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The first case corresponds to a 95-year-old Caucasian female patient who was referred to our hospital for eye redness in her right eye. Her medical history included hypertension and diabetes mellitus. For over 8 years she had been under ophthalmological treatment with artificial tears, autologous serum eye drops, and bandage contact lens due to continuous corneal epithelial defects as a result of neurotrophic keratopathy secondary to diabetes mellitus.\nAnterior segment slit lamp examination revealed conjunctival hyperemia, superficial punctate keratitis, and a positive-fluorescein-staining paracentral area of local corneal thinning one-by-two millimeters []. No signs of anterior chamber inflammation or infection were present and the patient referred no pain. A piece of twisted cotton was used to qualitatively examine corneal sensitivity, being difficult for the patient to tell if the cotton piece was touching or not her cornea and no blinking response was obtained.\nAs the corneal defect was not limited to the corneal epithelium, we considered that a surgical approach to mechanically cover the defect was needed in order to avoid perforation as medical treatment itself would fail to control the situation in the short term. However, a corneal transplant or conjunctival coating was not considered as the best options due to the patient's age, corneal neurotrophism, and the defect's location. We did not have access to amniotic membrane in that moment, so we decided to use Tachosil®. Using topical anesthesia, the Tachosil® sponge previously cut to the correct size, was placed over the corneal defect, and physiological saline solution was used to enhance the adherence of the collagen to the corneal tissue. It was then covered with a bandage contact lens for 24 hours in order for the patch not to move from place.\nThe treatment was repeated on three occasions spacing 3 days each patching. A layer of fibrin was formed over the corneal defect and beyond its margins and although a leukoma remained, the corneal thinning and therefore the perforation risk were corrected with no need of major surgical interventions [Fig. –]. Topical medication including autologous serum and artificial tears was continued. |
A 59-year-old male patient presented with ear pain and bleeding of the left ear, and upon closer investigation a reddish bulging mass extending through the left tympanic membrane from the middle ear was observed. The pure tone audiogram showed an 80-dB mixed hearing loss with an increased threshold of bone conduction in the high tone frequency range. The patient experienced no dizziness or facial palsy. The tympanum and mastoid were filled with an isodensity shadow indicating bone erosion, and the wall of the carotid artery canal and the jugular bulb appeared to be thick and erosive on CT (). The mass was close to the carotid artery and jugular bulb through the tympanum, and the mastoid space was enhanced in the early and late phases of the dynamic MRI. The enhanced mass also appeared on the underside of the promontory of the middle ear (). The patient had experienced a tympanomastoidectomy for tumors in the tympanum 7 years previously and the pathological diagnosis was adenoma of the middle ear.\nThe surgical findings revealed that a grayish-red tumor with a slight yellowish hue filled the mastoid. The upper construction of the stapes was conservative, although it was covered with granulation. We performed a canal wall-down mastoidectomy to expose the sigmoid sinus, which revealed the tumor mass close to the jugular bulb. The tumor had originated from the mucous membrane of the hypotympanum and progressed to destroy the bony portions of the posterior wall of the extra meatus through the underside of the cochlear promontory with communication between the hypotympanum and mastoid. There was bone erosion in the tympanic portion of the facial nerve canal, but no invasion to the facial nerve and jugular bulb was observed. Removal of the bony annulus and the residual tumors in the hypotympanum revealed the internal carotid artery with bony erosion, and the tumor was completely removed, sparing facial nerve.\nThe histopathological findings showed a solid sheet of homogenous cells, which was surrounded by a fibrous border. The tumor cells had round, oval, or slightly irregular nuclei with finely-dispersed chromatin, and occasionally formed glandular or tubular structures (Figures and ). They were typically positive for cytokeratin, chromogranin A, synaptophysin, and CD56, but were negative for S-100. The proliferative capacity of the tumor cells was assessed by observing the cells expressing the marker MIB-1, which is an antibody against antigen Ki-67. This was used to calculate the proliferation index for each tumor lesion by counting the total number of tumor cell nuclear profiles and the number of MIB-1-positive nuclear profiles in randomly and systematically selected fields. The first field in each tumor lesion was selected randomly, and the following fields were sampled systematically using a mesh []. The positive rate of MIB-1 was 6.6% (). The tumor was diagnosed as carcinoid tumor based on these pathological findings. |
A 27-year-old woman applied for a breast reshaping surgery for aesthetic purposes. There were no other complaints in her medical history. The patient underwent simultaneous mastopexy according to classical Lejour vertical scar technique and breast augmentation surgery using round silicone TSF - 415 mL implants under the pectoral muscle and a breast lift under general anesthesia (). During the operation proper hemostasis was achieved using electrocoagulation. Drains were removed next day after the operation with minimum serohemorrhaging fluid volumes. The later postoperative period was also uneventful.\nThe patient was discharged from the clinic on the second day after the surgery. Five weeks after the operation patient arrived at the clinic because of tenderness and swelling of the right breast. The patient stated that she had not sustained any traumas. During clinical examination, the upper right breast area was found to be significantly swollen and firm (). Ultrasound examination showed a 2.5 cm heterogeneous liquid strip accumulated around the implant (). The implant was intact. Complete blood count showed an increased amount of leukocytes, and red blood cells and hemoglobin were at the lower limit of the normal level.\nNo coagulopathies were found. The patient was taken to the operating room where she underwent revision surgery. The purpose was to remove the fluid and to find and stop the cause of its accumulation. During the operation (), a blood clot of 650 mL was removed (). Bleeding from one of the internal mammary artery branches in the implant pocket between the rib cage and the pectoral muscle lower pole was detected and stopped. After the revision, the implant was returned to the lodge. Vacuum drainage was used for one day only. One year after the surgery, there was no recurrence of bleeding, also no clinical evidence of the implant capsule contracture formation was found ().\nAll procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The patient gave her informed consent prior to her inclusion in this case report. Any details that might disclose the identity of the patient under study were excluded. |
An 84-year-old man was transported via emergency medical services to the emergency department (ED) following a motor vehicle collision. The patient was the restrained driver and was rear-ended while slowing down to make a turn. There was moderate damage to the rear of the patient's vehicle. The car was an old model vehicle and was not equipped with airbags. The patient was ambulatory at the scene but as a precaution he was transported via emergency medical services to the ED on a backboard and wearing a cervical collar.\nOn presentation, the patient was in no apparent distress and his vital signs were blood pressure of 157/78, pulse of 82 with a respiratory rate of 18 (unlabored), and pulse oximetry of 97% in room air. The patient initially complained of neck pain, bilateral upper extremity and shoulder pain, and numbness in both hands. A friend who accompanied the patient to the ED noted the patient's voice was raspy sounding. The patient's past medical history was significant for atrial fibrillation for which he took coumadin. He also had an automated implantable cardioverter defibrillator. The patient noted that his implanted defibrillator had not discharged today and he was in his normal state of health prior to the accident.\nHis physical exam was significant for midline cervical spine tenderness and his anterior neck exam was normal without tracheal deviation, jugular venous distention, or appreciable masses. The patient exhibited no neurological deficits except a complaint of bilateral hand numbness but his motor function was preserved and had full range of motion in all extremities. The patient had multiple abrasions over the anterior portion of both legs and over his right elbow. Laboratory studies revealed the patient had an INR of 3.5.\nA chest X-ray and plain film images of the patients shoulders were both normal without evidence of osseous injuries. An extended focused assessment with sonography for trauma was negative for pneumothorax and abdominal, pelvic, and pericardial free fluid. A computed tomography scan (CT) of the patient's cervical spine revealed an anterior C5 compression fracture with significant prevertebral tissue swelling and C2-C3 anterolisthesis. The fracture was causing an acute hematoma in the soft tissues to the right of the trachea and esophagus which extended inferiorly to the level of the right main bronchus (). After discussion with neurosurgery and radiology, the decision to further assess the compression fracture was made via lateral plain films (); note the patient could not undergo magnetic resonance imaging due to his implanted device.\nWhile in the ED, the patient developed worsening numbness in hands and worsening dysphonia, dysphagia, and difficulty managing his secretions. The decision was made to intubate the patient for potential airway compromise and what was surmised to be an expanding hematoma. Moreover, given the patient's progressively worsening situation, it was decided that the best option would be an awake fiber-optic intubation rather than direct laryngoscopy to minimize the risks of compressing an expanding hematoma or causing its rupture and thus airway compromise.\nThe patient was sedated with versed 2 mg IV and given 5 mL of 2% viscous lidocaine to drink. Further, 4% lidocaine in saline was atomized into his posterior oropharynx. The fiber-optic scope was passed and after some manipulation and copious suction of secretions which were pooling in the patient's pharynx, the vocal cords were visualized and an 8.0 mm endotracheal tube was passed through the cords without difficulty. Some bleeding was noted during procedure but acute hemorrhage or damage to the cervical hematoma was not evident.\nBecause the patient's INR was elevated at 3.5 and given his significant airway compromise, the patient was given four-factor prothrombin complex concentrates (PCC) and vitamin K which quickly reversed his hypercoagulable state. Repeat blood work 90 minutes after receiving PCC showed the patient's INR to be 1.3.\nSubsequent CT scan of his neck with intravenous contrast noted active contrast extravasation into the prevertebral soft tissues of the neck at the level of C4-5. This was in close proximity to the C4-C5 disc space to the right of midline and was likely stemming from a C5 compression fracture. No arterial feeder was noted and the hematoma was not increasing in size. The patient was later taken to the operating room for incision and drainage of the prevertebral space; however, no hematoma or fluid collection was found and only soft tissue edema was evident.\nThe patient was admitted to the intensive care unit for continued monitoring and extubated without incident after three days of observation. His oxygen saturation and vital signs remained stable throughout his stay. He was discharged from the hospital after 5 days of observation and has had no subsequent incidence including phonation, dysphagia, or breathing issues since discharge. |
A 50-year-old man was primarily operated on for extensive type B aortic dissection with acute bowel ischemia. The portion of the descending aorta from the origin of the left subclavian artery to just below the superior mesenteric artery was replaced by a tubular graft through an extended posterolateral thoracotomy. The patient recovered well; however, 10 years later, he was diagnosed with an aortic arch aneurysm and severe dilatation of the proximal descending aorta at the site of the anastomosis with descending tubular graft. Transverse thoracosternotomy (clamshell incision) was used to approach and replace the ascending aorta, the aortic arch, and the proximal part of the previous tubular graft in a single session as a re-do procedure. To facilitate the performance of the distal graft to graft anastomosis, the third and part of the fourth ribs on the left side were resected in addition to the transverse sternotomy. The sternotomy was closed with tension wires. Dehiscence of the transverse sternotomy developed during the early postoperative period. Two attempts of tension re-wiring followed by fixation with one-third tubular plate and conventional screws were performed to address the dehiscence, which all failed in a few weeks (Fig. a).\nThree years after the last unsuccessful revision surgery, the patient returned to our clinic symptomatic with pain and an unstable chest, with his lung herniating through the chest wall defect. Radiographs and a CT scan revealed an extensive chest deformation resulting from a widely displaced sternal nonunion, a bone defect of the sternum resulting from multiple operations, and the resections of the third and fourth ribs on the left side of the chest (Fig. b).\nThe patient was operated again by a team of cardiothoracic and orthopaedic trauma surgeons. An extensive adhesiolysis, the removal of the remaining wires, and debridement of the scar tissue were done. The sternal fragments were refreshed until the healthy bone and approximated, while the remaining 1 cm-large defect was filled with a structural iliac crest bone autograft. The fragments were then compressed by bone reduction clamps. The fixation was done using a titanium locking compression plate designed for the distal medial tibia (DepuySynthes, West Chester, Pennsylvania). The plate was intraoperatively shaped to match the markedly changed anatomy of the proximal sternum and fixed using 3.5 mm locking screws. The wider shape of the plate with multiple screw holes provided a good purchase in a smaller portion of the deformed proximal fragment. All the screws were carefully inserted under "finger control" as not to protrude at the back of the sternum. In the meantime, a 2 mm-thick, 20 × 10 cm Gore-Tex dual mesh membrane was attached to the ribs to cover the remaining defect caused by rib resection on the left side. The membrane was attached by using interrupted trans-costal sutures from heavy polypropylene. More than 60 sutures were placed along the two adjacent ribs above and below the defect to achieve the required strength. Another membrane of a 10 × 5 cm size was applied to the remaining soft tissue defect in the second intercostal space on the right side (Fig. c). The wound was then closed, the thoracic cavity was drained, and two drains were placed subcutaneously to prevent haematoma or a subsequent seroma formation.\nThe postoperative recovery of the patient was uneventful. The chest X-ray and the CT scan, performed 1 year after the operation showed the complete healing of the sternum and good positions of the membranes (Fig. ). After 3 years, the chest remained stable with no pain or lung herniation. There were also no implant-related problems and, therefore, no need for plate removal. |
Our patient is a 32-year-old African-American man with a history of T8M syndrome documented by chromosomal analyses at an outside hospital. His syndrome is characterized by dysmorphic facial features including saddle nose deformity and a large forehead as well as mild mental retardation. He presented to our clinic with complaints of right knee pain and inability to completely extend his right knee after injuring it several months ago. On examination of his right knee he was able to achieve full extension passively but was unable to actively perform a straight leg raise. On palpation, there was generalized tenderness and a high riding patella with a palpable gap beneath it consistent with a patellar tendon rupture. X-rays revealed marked patella alta with some mild HO in his distal quadriceps musculature (Figure ). Our patient was consented for right patellar tendon repair and possible excision of the HO.\nDuring the operative repair, his patellar tendon was found to be avulsed off the inferior pole of his patella. A repair was accomplished by weaving sutures through the patellar tendon and drill holes in his patella. Postoperatively, our patient was placed in a long leg cast. Our patient was not given any therapy for HO prophylaxis.\nPostoperative follow-up visits for the first six weeks revealed no obvious complications with proper wound healing and no complaints from our patient. At six weeks postoperatively, his cast was removed. Physical therapy was instituted at that time.\nFollow-up visits for the next three months demonstrated a decreasing range of motion of his right knee. X-rays taken three months postoperatively revealed extensive HO within his quadriceps muscles as well as the patellar tendon (Figure ). At four months postoperatively, our patient's knee was completely fused at 45 degrees. Despite the deteriorating range of motion, plantar and dorsiflexion remained intact. Sensation was intact and there was brisk capillary refill.\nAt this time our patient was given the option of leaving his knee locked at 45 degrees or performing a second surgery to fuse the knee in a more functional position. A total knee arthroplasty was not considered because our patient's quadriceps mechanism had ossified thereby eliminating active knee extension. After several additional opinions, our patient and his mother decided to proceed with a knee fusion.\nA second surgical procedure was undertaken. Compression arthrodesis of the knee was accomplished with an intramedullary interlocking nail from the hip to ankle (Stryker T-2 Fusion Nail System) after the distal femur and proximal tibia were transversely denuded of cartilage and subchondral bone. Images taken after the surgery revealed a successful procedure (Figure ). |
A 53-year-old man with history of liposarcoma had gallbladder tumor detected during routine ultrasonography () and was referred to us. He was first diagnosed as leg liposarcoma 5 years before and underwent complete resection. Despite of history of liposarcoma in his leg, he never developed any abdominal symptoms. Laboratory tests were unremarkable including carcinoembryonic antigen and carbohydrate antigen 19-9 (CA19-9) within their normal limits. Computed tomography (CT) scan demonstrated slightly enhanced 4-cm tumor located at gallbladder fundus (). On magnetic resonance imaging (MRI), the tumor had high signal in both of T2 and diffusion weighed image and low signal in both of T1 and apparent diffusion coefficient (). Based on these findings in imaging studies, our working differential diagnosis included liposarcoma and hemangioma rather than adenocarcinoma, and we recommended laparoscopic cholecystectomy. In the exploration, the tumor in the fundus appeared without serosal invasion (). Intraoperative ultrasonography revealed the tumor was contained in the gallbladder without infiltration into the liver, and in fact there was a distance between the liver bed and the tumor. Sonazoid enhanced ultrasonography ruled out liver metastasis and thus, we proceeded to laparoscopic cholecystectomy as planned. When the gallbladder was opened, yellowish white tumor was confirmed in the submucosal layer with the overlying mucosa intact (). Histopathological examination revealed, this tumor as myxoid liposarcoma consisting of lipoblasts and round cells. There was no necrosis and proliferations of spindle and round cell component were identified in the background of myxoid stroma (). These histological features were similar to the ones for the sarcoma in his leg that was resected before. The patient’s postoperative course was uncomplicated and was discharged home on Day 3. The adjuvant therapy was elected not to be given and he was placed on imaging surveillance with CT scan in every 3–6 months. In 16 months after this operation, he developed disease recurrence in the left popliteal fossa. There was no recurrence to date in the abdominal cavity at 28 months after the operation, he was alive with disease. |
An 80-year-old male patient was referred to the abdominal surgery department due to incarcerated ventral hernia and ileus. In the past he was operated due to perforated gastric ulcer. He also had arterial hypertension, chronic pulmonary obstructive disease and pulmonary hypertension, a history of smoking, he suffered an ishemic stroke in the past. He was urgently operated on the same day. Segmental resection of small bowel with end-to-end anastomosis was performed and the hernia defect was closed with direct sutures, without prosthetic mesh because the bowel was resected. There were no surgical or other complications after surgery and he was discharged from hospital after 8 days. 5 days later he was admitted to the hospital again due to early recurrence of ventral hernia. The content in hernia sac could however be reduced back to his abdomen. Laboratory findings showed leucocytosis and elevated C-reactive protein (CRP - 148 mg/l). Intestinal winding with a thickened wall up to 5 mm was found at the location of the ventral hernia by ultrasound examination. The patient underwent a second surgery 22 days after the first surgery due to obstructive ileus, which was seen on the abdominal computed tomography (CT) a day earlier. Due to additional diseases and disorders (ischemic stroke and insertion of stent in his left internal carotid artery in 2011, arterial hypertension, asthma, pulmonary fibrosis and hypertension, which were not properly treated, because the patient did not follow the prescribed treatment) the anaesthesiologist decided for the spinal anaesthesia, because the general anaesthesia would be to risky. The surgery was performed by an abdominal surgeon with 5 years experiences as a specialist and he performed more than 30 Rives-Stoppa ventral hernia repairs. The skin incision was made along the previous skin incision. In the subcutaneous tissue the small intestine was tightly adhered on to the skin. We managed to release it but unfortunately, a segment of the small intestine was damaged during adhesyolisis. Segmental resection of the damaged small bowel with end-to-end anastomosis was performed (). The small intestine was reduced back in to the abdominal cavity. Ventral hernia was repaired according to Rives-Stoppa technique with prosthetic mesh (). Other than postoperative tachycardia there were no reported issues. A couple of hours after the procedure apnoeic episodes appeared followed by unconsciousness. A computed tomographic angiography (CTA) of the brain vascular system was made and it showed a stenotic left vertebral artery (90% stenosis). Because of respiratory insufficiency and haemodynamic instability, the patient was transferred to the intensive care unit. Due to a worsening clinical condition, a CTA of the abdomen was preformed and an occlusion of superior mesenteric artery (SMA) was discovered. Interventional radiologist preformed an embolectomy and thrombus aspiration from the SMA with an insertion of a stent. The patient's condition continued to worsen so the abdominal surgeon decided for a “second look” abdominal exploration. At surgical revision we found a small intestinal and sigmoid colon gangrene. Because of the patients age, several other comorbidities and gangrene of the entire small bowel, the multidisciplinary team (abdominal surgeon, anaesthesiologist, intensivist) decided for conservative treatment. The patient died the day after surgery. |
This was a case report of a 30-year-old man who presented to the out-patient department of medicine with the complaints of itching and redness over the lower lip, right hand, left thigh and genitals. He had no other systemic complaints. Local examination revealed a red erythematous macule on the dorsal aspect of the right index finger and a similar lesion on the anterior aspect of the left thigh and the right corner of the lower lip [Figures and ]. Though he complained of itching over the genitals, no lesion was noted. General examination revealed normal vital signs, other systems being unremarkable. Patient had no notable past medical history. However, he had a clinical history of being prescribed oral clarithromycin for upper respiratory tract infection at a medical center 1 week back. Our patient denied consumption of any other drugs or of having a reaction to any drug in the past. At this point patient sought the opinion of a dermatologist and a diagnosis of lichen planus was made. He was treated with topical steroids and eventually the macules faded after 2 weeks leaving a violacceous pigmentation behind. At 4 months later, he again developed erythematous macules over the same sites mentioned above. This time patient had clinical history of consuming two tablets from the Helicobacter pylori Kit (H. pylori eradication therapy) as prescribed to him by a private practitioner. Within 2 h of consumption of the first-dose, he developed itching and redness over the same sites involved during the first episode. This time the patient suspected that the symptoms could have been caused by the drugs and visited us for a consult. Review of the contents of the H. pylori Kit revealed the presence of clarithromycin along with lansoprazole and amoxycillin. Further doses of the drugs were withheld and eventually the lesions faded away with hyperpigmentation within the next 2 weeks. An oral challenge test with the three drugs was conducted 4 weeks later, each drug of the three drugs being tested separately 2 weeks apart. Patient tolerated amoxycillin and lansoprazole without any adverse effects. However following challenge with oral clarithromycin, the patient developed similar skin changes 2 h after intake, hence supporting the diagnosis of FDE secondary to clarithromycin. Patient refused skin biopsy for further confirmation. We counseled him regarding the nature of the drug reaction and recommended avoidance of clarithromycin in future. |
An 86-year-old man on HD visited the emergency department (ED) of Okinawa Yaeyama Hospital with the complaint of worsening dyspnea. The symptom had started with mild shortness of breath on exertion several months ago and had gradually exacerbated. Given the frequency of dyspnea associated with volume overload and/or heart failure in HD patients, his doctor in the HD clinic assumed that the symptom was caused by heart failure associated with volume overload and attempted to empirically reduce his DW. The doctor failed to reduce his DW because of the frequent falling of the blood pressure during HD. Furthermore, while his dyspnea became worse, the doctor had to increase his DW by as much as 2 kg over 3 weeks to prevent blood pressure falling and leg cramping during HD. The patient visited our ED with the complaint of an acute worsening of dyspnea during the previous few days. He denied having chest pain, cough, sputum, orthopnea or paroxysmal nocturnal dyspnea, fever, chills, night sweats, or anorexia. He had a past medical history of end-stage renal disease (ESRD) due to unknown etiology and was on scheduled HD (4 h per day at 3 days per week) for 4 years with an uneventful course. He also had well-controlled essential hypertension and chronic atrial fibrillation which was treated by anticoagulation with warfarin for 4 years. He developed a hemorrhagic gastric ulcer that was probably associated with excessive anticoagulation 1 year ago. Helicobacter pylori was serologically negative at that time. Surgical repair of the left inguinal hernia was performed 10 years ago. He had no previous history of tuberculosis, pneumonia, or heart failure. He was not diabetic. He lived with his family. He worked as a construction worker up until several years ago. He smoked 2 packs of cigarettes a day for 30 years up until 30 years ago. He had no habit of regular alcohol consumption. Family history was noncontributory.\nOn physical examination, he was alert and appeared slightly sick. His body weight at the ED was 46.1 kg. His recent DW was 46.0 kg with an increase of 2 kg over 3 weeks. His blood pressure was 130/60 mm Hg, pulse was irregularly irregular with a rate of 50 beats per minute, respiration rate was 18 per minute, and body temperature was 37.4°C. Conjunctivae were pale. Cervical examination revealed nondistended jugular veins and no lymphadenopathy. Heart sounds were irregularly irregular without murmur or friction rubs. Chest auscultation revealed remarkably diminished breath sounds at the left thorax. A dull sound was heard at the left thorax on percussion. The abdomen was benign. There was no cyanosis, clubbing, or edema at the extremities. An arteriovenous fistula at his left forearm was unremarkable. Chest X-ray (CXR) showed massive left-sided pleural effusion without findings of pulmonary congestion or masses. (A CXR performed 7 months previous at the HD clinic showed no pleural effusion.) Immediate transthoracic echocardiogram showed no evidence of acute heart failure or volume overload. Laboratory findings are listed in table . Blood examination was remarkable for monocytosis (17.4%), hypoalbuminemia (2.5 mg/dl), elevated serum thyroid stimulating hormone over 10 μg/ml with positive anti-thyroid peroxidase antibody, and positive result for QuantiFERON-TB2G testing. Tumor marker testing revealed markedly elevated soluble interleukin-2 receptor. Thoracentesis with continuous pleural effusion drainage was immediately performed. The pleural effusion was grossly bloody and exudative with atypical cells, massive mesothelial pleocytosis, and elevation of lactate dehydrogenase (table ). The cytology of the effusion was class IV with strong suspicion of malignant lymphoma. Both fluorescent stain and Ziehl-Neelsen stain of the effusion detected no acid-fast bacilli. The culture of the effusion was negative. Computed tomography scans with contrast material of the neck, thorax, abdomen, and pelvis detected no findings suggesting a primary lesion of the malignant tumor or lymphadenopathy. Given the massive malignant pleural effusion at the unilateral side in the absence of solid tumor masses, we suspected PEL of the left thoracic cavity. We submitted cell surface marker testing and immunostaining for HHV8 of the effusion. CD45, CD38, and CD138 were dominantly detected as cell surface markers. HHV8 was detected by specific immunostaining. Enzyme-linked immunoassay (ELISA) for HIV was negative. Based on these results, we diagnosed the patient with PEL.\nThe patient's dyspnea subsided after the drainage. More than 6 liters of effusion were extracted over the first 3 days. We removed the chest tube on the 5th hospital day. We also prescribed 25 μg per day of levothyroxine for subclinical hypothyroidism due to Hashimoto thyroiditis, although the association with pleural effusion is unlikely. We notified his daughter of the diagnosis and prognosis and discussed his management. His family wanted no further invasive treatment or evaluation. With consideration of his age, underlying illness such as ESRD, the lack of established treatment for PEL, and the request from his family members, we gave up treating him with chemotherapy. As per the family's wishes, his daughter informed the patient of the diagnosis. We treated the patient with the goal of living at home with his family comfortably. He was discharged 2 weeks following his first admission. A CXR on the date of discharge revealed that there was no further accumulation of effusion. HD was continued at the HD clinic.\nThe patient was referred to our ED 1 month after the previous discharge with the complaint of dyspnea and left pleural effusion. After discontinuation of warfarin and administration of vitamin K, we performed another thoracic tube placement. Although he consequently developed re-expansion pulmonary edema (which presented as dyspnea with bilateral wheeze, crackles, and symmetric pulmonary edema on CXR) that required noninvasive positive pressure ventilation (NPPV) and bacteremia with Enterobacter cloacae treated with intravenous ceftriaxone (CTRX) for 14 days, his symptoms eventually subsided. Given the symptomatic accumulation of effusion after less than 1 month, we performed pleurodesis with 5 g of talc to the left thorax. He was discharged again after 1 month hospitalization.\nThree months following the second admission, he visited the ED due to general fatigue. He was evaluated for anemia (hemoglobin 7.6 g/dl) and was transfused with the diagnosis of mixed anemia of chronic disease due to PEL and renal anemia. His condition was fully improved with transfusion and rehabilitation and he was discharged after 10 days hospitalization.\nTwo weeks following the previous hospitalization, he was referred to the ED due to acute hypoxia following a productive cough for 2 days. We treated him with intravenous CTRX 1 g every 12 h, intravenous ciprofloxacin 200 mg every 12 h, intravenous nitroglycerin, NPPV, and emergent HD with the extracorporeal ultrafiltration method for clinical diagnosis of pneumonia combined with acute heart failure. Despite our intensive treatment, his condition rapidly deteriorated. Considering his underlying condition and the pain associated with treatment, we started intravenous morphine for palliation. He died peacefully the day after admission, 7 months after his diagnosis of PEL. |
A 69-year-old female reported to the department of oral pathology with the chief complaint of pain in the left side of the face for the past 1 month. History of presenting illness revealed a swelling which was initially small and gradually progressed 1 week before to the current state. Pain was sudden in onset, intermittent in nature, which aggravated on mastication and did not subside over medication (the patient was advised Combiflam tablet for 5 days, twice daily and intramuscular injection of cefixime for 3 days by physician whom she visited earlier). The patient revealed a past medical history of regular treatment for progressive SSc/lupus overlap syndrome, secondary Sjogren's with interstitial lung disease and PAH for the past 10 years. The patient was hospitalized for left lower limb cellulitis 8 years back and was discharged after complete observation and medication. Vitamin D insufficiency was suspected and corrected by calcirol granules.\nThe patient also had a complaint of difficulty in swallowing and spasm-type pain which was diagnosed as esophageal dysmotility and reflux disorder and was advised on prokinetic agents, low-fat diet and to avoid cold exposure to extremities. The patient also revealed a history of whitish skin lesion on both her legs and foot 20 years before these lesions have not progressed. The patient revealed no history of autoimmune disease in the family. The patient had undergone multiple uneventful extractions and complete prosthetic denture treatment. The patient also revealed a history of impaired taste sensation. On general examination, patient's vital signs were normal, and left submandibular lymph node was palpable, roughly oval, firm in consistency, tender and mobile.\nOn extraoral examination, facial asymmetry was seen in the left side of the face as a solitary diffuse swelling. The swelling extends from the middle third of the body of the mandible anteriorly to 1 cm behind the auricle posteriorly and superiorly at the level of the tragus to inferior border of the angle of the mandible inferiorly. Skin overlying the swelling appeared normal, with no other abnormalities detected []. Swelling was firm to hard in consistency, noncompressible, tender and warm on palpation.\nOn intraoral examination, inspection revealed inflamed parotid duct orifice on the left side and was tender on palpation. The presence of erythematous ulceration was seen at the right and left commissures of the lip suggestive of angular cheilitis []. A solitary ulcer was present in the lower labial vestibular region having erythematous margin with sloping border which was freely movable and tender on palpation suggestive of denture-induced traumatic ulcer. Hard-tissue examination revealed upper and lower completely edentulous arch.\nSalivary flow rate and pH were estimated to be 1 ml/min and 6.7, respectively. The quality of life of the patient was assessed using a questionnaire []. This self-evaluation was made to educate the patient about the impact of the oral dryness.\nPatient informed consent was obtained. Blood investigations were performed which were within normal limits. The patient was subjected to ultrasound examination which revealed minimal enlargement of left parotid gland with mild-to-moderate intraglandular ductal dilatation []. CT and MRI investigation revealed asymmetrical diffuse enlargement of left parotid gland with mild hyperintense signals, few specks of calcification and multiple branching and nonbranching cystic spaces. Mild irregular dilatation of the left parotid duct was elicited. Multiple enlarged submandibular level neck nodes were seen (largest – 2.5 cm × 1.1 cm, III level). Multiple calcific specks with few cystic spaces were seen in the superficial lobe of the right parotid gland with no significant duct dilatation [Figure and ].\nBased on the thorough history, clinical examination and investigations, the case was diagnosed as right chronic sialadenitis and left acute sialadenitis of parotid gland. The differential diagnosis considered was viral sialadenitis, pleomorphic adenoma and myositis ossificans. Ketorol DT 10 mg (analgesic) was prescribed for 1 week, and warm compression was advised to the patient following a review after 1 week the swelling reduced considerably (70%). Complete resolution was seen on the 2nd week. The patient was followed up for 2 months at an interval of 15 days. No recurrence was reported to date. |
An 11-year-old white girl with mixed dentition sought the stomatology outpatient clinic of our institution complaining of pain. The gums around permanent maxillary right and left incisors were compromised, including a purulent discharge and severe tooth mobility (). During anamnesis, there was no mentioning or suspicion of systemic and/or hereditary diseases, deleterious habits, or local injury.\nRadiography revealed severe vertical bone loss in both upper incisors (), which did not respond to electric or thermal pulp vitality tests. The diagnosis of an odontogenic abscess was ruled out due to the absence of trauma or caries in the teeth involved. However, the single location of the condition, its absence in posterior quadrants of the maxilla and/or mandible, its exudative appearance, and the radiographic demonstration of bone destruction were similar to the findings seen in Langerhans cell disease.\nTo our surprise, an elastic band involving the midportion of the roots of the two central incisors was found during biopsy (). When asked about the presence of the elastic band, the patient responded that she had obtained the band from the hair of her doll and had placed it around her teeth because a dentist had treated a friend with diastema in the same region using an elastic band.\nThe debris was removed and a metal wire was placed in permanent maxillary right and left incisors (). In addition, exfoliative cytology was performed which demonstrated a large number of neutrophils, macrophages, and red blood cells, few lymphocytes, sparse pavement epithelial cells with inflammatory alterations, filamentous mucus, and cell remnants. There were no signs of malignancy. The cytological diagnosis was class II Papanicolaou compatible with an acute inflammatory process.\nThe patient was followed up by clinical and radiographic examination, but no improvement in tooth mobility was observed. Bone loss increased and internal resorption and root exposure occurred. As a consequence, permanent maxillary right and left incisors were extracted after 2 months. The patient was referred for orthodontic treatment which consisted of a removable Hawley style retainer with an expansion screw in the middle for functional and esthetic improvement. |
We present the case of a 29-year-old male patient presented to our surgical clinic with a 5 month history of vague right upper quadrant pain. His background was significant for having undergone a radical pancreatico-duodenectomy (Whipple's procedure) 8 years earlier in another country for a pancreatic head islet cell tumor, which initially presented with recurrent episodes of hypoglycemia. At this time, histologic examination showed the tumor to be completely excised and his symptoms resolved completely. Follow-up scans postoperatively revealed 1 sub-centimeter lesion in the segment 6 of the liver, which was stable for 5 consecutive years and he attended no further follow-up thereafter.\nClinical examination and routine bloods were noncontributory; however a computed tomography abdomen revealed multiple low attenuation lesions in the right lobe of the liver, with no other obvious abnormalities. The main differential at this stage was metastatic disease. A double contrast magnetic resonance imaging (MRI) scan was subsequently performed, which revealed four distinct lesions []. The fat content and superparamagnetic iron oxide uptake were not specifically indicative of malignant tumors; however, considering the interval change in the size of the lesion in segment 6, the unusual nature of the suspected primary (insulinoma) and the vascular enhancement pattern of these lesions, the overall impression favored a diagnosis of four insulinoma metastases in the right lobe of the liver. An octreotide scan was performed, which failed to show any uptake but this was not surprising as not all tumors with somatostatin receptors will bind with octreotide. An ultrasound guided biopsy of one of these lesions was performed, which revealed a core of liver with mild fatty change only; no metastatic tumor was appreciated.\nGiven this patient's history, the nature of the lesions seen on MRI and the fact that one of the lesions had grown substantially since previous imaging, it was elected to proceed to formal resection. The liver parenchyma was transected to the right of the principle plain because of his previous Roux loop and satisfactory margins were achieved. An uneventful recovery ensued.\nHistologically [Figures , and ], these lesions were found to be hepatocellular adenomas. Each lesion was similar; unencapsulated, containing no portal tracts. The nonlesional parenchyma was normal without evidence of a co-existing glycogen storage disease. |
A currently 10 month old female had presented at the age of 10 days with multiple areas of purplish discolouration overlying an erythematous swollen base spreading extensively over her posterior trunk, proximal upper and proximal lower limbs. With a birth weight of 2.9 kg, she was a product of a full term unremarkable pregnancy and was delivered spontaneously through normal vaginal delivery to a healthy mother. However, her birth was complicated by hypothermia and perinatal asphyxia secondary to meconium aspiration. Her symptoms began when she was 10 days old and were associated with decreased oral intake, irritability and vomiting. Neither history of convulsions or loss of consciousness were present, nor the remaining review of her other systems remarkable for any abnormalities.\nOn examination, she was vitally stable, with multiple purple-red nodules noted bilaterally on the lateral and posterior aspects of the upper limbs extending through her back and towards the sacrum with involvement of the proximal thighs (). On palpation, the lesions were hot and tender to touch. The clinical picture was therefore consistent with subcutaneous fat necrosis. Her metabolic profile revealed hypercalcaemia with secondary hypoparathyroidism (). A renal ultrasound was ordered to evaluate the renal integrity and showed no presence of hydronephrosis or parenchymal calcifications. Additional brain ultrasound came back within normal as well.\nInitial protocol for the management of hypercalcaemia was instituted, with priority first allocated towards adequate patient hydration. Intravenous diuretics was initiated to achieve forced calcium diuresis but to no much improvement. Consequently, oral prednisolone was started at an initial dose of 2 mg/kg daily as indicated in the treatment of SCFN with normalisation of hypercalcaemia achieved within 2 weeks from presentation. On the other hand, her lesions took a comparatively and exceptionally prolonged clinical course, resolving after approximately 9 months of treatment. During the clinical course of the disease her steroid dosing was gradually tapered after the calcium level was restored to reference range and was continued on alternate days until the lesions were completely resolved. |
