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A 52-year-old male had presented to the emergency department with pain and swelling around the right shoulder region following a fall from stairs. The clinicoradiological evaluation suggested a two-part proximal humerus fracture. The patient had no other injuries, and systemic examination was normal. The patient was a known epileptic and was treated with regular anti-seizure medications. The fracture was managed operatively with an open reduction and internal fixation using a titanium proximal humerus locking plate (Figures , ). The standard deltopectoral approach was used for fracture exposure. The bone was osteoporotic and the screw purchase is an obvious issue in such fractures. We used specially designed locking head cancellous screws for the humeral head fragment fixation. These screws combine the advantage of cancellous screws, which offer better bone purchase, and the locking head helps in angular stable construct for the patients with such osteoporotic bone. The perioperative period was uneventful. Osteoporotic management in form of calcium and vitamin D supplementation was started right from the admission and was maintained throughout the follow-up. Postoperatively, the patient was kept on a guarded mobilization program because of the poor bone quality intraoperatively. The patient was discharged on the third postoperative day and was called for suture removal after two weeks of surgery. The patient underwent suture removal, and the postoperative wound healed completely at the end of the second week. After the third postoperative week, the patient sustained a seizure episode. Non-compliance with antiepileptic medications was the probable cause for the seizure episode. Following the violent contractions of the right arm at the time of seizure, the patient had redeveloped a painful swelling around the right shoulder. Clinicoradiological evaluation revealed a catastrophic failure of the fracture fixation. The proximal screws had migrated to the deltoid insertion region, and the fracture collapsed in varus (Figures , ). The violent abduction of the shoulder probably surpassed the fixation strength leading to such a failure.\nUnder antiepileptics cover, the patient was re-operated, and the same deltopectoral interval was used. The plate and migrated screws were removed. The bone was osteoporotic, and a major segment of the fracture margins was already damaged. We docked the distal fragment (shaft fragment) into the humeral head, and a temporary reduction was achieved. A simple pin-based external fixator was then used to span the fracture while additional K wires held the humeral head to the humeral shaft (Figures , ). The reduction was secured under direct vision. The wound was then closed in layers. Within two weeks, the wound healed satisfactorily. The fracture union was achieved in two months. The shoulder’s complete range of motion was restored within three months of the second surgery, and the patient did not face any limitations in daily activities. Strict compliance to the antiepileptic medications was ensured throughout the follow-up. |
A 72-year-old woman was referred to our department by a neurologist with a history of diabetes mellitus and hypertension. The patient had been suffering from headache for the past 8 months. The headache was throbbing and deep seated in nature. The physical examination and laboratory work-up were unremarkable with the exception of a slight increase in glycosylated hemoglobin indicating poor regulation of the blood glucose levels. The patient had used conventional analgesics and various other drugs for the past 3 weeks with no effect. There were no sinonasal symptoms like postnasal dripping, nasal discharge, or obstruction.\nNasal endoscopy revealed a small polypoid mass protruding from the ostium of the sphenoid sinus. Computed tomography (CT) scan of the paranasal sinuses showed opacification of the left sphenoid sinus containing calcifications and mucosal thickening on the right side (). Coronal images displayed in soft tissue window settings reveal a suspicious low attenuating mass lesion located in the sellar region (). Magnetic resonance imaging (MRI) was performed to rule out possible intracranial extension of sphenoid sinusitis and to evaluate coexisting masses in the sellar area. The left half of the sphenoid sinus was of low to intermediate signal intensity on T1-weighted (T1W) images and appeared with signal void on T2-weighted (T2W) images consistent with fungal sinusitis ( and ). The fungal disease appeared to be confined to the sphenoid sinus without any sign of intracranial involvement. A dynamic MRI study was undertaken to evaluate the pituitary gland and the mass lesion located in the right half of the cella. The mass lesion incidentally detected on the CT scan showed homogenous contrast uptake on late phase dynamic MR images and a dural tail was apparent on sagittal images suggesting the diagnosis of a meningioma. However, the mass lesion could not be differentiated from the pituitary gland. It was seated in the right half of sella turcica and extended to the suprasellar region where it pushed the right half of the optic chiasma superolaterally. Differential diagnosis included macroadenoma of the pituitary gland ( and ). It is worth noting that despite the indentation of the chiasma, the patient’s sight was not affected and headache was the only symptom.\nEndoscopic sphenoidotomy using transnasal approach was performed and a fungating mass was removed. The bony walls of the sinus were intact. Biopsies were taken from the sinus mucosa to rule out possible fungal invasion. Histopathologic evaluation demonstrated spore-forming structures confirming the diagnosis of fungal disease. Pathologic report stated healthy tissue margins confirming total excision of the fungal mass.\nThe patient was discharged the same day. On follow-up examination after 2 months rhinoscopy was normal and CT scan confirmed a sphenoid sinus free of disease. The patient’s headache still persisted but with decreased intensity. The incidentally detected mass lesion is under follow-up and is stable to date. Due to the patient’s age and medical status surgical excision was not feasible. It is not possible to decide which lesion was the actual cause of the headache in the first place. It appears that the mass lesion at least had contributed to the headache as the patient is still experiencing headache. Ironically, thanks to her headache the patient’s fungal infection was diagnosed and treated early enough to prevent any serious complications. |
A 70-year-old female with a past medical history significant for diabetes mellitus, peripheral neuropathy, Charcot neuroarthropathy, hypertension, hypercholersteremia, and renal insufficiency initially presented with an unstable Charcot joint about the rearfoot and ankle with two draining sinus tracts from the medial aspect of the ankle joint. The patient reported eight previous surgical attempts to the affected extremity. Two procedures approximately 8 months apart over 2 years ago were performed to achieve an ankle arthrodesis with external fixation that ultimately resulted in an infected nonunion and Charcot neuroarthropathy of the midfoot and rearfoot (A and B). A total of six procedures were then performed for infection that consisted of incision and drainage procedures with collection of bone and soft tissue cultures to guide systemic antibiotic therapy with no further attempts at reconstruction of the deformity. Multiple organisms were present from numerous cultures over the last 3 years. Recommendation was for below knee amputation prior to referral. The patient was initially treated with surgical excision and debridement of all non-viable and infected soft tissue and bone that resulted in a talectomy, distal tibia resection, and wide resection of the tarsal bones. Deep intra-operative soft tissue and bone cultures were obtained to determine the pathogenic organism(s) to guide further antibiotic therapy. A hybrid external fixation device was initially applied to provide osseous stability and to off-load the soft tissues. The patient returned to the operating room 7 days from the initial debridement for a second osseous debridement, thorough lavage of the wound, and placement of antibiotic beads with wound closure to treat the dead space and deep infection (A and B). The wounds healed at 4 weeks and revisional surgery was performed 6 weeks after the second surgical debridement. During this period, parenteral antibiotic therapy was maintained and the patient was kept non-weight bearing in the hybrid external fixator. Revisional surgery consisted of a tibio-calcaneal fusion with autogenous bone grafting harvested from the proximal tibia. Osseous stabilization for the tibio-calcaneal fusion was achieved with a multiplane circular external fixator (A and B). This was advantageous given the history of infection while providing the necessary stabilization required given the bone loss present from the previous debridement. The patient was maintained in the external fixation device for 110 days and was then immobilized in a non-weight bearing cast for 6 weeks, a weight bearing cast for another 4 weeks, then transitioned to a custom shoe with a double upright brace that she wears currently at 30 months post-operatively (A and B). |
A 32-year-old female recently diagnosed with gestational diabetes after an abnormal glucose challenge test (blood glucose level of 206 mg/dL at 1 h) presented to the sports medicine clinic at 30 weeks pregnant with lateral hip pain. She described the pain as a “burning that started in the lateral aspect of the left hip and radiated down the lateral aspect of the thigh.” The patient also reported lateral hip pain which was worse when rising from a seated position, climbing stairs, and lying on her left side. The pain was rated as a 2/10 in severity and was similar to the pain she had prior to pregnancy that was treated successfully with NSAIDs and a corticosteroid injection into the trochanteric bursa. Physical exam was notable for the absence of skin changes and absence of tenderness to palpation in the left groin but positive for tenderness to palpation in the lateral aspect of the thigh as well as directly over the greater trochanter. Range of motion testing was limited by her gravid habitus, but there was normal external and internal rotation of the left hip. Complete patient history and physical exam were indicative of meralgia paresthetica as well as trochanteric bursitis.\nAlthough her previous episodes had been treated successfully with NSAIDs and corticosteroid injections, NSAIDS are contraindicated in the third trimester of pregnancy and her gestational diabetes placed her at an increased risk of hyperglycemia from corticosteroid injections. Conservative measures such as physical therapy, education about weight loss, and instructions to wear loose clothing were recommended; however, the patient returned around 2 weeks later with no improvement in her symptoms. The patient’s daily blood glucose log was reviewed, and it showed an average fasting blood glucose level of 92 mg/dL and an average postprandial blood glucose level of 119 mg/dL. She was controlling her blood glucose by diet and exercise alone. Clearance was obtained by her OB/GYN to receive the corticosteroid injection, and subsequently, the patient received an ultrasound-guided injection of a sterile mixture containing 4 cc 0.5% marcaine and 40 mg methylprednisolone acetate into the left greater trochanteric bursa (Fig. ). Blood glucose levels were closely monitored for the next 48 h, and all readings were within normal limits. At a 1-month follow-up, the patient reported that the pain was significantly improved, now rated as 0/10, and therefore, she had returned to her normal exercise routine. Approximately 6 weeks after the steroid injection, the patient vaginally delivered a 3000-g male at 38 weeks and 1-day gestation. There were no complications, and the baby tolerated delivery well, recording APGAR scores of 7 at 1 min and 9 at 5 min. |
A 51-year-old Sri Lankan woman presented with epigastric pain for two months. Initial examination showed that she had tenderness in the epigastrium. There was associated loss of appetite as well. Her past medical, surgical and familial histories were unremarkable. Routine laboratory data on admission did not show any abnormal findings. Upper gastrointestinal endoscopy was normal up to 2nd part of the duodenum and revealed a normal mucosa of the stomach (Figure ). Abdominal CT scan showed a mass lesion (12×8×11 cm) of the left lobe of the liver with enhancement during arterial phase and washout during venous phase. The impression of the radiologist was a hepatocellular carcinoma, most probably of fibrolamellar type due to star sign within the lesion (Figure ). The operative plan was laparoscopy and left hemi hepatectomy. Laparoscopic exploration was performed for under general anaesthesia. On the laparoscopic examination, the appearance of the liver was completely normal. There was a large solid mass arising from the lesser curvature of the stomach. There were no regional lymph node enlargement. There were no peritoneal deposits and also pelvic organs were appeared normal on laparoscopy. Then patient underwent open surgery which involved ligation of the left gastric artery and separation of the tumour from the lesser curvature (Figure ) with a small cuff of the stomach. The weight of the surgical specimen was 0.92 kg. Later histology of the surgical specimen (Figure ) was suggestive of a GIST). The patient had an uneventful postoperative course and was discharged after seven days on orals. The histopathological examination of the surgical specimen (Figure ) revealed a tumour which composed of interlacing fasicles of spindle cells with wavy nuclei which are fairly monomorphic. There was no evidence of tumour necrosis. The mitotic activity of the tumour was 2 per 50 high power fields. It showed diffuse CD117 positivity in membrane and cytoplasm. The overlying gastric mucosa was histologically unremarkable and resection margins were free of tumour. The overall features were suggestive of GIST. She was not given postoperative chemotherapy. The patient was asymptomatic for past 2 years and currently been followed up as an outpatient in the clinic.\nGastrointestinal stromal tumours are believed to be originated from the interstitial cells of Cajal, a pace-maker cell that controls gastrointestinal peristalsis. The clinical manifestations of GISTs depend on the location and size of the tumours. Patients may present with pain, dysphagia, weight loss, gastrointestinal bleeding, bowel obstruction, or a palpable abdominal mass []. Surgical resection is the most effective treatment option for GIST. The 5 year survival rate after surgery amounts to 28–65% [-]. Tumour resection is the treatment of choice for GISTs. According to Lupescu et al. CT abdomen is sufficient to diagnose the location, extension, size, contours, structure of the tumours, hepatic metastasis of GIST []. Thus, it is important to be able to diagnose GISTs from pre-operative CT. According to Rosch et al. the sensitivity and specificity of endoscopy are 87% and 29%, respectively for distinguishing intramural lesion from extramural compression []. According to the best of authors knowledge there were no published case reports describes GIST of stomach mimicked hepatic tumour. But there are reports on extra gastro intestinal GIST involving lesser omentum [-]. Baskiran et al.[] described a large pedunculated GIST of posterior gastric wall mimicked a pancreatic tumour. Our patient’s upper gastro intestinal endoscopy showed a normal gastric mucosa. Therefore we didn’t plan for a upper GI endosonography for this patient. Here, our preoperative diagnosis was not GIST of stomach. Radiologically the wall of the stomach was not thickened and there was no intramural tumour in the gastric wall. Park et al. has described a left hepatic cyst may rarely mimic a submucosal tumour arising from the gastric cardia and fundus []. Our patient had a GIST of a stomach mimicking left hepatic tumour. In the best of our knowledge, this is the first report of a patient with gastric extra mucosal GIST that was misdiagnosed as a hepatocellular carcinoma of left lobe of liver and diagnosed by laparoscopy and treated by surgery. |
A 49-year-old man visited an ophthalmology clinic complaining of blurred vision in his left eye since 2 month ago. Optic disc swelling was observed in both eyes, and he was referred to our hospital. At the initial presentation, best corrected visual acuity (BCVA) was 25/20 in the right eye and 20/20 in the left eye. Central flicker was 37 Hz in the right eye and 36 Hz in the left eye. Intraocular pressure was 15 mmHg in both eyes. Both eyes had normal light reflexes and negative relative afferent pupillary defect. There was no abnormality in the anterior segment; however, papillary swelling in both eyes and optic disc hemorrhage in the left eye were observed on the fundus examination (). Fluorescein angiography (FA) revealed dye leakage from the papillae of both eyes ( and ) and multiple nodular staining along the veins (). Laboratory examination and chest X-ray were normal, and the tuberculin reaction was weakly positive. Brain CT showed no cerebral ventricular enlargement (), but dilation of the bilateral nerve (), 8 mm and 10 mm nodules on the left frontal skull (), and subcutaneous nodules in the right parietal region () were observed.\nAlthough systemic characteristics of sarcoidosis were not detected, ocular sarcoidosis was the most considered finding from the FA findings. Oral corticosteroids (prednisolone 40 mg/day) were initiated and tapered, but there was no improvement in papillary swelling. The frequency of dizziness further increased 2 months after the first visit. He visited neurosurgery 3 months after the first visit, and it was indicated that he had an abduction disorder in the left eye, right-sided central facial paralysis, mild weakness of the left little finger abductor, and increased tendon reflex in both legs.\nIn brain/myelography MRI, enhancement of contrast effect around both optic nerves, high signal of fatty tissue around the optic nerve, high signal of cerebral sulcus of the frontal parietal lobe, enlargement of the lateral ventricular angle, and high signal of cervical meninges were recognized (). Cerebrospinal fluid examination revealed markedly increased cerebrospinal fluid pressure (>30 cmH2O [not measurable]), cell counts 5/μL, cerebrospinal fluid protein 438 mg/dL, and cerebrospinal fluid sugar 45 mg/dL. Cytodiagnosis suggested highly atypical metastatic adenocarcinoma cells (Class IIIb) containing mucus-like substances in the cytoplasm (), and biopsy found that poorly differentiated adenocarcinoma with signet-ring cells originating in the gastroduodenum had metastasized to the brain, resulting in meningeal carcinoma, which caused bilateral optic disc invasion. Thereafter, a ventricular abdominal shunt (VP shunt) was performed, and the abduction disorder in the left eye and facial nerve palsy tended to improve. Whole brain irradiation started at another hospital 4 months after the first visit, and chemotherapy (TS-1+cisplatin: SP therapy) started 5 months after the first visit. BCVA at the final visit to our hospital (after 10 months after the first visit) after completing five courses of chemotherapy was 4/200 in both eyes, and central flicker could not be measured in either eye, although bilateral optic disc swelling had improved (). Later, his general condition gradually worsened, and he died 16 months after the first visit. |
We present a case of a 72-year-old male patient who presented for an elective cardiac catheterization for further evaluation of stable angina. His medical history was significant for diabetes mellitus and coronary artery disease (CAD) with a drug-eluting stent (DES) placed 16 years ago. He underwent cardiac catheterization through right radial artery approach which revealed multivessel CAD, no intervention was done and he was referred to cardiothoracic surgery team for evaluation of coronary artery bypass graft (CABG).\nTwo hours after the procedure, the patient suddenly started complaining of persistent visual disturbance and described it as seeing everything double; he had never experienced similar complaints in the past. He denied any headache, dizziness, speech difficulty or motor or sensory weakness in his extremities. The patient was hemodynamically stable and found to be in sinus rhythm. Ophthalmologic exam revealed mild ptosis of the left eyelid, sluggish pupillary reflex in the left eye and exotropia of the left eye on primary gaze along with impaired adduction of the left eye (Figures , ).\nThere were no abnormal findings in the right eye. The patient also complained of diplopia with downward and outward gaze, and these symptoms resolved transiently by covering his left eye. Rest of the exam for other cranial nerves was normal, and there were no focal motor or sensory deficits appreciated. Computed tomography angiography (CTA) of head and neck did not show any evidence of intracranial hemorrhage or territorial infarct, and revealed a small stable arteriovenous malformation (AVM) in right pons. MRI of the brain did not show any abnormal enhancement and third cranial nerve appeared normal bilaterally, and again demonstrated small cavernous malformation in right pons (Figure ) similar to CT scan findings. He was already on atorvastatin and aspirin for his CAD which was continued.\nThe patient continued to have persistent diplopia during his hospital stay, underwent CABG and was discharged 10 days later in stable condition. The patient followed up in ophthalmology clinic in four weeks; at this time, he complained of same visual complaints and there was no change in exam findings compared to his discharge from the hospital described above. The patient again followed up in clinic eight weeks later and his symptom of diplopia had now completely resolved. Ophthalmologic exam revealed his left eye ptosis had resolved, left pupil was round and reactive to light, and adduction of the left eye was normal. |
In July 2007, a 64-year-old woman sought evaluation at our hospital for a palpable lesion in the left breast which had been detected several days earlier. The medical history was significant for diabetes mellitus and hypertension. A 0.8-cm irregular, hypoechoic lesion near the left areola was demonstrated on sonography (). A biopsy was recommended due to the suspicious shape; however, the patient declined any procedures which would permit pathologic confirmation.\nIn December 2010, the patient visited our hospital with symptoms of exertional dyspnea of 1 month duration. Because of the underlying diabetes mellitus and hypertension, the imaging work-up focused on the pulmonary symptoms. A chest computed tomography (CT) showed multiple nodular septa, fissures, and pleural thickening of both lungs with a large bilateral pleural effusion and multiple enlarged lymph nodes in the paratracheal and axillary areas, suggestive of lymphangitic metastasis. There were no pulmonary nodules, mediastinal masses, or bronchial abnormalities. In addition to the pulmonary findings, a large irregular mass was incidentally detected in the left breast with overlying skin thickening and nipple retraction. The breast mass was evaluated by mammography and sonography. On mammography, an ill-defined hyperdense mass occupied the left subareolar area which was adherent to the areola (). Nipple retraction, diffuse skin thickening, and multiple enlarged lymph nodes in the left axilla were noted with shrinkage of the volume of the left breast. An irregular hypoechoic mass with invasion to the nipple was demonstrated on sonography (). Abdominal and pelvic CT and positron emission tomography-CT were obtained to check the other organs of the body, and showed no evidence of abdominal or pelvic lesions. The final diagnosis of the imaging studies was a primary breast tumor with pulmonary metastasis. We took a biopsy of the breast mass to confirm the pathology. Microscopic finding showed infiltrating nests of small cells in the fibrotic stroma, the tumor cells of which had small hyperchromatic nuclei and scanty cytoplasms (). Immunohistochemical stain showed strong positivity of the tumor cells for neuron-specific enolase, suggestive of small cell neuroendocrine carcinoma (). |
The patient was a 38-year-old male who became aware of weakness in the right side of the body for several months without a specific trigger. He also developed motor skills disorder in his right hand that made it difficult for him to drive a car. Weakness in his left lower extremity also gradually developed and he then visited the Department of Neurology at our hospital. At this visit, intracranial process was denied, but hyperesthesia of the right upper and lower extremities was confirmed. Since no other abnormal findings were observed, he was referred to our department for suspected cervical spine pathology.\nThe patient had a history of hospital visits for several weeks for cervical pain after he hit the top of his head severely while diving into a swimming pool at the age of 14 years old. At that time, cervical spine plain X-ray images showed no fracture and he was diagnosed with whiplash injury. After the use of a collar for several weeks, the cervical pain disappeared.\nAt his first visit to our department, his right and left grip strengths were 47 kg and 42 kg, respectively, and no objective muscular depression was apparent in the extremities. There was also no increased deep tendon reflex or pathological reflex. Cervical spine plain X-ray images showed no fracture but indicated an unstable atlantoaxial joint. The atlantodental interval (ADI) was increased to 8 mm during flexion and reduced to ≤3 mm in extension (). Computed tomography (CT) showed no bone chips at the attachment site of the transverse ligament of the atlas (). Magnetic resonance imaging (MRI) showed a high intensity area, which suggested fluid accumulation caused by instability between the anterior arch of the atlas and odontoid process of the axis ().\nBased on this information, we diagnosed delayed myelopathy due to atlantoaxial subluxation caused by the injury of the transverse ligament of the atlas. Transarticular screw fixation surgery was performed under X-ray fluoroscopic guidance with the atlantoaxial joint in a hat-hook position. After the decortication of the posterior arch of the atlas and lamina of the vertebral arch axis, autologous iliac bone grafting was performed.\nThe postoperative clinical course was uneventful. 4 days after surgery, walking exercises were initiated using a Philadelphia collar. Weakness of the extremities and motor skills disorder gradually improved. The patient was able to go back to work 3 months after surgery and showed no symptoms 6 months after surgery. Cervical spine plain X-ray images obtained 6 months after surgery showed union of grafted bone and no unstable cervical vertebra during flexion and extension (). |
A 48-years-old male presented with history of road traffic accident. He had sustained head injury, fracture of right femur and Gustillo type IIIc fracture of both bones right leg. He had anterior tibial artery injury and extensive crushing of anterior compartment muscles along with a soft tissue defect spanning across upper and middle thirds of leg and extending into the upper part of lower third of leg [Figure –]. Associated head injury and patient's hemodynamic instability did not allow early surgical intervention. As the management of wound was difficult due to multiple unstable fractures, after 48 hours of injury he was taken up for a short procedure of fracture fixation. His fractures were stabilised with external fixator, spanning across the knee. Soft tissue cover was not possible at this stage because of his general condition.\nAfter getting clearance from neurosurgery and the intensive care specialists, he was taken up for elective micro-vascular free tissue transfer on the fourth post injury day. On exploration of the wound, posterior tibial artery was found intact but both accompanying deep veins of both anterior and posterior tibial vessels were found thrombosed. The short saphenous vein was injured; the great saphenous vein (GSV) was patent and uninjured. Gentle irrigation with heparin saline into the vein (GSV) was possible without much resistance and the intima appeared healthy under microscope, hence it was selected as recipient vein for anastomosis.\nAfter confirming the recipient vessels, Vastus lateralis myo-cuteneous flap of dimensions 30 × 15 cm was harvested from the left thigh and was transferred to recipient site for coverage of the fracture site. After partial insetting, anastomosis was performed. The artery was anastomosed to the posterior tibial artery in an end to side fashion with 9/0 nylon. One of the flap veins was anastomosed to the great sephanous vein in an end to end manner. Due to non-availability of recipient veins in the vicinity, the second flap vein was clipped at the end of the procedure after confirming good flow. In setting was completed and good bleeding was observed from the flap at the end of the surgery. The patient was shifted to postoperative ward.\nAn hour after the surgery, the flap became congested. Clinically, it was evident by brisk and congested bleeding on scratching the flap with needle. The flap was explored immediately suspecting some compromise in the venous flow. On exploration, after exposing the anastomotic site, the vein anastomosis was found patent. Both the anastomosed flap vein and the GSV were distended and tense. The ligated second vein was also found to be tense and distended []. The clip at the end of the second vein was released to let out some blood, in order to decongest the flap. This manoeuvre improved the flap colour and bleeding after some time. The flap drainage through a single vein was probably inadequate, so nearby areas were re-explored for another recipient vein. We could not find a suitable vessel for the second anastomosis.\nAs there was no possibility of getting a second vein for anastomosis, it was decided to let the blood out periodically to decongest the flap. The blood flow through the second vein was checked and was found to be very high (22 ml/min); letting out so much blood in a patient with multisystem injury was not a suitable option. Collecting the blood and then transfusing it was another option but had risk of contamination and sepsis. After carefully considering all the options and weighing the risks, it was decided to drain the blood through an IV catheter and auto-transfuse it to the patient simultaneously. The idea was to create a closed system with lesser risk of contamination. One IV cannula was inserted over patients forearm and the other one in the second vein of the flap through the edge of the flap. In the leg the flap vein was ligated over the cannula under appropriate pressure with 7/0 polypropylene, also the position of cannula was secured over the skin so as to prevent it from accidently getting dislodged. The cannulas were connected with an extension tubing (100 cm) as shown in . A three way system was attached at the proximal end of the extension tube through which heparin (5000U in 500 ml of normal saline) infusion was started at a rate of 20 micro-drops per minute to keep the extension tube patent [].\nThe drainage of the flap vein through the external shunt was checked every 2 hours as shown in the accompanying video []. The patient was also quite comfortable with this position and was even able to take his hand to the mouth. Considering the fact that cannulating the flap vein and transfusion of blood from the site of injury directly into systemic circulation might lead to sepsis, stringent wound hygiene was maintained. As a precautionary measure the venous cannula draining the flap vein was changed once, after 3 days of drainage. By seventh day the flap developed enough collateral venous drainage and there was no congestion on blocking the venous cannula. At this time the drainage was stopped and the second vein was ligated.\nThe flap survived, except a small area of necrosis at the distal edge []. The final insetting was completed after 2 weeks. The distal necrotic portion was debrided and the residual raw area was managed with VAC and was covered with skin graft at a later date. Complete wound healing was achieved in 4 weeks []. |
A 37 year old male and current smoker (1 pack per day) with a history of prostate rhabdomyosarcoma at age 2 treated with partial resection and high dose external beam radiation presented to the emergency department (ED) with fever, groin pain and abdominal distension. His history was notable for numerous complications including bilateral osteonecrosis of the hip, lifelong complete urinary incontinence, a dense rectal stricture causing chronic colonic dilation and critical lower extremity ischemia due to bilateral iliofemoral disease. He had undergone 45 procedures to address these complications. He was initially managed conservatively for intestinal obstruction secondary to a rectal stricture. Rectal dilation was performed. Subsequently, he developed MSSA (methicillin sensitive Staphylococcus aureus) bacteremia and an abscess was noted in his left thigh (adductor region). The abscess was drained under ultrasound guidance by the interventional radiology team, and the drain was removed after a week. A month later, he presented to the ED with fluid draining from the former site of the IR drain. The fluid was found to be urine, with creatinine of 30.9 mg/dL. A Foley catheter was placed, and retrograde urethrogram (RUG) showed a urethrocutaneous fistula from the bulbar urethra to the left thigh (). Cystoscopy and subsequent biopsy of the fistula revealed no malignancy. Given the need of serial rectal dilations for his chronically dilated large bowel, the patient was formally recommended a permanent colostomy, but he declined. He was discharged with catheter in place and the fistula site dry.\nAs definitive treatment for the fistula, an ileal conduit urinary diversion with partial cystectomy was done. The patient was discharged postoperatively on day 12, with fistula dry and scant discharge per urethra. Two months after this surgery, the patient developed a cold lower limb for which he underwent a successful axillary-femoral artery graft thrombectomy. The next month, he also underwent a major hip joint reconstruction. 8 weeks later, he developed fever and chills followed by passage of feces per urethra. The next week, he developed fecal drainage from the left thigh as well. A RUG was performed and revealed a rectourethral fistula (). To treat this rectourethral fistula an end colostomy with distal mucus fistula was performed. Drainage from the urethra and thigh fistula persisted despite the surgery. Proctoscopy showed a tight rectal stricture at 8 cm. Colonic irrigation through a red rubber tube, passed through the distal mucus limb of his colostomy was done with the aim of clearing out the stool in his distal colon from his thigh fistula.\nThe cutaneous fistula remained dry for several weeks. Leakage per rectum remained minimal. He then reported passage of sutures per urethra and discharge of straw-colored peritoneal fluid from both the urethra and thigh fistulae. As a temporary measure, he underwent intraoperative cystoscopic identification of the fistula site and urethral bulking therapy using a dextranomer and hyaluronic acid (Deflux®, Salix Pharmaceuticals, Raleigh) applied to the fistula site. Debridement of the left groin fistula tract, and negative pressure wound therapy placement to control drainage from the peritoneo-urethral fistula was also done. Postoperatively, the drainage from the thigh and urethra persisted, so he underwent takedown of the previous descending colostomy and mucus fistula, descending colectomy and proctectomy, creation of a new permanent colostomy and simple cystectomy. As drainage still persisted after this, his urethra was closed perineally proximal to the fistula, and the defect was reinforced with a pedicled rectus femoris muscular flap. His incisions have healed well, and he no longer has drainage 5 months after surgery. |
We present a case of a 65-year-old female patient admitted to the Cardiac Surgery Department in Wroclaw in January 2018 with severe mitral regurgitation (MR) and the history of ischemic heart disease, after elective percutaneous coronary intervention of the circumflex branch of left coronary artery with two drug-eluting stents (DES) implantation 4 years earlier. Furthermore, the patient diagnosed with many chronic conditions, such as metabolic syndrome, obesity with BMI 33 and gastroesophageal reflux disease. Currently, with an exercise dyspnoea for about 2 years, intensifying in recent weeks, she was hospitalized in the Cardiology Department for further diagnostics. The transthoracic echocardiography (TTE) revealed non dilated left ventricle with a normal systolic ejection fraction of 60%, and no evidence of segmental wall motion abnormalities, severe MR with the prolapse of the A2 segment and systolic restriction of the posterior leaflet. Colour Doppler showed a highly distinctive eccentric turbulent jet directed towards the lateral wall and the base of the left atrium with ERO 0.6cm2 and regurgitant volume of 60 ml. Additionally, in the performed coronary angiography, hemodynamically significant narrowing was found in the area of the previously implanted DES. The patient was then consulted by the cardiac surgeon and qualified for surgery. After admission to the Cardiac Surgery Department, as part of the pre-operative preparation, TTE was again performed, in which the severe MR was confirmed and no pathological structures in the right atrium were described. Due to the unclear image in the right pulmonary field, described by the radiologist in the chest X-ray (Fig. ), diagnostics was extended by performing a computed tomography of the chest, which excluded the presence of pathological shadow in the lung parenchyma. There was no referral to the atrial septum in the CT report. The patient was scheduled for mitral valve repair surgery and coronary artery bypass grafting (CABG) with the use of saphenous vein graft to the circumflex artery. During the standard procedure of commencing the cardiopulmonary bypass (CPB) and bicaval cannulation, it was found difficult to insert the cannulas from the atrium into both vena cavas. Therefore the cannulation was performed using the smaller cannula sizes, which eventually allowed to go on bypass. On the free wall of the atrial septum, there was a thickening and an excess of adipose tissue with a firm consistency and the size of a walnut, significantly impeding access to the operated mitral valve through the left atrium, and probably completely preventing surgery by the transseptal approach. In the transesophageal echocardiography (TEE), a characteristic image of LHAS was confirmed by the presence of hypertrophy of the septum, up to 2.7 cm, an hourglass shape with a characteristic indentation at the place of the fossa ovalis (Figs. and ). Based on the intra-operative TEE, as well as by analyzing the chest X-ray and CT scans, the diagnosis of LHAS was made. Due to the asymptomatic course of the LHAS and the complexity of the scheduled operation, the decision was made to leave the change intact. The mitral valve was replaced through the left atrial approach. The surgery was completed in a standard manner and the weaning from the CBP went uneventfully. The patient’s early postoperative period was a routine. |
A well built 76-year-old otherwise healthy male who was a known case of node negative squamous cell carcinoma of the left buccal mucosa, involving left retro molar trigone region, anterior tonsillar pillar and soft palate, and was treated with 60 Gy of external beam radiation using photons over 30 fractions delivered using cobalt 60 source in 1998, presented six years later in 2004 with the gradually progressive swelling and pain in left malar region for last 3 months. He developed numbness of left cheek and blurring of vision in left eye along with epiphora of left eye and nasal obstruction of left nare. There were episodes of epistaxis. He was a chronic chewer and smoker for more than 30 years but quit the habit after diagnosis of squamous cell carcinoma in 1998. Examination showed a bony swelling in left malar region measuring 2 × 3 cm, with diffused margins and blunting of infra orbital rim. Parasthesia of cheek left side, epiphora, blurring of vision in left eye, and obstruction of left nare were also present. Overlying skin appeared normal. Intra orally a total edentulous mouth and tender bony swelling in left maxillary alveolar region involving both buccal and palatal aspect was noted. Swelling was more pronounced over the buccal aspect and extended along whole of the anterior wall of maxilla. Computed tomographic scan demonstrated radiodense mass in left maxillary sinus with expansion of the anterolateral and posterolateral wall (figure ). Tumor was seen to be infiltrating the orbital fat. At surgery the tumor was seen infiltrating ethmoid sinus, pterygoid muscles, lateral wall of nose and superomedial part of orbit. A local wide excision with left radical maxillectomy and orbital exenteration was carried out. The defect was grafted.\nMicroscopic examination of the resected specimen demonstrated, a neoplasm composed of sheets of spindle cells having eosinophilic cytoplasm and hyper chromatic nuclei (figure ). Laying of osteoid by the tumor cells was seen. Scattered mitotic figures were noted. The picture was consistent with osteogenic sarcoma of maxilla T3N0M0G1 AJCC stage III. The patient is disease free and on regular follow-up 6 months after the surgery. |
Patient n. 2 was a 60-year old female, diagnosed with suffering from POAG for ten years, under treatment with hypotensive drugs and regularly controlled IOP twice a year which appeared maintained within normal ranges. During a control visit in January 2016, the patient had reported irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand, mainly in her right eye (RE), over the last six months. The situation had been managed with the use of several types of tear substitutes, none of them successful in symptom relief. Symptoms were scored in the RE as OSDI of 74 out of 100 and a VAS score of pain of 85 mm out of 100. In the left eye (LE) the symptoms were defined by the patients as light and acceptable: OSDI score was 22 out of 100 and VAS 21 mm out of 100. Slit lamp evaluation had not shown epithelial damage, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 4 seconds in RE and 8 seconds in LE had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.\nIn March 2016, the patient was proposed to receive in her RE a treatment with topical CBS, as a compassionate unconventional therapy, with the aim to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful. The therapy for the contralateral LE was maintained with HA based tear substitutes.\nThe patient signed the specifically designed informed consent and started administration of the CBS eye drops in April 2016, with the posology of 0.4 ml (8 drops) in RE, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month.\nOn September 2016, the patient reported a significant relief from subjective symptoms of discomfort; in RE the OSDI was 28 out of 100, with VAS: 32 mm out of 100. Also in LE a reduction was observed, with OSDI score determined as 16 out of 100 and VAS 15 mm out of 100. During this visit, also the IOP was measured, which resulted in the normal range in both eyes and the analyses of the visual field tests performed since 2006.\nIn the mean deviation (MD) values recorded over several visits performed from December 2006 through September 2016 were graphed. A significant progressive lowering in MD values was observed which was followed by an important amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. It has to be noted that the increase in MD values was recorded either in the treated right eye or in the untreated left eye. Moreover, the MD values recorded in September 2016, four months after the end of the CBS eye drop administration, showed in both eyes a further amelioration.\nThe improvement is also demonstrated by the analysis of the PSD shown in . A progressive worsening in PSD values had been observed over ten years, whereas a rapid change was recorded in correspondence with the CBS eye drop treatment, either in the treated right eye or in the untreated left eye.\nIn the central 30-2 visual field tests before ((a): right eye RE; (b): left eye LE), at the end (c, d) and after four months (e, f) from the end of the CBS eye drop treatment were shown. An amelioration of the defect was observed in all the four quadrants in both the treated RE and the untreated LE.\nTaking together these observations, a positive effect also in the left untreated eye could be recorded, which suggests a neural cross-talk mechanism between the eyes. |
Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.\nThree months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().\nSurgical treatment called for the induced membrane technique.\nThe first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.\nThe second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().\nAntibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.\nThe second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
A 62 year-old male with an extensive head and neck cancer history and recent lower esophageal cancer presented for treatment of T3N0M0 squamous cell carcinoma of the left lateral tongue. He had a history of right tongue cancer surgically treated 20 years ago via right partial glossectomy, right modified radical neck dissection, and reconstructed with a left radial forearm free flap followed by adjuvant radiation therapy. He subsequently developed osteoradionecrosis of the mandible requiring a right fibula free flap. More recently, he was diagnosed with esophageal cancer treated with esophagectomy and gastric pull-up followed by chemoradiation. Patient had a left subclavian port placed for administration of chemotherapy, which remained until he presented for treatment of the new squamous cell carcinoma of the left oral tongue five months later.\nThe patient's case was presented at our multidisciplinary head and neck oncology conference, and recommendation was made to perform left partial glossectomy, left neck dissection, tracheostomy, and right ulnar forearm free flap. Intraoperatively, radiation-induced fibrosis was apparent during the left neck dissection but adequate vessels for microvascular surgery were identified. A right ulnar fasciocutaneous free flap was harvested, and microvascular anastomosis was performed to the left facial artery and left external jugular vein in end-to-end fashion. Initially, there was appropriate color and vascularity of the flap with no issues with the microvascular anastomosis. Progressive venous congestion of the flap, however, was recognized within several hours after procedure end. The patient returned to the operating room for neck exploration with revision of the venous anastomosis. Despite no evidence of thrombosis or kinking of the flap vein and a detectable flow with a Doppler, the flap remained congested. The vein was disconnected, and an end-to-side anastomosis to the left internal jugular vein was performed. Adequate venous flow was achieved, and the patient returned to the intensive care unit for monitoring.\nVenous congestion of the flap recurred (Figure ) within a few hours, and the patient was brought back to the operating room for exploration and free flap revision. At this point, the ulnar flap could not be salvaged. A left lateral arm free flap was then elevated to salvage the reconstruction. Again, microvascular re-anastomosis was uneventful with end-to-side anastomosis to the left internal jugular vein. Of note, the right neck was not explored for new recipient vessels due to likely vessel depletion on that side from prior neck dissection and two prior free flaps on that side as previously mentioned. Despite patent vessels as confirmed by visualization, palpation, and Doppler, there were still signs of early venous congestion intraoperatively. At this point, it was deduced that the left subclavian port was potentially contributing to elevated back pressure of the internal jugular vein. Review of a preoperative contrasted CT scan of the neck and chest revealed relative narrowing of the internal jugular vein and the subclavian vein at their junction point consistent with radiation-induced venous stenosis. The port catheter appeared to occupy most of the lumen of the subclavian vein causing occlusion of flow (Figure ,C). The port was subsequently removed intraoperatively, at which point the venous congestion immediately resolved.\nThe remaining postoperative course was uneventful. The left lateral arm free flap remained viable through the inpatient stay with discharge on postoperative day 10. The flap has healed well on long-term follow-up (Figure ). |
A 52-year-old woman was observed to have a pulmonary nodule in the left lower lobe on a chest X-ray, performed as part of preoperative testing for a dilatation and curettage. Computed tomography (CT) scans of the chest performed subsequently showed nodular opacities in the bilateral lower lobes. The patient was managed conservatively and a repeat CT scan performed 2 months thereafter showed almost complete resolution of the nodule in the left lower lobe and complete resolution of the nodule in the right lower lobe. At the time, these findings were thought to represent the resolution of inflammatory disease of the lung.\nIn October 2007, viral-type respiratory symptoms led to a CT scan of the chest which revealed a left lower lobe lung nodule. PET/CT scans in November 2007 revealed a 2.3×1.9-cm irregularly shaped nodule in the left lower lobe with minimal uptake (SUV 3.4) as well as a separate 7-mm non-avid nodule. Follow-up chest CT scans in December 2007 revealed that the left lower lobe nodule resolved into two separate nodules, in aggregates larger than the previous. Multiple new pulmonary nodules were observed in both lungs. A wedge resection of the left lower lobe in January 2008 revealed a mass composed of plasma cells admixed with lymphocytes, histiocytes and mesenchymal cells (). Review at the Pathology Department of the National Institutes of Health (Bethesda, MD, USA) confirmed the diagnosis of plasma cell granuloma. We have since performed ALK immunohistochemistry on this specimen, which identified no positivity.\nFollow-up CT scans of the chest were performed thereafter. In March 2009, there was progression of disease with new nodules compared with an assessment performed in December 2008. In April 2009, repeat CT showed increases in size of the left upper lobe and left lingular nodules. The patient underwent surgical resection of the left lingular mass in May 2009, again revealing plasma cell granuloma. Repeat scans performed one month after the second surgery demonstrated progression with multiple nodules involving bilateral lung fields. This prompted treatment with prednisone, initiated in June 2009 at 40 mg/day, which was reduced by October 2009. CT scans in September had revealed resolution of the nodules, however, in December 2009, a follow-up scan highlighted the recurrence of multiple lung nodules involving bilateral lung fields (). In January 2010, the patient was administered celecoxib 200 mg PO BID. By March 2010, CT scans showed improvement with resolution of a nodule and a reduction in size of others, with subsequent scans revealing continuing response to therapy. By February 2012, CT scans demonstrated resolution of all lung nodules (). The patient’s most recent CT scan in August 2012 revealed no progression of disease, 32 months after starting celecoxib. The patient has been reduced to 200 mg every other day.\nA literature search was performed in MEDLINE and EMBASE to identify all published studies using the search terms ‘inflammatory myoblastic tumor’, ‘plasma cell granuloma’, ‘inflammatory pseudotumor’, ‘NSAIDs’, ‘anti-inflammatory agents, nonsteroidal’ and ‘cyclooxgenase 2 inhibitors’. References for all retrieved studies were also reviewed to ensure that no studies had been missed in the primary search.\nAltogether, there have been a total of eight previous studies of IMTs managed successfully with NSAID monotherapy, comprising a total of ten patients (). In the first reported case, Hakozaki et al() treated a female with a 2.5×2.5-cm tumor in the left lobe of the liver. The biopsy revealed ‘edematous granulation tissue with capillaries and fibroblasts’. The patient had experienced abdominal pain for 6 months; one month of non-steroidal therapy induced complete remission (CR) of the mass and resolution of the abdominal discomfort. Su et al() reported on two patients with intra-abdominal masses. Tissue pathology was not described other than being consistent with an inflammatory pseudotumor (IPT). NSAID monotherapy induced CR after 2 months in one patient and after 4 months in the other patient. In both cases, NSAID therapy was discontinued at the time of achieving CR and the patients subsequently remained in remission. The case report of Chan et al() is the only case of NSAID therapy of pulmonary IMT other than our own case. The reported patient had an unresectable right lung mass, was febrile and toxic-appearing, with laboratory results indicative of acute inflammation with thrombocytosis, elevated ESR and elevated CRP. Histology revealed fibrous tissue, areas of necrosis and extensive lymphocyte and plasma cell infiltration. Rofecoxib therapy caused marked symptomatic improvement within 72 h; inflammatory parameters normalized and the patient achieved CR after 8 months of therapy. Przkora et al() published two cases of intra-abdominal IMT. Both patients received a short initial period of steroid treatment along with an NSAID, which was subsequently continued as monotherapy. The response trajectory is not described in either patient. One patient achieved CR after 14 months and the other had stable disease after 12 months. Both patients were maintained on NSAIDs at the time of publication. Colakoglu et al() reported on a case of a female with a solitary liver mass. NSAID therapy induced CR by 40 days. Vassiliadis et al() also reported on a patient with a solitary liver mass. In this case, the patient had fever, leucocytosis thrombocytosis and an acute phase reaction serology, i.e., the same clinical presentation as the case of Chan et al(). Naproxen induced a prompt reduction in temperature and was continued for one month. Off therapy, the mass continued to regress and the patient achieved CR. Mattei and Barnaby () reported a case in which ketorolac induced a significant response in a large retroperitoneal mass; the patient required surgery within 24 h due to rapid regression of the tumor resulting in a perforated duodenum. Finally, Colangelo et al() reported a case of a pancreatic head IMT in which ibuprofen induced CR after 6 months. Four years after ceasing drug administration, the patient remained in an unmaintained remission.\nThus, of the ten cases of NSAID therapy reported in the literature, nine were in CR and one had stable disease at the time of publication. The marked paucity of studies in the literature search is noteworthy, and likely reflects a publication bias, in that a study of unsuccessful use of NSAID therapy would not have been submitted for publication.\nFrom these studies, it is apparent that i) responses are not site-specific; ii) responses occur extremely soon after the onset of treatment, indeed as early as 24 h; iii) CRs to NSAID therapy may be extremely durable; iv) remission persists after termination of NSAID therapy. |
A 38-year-old otherwise healthy female presented with a two-year history of right hypochondrial pain consistent with the diagnosis of biliary colic. Her laboratory workup (including liver function tests) was normal. An ultrasound of her abdomen revealed multiple gallstones and a 1 cm x 1 cm single polyp in the body of her gallbladder. She underwent open cholecystectomy with an unremarkable postoperative outcome. The histopathology report of her gallbladder revealed a 1.2 cm x 1.5 cm lesion in the wall of her gallbladder consistent with Grade II (moderately differentiated) adenocarcinoma. It was a T1b lesion with uninvolved cystic duct margin. The case was discussed in the tumor board meeting of our hospital and it was recommended that CEC should be undertaken followed by gemcitabine-based adjuvant chemotherapy.\nCEC was planned and the patient was operated three weeks after the first surgery. Peroperatively, the patient was found to have a 1 cm x 1 cm hard mass at the cystic duct stump, which was a new finding. This mass was densely adherent to the common bile duct (CBD) and the first part of the duodenum. Because malignancy was suspected and the mass could not be freed from the duodenum, combined WPD and CEC was undertaken. The patient had an uneventful recovery and was discharged on her tenth postoperative day. The histopathology report of her second surgery revealed no evidence of malignancy and the hard mass was found to be due to FBGCR. However, no foreign body was identified in the specimen. Review of the operative notes of the first surgery revealed that the cystic duct had been ligated using silk suture.\nThe patient presented on her fourteenth postoperative day with complaint of on and off non-bilious vomiting for two days. The usual upper GI disturbances that occur after WPD were suspected and she was treated with dietary modification, pro-kinetic and anti-emetic agents. However, she had no relief in her symptoms. The symptoms increased in intensity and became persistent by the end of the third postoperative week. She did not develop jaundice during this period at any time. The patient was re-admitted for further evaluation. Her laboratory data was unremarkable including liver function tests. A diluted barium meal study was advised that showed hold up of barium in a dilated stomach with minimal dye passing into the efferent limb of the Roux-en-Y gastrojejunostomy (Figure ). Upper GI endoscopy was also performed and the gastrojejunostomy site was passed using the scope. While insufflation of air dilated the afferent limb and bile was visualized flowing in the expected direction, insufflation did not result in dilatation of the efferent limb. Additionally, the scope could not be negotiated further than 2 to 3 cm into the efferent limb where a sudden kinking was encountered.\nOn the basis of the above investigations, the patient was suspected to have efferent loop syndrome and was planned for urgent surgery. Peroperatively, an approximately 3 cm x 3 cm hard mass was found that was densely adherent to the inner side of the laparotomy incision and to the efferent limb of the gastrojejunostomy approximately 2 to 3 cm from the gastrojejunostomy site. There was significant kinking of the efferent limb because of this mass (Figures , ). Apart from some flimsy adhesions around the gastrojejunostomy site, the rest of the peritoneal cavity revealed no abnormalities.\nThis mass was dissected free from the small bowel and abdominal wall. As the previous histopathology report had revealed FBGCR and only three weeks had passed since her curative surgery, it was suspected that this may also be a mass of similar origin rather than peritoneal metastasis. Since the laparotomy incision had been previously closed with polypropylene (Prolene®) suture, it was decided to close the incision with polyglactin-910 (Vicyl®) suture this time. The patient had an unremarkable recovery and was discharged on her tenth postoperative day after she was tolerating oral soft diet. The histopathology report of the resected mass again showed FBGCR. The patient had no further symptoms and she was referred for adjuvant chemotherapy. Two and a half years after her adjuvant chemotherapy for gallbladder cancer, the patient is disease free and doing well. |
A twenty eight year old female delivered a baby 5 months before presentation. She started experiencing repeated episodes of blackouts in front of her eyes, since two weeks. These consisted of brief spells of diminishing of vision and inability to comprehend the surroundings lasting for a few seconds and passed off completely. She did not have falls or accidents due to these episodes. Along with these episodes, she developed a dull headache, lasting few hours every day, bilateral, frontal and was not associated with nausea or vomiting. She was able to continue her daily activities in spite of the headaches. The problem increased further when her relative noticed her to have a generalized tonic clonic convulsion, frothing at mouth and loss of consciousness lasting for 8-10 minutes. She remained confused for the next few hours and returned to normal. Her behavior gradually changed and she spoke only few words, low in volume without associated emotions. She would remain silent most of the day. She became slow in her routine activities. She found it difficult in maintaining her balance in sitting position, and would sway backwards whist sitting and standing. Her relatives noticed that when she smiled, her mouth deviated to left side. Gradually she became sleepier in daytime. Over these days she became further slow and eventually became bedbound. She could not stand without support. It was noticed that she had difficulty in holding objects with her right hand. She passed urine in clothes. Curiously, her relatives noticed fluctuation in her alertness in 3-4 days before coming to the hospital. During these days sometimes she used to be normal intermittently responding to verbal interactions, speaking few words and again after some time she would become drowsy. Relatives retrospectively felt that her disease could have begun earlier with some slowing and lack of interest which was put down to the puerperal setting and tiredness. There was no history of dysphagia, nasal regurgitation, fever or neck pain. There was no history of sensory complaints in the limbs, nor any visual impairment. She had not had any joint pains/recurrent abortions/deep venous thrombosis. There was no history of seizures or recurrent headache in past.\nPatient was afebrile with pulse 80/min regular and blood pressure of 124/80mmHg. There was no significant postural fall of blood pressure. There were no signs of meningeal irritation. She had no pallor, clubbing, cyanosis, lymphadenopathy, edema or pain in the lower limbs.\nWide fluctuations of level of consciousness were noted while she was in the wards. At times she would be conscious and responded appropriately to verbal interactions, with some impaired attention. At other time, often only few hours apart, she would become unresponsive, only opening her eyes to painful stimuli. These episodes of intermittent drowsiness continued for 2-3 days and then she became unresponsive. At that time her Glasgow Coma Scale was 10.\nExamination in the early stages before the persistent unresponsiveness developed, showed her speech output to be reduced with hypophonic, monotonous melody. Higher mental status could not be analyzed satisfactorily at any time due to her impaired attention, even when she was at her best. Pupils were bilaterally normal, equally reacting to light. External ocular movements were restricted in vertical gaze, upward more than downward, with preserved vestibulo ocular reflex suggestive of supranuclearophthalmoplegia.\nOn motor system examination she had right sided pronator drift, brisk reflexes in both upper limbs (right more than left) and extensor Babinski sign. Power examination was not possible in detail but still showed right more than left sided pyramidal pattern of weakness. Sensory examination was difficult to comment upon.\nHer complete blood count, liver function tests and kidney function tests were normal. Also her repeated electrolyte examinations during ward stay were normal. Her HIV, HBsAg and VDRL were non reactive. Her CSF examination was showing no cells with proteins of 26mg% and sugar 48mg% with normal opening pressure. MRI was done []. |
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
A 32 year-old male patient presented to our outpatient clinic with a 3-weeks history of insidious-onset annular skin lesions on the dorsal and palmar aspect of his hands with associated mild itching and fine scaling which did not regress with previously administered topical therapies. His general examination revealed generalized lymphadenopathy. Considering the age and clinical features of the patient, he was evaluated with the differential diagnosis of syphilis, sarcoidosis, erythema multiforme and granuloma annulare. His personal, and family history was unremarkable with no any complaints of respiratory distress or coughing. Dermatological examination revealed multiple erythematous, annular plaques varying in size from 1 to 3 cm in diameter localized on the dorsum and plantar aspect of palms with fine partly adherent dry scales. [Figures and ]. Skin biopsy was sent for histopathological examination. Histopathological examination of the biopsy material disclosed parakeratosis in the epidermis. Upper and mid dermis showed histiocytes dissecting between collagen bundles and areas of normal dermis intervening in between which is classically seen in the interstitial pattern of granuloma annulare. A mild perivascular and interstitial lymphocytic infiltrate was seen in the surrounding dermis with scattered neutrophils and eosinophils. The histopathological findings are shown in in the scanning view and shows the high-power magnification. On laboratory analysis, Erythrocyte Sedimentation Rate was raised and chest X-ray was suggestive of bilateral hilar lymphadenopathy. His serum ACE levels were increased and his Computed Tomography (CT) scan of chest and endoscopic biopsy of the hilar lymphnode showed features of sarcoidosis. The skin biopsy was reviewed again in view of the features of systemic sarcoidosis. Gram stain, Ziehl-Neelsen and periodic aid Schiff stains were negative for any microorganism on the skin biopsy. The histological findings from the hilar lymph nodes were suggestive of sarcoidosis, however no features of cutaneous sarcoidosis were detected. The patient was also investigated extensively for secondary syphilis due to the morphology of his skin lesions over the hands and the generalized lymphadenopathy of the patient. However Venereal Disease Research Laboratory (VDRL) and Treponema pallidum Haem Agglutination (TPHA) tests were negative for syphilis and his Enzyme-linked immunosorbent assay (ELISA) for HIV was also negative. |
A 23-year-old male presented with complaints of progressive facial asymmetry, difficulty in speech and mastication secondary to restricted movement of the mandible []. The patient also experienced intermittent pain in the right TMJ region. He reported progressive reduction in the vertical opening and increasing asymmetry of the face. The patient did not have any episode of trauma, and his medical history was noncontributory. Clinical examination revealed marked facial asymmetry with the mandible deviated to the left. The mandibular midline was deviated, indicative of asymmetric prognathism. Lateral movement to the right side was totally restricted. Significant dental compensations were evident. There was no translation of the right mandibular condyle, and positive tenderness of the left temporalis muscle was also noted on palpation. Preoperative calcium and alkaline phosphatase levels were within normal limits.\nPanoramic radiograph revealed a large radiopaque lesion of the right mandibular condyle, with the condyle resembling a mushroom and extending in front of the articular eminence. The ramus and mandible were elongated with a shift in the midline. Slight bowing of the body of the mandible was also observed radiographically on the right side. Left condyle and joint space were apparently normal. Computed tomography scan (CT) showed a large radiodense lesion on the right side of TMJ both in the coronal and axial sections. The lesion was clearly seen both in the plain and contrast images, extending on the right side up to the pterygoid plate medially and almost up to the carotid canal posteriorly measuring 33 mm mediolateral and 27 mm anteroposteriorly. The neck of the condyle was also enlarged and was found to be gradually merging with the vertical part of the ramus. CT scan reported tumor of the right condyle head [].\nSurgical removal of the enlarged condyle via preauricular approach was planned. Subsequently, with an extended preauricular incision placed through the skin and subcutaneous tissues to the level of the temporal fascia was carried out. Skin flap was extended by blunt dissection superoinferiorly and with preservation of the superficial temporal vessels by pushing it anteriorly. Vertical incision was placed in the temporal fascia and blunt dissection was carried out anteriorly until exposing the lateral part of the fossa and the enlarged condyle was exposed after opening the lateral ligament. With dissection, TMJ was exposed up to the neck of the condyle and internal maxillary artery on medial side was protected with condylar retractor. The bone was osteotomized and removed using a surgical bur []. The lesion including the condyle was removed and sent for histopathological examination. Postoperative recovery was uneventful and his mouth opening was fairly good. The patient has been regularly followed up every month for the initial 3 months and then once in 3 months with periodic radiographs. During the latest visit of the patient, it was observed that his facial appearance and mouth opening had returned back to normal and he had been symptom-free [Figures and ].\nThe entire specimen was decalcified in 10% formic acid and then processed for histopathological examination. Histopathology showed chondrocytes of the cartilaginous cap arranged in clusters in parallel, oblong lacunar spaces, similar to that of the normal epiphyseal cartilage along with regular bony trabeculae. There was no evidence of cartilage islands in underlying bony trabeculae. These features were suggestive of osteochondroma. Considering the size of the lesion, the diagnosis of giant osteochondroma was made []. |
A 33-year-old Chinese man was admitted to our department for sustainable foamy urine for more than one year. He also complained of intermittent hair loss and recurrence of oral ulcers.\nApproximately one year prior, the patient was hospitalized at a local hospital for the same reason, and routine urine tests indicated microscopic hematuria and proteinuria. He did not pay much attention, and there was no further diagnosis or treatment because of a lack of conscious symptoms. One month prior, his blood pressure rose to 145/91 mmHg for unknown reasons; microscopic hematuria and heavy proteinuria were again detected.\nThe patient had no comorbidities.\nThe patient's father had asymptomatic microscopic hematuria and proteinuria, as detected in a routine physical examination approximately 2 years prior. The patient had a daughter and a son; the daughter (7 years old) had asymptomatic microscopic hematuria, and the son had microscopic hematuria and proteinuria. His son had ever been diagnosed with chronic nephritis at a local hospital.\nThe patient's appearance was normal, without edema. His systolic and diastolic blood pressures were 141 mmHg and 90 mmHg, respectively; his pulse rate was 81 beats per minute, and his respiratory rate was 19 breaths per minute. No obvious abnormality, including growth retardation, was detected during physical examination, and no specific nervous system symptoms were recognized. The patient was also subjected to audiologic assessments, but no hearing impairments were detected, even at high frequency. Furthermore, no symptoms were found in either eye by comprehensive ophthalmic examinations.\nMicroscopic hematuria and proteinuria were confirmed by urine tests. The results of other tests, including routine blood tests and serum immunology, are listed in Table .\nNo obvious abnormality was detected by abdominal ultrasound examination, X-ray diagnosis, or electrocardiographic examination. However, heart echocardiography showed a small amount of pericardial effusion.\nTo further analyze the renal presentation, a histopathology study of renal biopsy was performed. By light microscopy, a total of 13 glomeruli were observed, with one glomerulus being enlarged and lobulated. Para-aminosailcylic acid staining and Masson staining were positive, showing mild mesangial matrix proliferation. The basement membrane was thickened. Three glomerular fibroblastic crescents and pericystic fibrosis of glomeruli were observed (Figure ). In addition, deposition of erythrotropin under the endothelium of the capillary loop was detected (Figure ). Electron microscopy revealed obvious basement membrane lesions including variable thickness and reticulation of the glomerular basement membrane, as well as irregular subepithelial protrusion of the lamina densa. Fine particles and electron-dense bodies were detected in the stratified basement membrane (Figure ). Immunological staining for IgG, IgA, IgM, C3, C4 C1q, К, and λ was positive in four glomeruli, with the signals being deposited in the vascular lumen and mesangial area in a granular or linear form (Figure ).\nA considerable investigation of family history was performed. The patient’s father had asymptomatic microscopic hematuria and proteinuria, as detected in a routine physical examination approximately 2 years previously. As mentioned above, the patient had a daughter and a son: The former had asymptomatic microscopic hematuria, and the latter had microscopic hematuria and proteinuria; his son had been diagnosed with chronic nephritis at a local hospital. Thus, three relatives had microscopic hematuria. Therefore, a diagnosis of ATS was highly suspected (Figure ). For a precise conclusive diagnosis, the patient and his children were recommended to undergo genetic testing, and WES was performed. Genomic DNA was extracted from blood samples; WES was performed as previously described. After sequencing, the coverage of the target sequence was over 99.12%, and the mean sequencing depth was approximately 147. The sequencing analysis revealed a heterozygous substitution, NM_000091 c.2657-1G>A (p. V294fs) in intron 22 of the COL4A3 gene, which was confirmed by Sanger sequencing (Figure ). The mutation was excluded from the single nucleotide polymorphism database but was included in the ClinVar database. As this mutation is located at an evolutionarily conserved splice site, this splicing mutation is thought to lead to the skipping of exon 23. In addition, this variant is classified as “likely pathogenic” according to the American College of Medical Genetics and Genomics standards and guidelines[] (Figure ). |
A 16-year-old girl underwent multiple operations for congenital gastroschisis at other hospitals. Primary skin closure was performed, and abdominal plasty was avoided. The patient presented with a complicated scar on her middle abdominal wall that measured approximately 5×12 cm, as well as other depressive scars resulting from drain insertions (). Computed tomography (CT) images demonstrated that part of her intestine was located immediately under an extremely thin postoperative skin layer, with suspected adhesion. In addition, a partial defect of the rectus abdominis muscle was detected ().\nTo perform a safe and effective operation, we positioned the skin incision to avoid intestinal trauma while considering the cosmetic elements related to postoperative scarring. In addition, we conducted a detailed investigation of the range across which retention sutures could be placed while maintaining the structure of the fascia. Using 3-mm-slice CT images as references, the range of the intestine located immediately below the thin postoperative skin, the range of stable fascia structure and intended retention sutures, and the range of the rectus abdominis fascia muscle intended for preservation were measured and drawn on graph paper (). The resulting shapes were cut from the paper, traced onto the patient’s body, and used to determine the skin incisional line (). The midline was designated from the xiphoid process to the umbilicus. We planned an operation involving the following elements: the range of intestine located immediately below the thin postoperative skin layer; a skin incision that would allow subcutaneous tissue preservation; de-epithelialization of the skin only near the midline; and gradual, bilateral deep undermining of the subcutaneous tissue beyond the stable fascia structure with retention sutures as needed.\nThe above-described operation was performed with the patient under general anesthesia, as planned. The skin surrounding the incision was carefully de-epithelialized in the middle dermal layer, gradual deep undermining of the subcutaneous tissue was performed bilaterally, and the bilateral edge of the stable rectus abdominis fascia was approximated to the extent possible in order to reduce tension on the skin sutures at the midline. Subsequently, skin suturing was performed in a layer-to-layer manner (). Three scars from previous drain insertion procedures were incised and sutured above the rectus abdominis fascia. One caudal scar was preserved as a substitute for the umbilicus. Intestinal trauma was avoided during surgery, and the patient did not experience postoperative complications. Two years later, she had achieved excellent cosmetic results (), with no exacerbation of herniation or ileus. |
A 48-year-old woman, gravida 1, para 1, visited the internal medicine department at another hospital with a complaint of abdominal fullness and weight loss of 10kg during the last year. A huge abdominal mass was palpated, and she was referred to the gynecology department to search for a tumor of uterine origin. She was premenopausal and had no significant past medical history. Physical findings revealed a large elastic hard mass extending from the xiphoid to the pubic bone. The magnetic resonance imaging (MRI) examination revealed a huge tumor on the uterine corpus, and a number of dilated vessels were observed between the tumor and the myometrium. Therefore, the tumor was suspected to derive from the uterus. The tumor showed an uneven signal on T2-weighted sagittal section (), and the enhanced MRI study showed that the tumor edge but not the center was enhanced (). As such, necrosis was suspected to have occurred in the center of the tumor. Uterine sarcoma was primarily suspected due to the large size, degeneration, and necrosis on MRI imaging. Computed tomography (CT) examination showed no lymph node swelling or distant metastasis. Preoperative laboratory testing revealed anemia (hemoglobin level, 5.6g/dl). We transfused 18 units of RCC before surgery. CT examination and ultrasonography on lower extremities indicated an absence of thrombosis. Preoperative serum levels of CEA, CA 19-9, CA 125, and LDH were within normal limits. A biopsy of the endometrium was not collected as the sounding examination of the endometrium was unsuccessful due to a deviated uterine cervix. At this point, preoperatively, we suspected the tumor was a leiomyosarcoma or leiomyoma with degeneration.\nThe patient underwent laparotomy, where we identified a huge tumor occupying a space from the pelvis to the diaphragm. The tumor surface was smooth and hard with many dilated veins (). A massive tumor with a diameter of 30 cm was observed arising from the posterior uterine wall with a smooth contour and invaded the retroperitoneal cavity under the mesentery. The tumor was firmly adhered to both the mesentery and right ovary. There were no findings of extra-uterine dissemination. The intraoperative frozen section report for the uterine tumor was of degenerated myoma with no findings indicating malignancy. A total abdominal hysterectomy (TAH) and right salpingo-oophorectomy (RSO) were performed. The operation duration and blood loss were approximately 216 minutes and 1000 ml, respectively. The excised specimen weighed 7600 g.\nMacroscopic findings of the tumor revealed a well-circumscribed tumor showing extensive continuity with the posterior wall of the uterus, measuring 28 × 23 cm (). On the sliced surface of the tumor, an obvious heterogeneous pattern was recognized within the mixture of a whitish homogeneous area, suggesting benign uterine fibroids, and a vulnerable area, due to bleeding and necrosis ().\nFor the intraoperative frozen section, we examined three areas, namely, a white homogenous part, a necrotic part, and a cystic part, of which all were findings of a leiomyoma. In the permanent histological examination, 10 additional sections were collected from the tumor. The basic histological findings of all the sections were the same. The tumor was comprised of spindle-shaped cells, homologous to smooth muscle cells, which were arranged in bundles with areas of hyalinization, consistent with a degenerated leiomyoma. The tumor was mostly comprised of degenerated uterine leiomyoma. However, enlarged blood vessels were observed within an area of approximately 2 cm × several mm, and proliferation of atypical cells showing a fine meshwork microvascular structure was observed in the blood vessel cavity (). These atypical cells consisted of various contours, such as cubic, polygonal, and short spindle shape. The nucleus was circular with a high degree of vacuolar enlargement and pleomorphism. Abnormal mitotic figures were also interspersed (). A tumor derived from a blood vessel was thus considered, and malignancy was suggested by the presence of nuclear atypia and abnormal mitosis.\nImmunohistochemical analysis revealed the atypical tumor cells to be positive for ERG, CD31, and AE1/3 (Figures and ), partially positive for Factor VIII, and negative for α-SMA, desmin, H-caldesmon, EMA, CD34, and D2-40. From the above, the atypical tumor cells were of epithelial origin and the final diagnosis was epithelioid angiosarcoma arising in a degenerated uterine leiomyoma.\nThe efficacy of postoperative adjuvant therapy for angiosarcoma has not been demonstrated and there is currently no established chemotherapy regimen. In this case, because the atypical tumor was observed in the blood vessel cavity, we thought it could have been spread hematogenously throughout the body. Hence, we selected adjuvant chemotherapy rather than adjuvant radiotherapy. Six courses of combination adjuvant chemotherapy with paclitaxel (150mg/m2), epirubicin (50mg/m2), and carboplatin (area under the curve = 4) were administered in the present case, following referral to previous reported cases. No recurrence has been observed 10 months after the primary surgery. |