A 55-year-old Indian woman reported to the hospital with complaints of dysphagia for solid foods for more than one year which had progressively increased in severity. At presentation, she had also developed difficulty in swallowing liquids and had a history of regurgitation of food after meals. There was no history of cough or difficulty in breathing during meals, thus ruling out the possibility of a tracheoesophageal fistula.\nA thorough physical examination revealed a lump in the left breast of approximately 3 × 3 cm in size. The lump was hard in consistency with irregular margins, and it was not fixed to the skin or to underlying structures. Two firm, mobile ipsilateral axillary lymph nodes with mild tenderness could be palpated. Our patient did not have any family history of breast or ovarian carcinoma. She had breastfed all three of her children and had been postmenopausal for eight years. There was also no history of oral contraceptive pills or hormone replacement therapy.\nUpper gastrointestinal endoscopy revealed a friable, ulceronodular lesion at the gastroesophageal junction involving the juxta-esophageal fundus. Endoscopic biopsy of the lesion was carried out. Histopathological examination of the biopsy tissue showed moderately differentiated squamous cell carcinoma. A computed tomography (CT) scan of our patient's thorax and abdomen showed a soft tissue density space-occupying lesion in the distal esophagus and cardiac end of her stomach. The lesion was characterized as an irregular thickening of the wall with a widened lumen. The length of involvement was approximately 6 cm.\nThere was no proximal dilation of the esophagus. Evidence of aspiration was seen in the right basal segment of her lung. No atelectasis or pneumanitic lung tissue was seen. Significantly sized bronchopulmonary lymphadenopathy was seen bilaterally.. Anterior and superior mediastinal facial planes were preserved. No significant anteroposterior or superior-mediastinal lymph nodes were found. No pleural collection of fluid was seen. An increased attenuating lesion was seen in the left breast superior to the nipple, measuring approximately 24 mm in diameter. The margins of this lesion were not sharply defined, and no calcification was seen. No other lesions were found. Her liver was mildly enlarged with no focal lesions and a normal portal venous system and intra-hepatic biliary radicles. The porta hepatis was also free of lymph nodes.\nSignificant lymphadenopathy was seen along the celiac trunk and the lesser curvature of the stomach along the gastric artery. The para-aortic and para-caval regions were normal. The splenic hilum was also free of lymph nodes. All other findings were within normal limits.\nFine needle aspiration cytology from the lump of the left breast suggested an infiltrating ductal carcinoma. In view of our findings, a transhiatal esophagogastrectomy and a left-sided modified radical mastectomy with axillary dissection were carried out. Ten centimeters of the esophagus as well as 5 cm along the lesser and 3 cm along the greater curvature of the proximal stomach were removed. Grossly, the tumor involved the adventitia of the lower end of the esophagus and also the pericardial fat.\nMicroscopic analysis showed moderately differentiated squamous cell carcinoma type with a predominantly pseudo glandular pattern, involving the lower third of the esophagus, the cardioesophageal junction and the cardiac end of the stomach (Figure ). The tumor had invaded the muscular layer of the esophagus and extended to the adventitia. Perineural infiltration and lymphatic emboli were noted. Circumferential cut margins were free of tumor. The tumor had infiltrated the full thickness of the wall of the cardia of the stomach and had invaded the peri-gastric fat. Seven out of nine nodes along the lesser curvature showed metastasis with perinodal extension. One node along the greater curvature showed metastasis with perinodal extension. Esophageal cut margin and gastric cut margin were free of tumor.\nHistopathological analysis of the left modified radical mastectomy revealed the tumor to be an infiltrating ductal carcinoma grade II (Figure ). A comedo-type of ductal carcinoma in situ of nuclear grade II was noted within the same tumor, the content of which was not clinically significant. Areas of necrosis and tumor calcification were noted. No perineural infiltration and lymphovascular emboli were found.\nThe nipple, areola, skin and base were free of tumor. All the 14 lymph nodes we dissected were reactive. Immunohistochemistry of the operative specimens was performed. The breast tumor was negative for estrogen and progesterone receptors, but was positive for human epidermal growth factor receptor 2 (HER-2 neu).\nThree months after surgery, our patient complained of dysphagia. A CT scan revealed a recurrence of the carcinoma in her esophagus (Figure ). Our patient had received four cycles of docetaxel and Adriamycin (doxorubicin)-based chemotherapy before the recurrence of the esophageal growth. As our patient was HER-2neu positive, a taxane-based regimen was planned, considering that the single agent docetaxel has previously been effective in treating esophageal carcinomas. Our patient was not prescribed Herceptin (trastuzumab) as she could not afford the drug. Instead, she was treated with locoregional radiation therapy for the recurrence of the carcinoma in her esophagus, taking care with the dosage delivered to the heart. She was given a total dose of 65Gy in 32 fractions over seven weeks. Initially, 40Gy was given by anterioposterior-posterioanterior fields. The remaining dose was delivered by three oblique fields. The remaining two cycles of chemotherapy were given after completion of the radiation treatment. Our patient is currently symptom-free and doing well one year and eight months after completion of the treatment. She had follow-up clinical examination monthly for one year. An upper gastrointestinal endoscopy and a CT scan of her thorax were performed every three months. Currently, our patient has follow-up appointments every two months and is advised to have six-monthly investigations. |
A 37-year-old male underwent a simple orthodontic preprosthetic treatment, after signing informed consent, with the aim to correct a scissor-bite between the upper second molar and the lower second molar on the right side (Figure ).\nA general dentist placed a buccal orthodontic button in the centre of the crown of the upper second molar and another button on the lower second molar lingual surface, and then instructed the patient to wear an orthodontic elastic to correct the scissor bite. The patient had to wear the elastic all day, except during meals and oral hygiene.\nThe patient was recalled monthly in order to check tooth movements, but after a few months he experienced discomfort in the gum at the level of the second lower molar, where the rubber band was applied. During a clinical examination, slight mobility of the lower molar was observed, which was attributed to the orthodontic force application, even if in presence of augmented periodontal probing (7 mm). A periapical radiograph revealed the presence of a small periodontal angular defect mesial to the molar, in absence of foreign bodies or tartar (Figure ). The elastic use was immediately suspended and the lingual button removed, in order to avoid any interference with oral hygiene maintenance. After consultation with a periodontist, subgingival root planning under anaesthesia, followed by chlorhexidine gel insertion and a systemic antibiotic therapy, was performed. The patient was than recalled one week later, at which point no improvement was found, but rather a significant mobility increase was noted. Periodontal probing was 12 mm all around the tooth. In a periapical radiograph, was obtained and worsening of the mesial angular defect was observed, affecting the root length (Figure ). The dentist then decided to extract the second molar.\nDuring the extraction procedure, the dentist found an intraoral elastic around the root, in proximity to the apex (Figure ). It was not possible to visualize this elastic in the radiological exams because this type of elastic is, unlike separator elastics, not radiopaque. After treatment failure, considering that the second upper molar was very buccally inclined and that the patient did not want to undergo a complete orthodontic treatment, the dentist decided to extract the molar to avoid occlusal interference and accumulation of food between 16 and 17, with the risk of upper first molar loss (Figure ).\nThree years later, a good bone level was noticed around the implant and the teeth adjacent to the 2nd molars extraction sites (Figure ). |
A 77-year-old man with a 2-month history of right shoulder and upper abdominal pain visited his physician at another hospital. A gallbladder tumor, about 3 cm in diameter, was detected by ultrasonography, and the patient was then referred to our hospital for further diagnosis and treatment. His laboratory findings at presentation were nearly normal, including the serum levels of tumor makers. Computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen showed the mass to be located at the cervix of the gallbladder and to involve the common and right hepatic arteries, with enlargement of the No. 12 lymph node (fig. ). A percutaneous biopsy of the tumor revealed an adenocarcinoma, and immunohistochemical analysis of the tumor showed positive staining for carcinoembryonic antigen (CEA) and cytokeratin 7, and negative staining for cytokeratin 20. Thus, the tumor was diagnosed as an unresectable gallbladder cancer.\nThe patient was initiated on treatment with gemcitabine at a dose of 1,000 mg/m2 administered on days 1, 8, and 15 of each 4-weekly cycle. After 13 cycles of treatment, a marked decrease of the tumor size on CT was noted (fig. ), and the treatment response was rated as partial response according to the Response Evaluation Criteria in Solid Tumors (RECIST) criteria, version 1.0. After 15 cycles of treatment, the tumor disappeared altogether, and CR was confirmed by CT after 20 cycles of gemcitabine monotherapy (fig. ). After CR had been achieved, the tumor was reevaluated for possible surgical resection by our surgeon. However, the patient was judged to be an unsuitable candidate for surgery, because he had severe bronchial asthma. Therefore, gemcitabine monotherapy was continued. After 27 cycles of treatment, corresponding to a further 12 months of treatment after CR had been confirmed, we held a discussion with the patient and his family about whether or not they would like the gemcitabine therapy to be continued. They made the decision for the gemcitabine therapy to be continued, for fear that the tumor might relapse. At this time, because of thrombocytopenia, the administration schedule of gemcitabine was changed to 3-weekly cycles, with the drug administered at the same dose as previously, but on days 1 and 8, followed by 1 week's rest. From the 40th cycle onward, the administration schedule was changed again to biweekly cycles, with no reduction of the drug dose, because of grade 1 malaise.\nAfter the completion of 5 years of treatment with gemcitabine, we again held discussions with the patient and his family and decided to discontinue the gemcitabine therapy, because the patient had remained relapse free for 45 months. Gemcitabine had been administered a total of 160 times, over 60 cycles, for 5 years, while the drug toxicities had remained rather mild in nature throughout the course of treatment, consisting only of grade 2 leukopenia and thrombocytopenia and grade 1 malaise.\nHowever, 11 months after treatment discontinuation, the patient presented with signs of obstructive jaundice. Brushing cytology of the bile duct by endoscopic retrograde cholangiopancreatography revealed an adenocarcinoma. MRI showed relapse of the tumor at the porta hepatis (fig. ). The metastases to the lymph nodes invaded the celiac artery and common hepatic artery. Thus, the patient was diagnosed as having unresectable relapse of gallbladder cancer, and gemcitabine therapy was restarted. Six months later, the tumors showed an increase in size and multiple liver metastases appeared. The second term of gemcitabine therapy was less effective against the disease as compared to the first, although the best response was stable disease. At the time this case report was written, the patient was receiving S-1 therapy and has remained alive for 79 months after the commencement of the first gemcitabine therapy. Serial changes of the serum levels of carbohydrate antigen 19-9 and CEA during the entire treatment are shown in fig. . |
A 65-year-old lady presented with complaints of blackening of third, fourth, and fifth fingers and gangrenous skin patch over the dorsum of right hand for the last 1 week, associated with tingling and numbness over the right hand and fingers. There was history of mild pain over the right hand that was intermittent and more marked when the patient performed manual work. Pain was occasionally associated with cold sensation of the right upper extremity. There was no previous history of such episodes in the past. There was no history of upper limb swelling or Raynaud's phenomenon in cold weather. The patient was a nonsmoker and a nonalcoholic. She was a diabetic on oral hypoglycemic agents. She was previously operated elsewhere for modified radical mastectomy malignancy on the right side 5 years ago, followed by 6 cycles of chemotherapy with no prior history of radiation therapy postmastectomy. No significant family history could be found.\nOn clinical examination, vitals were normal with no palpable pulsations in right radial and ulnar arteries; however, the brachial artery was palpable. No abnormal findings were noted in the rest of the arterial system and all the remaining pulsations were palpable. There was no suffusion of face or any dilated and engorged neck veins. Capillary refill was delayed in the right hand. Physical examination did not show evidence of any locally tender areas or neck masses. There was no evidence of muscular atrophy of the right upper extremity; however, there was mottling of skin over palmar aspect of ring finger and dry gangrenous changes on tips of third, fourth, and fifth digits. A complete neurological examination did not reveal any local motors or sensory deficits.\nPlain radiograph of the chest showed a cervical rib on the right side. Computed tomography scan (CT) of the neck confirmed right-sided cervical rib arising from the transverse process of C7 vertebra with fusion of the transverse process of C6 vertebrae that was projecting down. Right upper limb arterial Doppler showed an acute to subacute complete lumen occluding thrombus in the right ulnar artery, as well as in the right distal radial artery with good collateral to maintain distal flow. CT angiography of the right upper limb was suggestive of a focal thrombosis of the subclavian artery at the thoracic outlet in costoclavicular space due to extrinsic compression by the cervical rib. There was no evidence of any aneurysm or poststenotic dilatation.\nGangrene of the lateral fingers with low grade infection warranted local amputation; however, without treating the etiology, the expected result would have been unfavorable. Hence, initially patient was treated with antibiotics with analgesics. The patient received anticoagulation as per our institutional protocol. Patient was initially started on heparin for which was overlapped with warfarin for 5 days, followed by discontinuation of heparin after day 5. Patient was initially started on heparin which was overlapped with warfarin for 5 days, followed by discontinuation of heparin after 5 days. After optimization, she was operated for excision of the cervical rib through the supraclavicular approach (\n).\nA supraclavicular exploration was planned for cervical rib excision.\nUnder general anesthesia, the patient was positioned supine with an underlying shoulder bump for neck extension and head end was elevated to 30 degrees.\nWith the neck extended and turned to left side, a transverse skin incision was taken 2 cm above and parallel to the clavicle.\nSubplatysmal flaps were created and sternocleidomastoid muscle was retracted medially to identify anterior scalene fat pad. Fat pad opened and retracted laterally; inferior belly of omohyoid was identified and cut to visualize the thoracic outlet (\n).\nPhrenic nerve was identified and retracted medially along with the internal jugular vein to expose the belly and tendon of scalenus anticus.\nScalenus anticus was cut near its insertion after lifting with Mixter forceps and subclavian artery was identified.\nNeurolysis of the brachial plexus was performed to separate the cords from scalenus medius on the lateral side.\nLong thoracic nerve of bell was identified on posterolateral aspect of scalenus medius and secured by lateral retraction. Scalenus medius was cut near the first rib.\nThe cervical rib along with the long transverse process of the sixth vertebra was identified by palpation and osteotomy was performed using hammer and chisel. Bone fragments were excised followed by hemostasis.\nHemostasis was checked, and wound was closed with suction drain in situ. Ray's amputation of the right fourth finger along with debridement of the gangrenous skin on the right hand (\nand\nPatient was started on orals after 6 hours and mobilized. There was no weakness, loss of sensation, or winging of scapula on the right side. She was maintained on oral anticoagulation for 6 weeks and discharged on day 3 of the surgery. Arterial Doppler done after a week and 6 weeks showed radial artery biphasic pattern. Patient was continued on oral anticoagulation and was discharged on warfarin after ensuring the international normalized ratio is in therapeutic range. The wound on the hand healed with no progression of the gangrene at 3 months follow-up. At follow-up of 6 months, there was complete recanalization of the subclavian artery lumen on arterial Doppler (\n). |
A 19-year-old boy presented to us for evaluation with the complaints of easy fatigability. He was a known case of congenital heart disease with patent ductus arteriosus and had undergone device closure for the same at 4 years of age. He was a diagnosed as a case of CEPS at the age of 13 years, when he underwent preanesthetic workup, to repair an accidental tibial fracture, which revealed transaminitis and subsequent ultrasound abdomen revealed the presence of CEPS. Echocardiography done revealed no residual patent ductus arteriosus with device in situ. There was significant right atrial and ventricular dilatation with estimated high pulmonary arterial pressure (estimated right ventricular systolic pressure of 90 mmHg) with preserved right ventricular function. Presence of high pulmonary arterial pressures which was initially attributed to late closure of patent ductus arteriosus could be contributed by the presence of high-flow extrahepatic portosystemic shunt, so he underwent CT of the abdomen to determine the type of shunt. CT demonstrated the presence of Type 2 Abernethy malformation with large window type communication between the portal vein and IVC []. He was subsequently planned for angiography to assess hemodynamics as well as possible closure of communication. Hemodynamics data revealed pulmonary arterial pressure of 75/43 with the mean of 50 mmHg against systemic pressures of 111/64 with the mean of 80 mmHg. Before attempting to close the extrahepatic portosystemic communication, the mean portal venous pressure was documented to be 13 mmHg with no significant increase postballoon occlusion. Angiography revealed large window type communication with IVC [], so it was decided to use covered stent in the IVC to close the communication. A self-expandable aortic stent graft (Endurant II 36 mm × 36 mm × 49 mm – Medtronic) was used to close the communication. Poststent implantation, there was complete closure of the communication. Within 24 h of stent graft placement, ultrasound of the abdomen was done which revealed collapse of the stent graft and resultant reopening of the communication between the portal vein and IVC. He was again taken to catheterization laboratory and high-pressure balloon dilatation of the collapsed stent graft was attempted which was not successful. He then underwent a stent in stent implantation and a bare Andra Stent size 43 mm mounted on 18 mm × 5 cm Z-Med balloon after 1 month []. This time there was no flow seen across the communication. At last follow-up, after 3 months, the patient is symptomatically improved with echocardiogram showing preserved ventricular function and estimated right ventricular systolic pressure of 52 mmHg. He is planned for recatheterization in 6 months to assess the impact of closure of extrahepatic shunt on pulmonary hemodynamics. |
A 58-year-old woman presented with acute inflammatory features in the right iliac fossa. An ultrasound showed a small collection of fluid and debris in the free peritoneal cavity in the right iliac fossa with a thickened tubular structure, probably the perforated appendix. There was also a note in the ultrasound scan about mild pelvicalyceal dilatation of the right kidney. After discussing with the urologist, we proceeded with an emergency diagnostic laparoscopy and found a perforated appendix with abscess, inflammatory exudate and adhesions in the right iliac fossa []. By suction dissection, the appendix was freed from the surrounding structures and an appendectomy was performed. The peritoneal cavity was thoroughly washed out with normal saline and a drain was kept through the suprapubic port. The immediate postoperative period was uneventful. At follow-up after 2 weeks, she had right loin pain; a repeat ultrasound was taken, as advised earlier by the urologist, which showed more significantly dilated ureter accompanying the hydronephrosis. This dilated ureter was traceable up to the terminal portion about 2.5-3 cm proximal to the ureterovesical junction. There was no free fluid in the peritoneal cavity. We sent the patient for contrast-enhanced computed tomography (CT) scan, which showed diffuse inflammatory changes of the retroperitoneum on the right side. The dilatation of the ureter was confirmed up to its terminal portion, and there was no stone or intraluminal pathology obviously seen on the CT []. A thickening of the ureter was also noticed in relation to the terminal ileum and the appendicular area, and it was suggested that it was likely to be an inflammatory stricture of the right ureter [].\nAs the dilatation of the ureter had increased compared to the time of surgery and as the patient was quite symptomatic with right loin pain, we proceeded to decompress the dilated collecting system with an immediate ureterorenoscopy (URS), which showed narrowing of the distal ureter due to extrinsic pathology; hence, stenting of the right ureter was performed. This was duly performed within 24 h of the contrast-enhanced computed tomography (CECT) and the patient was discharged on the following day. At the follow-up 4 weeks after the stenting, she was found to have no symptom of right iliac fossa or loin pain. A follow-up ultrasound showed no dilatation of the ureter or the collecting system. The stent was removed after 6 weeks following which the patient has remained asymptomatic at 30 days follow-up. |
A 59 year-old female patient, who has been employed as rehabilitation worker, has observed gradually enlarging formations under both her right and left scapula for approximately eight months. In anteflexion, elevation of the upper extremities and when stretching the arms forward, swellings reaching up to the rear axillary lines appear bilaterally subscapularly. They were of soft consistency at palpation. The patient also described pain in the upper extremities, and in the region of arms. She had no recollection of any accident or fall. However, she had undergone neurosurgical operating procedures of disc extrusion in the cervical and thoracic spine, and the findings of bilateral resistances were present already pre-operatively. In the another surgical workplace repeated punctures and partial resection of the swelling on the right side were implemented 5 months ago, and it came to its subsequent recurrence.\nThe magnetic resonance ((b) and (c)) on thoracic wall showed in dorsolateral parts in subscapular regions in the level of 3rd to 7th rib symmetrical limited fluid collections with dimensions of 120 × 37 x 115 mm on the right side with a volume of 250 ml and on the left side 120 × 24 x 90 mm with a volume of 130 ml. The collections were localised in the intermuscular spaces between the external intercostal muscles and the heads of the muscle serratus anterior. The contents of collections were moderately heterogeneous with sporadic internal septa. Cystic formations had slightly distinct signal, native image in T1 weighing displayed hypersensitive contents on the right side. It could be a case of chronic post haemorrhagic changes. Postcontrastly the collections were without amplification of signal intensity. On the left side postcontrastly there was present a moderate reinforcement of capsule of fluid collection. In diffuse weighing the lesions were without marks of diffusion restriction. Axillary lymphatic nodes were of physiological size, the displayed pulmonary parenchyma was without inflammatory and focal changes, without mediastinal and hilar lymphadenopathy, the pleural cavities without effusion, the pleura was without hypertrophy, the recorded skeleton was without traumatic change.\nOwing to progressing swelling and increasing difficulties a surgical resection was indicated in the female patient. She was operated on under general anaesthesia, and a resection of the encapsulated collections of fluid was implemented bilaterally ((d)), two Redon drains were introduced. In the left collection serous fluid was present, on the right side also serous fluid with admixture of old blood was present. A histological examination of cystic collections proved that it concerned pseudocystic lesions with relation to subscapular bursa without marks of malignancy. Their walls were created by collagenous, hyalinised and vascularised connective tissue with predominately perivascular nonspecific chronic inflammatory cellulation ((a)), the internal surface of which was lined by a layer of fibrin and by a nonspecific granulation tissue with a focally accentuated xanthogranulomatous, siderophagous and giant-cell reaction without epithelium ((b)). In the lumen of the cysts there were remnants of blood clots with fibrinous or fibrinoid substances with dispersive admixture of siderophages, lymphocytes, neutrophils and giant polynuclear cells ((c)). The proof of amyloid by Congo red was negative. On the lesion periphery soft-tissue structures were caught, including striated muscularis. The drains were removed the 10th postoperative day due to higher production, the surgical wounds were healed-up per primam intentionem. After the operation the female patient had a full range of movements and was without trouble and pains. |
A 49-year old asymptomatic male was admitted to our institution after referral from his cardiologist in an outpatient care clinic, where he was monitored because of a dilated aortic root (46 mm). This was an incidental finding by transthoracic echocardiography (TTE) 6 months earlier at presentation due to non-specific chest pain. Except for hypertension, the patient had no other history of disease. Now 6 months later, the most recent TTE did not only show an increase in the aortic root diameter (50 mm), but also a hypo-echogenic structure in the right ventricular cavity (). The latter was interpreted as a right ventricular thrombus, position-wise possibly related to the tricuspid valve. Moreover, because of the rapid increase of the aortic diameter (4 mm in 6 months), the patient was referred to our hospital where a regular non-gated computed tomography angiography (CTA) scan was performed for acute aortic pathology. At the initial evaluation of the scan there was no suspicion of acute aortic pathology besides the dilation. After this CTA, the patient was discharged home with the intention to perform magnetic resonance imaging (MRI) a week later to determine the nature of the right ventricular structure. However, upon review of the CTA the next morning there was a suspicion of aortic dissection, based upon a possible tear in the aortic root. The patient was readmitted urgently for a new and dedicated electrocardiogram-gated (ECG-gated) cardiac computed tomography angiography (CCTA) scan () on which a structure of hypodense tissue was observed to derive from the sinus of Valsalva of the NCC bulging into the right ventricular cavity with involvement of the perimembranous septum. This suggested it to be a SVA filled with thrombus. As there was no continuation of contrast from the aortic root to the right ventricular cavity, a rupture was highly unlikely. However, given the potential risk for rupture with the rapid increase in aortic diameter and the thrombus suggesting the possibility of bleeding, the patient was referred for urgent cardiac surgery.\nDuring surgery, a SVA of the NCC bulging to the right ventricular cavity was observed (). There was no thrombus inside the aneurysm. As the native aortic valve was tricuspid and functioned well, a valve-sparing root replacement was performed. Histopathological analysis of the excised aortic tissue showed fragmentation of the elastic lamina in line with aortic media degeneration and no signs of connective tissue disease. This fits the general known cause of SVAs. There were no relevant complications in the postoperative setting and the patient was discharged 7 days after surgery. |
A 12-year-old boy came to us in emergency department with an injury to his left leg in the form of a direct impact to the anterior aspect of his proximal leg from an oncoming biker who was driving on the wrong side of the road. He was not able to put weight on the injured limb and had to be carried to the hospital.\nExamination showed a gross swelling in his knee region and posterior sag in his left leg, with a visible step distal to the joint line. There were some bruises and marked tenderness was present in the proximal tibial region. The calf had no tenderness and was soft on palpation; however, the periphery was relatively cold with absent peripheral pulses and, delayed pin prick, and capillary refilling time. He did not have any motor or sensory deficit in the limb.\nRadiographs revealed SH I proximal tibial physeal injury with posteriorly displaced tibial metaphysis (). Doppler sonography revealed a monophasic flow in the anterior tibial, posterior tibial, and dorsalis pedis arteries. The flow was normal above the level of the injury.\nHe was shifted to the operating room within 45 min of presentation. Under general anesthesia, in supine position, the fracture was reduced in a closed manner with traction and extension followed by flexion at the fracture site. Care was taken not to hyperextend or hyperflex the limb at any time during the maneuver. The reduction was held with two thick smooth K-wires in a crossed configuration (). The patient’s peripheral pulses had returned by the time the fixation was completed and hence the circulation was restored. Pin prick bleeding and capillary refill time returned back to normal. This was confirmed with a portable Doppler which showed biphasic flow in the peripheral arteries.\nPatient’s knee was immobilized in an above knee plaster cast. The post-operative period was uneventful except that the patient developed some blisters in the proximal leg on the 3rd postoperative day; however, there were no signs of any neurovascular compromise. The blisters were punctured, painted with Povidone-iodine and dressed in a dry manner. The skin healed well in a few days and the patient was discharged. The cast was removed at 3 weeks following which knee range of motion was started and the K-wires were removed at 6 weeks. Weight bearing was allowed at 3 months and gradually progressed as tolerable. Radiological healing was seen at 4 months (). At the last follow-up of 1 year, the patient had a good knee range of motion (ROM), no limb length discrepancy or deformity and was able to squat and sit cross legged without any difficulty. |
A 12-year-old woman presented with a painless swelling in the distal aspect of the right humerus of 1 month's duration. She did not report any trauma to the arm. Physical examination revealed a firm, nontender swelling deep to the triceps just above the elbow. The function of the right upper limb was normal, with a full range of movements in all joints and without distal neurovascular deficit and no regional lymphadenopathy.\nPlain X-rays showed a dense sclerotic bony protuberance arising from the posterior cortex of the distal aspect of the humerus (). The MRI showed an exostosis measuring 6 cm in its long axis terminating immediately proximally to the level of the olecranon and with involvement of the underlying medulla (). Chest CT scan and whole body bone scan confirmed no evidence of disease elsewhere. A core needle biopsy was performed which showed features consistent with a parosteal osteosarcoma.\nReconstructing a segmental defect at this site is fraught with difficulties. Although we have reported good results with distal humerus replacements [], we anticipated long term complications such as aseptic loosening using this option in a 12-year-old. Reconstruction with bone transport was not attractive because of the length of time that would have been required and the associated risk of elbow stiffness. Reconstruction with strut allografts requires an adequate bone banking service and presents inherent risks of graft rejection and imperfect fit of the donated graft to the recipient bone defect. Therefore we planned to do an en bloc resection of tumor bearing bone, extracorporeal irradiation of excised bone segment to eradicate the tumoral cells, and reimplantation of the autograft with preservation of the elbow joint. We resected 10 cm of the humerus, stopping 2 cm from the elbow joint and dividing the bone a further 10 cm proximal to this. We then stripped the tumor from the bone surface and underlying intramedullary canal using osteotomes, curettes, and a burr. Samples were sent for histology and microbiology. The humerus segment was wrapped in a sterile moist swab immersed in saline containing 2 grams of vancomycin and placed in a sterile bag. This was placed in another sterile bag and was securely packed in a sterile container. This container was taken to the radiotherapy suite, where the segment bone was irradiated with 90 Gy, and the container was returned to the operation theater. Transport and irradiation took about one hour, during which time the host bone was prepared for reconstruction. The irradiated humeral segment was reimplanted, inserting a nonvascularised fibular autograft through the middle of it to provide extra stability (Figures and ). The construct was held in place with 2 plates running up each side of the humerus (Figures and ).\nThe patient started mobilization exercises of the elbow from the third postoperative day. On the fifth postoperative day, the day of his discharge from our hospital, the range of motion of his elbow was 0–90 degrees. Six weeks after surgery, she had flexion of 0–120 degrees. About 4 months after surgery, she regained full flexion and extension of the elbow and full pronation and supination of the forearm. Elbow X-ray showed excellent union at both ends of the graft. At last follow-up ten years after surgery, she continues to have excellent function with full range of movement and can play sports without problems. X-ray of the right humerus showed full healing and consolidation of the graft (). The patient had no recurrence of the tumour. |
A 5-year-old girl was presented with a lump and pain in her right knee. The patient was with a history of falling from a bike two months prior to the admission with no significant injury related to the fall. General examination was within normal limit. The patient complains of unexplain loss of weight. Pain was felt out of proportion and worsen at night.\nPhysical examination of the right knee showed a solid circumferential mass (diameter 30 cm, contralateral 26 cm) on the proximal tibia (). The range of motion of the right knee is limited to 120° compared to 140° in the contralateral side due to the mass and pain. A plain radiograph was obtained which showed an osteoblastic lesion on the proximal tibia with a discrete margin and narrow transition zone. The cortex of the bone was intact with no periosteal reaction (). The margin of the lesion was further described on magnetic resonance imaging (MRI) with contrast (). A computed tomography scan of the chest showed no metastatic nodules. Histopathological and immunochemistry examination confirmed the diagnosis of osteosarcoma with a high degree giant cell-rich subtype ().\nThree cycles of neoadjuvant therapy regiment with cisplatin, ifosfamide, and adriamycin were administered to the patient. Multidisciplinary approach surgery with plastic surgeon and cardio thoracic vascular surgeon was conducted in this patient.\nThe surgery was performed with a torniquet in a supine position with an anteromedial approach on the proximal tibia. The popliteal bundle was then identified and preserved. Ligation and anastomosis of the vein involved with the tumor were performed prior to wide excision. Wide excision was performed with the respect to preserve the epiphyseal plate proximally and 13 cm of the epiphyseal plate distally. The proximal tibia segment was clear from the tumor with osteotome and bone curette ()\nThe proximal tibia segment was recycled using liquid nitrogen for 40 min. The recycled autograft was left to be thawed at room temperature for 15 min and soaked in 160 mg gentamycin saline for 5 min (). The recycled autograft was re-implanted and fixed with a locking 2.7 mm T-plate and a straight reconstruction 2.7 mm reconstruction plate (Heng Jie, China). The bone defect on the medial side was filled with a vascularized fibular graft which was harvested by prior to wide excision. The remaining bone defect was filled with bone graft and bone substitutes. A medial hemigastrocnemius flap was used for soft tissue coverage.\nPostoperatively we performed an X-ray to evaluate the surgery. From the X-ray, the plate and screws are well-fixated (). Postoperative histopathological examination confirmed the diagnosis of conventional osteosarcoma HUVOS 1 with free tumor margin.\nPostoperative condition showed good surgical and functional outcomes. Postoperative complications did not occur in this patient. Radiological union was achieved in 1 year followup with the ROM of 120° in the affected knee. Functional outcome was calculated with musculoskeletal society tumor score (MSTS) with the result of 76,6%. |
In September 2020, a 39 year-old man was admitted to Shanghai Ruijin Hospital complaining of a serious discharge from a small opening on his left flank. It had been occurring for 2 months without fever or pain. His medical history evidenced a laparoscopic left partial nephrectomy for clear cell renal cell carcinoma (ccRCC) in December 2015. Three weeks post-surgery, he had developed purulent discharge from the drainage tube orifice on his left flank. Seven months later, after three rounds of debridement and percutaneous drainage procedures, the wound was healed. There was no report of previous tuberculosis or pyelonephritis. Physical examination revealed that the fistula covered an area of 0.5 x 0.5 cm, and 6 cm deep on his left flank without tenderness or percussed pain (). Laboratory tests including renal function tests and urinalysis showed no unusual results. Urine culture and secretion culture were negative. To rule out tuberculosis, we conducted a fluorescent quantitative PCR test of the secretions from the fistula, which was also unremarkable.\nRenal dynamic imaging revealed normal function of the left kidney. Preoperative abdominal CT showed the sinus tract, extending from the surface of left kidney near the lower upper pole to the skin without renal/ureteral stone or hydronephrosis (). The three-dimensional reconstruction clearly showed the relationship between the sinus tract and the kidney (). Next, we performed a fistulous tract endoscopy, which showed a mass of white flocculus stuck to the lining of the sinus tract (). Given the frequent recurrence and complexity of NCF, we planned to repair the fistula and prevent relapse using complete sinus tract resection combined with omental flap grafting.\nSpecific steps in the surgical operation were as follows:\nComplete excision of the sinus tract under the guidance of methylene blue staining. The bottom of the sinus tract was stuck to the lower pole of the left kidney ().\nWe found a substantial amount of hard tissue similar to calcification, and a hemoclip at the lower pole of the left kidney. We removed all the abnormal tissue and hemostasis ().\nWe opened the posterior peritoneum and pulled part of the greater omentum tissue into the retroperitoneal space with oval forceps. Then, we tightly covered the left kidney with the omental flap and fixed it with sutures ().\nWe sutured the posterior peritoneum with the root of the omental flap. Then, we closed the posterior peritoneum incision. Finally, we closed the operation incision in layers in the usual manner.\nThe pathological investigation of the resected sinus tract showed chronic inflammation with granulation hyperblastosis and foam cells growth. The patient was discharged 6 days after the operation. Six months later, MR examination showed that the NCF had healed well with the omental flap tightly covering the kidney (). The patient was asymptomatic throughout 12 months of follow-up. |
A 74-year-old, Caucasian woman from Germany with a long history of local recurrences of DFSP on her right upper limb was readmitted for surgical intervention of a tumour of her right axilla. On clinical examination she presented with two firm tumour masses in the right axilla and on the thoracic wall with a size of 6 × 7 cm and 6 × 5 cm. Both lesions displayed central ulceration. In addition, the patient requested the resection of a small (1.7 × 1.6 cm) nodular skin lesion of her right elbow region.\nThe DFSP of her right shoulder region was first diagnosed in 1991. Following several incomplete tumour resections and local progressive tumour growth she was transferred to our hospital in 2001. Tumour resection was carried out and radiation therapy was suggested but refused by the patient. Despite clear resection margins the tumour recurred locally 10 months later and, at this stage, the tumour had infiltrated the brachial plexus. Since then, additional surgical interventions for local recurrences of the DFSP were required every 5 to 9 months. Fifteen years after the initial diagnosis of the DFSP, at readmission for a local recurrence, the tumour extended from the axilla along the thoracic wall to the lateral aspect of the ipsilateral scapular and an additional small (less than 2 cm) reddish blue subcutaneous nodular lesion was observed at the dorsal aspect of the right elbow.\nHistologically the two tumour masses in the axilla and thoracic wall confirmed the clinical diagnosis of a local recurrence of the known DFSP. The tumours were highly cellular with partly ovoid, partly spindle shaped nuclei with small fascicular cell formations. Due to these clear detectable fascicular areas, these recurrences were classified as DFSP with fibrosarcomatous transformation (Figure ).\nIn contrast, the tumour from the elbow microscopically presented as a highly cellular small cell tumour lacking fascicular formations (Figure ). Additional immunohistochemical analysis revealed punctual reaction of the vast majority of tumour cells for keratin MNF 116 with a cytoplasmatic distribution and a punctual perinuclear immune reaction for cytokeratin-20. The tumour was CD3, CD20 and LCA negative. A high proliferative activity index (Ki 67) of 70% was evident. The histological and immunohistochemical analysis verified the diagnosis of a Merkel cell carcinoma (pT1 pN0, pM0 R0).\nThe Merkel cell carcinoma was resected in total whereas a complete resection of the DFSP (pT2b, pN0, pM0, R2) with preservation of the extremity could not be achieved.\nSix months after the surgery the patient suffered a synchronous local recurrence of both the DFSP and MCC (Figure ). At this time the tumour specimen from the elbow histologically resembled the previously described MCC. The axillary specimen revealed an infiltration of a spindle cell-like tumour with a partial storiform, partial fascicular, growth pattern. Within this tumour, an area of up to 1.1 cm with a small cell tumour infiltrate, correlating with the described Merkel cell tumour mixed with a spindle cell tumour formation, was found. The histological findings displayed a collision tumour of a metastasis of the Merkel cell tumour (pT3, pN0, pM1) and the relapse tumour of DFSP (pT2b, pN0, pM0, R2) (Figure ). Further clinical and radiological tumour staging demonstrated no further evidence of metastatic disease.\nFor local tumour control, the option of an interthoracoscapular amputation was discussed but not accepted by the patient. Therefore, palliative radiotherapy and chemotherapy with the tyrosine kinase inhibitor Imatinib was initiated. |
We present the case of a nine-year-old girl who was brought to the emergency department (ED) with a complaint of abdominal pain for two days duration. The pain was started in the periumbilical region then shifted to the right iliac fossa. Her pain started gradually and has increased in severity. She described it as a constant pain that was not relieved by oral simple analgesic medications. The pain was exacerbated by movement and was not related to posture or meals. She scored the pain as 7 out of 10 in severity. The pain was associated with nausea and few episodes of vomiting that had food content with no blood. One day after the onset of pain, the patient developed a low-grade fever that measured 37.9℃ and was relieved by antipyretics. The parents reported that her daughter had decreased appetite and activity since the onset of pain. However, there was no change in her urinary or bowel habits.\nThe past medical history of the patient was significant for poorly controlled asthma. She required three admissions in the past year due to asthma exacerbation. She was on an inhaled corticosteroid therapy and short-acting inhaled salbutamol. The patient did not have any surgical history. She attained normal growth and development milestones at an appropriate normal age. The gynecologic history was not obtained from the patient. The patient was a primary school student. There was no history of diseases running in their family and the parents were not consanguineous.\nUpon examination, the patient appeared tired and sick. She was not in respiratory distress. She did not appear pale, jaundice, or cyanosed. Her vital signs were a pulse rate of 100 bpm, blood pressure of 98/65 mmHg, respiratory rate of 13 bpm, and temperature of 37.2℃. Abdominal examination revealed a soft and lax abdomen. There was a localized tenderness in the right iliac fossa with rebound tenderness. Cardiorespiratory and neurological examination findings were normal. Initial laboratory findings were suggestive of an active inflammatory process with elevated leukocyte count and inflammatory markers (Table ). In light of the clinical and laboratory findings, the initial diagnosis was acute appendicitis.\nThe patient underwent an abdominal ultrasound examination. It revealed a dilated blind-ended structure in the right iliac fossa with a distended bladder (Figure ). Such findings were in keeping with the diagnosis of acute appendicitis. The patient underwent a CT scan of the abdomen which revealed marked distension of the vagina with fluid and was displacing the urinary bladder anteriorly. These unexpected findings were in keeping with the diagnosis of hematocolpos and imperforate hymen (Figure ). The patient was then prepared for hymenotomy under general anesthesia. The procedure resulted in a complete resolution of the patient’s symptoms. She was discharged on the third postoperative day. In the follow-up visits, the patient had no active issues. |