A 68-year-old man with a history of a distal right femoral diaphyseal fracture that resulted in malunion and right varus knee deformity previously treated with a high tibial osteotomy (HTO) presented with severe osteoarthritic pain of the right knee. The patient had a distal femur fracture at the age of 18 years and was treated conservatively which resulted in malunion and shortening of the leg. At the age of 44 years, he was diagnosed with osteoarthritis and underwent a high tibial osteotomy. However, knee pain continued to get progressively worse especially for the last few years. The patient's social history included using an assisted walking device, being an active cigarette smoker, and combating chronic hypertension. Clinical evaluation and radiographic imaging confirmed the diagnosis of severe bone-on-bone tricompartmental osteoarthritis (). The patient's knee range of motion was significantly restricted from full extension to further flexion of only 90 degrees. The patient previously failed various conventional nonsurgical treatments and wanted to proceed with TKA. Given the complexity of this case, the RATKA MAKO-Stryker surgical arthroplasty system was advised as the surgical method in order to plan for implants that would better fit this patient's specific geometric bony anatomy with the plans of bringing the mechanical axis within 3 degrees of neutral biomechanical alignment. A thorough discussion of the risks and benefits of the surgical procedure and preoperative 3-D computed tomographic images of his knee was performed.\nThe surgical planning included obtaining a MAKO protocol CT scan. The robotic software converts CT images to a virtual model where a surgeon can plan on implant size, positioning, and joint alignment. As demonstrated from images, this patient had a distal femur fracture, which had resulted in malunion. Because of malalignment, the patient had recurvatum deformity. The use of the anatomical axis and jig-based technique would have resulted in the positioning of the knee implant into hyperextension. Also, it would have been difficult to use the jig-based technique in the setting of severe deformity of the distal femur. Also, deformity in the proximal tibia due to a previous high tibial osteotomy would have resulted in difficulties in establishing the mechanical axis. Because of robotic software, we were able to plan the implant position in a way that it would restore the mechanical axis.\nAfter exposure, femoral and tibial trackers were applied. The hip center of rotation was registered followed by medial and lateral malleolus registration. Bony landmark registration was done. Now, we proceeded with removing all the visible osteophytes and remaining ACL. Often times, it is difficult to remove the PCL completely without making the tibial cut in tight knees with longstanding deformities.\nWe next proceeded with identifying the existing deformity without any correction and with corrective maneuver and checking the balances on the medial and lateral side (). With the planned tibial cut of 7 mm from the highest point of the medial tibial condyle with 0-degree varus, we were looking at an extension gap balance of 19 mm laterally and 18 mm medially. This was enough to accommodate an at least 9 mm poly liner. However, in flexion, this gap was 12 mm and 15 mm on the lateral and medial side, respectively. But often, making a cut on the tibia removes the tight PCL and opens up the flexion gap. Also, we all know that tibial cut symmetrically affects the flexion and extension gap. So, we decided to proceed with this tibial cut first.\nNext, appropriate tibial cut was made. The previous existing hardware was removed. With the help of the lamina spreader, the balance of the ligament was checked again in extension and flexion. We were able to open up the flexion gap more. Moving the planned femur implant anteriorly and thereby planning to remove more bone posteriorly, we were able to get the numbers closer to the extension gap as evidenced by .\nNow, as evidenced by this figure, on the right bottom corner, the extension gap is 19 and 18 mm and the flexion gap is 19 mm laterally and 22 mm medially. At this point of time, the femoral implant is in 1° external rotation. So now, we needed to close down the medial gap in flexion. We were able to do this by pinning the femoral implant laterally and internally rotating the femoral component. By this maneuver, we were able to predict almost equal gaps in both flexion and extension. We proceeded with the femoral cuts. Trial implants were positioned.\nThe patient remained in the hospital for 2 days to ensure that adequate pain management was obtained prior to being discharged home. He returned for a follow-up visit in 3 weeks postoperatively (). During this visit, his range of motion was recorded as 0° to 95° flexion, with stable varus/valgus stress testing, negative anterior/posterior drawer signs, palpable distal pulses, and great strength within the quadriceps muscle group. At this point, the patient's knee replacement was deemed clinically stable and the patient was advised to return for a 2-month follow-up. |
A 14-year-old female had a bony defect on her head that was discovered by X-ray Photograph (xp) and computed tomography (CT) imaging, which were performed to investigate a head injury. The lesion was associated with scalp sutures she had received for a head injury she sustained at the age of 6. Immediately below the injury, a gradually enlarging bone-like tumor mass was observed. Upon admission, a subcutaneous mass with the same hardness as bone was found, which was palpable on the left occipital bone, and head xp showed a corresponding bone transmission image at the same lesion (Figure ). CT demonstrated a corresponding image of a bony defect and swelling of the outer table of the skull (). MRI revealed a corresponding bony defect and swelling at the subcutaneous tumor mass with cystic changes in the cavity of the bony defect, and a contrast study showed a partial enhancement around the cystic change (). 99mTc-HMDP bone scintigraphy demonstrated strong accumulation at a part of the cystic lesion (), and head DSA study revealed no tumor stain or feeder ().\nBased on clinical symptoms, history, and image observations, the differential diagnoses included aneurysmal bone cyst, simple bone cyst, eosinophilic granuloma with cystic change, growing skull fracture, and cystic FD. Therefore, a biopsy and tumorectomy under general anesthesia were performed to diagnose and radically cure the patient.\nIntraoperative Observations. A cutaneous scar due to past injury was observed at the surface of skin immediately above the skull mass (, arrowheads). The outer table of the skull beneath the skin of the scar was smooth without fracture but swollen (). After removing the outer table of the skull, the cystic cavity and bloody liquid component were observed. After aspiration of the liquid component, only bony tissue was grossly observed inside the cavity without macroscopic tumorous change, and the inner table of the skull was maintained with mild thinning (s). Finally, the cystic bony mass was completely removed, securing a margin of 5 mm around the mass. The dura mater and brain tissue just below the bony cyst was intact (). The defective part of bone was reconstructed with calcium phosphate paste.\nThere were no significant postoperative complications. Postoperative xp and CT showed that the tumor mass had been completely removed, and cranioplasty of the bone deficit after tumor removal was successful (Figures and ). Results of a pathological examination by H&E staining demonstrated an FD tissue image in a region where strong technetium accumulation was observed (). |
A 45-year-old female with history of Protein C deficiency and recent right thoracic outlet decompression surgery and first right first rib resection 3 weeks prior presented to an internal medicine clinic with dyspnea on exertion of one day duration. The patient was in good health up until 8 months before presentation when she developed recurrent transient ichemic attacks (TIAs). She underwent extensive evaluation at several hospitals and eventually was found to have thoracic outlet syndrome of the left upper extremity and possible thrombus at the subclavian artery. She successfully underwent left-sided thoracic outlet decompression and left first rib resection 7 months prior to presentation. Her post-op period was complicated by a subocclusive left external iliac deep vein thrombosis (DVT), and she was treated with apixaban. She recovered over the next several months and continued anticoagulation for her prior TIAsand DVT in the setting of Protein C deficiency. Two months prior to presentation, she developed right arm symptoms consistent with thoracic artery outlet syndrome on the contralateral side. She ultimately underwent thoracic outlet decompression surgery and right first rib resection 3 weeks prior to presentation, only missing full anticoagulation for 2 days. She was recovering as expected until she began to notice dyspnea on exertion 1 day prior to presentation.\nHer vitals were within normal limits. Her physical exam was notable for decreased breath sounds and dullness to percussion in the right lung base. To evaluate her complaints further, a Cardiac Limited Ultrasound Exam (CLUE) was performed with the phased array probe and revealed the right hemidiaphragm had decreased movement compared to the left hemidiaphragm with a normal CLUE exam otherwise ( and ; Supplement Videos 1 and 2) [].\nLabwork including troponin was normal, and electrocardigram revealed a new right bundle branch block but no evidence of ischemia. CT angiography of the chest was pursued due to her high risk of venous thromboembolism after surgery but did not show pulmonary embolism. It did confirm marked right hemidiaphragm elevation with associated compressive atelectasis. Sniff test confirmed abnormal motion during normal respiration of the right hemidiaphragm and abnormal paradoxical movement during sniff. She was diagnosed with postoperative unilateral diaphragm dysfunction that was initially found by point-of-care ultrasound during her clinic visit. |
In 2017, a 60-year-old female presented to her General Practitioner (GP) in Scotland with a slow-healing 1 cm wound on her right finger. She had sustained a minor burn to the finger 9 days prior to her attendance. A swab was taken by the attending clinician. This swab subsequently grew toxigenic . This result was telephoned to the out-of-hours local health protection team, 8 days after the case presented to her GP.\nThe case lived with her partner and owned two German Shepherd dogs. One dog had a history of a long-standing skin complaint, which had been documented as quiescent. The case worked in retail and had no recent travel history. She had not eaten unpasteurized dairy food nor visited any farms within the last 2 weeks. Her most recent diphtheria vaccination was in 2009.\nA nose and throat swab were collected from the patient and she was treated with diphtheria vaccine and a 2 week course of clarithromycin. She was excluded from work under the Public Health Act Scotland (2009) until completion of antibiotics and clearance swabs, one from each site (nose, throat and wound) were negative. Four close contacts were identified who required nose and throat swabs to assess for carriage of , of whom two were healthcare professionals who had dressed the wound. Contacts were all given diphtheria vaccine and a 1 week course of erythromycin. The two healthcare workers were excluded from work until nose and throat swabs were culture negative. One contact (husband) was identified as carrying in his nose and required a repeat swab following completion of antibiotics to check for clearance. His repeat swab was culture negative. Initial swabs for the fourth contact (a close friend) were negative.\nA local veterinary practitioner examined the dogs, which revealed one had an infected skin lesion, which was swabbed. Throat swabs were also collected from both dogs. Toxigenic was recovered in culture from the skin lesion and throat swabs of both dogs. They were commenced on antibiotic therapy and antimicrobial skin wash. Repeat swabs for the dogs were negative following completion of treatment. The dogs were identified as the most likely source of infection, however, it is possible human–human transmission may have occurred.\nWound swab was submitted for routine culture and was inoculated on Columbia blood agar (Thermo-Fisher, Perth, UK). Large numbers of \n were cultured and identified using MALDI-TOF (MALDI Biotyper, Bruker, Massachusetts, USA). MALDI-TOF generates unique mass spectrometry profiles, which are compared to a known database of micro-organism profiles, identifying the organism to genus and species levels []. The MALDI-TOF score was >2.0, which is an acceptable score for species identification. Susceptibility testing was performed by E-test (bioMérieux) using European Society of Clinical Microbiology and Infectious Disease (EUCAST) interpretative criteria. The isolate was sent to the Diphtheria National Reference Laboratory, Public Health England (PHE), Colindale, London, where the isolates were characterized by genotypic and phenotypic methods. In April 2014, a real-time PCR (qPCR) assay was formally introduced as the front-line test for putative toxigenic corynebacteria to inform public health action []. This assay provides confirmation of both identification of C. diphtheriae and / and detection of the diphtheria toxin gene. Phenotypic characterization was perfomed by culture on Columbia horse blood, Hoyle’s tellurite and Tinsdale agar plates (PHE Media Services, Colindale); API Coryne (bioMerieux) and additional differential biochemical tests (e.g. nitrate reduction, glycogen hydrolysis as required []. The modified Elek immunodiffusion test [] was used to confirm toxin expression. Further genotypic characterization of isolates was performed by MLST as previously described []. The clinical isolate from the index case was identified as /C.diphtheriae, diphtheria toxin gene positive by qPCR. The species was confirmed as C. ulcerans phenotypically and toxin expression was confirmed by the Elek test. Antimicrobial susceptibility testing results were also confirmed at the reference laboratory and it showed resistance to penicillin and clindamycin but sensitivity to vancomycin, erythromycin, linezolid, ciprofloxacin, doxycycline and rifampicin. was isolated along with C. ulcerans. S aureus. could be colonizing the skin but was also likely contributing to any skin and soft tissue infection.\nwas also isolated from a nose swab from the close contact of the index case and this was confirmed as toxigenic as above. Throat swabs from the two dogs and the skin lesion were collected by their veterinary practitioner and submitted to SAC Consulting Veterinary Services laboratory in Inverness where they were cultured on Columbia sheep blood agar and Hoyles tellurite medium (Thermo-Fisher, Perth, UK). was obtained in moderate growth from the throat swabs collected from each dog and in heavy growth from the wound. The wound also contained a heavy growth of subsp. coagulans, and an unidentified Gram-positive coccus. Suspect were identified with the API Coryne system. Follow-up samples were collected from the throats of both dogs after treatment was completed and was not detected. Furthermore, the skin lesion had resolved and neither , S. schleiferi subsp. coagulans nor the unidentified Gram-positive coccus were detected. The three canine isolates from the two dogs were also confirmed as toxigenic .\nThe six isolates (three human, three canine) were subjected to MLST analysis. Full profiles were not obtained, most likely due to variation in primer binding sites. However, of the partial alleleic profiles obtained (range 2 to 5 out of 7 alleles); all alleles occurring in >1 isolate matched and there were no mismatches, supporting an epidemiological link. All partial allelic profiles obtained (0, 41, 79, 49, 0, 45, 39) were consistent with sequence type 349 (42, 41, 79, 49, 49, 45, 39), which is present in the MLST database () [], from isolated from a cutaneous clinical case in 2005, from Toulouse, France. |
A 44-year-old Caucasian man presented with worsening swelling, tenderness, and erythema around his right eye, including the right side of his face. According to the patient, he woke up in the morning with swelling of the right eye and did not think anything of it. He thought it would improve. However, in less than 24 hours, he had extensive right facial swelling extending all the way to the submandibular region. On presentation, apparently, he had extension across to the left periorbital region and the premaxillary region as well. He did not note any leukocytosis; however, had bandemia. He was noted to have elevated liver enzyme tests (LETs), also noted to be in alcohol withdrawal with severe shakiness. It was found through intraop cultures that he tested positive for having group B Streptococcus pyogenes (Strep pyogenes), which indicated that he had necrotizing fasciitis. Subsequently, he underwent debridement and placement of wound VAC to the right upper and lower eyelids. The treatment involved using debridement and wound VAC placement in order to treat his septic shock in the setting of severe right facial cellulitis along with right periorbital cellulitis.\nThe patient was brought to the operating room and placed in a supine position under general anesthesia. He had an inspection. There was a VAC that was present with some sort of adhesive toward the nose, but essentially was drying at an adequate pressure. The patient had been getting IV antibiotics and once the operative report was obtained, the procedure began with attention centered to the right upper eyelid, which looks exceedingly improved compared to its original condition. The nasal aspect of the wound showed no necrosis. There was granulation tissue formation. The temporal aspect shows some level of necrosis and this was removed down to a vital muscularis region. The inferior area of necrosis looked well as far as the midline to the canthus, however, the lateral aspect required quite a bit more dissection down through, but external to the orbicularis. A bleeder was noted as dissection was made down through the maxilla and was cauterized. The area was dry, so a moist 4 X 4 wet-to-dry gauze was placed and then a Tegaderm was placed over it. If the nursing staff had been here, the VAC could have been placed, however, given the circumstances, we placed the VAC in the ICU with the patient being given proper sedation (Figure ). The patient tolerated the procedure well and went back into the ICU.\nPost-operation, he had interval improvement with his IV antibiotics. There was decreased swelling. His extraocular eye movements were full. There is a skin graft over the right upper eyelid, which extends through the areas of previous necrosis, and there is periorbital edema. He was able to open his eyes. There was no discharge in the conjunctiva. We recommended that the head of his bed should be elevated, as this will help decrease the edema around his left eye. The patient had pressures of 20 mmHg in both eyes. His cornea was clear and his anterior chamber was well-formed. His preseptal region was more edematous than previously reported two weeks ago when the sutures were removed. The patient was prescribed to have antibiotic bacitracin ointment once a day. The CT scan was noticeable for right lateral periorbital soft tissue swelling without any adjacent fracture. This was most likely the rotational flap. Otherwise, there was decreased swelling as compared with previous examinations. The maxillofacial CT scan was normal. He had interval improvement with his IV antibiotics. |
In October 2012, a 46-year-old woman was referred to our center for the presence of a large mass (50 × 70 mm) in the superior lobe of the left lung with homolateral pleural effusion. The patient was never smoker, without family history of cancer and without comorbidity. The cytological diagnosis was made using a CT-guided fine needle aspiration of the primary tumor and revealed an adenocarcinoma of the lung (TTF1+, CK7+) with the EGFR ex19del mutation. A PET-CT demonstrated the presence of liver and bone metastases and a nodule in the right breast, confirmed as a metastasis by fine needle aspiration. The patient received zoledronic acid 4 mg every 28 days and gefitinib 250 mg daily since November 2012 obtaining a partial response (PR). In August 2013, a disease progression (PD) was documented, with an increase in size of the primary tumor and size and number of liver metastases. A brain MRI revealed the presence of two cortical nodules, which were treated with stereotactic radiotherapy. The patient was enrolled in the IMPRESS trial and received 6 cycles of cisplatin and pemetrexed plus gefitinib obtaining again a PR that lasted until June 2014. Thereafter, a new lung metastasis appeared in the superior lobe of the left lung and the mammary nodule increased in dimensions. From June 2014 to December 2014 the patient received erlotinib 150 mg daily obtaining an initial stabilization of the disease (SD); however, within 6 months, she experienced again a PD with the increase of the mammary nodule and the appearance of a new bone metastasis in the sacrum. In December 2014, EGFR ex19del and T790M mutations were detectable in a new needle biopsy of the primary tumor; only at this time a digital PCR-based method was available for the analysis of circulating tumor DNA (ctDNA). Briefly, the method was optimized in order to recover a suitable amount of ctDNA for molecular analysis from 3 ml of plasma using the QIAmp Circulating Nucleic Acid Kit (Qiagen®, Valencia, CA). ctDNA was examined using the Prime PCR Probe Assay on a QX100™ Droplet Digital™ PCR System (BioRad®, Hercules, CA) for EGFR mutations (ex19del, T790M, and C797S) []. The ctDNA sample was considered as EGFR mutant when at least one droplet was above the fluorescence intensity threshold of 3000 and results were reported as copies/ml. The first plasma specimen was obtained in December 2014 and confirmed the presence of ex19del and T790M mutations (480 and 260 copies/ml, respectively; Fig. ). The patient was treated with atezolizumab from March to May 2015 and received stereotactic radiotherapy on the lung primary tumor and on metastases of the left superior lobe, breast and bone (sacrum and D10). Due to PD, the patient was given afatinib 40 mg daily from June 2015 to September 2015, but she experienced a further PD with an increase in size of the primary tumor and lung nodules, the appearance of new multiple bilateral lung metastases, lymphangitic infiltration and liver metastases. At this time, the presence of ex19del and T790 M was again demonstrated in a biopsy of a liver metastasis. Since osimertinib was available, the patient was enrolled in the ASTRIS trial and given 80 mg daily starting from October 2015 with an immediate clinical benefit. At the first evaluation a PR was documented with disappearance of most of the lung nodules and lymphangitic infiltration, reduction of the primary tumor and of liver metastases as well. A disappearance of ex19del or T790M was demonstrated in ctDNA in a blood sample obtained in October 2016. However, in April 2017, ex19del appeared again (400 copies/ml) and in July 2017 it increased to 1000 copies/ml, while T790M was also detectable (330 copies/ml, Fig. ). Then, ex19del and T790 M continued to increase to, respectively, 1600 and 510 copies/ml in October 2017, 2100 and 550 copies/ml in November 2017, and 6900 and 1900 copies/ml in December 2017.\nA radiological progression was demonstrated with increase in size and number of liver metastases in December 2017. The patient underwent a new liver biopsy that confirmed the PD and the presence of ex19del and T790M, whereas the ctDNA showed also the appearance of C797S mutation (170 copies/ml), in addition to ex19del and T790M. The patient started chemotherapy with carboplatin and pemetrexed and in February 2018 she obtained an SD associated with a drop of ex19del (800 copies/ml), T790 M (380 copies/ml), and C797S (90 copies/ml) and then a PR in May 2018, with disappearance of C797S and reduction of ex19del (760 copies/ml) and T790M (90 copies/ml). In July 2018, however, ex19del strongly increased to 2200 copies/ml, even though T790M and C797S were undetectable. Finally, in October 2018, when a PD was documented, ex19del increased to 5100 copies/ml, while T790M and C797S appeared again in plasma with 600 and 180 copies/ml, respectively (Fig. ). At this time a NextSeq 550 NGS platform (Illumina®, San Diego, CA) was available to analyse ctDNA by the AVENIO ctDNA Expanded Kit (Roche®, Pleasanton, CA). A plasma sample collected in December 2018 and analysed by NGS confirmed the presence of the ex19del, T790M and C797S and found, in addition, EGFR and c-MET amplifications, which were not present in tissue in the last re-biopsy of December 2017. |
We present the case of a 56-year-old female with history of syncope due to third degree atrioventricular heart block presenting initially with onset of stroke symptoms six days after pacemaker placement and two days after hospital discharge. At 5 PM she developed abrupt onset of left facial droop along with left upper and lower extremity weakness. The patient was initially treated at an outlying hospital and received alteplase at 6:35 PM for treatment of acute ischemic stroke.\nA chest radiograph performed at the outlying hospital prior to alteplase administration demonstrated an enlarged cardiac silhouette when compared to prior radiographs showing only borderline cardiomegaly. Upon administration, the patient reported mild chest pain and was given nitroglycerine and morphine. Her chest pain resolved and she was transferred to our comprehensive stroke center for admission. The patient presented to our emergency department at 10:10 PM with a heart rate of 122 beats per minute (bpm) and a blood pressure of 109/41 millimeters of mercury (mmHg).\nAt 11:20 PM the patient went for a computed tomography angiogram (CTA) after an initial assessment by the emergency physician in consultation with the stroke-team attending physician. After CTA at 10:28 PM, she was documented to have a blood pressure of 49/25 mmHg and heart rate of 109 bpm. She was returned to the resuscitation bay for re-evaluation. Cardiac tamponade was suspected due to the extreme hypotension in the setting of thrombolytic administration after recent pacemaker placement.\nOn reassessment, the patient had become confused with a Glasgow Coma Scale of 14. The emergency physician performed a POCUS, which demonstrated a pericardial effusion with features of cardiac tamponade including diastolic collapse of the right ventricle (). At that point the diagnosis of cardiac tamponade was made. The patient was alert and responsive, so an intravenous bolus of normal saline was given while a stat surgical consult was obtained. The surgical team evaluated the patient at the bedside within minutes and was able to review the POCUS findings. As the patient was conscious, they elected to take her immediately to the operating room rather than perform a bedside pericardiocentesis.\nWhile in the operating room, approximately 400 milliliters of coagulated blood were evacuated from the pericardial sac with 150 milliliters of surgical bleeding. The operative report notes resolution of tachycardia following this intervention with heart rate trending down to a range of 80–90 bpm with concomitant improvement in blood pressure. She was discharged two days post-operatively with a pericardial catheter in place. Echocardiogram performed on day of discharge noted a small, residual pericardial effusion. |
A 74-year-old female retired healthcare worker presented with abdominal pain and vomiting following a left inguinal hernia repair performed 7 days priorly. Symptoms began two days after her surgical procedure. Patient had a seven out of ten nonradiating, diffuse abdominal pain localized to the epigastric region described as burning in nature. It was accompanied by fever, nausea, and postprandial vomiting, which was clear and mucoid in nature, sometimes containing food particles. She did not have bowel movements since her inguinal hernia repair. Upon further inquiry, the patient mentioned that she had a decrease in appetite for all types of food for the past 6 months and began limiting her intake to liquids because they were easier to tolerate. The patient also noticed changes in stool caliber and noted an unintentional weight loss of 25 pounds in a one-month span. The patient stated that laboratory tests were unremarkable. A stool exam was not ordered since patient had no complaints of diarrhea. She had an esophagogastroduodenoscopy performed four months priorly due to similar symptoms and the results were essentially normal. The patient has a history of osteoarthritis. Surgical history includes umbilical hernia, a hysterectomy in 2012, and a left inguinal hernia in 2015. Family history includes diabetes mellitus and breast cancer. Patient is a retired home healthcare worker who resides in Grenada six months out of the year and frequently visits the United States. Patient has no other significant medical conditions and denies use of tobacco, alcohol, or illicit drugs. In review of systems, the patient complains of feeling dizzy and experienced a severe bout of malaise beginning around the time she began to lose weight. However, no signs of fever, pruritus, or erythema were noted. The patient was asked about any distinctive food choices or habits and she states that she plants and consumes her own vegetables in rural Grenada.\nUpon examination, the patient was an elderly female of the appropriately stated age; she appears apathetic and fatigued. The patient was malnourished and in mild distress with vital signs reading: blood pressure of 135/90 mmHg, a pulse of 110 bpm, a temperature of 99.8°F, and a respiratory rate of 18 breaths per minute. Lungs were clear to auscultation bilaterally and normal S1 and S2 sounds were heard with regular rate and rhythm. The abdomen was soft, nondistended, tender to light, and deep palpation in the umbilical area but no masses or organomegaly was felt. Bowel sounds were normoactive in all 4 quadrants and a surgical scar from umbilical hernia was noted. Patient's left inguinal surgical site was tender to touch with mild swelling and no drainage.\nThe abdominal X-ray and CT were ordered and suggested evidence of ileus and dilated stomach with small bowel distended up to 4.5 cm diameter without identifiable obstruction, respectively. No organomegaly or lymphadenopathy was noted. Upper GI follow-through was performed and impressions identified jejunal mural abnormality with thickening and loss of normal mucosal pattern, suggestive of broad region of infection or inflammation. Laboratory examination revealed an unremarkable absolute eosinophil count and blood percentage ().\nPatient presented with an elevated white blood cell count of 11,000/mm3 which rose to 16,000 mm3, following days of admission which is related to the infected surgical site (). Tumor markers for CA-19-9, CEA, and CA-125 were also measured and were within normal limits to rule out malignancy.\nThe patient was admitted for possible small bowel obstruction which was worked up with conservative treatment. Patient was placed on a regimen of nothing by mouth (NPO), lactated ringer 1000 mL intravenously at 125 mL/hr, NGT tube for intermittent decompression, and ketorolac 20 mg subcutaneously every 6 hours for pain. Another esophagogastroduodenoscopy was performed and the impression revealed duodenal mucosal thickening and diffuse redness. Biopsies were also taken: one sample from the antrum of the stomach and three samples from the duodenum which were submitted in formalin with the largest fragment measured up to 0.5 × 0.3 × 0.2 cm. Microscopic examination samples in the nonfundic type of gastric mucosal biopsy showed numerous parasites in the lumen of the glands (Figures and ). Parasite sections were noted in cross-sectional views and in other areas showing in long sections, which were identified as Strongyloides stercoralis. Eosinophils were identified in the infiltrate at 200x to 600x magnification, but a count was not taken. The diagnosis was confirmed with a stool ova and parasite, revealing rhabditiform larvae, which typically measure 200 to 300 microns by 10 to 20 microns []. |
A 34-year-old gentleman was first referred to orthopaedic services with a history of persistent knee pain, located over the right femoral condyle near the origin of the lateral collateral ligament. Eleven years prior to presentation, the patient was involved in a road traffic accident where he sustained a fracture of the right femoral neck and ipsilateral shaft. Initial management consisted of open reduction, fixation of the femoral neck fracture with cannulated screws and the ipsilateral shaft fracture with plating. The patient later had a revision of the plate to a femoral nail. Union was subsequently achieved with the femoral shaft fracture; however, a significant external rotation deformity was noted, and discomfort to the knee.\nThe patient was referred on to our tertiary referral orthopaedic centre for femoral de-rotation surgery. An initial CT scan performed revealed an external rotation deformity of 45 degrees. The patient procedure included removal of femoral nail, osteotomy with de-rotation surgery, and subsequent exchange nail. The femoral nail was fixed proximally and locked into place distally, following the corrective 45 degrees of internal rotation achieved to the distal femur during osteotomy, performed under intraoperative radiographic guidance. The post-operative rehabilitation regime consisted of initial touch weight bearing only, with gradual increments in weight bearing status. At six months follow-up, the patient complained of distal lateral femur pain. A repeat CT scan was performed, revealing a delayed femoral union. This was initially thought to be the cause of the patient’s symptoms. The patient was admitted for dynamization of the femoral nail with an injection of bone graft substitute, and iliac crest graft. Despite eventual union, the patient continued to complain of pain at the distal lateral femur, with a cracking sensation on movement now noted.\nRepeat clinical examination revealed a palpable crepitus over the distal iliotibial band with a snapping sensation, as it appeared to catch. A palpable small, solid swelling was noted at the posterolateral right knee. Ultrasound and CT revealed intra-articular loose bodies lying within the lateral para-patellar gutter. The patient subsequently underwent arthroscopy with removal of loose bodies. Despite this, at six-months follow-up to arthroscopy, the patient continued to complain of posterolateral knee pain. MRI showed no evidence of abnormality to the popliteus tendon or muscle, but highlighted the fabella embedded within the lateral head of gastrocnemius at the posterolateral corner of the knee. (Image ). The fabella was also notable on radiographs (Image ) and CT (Image ). Ultrasound scan confirmed the fabella as mobile on movement and associated with tenderness. The diagnosis of Fabella Syndrome was made, and the patient planned for a Fabellectomy procedure.\nAn initial arthroscopy was performed to review the knee joint, and to potentially assist in surgical excision of the fabella. A complete diagnostic arthroscopy was performed, with the fabella unable to be identified through the posterior capsule. After arthroscopy, open fabellectomy was performed. In view of the patient’s extensive scarring from prior orthopaedic procedures, a short 2cm incision was made directly over the palpable fabella (Image ). The common peroneal nerve was identified and spared. Subsequent incision left a cuff of biceps femoris to protect the nerve. Gastrocnemius was split and incised directly over the fabella, which was removed in entirety (Image ). Histopathology confirmed the diagnosis.\nThe patient was followed-up at two and ten months postoperatively. At both follow-ups, he described the complete resolution of his posterolateral knee pain. |
In March 2012, a healthy 50-year-old Caucasian male sustained a traumatic bifocal pertrochanteric and distal third shaft fracture of the left femur that was treated by another surgeon with a cephalomedullary long nail distally fixed with 2 screws. After 2 months, in May 2012, during the rehabilitation period, he suffered from the displacement of the fracture because of the loosening of the distal screws. He underwent a new operation by the same surgeon to reduce the displaced fracture and replace the distal screws. Six months after the last surgery, the fracture was still unconsolidated and he underwent pulsed electromagnetic fields and extracorporeal shockwaves therapy without success.\nOn February 2013, he referred to our department for persistent thigh pain and lameness that forced him to walk with a cane. The radiographic examination showed a hypertrophic nonunion of the distal femur (), refractory to the conservative treatment, which has been given an indication for surgical treatment. No limitation of the knee and hip range of movement was recorded. The limb was shortened to 3 cm.\nOur first surgical choice was the revision of the nail and the bone reconstruction with a corticoperiosteal pedicled flap from the medial femoral condyle.\nBefore the surgery, a CT angiography is performed to study the vascularization of the distal femur. The antibiotic preoperative profilaxis with gentamicin 160 mg (1 hour before the surgery) and cefazolin 2 g (just before the surgery) is administered.\nThe patient was placed in supine position with the hip and knee slightly flexed and externally rotated. A tourniquet is inflated at the proximal thigh. The removal of the intramedullary nail and screws was performed through the previous surgical access and the recipient site was prepared by debriding the bone, excising all scarred tissues and reaming of the femoral canal. A longitudinal incision was made on the medial side of the distal third of the thigh. The vastus medialis was reflected anteriorly, whereas the tendon of the adductor longus was retracted posteriorly, exposing the medial femoral condyle and its periosteal blood supply (). The dominant vessel was identified and dissected to its source. The design of the graft was outlined on the periosteum. The periosteum was cut with an electrocautery. Large vessels were ligated with hemoclips or absorbable suture threads. Then, using an osteotome or an oscillating saw, the outer cortex was cut and lifted, hammering slightly from the periphery towards the center (Figures and ).\nSimultaneously, the nail was removed from the femur bone and the recipient site was prepared by debriding the bone, excising all scarred tissues. The fracture was reduced and the osteosynthesis was performed by an anterograde intramedullary nail; the graft was overturned and wrapped around the nonunion and fixed with transbone stitches (). A radiograph was performed at the end of the procedure ().\nSubcutaneous enoxaparin sodium (4000 UI) was administered as soon as the recovery of full weight bearing took place. Wound inspection was done on the first and third postoperative days. Knee and hip mobilization, quadriceps exercises, and touch-toe weight bearing were started from the third postoperative day using a frame or crutches. The patient was discharged after 7 days from the surgery and stitch removal was done on the 15th postoperative day. The patient was followed at monthly intervals up to six months, then at three monthly intervals up to one year, and then every six months up to the last follow-up. Radiological assessment was done by taking serial X-rays as needed.\nProgressive to complete weight bearing was allowed on the 35th postoperative day after the appearance of callus on radiographs. The patient reached the complete weight bearing on the 60th postoperative day. We considered union to have occurred at 3.5 months of follow-up when radiologically anteroposterior and lateral radiographs showed bridging of at least three of the four bony cortices and clinically the patient was able to walk full weight bearing without any pain.\nAt the 20 months of follow-up, there was not recorded pain or excessive intoeing/out-toeing walking and the limb was shortened to 1.5 cm compared to the opposite leg. The radiographs showed complete union of the distal femur (). The patient was completely satisfied with the procedure. |
We present a case of a 65-year-old female patient admitted to the Cardiac Surgery Department in Wroclaw in January 2018 with severe mitral regurgitation (MR) and the history of ischemic heart disease, after elective percutaneous coronary intervention of the circumflex branch of left coronary artery with two drug-eluting stents (DES) implantation 4 years earlier. Furthermore, the patient diagnosed with many chronic conditions, such as metabolic syndrome, obesity with BMI 33 and gastroesophageal reflux disease. Currently, with an exercise dyspnoea for about 2 years, intensifying in recent weeks, she was hospitalized in the Cardiology Department for further diagnostics. The transthoracic echocardiography (TTE) revealed non dilated left ventricle with a normal systolic ejection fraction of 60%, and no evidence of segmental wall motion abnormalities, severe MR with the prolapse of the A2 segment and systolic restriction of the posterior leaflet. Colour Doppler showed a highly distinctive eccentric turbulent jet directed towards the lateral wall and the base of the left atrium with ERO 0.6cm2 and regurgitant volume of 60 ml. Additionally, in the performed coronary angiography, hemodynamically significant narrowing was found in the area of the previously implanted DES. The patient was then consulted by the cardiac surgeon and qualified for surgery. After admission to the Cardiac Surgery Department, as part of the pre-operative preparation, TTE was again performed, in which the severe MR was confirmed and no pathological structures in the right atrium were described. Due to the unclear image in the right pulmonary field, described by the radiologist in the chest X-ray (Fig. ), diagnostics was extended by performing a computed tomography of the chest, which excluded the presence of pathological shadow in the lung parenchyma. There was no referral to the atrial septum in the CT report. The patient was scheduled for mitral valve repair surgery and coronary artery bypass grafting (CABG) with the use of saphenous vein graft to the circumflex artery. During the standard procedure of commencing the cardiopulmonary bypass (CPB) and bicaval cannulation, it was found difficult to insert the cannulas from the atrium into both vena cavas. Therefore the cannulation was performed using the smaller cannula sizes, which eventually allowed to go on bypass. On the free wall of the atrial septum, there was a thickening and an excess of adipose tissue with a firm consistency and the size of a walnut, significantly impeding access to the operated mitral valve through the left atrium, and probably completely preventing surgery by the transseptal approach. In the transesophageal echocardiography (TEE), a characteristic image of LHAS was confirmed by the presence of hypertrophy of the septum, up to 2.7 cm, an hourglass shape with a characteristic indentation at the place of the fossa ovalis (Figs. and ). Based on the intra-operative TEE, as well as by analyzing the chest X-ray and CT scans, the diagnosis of LHAS was made. Due to the asymptomatic course of the LHAS and the complexity of the scheduled operation, the decision was made to leave the change intact. The mitral valve was replaced through the left atrial approach. The surgery was completed in a standard manner and the weaning from the CBP went uneventfully. The patient’s early postoperative period was a routine. |
A 58-year old Japanese male with type 2 DM and related end-stage chronic renal failure (CRF) was referred to our hospital for diagnosis and treatment of rhinosinusitis of undetermined etiology. The patient visited a local hospital complaining of headache, a depressed nasal bridge and nasal dryness with purulent rhinorrhea. He had a history of living renal transplantation for CRF. Removal of the grafted kidney had become necessary due to acute rejection one month before he became aware of sinonasal symptoms, and he had been treated by maintenance hemodialysis. Immunosuppressants started just after transplantation had already been discontinued by the day of his first visit.\nAt the onset, a computed tomography (CT) scan showed the partial fracture of septum and soft tissue density in the paranasal sinuses ( and ). The patient was initially suspected to have sinusitis, but the paranasal pyogenic lesion gradually progressed to development of nasal septum perforation, crust formation and saddle nose. Eventually necrotic tissue penetrated through the hard palate to the oral cavity after one month of therapy, and the lesion was considered to be a hidden tumor of extranodal natural killer/T cell lymphoma (ENKTL), nasal type. A biopsy of the necrotic tissue from the nasal cavity and hard palate had been performed at the previous hospital, but no diagnostic specimen was obtained. In the absence of definite histopathologic findings, the patient was suspected to have ENKTL, nasal type based on the macroscopic appearance and laboratory data. He was admitted to our hospital for further examination and treatment at three months after onset of rhinosinusitis.\nOn admission, the patient had a deteriorated condition and his Eastern Cooperative Oncology Group performance status was grade 3. Endoscopic examination revealed the perforation of nasal septum, large amounts of crusts in the nasal cavity (), and necrotic tissue on the hard to soft palate (). In addition, rotten bone appeared, when the crusts were removed. Blood tests revealed a normal leukocyte count of 6.20×109/L (normal range: 3.30–8.60×109/L) comprising 71.0% neutrophils (normal range: 39.8–70.5%) and 2.0% eosinophils (normal range:0.6–5.4%). Serological examination identified elevation of blood urea nitrogen to 79.1 mg/dL (normal range: 8.0–20.0 mg/dL) and creatine to 5.87 mg/dL (normal range: 0.65–1.07 mg/dL). Hemoglobin A1c (NGSP) was 5.9% (normal range: 4.9–6.0%). Soluble interleukin-2 receptor (sIL-2R) was markedly elevated to 3280 U/mL (normal range: 157–474 U/mL) and C-reactive protein was slightly elevated to 1.71 mg/dL (normal range: <0.20 mg/dL). β-D glucan was elevated to 18.1 pg/mL (normal: <11.0 pg/mL), while Aspergillus antigen, EBV-DNA and anti-neutrophil cytoplasmic antibody were negative. Nasal bacterial cultures failed to detect the pathogenic microorganism. Magnetic resonance imaging (MRI) showed destruction of the nasal structure with loss of the septum, and abnormal mucosal thickening and mucus retention in the sinuses, but no tumor was detected in the sinonasal region (). The destructive area did not reach the intracranial region, despite expansion and penetration towards the oral cavity. Repeated conventional biopsies of small tissue samples from the surface of sinus gangrene using biopsy forceps showed the presence of necrotic tissue with numerous fungi, including Candida species and Mucor, and Actinomycetes, which extended deep below the mucosa ( and ). However, it was difficult to conclude whether those microorganisms were truly pathogenic or merely contaminations, as the histologic finding lacked key-point features for the diagnosis of CIFRS, such as fungal invasion within the vessels or mucosal/vascular necrosis, perhaps due to massive tissue destruction by necrosis which caused the disappearance of findings for vascular invasion and mucosal/vascular necrosis. It was also difficult to make a definite differential diagnosis from malignant lymphoma, such as ENKTL, nasal type, due to the small tissue volume of the biopsied samples.\nAccordingly, we tentatively diagnosed CIFRS and started antifungal treatment with liposomal amphotericin B (L-AMB), while careful attention was paid to possible emergence of ENKTL. However, the gangrene still continued to expand towards the soft palate despite treatment with L-AMB for two weeks, and, indeed, CT examination showed no major improvement of sinus abnormal lesion ( and ). Therefore, we next performed endoscopic sinus surgery under general anesthesia with the aim of both removal of necrotic tissue and histopathologic examination. MRI after endoscopic surgery showed the removal of septum, but the residual soft tissue ( and ). The resected specimen showed extensive infiltration of Aspergillus niger in the background of necrotic tissue, but again without evidence of tumor tissue (–), based on which we made a final conclusive diagnosis of CIFRS, despite the absence of finding for fungal vessel invasion. Thereafter, the patient was treated with oral voriconazole and routine nasal cleaning as an outpatient, and his symptoms gradually improved. Follow-up CT scan ( and ) and MRI ( and ) showed no intracranial extension and the gradual improvement of the mucosal thickening, however, he unfortunately experienced a skin perforation of the nasal root after antifungal treatment for seven months, and an additional operation was performed to remove residual necrotic tissue on the palate and paranasal sinuses eight months after his first admission to our hospital. Presently, there is almost no necrotic tissue in his nasal cavity and paranasal sinus ( and ) and MRI shows only slight residual mucosal thickening ( and ). |
A 20-year-old Danish woman was referred with constant pain in the right proximal thigh that had progressed for 8 years. She had no other known medical conditions. Menarche was at age 13. There was no family history of sclerosing bone diseases. The limb pain was aggravated at night and was moderately relieved during physical activity and by cold baths. She was on daily analgesic medication with nonsteroidal anti-inflammatory drugs, paracetamol, and weak opioid agonists, and she was on sick leave from her university studies at the time of referral due to her symptoms. She was using shoe inserts because of leg length discrepancy.\nAt age 11 she had consulted a rheumatologist because of hip joint pain. Guided by ultrasound a hip joint puncture was performed due to fluid gathering; serology was negative. After the puncture, the joint pain ceased.\nSix months prior to referral to our clinic, she was involved in a traffic accident and hit by a car from the right at knee level. She was discharged from an emergency room (ER) with no suspected fracture and a radiological examination was not performed. She reported that there was significant worsening of the femoral pain after the traffic accident.\nA clinical examination revealed no skin lesions, café au lait spots, or redness; there was no increased temperature or swelling in her right thigh. Leg length was unequal but within normality.\nShe was instructed to fill out a diary with daily registration of pain on an analog scale from zero to 10, where increasing values were equivalent to more intensive pain. Orally administered analgesics were required when the pain exceeded a score of 6 or more, and the score 10 was reserved for symptoms that kept our patient from falling asleep and that did not respond adequately to orally administered analgesics.\nMagnetic resonance (MR) and positron emission tomography-computed tomography (PET-CT) scans revealed significant increased cortical thickness in the right femoral diaphysis that partially obliterated the medullary cavity (Fig. ). The surface of the sclerotic bone was uneven. A Tc99-bone scan showed a pathological increase in uptake in most of the right femoral shaft (Fig. ).\nBiochemical bone markers were normal, including normal alkaline phosphatase.\nScreening for the GNAS1 activating mutation R201 was negative in peripheral blood; as was mutation screening of the SQSTM1 gene, which has been reported to be mutated in Paget’s disease of bone.\nA bone biopsy was performed and showed thickened bone springs with preserved lamellar structure and slightly accentuated cement lines. There was no fibrosis, inflammation, or increased alcian positivity, which would be suggestive of active osteomyelitis. There were no signs of malignancy or neoplasia.\nA gynecological examination prior to referral had found a mild degree of polycystic ovaries (PCO) with normal androgen status. There were no endocrinopathies.\nDuring the clinical investigation, four tentative diagnoses were raised: atypical fibrous dysplasia, Paget’s disease of bone, chronic non-infectious osteomyelitis, and melorheostosis. The young age of our patient, normal alkaline phosphatase, and sparing of the metaphysis of the affected bone made Paget’s disease of bone highly unlikely.\nThe cortical thickening is atypical for the diagnosis of fibrous dysplasia. The absence of café au lait spots, the normal age at menarche, the absence of other endocrine disorders, and the negative GNAS1 mutation examination weighed against a diagnosis of fibrous dysplasia. The long bones are a common location for chronic non-infectious osteomyelitis and this can present in childhood; however, the radiological appearance and bone histology did not provide support for this differential diagnosis. With special emphasis on the uneven surface of the affected bone on X-ray (Fig. ), the diagnosis of melorheostosis, which was fully compatible with the relatively unremarkable histology, was found to be the most plausible diagnosis. In addition, the normal bone chemistry is in keeping with melorheostosis [] although in polyostotic cases alkaline phosphatase can be increased [].\nThe timeline for intervention and clinical outcome is presented in Fig. . Initially, zoledronic acid 5 mg was administered intravenously three times with 10-month and 12-month intervals; our patient had pain relief after the first administration, but a poor effect of the second and third injection. Using an analog pain scale she reported average pain of 9 prior to treatment, 6 after treatment with zoledronic acid, and 4 from 2 to 10 months after initiation of treatment with denosumab. Looking at the usage of pain medication, it went from daily dosage before treatment to 23% of days after treatment with zoledronic acid, and to no orally administered analgesics for 8 months following treatment with denosumab.\nIn October 2015, denosumab was chosen as the second line of treatment based on the hypothesis that the pain was caused by increased bone turnover. A dose of 60 mg was injected subcutaneously with some remission of pain that lasted for 6 weeks. At the same time as the pain relapsed, there was an increase in alkaline phosphatase, PINP, CTx, and osteocalcin in blood tests. Biochemistry before and during treatment is listed in Table . Based on these findings it was decided to shorten the interval between administrations to further strengthen control of a hypermetabolic state. The second dose of 60 mg was given after 8 weeks and this interval has been kept up to the present.\nBiochemistry after treatment with both zoledronic acid and denosumab showed suppression of bone-type basic phosphatase, osteocalcin, collagen I, and procollagen I, with a relative increase in parathyroid hormone, and plasma ionized calcium within the normal range. The injections with denosumab were well tolerated with no adverse effects reported. During the whole treatment period, the injections were administered from our out-patient clinic without cancellations.\nAfter the first three doses given 8 weeks apart, there was pain remission for 8 months; in this period our patient did not take any orally administered analgesics. At the latest follow-up in February 2018, she complained of a slight increase in pain and occasional use of paracetamol and nonsteroidal anti-inflammatory drugs.\nThe treatment with denosumab was well tolerated with no side effects observed.\nA Tc99-bone scan and computed tomography (CT) scan were performed in February 2017 and in February 2018, 5 and 17 months after the first injection with denosumab; the pathologically increased activity and cortical thickening were stationary. |
A 51-year-old female with a history of stage 1 hypertension was admitted to the hospital post cardiac arrest. She had a significant family history of premature coronary artery disease. Her father had a coronary artery disease diagnosed in his 40’s, her mother had a pacemaker inserted, and she had two first degree relatives diagnosed with an early cardiac disease. She is a nonsmoker, nonalcoholic drinker and denied recreational drug use. She was in her usual state of health and functionally active until her husband noticed her moaning and eventually unresponsive in the middle of the night. Prior to this, the patient did not complain of any chest pain, dyspnea, dizziness, lightheadedness or any other constitutional symptoms. Her husband started cardiopulmonary resuscitation and was taken over by the emergency medical staff (EMS). She was intubated and defibrillated five times by EMS for ventricular fibrillation then was given a bolus of amiodarone. She eventually had a return of spontaneous circulation and was transported to the hospital in which she was noted to have decorticate posturing with no purposeful movements. Electrocardiogram (ECG) during this time showed sinus rhythm (Figure ). She was admitted to the coronary care unit and was placed on targeted temperature management and was maintained on amiodarone drip. Transthoracic echocardiogram showed no valvular abnormalities, normal left atrium and left ventricular cavity size but with borderline concentric left ventricular hypertrophy with an ejection fraction of 56% and note of subtle regional wall motion abnormalities. About 12 hours after initiating the targeted temperature management, the patient was in severe bradycardia in the mid 30’s and a decision was made to hold the amiodarone at that point. The patient completed the targeted temperature management protocol and eventually had a full neurologic recovery thereafter.\nShe was extubated and subsequently transferred to the floor. She was scheduled for cardiac catheterization; however, she had witnessed ventricular fibrillation arrest on the floor (Figure ). Advanced cardiopulmonary resuscitation was initiated and she was revived immediately after defibrillation. A total of three minutes of resuscitation was performed and she was following commands afterwards. She was also given an amiodarone bolus and transferred back to coronary care unit. ECG did not show any ST elevations at this time. Subsequently, she had 23 more episodes of ventricular fibrillation arrest (Figure ) over a six-hour period which were intervened by timely defibrillation and administration of antiarrhythmic medications and inotropic agents. The antiarrhythmic medications administered were amiodarone and lidocaine boluses and drips. Intravenous magnesium was also given during the resuscitation course. Emergent cardiac catheterization showed minimal coronary artery disease. A transvenous pacemaker wire was placed due to bradycardia during a code in the catheterization laboratory, although the bradycardia did not initiate the arrest. An intra-aortic balloon pump was placed. Subsequently the balloon pump and temporary pacemaker wire were removed after she eventually became more hemodynamically stable. She once again had a full neurologic recovery and underwent an uneventful implantation of a dual-chamber implantable cardiac defibrillator (ICD). She was discharged from the hospital on amiodarone therapy and was closely followed up in the cardiology outpatient clinic. Cardiac magnetic resonance imaging was done which showed mild biventricular enlargement but with normal regional systolic function, no late gadolinium enhancement consistent with the absence of myocardial inflammation, infiltration or infarction and with no imaging features of ARVC. She eventually had a targeted genetic testing for idiopathic ventricular fibrillation and was found to have a mutation in the desmoglein-2 (DSG2) gene (c.338T > C, pVal1113Ala), which is a gene typically involved in ARVC.\nShe had been readmitted several times to the hospital due to palpitations, dizziness, lightheadedness, and defibrillator shocks. Her defibrillator had been interrogated as well as her antiarrhythmic medication had been adjusted which improved her symptoms and reduced defibrillator discharge. Because of intermittent episodes of defibrillator firing due to ventricular fibrillation despite antiarrhythmic medications and intracardiac device, a ventricular fibrillation radiofrequency catheter ablation was performed successfully. Thereafter, she had no repeated episodes of palpitations, dizziness, lightheadedness, and defibrillator shocks. |
A 59-year-old woman presented in July 2017 with extensive bleeding from her ileostomy site. Her history included locally advanced bladder cancer for which she had undergone pelvic exenteration and ileal conduit formation in November 2015. At that time, she had a known primary lung adenocarcinoma as well, but had no known liver metastases or other liver disease. Intravenous contrast-enhanced CT of the abdomen and pelvis performed in January 2016 raised the possibility of cirrhosis; however this was not biopsy-proven. In April 2016, she began to notice intermittent bleeding from her stoma which was initially thought to be mechanical tissue breakdown from the stomal flange. Concern for hepatic encephalopathy was raised when she had her first episode of confusion in December 2016. At that time CT of the abdomen and pelvis demonstrated strong radiographic suspicion for cirrhosis together with prominent vessels surrounding the urinary diversion site suspicious for portal hypertension. Despite not having a tissue biopsy, she was diagnosed clinically with cryptogenic cirrhosis in May 2017 during a hospitalization for fatigue, anasarca, and altered mental status. An upper endoscopy performed in June 2017 demonstrated portal hypertensive gastropathy but no esophageal varices.\nUpon presentation to the Emergency Department in July 2017 she had significant hemorrhage from her stoma resulting in hemodynamic instability. She was anemic with a hemoglobin of 8.3 g/dL that improved to 9.4 g/dL after blood transfusion, but gradually fell to 8.2 g/dL by the time of the procedure. Her MELD score was retrospectively calculated to be 19 at presentation, with an INR of 1.5 and total bilirubin of 4.3 mg/dl. She was emergently taken to the interventional suite for embolization with or without portal venous decompression via portosystemic shunt formation. A review of intravenous contrast-enhanced CT imaging showed extensive venous varices around the stoma involving the abdominal wall with a large draining varix arising from the portal system, likely the inferior mesenteric vein []. Also visualized was a variceal connection to the right common femoral vein. The portal and mesenteric veins were noted to be patent. Multiple approaches were considered for this patient. The transjugular intrahepatic portosystemic shunt (TIPS) and transjugular transhepatic approach with portosystemic shunt creation offered the benefit of portal decompression; however, the patient's recurrent hepatic encephalopathy was felt to be a relative contraindication. Transjugular transhepatic approach without formation of a permanent portosystemic shunt was also considered, since it would eliminate the risk of progressive hepatic encephalopathy. Percutaneous transhepatic approach would also eliminate the risk of progressive hepatic encephalopathy but was believed to pose increased risks of hepatic injury and bleeding. Transsplenic venous access to the portal venous system was considered as a viable, albeit technically challenging, option. The superficial nature of the abdominal wall stomal varix presented a less challenging and seemingly more time-efficient approach for access and was chosen as the target.\nUsing a micropuncture kit, the peristomal varix was directly accessed under ultrasound guidance and a micropuncture sheath was placed. Venography was performed and showed a large variceal collateral conglomerate around the stoma with variceal anastomosis with the right common femoral vein []. A wire was advanced and a 5F sheath was secured over the wire. A Kumpe catheter was introduced and advanced into the distal intra-abdominal aspect of the large draining varix. Catheter position was confirmed with repeat venography, and multiple coils were deployed []. This was followed by Gelfoam embolization. Postembolization venography showed sluggish flow in the draining varix with multiple filling defects within the visualized collaterals consistent with embolization []. The coils remained well-situated after placement and there was no evidence of migration. To ensure that there was no filling from the systemic venous system, the right superficial femoral vein was then accessed with a micropuncture kit and a femoral-iliac venogram and IVC venogram were both performed. These demonstrated brisk flow from the right common femoral vein through the iliac system and into the IVC. There was no filling of the stomal variceal collaterals visualized []. Hemostasis was thereby achieved, and the patient became hemodynamically stable shortly thereafter. At 6-month follow-up time no further imaging had been performed and the patient had not had any further episodes of hemorrhage from the ileostomy site |
A 67-year-old Japanese man gave a history of being diagnosed with a cystic lesion in the right third molar region of the mandible by X-rays 11 years earlier. Subsequently, a tooth extraction had been performed. However, cyst enucleation and histopathological examination had not been carried out at that time. Eight years after the tooth extraction, he noticed a gingival swelling which lasted for three years. The medical history was only prostatic hypertrophy. On examination, a slight swelling was palpable in the gingiva of the right third molar region of the mandible. There was no fistula but a part of the bone had a defect. A panoramic radiograph revealed a radiolucent cystic lesion, measuring 10 × 12 mm in the same area (Fig. a, yellow arrows). A computed tomography (CT) showed an unilocular radiolucent lesion along with cortical bone resorption of the mandible on the lingual side (Fig. b). On the basis of clinical and radiological findings, a presumptive diagnosis of an odontogenic cyst was made and a biopsy was performed (Fig. c). The incisional biopsy resulted in a diagnosis of central MEC arising from a GOC as described below. Chest and abdominal CT findings were within normal limits. A magnetic resonance imaging (MRI) revealed a contrast defect in the same area (Fig. d). Cervical lymph node metastasis was absent on MRI. Due to the malignant nature of the tumor, as well as a history of previous surgeries, a partial mandibulectomy was performed to remove the lesion with a sufficient surgical margin and the tumor was surgically excised under general anesthesia. Following a final diagnosis of central MEC, the patient made an uneventful recovery and demonstrated no clinical evidence of recurrence in the two years following the surgery.\nMicroscopic examination of the biopsy material showed an enlarged unilocular cyst (Fig. a). The cystic lumen was lined by epithelial cells and was surrounded by thick fibrous connective tissue. Additionally, a solid polycystic lesion was also seen on one side (Fig. a, black arrows). Numerous microcysts and mucous goblet cells were observed in the lining epithelium (Fig. b). The intraepithelial mucin in the mucous goblet cells was positive for mucicarmine staining (Fig. c). Eosinophilic cuboidal cells (Fig. d) and ciliated cells (Fig. e) were scattered within the non-keratinized squamous epithelial cells. These histopathological findings were suggestive of a GOC. In addition to the cyst wall consisting of fibrous connective tissue and the above-mentioned non-keratinized squamous epithelium coating the fibrous stroma (Fig. a, black square and 2f), the proliferation of many cystic nests containing mucous materials was observed in another part of the cyst wall (Fig. a, yellow square and 2 g). The lining epithelium inside several cysts consisted of a mixture of epidermoid, mucous, and intermediate cells (Fig. g). These findings served to confirm the diagnosis of central MEC arising from a GOC.\nWe evaluated the cytokeratin (CK) profile by immunostaining to compare the CK expression patterns between central MEC and GOC in the biopsy specimen. The lining epithelium comprising non-keratinized squamous cells in the GOC part (Fig. f) showed immunopositivity for CK 7 (Fig. a), CK13 (Fig. c), CK14 (Fig. e), CK18 (Fig. g), and CK19 (Fig. i). On the other hand, the central MEC part was positively stained for CK7 (Fig. b), CK14 (Fig. f), CK18 (Fig. h), and CK19 (Fig. j), whereas immunoreactivity for CK13 was not detected (Fig. d). In the final surgical specimen obtained after mandibular partial resection, the tumor with several cystic spaces could be seen to expand into the submucosal area under the gingival mucosa from the mandibular bone in the cut surface (Fig. a). The resected specimen contained only central MEC (Fig. b). The keratin immunohistochemical profiles of CKs were similar to those of central MEC in the biopsy specimen, which was not positive for CK13 (Fig. c) but was positive for CK18 (Fig. d). The histopathological examination of the final surgical specimen confirmed the presence of central MEC arising from a previous GOC after consideration of the histopathological findings of the biopsy specimen.\nWe sought to clarify the relationship of GOC to central MEC by performing MAML-2 molecular analysis of the lesion. Break-apart fluorescence in situ hybridization (FISH) for MAML-2 was performed. The component of central MEC in the biopsy specimen exhibited the MAML-2 rearrangement (Fig. a). In cystic areas of the GOC, the MAML-2-split was also present (Fig. b). Additionally, MAML-2 rearrangement was also detected in central MEC of the final surgical specimen (Fig. c). |
A 6-year-old girl came to our institution with a swelling over the left side of the face which had been increasing in size since 4 months. She had consulted a private dental surgeon six months earlier with a similar complaint of progressive swelling in the same region. Clinical examination, CT imaging of the lesion, and incisional biopsy were performed. The histopathological report then was suggestive of an aneurysmal bone cyst and curettage of the lesion was performed under general anesthesia.\nAt present the patient had a diffuse swelling over the left middle third of face []. The skin over the swelling was firm and mildly tender. Intraorally, the left side of the hard palate had a soft, nontender swelling of around 3 × 3 cms in size []. The upper left buccal sulcus was obliterated from the canine to second molar and egg shell crackling could be felt on palpation. No decayed or nonvital teeth were present.\nAspiration through buccal aspect yielded blood tinged fluid. All routine hematological and biochemical parameters were within normal limits.\nOrthopantamogram showed diffuse radiopacity in the left maxillary sinus region []. Computed tomography revealed a huge cystic mass occupying the left maxilla and the left alveolar process with expansion of the left maxillary sinus, scalloping and displacement of the hard palate on the left side, occlusion of the left nasal cavity [], and elevation of the floor of the left orbit [].\nBased on the past history, current clinical presentation, imaging findings, and the earlier histopathological report, a provisional diagnosis of recurrent aneurysmal bone cyst was made and an incisional biopsy was performed under local anesthesia.\nOn histopathological examination, the excised tissue showed fibrovascular connective tissue with dense collagen fibres interspersed by numerous actively proliferating fibroblasts. Some areas showed parallel arrangement of collagen fibres forming a fibrous capsule. Beneath this layer, areas of immature bony trabeculae with osteoblastic rimming and osteoid formation were seen []. Some areas showed cholesterol clefts with multinucleated giant cells. In the periphery of the lesional tissue, areas of dystrophic calcification were seen. In addition, sections from the central areas of the lesion showed blood filled spaces of varying sizes [] and giant cells []. These features were collectively suggestive of trabecular variant of juvenile ossifying fibroma with secondary aneurysmal bone cyst like changes. Due to the rapid growth and recurrent nature of the lesion, surgical resection was performed under general anesthesia. The patient recovered well and has been asymptomatic since 1 year. |
A 30-year-old man presented to the emergency department after “falling into a hole”. The patient was a construction worker and fell with his back in a 2-meter hole which was followed by a blunt abdominal trauma due to a falling of a 30 kg bag of cement on his abdomen. The patient had no past medical history and was not on any treatment or drug abuse. On primary survey, the airway was intact and a hard collar had been put on. The breathing, lung auscultation, and O2 saturation were in normal range. The blood pressure was 128/75 mmHg, pulse rate 90/min, and oxygen saturation of 96% while the patient was breathing ambient air. The patient underwent focused abdominal sonography for trauma patients (FAST) which showed small free fluid on lower pole of the right kidney. The patient was awake and fully conscious, the pupils were normal size and reactive, and there was not any neurologic deficit.\nThe secondary survey was significant for the mild abdominal tenderness on right upper quadrant. The chest, and lateral neck portable x-rays were normal. After surgery consultation, it was planned to have an abdominopelvic computed tomography (CT) scan with intravenous contrast agent in order to pin point the problem. In few minutes, the pain had been increased in intensity which was claimed to be 7 out of 10 in numerical rating scale (NRS). The blood pressure did not show any significant change but the patient pulse rate was increased up to 110. Serial FAST exam showed increase in free fluid volume. The patient underwent CT imaging (). It showed a moderate volume of free fluid in pelvic area, perihepatic, and also perisplenic spaces. Small bowel wall thickening due to mural hematoma of proximal part of jejunum with mural wall hypodensity in mid jejunal loop which are all suggestive of hollow viscus injury. There was no visible sign of solid organ damage or extra luminal free air. Regarding to the CT scan results, laparoscopy was performed. There was damage to the colon serosa and jejunal perforation which was primarily repaired. The patient was discharged 10 days later and had uneventful follow up. |
A 76-year-old lady was involved in a road traffic collision, after being dazzled by the sun, where she crashed into a parked vehicle and rolled her car twice. She was taken to her nearest emergency department and assessed following advanced trauma life support protocols. She was haemodynamically normal and had no neurological deficit. She complained of neck and left shoulder pain. Due to the mechanism of her injury and her complaint of neck pain, a CT scan of her cervical spine was performed along with an x-ray of her left shoulder.\nThe left shoulder radiograph showed no abnormality. The sagittal images of the cervical spine CT scan are shown in and and . is the mid-sagittal slice of the CT scan at presentation and shows no obvious malalignment.\nThere is an acute fracture of the antero-superior osteophyte of the body of C7 (). There is also opening of the cervical facet at C6/7 (). There were no fractures of the vertebral bodies. It can also be appreciated that there is significant degenerative change between C4 and C7.\nOn the basis of these imaging findings, the patient was reassured that no injury had been identified. Due to the COVID-19 pandemic, the patient was discharged home after this and no further follow up was arranged to ensure that both the patient and the hospital were protected from additional risk associated with COVID-19.\nTwo weeks later the patient was reviewed at home by her general practitioner due to ongoing neck pain, left shoulder pain and subjective numbness and weakness in her left arm. Upon assessment, it was noted that she held her neck in a flexed position and was unable to straighten the neck. The general practitioner arranged for an urgent review by the orthopaedic team in the nearest hospital. On assessment they recognized the potential for a significant injury and arranged an x-ray and a MRI of the cervical spine. shows the lateral cervical spine radiograph demonstrating an anterior subluxation of the C6 vertebrae on C7 and significant malalignment between the spinous processes of C6 and C7.\nThe MRI scan shows that the dislocation had reduced. demonstrates high signal in the C6/C7 intervertebral disc.\nalso demonstrates the injury to the posterior ligament complex at C4 to C7.\nThe case was discussed with the regional spinal surgery unit and subsequently urgently transferred. The case represented a missed unstable cervical spinal injury. Stabilization of the injury was performed with a two-stage procedure. First, an anterior\napproach was performed to reconstruct the anterior column using an anterior cervical discectomy and fusion through the C6/7 disc space. This was performed with a CeSPACE cage (B Braun, Hessen, Germany) and a Venture anterior cervical locking plate (Medtronic, Minneapolis, MN, USA). The patient was then turned prone and a posterior approach to the spine was performed. An instrumented fusion was performed using lateral mass screws in C4, C5 and C6 and pedicle screws in T1 bilaterally using Mountaineer (Depuy Synthes, Raynham, MA, USA). Grafton bone graft substitute (Medtronic, Minneapolis, MN, USA) was used in both the anterior and posterior stages.\nShe made a good post-operative recovery, and the upper limb neurological symptoms resolved. Post-operative radiographs are shown in . |
A 73-year-old male commercial driver was involved in a motor vehicle accident with significant facial trauma. Out in the field, the patient was hypotensive and tachycardic (systolic blood pressure, 80 mm Hg; heart rate, 128), with an estimated hemorrhage of 1,000 mL. The patient was transported to our institution and bled an additional 100 mL in the initial 10 minutes of assessment. Physical examination revealed a left zygomaticomaxillary complex fracture injury with significant bleeding in the nasal and oral cavities, and the patient was actively resuscitated with fluids. Packing of the nasal and oral cavity was not effective in controlling the bleeding, and the patient was intubated to protect the airway.\nAfter the initial fluid resuscitation with 2 liters of normal saline, the patient continued to have unstable vital signs and was transfused with 2 units of packed red cells, along with an additional liter of fluid. Despite vigorous resuscitation, the systolic pressure dropped further to 50 mm Hg. The initial laboratory study showed a hemoglobin level of 9.7. At this point, the patient's past medical history was not available, but the patient had been taking warfarin with a history of stroke three years prior to this.\nComputed tomography scan revealed a left zygomaticomaxillary complex fracture (). The patient was taken to the operating room for emergency reduction of the fracture with the primary goal of obtaining hemostasis. Upon exploration of the open wound in the oropharynx, the major source of bleeding was seen to originate from the left sphenopalatine fissure. During the 2-hour operation, the patient received an additional 3 units of packed red blood cells. Reduction of the facial skeleton was not sufficient in controlling the hemorrhage, and the patient was taken to the angiography suite. Angiogram of the external carotid artery revealed brisk extravasation of contrast from branches of the left sphenopalatine artery (). With embolization of this artery, the bleeding finally stopped, and the patient became hemodynamically stable without the need for further transfusion (). The patient was subsequently transported to the intensive care unit and recovered well enough to be extubated and transferred to the ward after 3 days. The patient was discharged home 3 months after the injury and returned to full employment. |