We report a case of a 76-year-old Caucasian male with extensive past cardiac disease history who presented to the hospital with a complete heart block. The patient felt weak and lethargic over the last 2 weeks and it worsened before the admission so he took his blood pressure at home and found his pulse to be very low on the blood pressure machine so he called the ambulance to go to the hospital. There were no symptoms of volume overload like dyspnea on exertion, leg edema, or other symptoms of congestive heart failure (CHF) exacerbation. The patient had a history of a sinus of Valsalva aneurysm (SVA) as well as coronary artery aneurysms; so in the past he had a Bentall procedure, which included placement of a mechanical aortic valve (#23 St. Jude prosthesis) and a conduit replacing the ascending aorta (aortic reconstruction). At that time, there was also a single-vessel bypass using a vein graft to the right coronary artery due to right coronary aneurysm. This was in 1997. He was following up with his cardiologist who did periodic CT/MRI of the chest with contrast to follow the previously diagnosed aneurysms. The repeated chest imaging was showing an ascending thoracic aortic aneurysm as well as the sinus of Valsalva and coronary artery aneurysms and was controlled with medical therapy and follow-up. Warfarin was given for anticoagulation for the metallic aortic valve. The size of thrombosed SVA was gradually increasing over time with 6 cm in diameter in 1999 and then progressed to 7.5 in 2000 and then to 7.3 × 6.5 cm by 2003. By 2004, size went up to 8.5 × 6.4 cm. The size remained stable on the subsequent CT scans that were done regularly. In 2009, the size went up to 8.1 × 7.2 cm. There were multiple cysts throughout the liver, the largest of which were seen in the right hepatic lobe measuring up to 14.7 × 11.3 cm and in the left hepatic lobe measuring 5.1 × 4.1 cm, and these were found unchanged on different CT scans. In 2010 due to uncontrolled hypertension on maximal number of antihypertensive agents, ultrasound of the renal arteries was done to exclude renal artery stenosis and came back negative for any finding. In 2010, patient had an attack of acute back pain and to exclude aortic dissection, CT scan of chest with contrast was done and it showed that the size of thrombosed sinus of Valsalva went up to 9.5 × 8.5 cm but with no actual dissection. MRI of the chest showed larger T1 and T2 hyperintense mass replacing the right lobe of the liver. This demonstrated a new thick wall and demonstrated evidence of hemorrhage within the lesion. This was indicating bleeding into the hepatic and splenic cysts at that time; anticoagulation was stopped till stabilizing the patient. It was found that his INR was 10.1 unexpectedly and could have been secondary to warfarin coagulopathy. INR was reversed with fresh frozen plasma and vitamin K administration. Patient was stabilized at that time and was discharged.\nMRI of chest and abdomen with and without contrast was done in April 2011 as a follow-up (Figures , , and ).\nIn 2013, he was admitted to a hospital for weakness and symptoms of CHF exacerbation and a transthoracic echocardiogram was done showing moderate concentric left ventricular hypertrophy. Left ventricular systolic function is mildly reduced. There is mild global hypokinesis of the left ventricle with ejection fraction 55%. The left atrium was moderately dilated in addition to mechanical aortic valve, moderate mitral regurgitation, prosthetic ascending aorta, a very large and thrombosed SVA, and normal-sized pulmonary artery. Transesophageal echocardiogram was done showing the same findings. This was decided to be managed medically at that time.\nOther past medical histories included uncontrolled hypertension with medications that were adjusted to reach the best numbers. He had chronic kidney disease secondary to ADPKD. His serum creatinine level was stable (baseline 157.5 μmol/L) on follow-up and never required dialysis. He had inguinal herniorrhaphy and surgical intervention for left sided hydrocele and spermatocele. He was a nonsmoker and nondrinker. He was living with his family. Family history was negative for premature coronary artery disease or other common cardiac diseases. His father died of a cerebrovascular stroke.\nOn admission to our hospital, EKG showed idioventricular rhythm (Figures and ) had replaced the previous sinus rhythm with ventricular rate decreased to 42/minute. Comparing previous EKGs showed that patient had right bundle branch block (RBBB) and left anterior fascicular hemiblock that progressed to complete heart block with AV dissociation and slow ventricular response. Medication list on presentation to us included azilsartan, eplerenone, Tamsulosin, Finasteride, clonidine, warfarin, and furosemide 80 mg tablet daily. He had a reaction to beta-blockers in the form of severe bronchospasm and for that reason he was not on beta-blockers on presentation to us.\nBy physical examination, the blood pressure was 144/63 mmHg. Heart rate was 40 beats per minute. Respiratory rate, oxygen saturation on room air, and temperature were normal. Chest was clear on auscultation and cardiac auscultation showed S1 and S2 were normal plus prosthetic mechanical sounds. There was a 2/6 systolic ejection murmur heard over the left mid sternal border. Metallic valve sound was heard. There were no diastolic murmurs or gallops noted. Trivial edema was noted in the legs bilaterally with mild jugular venous distension. Patient was awake and alert with no focal neurological deficit found.\nChest X-ray on admission showed a large soft tissue mass is again noted consistent with the mass present on the prior MRI that was done 6 months before abutting the left ventricle. Laboratory work showed anemia with HB 106 g/L with low MCV 79.8 fL and MCH 25.5 fL. Troponin was not elevated 0.65. Serum creatinine was 224 μmol/L; BUN was 29.64 mmol/L. Serum potassium was 4.9 mmol/L. This represents worsening of previous kidney chemistry and this was contributed to volume depletion either from poor oral intake or from worsening cardiac functions given his complicated cardiac history.\nINR was 5 with PTT 50.8 seconds as he was on warfarin for the prosthetic valve. The plan was to start the patient on dopamine infusion with external pacing pads for the complete heart block. Permanent dual chamber pacemaker was planned but the elevated INR needed to be corrected and fresh frozen plasma units and vitamin K were given. Next day during the stay in the ICU, patient started to develop dyspnea with wheezing and oxygen desaturation and the preliminary diagnosis was congestive heart failure with acute pulmonary edema that could be secondary to diastolic dysfunction exacerbated by plasma transfusion and the new onset complete heart block. Furosemide was given intravenously. Blood pressure was 163/70 with heart rate 53 while he was still on dopamine infusion. The patient was sent to the operation room immediately to place the permanent pacemaker and this was done under general anesthesia with endotracheal intubation (ETT). The patient returned back to the ICU on a ventilator and weaning was not possible postoperatively due to oxygen desaturation and altered mental status. Patient was put on propofol and fentanyl infusions. The repeated daily chest X-rays were showing cardiomegaly with hazy lung opacities at the base concerning pleural effusion and a mediastinal mass on left hemithorax representing the previously recognized thrombosed SVA.\nEchocardiogram was done showing a very large and thrombosed sinus of Valsalva aneurysm (). Trials of weaning from ventilator failed and patient seemed to still have persistent pulmonary vascular congestion that required continuous diuresis. A desperate trial was made by ordering Lyme disease antibody titer as a possible reason of the previously diagnosed heart block and it came back negative.\nBNP (B-natriuretic peptide level) was 3419 ng/L. Heparin infusion was started as patient needed anticoagulation for the mechanical aortic valve. Tube feeding was started via the orogastric tube (OGT). Eplerenone tablet was resumed via the OGT in an effort to help improve the worsening cardiac function. The daily diuresis with resultant negative fluid balance improved pulmonary vascular congestion and right-sided pleural effusion but there was a persistent opacification of the left hemithorax. BNP level was going down. There was a mass-like opacity on the left hemithorax which was interpreted with the preliminary differential diagnosis like pleural effusion or pneumonic consolidation. The furosemide intake improved the pleural effusion on daily chest X-rays but in the meanwhile blood pressure was dropping from overdiuresis and kidney functions started to worsen from the fluid shift away from the kidneys. So the diuresis was stopped. Hypernatremia (NA 157 mmol/L) developed due to free water loss and free water was given via the orogastric tube. Amlodipine, furosemide, and eplerenone were held given the low blood pressure and worsening kidney functions. Intravenous fluids were tried very cautiously due the complicated heart failure and hypervolemia. The goal was to keep INR between 2 and 3 and warfarin was stopped when INR was over that goal; then patient was kept on heparin infusion only given the worsening left-sided pleural effusion with the fear of developing hemorrhage into it. There was cream-colored moderate-to-large amount of secretions which was suctioned from his endotracheal tube. Sputum culture came back with Klebsiella pneumonia; so pneumonia was thought to be present and vancomycin plus ceftriaxone that was shifted to tazobactam-piperacillin was started intravenously. Vancomycin then was stopped. Then antibiotic was narrowed down to ceftriaxone according to the sensitivity result of the sputum culture. The decision was to do CT scan of the chest but without contrast due to worsening kidney functions. The CT scan of the chest showed moderate left-sided pleural effusion plus enlargement of the previously recognized thrombosed SVA (Figures , , , , and ). This leads to the idea that heart block happened secondary to enlarging SVA. Tapping of left-sided pleural effusion came back with 100 cc that was nonhemorrhagic. The analysis came back exudative and was thought to be secondary to a possible pneumonia on the same side of the lung. It was negative for malignant cells.\nLeukocytosis was getting worse and WBC went up to 26 × 109/L. Repeat sputum culture was sent again and showed Pseudomonas aeruginosa. Antibiotic was changed from ceftriaxone to ertapenem to cefepime according to the sensitivity result. Still there was no fever and actually leukocytosis improved after the change in antibiotics and went down to 12.9 × 109/L. Patient developed diarrhea and stool cultures came back positive for Pseudomonas aeruginosa. Kidney functions were worsening and serum creatinine went up to 267.75 μmol/L and BUN went up to 44.28 mmol/L. Kidney functions started to improve after gentle hydration and free water intake via orogastric tube but again pleural effusion on the left side was persistent on daily chest X-rays and BNP level was high so furosemide was given cautiously.\nThe cardiologist told the family that patient might need to be transferred to a specialized hospital where he will have a surgical repair of the enlarged thrombosed SVA. The prognosis was poor and weaning from mechanical ventilator failed and patient stayed on it for 15 days with no improvement. After discussion among family members and given the poor prognosis, the plan was cancelled and it was decided to do a terminal weaning and refer the patient to hospice.\nAfter terminal weaning, the patient was still surviving off the ventilator and oxygen saturation was stable on nasal cannula. His eyes were opened and he was noncommunicating and the differential diagnosis was a cerebrovascular stroke. So the patient was sent for a CT scan of the head and came back with a large retrocerebellar arachnoid cyst (). The confusion and unresponsiveness were then attributed to anoxic encephalopathy. The patient stayed for 2 days in the hospice ward and then died after his family had clarified that they do not wish to do a resuscitation to him. |
A 61-year-old woman who underwent bilateral TKA at a different institution, presented to our emergency orthopedic department on the third postoperative day with right knee and calf swelling, intolerable pain in the right lower limb, and weakness of right ankle dorsiflexors. Based on her surgery records, it was determined that the procedure involved an anterior midline skin incision followed by medial subperiosteal release along the proximal tibia via a medial parapatellar approach and implantation of a cemented knee prosthesis without tourniquet control (). There were no records available on the thromboembolic prophylaxis employed during the surgery, and the procedure was uneventful. She had no previous history of arterial disease or cardiovascular risk factors and trauma. However, the patient experienced weakness of right ankle dorsiflexors in the immediate postoperative period, which was diagnosed as common peroneal nerve palsy. The overall condition of the patient and the status of the right lower limb were good. Moreover, postoperative limb pulsation was present. The patient was immediately transferred to our emergency department and opioid analgesics were administered intravenously for pain relief. A thorough examination of the patient revealed a stable medical status and maintenance of vital parameters. On auscultation, there were no signs of adventitious sounds such as crepitus or rhonchi. Local examination revealed swelling over the entire right lower limb with painful restriction of motion of the right knee. In addition, we also noted swelling over the popliteal region, which was non pulsatile and without any signs of auscultation bruit. The postoperative status of the wound was good with no fresh discharge. On distal neurovascular examination, the right lower limb was found to be warm, and the pulsation of both the dorsalis pedis artery and posterior tibial artery was found to be normal. Neurologically, the ankle dorsiflexors were found to have motor grade 1, and there was a sensory deficit noted in the common peroneal nerve distribution. Based on these findings, a provisional diagnosis of common peroneal nerve palsy was made and a foot drop splint was applied immediately. The patient was then admitted to a ward for further observation. During the course of treatment, the patient reported decreased pain in the right lower limb with the use of analgesics, but the swelling of the entire right lower limb remained. A duplex examination of the right lower limb was performed as deep vein thrombosis was suspected; however, no evidence of deep vein thrombosis was noted. A computed tomographic (CT) angiography revealed a large popliteal artery pseudoaneurysm arising from the popliteal artery just above the knee with a large defect in the arterial wall, explaining the neurological symptoms (). The patient was immediately referred to a vascular surgeon for intervention. The lesion was treated with placement of a covered endovascular stent graft. After obtaining ante grade percutaneous access across the lesion, a covered 8×30 mm wall graft was placed. This resulted in a satisfactory sealing of the pseudoaneurysm ().\nHowever, even 2 weeks after the onset, the peripheral neuropathies were not completely resolved and were assessed with electromyography and somatosensory evoked potential studies to document the extent of the lesion. This examination indicated evidence of denervation, thus confirming the presence of peroneal and posterior tibial nerve dysfunction; the findings were used as a baseline for subsequent studies to track recovery.\nAt a 6-month follow-up examination, the patient remained asymptomatic without pain or swelling; however, the patient continued to exhibit a right foot drop with normal distal pulsation. |
An 8-year-old male child presented to us with a history of multiple bouts of massive hemoptysis and increasing dyspnea of 3-day duration. He had undergone bidirectional Glenn shunt at the age of 8 months for tricuspid atresia. He had an uneventful course till the present presentation. He was intubated and was mechanically ventilated in view of severe respiratory distress. His hemodynamics was supported with inotropes, and broad spectrum antibiotics were started. Initial workup showed normal blood counts with normal platelet count. Chest X-ray showed multiple heterogeneous opacities in right lung suggestive of hemorrhage []. In view of massive bleed from airway that was not controlled with positive pressure ventilation, he was taken up for an emergency angiography and collateral of 2.5 mm arising from left subclavian artery was occluded by coil embolization; following which there was temporary cessation of bleeding. However the next day, there was recurrence massive bleed from endobronchial tube. This time he underwent bronchoscopy that showed friable mucosa of right main bronchus and also computed tomography (CT) thorax [] which showed multiple tiny collaterals arising from the aorta that could not be embolized. Decision of bronchial artery embolization was deferred because of deteriorating general condition with recurrent bleeds.\nIn view of recurrent bleeding and inability to maintain optimum saturations, left side double-lumen endotracheal tube was inserted under bronchoscopic guidance. ILV was initiated by two separate ventilators. Right lung (bleeding) was put on the pressure controlled ventilation and left lung relatively normal, was managed with volume controlled ventilation. Both ventilators were time cycled with same settings and effort was made for maximum synchronization. With complete isolation of bleeding right lung from the left side and effective toileting of the right bronchus, bleeding gradually came down. The child was put back to a single conventional ventilator after 4 days and was extubated subsequently 2 days later, after confirming complete cessation of bleeding by bronchoscopy. The child was observed inward for few days and in the absence of any recurrence of bleeding, he was discharged in healthy condition []. There was no history suggestive of the pulmonary bleed during follow up visits. |
A 26-year-old Sudanese woman presented to a private hospital with painless swelling at the left lower ribs for 6 months' duration that showed rapid increase in size over the last 3 weeks before her presentation. She was 16 weeks pregnant at that time. Ultrasonography showed a well-defined complex mass at the left anterolateral costal margin measuring 2 × 1 × 1.4 cm. Fine needle aspiration done at the private institute showed atypical cells, followed by an unplanned excision done under local anesthesia 1 month later.\nReview of the paraffin blocks and hematoxylin and eosin–stained slides at the pathology department of our facility revealed multiple fragments of tumor composed of mononuclear stromal cells with abundant large osteoclast-type multinucleated giant cells. The tumor was extending to the adjacent soft tissue and skeletal muscle. There was no evidence of marked atypia, necrosis, or atypical mitotic figures. The morphological and radiological features were consistent with GCTB. The patient was then referred to our facility for more advanced care.\nTwo months after the initial procedure, clinical follow-up revealed reappearance of soft-tissue mass at the site of surgery. Ultrasonographic examination confirmed the presence of a heterogeneous lesion measuring 7.4 × 4.2 cm at the site of surgery involving the left 11th rib with increased vascularity on Doppler examination. The decision by the bone tumor multidisciplinary team was to closely follow the patient up clinically, with imaging studies to be postponed after delivery of the baby. After the birth of her child, CT and MRI of the thorax were done and showed a large heterogeneous soft-tissue mass measuring 17 × 12 × 8 cm in the left side of the chest arising from and destructing the 11th rib with intra-abdominal extension to the left side of the peritoneum, compressing the lower half of left kidney and displacing the bowel loop medially. Two small nodules were seen in the adjacent abdominal wall measuring 8 mm and 12 mm. The overall picture was suggestive of a local recurrence of the tumor which was confirmed by histopathologic examination of the ultrasound-guided biopsy taken from the lesion.\nPET-CT scan examination confirmed the presence of hypermetabolic large mass centered on the left lower chest wall along with peritoneal involvement and bilateral FDG-avid lung nodules consistent with lung involvement (Figure ). Complete excision of the recurrent mass was done with excision of the anterior parts of the 10th and 11th left ribs and releasing the tumor from the inferior surface of the left side of the diaphragm and the peritoneum. The tumor was ruptured during its release from the inferior surface of the left diaphragmatic copula. Repair of the diaphragm was done with mesh reconstruction of the defect at the left upper anterior abdominal wall. Histopathologic gross examination of the resected specimen showed that the tumor had heterogonous white, yellow to brown, and focally hemorrhagic cut surfaces with two subcutaneous skin nodules found in the vicinity of the tumor (Figure , A and B). Microscopically, the tumor showed morphological features similar to the initial tumor (Figure , A and B). No marked cytological atypia, atypical mitosis, tumor necrosis, or any other features suggestive of malignant transformation were noted (Figure , C).\nFour months after this procedure, another recurrence of the tumor occurred. The patient started to feel pain at the surgical site radiating to the lower abdomen. Examination revealed palpable mobile hard mass at the surgical bed. MRI confirmed the presence of multiple variable sized large intra-abdominal and retroperitoneal soft-tissue lesions, denoting recurrence of the tumor.\nPET-CT scan confirmed the presence of multiple peritoneal and abdominal wall lesions on the left with perihepatic peritoneal lesion on the right as well. The previously seen lung involvements showed increasing size and uptake. Ultrasound-guided biopsy from the recurrent lesion was done to rule any malignant transformation. However, no features of malignancy were noted.\nConsidering the atypical and very aggressive clinical behavior of the tumor, it was sent for DNA methylation study. Molecular testing was done on genomic DNA extracted from the tumor in formalin-fixed paraffin embedded tissue to detect hotspot mutations in the H3.1 gene (Hist1H3A and Hist1H3B). H3F3A G34W mutation was detected, but no copy number alterations were found.\nThe patient had another pregnancy, and rapid progression of the tumor was noticed. She travelled to another medical facility where she had termination of her pregnancy followed by wide massive resection of the left retroperitoneal local recurrence together with partial resection of left diaphragmatic copula, left-sided hemicolectomy with end-to-end transversosegmoidostomy, and extensive resection of the abdominal wall with mesh reconstruction.\nClinical and radiological follow-up of the patient (6 months after the latest procedure) showed no evidence of local recurrence and a stable course of the lung nodules. The patient was on denosumab for 3 months after the last surgery, and the latest PET-CT scan did not reveal any recurrence or change in the size of lung nodules. She is now on regular follow-up. |
A 25-year-old male reported to the hospital with an alleged history of road traffic accident and history of loss of consciousness and nasal bleed. The patient was conscious, stable, and oriented. The patient presented with a laceration wound measuring approximately 5 cm × 3 cm in the left forehead region running parallel to the eyebrow [], which was debrided immediately and sutured to control bleeding. Diffuse edema of the left upper and middle third of the face was evident along with circumorbital edema and subconjunctival hemorrhage of the left eye. The patient's mouth opening was adequate with bilaterally stable occlusion. Assessment of the computed tomography [] revealed an incomplete and undisplaced Le Fort I fracture and a left frontal bone fracture which was approximately 3 cm × 2.5 cm × 1 cm. The frontal bone fracture involved both the cortices without involvement of the frontal sinus, and there was no evidence of dural tear as discussed with the neurosurgical team. The patient was observed for any neurosurgical deficit and was found to be devoid of the same. Since the patient's mouth opening was adequate, occlusion was stable with no mobility of the maxilla, and the Le Fort I fracture was decided to be managed conservatively. The frontal bone was minimally depressed without involvement of the frontal sinus; therefore; bone cement was used as an alternate to the standard approach based on esthetic concerns.\nThe existing laceration was utilized to approach the fracture site []. The fracture site was exposed [] and isolated. A calcium phosphate-based cement[] (HydroSet™) which is a synthetic bone graft substitute was used in the reconstruction of the depressed frontal bone fracture. The bone cement [] consisted of powder and liquid components, was mixed to attain a flowable consistency and then was applied over the defect and was manipulated. The cement was designed to set in the presence of water, blood and cerebrospinal fluid. Once the cement had set, closure was done in layers with Vicryl 3-0 and ethilon 4-0 [].\nThe patient was assessed postoperatively [] for any pain, edema or bleeding at the surgical site. A postoperative computed tomography [] was done to evaluate the adaptation and contour of the bone cement placed over the defect. |
A 27-year-old woman applied for a breast reshaping surgery for aesthetic purposes. There were no other complaints in her medical history. The patient underwent simultaneous mastopexy according to classical Lejour vertical scar technique and breast augmentation surgery using round silicone TSF - 415 mL implants under the pectoral muscle and a breast lift under general anesthesia (). During the operation proper hemostasis was achieved using electrocoagulation. Drains were removed next day after the operation with minimum serohemorrhaging fluid volumes. The later postoperative period was also uneventful.\nThe patient was discharged from the clinic on the second day after the surgery. Five weeks after the operation patient arrived at the clinic because of tenderness and swelling of the right breast. The patient stated that she had not sustained any traumas. During clinical examination, the upper right breast area was found to be significantly swollen and firm (). Ultrasound examination showed a 2.5 cm heterogeneous liquid strip accumulated around the implant (). The implant was intact. Complete blood count showed an increased amount of leukocytes, and red blood cells and hemoglobin were at the lower limit of the normal level.\nNo coagulopathies were found. The patient was taken to the operating room where she underwent revision surgery. The purpose was to remove the fluid and to find and stop the cause of its accumulation. During the operation (), a blood clot of 650 mL was removed (). Bleeding from one of the internal mammary artery branches in the implant pocket between the rib cage and the pectoral muscle lower pole was detected and stopped. After the revision, the implant was returned to the lodge. Vacuum drainage was used for one day only. One year after the surgery, there was no recurrence of bleeding, also no clinical evidence of the implant capsule contracture formation was found ().\nAll procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The patient gave her informed consent prior to her inclusion in this case report. Any details that might disclose the identity of the patient under study were excluded. |
This is the case of a 21-years-old man who was diagnosed with β-thalassemia major at the age of 8 months. He underwent splenectomy at the age of 10 years and has been receiving monthly blood transfusions ever since. Three months prior to admission, he presented to a peripheral emergency department with a sudden episode of dyspnea and central cyanosis and was suspected to be infected with COVID-19. Chest CT scan was made and ruled out any interstitial lung involvement and revealed the presence of bilateral pulmonary emboli. Anticoagulation therapy with warfarin was started, but on follow-up the patient developed progressive exertional dyspnea, deteriorating to NYHA functional class III.\nThe patient was referred to our institution because of worsening symptoms. Physical examination showed clinical evidence of right heart failure and pulmonary hypertension, with a mild drop in arterial oxygen saturation (93%). Echocardiography revealed a dilated right atrium (RA) which contained a large 47 × 35 × 16 mm hyperechogenic mobile mass attached to the RA free wall near the orifice of the inferior vena cava. The right ventricle (RV) was severely dilated and dysfunctional, with tricuspid annular plane systolic excursion (TAPSE) of 13 mm and severe tricuspid regurgitation. Estimated systolic pulmonary arterial pressure (PAP) was 85 mm Hg. Lung perfusion scintigraphy was not available at our center. Chest CT angiography showed bilateral defects in the pulmonary arteries consistent with bilateral chronic pulmonary thromboemboli (Figure ).\nOn the basis of this evaluation, the diagnosis of CTEPH was established and PTE was performed, the technique of which was reported previously. Total circulatory arrest time was 45 minutes, and total cardiopulmonary bypass perfusion time was 94 minutes. A large organized clot was removed from the RA, in addition to removing the thickened intima and organized thrombi from lobar and segmental pulmonary arteries of the left middle and lower lobes, as well as from all major branches on the right.\nEarly recovery following surgery was uneventful, and the patient was discharged from ICU on the 4th postoperative day. Predischarge echocardiogram confirmed satisfactory hemodynamic results, with an improved RV function and an estimated PAP of 45 mm Hg. The patient was discharged home 19 days after surgery on warfarin but no requirement for supplemental oxygen. Three months after discharge, significant improvements in clinical status (NYHA class I) and oxygen saturation (99%) were noted despite a degree of residual pulmonary hypertension (PAP of 42 mm Hg). |
A 28-year-old female presented with pain in her right knee joint for the past 18 months. She was a housewife and had a history of twisting injury to the right knee two years prior to presentation. At presentation, she had pain in the right knee, both beyond 120-degree flexion and in terminal extension. On clinical examination, there was mild wasting of the quadriceps muscle. Range of movement was full but both terminal knee flexion and extension were painful. Clinical tests for cruciate knee ligaments and meniscal injuries were negative.\nMagnetic resonance imaging (MRI) was reported as large lobulated multiloculated cystic lesion near the posterior aspect of the femoral attachment of ACL suggesting encysted synovial collection or cystic synovial neoplasm.\nDuring arthroscopic evaluation, the cyst was visualised on the posterior extrasubstance aspect of ACL near femoral attachment measuring approximately 15 mm by 12 mm in diameter. A second cyst, not connected with the first, was noted anterior to ACL near the tibial attachment measuring around 5 mm by 3 mm in diameter which was not reported in the MRI. However, after surgery, a review of the same MRI also showed the presence of the anterior ACL cyst (Figures and ). The cysts were thin walled, transparent, freely mobile, and well demarcated and their origin from ACL was clearly visualized. There were no adhesions or signs of inflammation surrounding them. The medial and lateral menisci were normal, ACL, PCL were intact, and the rest of the joint was normal. The cysts were punctured and their walls broken by probing. Clear gelatinous fluid was seen exuding from the cysts when they were punctured. Arthroscopy resector was used to resect the majority of the cyst wall and the same was sent for histopathological examination. ACL was confirmed to be intact after the procedure. Postoperatively patient was mobilized in the hospital and there was no pain during terminal movements on the day of surgery itself. Biopsy of the lesion was compatible with that of the ganglion cyst (Figures and ).\nThe patient was followed up in orthopaedic outpatient department (OPD) regularly. At the final 2-year postoperative follow-up, the patient did not have any complaints and the entire range of joint motion was painless with a repeat MRI not showing presence of either of the ganglion cysts (). |
We report a 62-year-old man with mucormycosis who suffered from chronic hepatitis B for 20 years. A month before admission, trauma caused chest pain, wounds on the right upper limb and right knee joint. Six days before admission, there was no obvious inducement for aggravation of joint pain and chest pain. Chest CT was performed locally and showed the shadow of the lungs. The local diagnosis was rheumatoid arthritis, type 2 diabetes, and hepatitis B. He received related treatment in a local hospital. However, his joint pain was not significantly relieved, while coughing and coughing dark red sputum occurred at the same time, so he went to the rheumatic immunology department of our hospital.\nThe vital signs were normal on admission. Physical examination showed that the posterior pharyngeal wall was red and swollen. The shoulder and knee joints were swollen. An irregular wound could be seen on the inside of the upper limb and knee joint. Relevant examinations and tests were improved after admission. Test results are shown in . Chest CT showed multiple cavities in the lower lobe of both lungs (). Therefore, the patient was initially diagnosed with rheumatoid arthritis, pulmonary infection, and hepatitis B. He was given levofloxacin 0.5 g IV once daily (QD) for anti-infective treatment and metformin 50 mg orally (PO) two times daily to control blood sugar.\nThree days after admission, the patient still had a severe cough with dark red sputum. Therefore, he was transferred to the respiratory department for treatment on the third day of admission. The physical examination showed that there was a small amount of wet rale in the left lower lung and a large ulcer in the right upper limb. On the first day after the transfer, a large amount of purulent secretions from the left main bronchus and a small amount of purulent secretions from the right main bronchus were observed by tracheoscopy. Bronchoalveolar lavage fluid (BALF) was collected and tested for microbial culture and mNGS. On the third day after transfer, the patient developed fever, and the highest temperature was 38.5°C, which returned to normal after physical cooling. The fever of the patient was thought to be due to infection. Microbial culture and mNGS of BALF showed Klebsiella pneumoniae infection (). On the fourth day after transfer, according to the results of drug sensitivity, the antibiotic was adjusted to piperacillin sodium 4.5 g IV every eight hours (Q8h). Low blood glucose was detected at this point, so he stopped using metformin. On the seventh day after transfer, a blood routine examination showed that white blood cells were 4.8 × 109/L and C-reactive protein (CRP) was 85.6 mg/L.\nThe 9th day after transfer to respiratory department recommended debridement treatment and sent it to mNGS. In the process of waiting for the results, the patient coughed intermittently with grayish-brown sputum. During this period, we continued the anti-infection treatment and maintained good blood glucose control, so we did not continue to use drugs for blood glucose. Finally, the result of mNGS submitted by debridement tissue indicated R. microsporus infection (). Combined with upper limb mNGS and imaging results, the possibility of lung complicated with Mucor infection is considered. Therefore, the amphotericin B antifungal therapy was added on the 11th day after transfer.\nThirteen days after being transferred to the respiratory department, the chest CT showed that the lung lesions were worse than before (), and the empirical anti-infection treatment was poor. The diagnosis was further confirmed by CT-guided biopsy, and the punctured tissue was sent to pathology for mNGS detection. However, the patient suddenly developed massive hemoptysis on the day of the biopsy and died after the rescue was ineffective. The final pathology showed that a small amount of suppurative exudate with acute fibrinous tissue surrounding lung tissue causing pneumonia. The mNGS result returned as R microsporus infection (), which was consistent with the left upper limb wound test result. A brief summary of the patient's treatment is given (). |
A 15-year-old male with renal failure was referred to our center for dialysis treatment. Two weeks prior to referral, he was brought to an orthopedist for a suspected bone disorder characterized by worsening lumps on his left hand and ankle, which was noticed by his mother about 1 year before presentation. However, she had assumed that the swelling to his left hand was due to biting. In addition, swelling to his left ankle occurred after several leg strengthening exercises conducted by a physiotherapist. The orthopedist later recommended magnetic resonance imaging (MRI). On MRI preparation, the patient was found to have a high urea level and was thus referred to a pediatric nephrologist who subsequently initiated dialysis. His mother also claimed that the urine volume of her son had decreased within the last few weeks. On the second day of hospitalization, the swelling in his left ankle burst and thick masses containing blood, debris, and white crystalized materials were observed.\nThe patient was born to healthy non-consanguineous parents with no family history of HPRT deficiency (). At the age of 1 day, the patient developed stiffness in both hands. When he was 2 months old, he was diagnosed with severe psychomotor retardation and could only lie down. A pediatric neurologist diagnosed the patient with cerebral palsy at the age of 7 months based on computed tomography results showing cerebral atrophy. The patient has a habit of biting his lower lip, buccal mucosa, and left hand, which was evident since the age of 6 years, for which an occlusal bite guard was once used years before to protect his teeth. However, he refused to use it, which resulted in the extraction of some of his upper teeth. At present, he is unable to talk or communicate normally, but can express anger through agitating behavior. Moreover, his parents remarked about the occasional presence of orange sand-like sediments found in his diaper, which first occurred at the age of 3 years. However, a local pediatrician assumed that the patient was merely dehydrated and recommended increased water intake and intravenous fluid treatment, but neglected to measure his uric acid levels.\nOn a physical examination, deformities to the lower lip were noted (). A neurological examination revealed hypotonicity, dystonia, and clonus of all four extremities (). Single lump was seen on the antihelix of the earlobe (), along with multiple lumps on his extremities () and larger lumps similar to abscesses on his left hand and ankle (). He had scoliosis and flexion contracture of the hip, knee, and shoulder. He weighed 22 kg and was 140 cm tall (less than the third percentile of the Centers for Disease Control and Prevention, National Center for Health Statistics 2000 growth chart). The diuresis rate was 0.9 mL/kg/h. The laboratory workup results showed a hemoglobin level of 60 g/L (6.0 g/dL), serum uric acid level of 1608 µmol/L (27 mg/dL), urea level of 99.96 mmol/L (280 mg/dL), creatinine level of 671.8 µmol/L (7.59 mg/dL), and an estimated glomerular filtration rate of 7.6 mL/min/1.73 m2 (New Schwartz).\n24-h urine collection revealed a uric acid level of 1.1 mmol/24 h (186 mg/24 h) (with a total urine volume of 430 mL) and random urine uric acid to creatinine (UA/C) ratio of 2.7 (mg/mg). Electrolyte analysis indicated metabolic acidosis (bicarbonate level of 14.1 mmol/L), hyponatremia (sodium level of 129 mmol/L), hyperkalemia (potassium level of 7.14 mmol/L), and hyperphosphatemia (2.77 mmol/L). Other electrolytes were within normal limits.\nChest radiography showed thoracic scoliosis (). Arthrography or joint X-ray of the left hand and ankle showed revealed the presence of tissue masses (). After the lump on the left ankle burst, debridement and exploration sessions were performed in the left wrist and ankle, which revealed tophi of several joints (). Along with the debridement and exploration procedures, bone biopsy results confirmed juvenile gouty arthritis ().\nKidney ultrasonography revealed bilateral atrophic kidneys (left kidney: 5.4 × 2.9 cm; right kidney: 6.9 × 4.3 cm) with multiple stones in the right renal pelvis (). A diagnosis of LND was confirmed via genetic testing showing a homozygous deletion of exon 1 of the HPRT1 gene. The samples were sent to a foreign genetic laboratory and the results were obtained 1 month later. HPRT enzyme levels could not be measured in a timely fashion due to logistic challenges, such as unstable temperature for sample delivery and the inability to provide a short delivery time.\nHemodialysis was initiated, which was followed by regular hemodialysis within a week, but was switched to continuous ambulatory peritoneal dialysis to provide better long-term quality of life for the patient and his caregiver. The patient received a red blood cell transfusion, allopurinol treatment at a dose of 300 mg/day, a low-purine diet, and treatment for ESRD, which included subcutaneous erythropoietin (EPO) supplementation of 6000 U/week, oral calcitriol at a dose of 0.5 mcg daily, oral CaCO3 at 500 mg three times daily (with a meal), oral folic acid at 5 mg once daily, and one tablet of oral vitamin B complex daily. With treatment, serum uric acid levels decreased to 470 µmol/L (7.91 mg/dL) within 2 weeks. During a follow-up visit at 2 months after the tophi burst, the patient appeared to be more responsive when communicating with others. Moreover, the swelling to his ankle and wrist had significantly improved. The patient complied well to the treatment regimen and peritoneal dialysis caused no side effects. The results of a follow-up laboratory workup at the outpatient clinic were as follows: serum uric acid level of 336 µmol/L (5.65 mg/dL), serum urea level of 14.2 mmol/L (39.76 mg/dL), serum creatinine level of 323 µmol/L (3.65 mg/dL), and random urine UA/C ratio of 0.42 mg/mg. The parents were advised to undergo genetic consultation to identify if their daughters were carriers or if their other son also carried the mutation. The prognosis of the patient was poor because of the presence of ESRD accompanied with multiple irreversible abnormalities. |
A 16-year-old Caucasian girl with recessive dystrophic EB presented to our dental school for routine treatment. According to the clinical interview, hemorrhagic blisters in the mouth had been detected since early infancy. With the patient's growing and physical development, lesions extended to the face, feet and hands. Due to her great difficulty in performing adequate oral hygiene, almost all of her teeth had been destroyed by caries lesions and were covered with dental plaque ( and ). Her oldest sister had died one year before as a consequence of the same disease.\nThe patient's mental status was appropriate for her age. However, her physical development was delayed and her weight and height were compatible with that expected for a 9 year-old child. During physical examination, several blisters were observed in several areas of the body, and they quickly appeared right after mild pressure on her lips. Her fingernails and toenails were absent and her hands showed some degree of deformity. Her tongue presented no papillae. She also presented ankyloglossia and microstomia, probably due to secondary scars from repeated episodes of blistering and subsequent healing.\nAfter evaluation of the panoramic radiograph () and intraoral examination, a treatment plan was established. Extraction of maxillary right first molar, first premolar, lateral incisor and central incisor, maxillary left central incisor, lateral incisor and first premolar, mandibular left first molar, mandibular right first and second premolars and first molar, and restorative treatment of second premolar, canine, mandibular left first premolar and mandibular right lateral incisor were recommended. At beginning of the dental treatment the patient was poorly cooperative and did not permit even a simple dental examination. The option for dental extraction as part of the treatment plan was based on the clinical limitations associated with the patient's disease. Microstomia, blister formation and the poor patient cooperation all together made the surgical procedures even more difficult and challenging. Dental plaque was disclosed and the patient was taught how to improve her oral hygiene through better brushing techniques and the additional use of a 0.12% chlorhexidine solution applied topically with a cotton bud after lunch and at bed time for 7 days.\nFollowing the patient's conditioning therapy, the surgical procedures were carried out in 5 weekly appointments (). At all extraction sections, first a topical local anesthetic was applied for 4 minutes. Right after, one cartridge (1.8 mL) of local anesthetic solution, 2% mepivacaine with 1:100,000 adrenaline, was infiltrated. During the application of the local anesthetic solution, blister formation occurred. To avoid trauma to the fragile oral mucosa, precautions were adopted, including the use of gentle pressure during the surgeries and no suture usage. After each extraction, the healing process was very rapid and the patient did not complain about any pain ().\nA prosthetic appliance with two central incisors was prepared to replace the four maxillary anterior teeth, and it was delivered after the maxillary extractions had been completed (). The delay for delivering the appliance was purposeful, in order to give the patient motivation to cooperate with the required surgical and restorative procedures. The restorative treatment using glass ionomer cement was accomplished within 5 weeks.\nThe patient was followed for 3 years for control of the prosthetic appliance and oral health. |