A 48-year-old woman, gravida 1, para 1, visited the internal medicine department at another hospital with a complaint of abdominal fullness and weight loss of 10kg during the last year. A huge abdominal mass was palpated, and she was referred to the gynecology department to search for a tumor of uterine origin. She was premenopausal and had no significant past medical history. Physical findings revealed a large elastic hard mass extending from the xiphoid to the pubic bone. The magnetic resonance imaging (MRI) examination revealed a huge tumor on the uterine corpus, and a number of dilated vessels were observed between the tumor and the myometrium. Therefore, the tumor was suspected to derive from the uterus. The tumor showed an uneven signal on T2-weighted sagittal section (), and the enhanced MRI study showed that the tumor edge but not the center was enhanced (). As such, necrosis was suspected to have occurred in the center of the tumor. Uterine sarcoma was primarily suspected due to the large size, degeneration, and necrosis on MRI imaging. Computed tomography (CT) examination showed no lymph node swelling or distant metastasis. Preoperative laboratory testing revealed anemia (hemoglobin level, 5.6g/dl). We transfused 18 units of RCC before surgery. CT examination and ultrasonography on lower extremities indicated an absence of thrombosis. Preoperative serum levels of CEA, CA 19-9, CA 125, and LDH were within normal limits. A biopsy of the endometrium was not collected as the sounding examination of the endometrium was unsuccessful due to a deviated uterine cervix. At this point, preoperatively, we suspected the tumor was a leiomyosarcoma or leiomyoma with degeneration.\nThe patient underwent laparotomy, where we identified a huge tumor occupying a space from the pelvis to the diaphragm. The tumor surface was smooth and hard with many dilated veins (). A massive tumor with a diameter of 30 cm was observed arising from the posterior uterine wall with a smooth contour and invaded the retroperitoneal cavity under the mesentery. The tumor was firmly adhered to both the mesentery and right ovary. There were no findings of extra-uterine dissemination. The intraoperative frozen section report for the uterine tumor was of degenerated myoma with no findings indicating malignancy. A total abdominal hysterectomy (TAH) and right salpingo-oophorectomy (RSO) were performed. The operation duration and blood loss were approximately 216 minutes and 1000 ml, respectively. The excised specimen weighed 7600 g.\nMacroscopic findings of the tumor revealed a well-circumscribed tumor showing extensive continuity with the posterior wall of the uterus, measuring 28 × 23 cm (). On the sliced surface of the tumor, an obvious heterogeneous pattern was recognized within the mixture of a whitish homogeneous area, suggesting benign uterine fibroids, and a vulnerable area, due to bleeding and necrosis ().\nFor the intraoperative frozen section, we examined three areas, namely, a white homogenous part, a necrotic part, and a cystic part, of which all were findings of a leiomyoma. In the permanent histological examination, 10 additional sections were collected from the tumor. The basic histological findings of all the sections were the same. The tumor was comprised of spindle-shaped cells, homologous to smooth muscle cells, which were arranged in bundles with areas of hyalinization, consistent with a degenerated leiomyoma. The tumor was mostly comprised of degenerated uterine leiomyoma. However, enlarged blood vessels were observed within an area of approximately 2 cm × several mm, and proliferation of atypical cells showing a fine meshwork microvascular structure was observed in the blood vessel cavity (). These atypical cells consisted of various contours, such as cubic, polygonal, and short spindle shape. The nucleus was circular with a high degree of vacuolar enlargement and pleomorphism. Abnormal mitotic figures were also interspersed (). A tumor derived from a blood vessel was thus considered, and malignancy was suggested by the presence of nuclear atypia and abnormal mitosis.\nImmunohistochemical analysis revealed the atypical tumor cells to be positive for ERG, CD31, and AE1/3 (Figures and ), partially positive for Factor VIII, and negative for α-SMA, desmin, H-caldesmon, EMA, CD34, and D2-40. From the above, the atypical tumor cells were of epithelial origin and the final diagnosis was epithelioid angiosarcoma arising in a degenerated uterine leiomyoma.\nThe efficacy of postoperative adjuvant therapy for angiosarcoma has not been demonstrated and there is currently no established chemotherapy regimen. In this case, because the atypical tumor was observed in the blood vessel cavity, we thought it could have been spread hematogenously throughout the body. Hence, we selected adjuvant chemotherapy rather than adjuvant radiotherapy. Six courses of combination adjuvant chemotherapy with paclitaxel (150mg/m2), epirubicin (50mg/m2), and carboplatin (area under the curve = 4) were administered in the present case, following referral to previous reported cases. No recurrence has been observed 10 months after the primary surgery. |
A fourteen-year-old girl presented to her primary care physician with fever, photophobia, nausea, vomiting, meningeal signs, and loss of consciousness nine months after a motor vehicle accident that had resulted in frontal sinus fractures. She was treated empirically with ceftriaxone for presumed bacterial meningitis (her family refused lumbar puncture), and with acyclovir for documented herpes simplex infection in her oral cavity. Because of continuing intermittent CSF leak, she was transferred to Children's Hospital and Regional Medical Center. Computer tomography (CT) scan of her head showed sinusitis, bilateral dehiscence of the posterior frontal sinuses at the level of the anterior cranial fossa, and herniation of the frontal lobes into the frontal sinus skull defects. She subsequently underwent surgical debridement of the sinuses, elevation of old frontal sinus fractures, and closure of the dura with concurrent lumbar drain placement.\nA pruritic fine popular rash involving her arms and thighs, and gradually spreading to her face, complicated her perioperative course. The rash progressed into a confluent malar eruption that was thought to be related to ceftriaxone and prompted several changes in her antimicrobial regimen. Following her operation, her fevers and rash resolved, her headache improved, and her CT scan was reassuring. Yet, after removal of the lumbar drain, she developed severe postural headache accompanied by nausea, vomiting, and refusal to ambulate. Her CT scan showed bifrontal extradural CSF collection (). The pain service was requested to place an EBP.\nOur assessment revealed an otherwise healthy afebrile young teenage girl in moderate distress and a normal neurological exam. She had no signs of generalized or local infections, her coagulation studies were normal, and all her blood and CSF cultures since her initial presentation were negative. She was still receiving cefuroxime to finish her initial course of antibiotics.\nAfter informed consent was obtained, the patient was taken to the operating room and anesthetized. Under sterile conditions an EBP was performed one interspace above the lumbar drain insertion site using 15 mL (0.3 mL/kg) blood. When the patient recovered from anesthesia, she complained of mild backache, but stated that her headache had resolved.\nAbout 18 hours later, on a routine postprocedure visit the patient was noted to be flushed and febrile (maximum temperature 38.8°C). She had no headache at rest or with activity. She was able to ambulate for the first time in two weeks. The patient reported no nausea, vomiting, or meningeal signs. Her neurological exam and her complete blood count and differential were normal. Her head CT showed significant decrease of the size of the bifrontal extradural CSF collection (). After concerns were raised about the therapeutic intervention, discussion with the neurosurgical team followed, and expectant management with serial neurological checks was started. About three hours later, the patient reported pruritus on her arms and legs, and she was noted to have a fine maculopapular rash, very similar to the one she had shortly after admission. Review of her chart revealed that after the EBP her antibiotic was changed from cefuroxime to ceftriaxone by the resident on call to allow less frequent dosing. After discontinuation of the ceftriaxone and diphenhydramine administration, her rash improved and her fever resolved. Five days later she was discharged from the hospital and on followup two months later she continued to be healthy. |
A 9-month-old female infant with a bodyweight of 8.3 kg was admitted to our cardiac intensive care unit (CICU) due to failure to wean from invasive mechanical ventilation for respiratory failure. The patient was the second child of a young couple, without a history of consanguinity or a family history of vascular abnormalities. The fetal ultrasound at 35 weeks and 2 days of gestation revealed LPA arising from the RPA resulting in a PAS. Prenatal laboratory investigations were unremarkable including a normal amniotic fluid genetic microarray. Due to the prenatal diagnosis of PAS, the baby was delivered by cesarean section at 39 weeks of gestation with a birth weight of 3,000 g and normal Apgar scores.\nA neonatal transthoracic echocardiogram further confirmed the diagnosis of PAS without additional intracardiac anomalies. Since the baby was initially asymptomatic with normal clinical examination, no imaging modalities were performed, merely periodic outpatient follow-up being arranged in the local hospital. The patient was referred to our hospital for developing recurrent wheezing and progressive respiratory distress following acute respiratory infections since 7 months of age. There was no vomiting, feeding issues, and recurrent respiratory infections. To better delineate the anatomy of the PAS and to evaluate the airway, a computed tomography angiography (CTA) was undergone at 8 months of age, demonstrating that the LPA had its origin from RPA and coursed between the esophagus and trachea to the left lung hilum (shown in ). Despite segmental stenosis of proximal LPA was observed with the smallest inner diameter of 1.7 mm, no marked tracheal stenosis and complete tracheal cartilage rings were identified. Subsequently, the patient was hospitalized. Physical examination revealed moderate distress (nasal flaring and subcostal retractions) and tachypnea with respiratory rates of 56 breaths/min in the absence of dysmorphic features, heart murmurs, or extremity edema. Given the poor response to medical therapy, the patient was finally referred for surgical correction of the PAS by reimplantation of the reconstructed LPA to the left side of the main pulmonary artery (MPA) under cardiopulmonary bypass, but without tracheoplasty.\nDespite being hemodynamically stable under mechanical ventilation, the postoperative course of the patient was complicated with airway obstruction and hypercapnia. Repeat chest CT (three-dimensional reconstruction) on postoperative day 15 showed two bi-lobed lungs and anomalous lateralizing features of the right main bronchus (RMB), characterized by a more horizontal course and a long main bronchus. Additionally, it was noted that the trachea bifurcation occurred more distally at the level of T6 rather than T4-5, with the lumen diameter of 2.0 mm at the most stenotic part of the trachea. Moreover, several consolidative opacities in the inferior lobes of both lungs were found (shown in ). However, no features of cardiac heterotaxy and spleen anomalies were found in the subsequent ultrasound scan. The patient experienced three unsuccessful extubation within 18 days in one of which cardiopulmonary resuscitation was performed because of cardiac arrest. Thereafter, the patient was transferred to the CICU in our hospital under invasive respiratory support.\nOn CICU admission, the patient was sedated with sulfentanil (0.04 μg kg−1 h−1) and midazolam (8 μg kg−1 min−1) continuous infusion. SIMV mode of mechanical ventilation was employed with a peak airway pressure (Ppeak) of 22 cm H2O, a positive end-expiratory pressure (PEEP) of 7 cm H2O, a respiratory rate of 25 breaths, and an inspired oxygen fraction of 40%. The patient was hemodynamically stable without inotropic support. Her body temperature was 38.4°C. Clinical assessment reflected normal first heart sound, normal thoracic expansion, no hypotension (BP: 87/55 mmHg), and no hypoxia (SaO2: 95% on ventilation). Except for rales on pulmonary auscultation, the rest of her physical examination was unremarkable. Initial laboratory investigations revealed increased leukocytes (18,100/μm) with 74.5% neutrophils and 5.4% lymphocytes, normal hemoglobin (11.7 g/dl), normal platelets (17.1 × 104/μl), and elevated C-reactive protein (1.88 mg/dl). Liver/renal function, procalcitonin, myocardial troponin I, and electrolytes were normal. Results of respiratory culture (tracheal aspirates), chlamydia/mycoplasma antibody, PPD test, T-spot assay, serum glucan/galactomannan tests, and blood culture were negative. Arterial blood gas reported hypercapnia (pH 7.31, PaO2 96.2 mmHg, PaCO2 72 mmHg, and HCO3 22.3 mmol/L). Serial ultrasounds of the brain and heart were unremarkable, except for a pressure gradient of 16 mmHg across the anastomosis between the LPA and MPA. The chest X-ray revealed multifocal overinflation and increased opacities in the right infrahilar region, in part reflecting obstructive emphysema and atelectasis.\nImipenem (120 mg kg−1 d−1 divided every 6 h) and immunoglobulin (500 mg kg−1, single intravenous infusion) were given. To open the lung and prevent lung unit collapse, the patient was mechanically ventilated on SIMV mode, receiving a PEEP of 5–8 cm H2O with a tidal volume of 6–8 ml/kg. Three days later, the fever resolved, with an improvement of respiratory status. The patient was prepared for extubation with less sedation and a switch of ventilation mode from SIMV to PSV. However, a progressively increasing work of breathing was noted with noisy breathing and dramatical prolongation of the expiratory phase, a sign of airway obstruction and malacia. Ventilator-patient dyssynchrony was uncontrolled despite the adjustment of ventilatory parameters. A series of targeted tests were conducted to explore the underlying etiologies of ventilator dependence. First, systemic infections were not considered due to the absence of fever and negative results of blood routine, C-reactive protein, and procalcitonin. Second, new onset of lung infection, pulmonary edema, and plural effusion were excluded by a repeat chest radiography. Finally, unremarkable findings of ultrasounds did not support the diagnosis of pericardial effusion, low cardiac output syndrome, residual cardiac anomalies, intracranial hemorrhage/hypertension, and diaphragmatic weakness. Ultimately, a decision was made to assess the tracheal anatomy under general anesthesia. After preoperative preparation and anesthesia, electronic fiberoptic bronchoscopy was performed at the bedside of the patient on postoperative day 22. Complete tracheal cartilage rings were found from 1.5 cm below the glottis to 1.0 cm above the carina, with the stenotic length of 5.5 and 1.0 cm at the upper and lower stenotic segment, respectively (O-rings with the smallest inner diameter of 3.6 mm). Tracheal bifurcation occurred distally at the level of T6 with an inverted T-shape. No stenosis was seen in the left main bronchus (LMB); however, a long, stenotic, and collapsed RMB with only two distal bronchus orifices were observed (absence of a separate right middle lobe bronchus). Notably, multiple additional areas of variable narrowing in the subsegmental bronchi in both lungs were found. Additionally, extensive mucosal swelling with surrounding hyperemia and sticky secretions was prominent as well (shown in ). The assessment of tracheobronchomalacia was not available through the bronchoscopy, since spontaneous respiration was blocked by general anesthesia. Following bronchial washings, tests of the lavage fluid were performed, showing negative results for bacteria, fungi, viruses, protozoa, and malignant cells.\nThese bronchoscopy findings were consistent with a diagnosis of type IIb PAS. Tracheoplasty was not performed, due to the economic factor and the possible related complications. Under conscious sedation, she was managed conservatively by effective clearance of airways, massive physiotherapy, and nebulizer therapy (albuterol, budesonide, and epinephrine). In addition, methylprednisolone (3 mg kg−1 d−1) was given intravenously for 3 consecutive days prior to extubation. The sedatives were gradually tapered and discontinued. Fortunately, the patient was successfully extubated on postoperative day 34. While the respiratory condition of this case continued to improve following non-invasive respiratory support, low muscle tone and strength in the extremities were identified later, indicating neurological dysfunction probably related to hypoxic injury postoperatively. Trajectories of hypotonia over several weeks followed by generalized hypertonia were highly predictive of persistent neurological sequelae. MRI was performed to evaluate brain lesions. Symmetrical abnormal signals were widely detected throughout the gray matter, white matter structures, and deep nuclei in association with extensive cortical necrosis, cerebrum atrophy, and ventriculomegaly (shown in ). Finally, the parents of the patient abandoned treatment due to economic factors and took the baby home. summarized the timeline with relevant data from the episode of care in this case. |
A 61 year-old male was admitted for constipation and a palpable mass in the periumbilical area. His medical history revealed the surgery for acute perforative appendicitis 20 years ago and occasional intestinal obstruction afterwards. He denied any history of radiation or occupational exposure to chemicals. The small bowel series showed an external compressive lesion in the distal ileum and the computerized tomographic (CT) scan revealed a 6 cm, well-marginated mass in the anteromedial aspect of the ascending colon (Figure ). Under the suspicion of foreign body granuloma due to gauze (gossypibioma), an exploratory laparotomy was performed. The whole intestine was found to be covered in severe adhesion; however, ascites or blood in the abdominal cavity was absent. In the anteromedial aspect of ascending colon, there was an encapsulated mass 5 cm in size, and it was adhered to ascending colon and distal ileum (Figure ). The mass was filled with abscess and granulation tissue, and the frozen biopsy reported the diagnosis of abscess. Only resection of the mass was performed, and the patient was discharged without any complications.\nForty days after the discharge, he was admitted again with anemia, abdominal distension, and melana. The colonoscopy revealed the hematoma and the stenosis of the lumen directly above the cecum. The abdominal CT scan showed large volume of blood in the abdomen and multiple peritoneal nodules. The angiography and 99 mTc labeled RBC scan showed active bleeding around the ileocecal valve. Emergent laparotomy was performed and multiple nodules were found on the wall of ileum, liver, mesentery and peritoneum. In the distal ileum, two nodular lesions with 3 cm and 2 cm in size were bleeding actively, and they were adhered to each other. The nodules were diagnosed as sarcoma through the frozen biopsy, and the distal ileum including the pathologic lesion was resected. Seven days later, reoperation was performed due to substantial hemorrhage from the peritoneum, the mesentery, and the small intestine wall. Two days after the last surgery, the patient expired of uncontrollable bleeding due to disseminated intravascular coagulopathy.\nOn the macroscopic examination of resected ileum, there were two ill-defined tan solid tumors with mucosal ulceration, each measured 3 × 1.5 cm and 2 cm in diameter, involving the entire intestinal wall and extending to the subserosa of adhered loop. (Figure ).\nUnder microscope, spindle-shaped or epithelioid cells were arranged as a plate and the rudimentary vessel lumen were detected occasionally (Figure ). Separated from these two lesions, several small angiosarcomas containing foreign body granulomas were found in the subserosal layer of the intestine. Also, the nodules of liver and mesentery were diagnosed as metastatic angiosarcomas.\nImmunohistochemical staining was performed and tumor cells were positive for CD31, CD34, and vimentin (Figure ), whereas negative for factor antigen, CD117, and S-100. The tumor cells were also negative for cytokeratin (AE1/3) and EMA. The foreign body granulomas were surrounded by CD31 positive cells, partially or entirely.\nThe previously resected mass, which was diagnosed as abscess, was reviewed. In a low magnification field, abscess in the center and fibrosis with vascular proliferation in the periphery were noted (Figure ). However, when the periphery was examined under the high magnification, spindle-shaped or epithelioid cells were arranged as plate patterns, in some area, well-differentiated vessels were formed as similar to the lesions from the ileum. The additional serial sections revealed more foreign materials surrounded by epithelioid tumor cells and the invasion of tumor cells to the blood vessel. The tumor cells were positive for CD31 and CD34, and negative for cytokeratin. This supported the final diagnosis of a foreign body granuloma associated-angiosarcoma. |
A 75-year-old man was referred to our Saitama Medical University from a local hospital, where he had presented with gait disturbance and a computed tomography (CT) scan had demonstrated an extra-axial mass, suspected to be a calcified subdural or epidural hematoma. Three months previously, he had suffered a fall in the bathroom without any head trauma, and also had a history of knee joint inflammation. On admission, the findings of detailed neurological examinations were essentially normal, but the patient was unable to walk because of knee pain. A CT scan demonstrated a right frontal mass that compressed the frontal lobe, and calcifications at the border between the mass and the cortex (\n). There were erosions of the skull overlying the mass, as well as elsewhere on the cranial vault (\n). Myeloma with skull involvement was strongly suspected. Initial laboratory examinations revealed elevated serum levels of immunoglobulin G (4435 mg/dL) and immunoglobulin lambda chain (292.0 mg/L). Urinalysis was positive for Bence Jones protein (BJP). The tumor was well enhanced by gadolinium contrast, and a flow void with linear enhancement suggestive of large vessels within the tumor was demonstrated by magnetic resonance imaging (MRI) on admission. Some small skull lesions were also present (\nand\n). Based on a preoperative diagnosis of myeloma skull invasion, biopsy of the tumor was performed in the area of the cortical bone defect. The tumor was hemorrhagic, but the bleeding was well controlled by bipolar coagulation and compression by cottonoid, since the surgery involved a limited field. Pathological examination of frozen and permanent sections confirmed the diagnosis of myeloma cell invasion (\n). Local irradiation was started, and the patient was transferred to the Department to Hematology. After 37.5 Gy of irradiation in 15 fractions, the tumor showed marked shrinkage. During the irradiation, dexamethasone (20 mg × 4 days) was also administered. A systemic bone survey revealed erosion of the clavicle accompanied by pain, and therefore local irradiation of this lesion was also performed. After irradiation, bortezomib chemotherapy was started, but this led to disseminated intravascular coagulation and pneumonia as adverse events. Despite intensive treatment, the patient died 70 days after biopsy of the skull lesion. Autopsy was not performed. |
Patient 1 (P1), a 13-year old female with physician-diagnosed severe milk allergy began TCM in February 11, 2011 with the goal of preventing reactions while continuing on a dairy restricted diet.\nThis patient was diagnosed with milk allergy at 3 months of age after an ER visit due to anaphylaxis (widespread hives, vomiting and difficulty breathing) immediately following ingestion of cow milk based formula. She subsequently experienced numerous reactions; and the frequency and severity of these reactions worsened over time. Despite a dairy restricted diet, during the 2-year period prior to starting TCM she experienced >100 reactions (at times a reaction every few days, averaging 50 reactions/year). Fifty events required prednisone and epinephrine administration, 40 required ER visits, and 3 resulted in intensive care unit (ICU) admissions. In the three months before TCM, her reactions became even more severe; 5 of 10 events required epinephrine and ER visits. These reactions were characterized by hives, rash, lip swelling, throat tightness, wheezing, stomach pain, headache, weakness, dizziness, syncope, hypoxia, and drop in blood pressure. Her overall severity score during the 3-month period before beginning TCM was 29 (Figures and ). Her reactions were triggered by inhalation and contact in addition to accidental ingestion of trace amounts of dairy products. Anaphylaxis events were so frequent and severe that she was unable to attend school in the 4 months prior to her first TCM visit. She also complained of chronic stomach discomfort. Her milk protein specific IgE levels were elevated at 37.3 kU/L. She did not undergo milk protein skin testing because her history of severe skin contact induced reactions, raised concern that severe reactions might occur.\nThis patient lives in another state that outside New York state. The impact of these reactions on quality of life is illustrated by the fact that the patient’s father drove for three days to bring her to New York City for the first TCM visit because of fear of a possible reaction to trace amounts of milk protein while on an airliner. She was prescribed all 4 TCM remedies: Remedy A, started with 1 pill, gradually increasing to 5 pills, 10 pills and 12 pills, twice daily (b.i.d), and then three times daily (t.i.d.). 12 capsules t.i.d was the full dose for patients 12 and above based on the manufacturing extraction yield of raw herbs (Additional file : Herbal constituents and doses). The protocol to gradually increase the dose was to ensure the safety and tolerability. Remedy B, 3 pills, b.i.d.; Remedy C (bath additive) 2 packs per bath once a day (q.d.), and Remedy D (cream) applied to her entire body, including the face, q.d.. Since her reactions often began with throat tightness or pain, Fructus Arctii Lappae, which has been traditionally used for throat ailments [], was added during the treatment course. Acupuncture was also performed at each visit. She tolerated the medicine regimen very well and after approximately 3 months no longer complained of stomach discomfort that she used to have before TCM. Follow-up visits took place every 3–6 months, a total 8 visits. Her FSFA slightly improved after 6 months of TCM and greatly improved after 1.5 years of TCM, and she was able to resume school. After 2 years of treatment, the number of reactions was reduced by 90%. None required epinephrine, or ER visit. Symptom severity was reduced by 56%, based on total scores (Figures and ). She was able to work a summer job as a lifeguard, and she and her father no longer feared flying to New York for her two-year TCM visit. She experienced no reactions during the final 6 months of TCM (Years 2–2.5) (Table , Figures and ). Her baseline milk specific IgE level of 37.3kU/L has been declined to 19.0kU/L. Her FLIP score reduced to 25 from 35. No abnormal liver and kidney function test results before or after TCM therapy (AST, 20 and 18, reference 14-37u/L; ALT, 16 and 30, reference 8–36 U/L; BUN, 12 and 9, reference 7–25 mg/dL) were observed. She has reached her primary goal of avoiding reactions while on a dairy restricted diet, and is continuing TCM therapy with a new goal of maintaining the beneficial effect and to possibly become tolerant to milk allergens. |
A 45-year-old male with a previous history of tympanoplasty and functional endoscopic sinus surgery with septoplasty 10 years earlier presented to the ear, nose, and throat (ENT) clinic with several months of left moderate-to-severe otalgia and a sensation of ear blockage in his left ear accompanied by ipsilateral hearing loss. He gave a history of multiple failed ear wax removal in his left ear that had been performed at several ENT clinics, despite the use of alkaline ear drops.\nOn examination, the patient was comfortable and afebrile, and his vital signs were stable. Otoscopic examination of the left ear revealed impacted left ear wax covering the tympanic membrane, which could not be assessed. Otoscopic examination of the right ear also demonstrated mild ear wax, and the tympanic membrane was unremarkable. Oropharynx examination was unremarkable, the lymph nodes of the neck were not palpable, and all cranial nerves were intact upon examination. Nasal endoscopy revealed no pathologies. Ear wax removal under suction was attempted and failed. Another trial of removal after using alkaline ear drops for several days was also attempted but was unsuccessful. However, the surgeon became suspicious that the patient had KO rather than impacted ear wax because the wax was thick, had the appearance of keratin plugs, and was hard to remove after several attempts, despite the use of ear alkaline drops.\nBlood test results of the patient were within normal limits. The patient was planned for microscope-guided examination of the ears under general anesthesia. The examination revealed that the left ear was full of wax that was accumulating in the skin and contained a thick keratinous plug that had dilated the external auditory canal (EAC) with pockets and bone remodeling. Furthermore, the patient ear canal was circumferentially distended with a normal annulus. The tympanic membrane became visible and was intact. The keratinous plug was removed, and a diagnosis of KO was established (). An ear pack was draped with antibiotics and placed in the left ear. The patient was extubated, shifted to the ward without any complications, and discharged the same evening with the ear pack, which was removed after 3 weeks in the outpatient clinic. The patient was started on ciprofloxacin ear drops and analgesia for 1 week.\nIn the follow-up, the ear pack was removed, his hearing returned to normal level, and the pain disappeared. Pathological analysis of the removed plug revealed acellular lamellated keratin flakes and keratinous material (Figures and ), which confirmed our diagnosis. |
A 48 year old male with a long history of smoking presented with weight loss, anorexia and pelvic pain. He had a significant past history of a radical cystectomy ten years prior for a large multi-focal non-invasive, low-grade papillary (Ta) transitional cell carcinoma. The stated indications for cystectomy were large size of the mass and the anticipated inability to perform a complete resection. The pathological specimen which was reviewed at our institution was described as a low-grade non invasive papillary multifocal transitional cell carcinoma (TCC). The margins were clear and fourteen uninvolved lymph nodes were submitted. Postoperatively the patient recovered well and was under surveillance without any disease till above mentioned complaint. The patient's past history was also significant for a straddle injury requiring open surgical repair that occurred approximately 2 years prior to the diagnosis of bladder cancer.\nPhysical examination revealed a thin uncomfortable male with no other abnormal findings. Basic laboratory investigations were within normal limits. Imaging studies with CT scan of abdomen and pelvis revealed a right sided large heterogeneous pelvic mass with an area of central necrosis and evidence of bone destruction (right acetabular invasion) and distal rectal involvement (Figure ). There was no evidence of disease spread beyond this destructive pelvic mass.\nA CT guided biopsy of this mass revealed a low-grade urothelial carcinoma. Cisplatin based chemotherapy along with growth factor support was administered [dose dense methotrexate, vinblastine, doxorubicin and cisplatin (MVAC)]. After 3 uncomplicated cycles no tumor response was achieved. It was then decided that a curative intent en bloc resection represented the best option for patient.\nThe patient underwent surgical resection of the mass requiring a right hemipelvectomy, end colostomy and a myocutaneous flap closure with penile and scrotal reconstruction. The final pathology revealed an urothelial cell tumor with predominantly low-grade morphologic features, with focal areas of high grade tumor seen (Figure ; low magnification 10 × 10). The tumor invaded bone and soft tissue in a broad-based pushing fashion. The tumor formed nests with infiltration in the cortical bone, dissecting the pelvic soft tissue. There was no lymphovascular invasion and surgical margins were not involved. The patient is free from disease recurrence after more than one year following surgery. |
A 33-year-old male athlete with no significant past medical history presented with gradually worsening right arm and right handgrip weakness which started three days prior to presentation. The patient reported a painless swelling on the right side of the chest wall slowly growing over the last three months, and it was associated with malaise, intermittent night sweats, anorexia, and an unintentional weight loss of 15 pounds over the same period of time. The patient denied having fever, chills, or any other focal neurological deficits. Social history was negative except for the use of subcutaneous rhGH on a daily basis over the last 10 years with an intent to build lean body mass. The patient was actively involved in endurance physical exercise training and heavy weight lifting. The patient denied any trauma to left shoulder or chest wall except hearing a “pop” in his right shoulder during weight lifting four months ago that led to an acute onset of transient mild pain in the right shoulder for which he was worked up to rule out any musculoskeletal injury with CT and MRI that were negative for any musculoskeletal injury. Subsequently, the patient underwent physical therapy for a month with improvement in pain.\nOn admission, physical exam revealed a well-developed and well-nourished man with an athletic built and a 2 cm x 3 cm nontender mass at the anterior superior part of the right chest wall involving the right SCJ and soft tissues of the neck. There was the demonstrable weakness in the flexion and abduction of the right shoulder with a weakness in the right handgrip. The rest of the examination was normal.\nHis laboratory studies were significant for an elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) that were 122 mm/h and 6 mg/L, respectively. Blood cultures and oropharyngeal cultures were drawn on admission that came back negative. Initial imaging with chest X-ray revealed a right paratracheal haziness (see Figure ), and the right clavicle X-ray showed soft tissue inflammation of the base of the neck on the right side, but no fracture or dislocation of the clavicle was observed (see Figure ).\nA growing mass on the right side of the chest wall with focal neurological deficit i.e., weakness of right arm abduction and weak right hand-grip, associated with X-ray findings suspicious of a malignant lesion in the paratracheal area, prompted a CT scan of the chest with contrast that revealed a 2.2 cm x 2.6 cm x 2.9 cm anterior mediastinal mass in the superior-medial aspect of mediastinum invading the lung and the pleural cavity, and it also involved the right SCJ and manubrium sterni (see Figure ). Also, a significant hilar and mediastinal lymphadenopathy was noted suggestive of a highly aggressive inflammatory process due to either an advanced malignancy or an infectious process in the mediastinum.\nSubsequently, MRI of the right neck and SCJ with (see Figure ) and without contrast were performed that revealed inflammation of the right SCJ extending from the soft tissue at the base of the neck to the anterior superior mediastinum. No evidence of injury to the right brachial plexus was observed; however, the soft tissue around the brachial plexus was edematous due to the inflammation.\nTo further elucidate the etiology of inflammation observed on imaging studies, a CT scan guided fine needle aspiration (FNA) biopsy of the mass was performed which showed chronic inflammatory cells with a predominance of B and T lymphocytes, macrophages, and histiocytes with a few atypical cells. Furthermore, Gram stain, cultures, and acid-fast bacilli (AFB) smear of tissue biopsy were negative. Considering a high suspicion for malignancy i.e., lymphoma or teratoma, and low sensitivity of CT-guided FNA biopsy to diagnose mediastinal tumors, cardiothoracic surgery was consulted for mediastinoscopy with tissue biopsy. During the mediastinoscopy, an aggressive inflammatory process in the right anterior mediastinum was observed that invaded the right SCJ in cephalad direction, and posteriorly and caudally, it progressed to involve the right apical lung parenchyma. The patient underwent incision and debridement of the necrotic mediastinal tissue with a scraping of SCJ, and the tissue was sent for histology and culture which confirmed the diagnosis of complicated septic arthritis of SCJ. The histological analysis demonstrated the formation of germinal centers in the lung parenchyma and a predominance of CD 3 positive T lymphocytes, CD 45, and PAX 5 positive B cell lineage lymphocytes were observed in addition to macrophages, histiocytes, and dendritic cells (see Figure A-D). The tissue cultures grew methicillin-sensitive Staphylococcus aureus (MSSA) establishing an infectious process.\nThe patient was started on intravenous clindamycin and nafcillin during hospitalization, and later on, patient was discharged home on long-term intravenous antibiotics with nafcillin. The patient was advised to abstain from the use of any GH products, and avoid heavy weight lifting till complete recovery. At six weeks follow up, due to inadequate response to antibiotics, a follow-up MRI of the chest with and without contrast revealed an active inflammation of SCJ and signs of osteomyelitis of manubrium (Figure -B). A repeat mediastinoscopy with incision and debridement of the SCJ tissue and the lung pleura was performed and an extended parenteral antibiotic course was recommended.\nAt six months follow-up, the patient showed complete recovery with a resolution of SCJ septic arthritis and it was confirmed by the follow-up MRI of right SCJ with and without contrast (see Figure ). |
A 37-year old homeless male, with a past medical history of peripheral vascular disease, type 1 diabetes mellitus, hypertension, and depression, presented to the emergency department with intermittent chest pain and progressive shortness of breath for a few weeks. The patient denied intravenous drug use, although admitted to the use of recreational marijuana. The patient’s vitals on admission were stable except for low saturation on a pulse oximetry of 86% on room air. On physical examination, the patient was tachypnic and had fine crackles in the bilateral lung fields on auscultation. Laboratory results on admission showed a normal complete blood count and basic metabolic panel, but a urine drug screen test was positive for opioids. Because of persistent hypoxia, a D-Dimer was checked and came back highly elevated. The patient underwent a computed tomography angiography (CTA) of the chest to rule out pulmonary embolism. The CTA was negative for pulmonary embolism, however, it displayed extensive miliary densities throughout the bilateral lung fields (). The patient was admitted to the floor with a differential diagnosis of military tuberculosis versus fungal infection. Human immunodeficiency virus (HIV), fungal, and Quantiferron testing were negative. The cardiac work up and autoimmune serology were also unremarkable. The patient was then started on intravenous steroids and inhaled albuterol, although no improvement was seen. The patient remained hypoxemic despite therapy, and, therefore, underwent a bronchoscopy with a lung biopsy to find out the etiology of the disease process. The lung biopsy showed alveolated lung tissue with a miliary pattern of perivascular foreign body histiocytes containing refractory material suggestive of microcrystalline cellulose material (). There was no evidence of malignancy and there were no fungal or acid fast bacilli organisms identified on special stains. The histological features suggested intravenous injection of foreign material and upon further questioning the patient admitted to injecting oral opiates. The patient was started on intravenous steroids, although his clinical condition continued to decline. The patient developed hypercapnic respiratory failure, which required intubation, and eventually suffered from a cardiopulmonary arrest and passed away. |
History
The patient is a 23-year-old female who presented to the office with pain and numbness in her right foot and lower leg that had been gradually intensifying for the past year, following right plantar sesamoidectomy for non-healing fractures. She rated her pain as a 6/10 at rest and a 10/10 if aggravated or paroxysmal. Her pain was worse with prolonged activity and exercise but would also become exacerbated if she remained sitting for a long period of time. Light activity and firm pressure (compression stockings) provided temporary relief. Two months after surgery, she began experiencing pain around her right fibular head and sharp pain at her right first metatarsophalangeal joint. Her pain continued to intensify, developing a cramping-type character as well as increased muscle fatigue and the sensation of intense, localized pressure. She then developed progressive neuropathy in her right foot and lower leg and began experiencing deficits in temperature sensation and vacillations between hot and cold, starting at her popliteal fossa and radiating distally. She also reported paresthesia that would occur either at rest or as a result of light contact with her clothing or her bedsheets that would occasionally wake her from sleep. After nine months of the above symptoms, she began having episodes of severe pain and contracture in the flexor tendons of the plantar aspect of her right foot, which would also wake her from sleep. She had attempted physical therapy, medication trials, and a cam walking boot without relief of symptoms.
At the time of presentation to the clinic, she was lacking discriminatory sensation between hot and cold, and she reported that her leg had been alternating in color and tactile character throughout the day between red and purple and hot and cold, respectively. Her paresthesia was initially limited to her right great toe, the plantar aspect of her foot, the medial aspect of her ankle, and the posterolateral aspect of her right calf, but it had progressed to a � |
An 80-year-old male with diabetes mellitus, mild diabetic retinopathy, and mild dementia underwent reported uncomplicated, bilateral FESS for chronic sinusitis with polyposis at an outside hospital. He was provided an outpatient prescription for amoxicillin-clavulanic acid upon outpatient surgical discharge. Initial pathology specimen within the immediate postoperative period from the outside hospital was reported as non-invasive Aspergillus. He was seen by his original surgeon at the outside facility on postoperative day 3 without visual complaints or unexpected facial pain. On postoperative day 4, the patient reported sudden right-sided vision loss and presented to the walk-in ophthalmology clinic at the primary author’s tertiary care facility. An ophthalmology exam found no perception of light on the right and an abducens nerve palsy. Pupils were equal and reactive to light bilaterally with no pallor of the optic disc and normal ocular pressures. He was sent immediately to the emergency department for imaging, whereupon the otolaryngology team evaluated the patient. Intranasal exam by otolaryngology was only notable for moderate edema of the nasal mucosa without necrosis or pallor. There was no decreased sensation of the palate or midface. Computed tomography was performed and notable for violation of the lamina papyracea (Figure ). Preoperative imaging from the outside facility was not available for comparison. Magnetic resonance imaging suggested acute ischemic findings of the right optic nerve with diffusion restriction at the proximal optic nerve without evidence of thrombosis or hemorrhage. Subsequent imaging after admission noted an abnormal T2 signal without canalicular segment enhancement. There was, however, some adjacent enhancement extending to involve the optic and nerve sheaths in the orbital apex and superior orbital fissure. The enhancement of the right-sided nerve sheaths was thought to be contributing both to the vision loss and the abducens nerve palsy that was defining the OAS. In addition to those mentioned, the neurosurgery, neurology, and internal medicine teams evaluated the patient. It should be noted that repeated images were obtained at several time points due to poor images from motion artifact that did delay care. The clinical and radiologic findings were determined by the treating teams to be most suggestive of the surgical trauma from the FESS at the outside facility and not of an infectious origin. A non-surgical approach was initially pursued. The patient was provided broad-spectrum antibiotics and high-dose steroids to decrease inflammation with close neurologic monitoring. Re-evaluation by otolaryngology on flexible nasal endoscopy did not show any change in findings of the nasal mucosa.\nOn postoperative day 9 from the original FESS, the patient developed sudden vision loss of the left eye. An ophthalmology exam noted no light perception bilaterally, worsening ophthalmoplegia on the right with decreased supraduction, adduction, and infraduction in addition to the complete abducens palsy. These findings suggested now the involvement of the oculomotor and trochlear nerves. The posterior ocular exam now showed mild optic nerve pallor on the right with good color on the left and no evidence of edema. Repeat computed tomography and magnetic resonance imaging noted no new bony destruction; however, there was persistent inflammation of the right optic canal. An emergent multidisciplinary team meeting was held between all consulting specialties and infectious disease was also consulted as the initial diagnosis of traumatic neuropathy was questioned. The patient then underwent emergent FESS with otolaryngology and neurosurgery with decompression of both optic nerves, sphenoidotomy, septectomy, and mucosal stripping (Figure ). The lamina papyracea and bone adjacent to the root of the right orbital apex were dehiscent as had been suggested on imaging (Figure ). Additionally, there was a large amount of absorbable packing in the posterior ethmoid cavity on the right.\nA final culture from the initial FESS at the outside hospital was re-evaluated by the infectious disease team, which revised the diagnosis of Aspergillus to that of S. apiospermum. This pathologic diagnosis was made on postoperative day 10 (postoperative day 1 from emergent FESS). The patient was started on high-dose voriconazole and amphotericin B. The patient continued on maximum medical therapy and continued to worsen clinically and radiologically regarding the infection. Unfortunately, after prolonged hospitalization, he suffered a myocardial infarction and subsequent severe gastrointestinal bleeding. He passed away on postoperative day 47 from the initial FESS after being transferred to inpatient hospice services. |
A 65-year-old man with continuous irritable cough over 15 days was admitted to our department for a mass lesion in the right hilum. He presented with no face swelling and superficial varicose veins. He was a former smoker, and medical history included hypertension and diabetes mellitus. At bronchoscopy, the tumor was found occluding the right upper lobar bronchus and infiltrating the bronchus intermedius. It was diagnosed as squamous cell carcinoma by transbronchial tumor biopsy. Chest contrast computed tomography (CT) revealed that the mass of 80 mm in the maximum diameter located in the right upper lobe. The tumor directly invaded the arch of the right PA and a wide range of SVC. It was discovered that the left brachiocephalic vein (BCV) along the left margin of mediastinum walking down the line directly into the coronary sinus (). A venous flow was detected in the anterolateral descending aorta with dilatation of the coronary sinus (diameter of 21 mm) without other heart abnormalities by transthoracic echocardiography. Based on these findings, the diagnosis of PLSVC was made. There were no detectable metastases in other organs through brain magnetic resonance imaging (MRI), upper abdomen CT scan and bone nuclear scan.\nThe right posterolateral thoracotomy was performed on October 31, 2006. As expected, the tumor invaded the wall of SVC and no left brachial cephalic vein bifurcation was observed. The SVC was separated, clamped and resected following intravenous injection of 5, 000 IU of sodium heparin. The ringed PTFE graft of 12 mm in diameter was interpositioned between the right brachial cephalic vein and the origin of SVC by running sutures with 4-0 polypropylene. The tumor resection was completed by double sleeve lobectomy of right upper and middle lobes. The anastomosis between the right main bronchus and the cut end of the lower lobe bronchus was performed by interrupted suture using 3-0 Vicryl (Ethicon, USA). Right PA trunk and right lower PA reconstruction was completed by the running suture with 4-0 polypropylene (). And systematic mediastinal lymph node dissection was performed. All excised margins were tested microscopically negative for malignancy. As anticoagulation treatment, we began with low molecular Heparin on the operative day, then switched and continued to warfarin when thoracic drains removed. The postoperative course was uneventful, and the patient was discharged from the hospital on the 12th postoperative day. Final pathological diagnosis was poorly differentiated squamous cell carcinoma invaded the right SVC, right PA and mediastinal nodes were positive for malignancy.\nAfter 4 cycles of chemotherapy (Gemzar and cisplatin) and 1 cycle of radiotherapy, Chest MRI showed patency of the graft and PLSVC (). He died 21 months after operation because of remote metastasis. |
A 37-year-old male coughed up a large mass into the back of his mouth while swallowing. He pushed the mass back down his throat and he sought medical attention following this episode. He has chronic depression and smoked a pack of cigarettes a week for thirteen years but stopped smoking a year prior to presentation. A barium swallow was done which was reported as either an ‘… oesophageal duplication cyst of the cervical oesophagus … or less likely a polyp.’ A CT scan confirmed an intraluminal cervicothoracic oesophageal lesion consistent with a large fibrovascular polyp. He then underwent upper gastrointestinal endoscopy which confirmed a large mass on a stalk extending from the cervical oesophagus to 30 cm into the oesophagus. He was then referred for surgical resection. Clinical examination identified a Grade II/VI systolic murmur. Echocardiogram showed mild tricuspid, mitral and pulmonary regurgitation with mitral valve prolapse and a small secundum atrial septal defect. A preoperative diagnosis of a giant fibrovascular polyp (GFP) of the oesophagus was made, and the patient was scheduled for surgical excision.\nUnder general anaesthesia, he underwent resection of the oesophageal polyp. An incision along the anterior border of the left sternocleidomastoid muscle was made. An enlarged left lobe of the thyroid was mobilized and retracted medially, and the sternothyroid muscle was partially divided. The cervical oesophagus was dissected out circumferentially and encircled with Nylon tape. A longitudinal 6 cm full thickness oesophagotomy was made on the left lateral aspect of the oesophagus. The polyp was identified and delivered into the wound through gentle traction. The polyp measured 14 cm in length and 3 cm in diameter. The polyp stalk was excised down to the submucosa at its point of attachment to the proximal cervical oesophagus. The oesophagus was closed in two layers with absorbable sutures. He had an uneventful post-operative course and was discharged home after a swallow with water soluble contrast showed no leak on postoperative day seven. He developed hoarseness post-operatively, and indirect laryngoscopy 3 weeks post-operatively showed paralysis of his left vocal cord. This resolved with speech therapy over the following 6 weeks.\nThe histology report showed a polypoid lesion with a core of fibrovascular connective tissue surmounted by unremarkable stratified squamous epithelium. Scattered cells with large irregular hyperchromatic nuclei were noted throughout, including sections from the resection margin. The specimen was sent for immunohistochemical staining and was positive for CD34 and CDK4. The final diagnosis being of an atypical lipomatous tumour of the oesophagus. Repeat upper gastrointestinal endoscopy performed fourteen months post surgery was completely normal, with no evidence of recurrence. |
An 82-year-old man complained of a gradual increase in his abdominal girth over the past two years and of abdominal distension and intermittent nausea for the past six months. He was admitted to the hospital for repeated intractable hiccups having lasted two months. The patient had a history of intestinal necrosis due to an intestinal obstruction 25 years earlier and of occlusion of the inferior vena cava filter and left common iliac vein stent placement due to left common venous thrombosis five years earlier. Physical examination showed the patient was of average build with a thin, anemic appearance, and his vital signs were stable. Cardiopulmonary examination produced normal results. An abdominal examination revealed a longitudinal surgical scar in the middle of the upper abdomen. The swelling of the mass was evident on the left side of the abdomen (). Palpation showed the abdomen to be soft but not tender, and bowel sounds were normal. Ultrasonography and computerized tomography (CT) showed the presence of a massive hypoechoic lesion occupying almost the entire abdomen. This lesion exerted pressure on the stomach, liver, pancreas, and spleen. There was a high-density shadow visible about 1.5 cm from the middle pole of the medial edge of the mass (, ). An isotope kidney scan showed poor kidney function and only 5% normal renal function in the left kidney.\nAfter administration of two units of homologous red blood cells, the patient underwent what was initially laparoscopy and then open simple nephrectomy. Adhesion between the kidney and surrounding organs was evident during the procedure. About 7.5 liters of hydronephrosis fluid were drained. The left kidney and upper ureter were successfully removed (). The patient was diagnosed with GH caused by obstruction from a kidney stone at the junction of the ureter and renal pelvis, accompanied by renal cell transitional cell carcinoma (T2N0M0) (). The patient's intractable hiccup symptoms disappeared, and his condition and appetite recovered after the operation. During the six-month follow-up, the patient did not experience hiccups. |
A 16-year-old single girl, with 10 years of formal education, from rural background presented to surgery outpatient with complaints of pain in abdomen and vomiting for the last 6 months. She would have frequent bouts of vomiting, frequency of which increased in the last few days prior to presentation. On physical examination, she was found to have a mobile mass of 14 × 16 cm size in the upper abdomen, which was tender, with smooth surface and irregular margins. Ultrasound abdomen revealed a bizarre lesion in the right upper and middle abdomen suggestive of gastric bezoars. Upper gastrointestinal endoscopy did not reveal any abnormality in the esophagus and a large mobile mass made of hairs was seen in the stomach. Based on the findings of ultrasound and upper gastrointestinal endoscopy, a diagnosis of trichobezoar was considered and psychiatry consultation was sought. Psychiatric evaluation revealed that the girl was eating hairs and clay since early childhood. She would eat the hairs thrown here and there by her mother, other family members and neighbors. The patient would like the taste of hairs and had a strong and persistent desire to eat hairs. She would often go out searching for the same. Additionally, at times she would also eat clay. The description of the patient was corroborated by the mother too; who described that despite persistence reprimands, the patient persisted to eat hairs and occasionally would eat clay. However, the patient denied pulling out her own hairs. Physical examination of scalp and other body parts did not reveal any evidence of alopecia or pulling of hair/short hair. Past history did not reveal any evidence of depressive disorders, obsessive compulsive disorder, other anxiety disorders, eating disorders, emotional disturbances, mental sub-normality and other impulse control disorders. Also, there was no family history of any mental illness. Investigations in the form hemoglobin level, complete blood counts, serum electrolytes, liver function test, renal function test, pancreatic enzymes and routine stool examination did not reveal any abnormality. In view of large size of the mass, open laparotomy was done and the mass was removed. On examination, the mass comprised of entangled hairs. A psychiatric diagnosis of other behavioral and emotional disorders with onset usually occurring in childhood and adolescence (Pica of infancy and childhood) as per the ICD-10 was considered. The patient was counseled against eating hairs and clays and was informed about the consequences of the same. Behavioral analysis revealed that the patient would have urge to eat hair more so, when under stress. Additionally, behavioral principles with regards to management of urge and impulse control were discussed. Additionally, the psychotherapeutic intervention focused on teaching adaptive coping skills to deal with the stressful situations. Family members were also educated about the illness and were asked to be vigilant to note the patient behavior of eating hairs and other non-eatable items. The patient improved after the surgery and follow-up at 6 months did not reveal any behavior suggestive of trichophagia or pica. |
A 25-year-old lady presented to our hospital with complaints of productive cough and streaky hemoptysis for the last 4 weeks. She had been treated in the past for smear positive pulmonary tuberculosis in 2000 for 3 months and again in 2005 for 9 months with first-line antituberculous (ATT) drugs with poor drug compliance. She was diagnosed to have relapse in November 2007, and was being treated with second-line ATT drugs. Her detailed workup showed radiographic evidence of large cavitary lesion in the right middle zone and fibrocystic opacities in the right upper zone. Reports of sputum smear for acid-fast bacilli (AFB) and culture for mycobacterium tuberculosis were negative. Second-line ATT drugs were continued and, the patient was reviewed on a monthly basis in our outpatient department (OPD). She had a persistent large cavity in the superior segment of the right lower lobe after 18 months of treatment and bronchiectasis in right upper lobe. The patient underwent right upper lobectomy and excision of cavity in right lower lobe but developed a discharging sinus with air leak from the thoracotomy wound after 1 month. The patient was bronchoscopically evaluated and was found to have a fistula at the stump of right upper lobe bronchus. The fistulous tract was delineated by contrast injection under fluoroscopy.\nIn view of poor general condition, the patient was not a candidate for surgical closure and was offered device occlusion of the fistula with duct occluder device. It was decided that the approach to the site of BPF would be transcutaneous route and monitoring of the release of a PDA duct occluder device will be done under bronchoscopy and fluoroscopy.\nThe BPF was measured to be 8 mm in diameter and it was decided that the size of the occluder device will be increased to achieve good wall approximation and to avoid postdeployment migration. A Extra- stiff wire 0.035” (Cook medical, United States of America) wire was passed through the working channel of the bronchoscope under conscious sedation to gain access into the BPF opening and onwards to the pleural space and then the sinus track. The bronchoscope was withdrawn from the wire to be repositioned by the side of the wire for better visual control of the procedure. The wire was torqued to guide it along the sinus track till the skin wound and was made to exit by pull-and-push technique under local anesthesia. An 8F PDA delivery sheath was passed transcutaneously over the wire and its tip was positioned in the BPF under fluoroscopic and bronchoscopic control. After removing the wire, nitinol PDA device (Lifetech™, Shenzhen, PR China) of size 10 mm (proximal waist), 8 mm (distal waist), 7 mm (length), and retention skirt of 2 mm on either side was selected and loaded into the delivery sheath. The device was pushed up to the proximal level of fistula and released under fluoroscopic and bronchoscopic control to assume the predetermined sizes and to occlude the fistula snugly in the shape of a mushroom [].\nThe device position was confirmed and there was complete cessation of air leak and cough experienced by the patient earlier. Patient was managed with broad spectrum antibiotics and anti-inflammatory drugs. On the fourth postprocedure day, the device was bronchoscopically checked and found in situ with good approximation of the device—well-aerated right lower lobe with no clinical or radiological evidence of air leak or device migration []. The patient showed remarkable improvement in general outlook and nutrition with good effort tolerance. She was discharged on the 20th postprocedure day. Patient is asymptomatic with increasing opacification of residual cavity and closure device in situ. The cutaneous opening has completely healed, and the nutritional status and effort tolerance level of the patient have improved. |
A 61-year-old Japanese man with coxalgia had a medical examination. His previous history was uneventful; however, he was only 149 cm in height () and had suffered from coxalgia for a long time. Radiography revealed osseous projections at the knee and shoulder joints (). On radiographs and multidirectional computed tomography imaging of the hip joint a gigantic femoral head was observed, as well as increased shaft angle and large diameter of the femoral neck (). The patient had also developed coxarthrosis and severe pain of the right hip joint. The range of motion of the right hip joint was limited in flexion and abduction. His Japanese Orthopedic Association (JOA) hip joint function score was 45. Radiographs showed stage 4 arthritis based on the JOA hip score, with no ectopic ossification.\nRight total hip arthroplasty was performed using the direct lateral approach. The soft tissue appeared normal, and an incision was made in the articular capsule. A gigantic femoral head and large diameter of the femoral neck were noted. There was no evidence of exostosis with a cartilage hat. Hip dislocation was difficult because of the gigantic femoral head and caused a trochanteric fracture. The bone substance of the greater trochanter, femoral neck, and femoral trochanter was poor and showed bone fragility. The acetabular side cartilage and femoral head cartilage showed degeneration (). We implanted an acetabular cup by press fit without screws at the site of the original acetabulum, because the bone substance there was in good condition. We then performed an osteotomy in a more distal than usual part of the femoral neck, because the bone substance of the femoral trochanter was poor and exostosis was evident in the femoral trochanter on preoperative radiography. We implanted a femoral polished tapered stem using bone cement because of the poor bone substance of the femoral trochanter and the expanded medullary cavity of the proximal femur (). We fixed the fractured greater trochanter with tension band wiring using Kirschner wire and nylon tape (). We then repaired the articular capsule, gluteus minimus muscle, gluteus medius muscle, and tensor fasciae latae muscle.\nOn postoperative radiography and multidirectional computed tomography imaging, the implant setting was unproblematic, and the exostosis at the femoral neck had been removed (). Postoperatively we permitted walking with full weight-bearing. There were no complications such as dislocation or infection. At postoperative day 29, because we performed rehabilitation over long period so that the range of motion of the right hip joint was limited in flexion and abduction, the patient was discharged from the hospital with a crutch.\nThirteen months postoperatively the patient had no coxalgia, but the range of motion of the right hip joint was still limited in flexion and abduction. The patient had no limp and no interference with his activities of daily living. His JOA functional test score was 82. There was an ectopic ossification seen radiographically (). The patient had returned to his previous work and had no difficulties with everyday activities. |
The first patient is a 58-year-old man who was a previous smoker who presented to an outside hospital with abdominal pain and was diagnosed with gallstone pancreatitis. However, despite having a laparoscopic cholecystectomy, he continued to have episodes of pancreatitis yearly for the next 3 years. Computed tomography (CT) scan demonstrated a pancreatic duct dilated throughout its course to a maximum size of 8 mm without a discrete pancreatic mass in the head of the pancreas. Endoscopic retrograde cholangiopancreatography confirmed a dilated pancreatic duct with a suspected filling defect of the common bile duct, after which the patient underwent sphincterotomy and stent placement.\nHe then presented to our institution for consultation. Further workup with endoscopic ultrasound (EUS) was performed, which also confirmed the dilated pancreatic duct, but noted irregular contour of the duct. In addition, free mucin was seen exiting the main papilla, and there was a papillary growth noted in the pancreatic duct in the head of the pancreas, all of which was consistent with a main duct IPMN (). Preoperative laboratory values, including CA19-9, were within normal limits. Of note, it was unclear if the dilatation of the pancreatic duct was secondary to obstruction from the mass in the pancreatic head or due to main duct IPMN involving the entirety of the duct.\nHe was scheduled for surgical resection after the stated workup. He was planned for a Whipple procedure (pancreaticoduodenectomy) with possible total pancreatectomy if intraoperatively the main duct was found to be involved by IPMN with high-grade dysplasia. Transection of the pancreatic neck identified cells of high-grade dysplasia free floating near the margin (although exclusive of the margin). In addition, upon probing the pancreatic duct in the tail of the pancreas, the probe did not slide smoothly, suggestive of additional growths in the duct. Due to his young age and high risk of developing pancreatic cancer, a completion pancreatectomy and splenectomy were performed. He was reconstructed in a Roux-en-Y manner. He recovered well from surgery without any postoperative complications.\nHis final pathology demonstrated a 1.4 cm moderately differentiated colloid carcinoma arising within main duct IPMN (intestinal type) with high-grade dysplasia in addition to multiple foci of pancreatic intraepithelial neoplasia. Staining of the tumor was strongly positive for MUC2 and weakly positive for MUC1 and MUC5. The tumor involved the peripancreatic soft tissue, but all margins were negative. 0 of 39 lymph nodes was involved with tumor. His final pathologic staging was pT3N0Mx. He was, therefore, referred for medical oncology consultation. He was recommended for adjuvant chemoradiation with gemcitabine before and after fluorouracil-based chemoradiation, which he is currently receiving and tolerating well. |
Our patient is a 64-year-old left-hand dominant male who was involved in a motorcycle collision and sustained a right valgus impacted four-part proximal humerus fracture (). He also sustained a left small finger distal phalanx fracture. The patient’s medical history was significant for coronary artery disease. His past surgical history included a right shoulder arthroscopic superior labral repair and biceps tenodesis multiple years prior, and his social history included a history of cigarette smoking and social alcohol use. He denied having any pain or decreased range of motion in the right shoulder before the injury. The patient underwent open reduction internal fixation of the closed right proximal humerus 2 days after his initial presentation without complication. The fixation consisted of a lateral locking plate, separate plate, and screw fixation of the lesser tuberosity, and sutures into the supraspinatus, infraspinatus, and subscapularis that were anchored to the locking plate (). Pre-operative antibiotics included both 2 g of cefazolin and 600 mg of clindamycin per protocol for open shoulder surgery in the senior author’s practice, and this was readjusted throughout the procedure and continued for 24 h postoperatively. The patient was continued on the same antibiotics for the 24 h after surgery. He had a drain placed after surgery, and this was discontinued on post-operative day 2. He was discharged on post-operative day 3, after adequate pain control had been achieved on oral pain medications. His post-operative therapy regimen included a five-pound weight-bearing restriction for the first 6 weeks. He was encouraged to use his hand for activities of daily living. The patient was sent to physical therapy to work on active and active assisted range of motion, beginning with pendulums and progressing to full activity. At 4 months postoperatively, the patient was reporting continued pain and decreased range of motion in the right shoulder. His wound was well healed with no erythema or signs of infection. His motion measured 30o of external rotation with the arm at the side, 5o of internal rotation with the shoulder abducted to 90o, abduction to 60o, and forward flexion to 95o. His radiographs noted healing, with no change in the alignment of the proximal humerus from initial post-operative radiographs. The lateral locking plate appeared well maintained, but the partially threaded screw in the lesser tuberosity had backed out several millimeters (). As the patient had significant stiffness affecting the function of the extremity, a manipulation under anesthesia was performed 4 months after the index procedure. The manipulation was performed while holding counter-pressure over the lateral border of the scapula. Audible and palpable release of scar tissue was felt and heard. Post-manipulation range of motion included 70° of external rotation both at 0° and 90° of abduction, internal rotation of 45°, forward flexion of 160°, and abduction of 160° (). Imaging was performed after the procedure to ensure that there was no propagation of old fracture lines or any new fractures. The patient was given post-operative regional anesthesia for 24 h and was seen by the occupational and physical therapists for range of motion exercises during this time period. The patient was seen in the office on post-manipulation day 5, and a raised, erythematous and fluctuant 2-centimeter area was noted in the central portion of his prior incision. He denied any systemic symptoms such as fevers or night sweats. The assessment was that this was either a hematoma or deep infection. On post-manipulation day 10, this area converted into a draining wound (). His white blood cell count was 10.4/mm3, erythrocyte sedimentation rate (ESR) 26 mm/h, and C-reactive protein (CRP) 17 mg/L. The patient was taken to the operating room the following day, for irrigation and debridement, and partial removal of hardware. The prior deltopectoral incision was opened in line with the draining wound, the cavity was explored, and purulent fluid was noted. Multiple culture swabs were sent for microbiological analysis, and empiric vancomycin and piperacillin/tazobactam were started. In terms of the anterior hardware, the screws in the quarter tubular plate overlying the lesser tuberosity were noted to be loose and were removed, as were the independent partially threaded screw. The lateral locking plate and screws were covered in soft tissue that was adherent to the plate, and it was therefore not visible through the wound. The lateral plate was maintained in place as it appeared well-fixed on radiographs, and it may have still been supporting the fracture. An irrigation and debridement were performed. The infectious disease service was consulted, and on the 8th post-operative day, 4/4 cultures were positive for P. acnes. The cultures were beta-lactamase negative. The patient was placed on oral cephalexin for 6 weeks’ time. His wound healed, and 6 months later, the patient had the remainder of his hardware removed by another surgeon. His range of motion at this time was forward flexion of 150° with some assistance of the left arm, abduction of 90°, internal rotation to the lumbar spine, and external rotation of 40°. |
A 44-year-old female presented with numbness of the left upper limb and a pulsatile mass on the left chest at our outpatient service. She has been following up in our hospital since the age of 27 years when she underwent aortic root replacement for annuloaortic ectasia (AAE). Since then, she have been performed the aortic graft replacements for dissected aortic aneurysm total five times. A contrast-enhanced computed tomography (CT) scan revealed a highly tortuous left axillary aortic aneurysm (maximum diameter, 22 mm) (). We attributed the numbness to the compression of the brachial plexus due to the aneurysm. Accordingly, we performed an elective graft replacement with resection of the aneurysm to prevent aneurysmal rupture and thromboembolic events.\nDuring the operation, the aneurysm was exposed through two skin incisions (). The first skin incision was 4 cm on the supraclavicular area along the left clavicle. We carefully dissected the surrounding tissues and approached the aneurysm by avoiding any injury to the brachial plexus and internal jugular vein. In addition, we performed taping of the proximal anastomotic site. The second incision was 8 cm on the infraclavicular area from the sternoclavicular joint to the anterior axillary line. The major and minor pectoral muscles were not transected, but were separated along the length of the muscle fibers. The taping of three sites of the aneurysm was performed for segmental aortic clamping (). After intravenously injecting 5,000 IU heparin, we performed graft replacement using an expanded 6-mm polyester vascular prosthesis with external ring support after clamping of the proximal and distal arterial segments ( and ). We carefully located the vascular graft through the clavicle without touching the brachial plexus. Complete aneurysmal resection was performed after the ligation of branches from the aneurysm that were detected on a preoperative CT scan (). After the operation, we confirmed the blood flow to the left upper and significant improvement in the numbness. A post-operative CT scan revealed no problems at the reconstruction site. |
A previously fit and well 45-year-old man attended his local hospital with abdominal pain. He had raised inflammatory markers and serum lactate. Abdominal CT angiogram revealed a superior mesenteric artery embolus and evidence of infarcted bowel.\nThe patient underwent a laparotomy, superior mesenteric artery embolectomy and 30 cm of ischaemic jejunum was resected and a primary anastomosis was formed. The histology showed ischaemic bowel only. Transthoracic echocardiogram showed the presence of a large left atrial mobile mass attached via a pedicle, prolapsing through the mitral valve from the left atrium at 3.4 cm in length (.) It was unclear whether this was a myxoma or a thrombus. The patient’s ventricular function was normal and a coronary angiogram was reported as normal. He was transferred to a cardiac centre and underwent median sternotomy and aortobicaval cannulation 17 days after his laparotomy. The heart was arrested with anterograde and retrograde cardioplegia. The mass was found to be densely adherent to the left atrial free wall through to the right pulmonary veins and the superior vena cava, which was then detached from the right atrium for access. Ambiguity about the tumour’s nature made us send a frozen section specimen which revealed sarcomatoid appearances. The mass was peeled off the anterior mitral valve leaflet and excised with the invaded structures. The sinoatrial node had to be sacrificed. Bovine pericardium was used to reconstruct the left and right atrium and right pulmonary veins (). On coming off, bypass mitral regurgitation was observed via the transesophageal echo and so the neo left atrium was reopened and a 32 mm Cosgrove-Edwards ring implanted. The patient had an uncomplicated and short hospital stay. Histology was consistent with mildly pleomorphic spindle shape sarcoma supported by positive immunohistochemistry for SMA and the presence of MDM2 gene amplification. Despite the extensive tumour resection and chambers reconstruction, there was tumour margin involvement. The patient remains well five months later and is under the care of a sarcoma oncology team. |