A 38-year-old gravida 1, para 0 was transferred to our unit at 26 weeks of gestation with severe right iliac fossa pain and a suspicion of threatened preterm labour. She presented with a 3 day history of right iliac fossa pain, nausea, vomiting and diarrhoea. Vaginal examination and her fetal fibronectin test was negative. However, transabdominal and transvaginal ultrasound examination revealed a 6.0 × 5.5 × 5.9 cm vascular mass within the right pelvis with a clear arterial feeder and turbulent swirling intraluminal flow, leading to the diagnosis of a pseudoaneurysm (Fig. ). The diagnosis was subsequently confirmed by Magnetic resonance imaging (MRI) (Fig. ). The patient was discussed in our multidisciplinary team meeting with interventional radiologists, vascular surgeons, and anaesthetists. The risk of imminent rupture was assessed as high due to the severity of her symptoms and a 5–10 mm overall increase in the size of the pseudoaneurysm documented by sequential MRI 14 days apart. Surgery was considered high risk and so selective embolization of the pseudoaneurysm was performed. This was performed under local anaesthetic in the Interventional Radiology suite with full preparation for emergency delivery by the obstetric and anaesthetic team in case of fetal distress. The right uterine artery was identified angiographically from a contralateral femoral arterial puncture and selectively catheterised using a microcatheter. The artery was small but extravasation into the presumed pseudoaneurysm was identified near its proximal portion. The artery was embolised with a series of microcoils across the neck of the pseudoaneurysm to block flow. The procedure was uneventful and the fetus showed a continuous reactive heart rate pattern. Follow up ultrasound showed a completely thrombosed pseudoaneurysm with no flow. She had regular follow ups in the antenatal clinic with no sign of recurrence. A planned caesarean section was performed at 38 weeks gestation. A healthy baby boy was born weighing 2696 g with a blood loss of 1100mls. During the caesarean section, the thrombosed pseudoaneurysm was seen below the right broad ligament and all other pelvic organs looked completely normal (Fig. ). An ultrasound scan 3 months postpartum showed a small completely thrombosed pseudoaneurysm (Fig. ). |
A 50-year-old female came to our hospital for a regular examination, presented with distention in the epigastrium 1 week ago, the feeling lasted a few minutes and then spontaneously resolved. The patient had no gastrointestinal history before, and no special medical, family, genetic, and psychosocial history either. As there was no tenderness in the upper abdominal, no mass found in the examination by physician, plus chest radiography showed no abnormalities yet, she was suggested to take an ultrasound examination. Sonography on an empty stomach showed an indistinct hypoechogenic nodule in the area between liver, esophagus, and fundus of stomach. In order to show the nodule clearly, we asked her to take the exam again after drinking 700 mL water. The clearer image showed that the size of the nodule was 24.3 mm × 15.4 mm, with a clear boundary and even internal echo. Rich blood flow signal and arterial flow spectrum were also detected in it, in addition, contrast-enhanced ultrasound (CEUS) showed intense enhancement on the arterial phase and slightly reduced enhancement on the delayed phase. Since the nodule was very tight to the wall of the stomach, we supposed it as a gastrointestinal stromal tumor. The ultrasound images are shown in Figure .\nIn order to further clarify the relationship between the mass and stomach wall, the patient was taken a CT scan. The plain CT images showed a homogeneous nodule, whose density was slightly lower than that of liver nearby. In addition, intense enhancement on the arterial phase and equal enhancement on the venous and delayed phase were observed. Since a narrow gap between the nodule and the fundus was seen on enhanced CT image in venous phase, we inferred that the nodule was likely out of the stomach, perhaps a lymph node or a subserosal gastric stromal tumor. The CT images are shown in Figure .\nAlthough ultrasound-guided puncture biopsy would be more appropriate next, the patient wanted to have a laparoscopic surgery immediately. During the operation, the nodule was discovered closely adhered to the diaphragm; dilated and tortuous vessels were seen around and on the surface (shown in Fig. ). The tumor was thoroughly removed without a hitch (shown in Fig. ), and the subsequent frozen pathology suggested that it was a lymphoid tissue hyperplasia. The final paraffin pathology result was a hyaline-vascular type of CD, hematoxylin-eosin staining showed marked vascular proliferation and hyalinization of the abnormal germinal center, with a tight concentric layering of lymphocytes around the follicle, resulting in an “onion-skin” appearance (shown in Fig. ), along with the accumulation of CD34+ (vessel), CD21+ (FDC) consistent with hyaline-vascular type of CD (shown in Fig. ). During the follow-up at 15 months after laparoscopy, the patient recovered very well with no signs of recurrence, she was very satisfied with the process of diagnosis and operation. |
A 27-year-old woman had a renal biopsy because of intermittent macroscopic hematuria and proteinuria after tonsillitis. When she was 24 years old, the first episode of macroscopic hematuria and proteinuria (1.0 g/g Cr) occurred just 1 day after tonsillitis with a high fever. After treatment with antibiotics, all symptoms disappeared except for microscopic hematuria. Because such episodes recurred several times and there was continued microscopic hematuria without proteinuria, the patient underwent tonsillectomy when she was 26 years old. However, even after tonsillectomy, microscopic hematuria persisted, and she repeatedly had episodes of macroscopic hematuria and proteinuria following pharyngitis with a high fever. She had a renal biopsy under real-time ultrasonographic guidance with a 16-gauge automated biopsy device and was diagnosed with IgA nephropathy. The histology showed crescent formations in 2 out of 16 glomeruli, but she refused steroid therapy. Before being discharged from our hospital, evidence of only slight renal subcapsular bleeding in the left kidney could be found on routine ultrasonography. Three months after the renal biopsy, she had sudden macroscopic hematuria without any symptoms, which lasted for 3 days. After that, the macroscopic hematuria disappeared but the amount of microscopic hematuria was clearly increased as compared to before her renal biopsy. In the urinary sediment, red blood casts were always present and comprised of 20–40% dysmorphic red blood cells. Since an enlarged left renal pelvis was newly recognized on ultrasonography, enhanced computed tomography (CT) was performed and she was diagnosed with an arteriovenous fistula (AVF) in the lower pole of her left kidney (fig. ). As the amount of microscopic hematuria had decreased again, she chose not to receive therapy. When she was 34 years old, our patient got married and was concerned about her future fertility. Since repeated ultrasonography showed an increased dilatation of the left renal vein (fig. ), she decided to undergo treatment with percutaneous intervention. An arteriogram confirmed an AVF with an enlarged draining vein and aneurysmal dilatation (fig. ). Selective embolization of the feeding arterial branch closed the AVF with 8 detachable coils (fig. ). Follow-up magnetic resonance imaging (MRI) performed 3 months after the intervention did not show any abnormalities, including the dilatation of the renal vein (fig. ). Microscopic hematuria disappeared within another 3 months and complete remission of IgA nephropathy had been achieved. |
A 51-year-old Madheshi woman came to our neurology clinic with the chief complaint of sudden decrease in the tone and texture of her voice for the past 15 days. Her voice was very feeble but understandable and she noticed it was better by the time she got up from her bed only to worsen through the day to become nearly inaudible. She had noticed a slight change in her voice texture in the first several days which she had ignored in the beginning as it was not worth bothering about. She had no complaint of choking or coughing or aspiration or any throat discomfort. When asked for any other related and/or unrelated things she noticed in her habits, she complained about having constipation for many years otherwise she was apparently well. She had never visited hospital for any problem and no interventions had been done in the past. She denied smoking tobacco, drinking, or any other recreational drugs abuse. There was no one in her family or her parents’ family with any kind of known chronic disease. Her biological father had controlled hypertension with medication. Her psychosocial history was not significant. She came from a middle income family, and she had retired recently as an accountant for a small company. Her symptom progressively aggravated in later days to the extent that she not able to produce sound properly. It usually waned in the early morning or after enough voice rest only to wax throughout the day.\nOn physical examination, a systemic examination did not reveal any abnormality. On neurological examination, her muscle power was intact: 15/15 on Medical Research Council (MRC) scale. She had no imbalance and was able to tandem on walking. An examination of her gag reflex and other cranial nerves revealed no abnormal reflexes. Deep tendon reflexes were intact. Her speech articulation was intact and revealed no scanning of speech. Speech production was adequate and non-painful; her tone was non-nasal but the intensity was low and slow. Other neurological examinations also did not reveal any abnormality. Other systemic examinations were also non-significant.\nConcerned with her problem, she had visited an ear, nose, and throat (ENT) department for her problem and was screened for possible laryngeal disorder for hypophonia. There was no obvious laryngeal pathology found and the treatment initiated by the ENT department had no satisfying outcome. She visited our neurology clinic. The differential diagnoses considered were Parkinson’s disease (PD) or a bulbar variant of motor neuron disease (MND), and MG. MRI scanning of her brain and screening of her whole spine appeared normal. Laboratory investigations including a hematologic panel, infectious disease screening panel, and myopathy panel involving creatinine kinase and creatine phosphokinase-N-acetyl cysteine (CPK-Nac), were all within normal range. She was suspected to have MG as other diseases were ruled out. A Quantitative Myasthenia Gravis (QMG) test was performed on our patient and her QMG score was 1 (for speech). She was given neostigmine challenge test with intravenously administered injection of 2.5 mg neostigmine and the result was positive after more than 30 minutes. There was significant improvement in her voice quality and she felt her fatigability improved as well. She gained a good quality of speech within an hour. QMG scoring was repeated, and she scored QMG score of 0. The longer duration of the medicine gave us enough time to assess the improvement. The laboratory test for the AchR antibody (AchR ab) is not available in Nepal. We had to send AchR ab to India, and the report received after waiting several weeks was positive. Quantitatively, it was significantly higher, 1.11 nmol/l than the normal range (0.0–0.4 nmol/l). She was later maintained on 60 mg of orally administered pyridostigmine with 12 hourly administrations and subsequently increasing to 6 hourly along with steroids.\nPlain and contrast-enhanced computed tomography (CT) scans of her head, neck, and chest were done, and they revealed a mass in the anterior mediastinum: a well-defined, smooth outlined, approximately 4 × 2.5 × 2 cm, soft density nodule showing poor enhancement in the anterior mediastinum in the thymic space with maintained fat plane with the adjacent vessels and structure. Thymoma was suspected and she was sent to the surgical department for further surgical intervention.\nShe returned to our neurology clinic around 2 months after her first visit to us as a follow-up visit. She had undergone a successful surgery. She was doing well. Prednisolone was already tapered off. She was tapering down pyridostigmine as well, with a maintenance dosage of pyridostigmine 60 mg 12 hourly and planned to put off the medication. Her voice was clear and well maintained and she had no complaint of fatigue. She had no fresh complaint and was doing all her daily and professional activities very well. |
A 33-year-old male patient presented to a tertiary care hospital with intermittent abdominal pain, nausea, vomiting, constipation and numbness of bilateral lower limb extremities, of 3 days duration. He was conservatively managed in a surgical unit as partial intestinal obstruction and was awaiting diagnostic laparoscopy. The patient developed confusion and found to have systemic hypertension, and, therefore, was transferred to a medical ward for further management. He is a non-diabetic and did not have a previous history of hypertension. The drug history revealed usage of over-the-counter analgesics for 6 weeks.\nThe past medical and surgical history revealed similar neurovisceral attacks requiring five acute hospital admissions over 2 years, which ended up in questionable diagnoses. The sixth acute attack raised the suspicion of an acute porphyria. The first attack in January 2013 led to a diagnosis of appendicitis. Because the symptoms worsened following the surgery an emergency laparoscopic exploration was done. But the exploration revealed no cause to explain the worsening symptoms. The second attack was managed as sinus tachycardia and he was started on beta adrenergic blockers. The third attack which was associated with a fever was conservatively managed for a questionable renal colic. Forth attack was complicated with transient hyponatremia and transiently high serum creatinine levels. These complications were attributed to a questionable interstitial nephritis based on the fact that patient had used 50 mg of diclofenac sodium twice a day for 6 weeks, repeating the prescription given by a general practitioner. Another attack in 2015 was managed as partial intestinal obstruction and diagnostic laparoscopy was done. In all these presentations, findings from the ultra sound scans and diagnostic laparoscopy did not support a diagnosis of intra-abdominal pathology.\nOn examination he was thin built (BMI = 20 kg/m2) and pale. Brachial blood pressure was 160/90 mmHg. There were scars of previous appendectomy and laparoscopy surgeries on the abdominal, but, otherwise, the abdominal examination was unremarkable. Muscle power was 4/5 in all four limbs (could not move against a good resistance).\nThe laboratory investigations performed during this admission showed severe hyponatraemia of 115 mmol/L (136–145) with serum osmolality of 255 mOsmol/Kg (275–295) and urine osmolality of 460 mOsmol/Kg (50–1200 mOsmol/Kg). Serum creatinine concentration was 106 µmol/L (80–115) with blood urea level of 20 mg/dL (6–20). Hemoglobin concentration was 8.2 g/dL (13.5–17.5) and the red cell morphology was normochromic and normocytic. The total cholesterol level was 282 mg/dL (5th to 95th centile; 142–258) with LDL fraction of 225 mg/dL (5th to 95th centile; 78–185). Serum ferritin level was 646 ng/mL (20–250). Arterial blood gas analysis was suggestive of a metabolic acidosis. Echocardiogram showed evidence of left ventricular hypertrophy. There were no significant radiological findings in abdominal X-ray film or abdominal ultrasonography. Blood lead concentration was 3 μg/dL (< 5 μg/dL).\nA urine sample collected during the acute attack was sent to the Department of Chemical Pathology for biochemical analyses. On standing the urine sample gradually turned dark brown. The Watson and Schwartz test for urinary porphobilinogen (PBG) was positive (Fig. ). Spectrophotometry of urine for total porphyrins showed a “Soret band”. Urine total porphyrin level, calculated using Allen corrected absorbance of the urine sample was 5505.5 nmol/L (< 300 nmol/L). Genetic studies were carried out in an overseas laboratory. The full analysis of HMBS gene was performed by PCR amplification of extracted DNA followed by exon specific primer extension analysis of all exons, exon intron boundaries and promotor regions. The gene analysis revealed a previously reported missense mutation, c.517C>T encoding p.R173W in the HMBS gene. Targeted mutation analysis was performed by PCR amplification of extracted DNA followed by allele specific primer extension analysis, in five first-degree relatives. Among these, four were heterozygous for the same HMBS gene mutation (Fig. and Table ).\nSince heme arginate is not available in Sri Lanka the patient was managed only symptomatically. Carbohydrate loading with intravenous dextrose and oral carbohydrates was the only feasible option. All the medications used for symptomatic management were checked for safety in acute porphyrias. Patient was discharged from the ward after symptoms gradually improved over 6 days to a degree that he can be managed as an out-patient. Response to treatment could not be assessed due to unavailability of quantitative tests to measure urinary aminolevulinic acid (ALA) and PBG in Sri Lanka. The patient was educated regarding precipitating factors of acute porphyria. A diagnostic card with information regarding medications to avoid was provided to the patient. Patient was followed up at the clinic with regular renal functions, hemoglobin and blood pressure monitoring. Follow up of the patient over 1 year following diagnosis revealed that patient suffered from two mild attacks which didn’t require in-patient management. Nerve conduction studies were not carried out because neurological symptoms were not observed in-between acute attacks.\nPre-symptomatic relatives who inherited the HMBS mutation were also advised to avoid the trigger factors of acute attacks such as certain medications, fasting, alcohol and hormones. The brother of the proband was counseled regarding the risk of his children inheriting the HMBS mutation and recommended targeted mutation analyses for both children. |
A 24-year-old male patient was referred to our hospital from a rural peripheral health center with complaints of abnormal behavior and seizures. He was born of a nonconsanguineous marriage and was the only brother of five sisters, all of whom were healthy. No history of any developmental delay or mental retardation was present. On examination in psychiatric clinic, he confided to having severe anxiety related to being alone outside his home and in crowded places. He was also fearful of traveling by road and particularly so in buses. Exposures to these situations lead to severe anxiety or panic attacks. His daily lifestyle was severely restricted because of avoidance of these situations. He also had partial seizures for the past 5 years for which he was under treatment. Facial asymmetry was noted on the left side of face involving the maxillary and periorbital region. A line was also noticed running obliquely in the left frontoparietal region. The cause of his facial asymmetry was not immediately apparent. The electroencephalogram and routine laboratory tests were normal. A contrast computed tomography (CT) of the head was requested for ruling out any organic cause for seizure activity. CT head showed presence of a lipoma of size 6.7 mm × 4.5 mm × 5.5 mm in the right quadrigeminal plate cistern with CT attenuation values ranging from −50 HU to −60 HU []. No other intracranial abnormality such as calcification or white matter hypodensity was noted. Careful inspection of CT images revealed atrophy of the subcutaneous soft tissues and skin in the left frontal region []. The volume rendering showed it to be extending in a diagonal fashion along the left frontoparietal location []. The left supraorbital ridge and frontal bone were also found to be depressed []. Inquiry into history from parents revealed that the patient had undergone spontaneous regression of the left-sided facial soft tissues at the age of 5 years. The process had arrested by itself after 2 years and was stable since then. The seizure activity and the psychiatry complaints had developed later at the age of 19 years. Based on the presence of facial hemiatrophy, a diagonal line on scalp of forehead, the CT findings and typical history, diagnosis of PRS was made having possible overlap with linear scleroderma or en coup de sabre. The psychiatric ailment was diagnosed as agoraphobia with panic attacks. The patient was scheduled for magnetic resonance imaging (MRI) brain; however, the study had to be aborted as he developed panic attack during the scan. |
A 24-year-old male, unmarried, agricultural worker by occupation and without any drug habits had come with complaint of erectile dysfunction and depressive symptoms secondary to that. These complaints started around 4 months back, when his father was looking for a bride. Prior to this, he reported never having had sexual ideas and his friends tried to give him advice on how to show sexual prowess to a woman on her first night. That night when he went to sleep he felt some shadow crawling over his body and he had spontaneous ejaculation. This was first time such thing had happened to him. His father reassured him that night. Next two nights also he had spontaneous ejaculation at night and this terrified the patient that all his semen may have drained out and consequently, his ability to impregnate a woman and his energy to carry out physical work had been depleted significantly. His friends had advised him to masturbate and told him to continue the technique to get semen out at least once a month. To his dismay, he found that he was unable to do as there was ‘no rigidity’ in the penis. When questioned about the nature and importance of semen, he said that semen is important for producing children and having a family but added that he had no idea where it came from. He consulted a Siddha practitioner for his problems and was told that if semen was not coming out through its proper channel, it would fill other body fluids and also come out in the saliva. That doctor took sample of saliva in small bottles and told the patient that after testing he had come to the conclusion that they are containing sperms. Along with this presumed ‘imbalance in bodily fluids’, he had developed depressive symptoms secondary to semen loss when he presented to us and had attempted suicide once during this period.\nAfter evaluation, he was admitted as a case of severe depressive disorder. During ward stay and with various treatments there was improvement in biological functions and consultations also revealed acute gastritis for which he was treated adequately. He continued to believe that semen is coming out through saliva. By its taste and smell he was able to confirm what the previous Siddha doctor had told him. Few weeks later, he observed that the size of his penis had shrunken and corroborated this observation by taking his measurements. He began to worry about how he was going to live without his own family and how he would become a laughing stock among people for losing his ‘maleness’ or ability to impregnate a woman. He also worried about earning his livelihood by farming due to accompanying weakness.\nDuring assessment we found that he has hypolibidinal state prior to onset of these problems. There were no local pathology and sex hormone study and thyroid function tests did not show any abnormalities. |
A 59-year-old woman was referred to the Department of Oral and Maxillofacial Surgery at Osaka University Dental Hospital with the chief complaint of a radiolucent lesion in the left body of the mandible, detected on a routine radiologic dental checkup []. On clinical examination, there was no evidence of buccal swelling or bony expansion. Mobility of the teeth in the region was not observed. Further diagnostic studies included computed tomography (CT) and magnetic resonance imaging (MRI). Preoperative diagnosis was strongly suggestive of a benign tumor of the nerve or vascular origin and probably schwannoma, neurofibroma, or hemangioma located in the left body of the mandible. The patient wished for follow-up rather than operation. After 2 years’ follow-up, the radiolucent lesion had slowly grown []. Preoperative CT scan revealed the lesion in the left body of the mandible []. The patient decided to have an operation on the lesion. Since the lesion was suspected to be schwannoma, neurofibroma, or hemangioma, a preoperative biopsy was performed, leading to a diagnosis of venous malformation from histopathological analysis. Under general anesthesia, the lesion was approached with a submarginal incision from the lower left second premolar tooth to the lower left third molar tooth. The lower left second and third molar teeth were extracted. As we were considering conservation of the inferior alveolar nerve or digital nerve reconstruction of the inferior alveolar nerve with a biodegradable conduit, the mental nerve was identified, and the inferior alveolar nerve was freely separated from the surrounding mandibular bone between the mental foramen and the lesion using the piezosurgery system [Figure and ]. The lesion was removed surgically with no significant bleeding, and the nerve could be conserved []. The nerve was returned to its original place. The surgical specimen revealed a red tumorous mass of the lesion []. Histopathological findings showed irregularly sized vascular spaces surrounded by dense connective tissue, which led to a diagnosis of venous malformation []. After operation, she reported an initial change in sensation (paresthesia). To evaluate sensation, the Semmes–Weinstein mechanical esthesiometer is usually used.[] The sensitivity recovered from 3.61 to 2.36 after 6 months evaluated by Touch Test® Sensory Evaluators. At the time of writing, the course of the patient has been good, with no symptoms of recurrence [Figure and ]. |
A 57-year-old man had an industrial accident in August 2009. He was diagnosed with a C2 vertebral body fracture and high cervical SCI at the C3 level. On physical examination, he did not have key muscle activity in all extremities and the last intact sensory level was the C3 dermatome. He felt deep anal pressure but he did not have voluntary anal contraction. According to the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), he was classified as C3 AIS B (American Spinal Injury Association impairment scale B). In February 2010, he had his pulmonary function tested for the first time and his pulmonary function was severely decreased. The tidal volume, vital capacity, and peak cough flow were 220 mL, 800 mL, and 130 L/min in the supine position, respectively. Continuous hypercapnia, defined as partial pressure of carbon dioxide in arterial blood (PaCO2) of over 45 mmHg, was also recorded. In the diaphragm fluoroscopy, the average length of the diaphragm apex was decreased on both sides up to 10 and 15 mm during tidal breathing and 20 and 40 mm during deep breathing []. We initially applied a portable ventilator with volume controlled assisted ventilation mode. Respiratory function gradually improved through active pulmonary rehabilitation techniques such as air stacking exercise with an Ambu bag and sputum expectoration by mechanical insufflation-exsufflation and accessory respiratory muscle training. However, he was readmitted to the intensive care unit due to septic shock caused by urinary tract infection one year after the last discharge from the hospital. Unfortunately, a tracheostomy was performed again, and then the step-by-step pulmonary rehabilitation program was prescribed, as mentioned above. Although we could remove the tracheostomy tube, total weaning did not take into account respiratory insufficiency in SCI, concomitant sleep apnea and the aging. We applied an intermittent NIV with pressure support ventilation mode during the night time to solve the problem of hypercapnia. Initially, we used a nasal mask but he could not adjust to the mask due to mouth opening during sleep (). Then, we applied a full face mask, which can cover the nose and mouth at the same time, for reducing oral air leak. However, he could not take off the mask by himself, which blocked his voice, and this situation caused great fear. Because the main problem was a limitation of communication, we focused on the key motion which the patient could make for developing a solution.\nWe designed a novel alarm system after discussion with the Department of Biomedical Engineering. The system consisted of a microcontroller board (Arduino UNO; Arduino, Somerville, MA, USA) which is an open source electronics platform based on an easy-to-use hardware and a sound generator, a pressure transducer using an existing endotracheal tube (). The system had been programmed with the open-source software (Arduino software). The best clear and easy intended motion of the patient was neck rotation. Sensing of the intended motion was performed by a balloon of the endotracheal tube placed under the pillow or head of the patient (). The sensor reacted only when the pressure exceeded 10 cm H2O. In this setting, the sensor ignored unnecessary signals such as a smooth ascending pressure or an unintended pressure below this threshold value. If the pressure exceeded 10 cm H2O, an intended neck rotation by the patient provoked a single beep sound made by the sound generator for the patient. More than three consecutive beeps within 3,000 ms created a loud alarm sound through speakers for the nurse or caregiver.\nAfter applying this pressure alarm system, he could easily adapt to the full face mask without anxiety and was discharged home without any complications. |
Seventy-one years old male patient was admitted to the hospital with the complaints of a swelling and discharge on the anterior neck wall. These complaints were present for 10 days. In the medical history of the patient, similar complaints were present 15 to 20 days after FNAB of thyroid gland performed four years ago, and these complaints had spontaneously resolved with antibiotics within one month. At that time, no surgical intervention related to this complaint was performed.\nPhysical examination of the patient revealed a sinus opening with seropurulant discharge located at midline, two cm caudal to thyroid cartilage and the tissues surrounding the sinus opening were moderately swollen and hyperemic (). There was also a palpable nodule four cm in size with circumscribed margins located in the left lobe of thyroid gland. There were no palpable lymph nodes in the cervical region. Other systemic examinations were normal. White blood cell count, neutrophil, eosinophil, C reactive protein, sedimentation rate, liver and thyroid function tests, and antithyroid antibodies were all in normal range. In cervical USG, there was a nodule 46 × 32 mm in size with multiple calcifications and mixed echogenities in the left thyroid lobe and it was pushing trachea away from the midline. At thyroid scintigraphy, the nodule was hypoactive and almost filling the whole lobe of the thyroid gland. In addition to this nodule, there was asymmetrical thickening of tracheal wall on the left side starting from the level of epiglottis and obliterating lateral recess at the level of Eustachian tube in computerized tomography (CT).\nSwab culture of the wound revealed no microorganisms. The contrast material given through the opening of the sinus passed neither to trachea nor to esophagus and the sinus was completely intrathyroidal (). USG-guided FNAB with 25 Gauge needle was performed for the nodule located in the left lobe of thyroid gland. The cells, with prominent nucleus and elongated cytoplasm, fragmented connective tissue elements and polimorphonuclear cells were noted at cytological evaluation. The result of the FNAB was suspicious for malignancy. PET-CT was also performed with the suspicion of undifferentiated thyroid malignancy infiltrating the skin. Moderate 18-FDG uptake seen on the left side of the neck from epiglottic level to cephalad direction was interpreted as an inflammatory process. However, 18-FDG uptake by thyroid nodule was reported to be suspicious for malignancy. With these radiological and clinical findings, bilateral total thyroidectomy was performed. Methylene-blue dye was injected through the opening of sinus tract at the beginning of surgery. Although the dye was totally intrathyroidal, the sinus tractus, prethyroidal muscles, and the skin were resected with the specimen. The sinus tract was continous with the left lobe of thyroid gland and there were severe inflammatory adhesions around the ligament of Berry at this region. The dissection of the left lobe was quite difficult, and central lymph node dissection was also performed.\nEsophagocutaneous fistula developed at the third postoperative day. Oral food intake was stopped and the fistula spontaneously resolved with parenteral nutrition. The histopathological examination of the specimen revealed that the sinus tract was formed by spindle cell proliferation and the tract was fixed to prethyroideal muscles. Spindle cell proliferation and inflammatory cell infiltration within vascular structures were responsible for adhesions between thyroid gland and prethyroideal muscles. The spindle cells had myofibroblastic features with no nuclear atypia. Hurtle cell changes confined within the bounderies of the nodule were noted outside the fistulous tract. These areas were stained with CD56, CK19, and HBME1 and not with Gal3 and P53. The other lobe and isthmus were made up of normal thyroidal tissue. All nine lymph nodes dissected from central region were reactive in nature. The diagnosis of the nodule was benign and the patient is still followed with no complications. |
A 4 year old male presented to the emergency department with the history of falling off a ladder 3 hours prior. He landed on his face on a wooden spike that embedded itself in the right orbit. His local hospital is not equipped to deal with ocular emergencies so he was transported by car for approximately 40 km to an eye hospital. Unfortunately, no anesthetist was available and the patient was referred to our emergency department. No treatment was instituted prior to referral. To reach our hospital, the patient had to backtrack 22 km and travel an additional 18 km, 40 km in total over rough roads (Figure ).\nOn arrival in our emergency department the child was fully conscious. He was hemodynamically stable with no airway or respiratory compromise. He had no past medical history and was not taking any regular medication. History revealed he was up to date with his immunizations, but history was considered unreliable. He lived with his parents and younger brother in a town in Eastern Nepal. On further examination, no other injuries were found specifically the cervical spine. Gross neurological examination was normal. The foreign body, still in situ, was a sharp wooden peg. It had penetrated the right orbit along the medial side. The cumbersome protruding object constituted a rock and a wire mesh attached to the wooden peg. The weight (approx. 1 kg) and shape of the object made stabilization of the object particularly difficult. It was impossible to evaluate the eye further due to the eye being obscured by the protruding object (Figure ).\nIntravenous access was established. Pain was managed with morphine and prophylactic antibiotics were initiated. CT scan was arranged. Patient was sedated with ketamine and midazolam. The patient was transferred to CT scan with the relative supporting the foreign body all the time. The contrast CT was initially reported by a radiology resident as follows, “foreign body lateralizing globe and penetrating through the medial part of the orbit extending through orbital apex to middle cerebral fossa and just impinging right cerebellar hemisphere. Minor contusions in right cerebellum and hemi sinus ethmoidal sinus are suspected. The length of the penetrating part was around 9 cm” (Figure ). Upon immediate ophthalmology consultation, a decision to remove the foreign body was made. Again, anesthetists were unavailable for timely removal of the foreign body as they were busy in theatre. Due to the time delays pre presentation, it was decided that removal of the foreign body should occur as quickly as possible. The ophthalmologist and the emergency physician removed the foreign body, with the aid of sedation (ketamine, morphine and thiopentone). A lateral canthotomy was performed (Figure ).\nAfter removal of the foreign body, the eye examined as follows: axial proptosis, mid dilated pupil, clear lens, and clear media. There was no evidence of globe penetration. Pilocarpine drops were instilled and ciprofloxacin ointment used before padding and bandaging the eye. Oral acetazolamide was started. Otolaryngologist and Surgical specialties were consulted with no further management advised. There was no neurosurgeon at the hospital available for consultation. The patient remained in the emergency department for observation (Figure ).\nAt 48 hours the patient remained neurologically intact. His dressing was removed and his eye examined as follows: subconjunctival hemorrhage, clear cornea, mid dilated pupil, clear lens. Patient had no light perception in the right eye. A repeat CT showed no deleterious changes. On discharge on day 3, patient was advised to follow up with a neurosurgeon in another city.\n3 month follow up revealed the following examination: mild proptosis, no blinking, no eye movement, pupil dilated and non-reactive, cornea clear and no light perception. Fundoscopy revealed a pale optic disc. Eye taping was advised. At 6 months, he had regained some blinking, but had not recovered any light perception. |
A 33-year-old male patient presented to a tertiary care hospital with intermittent abdominal pain, nausea, vomiting, constipation and numbness of bilateral lower limb extremities, of 3 days duration. He was conservatively managed in a surgical unit as partial intestinal obstruction and was awaiting diagnostic laparoscopy. The patient developed confusion and found to have systemic hypertension, and, therefore, was transferred to a medical ward for further management. He is a non-diabetic and did not have a previous history of hypertension. The drug history revealed usage of over-the-counter analgesics for 6 weeks.\nThe past medical and surgical history revealed similar neurovisceral attacks requiring five acute hospital admissions over 2 years, which ended up in questionable diagnoses. The sixth acute attack raised the suspicion of an acute porphyria. The first attack in January 2013 led to a diagnosis of appendicitis. Because the symptoms worsened following the surgery an emergency laparoscopic exploration was done. But the exploration revealed no cause to explain the worsening symptoms. The second attack was managed as sinus tachycardia and he was started on beta adrenergic blockers. The third attack which was associated with a fever was conservatively managed for a questionable renal colic. Forth attack was complicated with transient hyponatremia and transiently high serum creatinine levels. These complications were attributed to a questionable interstitial nephritis based on the fact that patient had used 50 mg of diclofenac sodium twice a day for 6 weeks, repeating the prescription given by a general practitioner. Another attack in 2015 was managed as partial intestinal obstruction and diagnostic laparoscopy was done. In all these presentations, findings from the ultra sound scans and diagnostic laparoscopy did not support a diagnosis of intra-abdominal pathology.\nOn examination he was thin built (BMI = 20 kg/m2) and pale. Brachial blood pressure was 160/90 mmHg. There were scars of previous appendectomy and laparoscopy surgeries on the abdominal, but, otherwise, the abdominal examination was unremarkable. Muscle power was 4/5 in all four limbs (could not move against a good resistance).\nThe laboratory investigations performed during this admission showed severe hyponatraemia of 115 mmol/L (136–145) with serum osmolality of 255 mOsmol/Kg (275–295) and urine osmolality of 460 mOsmol/Kg (50–1200 mOsmol/Kg). Serum creatinine concentration was 106 µmol/L (80–115) with blood urea level of 20 mg/dL (6–20). Hemoglobin concentration was 8.2 g/dL (13.5–17.5) and the red cell morphology was normochromic and normocytic. The total cholesterol level was 282 mg/dL (5th to 95th centile; 142–258) with LDL fraction of 225 mg/dL (5th to 95th centile; 78–185). Serum ferritin level was 646 ng/mL (20–250). Arterial blood gas analysis was suggestive of a metabolic acidosis. Echocardiogram showed evidence of left ventricular hypertrophy. There were no significant radiological findings in abdominal X-ray film or abdominal ultrasonography. Blood lead concentration was 3 μg/dL (< 5 μg/dL).\nA urine sample collected during the acute attack was sent to the Department of Chemical Pathology for biochemical analyses. On standing the urine sample gradually turned dark brown. The Watson and Schwartz test for urinary porphobilinogen (PBG) was positive (Fig. ). Spectrophotometry of urine for total porphyrins showed a “Soret band”. Urine total porphyrin level, calculated using Allen corrected absorbance of the urine sample was 5505.5 nmol/L (< 300 nmol/L). Genetic studies were carried out in an overseas laboratory. The full analysis of HMBS gene was performed by PCR amplification of extracted DNA followed by exon specific primer extension analysis of all exons, exon intron boundaries and promotor regions. The gene analysis revealed a previously reported missense mutation, c.517C>T encoding p.R173W in the HMBS gene. Targeted mutation analysis was performed by PCR amplification of extracted DNA followed by allele specific primer extension analysis, in five first-degree relatives. Among these, four were heterozygous for the same HMBS gene mutation (Fig. and Table ).\nSince heme arginate is not available in Sri Lanka the patient was managed only symptomatically. Carbohydrate loading with intravenous dextrose and oral carbohydrates was the only feasible option. All the medications used for symptomatic management were checked for safety in acute porphyrias. Patient was discharged from the ward after symptoms gradually improved over 6 days to a degree that he can be managed as an out-patient. Response to treatment could not be assessed due to unavailability of quantitative tests to measure urinary aminolevulinic acid (ALA) and PBG in Sri Lanka. The patient was educated regarding precipitating factors of acute porphyria. A diagnostic card with information regarding medications to avoid was provided to the patient. Patient was followed up at the clinic with regular renal functions, hemoglobin and blood pressure monitoring. Follow up of the patient over 1 year following diagnosis revealed that patient suffered from two mild attacks which didn’t require in-patient management. Nerve conduction studies were not carried out because neurological symptoms were not observed in-between acute attacks.\nPre-symptomatic relatives who inherited the HMBS mutation were also advised to avoid the trigger factors of acute attacks such as certain medications, fasting, alcohol and hormones. The brother of the proband was counseled regarding the risk of his children inheriting the HMBS mutation and recommended targeted mutation analyses for both children. |
The subject was a 14-year-old female patient with EDS hypermobile type. She had perceived her recurrent shoulder dislocation from childhood. She complained of shoulder pain and restriction of activity in daily life (ADL) during shoulder movement due to shoulder instability. There was no abnormal finding from radiological examination. There were no psychiatric diseases. She could dislocate her bilateral shoulder joint voluntarily. Her chief complaint was the bilateral shoulder discomfort and instability during writing motion. On her first examination by a doctor, she was treated conservatively, using the exercise program for 6 months []. On her second examination by a doctor at another hospital, she was also treated conservatively, adding an orthosis for MDI to the exercise program (intervention A) []. The patient received an explanation about this report and provided informed consent that complied with the Declaration of Helsinki.\nThe patient had a slight build with a height of 156 cm and weight of 44 kg. To evaluate her bilateral shoulder function, her active range of motion (ROM) (), sulcus sign, and shoulder instability in each direction were measured on the time point at the beginning of intervention A. Her passive ROM was not able to be measured because her humeral head was dislocated so easily with passive ROM exercise. Muscle strength was also difficult to measure due to dislocation with ease. Although her humeral head dislocated many times during rehabilitation, the doctor allowed her rehabilitation because there was no increase of persistent pain and she could centralize her humeral head voluntarily. The modified Rowe score was measured to evaluate shoulder instability [] ().\nEDS patients usually suffer from many musculoskeletal symptoms, even when they grow up []. On the other hand, it has been demonstrated that patients who have shoulder MDI will recover shoulder function during movement after rehabilitation programs [, ]. However, the prognosis of this patient was challenging because patients with EDS were excluded from the rehabilitation programs.\nThe AB design includes some measurement of outcome variables throughout a baseline control/comparison phase A and then throughout another intervention phase B []. In the early part of rehabilitation, the patient was provided with an exercise program for MDI with orthosis [] (intervention A). The term was continued for 3 months. In summary, she performed shoulder isometric muscle exercises using the TheraBand (Hygenic, Akron, Ohio) for the rotator muscles and scapula stabilizers with supervision by a physical therapist. The program includes five exercises: resistance exercises of shoulder abduction, internal rotation, external rotation, extension, and flexion. Assistance from a physical therapist to stabilize the humeral head was often needed so as not to dislocate it. The resistance during exercise was gradually increased according to her performance regarding shoulder dislocation. A physical therapy session was provided one to two times a week. Each session was 40 minutes. Compliance of home exercises was no more than about twice a week.\nIn the latter part of rehabilitation, the patient was instructed to perform the Watson program for MDI [, , ] (intervention B). The Watson program is an exercise program that aims for coordination of scapular muscles with strengthening rotator cuff and deltoid muscle. This phase also lasted for 3 months. In summary, this program consists of six stages that focus on retraining specific scapular control before any deltoid and rotator cuff exercise. The patient should make progress with exercise into functional and sport-specific ranges. She also received a physical therapy session one to two times a week. Each session was 40 minutes. In the first stage, correction of her humeral head by a physical therapist was effective during shoulder movement. The scapular control exercise was performed with assistance. She could make progress to stage 2 within 1 month. She could finally perform stage 5 exercises after 2 more months of rehabilitation. Compliance of home exercises was more than 3 days a week.\nThe intervention was performed from July 2017 to January 2018. The patient was followed up until July 2018. The active ROM increased, while the passive ROM was not increased, due to easy dislocation (). The sulcus sign was still positive after intervention and at the time point of the final evaluation. The shoulder instability score was improved after intervention B (). She felt no pain and discomfort during writing motion after the time point. She could also control both humeral heads within proper position during ADL. A fair outcome was achieved by the final evaluation. |
The patient was a 32-year old woman with a one-year history episodes of cholecystitis treated conservatively. She did not have any other disease history. After an abdominal magnetic resonance imaging that confirmed multiple gallbladder stones (Fig. a), an elective LC was performed without intra-operative complications. The recovery was uneventful and the patient was discharged two days after operation. On the second day after discharge, the patient developed severe right upper abdominal pain and she was sent to our emergency department at 8:30 pm. At arrival, her heart rate was 110 bpm and the blood pressure was 80/55 mmHg. The hemoglobin dropped to 86 g/l from 127 g/l. The CT scan showed a 10.9 × 12.5 × 6.6 cm ISH in the right liver without obvious free fluid in abdominal cavity (Fig. b). Two hours after fluid resuscitation including 2 U red blood cell, the hemoglobin further declined to 78 g/l and the hemodynamics remained unstable. The abdominal pain was not relieved, after intravenous analgesics. A Doppler ultrasound was performed, two hours later and it found the hematoma had increased in size. Active intrahepatic bleeding was suspected. We called radiologist for consultation, however, the interventional angiography and embolization was not available at mid night. We explained the potential risk of sudden rupture of hematoma during conservative methods which may cause sudden death, to the patient and her relatives. After careful consideration of the continuous decline of hemoglobin, unstable hemodynamics after fluid resuscitation, we explained our surgical plan to the patient and her relative. We planned to perform laparoscopic exploration at first, if the hematoma continued to expand, we would evacuate or drain it, if not, we would put a drainage tube under liver which could serve as an early warning of rupture. The patient requested surgical method to reduce the risk of sudden death. Therefore, an emergency laparoscopic exploration was performed under general anesthesia. The ISH was confirmed (Fig. c). Four U red blood cell and 400 ml fresh frozen plasma were transfused. After fluid resuscitation and blood transfusion, her hemodynamic became stable. During the 3-h intra-operative observation, the hematoma did not expand. Therefore, a non-sucking drainage tube was placed under the liver and she was sent to ICU ward. Next morning, she was transferred to the ordinary ward. The upper abdominal pain gradually relieved. Five days after the laparoscopic exploration, another CT scan showed that the hematoma was largely resolved and we removed drain tube (Fig. d). She was discharged, 10 days after readmission.\nTotally, 13 papers, including 16 cases of ISH after LC were reported from 1994 to 2015 (Table ). Nearly half of the patients had instability of hemodynamics. All of the cases were female patients. Age of patients ranged from 25 to 78. All hematomas were mainly located in the right lobe of liver, and some of them extended to the left lobe of liver. Only one case was ruptured at diagnosis. Hepatic capsule laceration was found in two cases, one of whom also took NSAIDS (non-steroids anti-inflammatory drugs) to control the pain after operation. Totally, 58.8% of patients took NSAIDS to control the post-operative pain, and most of them used Ketorolac, however, 35.3% of the patients still did not have definitive risk factors. The time interval to diagnose ISH after LC ranged from seven hours to six weeks. They were diagnosed most commonly (35.5%) within one day after LC. All patients had abdominal pain and 47.1% of the cases developed hypovolaemic shock.\nTreatment strategies included: conservative treatment (antibiotics, blood transfusion, strict bed-reset), percutaneous drainage under CT or B ultrasound guidance, selective embolization of the bleeding vessel, laparoscopic exploration and laparotomy. Eighteen percent of patients had stable condition without fever and underwent conservative treatments. The only case of angioembolization was complicate by infection and required percutaneous drainage. For the patients with stable condition, fever and serious compression of inferior venal cava (IVC) always were indications for percutaneous drainage under CT or B ultrasound guidance. In these 17 cases, 29.4% of the patients underwent percutaneous drainage. For the patients with hemodynamic instability, emergent reoperation was adopted. Totally, nine cases underwent reoperation, including two case of laparoscopic operation and seven cases of laparotomy. For our case, we only performed laparoscopic exploration and did not perform evacuation or drainage of the hematoma, since the hemodynamic became stable after plenty fluid resuscitation and the hematoma did not expand, during the 3 h of intra-operative observation. For another case, laparoscopic exploration found small capsule laceration, and hemostasis was performed. In the seven cases of laparotomy, six patients underwent evacuation and drainage of hematoma, only one case underwent only laparotomy without evacuation or drainage.\nAll patients survived. Most of patients stayed one to two weeks after readmission, however, the longest hospital stay was up to 31 days after reoperation. |
A 56-year-old male patient presented with right aural fullness and nasal obstruction that had been evident for three years. He had no juvenile history of nasal disease or otitis media. At the first admission, the secretory otitis media (SOM) in the right ear was investigated, and a solid, smooth and poorly mobile tumor was detected in the posterior part of the nasal cavity and nasopharynx (). Initial diagnosis of a transnasal biopsy sample suggested fibroma. Computed tomography (CT) examination showed that the tumor occupied the nasopharynx, and a high-intensity shadow was evident within it (, and ). Although CT was unable to reveal the origin of the tumor, the high-intensity shadow extended in the direction of the ET () and thus the tumor was thought to have arisen from the ET. Magnetic resonance imaging (MRI) showed that the tumor consisted of heterogeneous components with T1 enhancement (), and that the inside of the tumor appeared more contrasted with T2 enhancement (). The T2 enhanced image showed a smooth line of the nasopharyngeal mucosa, suggesting no tumor invasion in the nasopharynx. Enhancement with intravenous contrast medium revealed no remarkable features by both CT and MRI.\nUnder a preoperative diagnosis of nasopharyngeal tumor possibly involving the Eustachian tube, partial resection of the nasopharyngeal tumor was performed by transnasal endoscopic surgery to obtain a definitive diagnosis and to relieve the nasal obstruction. A hard mass with a smooth surface was seen to arise from the pharyngeal ostium of the ET (). The mass was reduced piecemeal using scissors and sharp forceps, taking care to preserve the normal mucosa of the nasopharynx. The tumor was not attached to the nasal and pharyngeal mucosa and protruded into the ET. However, its point of origin could not be detected precisely because the surgical devices were unable to reach the inside of the ET. Part of the tumor in the ET therefore remained, and a tympanostomy tube was inserted into the right ear to resolve the SOM. Pathological examination showed that the tumor consisted of bony tissue, fat cells and proliferated fibroblasts, and was diagnosed as osseous hamartoma arising from the ET (). Ten months after the reduction surgery, the tumor was not markedly enlarged (). The nasal obstruction, right aural fullness and conductive hearing loss that were evident on initial presentation were diminished after the surgery. |
A 51-year-old Madheshi woman came to our neurology clinic with the chief complaint of sudden decrease in the tone and texture of her voice for the past 15 days. Her voice was very feeble but understandable and she noticed it was better by the time she got up from her bed only to worsen through the day to become nearly inaudible. She had noticed a slight change in her voice texture in the first several days which she had ignored in the beginning as it was not worth bothering about. She had no complaint of choking or coughing or aspiration or any throat discomfort. When asked for any other related and/or unrelated things she noticed in her habits, she complained about having constipation for many years otherwise she was apparently well. She had never visited hospital for any problem and no interventions had been done in the past. She denied smoking tobacco, drinking, or any other recreational drugs abuse. There was no one in her family or her parents’ family with any kind of known chronic disease. Her biological father had controlled hypertension with medication. Her psychosocial history was not significant. She came from a middle income family, and she had retired recently as an accountant for a small company. Her symptom progressively aggravated in later days to the extent that she not able to produce sound properly. It usually waned in the early morning or after enough voice rest only to wax throughout the day.\nOn physical examination, a systemic examination did not reveal any abnormality. On neurological examination, her muscle power was intact: 15/15 on Medical Research Council (MRC) scale. She had no imbalance and was able to tandem on walking. An examination of her gag reflex and other cranial nerves revealed no abnormal reflexes. Deep tendon reflexes were intact. Her speech articulation was intact and revealed no scanning of speech. Speech production was adequate and non-painful; her tone was non-nasal but the intensity was low and slow. Other neurological examinations also did not reveal any abnormality. Other systemic examinations were also non-significant.\nConcerned with her problem, she had visited an ear, nose, and throat (ENT) department for her problem and was screened for possible laryngeal disorder for hypophonia. There was no obvious laryngeal pathology found and the treatment initiated by the ENT department had no satisfying outcome. She visited our neurology clinic. The differential diagnoses considered were Parkinson’s disease (PD) or a bulbar variant of motor neuron disease (MND), and MG. MRI scanning of her brain and screening of her whole spine appeared normal. Laboratory investigations including a hematologic panel, infectious disease screening panel, and myopathy panel involving creatinine kinase and creatine phosphokinase-N-acetyl cysteine (CPK-Nac), were all within normal range. She was suspected to have MG as other diseases were ruled out. A Quantitative Myasthenia Gravis (QMG) test was performed on our patient and her QMG score was 1 (for speech). She was given neostigmine challenge test with intravenously administered injection of 2.5 mg neostigmine and the result was positive after more than 30 minutes. There was significant improvement in her voice quality and she felt her fatigability improved as well. She gained a good quality of speech within an hour. QMG scoring was repeated, and she scored QMG score of 0. The longer duration of the medicine gave us enough time to assess the improvement. The laboratory test for the AchR antibody (AchR ab) is not available in Nepal. We had to send AchR ab to India, and the report received after waiting several weeks was positive. Quantitatively, it was significantly higher, 1.11 nmol/l than the normal range (0.0–0.4 nmol/l). She was later maintained on 60 mg of orally administered pyridostigmine with 12 hourly administrations and subsequently increasing to 6 hourly along with steroids.\nPlain and contrast-enhanced computed tomography (CT) scans of her head, neck, and chest were done, and they revealed a mass in the anterior mediastinum: a well-defined, smooth outlined, approximately 4 × 2.5 × 2 cm, soft density nodule showing poor enhancement in the anterior mediastinum in the thymic space with maintained fat plane with the adjacent vessels and structure. Thymoma was suspected and she was sent to the surgical department for further surgical intervention.\nShe returned to our neurology clinic around 2 months after her first visit to us as a follow-up visit. She had undergone a successful surgery. She was doing well. Prednisolone was already tapered off. She was tapering down pyridostigmine as well, with a maintenance dosage of pyridostigmine 60 mg 12 hourly and planned to put off the medication. Her voice was clear and well maintained and she had no complaint of fatigue. She had no fresh complaint and was doing all her daily and professional activities very well. |
An 88-year-old woman with dementia was brought to our emergency department (ED) due to two rapidly growing painful nodules on her right lower leg. Due to her dementia, the medical history was mostly obtained from her son, and the informed consent for this case report was signed by her son. She had poor performance status and was capable of only limited selfcare (Eastern Cooperative Oncology Group Performance Status Scale grade 3). She was confined to the bed or chair most of the time and was normally cared for at a nursing home. According to her son, the nodule on her right anterior lower leg appeared about 3 weeks previously, and she had begun to complain of right lower leg pain. The other tumor was located at the medial aspect of her right lower leg, and it had appeared only 1 week previously. The two nodules had grown rapidly, with increasing pain. She denied experiencing fever, night sweats or weight loss in recent months. She had been seen at a local clinic prior to her ED presentation. At the clinic, antibiotics had been prescribed and a needle aspiration of the larger skin lesions was attempted. No pus or fluid could be aspirated, and despite antibiotic treatment, the lesions were getting larger. Thus, she was transferred to our ED for further evaluation and treatment.\nPhysical examination revealed two red and violaceous skin lesions over the right lower leg, ~9 and 3 cm in size, respectively. The lesions were erythematous, had ill-defined borders and were tender on palpation. Local heat and swelling of the skin around the lesions were also noted. Laboratory data, including a complete blood count and biochemical studies, revealed no significant findings. Contrast-enhanced computed tomography of the lower limbs revealed an abnormal space-occupying lesion about 9 cm × 7.5 cm × 3.3 cm in size, at the anterior aspect of the right lower leg. It showed heterogeneous density, heterogeneous contrast enhancement, and involvement of the subcutaneous area, superficial fascial planes and muscle layer. Another small non-specific lesion about 3 cm in size was located at the medial aspect of the right lower leg, with similar radiological features (). With a provisional diagnosis of skin tumors with associated cellulitis of the right lower leg, she was subsequently admitted to our institution for further investigations and antibiotic treatment.\nAfter admission, we performed incisional biopsy of the skin tumors () and the specimens were sent for histopathological examination and bacterial/tuberculosis/fungal culture. An antibiotic (oxacillin 2 g every 6 hours) was administered as empiric therapy for cellulitis. Three days later, bacterial culture and tuberculosis acid-fast stain testing provided negative results. A week later, histopathological results indicated a malignant lymphoma. There was diffuse infiltration of large, atypical lymphocytes with centroblast and immunoblast features in the whole dermis without epidermal involvement. Immunohistochemical staining revealed the following results: CK AE1/AE3(−), CD3(−), CD20(+), CD30(−), BCL-2(+), CD10(− to dim, −/+), BCL-6(dim,−/+), MUM1(+), Cyclin-D1(−), Ki-67(85%) and IgM(−) (). The results were compatible with a diffuse large B-cell lymphoma, either primary or secondary. There was no evidence of lymphadenopathy elsewhere. Based on clinical and histopathological findings, a diagnosis of PCDLBCL, LT was made. The case was later referred to a team of oncologists, and her family refused further management due to old age. Therefore, the patient then received hospice palliative care. |
A 41-year-old male was admitted to our hospital because of progressive pain and discomfort during swallowing. His past medical history was characterized by surgery for clival giant cell tumor complicated by ICA rupture 10 years ago. Indeed, in 2008, the patient was operated on by a fully endoscopic neuronavigation-guided EEA [, , , , , , , , , , ]. Thanks to the working angle and the better visualization offered by the EEA, we were able to maximize the tumor resection preserving at the same time craniovertebral junction stability and avoiding posterior fixation with his relative complications [, , , , , , , , ]. Indeed, the lesion was completely removed, but during the drilling of the surrounding healthy bone, there was a little tearing of the left ICA. Local control of the bleeding was challenging, but it was obtained by using topic hemostatic agents and balloon catheter for nasal tamponade. Immediately after surgery, the patient underwent angiography disclosing a pseudoaneurysm originating near the inferior meningohypophyseal trunk, which was successfully treated by embolization with coils (Fig. ). After 2 days of intensive care unit stay, the patient was transferred to our department, showing no neurological deficit and no signs of CSF leak or other CSF disturbances [, , ]. The nasal tamponade was removed 4 days after surgery, and no bleeding was observed coming out from the nasal cavity. The patient was dismissed and sent home 1 week later. Histologic evaluation of surgical specimens revealed the aspect of a giant cell tumor with no infiltration of the surrounding bone [].\nRadiation therapy was not performed. Brain magnetic resonance imaging with angiographic sequences was done regularly 3, 6, and 12 months after surgery in the first year and every 6 months for a further 2 years, then annually for a further 5 years. All these examinations showed no tumor recurrence or pseudoaneurysm repermeabilization.\nFor the presence of pain and discomfort during swallowing, the patient was referred to an otorhinolaryngology department, where, during endoscopic examination of the upper airways, a prolapse of the distal tip of the embolization coil wire was detected in the naso- and oropharynx. Then, after neurosurgical consultation, an urgent CT angiogram was performed which confirmed the extrusion of endovascular coils into the nasal and oropharyngeal cavity (Fig. ). The pseudoaneurysm was fully thrombosed despite the coil migration. For this reason, under local anesthesia and with the endoscopic technique, the extruded segment was trimmed at the level of its origin in the nasal mucosa. No bleeding, thrombosis or respiratory complications were observed. Endoscopic endonasal office-based control examinations were then performed regularly 1, 3, and 6 months after this event disclosing another coil migration. |
A 53-year-old woman was seen for transfer of care for metastatic lacrimal gland carcinoma. She had been diagnosed 2 and 1/2 years earlier in another state. She originally presented with progressive headaches, blurred vision, and right eye proptosis. CT imaging showed 2 separate masses involving the right orbit (a 2.5–3 cm mass under right orbital roof in the lacrimal area, also a 1 cm mass in the right orbital apex and superior orbital fissure) with possible intracranial extension. MRI imaging showed a right superior lateral orbital mass apparently originating from the lacrimal gland, invading into surrounding bone and possibly the brain parenchyma. The patient underwent subtotal resection of the locally advanced right orbital mass. The pathologic diagnosis was metastatic carcinoma of lacrimal gland origin. Immunohistochemistry was positive for keratin, CK7, and mammaglobin; negative for CK20, TTF-1, BRST-2 or estrogen or progesterone receptors.\nThe patient then received adjuvant radiation treatment to the right orbital area. She developed symptoms suggestive of metastatic disease less than a year later. MRI imaging of the thoracic spine showed 3 separate areas of intramedullary metastatic disease, at the T1 level, T6-7, and T12. There was no metastatic disease seen in the lumbar spine by MRI, and CT imaging of the chest and abdomen done at the same time also showed no evidence of metastases. The patient was again treated with radiotherapy. She later received radiation to new metastatic sites in the left cerebellum and an intramedullary lesion in the cervical spine.\nPET scan done following transfer of care showed evidence of disease progression in the cervical spine and proximal thoracic spine, as well as bilateral lung nodules and thoracic lymphadenopathy suggestive of systemic metastases. A biopsy was performed of one of the lung nodules, confirming metastatic adenocarcinoma. Due to the intramedullary location of spine mets, as well as prior radiotherapy to these sites, the patient was not considered a candidate for palliative surgery or radiation. Additional testing performed on the lung biopsy specimen showed no evidence of an epidermal growth factor receptor (EGFR) mutation; however, Her-2/neu expression was found to be amplified by FISH assay (Her-2/cen 17 ratio of 6.0). The patient was started on lapatinib, an oral tyrosine kinase inhibitor with systemic and central nervous system (CNS) activity against both Her-2/neu and EGFR, at a dose of 1500 mg daily. She tolerated lapatinib without significant side effects. CT imaging after 2 months of treatment showed stable disease.\nAfter approximately 5 months of treatment, the patient developed progressive weakness in both lower extremities. MRI imaging showed a moderate increase in size of the intramedullary lesion at T1, as well as increased cord edema and a syrinx extending to the C3 level. The findings were felt to be indeterminate for progression of malignancy versus radiation myelitis from prior radiotherapy. PET imaging was ordered to further evaluate this lesion. The PET study showed grossly stable size of multiple metastatic lesions but significant decrease in standardized uptake value (SUV) at all disease sites, consistent with a response to treatment. For example, the SUV of the largest lung lesion had decreased from 28.8 to 6.2, while the SUV of the T1 lesion had decreased from 18.6 to 6.6. Lapatinib was continued, based on the evidence of response and also as the patient was not considered a candidate for more aggressive treatment such as high-dose IV methotrexate. She was also treated with oral dexamethasone. Unfortunately, the patient's lower extremity weakness progressed to the point of paraplegia, and she had a steady clinical decline related to this. She ultimately died approximately 10 months after starting lapatinib and more than 4 years after original diagnosis. |
A 35-year old female patient reported to the Department of Oral and Maxillofacial Surgery with the chief complaint of pain in the lower right back tooth region of the lower jaw. The clinical and radiographic examination showed the lower right third molar to be impacted and surgical removal of the same was planned. Case history was documented. Patient’s general condition was fine and routine blood investigations were performed which were in normal limits. The surgical removal of the impacted lower right third molar was planned under local anesthesia and the case was assigned to a resident doctor. After pre-surgical aseptic procedures, the resident in charge loaded the syringe for injecting local anesthetic with 2.5 mL clear liquid in a vial which he assumed was local anesthetic solution. Within minutes after the injection was made (approximately 2 min), the patient started complaining of sharp burning pain and a feeling of intense discomfort at the site of injection. The procedure was stopped immediately. A quick clinical examination followed including monitoring of the vital signs which were within normal limits. The resident reported immediately to the consultant who examined the contents of the vial from which the syringe was loaded. A cursory examination revealed that the vial contained a clear liquid having an offensive pungent odor (resembling that of cadaveric chambers in anatomical dissection halls). Immediate surgical exploration of the site was planned under local anesthesia in order to limit the necrotic tissue damage. The pterygomandibular space was approached intraorally and extensive exploration and meticulous debridement were carried out along the track of possible chemical trickle down, a corrugated rubber drain placed and prophylactic broad spectrum antibiotics along with analgesics were prescribed for 7 days. While discharging, the patient was instructed to maintain good oral hygiene by using 15 mL povidine-iodine gargles twice to thrice daily. The patient was recalled on the fifth post operative day and the drain was removed. The patient didn’t however, report for any further follow ups for up to 26 days. After 26 days, the patient reported with swelling and discharge on the right submandibular region and limited mouth opening. The swelling was soft in consistency, with intermittent pus discharge. The discharge was collected and sent for culture and sensitivity. Interincisal distance at this time was measured and recorded as 15 mm. Orthopantomogram (OPG) and computed tomographic (CT) scans were advised. The OPG showed slight radiolucency in the angle region on the affected side, changes suggestive of osteolysis (). The CT scan on the affected side showed bulky medial pterygoid muscle with irregular margins and exhibited low attenuation. Anteriorly, an obliteration of fat plane i.e. fatty infiltration and diffusion was evident. Clinically correlated, these changes were suggestive of active inflammation of the medial pterygoid and subsequent necrosis (). The patient was admitted and empirical intravenous antibiotics were started and the same were continued as no growth was reported on the pus culture and sensitivity. A surgical intervention and re-exploration were planned under general anesthesia at this stage based on clinical and radiographic investigations. During the surgical procedure, the patient was intubated by fiber optic intubation due to decreased mouth opening. A submandibular incision was placed and the angle region exposed. Upon exploration, the right medial pterygoid muscle attachment on the medial aspect of angle of mandible was found to be necrosed (). The site was then simultaneously carefully exposed intra orally using Ginwalla’s anterior border of ramus approach () and necrotic muscle tissue was excised. Following surgical excision of the necrotic medial pterygoid muscle the surgical wound was closed intraorally with 3-0 vicryl and submandibular incision was closed in layers with 3-0 vicryl and skin with 4-0 prolene. The patient had an uneventful recovery and was discharged free of symptoms on the 6th post operative day. Gradually the mouth opening improved by the end of 2nd month and the patient is completely asymptomatic after 1 year of follow up. |
For more than seven years a 34-year old male patient had been complaining about recurrent discomfort of the upper abdomen and pain emanating to his back. Due to an increase of the preexisting disorders an abdominal CT-scan was conducted, which showed a cystic retroperitoneal tumor (figure ).\nThe patient was referred to us with the suspicion of a pancreatic neoplasia. After reviewing the CT scans we suspected a retroperitoneal neoplasia and completed staging which showed no distant metastases. An exploratory laparotomy was performed. However, no pancreatic mass was palpable intraoperatively. After mobilization of the right hepatic lobe and the right colic flexure, the tumor was located between the inferior vena cava and the left renal vein compressing and infiltrating those vessels. To obtain cranial and caudal control, the IVC was longitudinally exposed and secured with vessel loops. Subsequently, the IVC was gradually clamped. Prior to exclusion of the venous segment the patient received heparin intravenously. Due to the predominantly extravascular tumor growth (figure ) partial semi circumferential resection of the inferior vena cava, including the confluence and approximately 1.5 centimeters of the terminal left renal vein was performed and the tumor was resected 'en bloc'.\nIntraoperative assessment of the surgical margins by frozen section confirmed that a complete R-0 resection had been achieved. Vascular reconstruction was performed by a running suture of the caval resection margin and by reinsertion of the left renal vein into the proximal portion of the IVC at the site of the suture, since the length of the remaining left renal vein was sufficient after mobilization for a tension-free anastomosis. Intraoperative duplex ultrasound of the cavoplasty presented a good venous flow in the reconstruated vessels.\nThe postoperative course was uneventful, without impairment of the renal function or swelling of the lower limbs. Due to the vascular surgical procedure, the patient was therapeutically anticoagulated with heparin. Histopathology revealed a lowly differentiated leiomyosarcoma with an extension of approximately 7 cm in diameter; the resection margins were tumor-free. After interdisciplinary discussion of the case adjuvant radiotherapy was conducted with 57.4 Gy in particular due to the advanced tumor size.\nDuring the first follow-up after three months, the patient presented in good general condition. A CT-scan of the thorax and an abdominal MRI showed no indication for tumor recurrence. Under sustained anticoagulation with warfarin, the reconstructed vessels constituted without any pathological findings. Yet after six months the patient was still free of local recurrence, however, CT-scan showed a central hepatic lesion. A subsequently performed explorative laparotomy revealed inoperable disseminated hepatic metastases. According to interdisciplinary consent, the patient received regional hyperthermia as well as chemotherapy including ifosfamide, adriamycine, and etoposid during the following months. |
A 60 year old Caucasian male patient was referred to general surgery for multiple unrelated complaints including umbilical hernia and left arm lipoma. The patient also had an additional complaint of a fast-growing right leg mass located on upper lateral right calf distal to the knee. The patient had no other suspicious skin lesions and admitted to having the lesion shave biopsied two years prior by dermatologist with benign findings. No picture was taken of the lesion prior to surgical intervention as it was expected to be benign based upon prior dermatological findings. The patient stated that the leg lesion was non-painful in nature but was concerned that it may have increased in size over the previous 2 months and had a brown-gray discoloration. No prior imaging was obtained for the leg lesion. A wide margin elliptical excision was performed with a minimum goal of 1 cm margins on all sides of the lesion and the depth was resected to the muscle layer. The full specimen was marked for orientation and submitted to pathology. The excised elliptical portion measured 4.3 cm in length and 2.5 cm in width at widest points. The nodular lesion measured 2.4 × 1.8 × 0.9 cm. Ancillary studies showed that the lesion was CD31 positive, CD34 positive, and negative for cytokeratin markers. The pathology report confirmed EHE with tumor close to circumferential margins and present at the deep margin. The lesion was staged as pT1a pNX in accordance with AJCC staging. Given the deep margin extending to the thin layer of muscle just distal to the knee, the patient was referred to orthopedic surgery for further evaluation and operative intervention. The mainstay of treatment for invasive sarcoma is surgery often coupled with radiation and/or chemotherapy. A second surgical excision 19 days later following the original surgery was performed into deeper tissue. The second lenticular ellipse measured 8.1 cm in length and 2.1 cm in width at widest points at a depth of 1.4 cm. Multiple frozen sections were examined and clean margins of a minimum of 1 cm was determined in all directions.\nDue to the diagnosis of EHE, it was prudent to obtain additional imaging to determine if the malignancy had metastasized. Although incredibly rare, there have been documented cases of pulmonary epithelioid hemangioendotheliomas, as well as cases of tumors found on the liver. A CT scan was performed with IV and oral contrast of the chest and abdomen. The findings were unremarkable for the chest but multiple hepatic cysts and an enhancing lesion in the right lobe of the liver were identified. A follow up MRI with and without gadolinium confirmed a 2 cm well-defined focal area of delayed enhancement within the posterior segment of the right lobe of the liver corresponding to the CT findings, likely representing a cyst and not a metastatic lesion. These findings suggest that no metastasis had occurred and that the leg skin lesion appeared to be the primary site of EHE. |
A 65-year-old man with a background of sarcoidosis presented with an acute myocardial infarction and underwent emergency percutaneous coronary intervention and revascularization of his left anterior descending artery with an 8-Fr sheath access of the right common femoral artery. The patient was anticoagulated with unfractionated heparin during the procedure. The intervention went uneventfully and the arteriotomy site was closed with an Angio-Seal vascular closure device. The patient recovered well and was discharged after 2 days and was commenced on dual antiplatelet therapy.\nOne week following his cardiac intervention, he was noted to have a pulsatile mass in his right groin with an associated bruit. He underwent a Duplex arterial ultrasound scan, which showed a bilobed pseudoaneurysm (superficial aneurysm measures 20 mm in transverse diameter with a shallow neck 0.8 mm; deep aneurysm measures 12 mm in transverse diameter which extends off a 6 mm neck) arising just lateral to the femoral artery puncture site [].\nHe went on to have an ultrasound-guided percutaneous injection of the pseudoaneurysm with thrombin. The procedure proved to be extremely challenging due to rapid blood flow and the wide pseudoaneurysm neck (6 mm). Following 10 min of direct sonographic probe pressure, complete occlusion of the superficial pseudoaneurysm was observed but a small residual fleck of flow on power Doppler was evident to suggest an incomplete occlusion of the deep component of the pseudoaneurysm []. This was confirmed on repeat Duplex ultrasound scan the following day [].\nThe patient refused surgical repair and was offered a novel approach using an Angio-Seal vascular closure device. Informed consent was obtained from the patient for the “off-label” use of this device. A 6-Fr contralateral sheath was positioned in the distal right external iliac artery from the left common femoral artery and angiography was performed which demonstrated the pseudoaneurysm arising from the anterolateral aspect of the right common femoral artery []. Percutaneous right access of pseudoaneurysm was achieved using a wire capture technique. The pseudoaneurysm neck was catheterized and the catheter directed anteriorly. A 0.014-inch straight wire was advanced into the wall of the pseudoaneurysm, which acted as a marker to the location of the pseudoaneurysm neck. A parallax was formed via multiple projections based on the straight wire to guide the entry point of the micropuncture needle at the skin surface. An 18G × 15 cm needle was inserted percutaneously, and advanced with rotation in order to engage the straight wire end []. Following successful wire capture, a 0.014-inch wire was advanced through the 18G needle and a 5-Fr microsheath was inserted over the wire into the right external iliac artery. This allowed a 0.035-inch wire to be advanced through the sheath into the right external iliac artery. An 8-Fr Angio-Seal device was inserted in a retrograde manner ensuring that the polymer anchor is pulled firmly against the neck of the pseudoaneurysm. The toggle of the Angio-Seal device failed to hold at the artery wall. The procedure was repeated with a similar result. It was thought that the wide pseudoaneurysm neck was the reason for the failure of Angio-Seal deployment. A decision was made to use an Amplatzer 4 (St Jude Medical, St Paul, MN, USA) vascular plug device to occlude the pseudoaneurysm. A 0.038-inch guidewire was advanced via the left common femoral artery access and a 7-Fr sheath was inserted over the wire and advanced to the pseudoaneurysm neck at the right common femoral artery. The Amplatzer 4 vascular plug was deployed successfully []. Post deployment, angiography demonstrated effective sealing of the right common femoral artery at the level of the vessel wall []. Repeat of Duplex ultrasound scan the next day demonstrated successful embolization with no flow within the pseudoaneurysm []. The patient did well with no immediate complications and was discharged home 24 h postprocedure. |
The patient is now an 18-year-old. He was referred at 3 years of age with a complaint of a chronic wound in the medial region of the right thigh and systemic symptoms, including lethargy, weight loss, and intermittent fever since 2 months prior. The lesion was initially a small red ulcer, which rapidly progressed in size with yellowish nonpurulent bloody discharge. Initially, the patient was hospitalized and treated with topical and systemic antibiotics. Blood studies for systemic vasculitis and chronic granulomatous disease and all investigations to rule out any background conditions, such as inflammatory bowel disease, were normal. His skin lesion improved with local care and topical antibiotic for 2 months, and the patient was discharged without any specific diagnosis. However, after a few months he found new lesions on his right hand and abdomen. Biopsy of the lesions showed subacute necrotic dermatitis, and then prednisolone and azathioprine were started. Several months later, when his medications were tapering, a new lesion of 15×15 cm developed in the left thigh, and this time the patient was diagnosed with PG, based on the second biopsy. Reevaluation of the patient for systemic vasculitis and inflammatory bowel disease again were negative. With a final diagnosis of PG, he received cyclosporine and dapsone. However, despite treatment with these medications for consecutive years, he suffered a recurrent and relapsing course. We also tried different combination therapies, such as methotrexate, cyclophosphamide, and mycophenolate mofetil, for several courses, with unfavorable responses. Based on an unpublished report by Göknur Kalkan on the association of familial Mediterranean fever and PG in the Mediterranean area, we assessed MEFV gene mutations, which showed normal results. Because of refractory courses with different relapses and temporary recoveries, we decided to start infliximab as a biologic drug at 100 mg at weeks 0, 2 and 6 and then every 4 weeks. He showed dramatic clinical improvement in a few months, without any known side effects over the previous 2 years. There was not any recurrence, and all previous medications were discontinued. During 2 years; follow-up at the rheumatology clinic, he achieved full recovery. |
A 32-year-old, male presented with a history suggestive of schizophrenia of 9 years duration. To start with he developed symptoms in the form of auditory hallucinations, suspiciousness, fearfulness, decreased interaction with family members and social withdrawal. Three months after the onset of the illness he was treated with T. haloperidol 10 mg/day along with clonazepam 3 mg/day with which his positive symptoms subsided, but negative symptoms continued. However, he discontinued the medications and after few months had a relapse of symptoms and was again started on treatment. Over the years, he developed a pattern in which he would have florid positive symptoms for 3–6 months, following which treatment would be started with which his positive symptoms would resolve, but negative symptoms would persist. After taking medications (olanzapine 20 mg/day or risperidone 3 mg/day) for 3–4 months after improvement of positive symptoms he would discontinue the medications and then remain in the same deficit state for varying periods of 6 months to 2 years. About 4 months prior to presentation at our center, following one of the relapses, he was started on clozapine by a psychiatrist with which he improved over the period of 3 weeks. However, at the dose of clozapine 200 mg/day he started to complaint of feeling restless and would have tingling sensation in his feets and hands; continuously feel an urge to move about and would not be able to sit at a place. While sitting at one place would keep on moving his feet and keep on shifting positions. He presented to our center with these symptoms. At the time of presentation his physical examination did not reveal any evidence of rigidity, tremors and orofacial movements. In the mental status examination he was restless throughout the interview, kept on moving his both upper and lower limbs after every few minutes and often got up while being interviewed and on inquiry reported inner restlessness and an urge to move his limbs.\nOn the basis of available information, a diagnosis of undifferentiated schizophrenia with akathisia (possibly clozapine induced) was considered. On Barnes Akathisia Rating Scale, his total score was 11. His routine investigations did not reveal any abnormality and magnetic resonance imaging of the brain did not reveal any abnormality. Initially tablet propranolol 40 mg/day was added but with this he did not perceive any improvement. After 2 weeks of introduction of propranolol, the dose of clozapine was slowly reduced to 150 mg/day with close monitoring of psychopathology. Reduction of clozapine led to subsidence of akathisia with Barnes Akathisia Rating Scale scores coming down to 0 after 3 weeks of reduction of clozapine to 150 mg/day. According to the WHO-UMC causality criteria, association of akathisia with clozapine was rated as “probable.” |