A 70-year-old Sri Lankan Tamil male with a history of well-controlled type 2 diabetes mellitus and a goitre of 30 years presented with a painful enlargement of goitre on the left side for one month. He had progressively worsening difficulty in breathing with intermittent dysphagia for solids. He did not have any symptoms of local infiltration and was clinically euthyroid. Examination revealed a hard mass arising from the left thyroid lobe measuring 8 cm × 7 cm in size with gross tracheal deviation to the right side. In addition, there were two mobile lumps anterior to the mass in the subcutaneous tissue plane (). There was no retrosternal or retroclavicular extension on the left side. The right thyroid lobe was moderately enlarged and had multiple palpable nodules. There was no cervical lymphadenopathy. Ultrasound scan showed a large calcified left thyroid nodule and few superficial nodules. The outer surfaces of the nodules were delineated by an echogenic line suggestive of surface calcification. The internal echotexture of the nodules was not clearly appreciated due to artefacts from the surface calcifications. Right thyroid lobe showed only benign characteristics. Neck X-ray radiography showed a calcified left lobe with significant tracheal deviation to the opposite side (Figures and ). Thyroid stimulating hormone (TSH) and free thyroxine (T4) levels were within normal limits. Ultrasound-guided fine needle aspiration cytology (FNAC) showed scattered cyst macrophages, lymphocytes, and multinucleated giant cells in an eosinophilic background with scanty colloid. The features were compatible with a benign cyst (Thy 2).\nThe patient underwent a total thyroidectomy. Two confluent nodules were noted in the subcutaneous tissue plane extending through the deep fascia between the strap muscles to the calcified left lobe nodule. The deep fascia and strap muscles were thinned out and were adhered to the calcified left lobe (). Division of strap muscles on the left side was required to mobilize and deliver the left lobe containing the calcified nodule.\nMacroscopic assessment of the specimen consisted of the thyroid gland with the right lobe measuring 45 × 25 × 20 mm, the isthmus measuring 65 × 15 × 4 mm, and the enlarged left lobe measuring 80 × 75 × 55 mm. The outer surface of the gland was smooth. There were two confluent nodules over the anterior surface of the left lobe measuring 12 × 8 × 8 mm and 10 × 8 × 6 mm with a smooth outer surface.\nHistology of the thyroid revealed an encapsulated left lobe lesion composed of a thick fibrous wall with foci of calcification. A dense inflammatory reaction comprising lymphocytes, foamy histiocytes, and scattered multinuclear giant cells was present within the capsule. The lumen was filled with amorphous, eosinophilic material with cholesterol clefts. A thin rim of compressed thyroid tissue was noted outside the fibrous wall. Sections from the confluent nodules revealed similar histopathological features and showed encapsulated lesion surrounded by a thin fibrous capsule. They were filled with numerous foreign body type giant cells and foamy histiocytes admixed with amorphous eosinophilic material and cholesterol clefts. No thyroid or lymphoid tissue was seen. The right lobe and isthmus showed features of a colloid storing goitre. There was no evidence of malignancy in the entire specimen. Overall, features of the main calcified nodule of the left lobe and the two smaller confluent nodules were compatible with a colloid cyst with secondary changes including calcification and chronic inflammation. The patient had an uneventful postoperative recovery with the alleviation of the obstructive symptoms. He was discharged on the first postoperative day on thyroxine 100 mg daily and remained healthy without any obstructive symptoms during a three-month routine outpatient clinic review. |
A 37-year old homeless male, with a past medical history of peripheral vascular disease, type 1 diabetes mellitus, hypertension, and depression, presented to the emergency department with intermittent chest pain and progressive shortness of breath for a few weeks. The patient denied intravenous drug use, although admitted to the use of recreational marijuana. The patient’s vitals on admission were stable except for low saturation on a pulse oximetry of 86% on room air. On physical examination, the patient was tachypnic and had fine crackles in the bilateral lung fields on auscultation. Laboratory results on admission showed a normal complete blood count and basic metabolic panel, but a urine drug screen test was positive for opioids. Because of persistent hypoxia, a D-Dimer was checked and came back highly elevated. The patient underwent a computed tomography angiography (CTA) of the chest to rule out pulmonary embolism. The CTA was negative for pulmonary embolism, however, it displayed extensive miliary densities throughout the bilateral lung fields (). The patient was admitted to the floor with a differential diagnosis of military tuberculosis versus fungal infection. Human immunodeficiency virus (HIV), fungal, and Quantiferron testing were negative. The cardiac work up and autoimmune serology were also unremarkable. The patient was then started on intravenous steroids and inhaled albuterol, although no improvement was seen. The patient remained hypoxemic despite therapy, and, therefore, underwent a bronchoscopy with a lung biopsy to find out the etiology of the disease process. The lung biopsy showed alveolated lung tissue with a miliary pattern of perivascular foreign body histiocytes containing refractory material suggestive of microcrystalline cellulose material (). There was no evidence of malignancy and there were no fungal or acid fast bacilli organisms identified on special stains. The histological features suggested intravenous injection of foreign material and upon further questioning the patient admitted to injecting oral opiates. The patient was started on intravenous steroids, although his clinical condition continued to decline. The patient developed hypercapnic respiratory failure, which required intubation, and eventually suffered from a cardiopulmonary arrest and passed away. |
A 60-year-old male patient was admitted with complaints of low back pain and loss of appetite. He had a history of several diagnostic and therapeutic interventions for low back pain. At last, an abdominal computed tomography revealed a solid lesion (5 × 3.5 cm) at the pancreatic corpus and tail, which had indistinguishable fatty interplains with superior mesenteric artery (). Right hepatic artery was observed to be originating from proximal portion of the superior mesenteric artery (). The patient and his relatives were informed that optimum clarification of the tumor whether it was resectable or not could be done only during surgical exploration due to the close relationship of the tumor and the main abdominal branches of the aorta. The patient was operated with his relatives' consent. During exploration it was noted that the mass had invaded transvers mesocolon on the splenic flexure site and at the level of Treitz ligament. The mass was in close contact with superior mesenteric vein as well. Following division of the gastrocolic ligament, it was noted that the mass was adherent to the celiac axis and invaded the left renal vein, but it was completely released from the superior mesenteric artery. In this state (situation), distal pancreatectomy (pancreatic tissue on the left portion of portal vein), splenectomy, celiac axis resection, partial gastrectomy, left nephrectomy, left adrenalectomy, and left hemicolectomy with end colostomy by closure of the distal stump were performed (). Before deciding on celiac axis resection, we occluded the celiac axis with a vascular clamp and checked and felt the hepatic arterial pulsation at the hepatic hilum. Tumor adhesions to the celiac axis were not divided and en-bloc resection of the celiac axis was done (along) with the tumor. For gastric wall invasion, at first, we only performed a localized posterior gastric wall resection, but this procedure was completed to near-total gastrectomy due to ischemia of the remaining part of stomach. Gastric continuity was obtained by a Roux-en-Y gastrojejunostomy. Duration of the whole surgical procedure was 470 minutes.\nOn postoperative day one, the patient was extubated, and the nasogastric tube was removed. During the whole postoperative course liver enzyme levels were always within normal range (). Pancreatic leakage was not observed. On postoperative day eight, the patient was discharged uneventfully and free of any complication.\nHistopathological examination reported as pancreatic ductal adenocarcinoma with an extensively invaded peri-pancreatic tissue with perineural, lymphatic, and venous invasion. It had invaded adrenal gland, stomach wall, mesocolon, and renal vein. There was no histopathologically confirmed celiac axis and splenic tumor invasions, but there were fibrotic adhesions to the celiac axis. Of 29 lymph nodes resected, two had tumor metastasis; among nine lymph nodes resected around superior mesenteric artery, none of them had metastasis. Following medical oncology consultation, the patient was given chemotherapy, and after that he was planed for reversal of end colostomy. His back pain was completely relieved in the postoperative period. While this paper was prepared, the patient was at the end of the third month of his followup. |
A 50-year old female patient visited the hospital, referred from the obstetrics and gynecology department, with the chief complaints of pain at the right hip and leg which had continued from five to six months ago. The strength of the pain was around 6/10 by visual analogue scale (VAS). The pattern of the pain was an ache spreading from the right part of the anus to the right thigh and the rear side of the calf. She felt the pain in all types of positions, but it was even more serious when she sat or walked for a long time. Physical examination, including the sensory and motor neurologic test, deep tendon reflex test, straight leg raise test, and flexion, adduction and internal rotation (FAIR) test, revealed no specific findings except tenderness at the right piriformis muscle and greater sciatic notch. The patient had previously had a total hysterectomy, due to cervical intraepithelial carcinoma 18 years before, as well as a few treatments of radiotherapy and chemotherapy for local recurrence and metastasis. Magnetic resonance imaging (MRI), performed at the time of the visit, showed no specific findings except a minimal diffuse bulging disc at the L3-4 and L4-5. There was no specific finding from the electromyography. There were no findings either by the pelvis MRI or the PET-CT, which may cause lumbosacral plexopathy or sciatic neuropathy by local recurrence and metastatic lesion. No inflammatory finding was revealed in the blood test. Although trigger point injection, epidural steroid injection, lumbar sympathetic ganglion blockade, and sacroiliac joint injection were performed to treat the pain, they only resulted in temporary pain relief with no continued improvement. In spite of oral administration of narcotic analgesics, the pain became even more severe. Seven to eight months later, the patient reported decreased leg strength and severe (VAS 7-8/10 score) pain at the buttock and thigh. The decrease in leg strength was limited to under the knee, and the dorsiflexion of the ankle and the plantar flexion was lowered to 3/5 grade, but no muscular atrophy was observed. Though the lumbar spine MRI at that time showed no significant change in spinal lesion, fatty atrophy was found at the right piriformis muscle. In addition, the electromyography showed right sciatic neuropathy. Suspecting the nerve entrapment syndrome by fatty muscle atrophy, local anesthetic and steroid were injected to the piriformis muscle under the fluoroscopic guide (). After the injection, the pain was maintained at the level of VAS 1-2/10 for a few months, but the reduced leg muscle strength was not recovered. After that, no additional treatment was performed because the patient rejected further treatment. |
A currently 12-year-old boy was born to a Dutch couple. There was a possible positive family history for Duchenne muscular dystrophy, as the mother of the patient had 2 uncles who presumably had a muscle disorder, most likely Duchenne muscular dystrophy. However, a diagnosis was never made, and the men died at the age of 17 and 27. Because of the family history, the mother had undergone genetic screening for Duchenne muscular dystrophy in 1992 by means of creatine kinase screening, DNA screening of both X-chromosomes, and a muscle biopsy. The DNA screening was performed by means of genomic DNA (gDNA) hybridization with dystrophin probes, which did not detect any deletions in the dystrophin genes. Since all 3 investigations yielded normal results, the mother of our patient was explained that she was probably not a carrier of Duchenne muscular dystrophy (the estimated risk of being a carrier was therefore 10%). However, it was mentioned that this possibility was not completely ruled out, since dystrophin expression and creatine kinase serum levels can be normal in carriers. Furthermore, the sequence of the possible DMD gene mutation in the family was unknown, thus impeding a fully accurate—that is, with 100% certainty—detection of possible DMD gene mutations in the maternal X-chromosomes.\nThe boy was delivered by a Cesarean section at the premature age of 32 weeks and 2 days because of fetal distress based on a maternal infection and asphyxia. His body weight was 1955 g. Despite that mentioned earlier, he initially obtained a high Apgar score of 8 over 9.\nAt day 8, he was admitted to a neonatal intensive care unit with a neonatal necrotic enterocolitis. He remained here for 4 weeks, after which he was discharged with a weight of 2400 g and a frontal occipital circumference of 33.1 cm. An initial ultrasound of the brain did not reveal any abnormalities. However, after 1 month, another ultrasound demonstrated cyst formation, particularly frontal and parietal (periventricular leukomalacia degree 3), with enlarged periventricular liquor space. Subsequently, an electoencephalography (EEG) was made, which showed a normal background pattern according to his age without epileptiform activity yet with moderate focal abnormalities. An MRI made at the age of amenorrhea duration + 40 weeks (AD+40) showed a PVL degree 4 with cyst formation, mainly temporally located and left more than right (). The parents were informed about the prognosis, which likely included that their son would become spastic in his lower limbs. Additionally, it was mentioned that epilepsy and cognitive problems could possibly develop at a later age.\nAt the age of 3 months abnormal repeated general movements were present. After 6 months, cerebral palsy was diagnosed as he clinically presented with axial hypotonia while having hypertonia in his extremities. Moreover, he presented with hyperreflexia and general movement abnormalities at different moments during follow-up.\nFrom the age of approximately 7 months, he developed Salaam spasms, and in a later stage absence seizures with head nodding followed by tonic rigidity as based on multifocal epilepsy. This was subsequently confirmed by EEG registration, which revealed the epilepsy was originating from left and right occipital areas. The clinical picture further deteriorated, and he lost his visual ability. He was then diagnosed with a symptomatic West syndrome, since the EEG did not show any signs of hypsarrhythmia.\nIn line with the clinical picture of PVL, the boy was, at the age of 3½, not able to stand and walk independently. At the age of 3 years and 10 months, he was evaluated by a pediatrician for constipation, probably as a side effect of the use of the antiepileptic drugs. An extensive hematological and biochemical workout revealed increased liver enzymes: aspartate transaminase, 299 IU/L; alanine transaminase, 206 IU/L; lactate dehydrogenase, 1644 IU/L; and creatine kinase, 13 485 IU/L (normal <200 IU/L). Initially, liver disease was considered and tested for (i.e. hepatitis A, B, and C-; Epstein Barr virus; and cytomegalovirus serology, which turned out to be all negative) and also the family history of the mother was taken into account. On physical examination, a boy with severe growth and mental retardation was seen. The frontal–occipital circumference was 48.0 cm. Furthermore, he was hypotonic, which was in sharp contrast to the past when he was initially hypertonic. Moreover, his reflexes were diminished or even absent, while they used to be vivid at the early stages of his life. Additionally, no (pseudo)hypertrophic calves were observed nor were contractures present.\nWorking out the differential diagnosis, some of the aforementioned signs, such as the family history and the increased creatine kinase levels, pointed in the direction of a neuromuscular disorder, whereas other features did not. This prompted, however, additional investigations at the age of 4; a muscle biopsy and genetic testing were initiated. The muscle tissue appeared to be devoid of dystrophin. The genetic testing also confirmed the suspected Duchenne muscular dystrophy, as exon 61 of the Duchenne muscular dystrophy gene was duplicated. The mother, who never had any complaints, subsequently decided not to get tested for this mutation, as it would not have consequences for her or her offspring. That is, her son born with Duchenne muscular dystrophy was her second child (the oldest son did not have Duchenne muscular dystrophy), and she and her husband did not have a desire for more children. Therefore, it cannot be ascertained whether the dystrophin mutation of our patient was inherited from the mother or whether it was a de-novo mutation. After the diagnosis of Duchenne muscular dystrophy was made, our patient was eventually able to stand up individually and walk a short distance at the age of 5½ years. It was noticed that for standing up, he pulled himself in upward direction, thereby resembling Gower’s maneuver. At the age of 7, he lost the ability to walk again.\nAfter having established the diagnosis for Duchenne muscular dystrophy, he was psychologically tested at the age of 8 years, which revealed a developmental age of 9 months (Kent Infant Development Scale), showing serious retardation. He started rehabilitation therapy, physiotherapy, and physical therapy. The parents eventually decided not to start prednisone treatment. The patient currently visits a day care center for mentally retarded children. |
The first case was a 59-year-old African American male with a past medical history notable for schizoaffective disorder, depression, and substance abuse who was brought in to the emergency room for disorganized behavior and agitation in the community. At the time of admission the patient demonstrated disorientation, repetitive motor behavior, and an alternation between agitation and psychomotor retardation. He had poor response to communication and tactile stimuli. A suspicion of altered mental status due to organic causes was suspected with the possibility of catatonic excitement and retardation. He was admitted to the medical floor, with a work-up revealing a positive toxicology screen for cocaine and opioids. The patients CBC and BMP were within normal limits except for his ammonia level which was 80 mg/dl. The patient was initially treated with Chlorpromazine Hcl 50 mg orally daily for his agitated behavior as well as Naltrexone 50 mg orally daily for his opiate intoxication.\nThe patient exhibited incoherent thought process in addition to mumbled speech that made a significant portion of his assessment evaluation difficult. During evaluation, he displayed abnormal movements of his arms and face, with tremors and restlessness. His affect was flat. He did not display any perceptual disturbances or delusions. An assessment for cognitive impairment was noncontributory during his most recent admission. The patient received Mirtazapine 45 mg orally at bedtime and Olanzapine 10 mg orally daily in his treatment and by day three of admission had shown improvement in his disorganized behavior with supportive care. The patient demonstrated more effort to directly communicate with house staff after treatment began.\nThe patient reported a past history of psychiatric illness that was late in onset. His first presentation at the age of 51 years was significant for depressed mood, paranoid delusions, and auditory hallucinations for which he was diagnosed with a major mood disorder. His symptoms responded poorly to medications including antidepressants. His disease course involved increasing periods of impulsive behavior and agitation. He became noncompliant with his prescribed medications. He was later admitted to the medical floors at the age of 54 years for “repetitive behavior” during which he was found moving from his bed to the bathroom repeatedly as if he wanted to use the bathroom all the time. He also showed some abnormal rocking movements during this time period. A medical work-up for seizure was negative. He was discharged with a presumptive diagnosis of a psychotic disorder. Thereafter, at the age of 56 years he had an episode of property destruction in the community and it was noted that he had “abnormal body movements” in addition to lability of mood. His diagnosis was revised to schizoaffective disorder and he was treated for mood lability at the time with risperidone.\nGiven the late onset of his neuropsychiatric symptoms, a computed tomography scan (CT) of his brain was done during his presentation, as seen in . Reviewing his chart, it was noted that the calcifications were apparent in his first head CT taken in January of 2012 with no changes to the current CT in January of 2019. |
The first case was a 59-year-old African American male with a past medical history notable for schizoaffective disorder, depression, and substance abuse who was brought in to the emergency room for disorganized behavior and agitation in the community. At the time of admission the patient demonstrated disorientation, repetitive motor behavior, and an alternation between agitation and psychomotor retardation. He had poor response to communication and tactile stimuli. A suspicion of altered mental status due to organic causes was suspected with the possibility of catatonic excitement and retardation. He was admitted to the medical floor, with a work-up revealing a positive toxicology screen for cocaine and opioids. The patients CBC and BMP were within normal limits except for his ammonia level which was 80 mg/dl. The patient was initially treated with Chlorpromazine Hcl 50 mg orally daily for his agitated behavior as well as Naltrexone 50 mg orally daily for his opiate intoxication.\nThe patient exhibited incoherent thought process in addition to mumbled speech that made a significant portion of his assessment evaluation difficult. During evaluation, he displayed abnormal movements of his arms and face, with tremors and restlessness. His affect was flat. He did not display any perceptual disturbances or delusions. An assessment for cognitive impairment was noncontributory during his most recent admission. The patient received Mirtazapine 45 mg orally at bedtime and Olanzapine 10 mg orally daily in his treatment and by day three of admission had shown improvement in his disorganized behavior with supportive care. The patient demonstrated more effort to directly communicate with house staff after treatment began.\nThe patient reported a past history of psychiatric illness that was late in onset. His first presentation at the age of 51 years was significant for depressed mood, paranoid delusions, and auditory hallucinations for which he was diagnosed with a major mood disorder. His symptoms responded poorly to medications including antidepressants. His disease course involved increasing periods of impulsive behavior and agitation. He became noncompliant with his prescribed medications. He was later admitted to the medical floors at the age of 54 years for “repetitive behavior” during which he was found moving from his bed to the bathroom repeatedly as if he wanted to use the bathroom all the time. He also showed some abnormal rocking movements during this time period. A medical work-up for seizure was negative. He was discharged with a presumptive diagnosis of a psychotic disorder. Thereafter, at the age of 56 years he had an episode of property destruction in the community and it was noted that he had “abnormal body movements” in addition to lability of mood. His diagnosis was revised to schizoaffective disorder and he was treated for mood lability at the time with risperidone.\nGiven the late onset of his neuropsychiatric symptoms, a computed tomography scan (CT) of his brain was done during his presentation, as seen in . Reviewing his chart, it was noted that the calcifications were apparent in his first head CT taken in January of 2012 with no changes to the current CT in January of 2019. |
A 32-year-old woman was admitted to the emergency room with suddenly occured dispnea and orthopnea. On admission she had a blood pressure of 110/70 mmHg, a regular pulse of 134 beaps per minute, and a breath rate of 34 per minute with 68% of partial oxygen saturation. There was a grade 2 midsystolic murmur in the mesocardiac region and bilateral pulmonary ralles throughout the lung fields.\nThe electrocardiogram (ECG) revealed sinus tachycardia (). Chest X-ray showed bilateral extended hilar and paranchimal infiltration and lines of Kerley B. The patient was dramatically rapidly improved with diuretic therapy and partial oxygen saturation increased up to 95%. Transthoracic echocardiography (TTE) showed normal left ventricle ejection fraction with severe hypertrophy of left ventricular posterior, lateral free wall, and mid ventricular septum ( A, B). The maximal wall thickness of posterior free wall and mid ventricular septum were 25 mm and 19 mm respectively. There was a significant peak intraventricular gradient of 91 mmHg at rest (). The color doppler imaging demonstrated flow acceleration due to mid ventricular obstruction (). The coronary angiography performed because of elevated troponin I levels, that was demostrated normal coronary arteries. Left ventriculography showed totally midventricular obtruction at systolic phase (). The cardiac MRI was also performed to confirm the diagnosis. The cardiac MRI images were clearly showed the asymetric thickening of the left ventricular posterior, lateral free wall, and mid ventricular septum (). The 24 hour Holter ECG monitoring performed and any significant arrhythmias was detected. She was discharged with the prescription of metoprolol and acetyl salisilic acid. After one month of discharge she was admitted to the emergency room with palpitation. The ECG showed atrioventricular nodal reentrant tachycardia. The rhythm was converted to sinus after the administration of metoprolol. The control ECG revealed short PR interval and delta waves as the findings of Wolf Parkinson White syndrome which is different from the ECG on the first admission to the hospital (). The electrophysiologic study performed and radiofrequency ablation of accessory pathway was applied. Two weeks after hospital discharge, she was admitted to our hospital with ventricular tachycardia which was converted to sinus rhythm after electrical cardioversion. According to the European Society of Cardiology guidelines, ICD implanted because of the risk of sudden death. |
A 43-year-old man with a history of moderate hypertension and hypercholesterolemia consulted his physician for non-specific retrosternal pain. The first diagnostic tests ruled out myocardial ischemia. Due to the persistence of the symptoms, the patient underwent endoscopy of the upper gastrointestinal tract which revealed the presence of gastric adenocarcinoma in the antrum and corpus. The patient underwent a laparotomy. However, the direct infiltration of the tumor into the pancreatic head and the presence of small metastatic nodules in the omentum did not allow for a surgical resection.\nAt the first examination by the medical oncologist the patient complained of moderate pain in the epigastric region with dorsal irradiation and dyspepsia. Codeine and acetaminophen were prescribed for symptom control. To complete tumor staging, a contrast-enhanced spiral computed tomography (CT) of the chest, abdomen, and pelvis was carried out which showed the presence of three pathological lymph-nodes. The lymph nodes had diameters larger than 10 mm and were located in the lesser curvature of the stomach, in the peripancreatic region, and in the mediastinum. Three pulmonary nodules that were smaller than 10 mm were also observed. A very small amount of ascites was present in the abdomen, but neither hepatic nor lytic bone lesions were detected. A representative bone window scan of the pelvis is shown in Figure . The results of hematological and biochemical tests were normal with the exception of serum alkaline phosphatase, which was 2.5 times the upper limit of normal.\nAfter a frank discussion of the benefits and the morbidity of chemotherapy, the patient agreed to enroll in a controlled phase II trial and was treated with pemetrexed 500 mg/m2 and oxaliplatin 120 mg/m2, both repeated every 3 weeks. The first tumor re-evaluation was carried out 6 weeks after the beginning of the treatment, before the administration of the third cycle of chemotherapy. The three lymph-nodes, which were defined as target lesions, showed a 43% decrease in the sum of the longest diameters, classifying the response as partial. The pulmonary nodules, which were defined as non-target lesions, were no longer visible. The bone-window scans, however, revealed osteoblastic metastases in almost all vertebral bodies and in the pelvis, as shown in Figure .\nFrom the clinical point of view, the treatment was beneficial to the patient because it led to a significant reduction in the use of analgesics; moreover, the CA 19.9 level decreased from 222 U/ml to 66 U/ml.\nWe reasoned that the observed bone lesions were due to the strong osteoblastic response of preexisting bone metastases not visible on the baseline CT scan. In agreement with the patient, treatment was continued for two additional cycles. At the second tumor re-evaluation 6 weeks later, the target lesions were in confirmed partial response, and the bone metastases remained stable and no new blastic lesions appeared.\nAfter the fifth chemotherapy cycle, the patient decided to go off the protocol because of intolerable side effects, particularly a pharyngeal disesthesia induced by oxaliplatin.\nTwo months later, the first follow-up CT showed new lymph-nodes in the mediastinum and in the mesentery, while the patient's performance status had deteriorated due to persistent vomiting. He was then referred to palliative care. |
An 81-year-old healthy woman presented to a general neurology clinic in 2014 for a second opinion regarding pain on the left side of her face. She described the pain as electric shocks in her cheek from below her orbit to her chin. The pain was only present on the left and could be triggered by eating, brushing teeth, and other mouth movements. The pain was severe and lasted 20-30 seconds at a time. The pain came in cycles lasting about three months each. Her first cycle was in 2006, and there was no preceding injury or associated rash. When she presented in 2014, the cycle was not resolving spontaneously and the pain was more intense than previous cycles. She had no improvement with acetaminophen with codeine or tramadol. Her neurological examination was unremarkable with the exception of mildly decreased pinprick sensation in the V2 distribution on the affected side. Her previous neurologist prescribed carbamazepine, but she never tried it due to concerns over listed side effects. She was evaluated with magnetic resonance imaging (MRI) of her brain and magnetic resonance angiography (MRA) of the head. No structural or vascular etiology of her symptoms was identified.\nShe was given a diagnosis of idiopathic trigeminal neuralgia and started on gabapentin as a more palatable alternative to carbamazepine. Gabapentin helped her pain but led to weight gain, flattened affect, impaired memory, and poor coordination. She weaned herself off gabapentin prior to her follow-up visit. At that time, her pain had resolved, so she opted not to continue any prophylactic medications. She continued to have cycles of pain every one to two months for which she was given a prescription for low-dose gabapentin to be used as needed.\nIn May 2016, she developed on outbreak of zoster on her torso. She was treated for this with valacyclovir with good results, and she did not develop post-herpetic neuralgia. She observed that her facial pain diminished during antiviral treatment and returned upon completion. At her husband’s urging, she was vaccinated for the first time with Zoster Vaccine Live (Zostavax, Merck & Co. Inc., NJ, USA) in July 2016. She noticed that over the subsequent week, her left cheek pain gradually resolved, and at the present time, it has not recurred. This is now the longest period she has gone without trigeminal pain since 2006. |
A 57 -year-old female presented with incidental ultrasonic evidence of left upper pole renal mass in Nov. 2001. Further evaluation with abdominal Magnetic Resonance Imaging revealed a mass in the upper pole of the left kidney with radiologic characteristics of renal cell carcinoma (). Left radical nephrectomy was performed sparing the left adrenal gland. The pathology specimen analysis showed a cystic mass 3 × 3 × 5 cm with yellowish friable tissue. Sections showed malignant epithelial cells, arranged in sheets. The picture was consistent with renal cell carcinoma, Grade II Fuhrman nuclear characteristics, confined to the capsule, neither pelvicalyceal nor vascular invasion was found (pT1bN0M0). Postoperatively she did not receive immunotherapy or chemotherapy. Apart from her hypertension which was well controlled with amlodipine and valsartan, subsequent clinical and radiological follow up showed no local or metastatic recurrence till 5 years after the operation then she stopped her visits. On October 2016 an incidental mass was found in the right adrenal gland during a checkup visit for the status of her right solitary kidney. Abdominal and pelvic computed tomography scan was done, revealing a well-defined mass with a smooth outline in the right adrenal gland measuring 54 × 48 × 39 mm with a central necrosis. The density of the solid component was 38 HU. In dynamic study the solid component showed significant enhancement after intravenous contrast administration (). Thorough hematological, biochemical and hormonal investigations were performed; all were within normal range. The results of laboratory examination showed the adrenal mass to be nonfunctional. The condition was well clarified for the patient and consent was taken to do right adrenalectomy. Under general anesthesia, in left lateral position through right transcostal incision, right adrenalectomy was done (). No any perioperative complications were recorded and she was discharged home on 4th post-operative day. Pathological examination revealed morphological and immunohistochemical findings in line with metastatic renal cell carcinoma, including positive staining for AE1/AE3, cytokeratin 7, vimentin, and CD10, and negative staining for CDX-2, inhibin, and synaptophysin (, ). During the last 2 years she has being on regular follow up. Whole body Positron Emission Tomography-Computed Tomography with fluorodeoxyglucose was performed, neither local nor metastatic recurrence was observed in any system. |
Nurse
The patient was a 50-year-old male with chronic leukemia. Onset was about six years ago, and he had been visiting the hospital ever since. He was eventually hospitalized for chemotherapy and thereafter continued the routine of repeated hospitalization and home care. While chemotherapy dramatically reduces the size of enlarged lymph nodes and the spleen, bodily damage also increases with dosage escalation. For the first few months, the patient recuperated at home, but this gradually decreased to one month, then to one week, and after about one year, he could not be discharged from the hospital. Although the patient knew that a complete recovery was unlikely, chemotherapy was having an effect, thus both he and his wife desired continued treatment. When the effects finally began to wane, and it became difficult to predict whether bodily damage or the curative effects would be greater, he was told that he had about one month to live if therapy was discontinued. To the patient, choosing to discontinue chemotherapy was tantamount to giving into death, and thus he desired, as a last resort, to undergo aggressive treatment in a sterile room. Although the attending physician and I doubted the wisdom in carrying out chemotherapy, the disease specialist suggested that the choice be left to the patient. Since the specialist’s opinion was prioritized over the attending physician’s opinion within the medical team, chemotherapy was initiated. Even among the nurses, there were differing views on whether the therapy should be carried out. The patient experienced delirium and panic due to limited freedom in the sterile room. Yet, the healthcare team, in collaboration with the psychiatric department and palliative care team, provided care to maintain the patient’s life, as well as care for the wife. The treatment was covered by insurance. We were also able to secure a bed in the sterile room without issues. One month after initiating treatment, the patient desired further treatment, although he could not leave the sterile room. However, |