A 34-year-old right-hand dominant Greek woman, presented at the Upper Limb Clinic of the Hospital complaining of persisting pain and stiffness in her right shoulder. The symptoms began 3 months earlier after a fall on her outstretched hand from a height of approximately 3 metres. The patient reported that the initial clinical assessment in the local emergency department and the anteroposterior radiograph of the right shoulder did not reveal any significant abnormality and a diagnosis of shoulder sprain and contusion was established. Pain medication was prescribed and a sling was applied for 10 days. After that time, the patient was re-examined and physical therapy with active and passive shoulder and upper limb exercises was commenced. As there was no improvement in pain and shoulder mobility, she was finally referred to our clinic for a second opinion and further evaluation.\nOn physical examination, her shoulder looked flattened anteriorly and both acromion and coracoid processes appeared to be prominent at the anterior part of the shoulder. There was an internal rotation deformity of 30° and any effort to passively or actively move the glenohumeral joint was extremely painful. Forward elevation of 40°, no external rotation and inability to completely supinate the forearm were also identified. The patient did not have any neuromuscular deficit and her medical history was unremarkable in terms of previous injuries in the shoulder region or other medical comorbidities. The anteroposterior radiograph of the right shoulder illustrated the marked internal rotation of the proximal humerus and the typical "lightbulb sign". The greater and lesser tuberosities were fractured and displaced from each other and from the humeral head. A further undisplaced fracture line at the anatomic neck of the proximal humerus was also evident (Figure ). Because of the inherent patient difficulty to abduct the arm, an axillary view was not performed. The transthoracic lateral roentgenogram showed posterior extrusion of the humeral head from the glenoid fossa (Figure ). Furthermore, the computed tomography (CT) scan clearly delineated the locked posterior shoulder dislocation with the large anteromedial head defect (50% of the articular surface) and the comminuted fractures of both tuberosities (Figure ).\nAccording to these findings, open reduction and reconstruction of the proximal humerus was considered necessary. Under general anaesthesia, the patient was placed in a beach chair position and the glenohumeral joint was assessed via a deltopectoral approach. The axillary nerve was palpated to ascertain its position but it was not mobilised. The long head of the biceps was still intact and both tuberosities were localised and circumferentially released from the newly formed granulation tissue and immature callus. As the capsule was torn and detached along with the lesser tuberosity, mobilisation of the bone fragment in a "trap-door" manner allowed easy access and visualisation of the glenohumeral joint. The humeral head was found to be dislocated posteriorly, the posterior labrum was pulled out from the glenoid and a layer of fibrous tissue covered the glenoid cavity (Figure ). After meticulous removal of the scar tissue, the glenoid articular cartilage looked to be in good condition and the humeral head was reduced using long Darrach retractors in combination with extra-articular pressure. However, the joint was unstable even with a few degrees of internal rotation. Using three Panalok RC (Mitek Products, Ethicon) absorbable anchors with number-2 polyester braided sutures, the posterior capsule and labrum were repaired to the posterior glenoid rim. The large reverse Hill-Sachs lesion was addressed with transfer of the fractured lesser tuberosity and its attached subscapularis muscle to the anteromedial defect according to McLaughlin's technique modified by Hawkins et al. []. Aiming to restore the sphericity of the humeral head and enhance the healing process, the bone bed of the defect was augmented with demineralised bone matrix allograft (Grafton® DBM Putty, Osteotech, Eatontown, NJ) and stable fixation of the lesser tuberosity was achieved with two partially threaded 4.0 mm titanium screws (Figure ). The greater tuberosity and anatomic neck fractures were subsequently stabilised using three screws of the same type. Repair of the rotator interval was the last step performed and routine closure of the wound over a drain was achieved.\nPostoperatively, the extremity was placed in a sling with the shoulder in neutral rotation and slight abduction. At 4 weeks, passive shoulder and pendulum exercises were initiated and the patient was advised to use the sling for another 4 weeks. At 8 weeks, a more aggressive physical therapy with active assisted range-of-motion and strengthening exercises was instituted as plane X-rays showed maintenance of joint congruency and early signs of bone healing. Despite the instructions for examination at regular intervals, the patient did not return for follow-up until two and a half years postoperatively. She reported that her shoulder was totally painless without any limitations during daily activities. She could actively elevate and abduct her arm 150° and 120°, respectively. In internal rotation, she reached the L2 vertebra and external rotation was 40°. Plane radiographs (Figure ) and CT scan (Figure ) confirmed a good clinical result and absence of devascularisation or instability of the humeral head. |
A 23-year-old sighted woman visited our hospital seeking treatment for a delayed sleep phase disorder that progressed each day.\nHer development was not typical. Since early childhood, although her mother darkened the room to put her to sleep, she was active and found it difficult to sleep at night. She had difficulty getting up in the morning but was able to attend school. When her mother called her, she sometimes gave no reply; thus, the mother initially thought she was deaf, but she was not. She covered her ears when watching TV, which might mean hypersensitivity. Because she tried to stick to her own interpretations and methods, her mother thought of her as a difficult child to raise. Since she was a child, she tended to repeat the same play endlessly and liked reading books rather than playing with her friends. She realized that she did not get along well with her friends but tried to think that "I have to get along with my friends well" and tried to integrate. However, in junior high school, she was left out of her group of friends and was unable to attend school. There was no noticeable bullying but she later attended a special classroom, after which she went to a correspondence high school. She entered college, left her parents, and lived with her sister. However, she often felt anxious, calling her mother with a pessimistic view of life and said that "I hate living." She was so embarrassed to write her thoughts on a class report that she could not submit it and dropped out of college. Since then, she has lived at her parents’ home, sometimes helping with housework. After dropping out of college, her sleeping hours became different day by day and her circadian rhythm was often completely reversed. Initially, she was sleepy but could not sleep for approximately 2 hours when she went to bed. The sleep cycle is now delayed by one cycle every 20 to 30 days (approximately every 25 days). She tried to control herself for 3 to 4 years but could not do so; therefore, she visited our hospital. She brought her sleep records to date, which show that she had six of these delayed cycles in 154 days; thus, her sleep rhythm averaged one of these cycles every 25.6 days (Figure ).\nShe was diagnosed with ASD using the Diagnostic and Statistical Manual of Mental Disorders version 5, characterized by persistent challenges with social communication and social interaction, and by the presence of restricted, repetitive patterns of behavior, interests, or activities. Additionally, using International Classification of Sleep Disorders (ICSD) criteria, she was diagnosed with N24SWD. She had no other notable physical illnesses.\nShe received behavioral education, but her sleep rhythm did not improve. Then, we initiated treatment with 8 mg of ramelteon every night around 11:00 PM, and the N24SWD cycle was extended from 25.6 days to 42 days, but did not completely recover. Therefore, we added 15 mg of suvorexant, and the sleep rhythm was restored (Figure ). Subsequently, she began to take steps to go outside and can now go to a social rehabilitation facility once or twice a week. |
A 57-year-old woman with diabetes and hypertension presented to our hospital complaining of severe right upper quadrant pain and vomiting. She reported having an elective open cholecystectomy done seven years before and noted that the symptoms she now experienced were similar. She had been hospitalized six times in the preceding two years for similar episodes. Although abdominal ultrasounds were done on these occasions, the emergency room physicians did not entertain the possibility of a stump cholecystitis because the patient insisted that her gallbladder had already been removed. Operative notes were not available but she reported that the operation was uncomplicated and she was discharged 72 hours after open surgery.\nUpper gastrointestinal studies excluded the presence of upper gastrointestinal pathology. Liver function panel and serum amylase levels were normal. Abdominal ultrasound suggested that stones were present in a thick walled gallbladder.\nThe patient's history was revisited on this presentation. She insisted that open cholecystectomy was performed seven years before and there was a surgical scar at the right upper quadrant, but no operative records or histology reports were available to corroborate the surgical history. Once the symptomatology resolved, the patient was discharged with a request for magnetic resonance cholangiography (MRCP) from another institution. This confirmed the presence of a gallbladder remnant with normal biliary anatomy. There was no evidence of choledocholithiasis ().\nShe was prepared for general anaesthesia and taken to the operating theatre for laparoscopic completion cholecystectomy. Laparoscopic shears were used to perform adhesiolysis in order to visualize the structures in the right upper quadrant. There were dense adhesions at gallbladder bed precluding clear visualization of relevant anatomy (). Careful and patient dissection with the cautery hook eventually presented the gallbladder remnant (). The remnant was followed in an anterograde fashion down to the cystic duct to demonstrate Strasberg's critical view of safety (). The cystic duct and artery were then individually ligated and the gallbladder separated completely (). Her recovery was uneventful and she was discharged home after 8 hours. |
A 51-year-old Madheshi woman came to our neurology clinic with the chief complaint of sudden decrease in the tone and texture of her voice for the past 15 days. Her voice was very feeble but understandable and she noticed it was better by the time she got up from her bed only to worsen through the day to become nearly inaudible. She had noticed a slight change in her voice texture in the first several days which she had ignored in the beginning as it was not worth bothering about. She had no complaint of choking or coughing or aspiration or any throat discomfort. When asked for any other related and/or unrelated things she noticed in her habits, she complained about having constipation for many years otherwise she was apparently well. She had never visited hospital for any problem and no interventions had been done in the past. She denied smoking tobacco, drinking, or any other recreational drugs abuse. There was no one in her family or her parents’ family with any kind of known chronic disease. Her biological father had controlled hypertension with medication. Her psychosocial history was not significant. She came from a middle income family, and she had retired recently as an accountant for a small company. Her symptom progressively aggravated in later days to the extent that she not able to produce sound properly. It usually waned in the early morning or after enough voice rest only to wax throughout the day.\nOn physical examination, a systemic examination did not reveal any abnormality. On neurological examination, her muscle power was intact: 15/15 on Medical Research Council (MRC) scale. She had no imbalance and was able to tandem on walking. An examination of her gag reflex and other cranial nerves revealed no abnormal reflexes. Deep tendon reflexes were intact. Her speech articulation was intact and revealed no scanning of speech. Speech production was adequate and non-painful; her tone was non-nasal but the intensity was low and slow. Other neurological examinations also did not reveal any abnormality. Other systemic examinations were also non-significant.\nConcerned with her problem, she had visited an ear, nose, and throat (ENT) department for her problem and was screened for possible laryngeal disorder for hypophonia. There was no obvious laryngeal pathology found and the treatment initiated by the ENT department had no satisfying outcome. She visited our neurology clinic. The differential diagnoses considered were Parkinson’s disease (PD) or a bulbar variant of motor neuron disease (MND), and MG. MRI scanning of her brain and screening of her whole spine appeared normal. Laboratory investigations including a hematologic panel, infectious disease screening panel, and myopathy panel involving creatinine kinase and creatine phosphokinase-N-acetyl cysteine (CPK-Nac), were all within normal range. She was suspected to have MG as other diseases were ruled out. A Quantitative Myasthenia Gravis (QMG) test was performed on our patient and her QMG score was 1 (for speech). She was given neostigmine challenge test with intravenously administered injection of 2.5 mg neostigmine and the result was positive after more than 30 minutes. There was significant improvement in her voice quality and she felt her fatigability improved as well. She gained a good quality of speech within an hour. QMG scoring was repeated, and she scored QMG score of 0. The longer duration of the medicine gave us enough time to assess the improvement. The laboratory test for the AchR antibody (AchR ab) is not available in Nepal. We had to send AchR ab to India, and the report received after waiting several weeks was positive. Quantitatively, it was significantly higher, 1.11 nmol/l than the normal range (0.0–0.4 nmol/l). She was later maintained on 60 mg of orally administered pyridostigmine with 12 hourly administrations and subsequently increasing to 6 hourly along with steroids.\nPlain and contrast-enhanced computed tomography (CT) scans of her head, neck, and chest were done, and they revealed a mass in the anterior mediastinum: a well-defined, smooth outlined, approximately 4 × 2.5 × 2 cm, soft density nodule showing poor enhancement in the anterior mediastinum in the thymic space with maintained fat plane with the adjacent vessels and structure. Thymoma was suspected and she was sent to the surgical department for further surgical intervention.\nShe returned to our neurology clinic around 2 months after her first visit to us as a follow-up visit. She had undergone a successful surgery. She was doing well. Prednisolone was already tapered off. She was tapering down pyridostigmine as well, with a maintenance dosage of pyridostigmine 60 mg 12 hourly and planned to put off the medication. Her voice was clear and well maintained and she had no complaint of fatigue. She had no fresh complaint and was doing all her daily and professional activities very well. |
A 25 year old male was originally seen in our office after the diagnosis of chronic exertional compartment syndrome (CECS) of the anterior and lateral compartments had been made by invasive pressure measurements of those compartments. He was originally referred to our office for the treatment of chronic leg pain due to a neuroma of a superficial peroneal nerve, injured during an anterior and lateral compartment fasciotomy to treat his CECS. This painful neuroma was treated successfully by neuroma resection and implantation of the proximal end of the superficial peroneal nerve into the extensor digitorum communis muscle []. His anterior and lateral compartment pain had resolved with the original fasciotomies. He was then discharged from our care.\nHe returned to our office one year later with complaints of bilateral exercise induced pain in the backs of his legs from the lower calf to the ankle that he stated felt "just like the front of my legs did, though slightly less intense." After five minutes of running he began to complain of tightness and a dull aching pain that progressed to severe pain eventually causing him to stop exercising. His pain was also associated with paresthesias and numbness in the soles of his feet. The pain and numbness persisted for five to ten minutes after stopping his exercise, but the tightness lasted longer.\nOn exam, the patient was an athletic appearing male with normal pulses in dorsalis pedis and posterior tibial vessels. He was tender to pressure applied immediately posterior to the tibia overlying the distal deep posterior compartment. He had no tenderness to percussion of the tibia itself or to palpation of the tibial edge. He was not tender in the midline of the posterior calf over the proximal tibial nerve []. His gastrocnemius muscle was slightly tender. He did have a Tinel sign over both tarsal tunnels with radiation to the sole of his feet.\nDue to his symptoms of exercise induced numbness and paraesthesias, non-invasive, non-painful neurosensory testing was performed with the Pressure Specified Sensory Device™ (Sensory Management Services, LLC, Baltimore, Maryland, USA) at rest to measure base line cutaneous pressure thresholds for one and two point static touch and to measure two point discrimination in the skin innervated by medial plantar and medial calcaneal branches of the tibial nerve (Figures . and ). The anterior lateral dorsum of the foot and the dorsal web-space between the first and second toe – the usual distribution of the superficial peroneal and deep peroneal nerve branches respectively – were also measured. The study was repeated immediately after 10 minutes of running on a treadmill – the time interval to reproduce his symptoms. Following the running, there was widening of two point discrimination in the distribution of the calcaneal nerve and the medial plantar nerve indicating loss of large fiber tibial nerve function suggesting the diagnosis of exertional compartment syndrome of the deep posterior compartment causing compression of the tibial nerve (Table ).\nTo confirm the diagnosis, traditional invasive, immediate, post-exercise compartment pressures of the superficial and deep posterior compartments were obtained using a device with a side port needle measurement system (Stryker Instruments, Kalamazoo, Mich.). The superficial posterior compartment (SPC) measured 40 mmHg on the right and 24 mmHg on the left. The deep posterior compartment (DPC) measured 62 mmHg on the right and 28 mmHg on the left. To rule out other causes of posterior leg pain an MRI was performed and demonstrated no vascular anomalies, no evidence of stress fractures, medial tibial periostitis, tumors, or other abnormalities.\nBilateral superficial posterior and deep distal posterior fasciotomies were performed through a proximal and distal two incision medial approach. Postoperatively, the patient recovered without incident. However, at three months he still complained of similar symptoms, but they were more isolated to the posterior distal half of the lower extremity over the distal deep compartment muscles. The patient's exam still demonstrated pain with compression just posterior to the tibia in the lower half of his legs. Due to his complaints of persistent pain and numbness, his non-invasive neurosensory testing was repeated before and after running 10 minutes on a treadmill (Table ). Again he demonstrated loss of two point discrimination in the calcaneal and medial plantar nerve that suggested continued tibial nerve dysfunction brought on by exertion.\nTherefore he was taken back to the operating room for a repeat fasciotomy of the distal deep compartments. It was discovered that the patient had an unusual anatomic variant of his deep distal compartment as described by Detmer [], and therefore the compartment had not been fully released during the first operation. The soleus muscle wrapped around medial side of the tibia unusually far, and it completely obscured the deep distal compartment. The fascia that had originally been released turned out to be the fascia overlying the unusually large and medially placed soleus. Only after peeling the soleus completely off the medial edge of the tibia in the distal lower leg was a second deeper layer of thickened fascia found beneath it. This too was released longitudinally to open the true deep distal compartment that encased the posterior tibial neurovascular bundle, the flexor digitorum longus, posterior tibialis, and flexor hallucis muscles.\nThe patient recovered well from his second operation and was allowed to progress in his exercise regimen starting three weeks after surgery. After his first attempted posterior distal compartment release, he was able to run only a half of a mile before he would need to rest and allow his legs to recover. Three months after his second posterior distal compartment release, he was able to run over three miles with out resting. At 15 months after the second posterior distal compartment fasciotomy, the patient states that he had a 90% improvement in the numbness and posterior leg pain since surgery.\nWe tested him a third time with the non-invasive neurosensory testing before and after running on a treadmill for 12 minutes and this demonstrated minimal change in two point discrimination indicating minimal change in tibial nerve function, thus demonstrating resolution of nerve compressions caused by his deep distal posterior exertional compartment syndrome. |
A 33-year-old male patient presented to a tertiary care hospital with intermittent abdominal pain, nausea, vomiting, constipation and numbness of bilateral lower limb extremities, of 3 days duration. He was conservatively managed in a surgical unit as partial intestinal obstruction and was awaiting diagnostic laparoscopy. The patient developed confusion and found to have systemic hypertension, and, therefore, was transferred to a medical ward for further management. He is a non-diabetic and did not have a previous history of hypertension. The drug history revealed usage of over-the-counter analgesics for 6 weeks.\nThe past medical and surgical history revealed similar neurovisceral attacks requiring five acute hospital admissions over 2 years, which ended up in questionable diagnoses. The sixth acute attack raised the suspicion of an acute porphyria. The first attack in January 2013 led to a diagnosis of appendicitis. Because the symptoms worsened following the surgery an emergency laparoscopic exploration was done. But the exploration revealed no cause to explain the worsening symptoms. The second attack was managed as sinus tachycardia and he was started on beta adrenergic blockers. The third attack which was associated with a fever was conservatively managed for a questionable renal colic. Forth attack was complicated with transient hyponatremia and transiently high serum creatinine levels. These complications were attributed to a questionable interstitial nephritis based on the fact that patient had used 50 mg of diclofenac sodium twice a day for 6 weeks, repeating the prescription given by a general practitioner. Another attack in 2015 was managed as partial intestinal obstruction and diagnostic laparoscopy was done. In all these presentations, findings from the ultra sound scans and diagnostic laparoscopy did not support a diagnosis of intra-abdominal pathology.\nOn examination he was thin built (BMI = 20 kg/m2) and pale. Brachial blood pressure was 160/90 mmHg. There were scars of previous appendectomy and laparoscopy surgeries on the abdominal, but, otherwise, the abdominal examination was unremarkable. Muscle power was 4/5 in all four limbs (could not move against a good resistance).\nThe laboratory investigations performed during this admission showed severe hyponatraemia of 115 mmol/L (136–145) with serum osmolality of 255 mOsmol/Kg (275–295) and urine osmolality of 460 mOsmol/Kg (50–1200 mOsmol/Kg). Serum creatinine concentration was 106 µmol/L (80–115) with blood urea level of 20 mg/dL (6–20). Hemoglobin concentration was 8.2 g/dL (13.5–17.5) and the red cell morphology was normochromic and normocytic. The total cholesterol level was 282 mg/dL (5th to 95th centile; 142–258) with LDL fraction of 225 mg/dL (5th to 95th centile; 78–185). Serum ferritin level was 646 ng/mL (20–250). Arterial blood gas analysis was suggestive of a metabolic acidosis. Echocardiogram showed evidence of left ventricular hypertrophy. There were no significant radiological findings in abdominal X-ray film or abdominal ultrasonography. Blood lead concentration was 3 μg/dL (< 5 μg/dL).\nA urine sample collected during the acute attack was sent to the Department of Chemical Pathology for biochemical analyses. On standing the urine sample gradually turned dark brown. The Watson and Schwartz test for urinary porphobilinogen (PBG) was positive (Fig. ). Spectrophotometry of urine for total porphyrins showed a “Soret band”. Urine total porphyrin level, calculated using Allen corrected absorbance of the urine sample was 5505.5 nmol/L (< 300 nmol/L). Genetic studies were carried out in an overseas laboratory. The full analysis of HMBS gene was performed by PCR amplification of extracted DNA followed by exon specific primer extension analysis of all exons, exon intron boundaries and promotor regions. The gene analysis revealed a previously reported missense mutation, c.517C>T encoding p.R173W in the HMBS gene. Targeted mutation analysis was performed by PCR amplification of extracted DNA followed by allele specific primer extension analysis, in five first-degree relatives. Among these, four were heterozygous for the same HMBS gene mutation (Fig. and Table ).\nSince heme arginate is not available in Sri Lanka the patient was managed only symptomatically. Carbohydrate loading with intravenous dextrose and oral carbohydrates was the only feasible option. All the medications used for symptomatic management were checked for safety in acute porphyrias. Patient was discharged from the ward after symptoms gradually improved over 6 days to a degree that he can be managed as an out-patient. Response to treatment could not be assessed due to unavailability of quantitative tests to measure urinary aminolevulinic acid (ALA) and PBG in Sri Lanka. The patient was educated regarding precipitating factors of acute porphyria. A diagnostic card with information regarding medications to avoid was provided to the patient. Patient was followed up at the clinic with regular renal functions, hemoglobin and blood pressure monitoring. Follow up of the patient over 1 year following diagnosis revealed that patient suffered from two mild attacks which didn’t require in-patient management. Nerve conduction studies were not carried out because neurological symptoms were not observed in-between acute attacks.\nPre-symptomatic relatives who inherited the HMBS mutation were also advised to avoid the trigger factors of acute attacks such as certain medications, fasting, alcohol and hormones. The brother of the proband was counseled regarding the risk of his children inheriting the HMBS mutation and recommended targeted mutation analyses for both children. |
In 2003, during a routine health checkup, a pancreatic cyst measuring 8 cm in diameter was detected in a 51-year-old woman. The cyst was subsequently managed with follow-up care by the previous physician on an outpatient basis. In 2010, the cyst had increased to 12 cm in size and then to 19 cm within approximately the next 6 months. At this time, the patient was referred to our hospital for treatment. At the initial visit, she reported abdominal bloating. However, no medical problems were evident from the patient's health history, and she had no history of pancreatitis, abdominal trauma or alcohol intake. A physical examination showed no sign of anemia in the palpebral conjunctiva or jaundice of the bulbar conjunctiva; the abdomen was soft, flat, and nontender, and no obvious tumor mass was detected by palpation. Blood tests revealed no elevation of pancreatic enzyme levels or tumor markers. Abdominal ultrasonography and contrast-enhanced abdominal computed tomography (CT) scans showed the presence of a partially septated giant cystic lesion measuring 20 × 11 × 13 cm in the abdominal cavity (fig. ). The lining membrane of the cyst was thin, and no solid components or calcifications were noted in the cyst. A beak sign was observed in the pancreas, and the main pancreatic duct in the tail of the pancreas was slightly dilated. Therefore, the mass was considered a cystic tumor of pancreatic origin. On abdominal magnetic resonance imaging, the pancreatic body lesion detected by the CT scan was depicted as high-intensity area on T2-weighted images. These findings were consistent with the characteristics of cystic lesions (fig. ). A septum-like structure was present in the cyst, and dilation was noted in the pancreatic duct in the tail of the pancreas. Endoscopic ultrasonography showed extramural compression of the middle part of the gastric body. Although imaging studies of the same site could not reveal the entire extent of the cyst because of its enormous size, the cyst demonstrated low-level internal echoes and did not show any obvious solid component. A septum-like structure was observed, but there was no microcystic lesion with a characteristic honeycomb pattern. Endoscopic retrograde cholangiopancreatography displayed only the main pancreatic duct in the head of the pancreas; the cyst compressed the tail side. Based on these findings, the differential diagnosis included serous cystic neoplasm (SCN), mucinous cystic neoplasm, and branch duct-type intraductal papillary mucinous neoplasm in the neoplastic category as well as pancreatic pseudocyst in the nonneoplastic category. Because this mass was increasing in size and abdominal bloating was present, surgical resection was deemed appropriate. Therefore, a distal pancreatectomy with splenectomy was performed. The cystic content was serous fluid of a dark, muddy color, suggesting previous bleeding (fig. ). On histopathological examination, the lining membrane had a papillary structure composed of cuboidal cells with round nuclei. The cells stained PAS-positive and diastase PAS-negative, thus indicating the presence of glycogen (fig. ). These findings led to the definitive diagnosis of SCN. |
A 46-year-old man presented in early 2012 having a 16-year history of recurrent respiratory tract infections. He had originally presented to the respiratory physicians in 1996, and in 2004, he developed an empyema which required thoracotomy and drainage. Following drainage, he remained relatively well requiring infrequent oral antibiotics for exacerbations of bronchiectasis on a background of repeated episodes of right basal pneumonia. A sputum specimen from the patient cultured positive for Haemophilus influenzae. A high-resolution computed tomography scan (HRCT) in 2004 demonstrated bronchiectasis and this was confirmed on repeat imaging eight years later. His background history included right lower lobe childhood bronchiectasis diagnosed after an episode of haemoptysis. A further HRCT scan demonstrated an area of high attenuation in the right lower lobe bronchus intermedius ( and ). This was intra-luminal and solid in keeping with appearances of a foreign body or simply a calcified secretion: it looked like a toy traffic cone.\nOn enquiry, the patient recalled swallowing a toy traffic cone over 40 years ago when he was six years old. He had choked at the time but assumed it had been swallowed. Ongoing respiratory review resulted in a referral to our thoracic department for surgical assessment. A rigid bronchoscopy showed the right lower lobe bronchus was narrowed to a tiny orifice continually discharging pus. The bronchus intermedius was short and the middle lobe was inflamed. The right main bronchus and upper lobe were both normal as was the apical segment of the right lower lobe.\nFurther surgical management included a flexible bronchoscopy under general anaesthetic to assess the bronchus to the lower lobe. Due to the protracted clinical history, it was doubtful that extraction would be possible and the patient was scheduled for an elective thoracotomy. Preoperative investigations included a chest X-ray which demonstrated obliteration of the right costodiaphragmatic angle and normal pulmonary function tests (). At operation, a bronchoscopy revealed an indurated area at the bifurcation of the middle lobe and right lower lobe bronchi. This area still discharged pus and bronchial lavage and suctioning revealed a yellow foreign body which was extraluminal. The surgical team proceeded to perform a right lower lobectomy and wedge excision of the middle lobe.\nIntraoperatively, multiple inflammatory adhesions were discovered. The right inferior pulmonary vein and right pulmonary artery were both identified and divided. The right apical segmental artery and right basilar artery were also identified and divided enabling the posterior hilar artery to be visualized and the bronchus exposed. During dissection of the bronchus, profuse quantities of pus were aspirated (). The toy traffic cone was identified in the same location as that determined on CT (). Once removed, the right lower lobe bronchus was stapled and oversewn using a 3-0 Vicryl suture. A small section of the middle lobe was excised after dividing the inferior segmental bronchus of the middle lobe.\nThere were no postoperative complications reported and a short course of intravenous antibiotics was given. Histopathological examination revealed a significantly underdeveloped right lower lobe having areas of focal consolidation and changes consistent with bronchiectasis. The right lower lobe bronchi had diffuse dilatation and associated peribronchial fibrosis and scarring. The toy traffic cone was returned to the patient (). |
A 39-years-old man was referred to our clinic with complain of inability to sit on and use wheelchair for ambulation due to the lack of flexion in both knees. He was a victim of a diving accident 5 years before presentation after which he had been quadriplegic. With appropriate care and surgical intervention upper extremities regained some function (sensory and motor) but the paraplegia remained. During the first 6 months following the injury he noticed progressive lack of flexion of both knees and finally total ankylosis of both knees in full extension. The problem severely impacted his lifestyle and mobility due to impaired sitting ability. The problem bothered the patient so that he would request an amputation if the position of the knee joint could not be corrected.\nOn physical examination, the knees had no passive movement and both ankles were fixed in equinus position (Figure ). A burn scar was seen on the lateral aspect of the right knee. Distal posterior tibialis and dorsalis pedis pulses were palpated and were symmetric. On neurologic examination there were no voluntary contraction in his spastic lower limbs and complete sensory deficit was evident. The patient was under treatment with warfarin due to previous deep vein thrombosis. The medication was changed to heparin before operation.\nOn radiographic examinations, large masses of heterotopic bone were seen bridging the knee joints from posterior distal femur to proximal tibia in the popliteal fossa (Figure ). To determine the vicinity of neurovascular structures with the heterotopic bone a CT-angiography was performed which showed both popliteal arteries displaced posteriorly and encased in grooves of heterotopic bone (Figure ). On the right side the mass was larger (220 × 50 mm) starting more proximally from about the Hunter's canal down distal to the level of trifurcation of popliteal artery. On left side the mass (180 × 65 mm) ended just proximal to the level of trifurcation. An MRI study was done to assess the integrity of articular structures and to rule out the articular involvement.\nOn laboratory data, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) and alkaline phosphatase (ALP) levels were normal. Bone scintigraphy was not performed.\nVascular surgical consultation was requested regarding the vicinity of popliteal vessels to the mass, and the risks of surgery was discussed with the patient. The left knee was operated on first due to presence of fresh scar and ulceration on lateral side of the right knee. With the patient in prone position and under general anaesthesia, posterior approach with lazy-S incision was used. Medial head of the gastrocnemius muscle was released. The gastrocnemius and the hamstrings were atrophic but not involved in the heterotopic bone (Figure ). After ligation of the superior medial genicular branch, the popiteal artery was explored and dissected free in its entire length. The mass was excised using osteotome in its base. The posterior knee capsule was involved in the mass and was resected partially. Posterior cruciate ligament was seen intact. We gained 0 to 95 degrees of flexion intraoperatively. The tourniquet was deflated and hemostasis done. Posterior tibialis and dorsalis pedis pulses were checked. Suction drain was placed and wound closed in usual manner. A hinged knee brace was placed locked in 60 degrees flexion.\nPostoperative prophylaxis was done with a single dose administration of 700cGy irradiation on the first day. Indomethacin was given 75 mg daily and continued for 6 weeks. Prophylactic administration of Enoxaparin 40 mg daily (for deep vein thrombosis) started on first postoperative day. The drains were removed on second postoperative day and the brace unlocked to start full gentle range of motion. On the fourth postoperative day the patient developed serousanguinous discharge from the wound which resolved after 2 days. On the third postoperative week the patient referred with a pitting edema of the left foot. Color doppler ultrasonography revealed deep vein thrombosis of calf which mandated medical treatment of the thrombosis. The postoperative course was otherwise uneventful. Pathologic study was compatible with heterotopic ossification. On sixth postoperative month the range of motion was 0 to 80 degrees of flexion. The right knee was operated 3 months after the left one with the same surgical technique and the same surgeon. Immediate postoperative range of motion was 0 to 100 degrees of flexion. The postoperative follow up was the same as the left one with no complications. After sixth months, the range of motion of right knee was 0 to 75 degrees of flexion. |
A 47-year old woman visited our hospital with a chief complaint of visual impairment in both eyes. The patient had muscle weakness and atrophy in distal lower extremities from about 7 years old, and used a wheelchair due to a disability that makes ambulation impossible as symptoms deteriorated. Muscle weakness and atrophy in both hands developed from about 20 years old. Her symptoms gradually deteriorated and the affected area expanded to the entire upper extremities. Four months before her visit to our hospital, visual acuity in both eyes had gradually reduced. Recently, she had a difficulty in recognizing people's faces from a 5m distance, but had no color vision deficiency or ocular pain. She had normal cognitive function development and achieved outstanding academic grades. After finishing graduate school, she is currently working as a teacher.\nPhysical examination revealed that the patient had normal cognitive function, and her pupils were 3 mm/3 mm, symmetric and reactive to light. According to ophthalmologic examination, corrected visual acuity was 20/130 in the right eye and 20/200 in the left eye, and uncorrected visual acuity was 20/500 in the right eye and 20/1,000 in the left eye. Although the retina was normal in all eyes on slit lamp examination, bilateral optic disc pallor was detected on the temporal sides in fundus photogram (). Visual field constriction was not observed, but central scotoma in both eyes was found on Goldmann perimetry (). She had no motor disturbances in the extraocular and facial muscles. Dysarthria and dysphagia were not detected. Diffuse muscle weakness and atrophy were observed in both upper and lower extremities, and the symptoms were more severe in the distal extremities. The sensory function was normal, and tendon reflexes were absent in both upper and lower extremities. Babinski reflexes were negative.\nThere were no electric pontentials in sensory and motor nerve conduction studies and needle electromyographic examinations revealed giant motor unit action potentials and reduced interference patterns. Pattern reversal visual evoked potentials were significantly prolonged in both eyes. MRI scanning of the cerebral hemispheres, orbits and optic nerves was normal, but high signal intensities were found in bilateral middle cerebellar peduncles on diffusion-weighted and fluid attenuated inversion recovery (FLAIR) imaging (). Her routine blood laboratory studies were normal, and serum lactate level was also normal at 2.5 mmol/L. She is the eldest daughter of a son and five daughters. No family members had symptoms of neuromuscular disorders.\nWe analyzed exons 1~19 of MFN2 and their franks region by direct cycle sequencing, and identified heterozygous transversion of cytosine to thymine (c.617C>T) in the seventh coding exon, when compared with the reference sequences(NC_000001.10, NM_014874.3). This mutation has already been reported in CMT []. Mitochondrial DNA mutations associated with Leber's hereditary optic atrophy (3460G>A, 11778G>A, 14484T>C and 4171C>A) were not found. Because the patient had not only peripheral neuropathy but also optic atrophy, she was diagnosed with HMSN VI caused by MFN2 mutation. |
A 57-year-old man was admitted to our acquired brain injury (ABI) rehabilitation unit 8 months after he suffered a large right cerebellar hemorrhage with significant mass effect requiring an emergent posterior fossa decompressive craniectomy. His past medical history included hypertension, vertigo, a remote concussion, and history of a fractured jaw. He developed significant dysphagia early following his stroke, including difficulty with secretion management. The dysphagia was likely multifactorial, related to the cerebellar bleed with initial mass effect and possible subsequent structural injury to the brain stem (central) and cranial nerves (peripheral) as well as an early altered level of consciousness (drowsiness, somnolence) following his stroke. A percutaneous endoscopic gastrostomy (PEG) tube was placed 11 days after his stroke, but refractory vomiting and aspiration pneumonia eventually necessitated gastrojejunostomy (GJ) tube placement. He continued to have ongoing issues with vomiting despite a trial of metoclopramide. Imaging performed 1 month after placement of the GJ tube revealed retrograde migration with the tip of the catheter visualized within the oropharynx, which was approximately 14 weeks after his stroke. The GJ tube was then replaced under fluoroscopic guidance. Within 2 weeks the tube had again migrated and was visualized within the esophagus on imaging. He underwent endoscopic placement of a gastrostomy tube with addition of a jejunal extension. Ten weeks after placement, the GJ tube had migrated with the tip now located in the duodenum. At this point, his chronic vomiting appeared to be improving with a trial of erythromycin so no further adjustments to the tube were made.\nUpon admission to our ABI unit he had ongoing dysphagia, significant hypophonia, and intermittent episodes of regurgitation and vomiting, though often only small amounts of bile or fluids. The vomiting was not stereotyped, nor did he have long episodes where he was completely symptom free, as seen in cyclic vomiting syndrome []. He had two further episodes of retrograde tube migration requiring replacement by interventional radiology. A video fluoroscopy study was performed 10 months after his stroke that revealed rhythmic pulsations of the palate, pharynx, and larynx involving the vocal cords, in keeping with palato-pharyngo-laryngeal myoclonus (see ). Subsequently, a brain MRI was completed showing apparent unilateral hypertrophy and increased FLAIR signal in the left inferior olive suggestive of hypertrophic olivary degeneration.\nSeveral medication trials including clonazepam, divalproex sodium, and gabapentin, were undertaken to help control his palato-pharyngo-laryngeal myoclonus. Medications were administered independently with no combination therapies trialed. Clonazepam was poorly tolerated due to drowsiness and thus discontinued. Divalproex sodium titrated up to 1000 mg per day and gabapentin titrated up to 600 mg per day did not lead to significant changes in the frequency or intensity of his myoclonus on repeat video fluoroscopic studies. However, his GI symptoms improved significantly with these medication trials such that he no longer vomited and there were no further incidents of retrograde migration of the feeding tube. Imaging performed 3 months after the initiation of the medication trials and 2 months after the most recent GJ tube replacement demonstrated the tip of the tube appropriately located within the jejunum. |
A 27-year-old woman applied for a breast reshaping surgery for aesthetic purposes. There were no other complaints in her medical history. The patient underwent simultaneous mastopexy according to classical Lejour vertical scar technique and breast augmentation surgery using round silicone TSF - 415 mL implants under the pectoral muscle and a breast lift under general anesthesia (). During the operation proper hemostasis was achieved using electrocoagulation. Drains were removed next day after the operation with minimum serohemorrhaging fluid volumes. The later postoperative period was also uneventful.\nThe patient was discharged from the clinic on the second day after the surgery. Five weeks after the operation patient arrived at the clinic because of tenderness and swelling of the right breast. The patient stated that she had not sustained any traumas. During clinical examination, the upper right breast area was found to be significantly swollen and firm (). Ultrasound examination showed a 2.5 cm heterogeneous liquid strip accumulated around the implant (). The implant was intact. Complete blood count showed an increased amount of leukocytes, and red blood cells and hemoglobin were at the lower limit of the normal level.\nNo coagulopathies were found. The patient was taken to the operating room where she underwent revision surgery. The purpose was to remove the fluid and to find and stop the cause of its accumulation. During the operation (), a blood clot of 650 mL was removed (). Bleeding from one of the internal mammary artery branches in the implant pocket between the rib cage and the pectoral muscle lower pole was detected and stopped. After the revision, the implant was returned to the lodge. Vacuum drainage was used for one day only. One year after the surgery, there was no recurrence of bleeding, also no clinical evidence of the implant capsule contracture formation was found ().\nAll procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The patient gave her informed consent prior to her inclusion in this case report. Any details that might disclose the identity of the patient under study were excluded. |
Mr. X is a 63-year-old male with a 40-year history of schizophrenia who was well controlled on clozapine for his psychosis and disorganized thought. Due to the severity of his mental illness, his sister is his conservator, which means that she makes all of his legal and medical decisions. Mr. X has a family history of colon cancer, where his mother was diagnosed with colon cancer in her 60s. The course of his disease started with symptoms of recurrent diarrhea and right lower quadrant abdominal pain. Since Mr. X is conserved, Mr. X’s sister gave informed consent for Mr. X to undergo an initial colonoscopy. Mr. X did not understand the instructions on how to perform the bowel prep and he did not finish drinking the bowel prep solution. This resulted in a failed colonoscopy due to poor visualization as there was too much stool in his colon. A secondary test utilizing barium enema revealed an apple core stenosing mass distal to the cecum. The discovery of an apple core lesion of the colon can be due to several etiologies, including but are not limited to inflammatory bowel disease, infection, and cancer []. A second colonoscopy was performed but did not find the lesion that was described in the barium enema and did not identify any additional lesions. Mr. X was symptomatically treated for pain and diarrhea and his symptoms spontaneously abated. There was no further evaluation until 10 months later when Mr. X experienced a resurgence of abdominal pain. Computed tomography of the abdomen and pelvis revealed a 17.6-cm mass originating from the cecum and extending superiorly, invading both the duodenum and pancreatic head. He was diagnosed with stage III colon cancer and the surgeon informed both the patient and his caregivers that he required a complicated surgery as a potential cure for his cancer.\nAfter undergoing an open Whipple procedure with right colectomy en bloc resection, he was hospitalized for eight days. The psychiatric consultation-liaison team worked closely with the surgical team to help Mr. X maintain psychiatric stability. In the months after discharge, Mr. X suffered physical complications from his surgery. These complications included severe malnutrition that led to total parenteral nutrition and gastronomy tube placement. Mr. X then suffered from paralytic ileus, followed by severe bacterial pneumonia. As a result, Mr. X had to be admitted and discharged repeatedly from multiple hospitals and skilled nursing facilities (SNF). During one of his stays at an SNF, he refused to take his clozapine; over time, he became agitated, aggressive, and began physically assaulting the nursing home staff and his family caretakers.\nMr. X then demonstrated both disorganized speech and thought, ultimately declaring suicidal ideation. At that point, Mr. X was admitted to our inpatient psychiatric ward for suicidal ideation and decompensation of his chronic schizophrenia. Mr. X’s sister reported that he was on clozapine prior to admission and that he was uncooperative because of the weekly blood draws. Thus, he was started on oral olanzapine, negating the need for weekly blood draws, as part of a regimen targeted toward reducing his psychosis and impulsiveness. However, Mr. X refused to take oral olanzapine. Repeated attempts to restart oral antipsychotic medications failed as Mr. X refused to take oral medications alone; only after both intramuscular and oral formulations of olanzapine were utilized did Mr. X regain compliance with psychiatric treatment. After several weeks within the inpatient psychiatric unit, Mr. X’s schizophrenia symptoms lessened to the point where he no longer expressed aggression and suicidal ideation. |
An 80-year-old male patient was referred to the abdominal surgery department due to incarcerated ventral hernia and ileus. In the past he was operated due to perforated gastric ulcer. He also had arterial hypertension, chronic pulmonary obstructive disease and pulmonary hypertension, a history of smoking, he suffered an ishemic stroke in the past. He was urgently operated on the same day. Segmental resection of small bowel with end-to-end anastomosis was performed and the hernia defect was closed with direct sutures, without prosthetic mesh because the bowel was resected. There were no surgical or other complications after surgery and he was discharged from hospital after 8 days. 5 days later he was admitted to the hospital again due to early recurrence of ventral hernia. The content in hernia sac could however be reduced back to his abdomen. Laboratory findings showed leucocytosis and elevated C-reactive protein (CRP - 148 mg/l). Intestinal winding with a thickened wall up to 5 mm was found at the location of the ventral hernia by ultrasound examination. The patient underwent a second surgery 22 days after the first surgery due to obstructive ileus, which was seen on the abdominal computed tomography (CT) a day earlier. Due to additional diseases and disorders (ischemic stroke and insertion of stent in his left internal carotid artery in 2011, arterial hypertension, asthma, pulmonary fibrosis and hypertension, which were not properly treated, because the patient did not follow the prescribed treatment) the anaesthesiologist decided for the spinal anaesthesia, because the general anaesthesia would be to risky. The surgery was performed by an abdominal surgeon with 5 years experiences as a specialist and he performed more than 30 Rives-Stoppa ventral hernia repairs. The skin incision was made along the previous skin incision. In the subcutaneous tissue the small intestine was tightly adhered on to the skin. We managed to release it but unfortunately, a segment of the small intestine was damaged during adhesyolisis. Segmental resection of the damaged small bowel with end-to-end anastomosis was performed (). The small intestine was reduced back in to the abdominal cavity. Ventral hernia was repaired according to Rives-Stoppa technique with prosthetic mesh (). Other than postoperative tachycardia there were no reported issues. A couple of hours after the procedure apnoeic episodes appeared followed by unconsciousness. A computed tomographic angiography (CTA) of the brain vascular system was made and it showed a stenotic left vertebral artery (90% stenosis). Because of respiratory insufficiency and haemodynamic instability, the patient was transferred to the intensive care unit. Due to a worsening clinical condition, a CTA of the abdomen was preformed and an occlusion of superior mesenteric artery (SMA) was discovered. Interventional radiologist preformed an embolectomy and thrombus aspiration from the SMA with an insertion of a stent. The patient's condition continued to worsen so the abdominal surgeon decided for a “second look” abdominal exploration. At surgical revision we found a small intestinal and sigmoid colon gangrene. Because of the patients age, several other comorbidities and gangrene of the entire small bowel, the multidisciplinary team (abdominal surgeon, anaesthesiologist, intensivist) decided for conservative treatment. The patient died the day after surgery. |
A 16-year-old adolescent male, a Sikh by religion and educated up to 2nd standard, belonging to a middle socio-economic status with non-vegetarian dietary habits was brought to our psychiatry outpatient department by parents with chief complaints of generalized tonic-clonic seizures since 2 years of age, low-intelligence since childhood and behavior problems like stubbornness and aggression since past 5-6 years.\nHistory of present illness revealed uneventful prenatal and perinatal course along with delayed motor, speech, and adaptive milestones. The patient had his first episode of grand-mal seizures at around 2 years of age and has been having them regularly since then but with variable frequency and duration. There has been no seizure free period since the onset of seizures. The patient started going to a school at around 6 years of age but discontinued studies after 2 years of age due to poor performance at school and being ridiculed by his peers for poor performance and for marks on his face. The patient would spend most of the time at his home playing with his younger siblings and occasionally playing with other younger children in his neighborhood. For the past 5-6 years the parents have noticed increasing behavior problems like stubbornness and not following parental instructions along with frequent episodes of verbal aggression and occasional episodes of physical aggression mostly directed towards parents and younger siblings generally when his wishes were thwarted or demands were not met.\nThe patient had been shown to many doctors ever since his seizures started and his treatment prescriptions showed that for past 1 year he had been taking orally sodium valproate 500 mg twice daily, phenobarbital 60 mg twice daily, carbamazepine 200 mg thrice daily, and clonazepam 0.5 mg twice daily. The family reported only a small decrease in seizure frequency even after taking these medications. There was no history of any co-morbid medical and surgical illness in the patient or any history of similar features in any of the family members.\nGeneral physical and systemic examination was within normal limits apart from the findings of facial angiofibromas [] in the facial region. His body weight was 65 kg. Mental status examination was suggestive of low cognitive profile and did not reveal any other psychopathology.\nComputed tomography scan and magnetic resonance imaging of the patient displayed multiple subependymal nodules and small multifocal discreet lesions in bilateral cerebral hemispheres suggestive of cortical and sub-cortical tubers [Figures and ]. Electroencephalogram (EEG) of the patient was non-specific.\nManagement of the patient was carried out keeping in mind the following objectives:\nControl of grand mal seizures through optimization of treatment regime Assessment of patient's cognitive abilities and achievement of optimum level of adaptive functioning according to patient's cognitive abilities Behavioral modification aimed at reduction of undesirable behaviors and promotion of adaptive and socially desirable behaviors Detection and timely treatment of other manifestations of tuberous sclerosis to prevent complications.\nAs part of the management plan, the patient's medications were optimized and he was started on a single anti-epileptic drug sodium valproate at the dosage of 1000 mg/day (15 mg/kg/day). The rationale behind this step was that the patient's current medications regime comprised of multiple anti-epileptics medications two of which were enzyme inducers, i.e., carbamazepine and phenobarbital, which were reducing the effective dose of sodium valproate as well as carbamazepine resulting in reduced plasma levels. It was decided to subsequently adjust the dose of sodium valproate during subsequent follow-ups and to increase the dose gradually depending on the patient's tolerability and response to a maximum of 60 mg/kg body weight. Valproate was increased to 2 g/day in divided doses. Though, the patient's seizure frequency decreased markedly on this dose but he was not completely seizure free. Any effort to increase the dose of valproate resulted in intolerable gastrointestinal (GI) side-effects. Therefore, clobazam 10 mg/day was added to valproate and this resulted in complete improvement of patient's seizures. Currently, he is seizure free for past 2 months.\nThe patient's cognitive assessment (for Intelligence Quotient (IQ) assessment first parents were interviewed for development behavior on “Vineland social maturity scale” and then child was evaluated first on “Seguin form board test” and then on “Colored progressive matrices.” For cognitive assessment “bender gestalt test” and “Weschler memory scale” were done) revealed an IQ of 45, i.e., moderate mental retardation and a mental age of approximately 7 years with relative strength in visual-spatial domain. Parents and family members were psycho-educated regarding the patient's limited cognitive abilities and to lower down their expectations from the patient to bring it in coherence with the patient's cognitive abilities. Behavior therapy was started to reduce the frequency and duration of undesirable behaviors and to promote desirable behaviors by using the principles of negative and positive re-enforcement. The patient was given remedial education and was started from his current level of knowledge. After 3 months of behavior therapy and remedial education, there was marked improvement in the patient's behavior as evidenced by reduced frequency and duration of anger episodes and reduced need for help in daily routine activities. By the end of 3 months, the patient was able to read road signs in English and Hindi and write his name in both the languages and was able to do simple addition and subtraction problems.\nThe patient also underwent following investigations: Complete hemogram, liver function tests, renal function tests, ultrasonogram whole abdomen, echocardiography, and ophthalmic examination including, retinal and fundus examination. This was carried out to detect any other systemic manifestations of tuberous sclerosis, which might need treatment. These investigations were within normal limits.\nA dermatological consultation was also carried out for the treatment of cosmetically disfiguring facial angiofibromas since that was adding to the social embarrassment of the patient. The patient was started on tacrolimus 0.1% w/w ointment for topical application on the affected area. This resulted in visible reduction in the facial angiofibromas. No major adverse effects were noted except transient burning sensation at the site of application. |
A 22-year-old man presented with a swelling in the left ramus of the jaw 2 years ago. Examination revealed a unilocular radiolucent lesion, with a scalloped inferior border []. The CT scan revealed a well defined hyperdense soft tissue seen in the region of and below the left coronoid process of mandible, with suspicion of sclerosis. A partial mandibulectomy was performed and a reconstruction plate with a mini plate at the anterior region along with a fibular graft in the jaw was inserted to repair the defect []. Microscopy of the biopsied specimen revealed a diagnosis of central giant cell granuloma.\nAfter one year, the patient, now 23 years old, complained of a recurrent swelling in the same region. Intraorally, the patient presented with a growth in the left buccal mucosa at the level of the occlusal plane, which was excised and microscopically reviewed. Histopathological examination revealed it as a granuloma []. The first molar along with the premolars were removed, the region was curetted and a new reconstruction plate was given.\nA year later, the patient now 24 years old, was referred to the Department of Oral Surgery with the complaint of pain and recurrent swelling of the left jaw []. The patient had difficulty in opening the mouth. There was no paresthesia and both medical and familial histories were non contributory.\nClinically the lesion extended from the corner of the mouth to the anterior part of tragus on the left side, which was 4 × 4 cm in size, irregular in shape with a rough texture. The swelling was hard in consistency, showed no secondary changes and was non tender on palpation.\nIntraoral examination revealed an exophytic growth present posteriorly near the junction of the buccal mucosa and pterygomandibular fossa region, at the level of the occlusal plane, sized 1 × 1.5 cm and soft in consistency. It had a smooth surface with no fluctuation on palpation []. Presently the CT scan revealed an evidence of an expansile destructive mass (4.3 × 3.8 × 4.3 cm in the maximum anteroposterior, transverse and superoinferior dimensions) in the expected location of the left coronoid process, with thin residual septae like areas of osseous density seen in a large soft tissue mass. This soft tissue mass showed near isodensity compared to the adjacent muscles of the left masseteric space. The lesion expanded the insertion of the left temporalis muscle and bulged anteriorly into the left buccal space and posteriorly into the left condylar head and neck and left parotid gland. Medially, the lesion led to mild pressure erosion with thinning of the buccal cortex of the left maxillary tuberosity and bulged against the left medial pterygoid muscle [].\nRoutine hemogram and urine examination were normal. On the basis of clinical and radiological examination a provisional diagnosis of CGCG was made. The serum chemistry of calcium, phosphorous, parathyroid hormone was normal, there by excluding the possibility of hyperparathyroidism.\nSurgery was performed by a submandibular incision at the site of the previous scar, with the removal of the reconstruction plate, mini plate and graft, along with the condyloid process. The tumor mass and the margins of the normal tissue were removed. A careful and thorough curettage of the residual bone cavity was performed. The defect was repaired by a reconstruction plate attached to a condylar graft.\nHistopathological examination of excised specimen revealed evenly dispersed (2-3/HPF) giant cells each having 2-8 nuclei in them, in close approximation with proliferating blood vessels admixed with areas of haemorrhage. The connective tissue was minimal with vesiculated fibroblast proliferation []. The tumor mass had infiltrating margins and residual bony spicules towards the periphery. Even the bone graft attached to the condyle showed the presence of tumor giant cells []. No recurrence was noticed in post operative follow-up phase of 3 years [Figures and ] and further reconstruction of mandible using iliac crest graft is intended. |
A 64-year-old Japanese woman was brought to our emergency department after ingesting an unknown dose of glyphosate surfactant herbicide in order to attempt suicide. The woman had no history of psychiatric illness or neurological disease, however, she had a history of hypertension and hyperlipidemia, both of which were being medically controlled.\nInitially, she did not present with headache, dizziness, numbness, or any other neurological symptoms. She was also able to acknowledge the date and her hospitalization, and was able to describe her attempted suicide. The patient also described being depressed after losing her job three months prior to the event, and that she was not taking any antidepressants.\nSeveral hours after admission she became agitated and confused, apprehensively questioning where she was and why she was at a hospital. Following the manifestation of these symptoms, she was admitted to our psychiatric department.\nThe patient was found to be disoriented with regards to time and place, did not understand why she was in the hospital, and denied having attempted suicide. Although we initially thought this might have been due to depression or psychiatric illness, it became apparent that she was not able to remember events that had occurred after hospitalization. The patient was conscious of her memory impairment and disorientation, and became agitated about her condition to the point of delirium. As a result, she needed to be confined to a protection room for several days.\nWhile she was clearly in a state of delirium, a physical examination revealed her to be in a good general condition, with stable vital signs. We observed no focal neuropathological signs except for her memory deficit. Blood tests (including tests for complete blood count, electrolytes, urea, creatinine, and thyroid hormone) and serological test for syphilis yielded normal results. The results of her urine analysis and toxicological screens were also normal. A computed tomography (CT) scan of the brain showed only an old infarction in the bilateral basal ganglia, and we were unable to immediately perform a magnetic resonance imaging (MRI) scan due to her agitated state.A brain MRI scan without contrast was performed on day nine of her hospitalization and revealed a small high-intensity lesion in the dorsal part of the left hippocampal body on the diffusion-weighted image with a corresponding focus of low signal on the apparent diffusion coefficient map. Fluid-attenuated inversion recovery and T2-weighted sequences also revealed a high-intensity signal in a similar area (Figures and ). An electroencephalogram exhibited regular alpha activity, and the results of her cerebrospinal fluid analysis were within normal limits.\nWe diagnosed the patient with a left hippocampal infarction and administered a course of 75mg of clopidogrel once daily to be taken orally. Within three weeks since hospitalization, her disorientation to place and state of agitated delirium slightly improved. However, she still could not retain any memory of new events, and her orientation to time was still impaired. We performed the Wechsler adult intelligence scale-third (WAIS-III) and the Wechsler memory scale (WMS-R) for neuropsychological assessment. The WAIS-III revealed an IQ of 70, while the WMS-R yielded the following scores: Verbal memory 52, General memory 64, Delayed memory 65 (Table ). These results suggest that she had both retrograde and anterograde amnesia.\nA follow-up WMS-R performed two months later revealed a partial improvement in some domains (Table ), and no abnormalities were found on her follow-up MRI scan. Therefore, she was discharged from the hospital on day 77. Despite a half year of rehabilitation at another hospital, the patient continues to exhibit memory impairments, requiring a notepad to supplement lapses in her ability to recall. |
A 31-year-old male bomb-blast victim was brought to the emergency room. On examination, his heart rate was 64 beats/min, blood pressure 134/74 mmHg, and oxygen saturation 99% on room air. He was found to have penetrating wounds on the right side of the neck and the lateral side of the middle third of the right arm, with no active bleeding or hematoma formation. There was another wound in the right gluteal region without active external bleeding or hematoma formation. Neurovascular examination of the upper and lower limbs was unremarkable. The abdomen was soft without distension or tenderness.\nX-rays of the chest, abdomen, and right upper and lower limbs showed radiopaque foreign bodies in the right arm, right gluteal region, right thigh and right lower abdomen []. The radiopaque foreign body in the abdominal region was the largest one measuring 2 cm × 1 cm. An ultrasound of the abdomen revealed no free fluid in the peritoneal cavity. As the foreign body in the abdominal region appeared to be intraperitoneal on a plain X-ray, a CT scan of the abdomen was done which showed a dense metallic body within the lumen of the cecum. However, the CT scan did not rule out perforation of the caecal wall. Based on the outcome of this investigation, an exploratory laparotomy was decided and performed on the assumption that the intraluminal foreign body was shrapnel that had entered the cecal lumen as a result of the explosion and had caused an undetected perforation [].\nThe peritoneal cavity was explored through a midline incision, but no intraperitoneal bleeding, fecal contamination, or any sign of solid organ injury was found. The gastrointestinal tract from the esophagogastric junction down to the rectum was normal without any perforation or expanding hematomas. However, on palpation of the cecum and the ascending colon, a solid metallic object was felt in the distal part of the ascending colon. A C-arm image intensifier was used to confirm its presence. After confirmation of the presence of the metallic foreign body, the cecum and the ascending colon were mobilized. The posterior wall showed no evidence of perforation [].\nOn the 2nd postoperative day, an abdominal X-ray was done, which showed the radiopaque foreign body to be present on the right side of the abdomen. On the 5th postoperative day, the foreign body was no longer visible [].\nThe patient subsequently reported that he had passed a metallic object in the feces on the night of the 4th day. On a subsequent interview, he reported that at the time of explosion he felt a metallic object in his throat, which he swallowed reflexly. It was concluded that this metallic object was the shrapnel from the bomb explosion.\nPostoperatively, the patient was stable, tolerated a normal diet and was discharged home to convalesce uneventfully. |
A 79-year-old man had a history of hypertension, chronic kidney disease, distal gastrectomy due to gastric cancer at 62 years of age, and right spontaneous pneumothorax at 63 years of age. Aortic regurgitation was identified from a heart murmur and was followed up by a nearby doctor. He had no shortness of breath; however, he had gradually decreasing cardiac function and exhibited significant depression of aortic valve regurgitation. He was referred to our hospital for cardiac surgery. Doppler echocardiography showed severe aortic regurgitation and the right coronary cusp prolapsed toward the left ventricular outlet trunk in diastole and aortic valvular prolapse. There was no cutaneous abnormality upon physical examination. No major abnormality was found in the preoperative laboratory results.\nWe performed cardiac surgery via a lower partial median inverted L-shaped sternotomy that cut into the right second intercostal space. Since the right internal thoracic artery was exposed and stretched, we ligated and transected it. Cardiopulmonary bypass was established with right atrial drainage and ascending aorta return. After cooling to 33℃, the ascending aorta was clamped under ventricular fibrillation, an aortotomy was made, and the selective coronary perfusion resulted in cardiac arrest without problems. We replaced the aortic valve with a 23-mm bioprosthetic valve. Weaning from the cardiopulmonary bypass was without difficulty. The surgery was completed without blood transfusion. The patient’s postoperative blood pressure and heart rhythm were stable. However, a postoperative chest radiograph indicated a hematoma in the right upper chest wall and pleural effusion (Fig. ). This site did not appear to be damaged during the operation, although it was thought that the cause was blood dripping due to damage to the pleura during the operation. Therefore, we inserted a drainage tube immediately and transferred him to the intensive care unit.\nApproximately 2 hours after the surgery, the patient became anemic, and his blood pressure gradually decreased. This prompted us to transfuse blood. A small amount of blood drainage from the tube was observed. Blood retention was suspected and subsequently confirmed by Doppler ultrasounds. Then, chest radiography was performed again; findings were compared with the first postoperative chest radiograph. The second radiograph indicated enlargement of the hematoma (Fig. ). Considering the possibility of iatrogenic bleeding, we removed the central venous catheter and the Swan-Ganz catheter immediately. We observed a gradual drop in his blood pressure; however, we performed contrast-enhanced computed tomography (CT). This revealed massive hemorrhage and extravasation from the fourth right posterior intercostal artery (Fig. ), and no obvious fractures were found under bone conditions of the CT. Bleeding was immediately stopped because the patient was in shock. We performed emergency surgery through the previous lower partial sternotomy. We made an incision in the right visceral pleura, evacuated the 275-g hematoma from the cavity, and examined it carefully. The origin of the bleeding was identified in the fourth right posterior intercostal artery, although no rib fracture was noted on palpation of the lesion. The artery was cauterized with an electric scalpel and ligated. The patient’s postoperative course was uneventful, with no resumption of bleeding. |
Patient’s history
A 14-year-old girl presented to the tertiary referral Children’s Hospital orthopedic outpatient clinic due to dull pain and periodic wound drainage on the lateral side of her right thigh. Her mother noted two previous surgeries on that area performed at a county hospital. The first one, eight years ago, was performed due to right thigh swelling and fluctuance. These symptoms were initially suspected to be caused by pyomyositis of the vastus lateralis. A small longitudinal incision on the lateral side of the thigh was done and a purulent cyst was evacuated. Samples were sent for microbiological, cytological, and pathological examination. The laboratory reports were not evaluated properly by the treating surgeon, and the girl was discharged from the hospital with recommendations for oral antibiotics to treat pyomyositis. A month after the operation, purulent wound drainage appeared. The child was continued to treated with oral antibiotics and underwent frequent dressing changes, until complete wound closure was achieved. However, 10 months later, swelling, pain and purulent drainage reappeared at the place of the initial incision. The patient underwent repeated surgical incision and evacuation of what was considered to be a recurrent subfascial abscess. After surgery, the patient was instructed to continue with antibiotics and to dressing changes while waiting for wound closure. Unfortunately, her wound was not completely closed. For the next seven years, she suffered from monthly purulent drainage accompanied by moderate swelling and dull thigh pain that was most intense a few days before the appearance of drainage. During that period, there were no complaints about fever or malaise.
Physical examination and initial considerations
Seven years after the second surgery, physical examination performed at our institution showed a chronic wound on the lateral right thigh with mild drainage and local erythema. Palpation was slightly painful; however, there was full, painless range of motion of the hip and knee. A swab was taken for microbiological examination and an initial laboratory work-up was done. Microbiology results became positive for Pseudomonas stutzeri |
A 57-year-old female with mental retardation initially presented to a private hospital emergency room with complaints of left shoulder pain and decreased range of motion. The patient was a resident of a group home where she used to ambulate minimally and had no witnessed history of trauma, fall, or seizure. She had a medical history significant for mitral valve prolapse, hypercholesterolemia and bipolar disorder. Additionally, the patient had no reportable history of previous shoulder dislocation or instability. A focused history and exam at the time of presentation revealed the patient to be a poor historian given her mental retardation. On examination, she was found to be obese, with fullness about her left shoulder, and she was neurovascularly intact in the left upper extremity with limitation of range of motion in all planes. Neurologic exam and MRI were found to be negative for stroke. However, radiographic imaging of her left shoulder was found to be significant for an anterior shoulder dislocation []. At this time, the patient was evaluated by an orthopedic resident and she underwent an attempted closed reduction of her left shoulder under conscious sedation. Post-reduction imaging, including plain radiographs and CT, revealed that the shoulder was now dislocated posteriorly []. Again, the patient underwent a closed reduction with conscious sedation, with post-reduction radiographs revealing that the humeral head was now again dislocated anteriorly. Further attempts at reduction of this anterior glenohumeral dislocation under conscious sedation were unsuccessful. The patient was then medically cleared for surgery and repeat CT scan of the left shoulder was performed to assess for any bony defects and reasons for shoulder irreducibility. Oblique coronal and sagittal 2D reconstructions demonstrated the long head of biceps tendon dislocated lateral and posterior to the humeral neck and head. A small Hill-Sachs defect was also noted with a subcoracoid glenohumeral dislocation [Figures and ].\nAt this time, the patient was brought to the operating room for attempted closed reduction under general anesthesia. After general anesthesia was administered, attempt at closed reduction was again unsuccessful and open reduction was performed. A standard delto-pectoral approach was then performed with the patient in a beach-chair position. The interval was identified, the cephalic vein retracted laterally, and dissection down to the humeral shaft was performed. The humeral head was noted to be anteriorly dislocated in a sub-coracoid position. An attempt at reduction was again unsuccessful. The biceps tendon was then identified distally in the bicipital groove and followed proximally where it was noted to be incarcerated in the glenohumeral joint. The proximal tendon was dislocated from its anatomic position in the bicipital groove proximally and was tethered laterally around the humeral head, preventing reduction of the glenohumeral joint []. The biceps tendon was tenotomized from its origin at the superior glenoid tubercle and the portion of interposed tendon was excised, with a soft tissue tenodesis being performed at the level of the pectoralis major tendon. Next, the subscapularis was separated from the joint capsule, which was incised and inferiorly dissected off the humeral neck. The labrum was clearly visualized and was found to be intact inferomedially. At this point, the glenohumeral joint was reduced and taken through a full range of motion of internal rotation and external rotation with the arm in adduction and at 90° of abduction. The subscapularis and capsulolabral complex was then repaired through bony tunnels in the lesser tuberosity with #2 Fiberwire (Arthrex, Naples, FL, USA). The deltopectoral interval was then closed and a standard subcuticular closure performed. Intraoperative and postoperative radiographic imaging confirmed glenohumeral reduction. This patient had an uneventful postoperative course and was discharged back to her group home with visiting nursing services on the fourth postoperative day. Immediate hand, wrist, and elbow exercises were instituted, with passive range of motion exercises instituted at 2 weeks followed by gentle active assisted range of motion exercises. Active internal rotation was limited for 4 weeks. |
A 60 year old Caucasian male patient was referred to general surgery for multiple unrelated complaints including umbilical hernia and left arm lipoma. The patient also had an additional complaint of a fast-growing right leg mass located on upper lateral right calf distal to the knee. The patient had no other suspicious skin lesions and admitted to having the lesion shave biopsied two years prior by dermatologist with benign findings. No picture was taken of the lesion prior to surgical intervention as it was expected to be benign based upon prior dermatological findings. The patient stated that the leg lesion was non-painful in nature but was concerned that it may have increased in size over the previous 2 months and had a brown-gray discoloration. No prior imaging was obtained for the leg lesion. A wide margin elliptical excision was performed with a minimum goal of 1 cm margins on all sides of the lesion and the depth was resected to the muscle layer. The full specimen was marked for orientation and submitted to pathology. The excised elliptical portion measured 4.3 cm in length and 2.5 cm in width at widest points. The nodular lesion measured 2.4 × 1.8 × 0.9 cm. Ancillary studies showed that the lesion was CD31 positive, CD34 positive, and negative for cytokeratin markers. The pathology report confirmed EHE with tumor close to circumferential margins and present at the deep margin. The lesion was staged as pT1a pNX in accordance with AJCC staging. Given the deep margin extending to the thin layer of muscle just distal to the knee, the patient was referred to orthopedic surgery for further evaluation and operative intervention. The mainstay of treatment for invasive sarcoma is surgery often coupled with radiation and/or chemotherapy. A second surgical excision 19 days later following the original surgery was performed into deeper tissue. The second lenticular ellipse measured 8.1 cm in length and 2.1 cm in width at widest points at a depth of 1.4 cm. Multiple frozen sections were examined and clean margins of a minimum of 1 cm was determined in all directions.\nDue to the diagnosis of EHE, it was prudent to obtain additional imaging to determine if the malignancy had metastasized. Although incredibly rare, there have been documented cases of pulmonary epithelioid hemangioendotheliomas, as well as cases of tumors found on the liver. A CT scan was performed with IV and oral contrast of the chest and abdomen. The findings were unremarkable for the chest but multiple hepatic cysts and an enhancing lesion in the right lobe of the liver were identified. A follow up MRI with and without gadolinium confirmed a 2 cm well-defined focal area of delayed enhancement within the posterior segment of the right lobe of the liver corresponding to the CT findings, likely representing a cyst and not a metastatic lesion. These findings suggest that no metastasis had occurred and that the leg skin lesion appeared to be the primary site of EHE. |
A 60 year old Caucasian male patient was referred to general surgery for multiple unrelated complaints including umbilical hernia and left arm lipoma. The patient also had an additional complaint of a fast-growing right leg mass located on upper lateral right calf distal to the knee. The patient had no other suspicious skin lesions and admitted to having the lesion shave biopsied two years prior by dermatologist with benign findings. No picture was taken of the lesion prior to surgical intervention as it was expected to be benign based upon prior dermatological findings. The patient stated that the leg lesion was non-painful in nature but was concerned that it may have increased in size over the previous 2 months and had a brown-gray discoloration. No prior imaging was obtained for the leg lesion. A wide margin elliptical excision was performed with a minimum goal of 1 cm margins on all sides of the lesion and the depth was resected to the muscle layer. The full specimen was marked for orientation and submitted to pathology. The excised elliptical portion measured 4.3 cm in length and 2.5 cm in width at widest points. The nodular lesion measured 2.4 × 1.8 × 0.9 cm. Ancillary studies showed that the lesion was CD31 positive, CD34 positive, and negative for cytokeratin markers. The pathology report confirmed EHE with tumor close to circumferential margins and present at the deep margin. The lesion was staged as pT1a pNX in accordance with AJCC staging. Given the deep margin extending to the thin layer of muscle just distal to the knee, the patient was referred to orthopedic surgery for further evaluation and operative intervention. The mainstay of treatment for invasive sarcoma is surgery often coupled with radiation and/or chemotherapy. A second surgical excision 19 days later following the original surgery was performed into deeper tissue. The second lenticular ellipse measured 8.1 cm in length and 2.1 cm in width at widest points at a depth of 1.4 cm. Multiple frozen sections were examined and clean margins of a minimum of 1 cm was determined in all directions.\nDue to the diagnosis of EHE, it was prudent to obtain additional imaging to determine if the malignancy had metastasized. Although incredibly rare, there have been documented cases of pulmonary epithelioid hemangioendotheliomas, as well as cases of tumors found on the liver. A CT scan was performed with IV and oral contrast of the chest and abdomen. The findings were unremarkable for the chest but multiple hepatic cysts and an enhancing lesion in the right lobe of the liver were identified. A follow up MRI with and without gadolinium confirmed a 2 cm well-defined focal area of delayed enhancement within the posterior segment of the right lobe of the liver corresponding to the CT findings, likely representing a cyst and not a metastatic lesion. These findings suggest that no metastasis had occurred and that the leg skin lesion appeared to be the primary site of EHE. |
A 26-year-old female presented to the Breast & Endocrine Clinic after being referred from a district hospital with an increasing anterior neck swelling over a period of 6 months with symptoms of superior vena cava syndrome. She was previously well with no known medical, surgical or genetic problem. On physical examination, the patient had a congested facial appearance, with a large hard fixed anterior neck mass. She was ambulating well with neither features of respiratory compromise nor signs and symptoms of carcinoid syndrome. Initial differentials include a large multinodular goiter or thyroid malignancy. Chest radiography showed a widened mediastinum. In view of the large nature of the mass, we opted for a computed tomography (CT) of the neck and thorax instead of an ultrasound neck, as the CT scan would be able to delineate the anatomy of the mass in relation to its surrounding structures. The CT thorax showed a huge anterior mediastinal tumour with mass effect to the surrounding structures associated with multiple cervical and mediastinal lymphadenopathy (). The thyroid, however, is grossly normal on the CT scan. The differential diagnosis has subsequently shifted to primary mediastinal lymphoma. A core biopsy of the neck mass was taken under ultrasonography guidance and histopathological examination showed nests of tumour cells, scant cytoplasm and salt and pepper chromatin. The tumour cells were diffusely positive for synaptophysin, chromogranin and the Ki-67 proliferative index is 50%. These constellations of finding were suggestive of high-grade small cell NETs.\nAs her tumour was unresectable, she was referred to our oncology team for chemotherapy. She had first completed 6 cycles of Etoposide and Cisplatin. However, the response was poor and she subsequently underwent a further 3 cycles of Irinotecan and Carboplatin. Follow up CT showed stable disease initially, but had turned into a disease progression a few months later. She subsequently underwent radiotherapy to the neck and mediastinum with a total of 30 Gy in 10 fractions.\nRestaging CT scan showed enlarging anterior mediastinal mass with multiple new enhancing bilateral breast lesions and hilar lymphadenopathy (A). Upon reviewing the CT scan with the patient in the clinic, she complained of enlarging bilateral painless breast lumps over the past 3 months which was not previously present. She denied family history of breast and ovarian malignancies. Clinical examination revealed multiple mobile breast lumps with a firm consistency over bilateral breast of varying sizes. There was no axillary lymphadenopathy. This clinical picture is typical of a breast fibroadenoma. However, with her underlying history of a mediastinal NET, the diagnosis of breast metastases was kept in mind. Sonographic assessment of the breast was done to further characterize the lesions and showed multiple oval-shaped hypoechoic lesions with irregular margins and posterior acoustic enhancement (B). These suspicious features may represent primary breast malignancy or breast metastases.\nWith the possible diagnosis of breast malignancy, core biopsy of the bilateral breast lesions was performed instead of fine needle aspiration cytology (FNAC). Histopathological examination of the lesions showed malignant cells infiltration in sheets and nesting patterns (A). Immunohistochemistry studies show that the malignant cells were positive for synaptophysin (B) and chromogranin (C), and the Ki-67 proliferative index was 50%. These cells were negative for mammaglobin (D). These features confirmed the diagnosis of metastatic NETs to the breast.\nDespite the prompt initiation of second-line chemotherapy and radiotherapy, her tumour showed no signs of regression. She subsequently refused further treatment and was followed-up 3 monthly at our clinic to review her general well-being. She succumbed one year later due to neoplastic progression. |
A 29-year-old male patient was referred to our department for evaluation and treatment of a swelling in the left anterior maxillary region. The patient complained of a slow growing swelling over the left cheek which had increased over a period of 1 year. The patient had noticed an intraoral swelling 25-30 days back for which he had consulted his dentist. He also noted teeth displacement over a period of 1 year. The patient denied any history of trauma and was asymptomatic.\nThe patient's medical and family history was unremarkable. He had the habit of chewing tobacco and areca nut since 5 years. Clinical examination revealed a diffuse swelling on the left side of the face extending from the ala of the nose up to the zygomatic region measuring approximately 3 × 3 cm. On palpation, the swelling was hard with defined borders. The overlying and adjacent skin was normal; no regional lymphadenopathy was noted.\nOn intraoral examination, an irregular lobulated swelling was noted involving the entire left side of the palate, which had crossed the midline []. Labially the swelling extended from 21 to 24 with obliteration of the labial vestibule; the teeth in this region were displaced with mobility in relation to 21 and 22. The consistency was firm to hard and the mucosa was intact.\nWater's view showed a well-corticated radiolucent lesion destroying the inferior border of the maxillary sinus on the left side. A mixed lesion was seen in 22 and 23 region in continuity with the above mentioned lesion. Intraoral periapical radiographs showed displaced teeth i.r.t 21, 22, and 23. Computed tomography (CT) imaging showed an irregular soft tissue mass causing osteolytic destruction of left upper maxillary alveolus, floor and lateral wall of left maxillary sinus, medial wall of right maxillary sinus, and posteromedial part of right orbit [].\nThe differential diagnosis included odontogenic tumors since lesions like adenomatoid odontogenic tumor are common in this location and present a mixed appearance radiographically and therefore was considered. The clinical features such as displacement and mobility of teeth and the radiographic extent of the lesion suggested that it may be a non-odontogenic tumor of the bone.\nFine needle aspiration biopsy smears showed a matrix dominated lesion with moderate cellularity. The matrix was chondromyxoid in nature. Cells either single or in groups were embedded in the matrix. The cells showed abundant to moderate amounts of well-defined cytoplasm. The cytoplasm was eosinophilic and vacuolated. Binucleation and trinucleation was seen. Moderate degree of nuclear pleomorphism was noted. Some cells showed large nuclei with increased nuclear cytoplasmic ratio. Cartilage with cells in lacunae were not seen []. In view of the clinical and radiological data, a diagnosis favoring well-differentiated chondrosarcoma was offered. Following this, an incisional biopsy was carried out. |
We furthermore report on the case of a 58-year-old female patient who was referred to our department with a retroperitoneal tumor which was diagnosed in an external hospital. This finding had resulted from an abdominal ultrasound, which was carried out because of unspecific abdominal pain. A CT-scan described a tumor that likely originated from the IVC, however not being clearly distinguishable from the right adrenal gland.\nAfter mobilization of the liver, including segment one, the subsequent intraoperative finding revealed a large inferior caval tumor with an infrahepatic suprarenal localization that was consistent with the radiological statement. The tumor could not reliably be separated from the right adrenal gland. Thus, apart from a partial resection of the IVC, a right adrenalecomy en bloc was required. In contrast to patient 1, this tumor presented with a larger proportion of intravascular growth. Therefore, adequate oncologic resection required a complete circumferential resection of the IVC in addition to a right adrenalectomy en bloc, which was performed. Because the caval confluences of the renal veins were unaffected by the tumor on both sides, approximately 3 cm of the entire circumference of the infrahepatic IVC were resected as far as slightly above the renal confluences. Due to the low pressure in the IVC, the vascular continuity was reconstructed using a ring-enforced PTFE prosthesis 19 mm in diameter to optimize caval flow.\nBecause of the exogenous material implanted, the patient also received effective anticoagulation with heparin. On the second postoperative day, a relaparotomy was required under clinical suspicion of a secondary hemorrhage. Whereas this was confirmed, and hematomas in the right upper abdomen as well as within the omental bursa were detected, exploration of the entire abdomen could not reveal an origin of the bleeding. In particular, the exposure of the prothesis, including the anastomosis showed proper conditions. Thus, besides the removal of hematoma with abdominal lavage, no further intervention was performed. During the following course the patient recovered without complications.\nHistopathological examination showed a moderately differentiated caval leiomyosarcoma. with a maximum extension of 4 cm in diameter, and almost complete luminal obliteration, as well as tumor-free resection margins. In compliance with interdisciplinary consent, no adjuvant therapy was indicated. The follow-up examinations currently lasting up to a year after surgery showed no signs of tumor recurrence. |
A 67-year-old female patient presented with increasing pain in the right groin and severe weakness in the ipsilateral lower extremity, rendering her unable to bear weight or walk for 4 days. She underwent coronary angiography via a right groin puncture 4 days prior to admission in another hospital. She had a history of hypertension, coronary artery disease, and a body mass index of 32 (weight, 74 kg; height, 152 cm). She was not on anticoagulants. The medical records confirmed that femoral artery access was difficult and that it was performed without ultrasound guidance and required multiple puncture attempts. No closure device was used. However, it was noted that she was asymptomatic at the time of discharge.\nPhysical examination revealed tender induration and bruising in the right groin with no unusual pulsations or bruits. The patient was unable to extend her knee straight () and demonstrated significant weakness of the quadriceps femoris muscles with only grade 1/5 power according to the Medical Research Council scale of muscle strength. Numbness and tingling were also noted on the anteromedial aspect of the thigh and upper leg, suggesting severe femoral nerve dysfunction. The adductor and extensor compartments of the thigh did not exhibit any dysfunction. The increasing severity of local pain despite four analgesics and the onset of paralysis at 4 days post-puncture were suggestive of an expanding pseudoaneurysm stretching the adjacent femoral nerve rather than an intraprocedural direct nerve injury. Computed tomography angiography confirmed a DFA pseudoaneurysm measuring 4 cm×5.5 cm deep to the proximal part of the DFA ().\nBased on the perceived need to evacuate the hematoma and release compression on the nerve, we proceeded with urgent open surgery rather than an endovascular treatment. Surgical exploration confirmed that the underlying pseudoaneurysm had overstretched the entire femoral neurovascular bundle. The common femoral artery (CFA) and superficial femoral artery (SFA) were dissected off the floor of the femoral triangle and controlled with clamps. A direct incision was used to evacuate the bulging hematoma, and the bleeding DFA branch was sutured and ligated (). The surgical site healed without any complications. Following intensive rehabilitation with electrical stimulation and quadriceps strengthening exercises, complete muscle recovery was achieved in 12 weeks (). |
A 30-year-old male presented to emergency hospital complaining of penile pain, weak urinary stream and dribbling at the end of micturition for 2 months duration. The pain was constant pain and aggravated with movement, urination, and sexual intercourse and relieved by rest. The color of the urine was normal during this period.\nIn the last 2 weeks the patient developed severe dysuria along with development of a tender nodule over the dorsal surface of the penis. The patient visited the emergency department. An attempted urethral catheterization was failed at that time.\nThere was no history of urethral trauma or urethral instrumentation. The past medical and surgical histories were negative, and the drug history were negative.\nThe family history for any relevant genetic information and psychosocial history was negative.\nThe general examination was unremarkable. Examination of the genitalia revealed a normal urethral meatus but there was a fistula at dorsal mid penile shaft and the urine were coming out from that opening, there was surrounding redness and edema with palpable firm nodule in the penile shaft ().\nThe patient was then sent for investigations, the renal function was normal with normal complete blood count. The urinalysis showed full pus cells and red blood cells in urine. Ultrasonography of abdomen revealed normal bladder wall with normal ureters, no stones and no hydronephrosis. A pelvic x-ray revealed a mid-urethral radiopaque shadow ().\nThe patient underwent an emergency cystoscopy which revealed an impacted stone in mid bulbar urethra, attempts of stone extraction was failed, then decision was made to remove the stone by the open procedure ( & ).\nAn open urethral incision was made at ventral surface of the penis and then removal of the stone was done along with dorsal fistulectomy and repair was performed. The patients received broad spectrum antibiotics parentally. Foley's catheter was placed and removed later after 21 days ().\nThe patient had uneventful postoperative period and the follow up was done up to 6 months with no postoperative complications. The patients was informed to visit the hospital if he develop any urinary symptoms like difficulty in urination, dysuria, poor stream, and difficult in initiating the urination. |
This patient is a 54-year-old homeless man with a history of schizophrenia who presented to the emergency department in November 2020 after being found down with a penetrating injury to the left anterior chest. On arrival, his GCS was 3, and he was being ventilated via bag valve mask by EMS. He was promptly intubated for airway protection. His vital signs were stable. On further exposure, he had an approximately 3 cm stab wound to his left anterior chest, medial to the nipple line, at approximately the level of the 7th rib. Otherwise, he was noted to have ecchymosis of the occiput and superficial lacerations to his right flank. Also concerning was a fixed and dilated left pupil. A pan CT scan revealed a left subdural hematoma with bilateral frontal hemorrhagic contusions and 5 mm of midline shift. Additionally, he was found to have a moderate left-sided pneumothorax and associated left anterior 7th rib fracture for which he underwent chest tube placement with resolution of his pneumothorax (). A cerebral oxygenation monitor and drainage device was placed by neurosurgery revealing an elevated opening pressure of 31 mmHg. At this time, there was also concern for possible diaphragmatic injury given the location of his stab wound and imaging findings; however, in the setting of his elevated intracranial pressures, he was deemed unsafe for the OR and admitted to the neuro ICU for further monitoring.\nOn hospital day 0, his routine workup included a negative COVID-19 nucleic amplification assay. His intracranial pressure was controlled using mannitol and hypertonic saline bullets while he was started on levetiracetam for seizure prophylaxis. Repeat CT imaging the following day showed stability in his intracranial bleeds and a decrease in midline shift. His mental status improved, and he self-extubated on hospital day 3. After his intracranial pressures normalized his drains were removed, and on hospital day 6, he underwent diagnostic laparoscopy to rule out diaphragmatic injury given the location of his initial stab wound. Prior to this procedure, he had a repeat COVID nucleic amplification assay as per institutional policy, which again resulted negative.\nFollowing this intervention, his condition continued to improve. His diet was advanced as tolerated and he was otherwise normalized, although he continued to have issues with agitation and altered mental status secondary to his traumatic brain injury and underlying schizophrenia. Psychiatry was consulted for titration of his medications, and plans were made for discharge to a TBI rehab; however, his discharge was complicated by his homelessness status and frequent behavioral outbursts, occasionally requiring chemical sedation. Beginning on hospital day 13, he was given a one-to-one bedside nursing assistant and was occasionally placed in a SOMA Safe Enclosure bed.\nIn anticipation of discharge, repeat COVID nucleic amplification assay was done in compliance with rehabilitation center policies. Tests sent on hospital days 49 and 50 both resulted negative; however, an additional test sent on hospital day 55 resulted positive. As before, the patient was given an isolated room and placed on contact, droplet, and airborne precautions. Later that evening, the patient became febrile to 103°F but refused additional fever workup. A second COVID nucleic amplification assay was repeated the following day, which confirmed his COVID-positive status. He was treated with a 10-day course of dexamethasone as per infectious disease recommendations, and his fevers resolved. Repeat COVID testing on hospital day 66 remained positive, and he had his first negative test on hospital day 72. Shortly thereafter, he was accepted to a facility for ongoing rehabilitation. |
-Case 1: a 29 years old man consulted the emergency service of our hospital for tooth pain, swelling of the submandibular region and fever. The only important information that the patient referred was tooth pain (tooth 48) since four days. Patient exploration revealed macroscopic tooth decay of 48, trismus, floor of the mouth inflammation and diffuse pain on palpation of the submandibular zone. In view of that, intravenous antibiotic treatment was administered and a CT of cervical area was carried out. CT images showed the presence of a serious infection which spread from the tooth 48 to the posterior mediastinum. The patient was admitted in intensive care unit care unit of our hospital. A few hours later, thoracic and maxillofacial surgeons carried out the surgical drainage of the abscess. Moreover, we decided to performa surgical scrubbing of the infected area every 8 hours. In addition, a long treatment with intravenous broad-spectrum antibiotics was established. After surgery, the situation began to improve. Almost two months later, patient was discharged from the hospital.\n-Case 2: a 43 years old man consulted the emergency service of our hospital for tooth pain and swelling of the right submandibular region. Patient referred that symptoms began two days before. Clinical exploration revealed trismus and hardening of the floor of the mouth tissues. A careful oral examination was hampered due to the intensive trismus. Thus, we decided to perform orthopantomography and a CT of cervical area in order to achieve a prompt diagnosis. Orthopantomography shows a wide tooth decay of 48. In this light, CT images showed a severe infection that spread from the tooth 48 to the posterior mediastinum. Against this backdrop, patient was admitted in intensive care unit care unit of our hospital. On the same night, a group of thoracic and maxillofacialsurgeons carried out the surgical drainage of the abscess. On this occasion we also performed the cleansing of the infected area every 8 hours. Moreover, a long treatment with intravenous broad-spectrum antibiotics was established. In the following days, patient evolved favorably and was discharged after almost forty-day of hospital stay.\n-Case 3: a 61 years old man consulted the emergency service of our hospital for tooth pain, trismus, swelling of bilateral parotid and submandibular region, dyspnea and hardening of the floor of the mouth tissues. Considering the above, an orthopantomography and a CT of cervical area were immediately carried out. This tests showed a grave infection that originated from the tooth 38. Importantly, CT images evidenced the presence of a large amount of air into parotid and submandibular region. Moreover, this test showed a collection of pus that extended from the tooth 48 to the posterior mediastinum. In the light of these developments, patient was admitted in intensive care unit care unit. Thoracic and maxillofacial surgeons carried out the surgical drainage of the abscess as quickly as possible. As before, we performed the cleansing of the infected area every 8 hours. Furthermore, a long treatment with intravenous broad-spectrum antibiotics was also established. Fortunately, infection evolved satisfactorily in this patient too. Approximately two months later, he was discharged from the hospital, (Figs. -). |
A 37-year-old woman, gravida 3, para 2+0, at 27-week gestation was transferred from a peripheral hospital with a three-day history of haematemesis and melaena requiring 10 units of red blood cells. An endoscopy prior to transfer showed numerous clots in the stomach but no bleeding source was identified. She had no significant past medical or family history and her pregnancy had been uncomplicated to date. Prenatal investigations had revealed a diagnosis of trisomy 21 and a congenital cardiac defect.\nOn presentation, she was haemodynamically stable; her haemoglobin was 9.3 g/dL. Ultrasonography confirmed a viable pregnancy. Urgent upper gastrointestinal endoscopy revealed extensive gastric varices but no bleeding point and no oesophageal varices (). Contrast enhanced magnetic resonance imaging (MRI) with diffusion-weighted sequences of the abdomen was subsequently performed which revealed a 9 × 7 × 8 cm mass infiltrating the pancreatic tail and spleen with splenic vein invasion, as well as consequent massive gastric varices along the greater curve and fundus (). There was no evidence of liver metastases. Completion staging computed tomography (CT) of the thorax revealed no pulmonary metastases.\nFollowing consultation with the obstetrics team and the patient and her family, a plan was made to proceed with early surgical resection, given the high risk of rebleeding, with delivery of the foetus planned for the same day. She was given corticosteroids for 4 days and then underwent initial uncomplicated caesarean section followed by a radical en bloc partial pancreatectomy and splenectomy with resection of the fundus of the stomach and ligation of massive short gastric varices (). Her postoperative course was uncomplicated and she was discharged home after two weeks. The neonate was transferred to a specialist neonatal unit and then discharged to the patient's local hospital for supportive care. He was discharged home well 6 weeks later.\nHistology revealed a well differentiated grade 2 neuroendocrine tumour with a Ki67 of 10% and a mitotic rate 6 per 10 HPF. The surgical margins were free of tumour and 0/3 lymph nodes were involved. Final staging was T4N0. Her case was discussed at the gastrointestinal cancer multidisciplinary meeting and no further treatment was recommended.\nInterval imaging with contrast enhanced CT thorax, abdomen, and pelvis at 3, 6, 9, and 18 months was performed with no evidence of disease recurrence or metastases. At 3 months postoperatively, an indium-111 octreotide single-photon emission CT scan (OctreoScan) demonstrated no evidence of metastases. At last follow-up and 24 months postoperatively the patient was well and free of disease. |
A 58-year-old male patient was admitted to hospital because of right kidney calculi that were found during a routine examination over 3 months before hospital admission. Relevant examinations such as a plain X-ray of the abdomen (KUB) (), intravenous urography (IVU), and abdominal computed tomography (CT) were performed after admission to our hospital. Percutaneous nephrostomy was performed to remove the stone. The prone position was used to perform the PCNL. Between the posterior axillary line and scapular line, the puncture site was localized to the 11th intercostal space. Percutaneous punctures that were guided by ultrasound were performed using an 18-gauge needle. Urine in the renal pelvis was not completely aspirated after the puncture. A zebra guide wire was inserted into the collecting system. We judged that the guide wire should be coiled in the renal pelvis after a successful puncture. Access to the excretory system was achieved gradually by fascial dilators ranging from the smallest size to 20F. Severe bleeding from the sheath immediately after dilator removal led to a sudden interruption of the procedure. An 18 F nephrostomy catheter was promptly inserted and closed to control the bleeding.\nA quick nephrostogram was not performed after placement of a nephrostomy tube to locate the position of the nephrostomy tube. Blood loss was estimated to be from 400 to 500 mL, which was mainly due to outflow from the fistula channel via the skin of the nephroscope, and it was mixed with some flushing water and urine. Hemoglobin levels decreased by 15 g/L after surgery, and the patient also had mild hematuria. His blood pressure did not decrease. We thought that the bleeding was caused by damage to the renal parenchyma and microvascular structures. A plain CT scan of the kidney that was performed 5 days after surgery showed that there was a great possibility that the nephrostomy catheter had traversed from the middle to lower pole of the right kidney directly into the right renal vein and ending in the IVC ().\nAbdominal computed tomography angiograph (CTA) and three-dimensional reconstruction () were immediately applied to assess the patient’s specific condition, which revealed that the nephrostomy tube had pierced the renal parenchyma, entered the right renal vein, and extended up into the IVC. We immediately ordered the patient to remain on strict bed rest until 2 weeks after surgery. In the postoperative 2 weeks, the nephrostomy tube was withdrawn from IVC and right renal vein into the collecting system, guided by X-ray with interventional treatment, and during this time, a surgical team was on emergency standby. Right renal artery, right renal vein, and IVC angiography were performed during the catheter withdrawal. The tube withdrawal process was smooth and no bleeding occurred, and the patient’s postoperative course was uneventful. Five days after the catheter was withdrawn into the collecting system, the patient underwent an unenhanced CT (). He was discharged on day 7 after tube withdrawal, and the residual stones were treated using PCNL 1 month after discharge. |
In april 2010 a 16-year old female patient showed herself in the Central Interdisciplinary Ambulance in the School of Dentistry, University of Münster for consultation. Her dentist intended to perform a prosthetic full crowning of all teeth due to the generally hypoplastic enamel (Fig. ). The parents of the patient asked for the necessity of prosthetic treatment at that point of time. An orthodontic treatment to regulate a minor existing malocclusion was completed at that time. Her dentist recognized the alteration of the teeth upon eruption of the deciduous teeth and later the enamel malformation was also evident in the permanent teeth.\nThe medical history was unremarkable. Since the diagnosis of AI the patient got frequent appointments at her dentist for high fluoride varnish and professional dental care every 6 weeks. Beside that the patient performed an in-house fluoridation once a week with high dose fluoride and daily use of fluoride containing tooth paste for tooth brushing.\nA familial clustering was reported by the patient and her parents. Affected subjects were known in different branches of the family. Her sister was also affected in an equal manner. No further dental findings or caries were recognizable.\nThe suspected diagnosis was amelogenesis imperfecta type I according to the classification of Witkop []. The diagnosis was based on the phenotype and family history of the relevant findings of amelogenesis imperfecta.\nDue to the partially exposed dentin and lack of regularly formed overlying enamel the teeth of the patient showed hypersensitivity upon chemical and physical stimuli. Especially sweet food and beverages caused increased disturbance by pain. As a consequence, the patient altered her alimentary habits and avoided the consumption of all kind of food that caused pain.\nBeside the limitation of food consumption, the major discomfort was the reduced esthetic appearance due to the malformed tooth shape and appearance of the visible anterior teeth of the upper and lower jaw. Due to the malformed tooth shape and the discolouration the patient reported negative social influence when trying to hide the altered teeth during talking and laughing to each other.\nDue to the well done frequently appointments and fluoridation at her dentist and the good oral hygiene no signs of gingivitis were present. The only filling was found within the lower left second molar.\nOn dental examination a complete adult dentition without the upper and lower third molars was recognizable. Due to a former defect the last lower left second molar was restored with a composite resin filling. No other restorations were recognizable.\nThe shape of the visible clinical crowns of the permanent dentition was altered by the hypoplastic enamel. As reported before the patient mentioned similar alteration within the deciduous dentition.\nPrior to the suggested treatment an orthopantomogram was done by the former dentist (Fig. ). A complete permanent dentition was recognizable, but the lower third molars were still unerupted. The orthopantomogram revealed that there was an agenesis of the upper left and right third molars. The residual covering enamel showed no reduced radiolucency but reduced thickness compared to the underlying dentin. No radiolucency corresponding to carious lesions was found. The pulp chambers especially of the upper and lower first and second molars showed reduced volumes compared to the mean volume of such teeth in patients of the same age not affected by AI. The length of the tooth roots appeared to be in normal range, while the interdental spaces, especially in the lower jaw appeared enlarged.\nIn advance of the first presentation in the central interdisciplinary ambulance an orthodontic treatment was performed by the orthodontist near the patient’s home. Due to the hypoplastic and partly aplastic enamel the orthodontic treatment was done by using the Invisalign system (Align Technology Switzerland GmbH, Rotkreuz, Switzerland). The aim of the orthodontic treatment was a pre-prosthetic alignment of the teeth suggested by the former dentist. The upper and lower dental arches were harmoniously shaped, and a slight gap position of the approximal spaces was also adjusted. However the designated prosthetic restoration was not carried out.\nCast models were made after dental impressions with alginate to preserve the initial oral situation prior to further treatment. To determine possible alterations of the esthetic aspects a measurement of the upper face third from hairline to glabella (5 cm), middle face third from glabella to subnasal (5 cm) and lower face third in maximum intercuspidation from subnasal to menton (4.7 cm) was done. Additionally, a deep bite of 5 mm was measured, whereas an overbite of 1–2 mm can usually be measured; the difference corresponds approximately to the sum of the missing enamel thickness, which in non-pathological cases is found in the posterior region []. The vertical dimension was not elevated in agreement with the patient.\nThe restoration of all teeth of the upper and lower jaw except upper left lateral incisor and the lower left premolars was done by direct composite resin fillings. Due to existing occlusal contacts located in the areas of residual enamel within the maximum intercuspidation the occlusal relation was secured without further alterations and without the need of elevating the vertical dimension. Five appointments were scheduled for the entire treatment. During each appointment the teeth of a quadrant and the anterior upper incisors were treated successively. During each appointment the restoration of the teeth was done after adaption of rubber dam and additionally insertion of a braided retraction thread of size 00 (Ultrapak Cord, Ultradent, South Jordan, UT, USA) impregnated in Orbat (lege artis Pharma, Dettenhausen, Germany) in the sulcus gingivalis in order the prevent efflux of sulcus fluid and blood during restorative therapy.\nAfter total etch of the tooth surfaces by phosphoric acid (38%) (Orbis Dental, Münster, Germany) the chosen dentin adhesive Optibond FL (Kerr, Orange, CA, USA) was applied to the etched surfaces according to the manufacturer’s specifications to ensure an appropriate bonding of the composite resin restoration to the residual enamel and underlying dentin. For restoration of the hypoplastic teeth the nanofilled hybrid composite resin Grandio (VOCO, Cuxhaven, Germany) was used. In agreement with the patient the molars and canines were restored with the shade Vita A3 whereas the premolars and incisors with the shade Vita A2.\nThe exposed dentin and residual enamel of the dysplastic teeth was covered in anatomic shape by the use of composite resin restoration and light cured with a LED polymerization light at wavelength of 450 to 490 nm (SmartLite PS, Dentsply, Konstanz, Germany) for 20 s per increment. The restoration of the occlusal, buccal and oral surfaces was done by free-hand modelling whereas the proximal parts of the teeth were modelled with matrices (Orbis Dental, Münster, Germany) and interdental wedges (Kerr Hawe, Bioggio, Switzerland).\nAfter completion of the restorations all restored surfaces were contoured, the occlusion was corrected by yellow coded diamond burs (Brasseler, Lemgo, Germany) and polished with Identoflex Composite Polishers and Occlubrush (both KerrHawe). The treatment of the upper jaw required 3 appointments whereas the treatment of the lower jaw required 2 appointments.\nIn agreement with the patient, some teeth (upper left lateral incisor and the lower left premolars) and some lingual and palatal surfaces were not treated due to an existing enamel layer. Due to insufficient distal margin the filling of the second left lower molar was retreated (Fig. ).\nAfter the completion of the treatment the patient got intensive oral hygiene instructions. The fluoridation schema containing fluoride varnish in the dental office once every 6 weeks, application of high dose fluoride formulation once a week with Elmex Gelée (GABA, Lörrach, Germany) or Sensodyne PROSCHMELZ Fluorid Gelée (GlaxoSmithKline, Bühl, Germany) and daily oral hygiene with fluoride containing tooth paste, toothbrush and dental floss was recommended. Beside also appointments for dental control every 3 months in the dental office were recommended.\nAfter carrying out the restoration the patient decided to undertake the recommended regular appointments and dental care at her family dentist near to her home in order to avoid frequently long travels for appointments to the School of Dentistry of Münster.\nSeven months after completion of the restoration the first follow-up was performed. All restorations were sufficient and no discolouration was visible. Solely a slight bleeding on probing located in the sulcus gingivalis of the right lower canine was visible but no other signs of gingival inflammation were recognizable.\nAfter about 2 years (28 months) without regular appointments for control at the School of Dentistry in Münster the patient showed again for retreatment. Some restorations showed secondary caries especially located in the interdental spaces of the both upper first molars and the second left lower premolar. The defects of the teeth were again treated in the same manner as described with dentin adhesive fillings. The composite resin used was Grandio SO (VOCO, Cuxhaven, Germany).\nAbout 4 years (46 months) after initial treatment the patient showed herself for dental control. Bitewing radiographs of the right and left side were taken (Fig. ). Some restorations showed radiolucency of the margins due to secondary caries and caries without association to former restorations especially within the interdental spaces. A restorative therapy of the affected teeth was done in the same manner as initially performed with total etch technique, dentin adhesive and composite resin (Tetric Evoceram, Ivoclar Vivadent, Schaan, Lichtenstein). Treatment was necessary to the upper right jaw (second premolar to the second molar), the upper left jaw (first premolar and second molar), the lower left jaw (second premolar to the second molar) and the lower right jaw (second premolar and fist molar).\nSixty-eight months (5.5 years) after restoration the patient showed herself for another follow-up control. Due to secondary caries the restoration of the second lower right molar showed an insufficient margin. A retreatment was done with total etch technique, dentin adhesive and composite resin (IPS Empress Direct, Ivoclar Vivadent).\nThe last follow-up was done about 9 years (110 months) after initial treatment. During the appointment some composite resin fillings showed insufficient margins (second upper left premolar, first lower left molar, first and second lower right molar) and had to be retreated (Fig. ). |
A 58-year-old male patient presented with a one-week history of bloody otorrhea from the right side. In the past 34 years, the patient had undergone four surgeries to remove the masses from his right parotid gland. Histopathologic examinations performed after the first three surgeries indicated the presence of benign pleomorphic adenomas. The latest surgery was performed nine months ago to remove a 3.3 cm mass from the parotid gland. Following this surgery however, a histopathologic examination revealed a true malignant mixed tumor (carcinosarcoma) composed of poorly differentiated carcinoma and osteosarcoma. Immunohistochemical studies were positive for P53, Ki67, and vimentin, weakly positive for cytokeratin, and negative for Actin and the S100 protein. The patient was given adjuvant radiotherapy after the latest surgery and was doing well except for symptoms associated with irradiation.\nThe patient underwent a follow-up high-resolution CT scan of the temporal bone for the presented symptoms and showed extensive opacification of the right mastoid air cells, posterior petrous bone, middle ear, and the external auditory canal. The imaging appearance raised the suspicion of a recurrent tumor. Excisional biopsy of the external auditory canal mass was performed and confirmed the diagnosis of a recurrent true malignant mixed tumor.\nDuring this hospitalization, a chest radiograph indicated the presence of patchy opacities in his left lower lung field () not seen in previous chest radiographs; this warranted a CT scan for further characterization. A CT scan of the chest showed parenchymal consolidation with some amorphous calcifications (); hence, the possibilities of fungal infection or granulomatous disease were considered initially. This patient had no fever and did not report associated symptoms of infectious or inflammatory respiratory tract diseases; therefore, a CT-guided biopsy of the pulmonary lesion was performed because of the concern of atypical pulmonary metastases. The biopsy generated two pieces of specimen that were used for histopathologic examination.\nMicroscopically, the specimen showed nearly total necrosis and the presence of atypical cells; within the mesenchymal element, some osteoid materials and foci of osteonecrosis were noted (). Malignant osteoblastic cells surrounding osseous materials were also noted (). Immunohistochemical studies were positive for vimentin, negative for TTF-1 (thyroid transcription factor-1) and cytokeratin. A review of the surgical specimen from the patient's right parotid gland nine months ago revealed similar findings. The specimen obtained during the lung biopsy showed no carcinomatous component, and did not demonstrate tumor emboli in pulmonary vessels. A radionuclide bone scan did not reveal increased activity at locations other than right neck and lung. The histopathologic analysis of the lung biopsy suggested a metastatic true malignant mixed tumor. Retrospective reading of his neck CT nine months ago clearly depicted calcification within the right parotid gland tumor (). |
A previously healthy 37-year-old male was referred to our hospital with a diagnosis of dengue hemorrhagic fever with shock. His clinical course began 6 days prior to transfer when he presented to an outside hospital with fever. He was found to have left sided hemothorax on arrival causing respiratory distress in which intercostal drainage was promptly placed. However, his condition remained unstable following the procedure requiring intravenous norepinephrine for hypotension. His clinical course was shortly complicated by acute renal failure necessitating renal replacement therapy via right internal jugular catheter on hospital day 2. Despite supportive treatment, lactic acidosis was newly demonstrated together with the need for higher dose of vasopressive drug, various antibacterial regimens were prescribed throughout hospitalization and meropenem and vancomycin were selected as initial combination.\nOn hospital day 3, due to progressive respiratory distress and failure on lung recruitment maneuver, extracorporeal membrane oxygenation (ECMO) was initiated via right femoral catheter. He was also noted to have pancytopenia. Bone marrow study findings were compatible with hemophagocytosis syndrome secondary to dengue infection. Intravenous dexamethasone (10 mg/m2) and intravenous immunoglobulin were started and plasmapheresis replaced immunoglobulin therapy on the following day. Hematologist was able to reverse pancytopenic event 7 days later but profound lymphopenia persisted throughout his hospital course with the highest absolute CD4 count of 90 cells/mm3.\nOn hospital day 11, ECMO was discontinued and right femoral catheter was removed after stabilization of overall condition. However on hospital day 13, he underwent left below knee amputation for better control of limb ischemia presumably secondary to ECMO complication that had been documented since hospital day 8. His clinical course was also complicated by 1.) Cytomegalovirus reactivation during dexamethasone therapy and required brief duration of ganciclovir treatment given no specific organ involvement identified and 2.) Chromobacterium viloaceum pneumonia and secondary bacteremia on hospital day 18. Bacterial pneumonia was very difficult to control that he required a prolonged course of antibiotics consisting of piperacillin/tazobactam and ciprofloxacin to the point that left pneumonectomy was indicated on hospital day 46. After the surgery, he was prescribed several short courses of antibiotic treatment mainly in order to prevent gut microbial translocation following numerous events of gastrointestinal bleeding. Hemorrhagic complication was later discovered to be secondary to large stress associated rectal ulcer. He died on hospital day 71 due to massive esophageal bleeding that autopsy findings failed to identify infectious etiology at the bleeding site.\nRegarding fungal infection risk particularly IA following severe dengue infection, micafungin was begun on hospital day 3 after serum galactomannan (GM) was obtained which later reported to be 2.3 by enzyme-linked immunosorbent assay (ELISA). Liposomal amphotericin B replaced micafungin for new diagnosis of probable IA that chest radiograph now displayed faint opacities in right middle lung field. In addition, anidulafungin was added in response to rapid progression of pulmonary lesions on chest radiograph. After 6 days of combination therapy, serum GM level declined to 0.55 as the lowest value but no change on chest radiograph findings and respiratory status.\nOn hospital day 18, micafungin replaced anidulafungin in light of possible cardiac toxicity. Bronchoscopy was done to determine the potential pathogenic fungi but biopsy was avoided given very compromised respiratory status. Antifungal regimen had been switched to and fro between liposomal amphotericin B and amphotericin B deoxycholate due to a fear of drug induced cholestatic jaundice from hospital day 21 until day 31 when polyene antifungals were halted completely after a recognition of A. terreus growth on every respiratory specimen. Intravenous and nebulized voriconazole and oral flucytosine via nasogastric tube were now chosen. Despite of therapeutic voriconazole levels in the range of 3.29–4.89 mg/L, pulmonary lesions were still growing in size with increment of serum GM to 9.73 and worsening respiratory parameters over a period of 10 days. A decision to switch treatment to intravenous posaconazole was made in view of potential SBECD accumulation. Plasma levels of posaconazole were measured to assure drug exposure adequacy. MICs of current A. terreus infection to voriconazole, posaconazole, caspofungin, anidulafungin, micafungin were 0.75, 0.125, 1, 0.004, and 0.064 mg/L, respectively.\nDetermination of posaconazole concentrations in plasma was performed using validated Ultra Performance Liquid Chromatography-Photo Diode Array (UPLC/PDA), according to the US Food and Drug Administration guidance for bio-analytical method validation []. Blood samplings for plasma posaconazole levels were performed dividing into two phases. The first phase took place when posaconazole 300 mg was given every 24 h, spotted plasma levels were obtained to determine the appropriate timing of the following doses in which resulted in a new dosing regimen of every 48-h interval. In the second phase happened when posaconazole 300 mg was given every 48 h, blood samples were collected for pharmacokinetic analysis at 0 h (predose), immediately at the end of infusion, approximately 15 min after the end of infusion, and approximately 4, 8, 12, 24 and 48 h after the start of infusion. After 34-day course of posaconazole with confirmation of therapeutic plasma posaconazole levels almost the entire period of time during posaconazole treatment (0.956–7.099 mg/L), his infection still did not subside. Serum GM prior to his death was 8.97. Autopsy result confirmed diffuse IA in his right lung without evidence of dissemination to other organs as well as no bacterial pneumonia documented. Additional immunological tests also declared impairment of natural killer T-cell function. Plasma posaconazole levels and serum GM are shown in Additonal file : Figure S1. An additional figure file shows summary of his clinical course [see Additional file ].\nLiver function tests and QTc interval on electrocardiography data prior and during posaconazole treatment are displayed in Table . The findings demonstrated a decline in aspartate transaminase (AST) and alanine transferase (ALT) levels significantly after posaconazole use (p =0.005 and 0.028, respectively). On the other hand, there was an increase in alkaline phosphatase (ALP) and gamma-glutamyl transpeptidase (GGT) levels after posaconazole use with statistical significance (p = <0.001 and 0.039, respectively). There were no crucial changes on total bilirubin (TB), direct bilirubin (DB) and QTc interval. Statistical analyses on relevant parameters to compare the differences prior and during posaconazole therapy were performed using SPSS version 21.0 for Windows (IBM Corp., Armonk, New York). The distribution of continuous variables was determined by Kolmogorov-Smirnov test. All continuous variables were assessed by the Student's t-test or Wilcoxon Rank Sum test as appropriate. For normally distributed variables, data were described by mean ± standard deviation (SD). For skewed variables were described as median (range). A p value of less than 0.05 was considered significant for all statistic values. |
A 43-year-old woman was admitted under the surgical team with abdominal pain, vomiting and 2 kg unintentional weight loss in past 3 months. She was diagnosed as a new case of Crohn’s disease. CT abdomen-pelvis performed during admission revealed complicated 9 cm terminal ileal wall thickening with narrow lumen and loculated perforation of the terminal ileum with a small abscess in the right iliac fossa. Thereafter, she underwent a right hemicolectomy, followed by another laparotomy 2 weeks later to perform adhesiolysis and refashioning of the anastomosis as she was found to have terminal ileal adhesion to with the pelvic wall along with a small hole in anastomotic site. She was started on parenteral feeding after the laparotomy as was not able to tolerate enteral feeding. A PICC line was inserted in her left internal jugular vein following which antero–posterior chest X-ray confirmed satisfactory position of the PICC line. She had no respiratory distress for the first couple of days post PICC line insertion. Two days after the commencement of total parenteral nutrition (TPN), she was noted to become breathless. ABG showed hypoxic respiratory with a po2 of 6.9. Initial thought was whether she developed a pulmonary embolism considering recent surgery and immobility despite remaining on prophylactic anticoagulation while stay in the hospital. Immediate CT-pulmonary angiogram was performed which revealed PICC line punctured left subclavian vein with an extra luminal extension of about 2 cm in the mediastinum leading into bilateral pleural effusion and pneumomediastinum with collapse of both lower lobes (). Considering the complexity of the CT findings, it was referred to the medical on call team overnight. After stopping the TPN infusion, medical registrar on call discussed the case with the cardio-thoracic surgeon who recommended the immediate transfer of the patient for a possible sternotomy and removal of the PICC line with subclavian vein repair to be performed by the vascular surgeons. Prior to transfer, she would require the insertion of bilateral chest drains to relief her symptoms and also to aid in for any possible haemothorax. Thereafter bilateral 18F chest drain tube were inserted by the medical registrar overnight by seldinger method. A 1 L white, milky fluid was drained from the left-hand side and other similar consistency 1 L red tinged fluid was drained from right-hand side (). Both drains were clamped to avoid re-expansion pulmonary oedema considering the volume and rapid drainage of fluid. Pleural fluid sample was sent to the laboratory and post chest drain chest X-ray was performed which showed resolving bilateral pleural effusion (). She was immediately transferred to the cardiothoracic centre where she underwent a sternotomy and removal of the PICC line with primary repair of the left subclavian vein. She was later treated with antibiotics, daily pleural drainage and parenteral nutrition through a PICC line inserted in her right internal jugular vein, which was later removed without any further complications once she was able to tolerate enteral feeding. The two pleural fluid sample was consistent with TPN contamination: visibly lipaemia (left more than right as right pleural fluid was red tinged colour) with triglycerides of 4.40 mMol/L and 4.70 mMol/L. |
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