A 61-year-old female with past medical history significant for type 2 diabetes mellitus, hypertension, and dyslipidemia and family history of breast cancer in her brother and maternal aunt presented to the emergency room for recurrent chest pain. She also reported fatigue for the past few months and was being evaluated for worsening anemia by her primary care physician. Her hemoglobin was 6 gm/dl with iron panel consistent with anemia of chronic disease. Her electrocardiogram (ECG) at arrival showed an “RSR pattern” in V1 and V2 leads but was otherwise normal. She underwent a stress myocardial perfusion imaging (MPI) which was negative for cardiac ischemia. A transthoracic echocardiogram revealed a 1.6 cm × 1.5 cm atrial mass attached to the anterior wall of the left atrium, which appeared to cross the mitral valve in ventricular diastole. The left atrial mass was further characterized by a transesophageal echocardiogram (TEE) as a solid, irregularly shaped, partly mobile mass attached to the atrial septum and extending to the anterior mitral valve leaflet (). The segment attached to the septum measured 2.6 cm × 1.43 cm, and the segment attached to the valve measured 1.4 cm × 2.22 cm. In addition, MRI of the heart with gadolinium was done preoperatively which confirmed the circumscribed hypodense mass with speckled appearance which originated at the atrial septum and extended along the anterior mitral valve leaflet (). The mitral valve flow was normal with no evidence of obstruction, stenosis, or regurgitation. The patient underwent a minimal incision valve surgery for resection of the mass which was presumed to be myxoma due to its location. Intraoperatively, on open examination of the left atrium, it was noted that the mass originated from the fossa ovalis region of the interatrial septum and infiltrated the atrial wall down onto the entire anterior leaflet of the mitral valve. A fibrotic density that surrounded the tumor was also noted. The mass along with a portion of the interatrial septum and the mitral valve was resected. The mitral valve was replaced using a 27 mm Hancock II bioprosthetic valve. The atrial septal defect caused by the resection was repaired with a bovine pericardial patch. A postoperative TEE was performed which confirmed the successful placement of the bioprosthetic valve with no paravalvular leak. The patient had an otherwise unremarkable postoperative recovery and was discharged home after fourteen days of hospital stay.\nTwo weeks following discharge, the patient presented to the emergency with cough, diaphoresis, and palpitations. She was noted to have jugular venous distention and bibasilar crackles on auscultation of the lungs and was found to be in acute heart failure. An urgent transesophageal echocardiography demonstrated severe mitral regurgitation with paravalvular leak (Figures , , , and ). At the same time, the histopathologic examination of the atrial mass showed a high-grade sarcoma consistent with dedifferentiated liposarcoma. The tissue exhibited spindle cells with pleomorphism, multinucleated giant cells, and inflammatory cells. Immunohistochemical stains demonstrated that the neoplastic cells were positive for vimentin, focally positive for S-100, and weakly positive for CDK4 and negative for p53 (). FISH studies performed showed an MDM-2 gene amplification in 95–200 nuclei examined. The patient was aggressively treated with intravenous diuretics and afterload reduction using furosemide and nicardipine infusion. Blood cultures were obtained with suspicion of postsurgical infective endocarditis causing valvular dehiscence. However, cultures did not grow any bacteria. The patient clinically deteriorated due to new-onset atrial fibrillation and worsening heart failure despite medical treatment in CCU. CT chest obtained showed a 1.3 cm lytic iliac bone lesion and 3.1 cm × 2.5 cm right upper mediastinal soft tissue density. Due to high suspicion of metastatic disease, MVR and cardiac transplant were not offered until further evaluation for metastasis. Unfortunately, due to rapid clinical decline with a new diagnosis of high-grade cardiac tumor with possible metastases, the patient opted for hospice care. PET study was not obtained. |
A 57 -year-old female presented with incidental ultrasonic evidence of left upper pole renal mass in Nov. 2001. Further evaluation with abdominal Magnetic Resonance Imaging revealed a mass in the upper pole of the left kidney with radiologic characteristics of renal cell carcinoma (). Left radical nephrectomy was performed sparing the left adrenal gland. The pathology specimen analysis showed a cystic mass 3 × 3 × 5 cm with yellowish friable tissue. Sections showed malignant epithelial cells, arranged in sheets. The picture was consistent with renal cell carcinoma, Grade II Fuhrman nuclear characteristics, confined to the capsule, neither pelvicalyceal nor vascular invasion was found (pT1bN0M0). Postoperatively she did not receive immunotherapy or chemotherapy. Apart from her hypertension which was well controlled with amlodipine and valsartan, subsequent clinical and radiological follow up showed no local or metastatic recurrence till 5 years after the operation then she stopped her visits. On October 2016 an incidental mass was found in the right adrenal gland during a checkup visit for the status of her right solitary kidney. Abdominal and pelvic computed tomography scan was done, revealing a well-defined mass with a smooth outline in the right adrenal gland measuring 54 × 48 × 39 mm with a central necrosis. The density of the solid component was 38 HU. In dynamic study the solid component showed significant enhancement after intravenous contrast administration (). Thorough hematological, biochemical and hormonal investigations were performed; all were within normal range. The results of laboratory examination showed the adrenal mass to be nonfunctional. The condition was well clarified for the patient and consent was taken to do right adrenalectomy. Under general anesthesia, in left lateral position through right transcostal incision, right adrenalectomy was done (). No any perioperative complications were recorded and she was discharged home on 4th post-operative day. Pathological examination revealed morphological and immunohistochemical findings in line with metastatic renal cell carcinoma, including positive staining for AE1/AE3, cytokeratin 7, vimentin, and CD10, and negative staining for CDX-2, inhibin, and synaptophysin (, ). During the last 2 years she has being on regular follow up. Whole body Positron Emission Tomography-Computed Tomography with fluorodeoxyglucose was performed, neither local nor metastatic recurrence was observed in any system. |
A 51-year-old female with thoracic SB presented to our hospital with fever, pyelonephritis and pneumonia. A vascular surgical opinion was sought as the patient developed recurrent infected pressure ulcers in the right hip, right thigh and right heel with exposed calcaneal bone, and cellulitis of the right leg.\nThe patient had a problematic medical history secondary to thoracic SB. Her comorbidities included neurological bowel and bladder dysfunction, orthopaedic abnormalities and chronic osteomyelitis. She had a functional level at T12 with paraplegia and mobilized with a wheelchair. Since birth, she had undergone multiple operations including a Girdlestone of the right hip for osteomyelitis secondary to pressure ulceration. She was hospitalized eight times due to chronic osteomyelitis in her hip and lower limb. She had attended 12 outpatient clinic visits and over 100 primary care visits for the same condition over a 23-year period.\nMagnetic resonance imaging and a SPECT-CT (single photon emission computed tomography-CT) were requested which showed osteomyelitis in the bone around the Girdlestone and in the calcaneum. Vascular assessment revealed a normal arterial supply (Fig. ).\nShe was admitted and treated with intravenous antibiotics; pressure relieving measures for the right hip and thigh; and negative-pressure wound therapy for the right heel. Her condition improved with nearly complete granulation tissue growth in the right heel wound. She was discharged after 29 days with a further 6 weeks of intravenous antibiotics.\nDespite intravenous antibiotic treatment, healing remained poor and she suffered from chronic pain. A referral was made to rehabilitation medicine for consideration of leg amputation. Other than minor modifications needed to wheelchair set up, there would be no functional deficit from amputating the limb.\nWith antibiotic cover and with all other wounds now epithelialized, she underwent a right above knee amputation through a fish mouth incision (Figs and ). The operation was uneventful and the patient was discharged at 5 days with no complications.\nShe was followed up at 12 months post-amputation. There were no further hospitalizations or primary care visits for problems related to ulcers or soft tissue infection. All pressure areas and surgical wounds remain healed. She completed two RAND 36-Item Health Survey (SF-36) questionnaires [] to investigate how she perceived her quality of life (QOL) before and after the amputation (Table ). She reported increase ease of mobility using her wheelchair after the amputation. |
A female baby who was 45 days old reported to the craniofacial center with unilateral cleft lip and palate []. After clinical examination, NAM was planned for the patient. The parents were informed about the treatment procedure and the duration, with more emphasis placed on their daily involvement during the active phase of the NAM procedure.\nThe parents were asked not to feed the baby at least 3 h before taking impression. Impression was taken with the child secure in the mother's lap. Primary impression of the maxillary arch was taken with silicone impression material in a special pediatric impression tray []. Caution was taken to avoid any airway obstruction. After the impression had been taken, oral and nasal cavities were inspected for any residual impression material. The impression was poured in dental stone for making a working model [].\nThe plate was fabricated on the impression cast after the undercut areas were blocked with utility wax. Separator medium was placed onto the cast. The plate was made of self-cure acrylic material. The plate should be at least 2 mm in thickness to provide rigidity during the NAM procedure. Nasal stent was made in. 032-inch titanium molybdenum alloy (TMA) wire, the retentive part was acralyzed in the plate during the plate fabrication [Figure and ]. The plate is placed in the infant's mouth, and from the wire, a coil is made which is 3–4 mm in diameter. The upper part of the wire reaches the dome of the nose which is bent into bean shape to incorporate acrylic bulb. The acrylic bulb is coated with soft acrylic for ease of insertion and also to prevent irritation during activation [].\nAll the surfaces of the plate are smoothed any rough surface, or hard edges may irritate the soft tissues and may cause ulcerations. The plate was placed in the mouth and checked for fitting. Properly seated appliance will have the plate seated in the mouth and the nasal stent soft acrylic placed in the medial alar wall of the nose on the affected side. The baby was kept under observation for the initial few minutes to check for the stability of the plate. Steri strips are placed from one side of the cheeks to the other side []. After this procedure, the parents were asked to bottle feed the baby to ensure there is no gag reflex.\nThe baby was seen once in 2 weeks for appliance adjustment. The plate is adjusted to facilitate the approximation of the alveolar process. The nasal stent is adjusted to align the nasal dome, improve nasal projection, and increase the length of the columella. The adjustment of the nasal stent is done with orthodontic pliers. The parents were advised to clean the plate every day and keep the plate clean. |
A 59-year-old woman presented in July 2017 with extensive bleeding from her ileostomy site. Her history included locally advanced bladder cancer for which she had undergone pelvic exenteration and ileal conduit formation in November 2015. At that time, she had a known primary lung adenocarcinoma as well, but had no known liver metastases or other liver disease. Intravenous contrast-enhanced CT of the abdomen and pelvis performed in January 2016 raised the possibility of cirrhosis; however this was not biopsy-proven. In April 2016, she began to notice intermittent bleeding from her stoma which was initially thought to be mechanical tissue breakdown from the stomal flange. Concern for hepatic encephalopathy was raised when she had her first episode of confusion in December 2016. At that time CT of the abdomen and pelvis demonstrated strong radiographic suspicion for cirrhosis together with prominent vessels surrounding the urinary diversion site suspicious for portal hypertension. Despite not having a tissue biopsy, she was diagnosed clinically with cryptogenic cirrhosis in May 2017 during a hospitalization for fatigue, anasarca, and altered mental status. An upper endoscopy performed in June 2017 demonstrated portal hypertensive gastropathy but no esophageal varices.\nUpon presentation to the Emergency Department in July 2017 she had significant hemorrhage from her stoma resulting in hemodynamic instability. She was anemic with a hemoglobin of 8.3 g/dL that improved to 9.4 g/dL after blood transfusion, but gradually fell to 8.2 g/dL by the time of the procedure. Her MELD score was retrospectively calculated to be 19 at presentation, with an INR of 1.5 and total bilirubin of 4.3 mg/dl. She was emergently taken to the interventional suite for embolization with or without portal venous decompression via portosystemic shunt formation. A review of intravenous contrast-enhanced CT imaging showed extensive venous varices around the stoma involving the abdominal wall with a large draining varix arising from the portal system, likely the inferior mesenteric vein []. Also visualized was a variceal connection to the right common femoral vein. The portal and mesenteric veins were noted to be patent. Multiple approaches were considered for this patient. The transjugular intrahepatic portosystemic shunt (TIPS) and transjugular transhepatic approach with portosystemic shunt creation offered the benefit of portal decompression; however, the patient's recurrent hepatic encephalopathy was felt to be a relative contraindication. Transjugular transhepatic approach without formation of a permanent portosystemic shunt was also considered, since it would eliminate the risk of progressive hepatic encephalopathy. Percutaneous transhepatic approach would also eliminate the risk of progressive hepatic encephalopathy but was believed to pose increased risks of hepatic injury and bleeding. Transsplenic venous access to the portal venous system was considered as a viable, albeit technically challenging, option. The superficial nature of the abdominal wall stomal varix presented a less challenging and seemingly more time-efficient approach for access and was chosen as the target.\nUsing a micropuncture kit, the peristomal varix was directly accessed under ultrasound guidance and a micropuncture sheath was placed. Venography was performed and showed a large variceal collateral conglomerate around the stoma with variceal anastomosis with the right common femoral vein []. A wire was advanced and a 5F sheath was secured over the wire. A Kumpe catheter was introduced and advanced into the distal intra-abdominal aspect of the large draining varix. Catheter position was confirmed with repeat venography, and multiple coils were deployed []. This was followed by Gelfoam embolization. Postembolization venography showed sluggish flow in the draining varix with multiple filling defects within the visualized collaterals consistent with embolization []. The coils remained well-situated after placement and there was no evidence of migration. To ensure that there was no filling from the systemic venous system, the right superficial femoral vein was then accessed with a micropuncture kit and a femoral-iliac venogram and IVC venogram were both performed. These demonstrated brisk flow from the right common femoral vein through the iliac system and into the IVC. There was no filling of the stomal variceal collaterals visualized []. Hemostasis was thereby achieved, and the patient became hemodynamically stable shortly thereafter. At 6-month follow-up time no further imaging had been performed and the patient had not had any further episodes of hemorrhage from the ileostomy site |
A 55-year-old healthy woman was referred to our institution with a two-year history of progressive dysphagia to solids (). She reported a recent episode of solid food getting stuck in her throat, which prompted presentation to an outside endoscopist. The patient reported no alcohol use. She was a former smoker with a 15 pack-year history, but had quit over 20 years prior. The patient had a past medical history of gastroesophageal reflux disease, for which she was taking omeprazole, and hypothyroidism. She had no known history of any esophageal dysmotility disorder. There was a history of diabetes mellitus in her mother and son.\nPhysical exam and laboratory testing were unremarkable. Esophagography demonstrated a filling defect in the upper thoracic esophagus. Computed tomography (CT) demonstrated an 8 cm mass. Endoscopic ultrasound (EUS) demonstrated a pedunculated mass with a submucosal origin beginning at 20 cm from the incisors on the right side of the neck (). The lesion was felt to have the characteristic appearance of a FVP and the patient elected to proceed with resection.\nThe exploration began via a right cervical approach. The recurrent laryngeal nerve was identified and the cervical esophagus was mobilized. The mass was palpable on the posterior esophageal wall at the thoracic inlet. Upon a short myotomy, no stalk was identified and the mass could not be delivered to the neck. The cervical incision was closed and a right thoracotomy was performed. The mass was seen extending from the level of the azygos vein to the thoracic inlet. The esophageal muscular layer was intact. Following myotomy, the soft mass, which was densely adhered to the mucosa, was visualized and dissected from the underlying mucosa. It became evident that the mass maintained its attachment to a portion of the mucosa. Complete mobilization revealed the mass to be a lipoma at the tip of a large midesophageal diverticulum traveling in a submucosal plane. Repeat endoscopy demonstrated an ostium in the esophageal wall opening into a blind-ending pouch. The diverticulum was fully mobilized and resected using a stapler (). Mucosal closure was reinforced with overlying muscle and a pleural flap.\nThe patient was diagnosed with a large midesophageal diverticulum with a lead point lipoma. The patient’s postoperative course was uncomplicated. A postoperative esophagogram demonstrated no esophageal leak or obstruction. Pathology demonstrated a 7.5 cm diverticulum with a 4.5 cm lipoma without malignancy. At follow-up on the nineteenth postoperative day, the patient was tolerating a diet without dysphagia. |
The patient was a 65-year-old man who presented with food obstruction, hoarse voice and choking on drinking water for 2 months, and pain behind the breastbone for 1 month. Gastroscopy revealed squamous cell carcinoma 23 to 25 cm from the incisors. Positron emission computed tomography examination showed that the middle and upper part of the esophagus was occupied, accompanied by multiple lymph node metastases in the right neck root and mediastinum. FDG uptake of the right lobe nodules of the liver was increased. Therefore, metastasis was considered. According to the eighth edition classification of UICC-TNM, the clinical stage of this patient was cT4bN1M1 (stage IVB). The patient had no indications for surgery in stage IVB and required local radiotherapy to relieve the current obstructive symptoms, hence CRT was planned. A single dose of 2Gy of radiotherapy was performed 5 times per week and the chemotherapy regimen was based on platinum drugs. Scored Patient-Generated Subjective Global Assessment score was 9. The nutrition group formulated a nutritional support treatment plan. According to the follow-up, stereotactic body radiotherapy treatment was considered optional for liver lesions. After obtaining the consent of patient and his family, the treatment was started. During the treatment process, symptoms such as low back pain had been occurred. Meanwhile, the liver lesions were larger than before in the liver magnetic resonance imaging (MRI) images, and lumbar MRI and bone ECT revealed new multiple bone metastases. Head MRI was normal without lesions. The lesions at the radiotherapy site (right neck root, mediastinal lesions) were absorbed and the range was reduced. All these results were observed in 1 week from the start of the first concurrent chemoradiotherapy. After discussing and evaluating within the department, the progression of the disease was considered. After communicating with the patient and family members, it was decided to add immunotherapy. Although the patient has not been tested for immunotherapy-related indicators, immunotherapy could be used as part of comprehensive treatment for patients with stage IV esophageal cancer. After obtaining the consent of the patient and family members, he was given camrelizumab 200 mg for one cycle. After 5 days, the patient began to develop cough, sputum, and bloodshot sputum. Chest computed tomography showed multiple lung nodules and metastasis was considered. The patient and his family gave up treatment, and his condition deteriorated rapidly after discharge. It took only 3 months from the onset of symptoms to death. In order to further find the cause of the patient's rapid progress, we performed genetic testing with his blood samples through high throughput DNA sequencing methodology (next generation sequencing; NGS) and found some meaningful somatic gene mutations as shown in Table . |
A 36-year-old-male patient had a background of Prader-Willi syndrome. He had the associated learning difficulties and hyperphagia seen with the syndrome. His dietary intake was carefully controlled within the care home where he lived and his body mass index was maintained at around 30. He also suffers from asthma and had orchidopexy as a child.\nThe patient was admitted as an emergency with abdominal pain and vomiting, following an episode of binge eating. A CT scan showed a grossly dilated stomach and a small volume of free gas in the abdomen (Fig. ). Diagnostic laparoscopy did not reveal a perforation. His condition improved with antibiotics and decompression of the stomach with an NG tube. He was discharged after a 9-day admission.\nThe patient was admitted a second time, 7 months following the initial presentation with recurrent symptoms of abdominal pain, vomiting and respiratory compromise, after another episode of binge eating. The provisional diagnosis was of acute gastric dilatation with aspiration pneumonia and dehydration. He was admitted to the high dependency unit for stabilization, with oxygen, intravenous fluids and insertion of an NG tube was passed to decompress the stomach. The NG tube drained 1500 ml immediately. Fourteen hours after admission, he suffered a pulseless electrical activity cardiac arrest but was successfully resuscitated. A CT pulmonary angiogram and CT abdomen/pelvis showed a massive pulmonary embolus, pleural effusion, grossly distended stomach and ascites. The patient was sedated and ventilated in intensive care for 11 days. Four days after admission, a trial of NG feed was started. Despite the use of neostigmine and metoclopramide, NG aspirates remained high and total parenteral nutrition was subsequently started. He was discharged to the ward after 13 days in intensive care. Cautious oral intake was started after a swallowing assessment. He was unable to tolerate the NG tube and also found the central venous access distressing. After 16 days on the ward, he developed a further episode of respiratory failure and was readmitted to intensive care. The picture was of aspiration pneumonia and the patient required reintubation, ventilation and inotropic support. An upper gastrointestinal endoscopy showed significant residual food debris in the stomach, regurgitating to the oesophagus. The presumed aetiology of the recurrent aspiration pneumonia was gastric stasis causing food reflux into the oesophagus and bronchial aspiration.\nAfter consultation with our local upper GI surgery unit, it was agreed that medium- to long-term decompression of the stomach would be required. As he was unable to tolerate an NG tube, a PEG tube with a jejunal extension was placed to vent the stomach and to permit enteral feeding.\nThe patient was successfully extubated 1 week later and discharged to the ward.\nOver the course of a further 18 days, his oral intake was slowly increased with regular aspiration of the PEG-J tube.\nHe was subsequently discharged to the community, re-established on his normal dietary regimen and anitcoagulated with warfarin. At outpatient clinic follow–up, the PEG-J tube was planned to be removed under a direct vision at a further endoscopy. |
This 55-year-old man was admitted for a several-month history of personality change and more recently had been becoming increasingly drowsy and was noted to be dragging his left foot. A CT scan of the head revealed a large cystic mass in the right frontal lobe with compression of the frontal horn of the right lateral ventricle and right-to-left shift. Decreased signal was seen extending throughout the white matter of the frontal lobe across the genu of the corpus callosum into the left frontal lobe. An MRI of the head confirmed the large complex mass with contrast enhancement of the solid component, as well as a portion of the rim of the cystic component. However, the enhancing component did not extend into the corpus callosum. The differential diagnosis was glioblastoma or possibly a metastatic deposit. While in the hospital, his condition deteriorated and he was given dexamethasone with some improvement. A CT scan of the head revealed a new large intratumoral hemorrhage. An emergent craniotomy was performed with drainage of the cyst and a subtotal resection of the tumor, which appeared to extend into the temporal lobe. The patient received adjuvant external beam radiotherapy with a total dose of 59.4 Gy in 33 fractions. MRI studies performed 6 months post-operatively revealed a new focus of contrast enhancement suspicious for recurrence, which decreased in size on the most recent imaging 2 months later. However, the extent of the surrounding non-enhancing T2/FLAIR signal abnormality had increased. The latest plan was to see him again in 3 months, with the possibility of chemotherapy if imaging revealed tumor progression.\nThe pathology specimen was very similar in appearance to case 1, except that the oligodendroglial component appeared low-grade (WHO grade II), with low mitotic activity and no evidence of either microvascular proliferation or necrosis (Fig. a). Dysmorphic ganglion cells (Fig. b) displayed strong cytoplasmic and membrane pattern positivity for synaptophysin (Fig. d), but were nearly uniformly negative for Neu-N (Fig. f). No EGBs were seen. Weak cytoplasmic positivity for synaptophysin was seen in occasional oligodendroglioma cells as well, but mostly highlighted entrapped neuropil (Fig. c); Neu-N stained only entrapped cortical neurons (Fig. e). Scattered GFAP-positive cells were seen in both components, although in the GGLF they appeared consistent with tumoral astrocytes (Fig. h), while in the oligodendroglioma portion most positive cells looked like reactive astrocytes (Fig. g). Occasional gliofibrillary oligodendrocytes were also noted. Tumor cells were CD34 negative, while intratumoral endothelial cells were positive. The Ki-67 stain reached a maximal labeling index of roughly 8% in the oligodendroglioma component, but stained only rare small perivascular cells in the GGLF (similar to Fig. h). FISH studies showed chromosome 1p deletion in both components (Fig. a, b), while chromosome 19 studies showed a normal disomic (2 copies) dosage in both regions (Fig. c, d), i.e., there was no evidence of 19q deletion. |
A 45 years old male patient presented with a history of resection surgery of the right side of the body of the mandible for the extirpation of lower buccal right side squamous cell carcinoma lesion along with functional neck dissection two years back. The defect was reconstructed by a vascularized free fibula graft with the skin island covering the intra oral defect successfully along with tumor ablation surgery done simultaneously. The patient was administered therapeutic radiotherapy post ablative surgery. Maxillary complete dentition was present and in the lower left jaw, canine until the left second molar was present. Right side free fibula graft was present stabilized by osteosynthesis plates. There was excess of skin flap over the fibula graft restricting the inter-arch space with opposite dentition and was difficult to occlusally rehabilitate with prosthesis. There was no vestibular sulcus and mucosa with restricted tongue movement because of lingual space occupied by skin flap. In the radiograph and CT scan, it was evident of the space present between the fibula and the symphysis indicating of delayed union/non-union.Pre operative panorama\nPre operative computerized scans\nIntra oral picture\nFor successful prosthetic rehabilitation, there is a need to understand and evaluate the extent of the defect and its limitations. Various classifications for evaluation of mandibular defects have been described such as Jewer’s classification of Mandibular defects [] Boyd classification of mandibular defects [], Urken et al. [] Classification of mandibular defects [], Cantor and Curtis classification of mandibular defects [, , ], but the most latest and simplified one reported is by James S Brown et al. The limitation of James S Brown et al. [] classification is that soft tissue and type of dentate status has not been described. Our paper presented a class 2 defect of James S Brown classification.\nAfter the routine blood examination and written consent was obtained from the patient, the patient was operated in a routine dental operatory under local anesthesia. Lignox® 2% A [lignocaine with adrenaline 1:80000] was infiltrated around the fibula. Following the manufacture instructions, five BECES® implants [Manufacturer: Simpladent GmbH, Switzerland] and one ZDI® implant [Manufacturer: Simpladent GmbH, Switzerland] were placed via a flapless procedure. From distal to mesial, the dimension of BECES® implants engaging the lower cortex of the fibula were of 3.6 mm diameter but length of 29 mm, 26 mm, 29 mm, 26 mm and 23 mm. The ZDI® implant of 4.6 mm diameter and length 35 mm was angulated towards the left symphysis from the mesial aspect of fibula flapless and bended by AHB® adapter for the restorative acceptable position. The placement of implants were itself a challenging task as the implants were long and limited inter arch clearance resulted in difficulty of the implant insertion. There were more than 10 mm of the dead space because of mobile skin island over the fibula.\nImpression was made on pickup impression caps supplied along with implants by polyvinyl additional silicon putty impression material (Aquasil® - dentsply), the same day after the implant placement. Next day metal framework fit was checked over the implant abutment intraorally and in the evening metal to acrylic semi-permanent hybrid prosthesis was delivered keeping the occlusion lingually inclined. The surgeon skillfully placed all implants free hand avoiding the osteosynthesis screws present for the fibula fixation.\nAfter a year, the prosthesis was removed by cutting the metal framework carefully by carbide burs and all implants were checked for the stability. One loose implant was found to be in contact with the osteosysnthesis screws and thus failed to osseointegrate and was hence, removed. The prosthesis was refabricated keeping sanitary intaglio profile. Tissue around the implants were asymptomatic with no complaints from the patient. The surgeon was successful in achieving the union of the fibula to native mandible and was evident radiographically. On three years follow up, the patient complained of some growth without pain around the neck of the implants on the transplanted fibula. A biopsy was then performed that reported negative. On examination, it was observed that complete mucosalization of the skin graft had occurred with no observable distinction from the mandibular mucosa of the patient. It was concluded that the growth was the granulation tissue around the implants which was then excised by soft tissue 810 nm laser with 400 μm fibre. Presently, the patient is completely satisfied and doesn’t have any complaint up till now and there is a healthy mucosa all around the intaglio surface of the functional prosthesis.BECES® and ZDI® implants placed flapless\nPost operative panaorama\nOne year follow-up\nTwo year follow-up\nThree year follow-up up to 04/2108\nGrowth around the implant neck\nGranulation tissue excised by soft tissue 810 nm laser\nHealthy mucosalized tissue appreciated with sanitary prosthesis |
An 18-year-old male patient presented with a large 10 × 12 × 10 cm fungating mass over scalp in the midline, more toward the left side []. There was past history of flame burn over face and scalp around 15 years back when he was a kid. He had been treated with dressings at home by his local medical practitioner. The wound had been closed by scarring during a period of months. The area had remained unchanged until last 2 months, had developed an ulceration over the scar tissue, and there was a serosanguinous discharge since then. Due to personal neglect and a low socioeconomic status, no treatment was taken. Since last 15 days, he had developed a purulent discharge; over last 4-5 days, he had developed a very soft swelling over the scalp which was gradually increasing in size. Neurologically, the patient was normal previously but was now bedridden with increased spasticity in both upper and lower limbs with no power in the lower limbs and 2/5 power in both upper limbs.\nOn examination, the swelling was very soft, which was probably a brain tissue protruding out through the scalp and the bony defect. Computed tomography (CT) scan confirmed that there was a bony defect present of size about 8 × 6 cm and the brain matter was fungating out. There were areas of intra cerebral hematomas inside the fungating brain tissue [Figure –]. CT angiography was also done to know about the major blood vessels. Superior sagittal sinus going through the defect was already thrombosed [].\nThe patient was subjected to surgery for resection of the protruding necrotic brain matter with freshening of the defect with duraplasty using fascia lata of right thigh with forehead transposition flap. The forehead donor site was covered with the spilt skin graft harvested from the left thigh [Figure –].\nHistopathology of the specimen taken from the margins of the defect revealed well-differentiated squamous cell carcinoma, while that from the excised brain tissue showed marked edema, dense infiltration with acute and chronic inflammatory cells, granulation tissue, and abscess formation. At one focus, there was an area of well-differentiated metastatic squamous cell carcinoma.\nPostoperatively [], initially the patient recovered well, but later he dramatically deteriorated and expired within 10 days. |
A 34-year-old healthy woman presented with a rapidly enlarging anterior abdominal wall mass noticed over three months. She was otherwise well apart from occasional pain over the lesion. There was no family history of similar conditions. Physical examination revealed a large subcutaneous mass that was firm and mobile with increased prominence upon muscle contraction. The overlying skin was normal. An ultrasound showed a solid lobulated mass with vascularity indenting the abdominal wall. A tru-cut biopsy revealed inflamed fibrocollagenous tissue infiltrated by epithelioid tumor cells. A computed tomography (CT) scan of the abdomen showed a heterogeneous lobulated subcutaneous lesion at the left anterior abdominal wall measuring 54 × 73 × 68 mm with central necrosis []. There was no calcification. There was a poor plane of demarcation with the abdominal muscles but no evidence of distant metastasis.\nShe was elected for a wide local excision, which was performed under general anesthesia as she has an acceptable anesthetic risk. An elliptical incision was made on a previously made skin marking []. Intraoperatively, there was a mobile tumor measuring 8 × 6 cm with the posterior part attaching to the anterior rectus sheath. A wide local excision with 1 cm margin was performed, resecting along the involved anterior rectus sheath. The feeding vessel is from the superior epigastric artery, and it was carefully identified, doubly ligated, and divided. A non-degradable polypropylene mesh was incorporated on the breached segment to avoid future herniation []. A vacuum drain was inserted to avoid the risk of deep surgical site infection from hematoma collection.\nThe resected specimen revealed a solitary tumor measuring 75 × 55 × 90 mm [ and ]. The cut section of the tumor showed a friable lesion with golden yellow tan orange cut surface and hemorrhage area. All resection margins were tumor-free, with the closest margin of 5 mm at the deep margin. Histology demonstrated epithelioid cells in nests with rounded nuclei, irregular nuclear membrane, and abundant eosinophilic to clear cytoplasm with thin fibrovascular septa and some spindle cells with evidence of lymphovascular invasion [-]. It showed moderate to marked nuclear atypia and nuclear pleomorphism with many large and bizarre nuclei. Less than 50% of coagulative necrosis was observed with mitotic figures (3 mitoses/10 high power fields (HPFs)). Immunohistochemistry (IHC) revealed that the tumor cells were positive for human melanoma black (HMB)-45 [], but negative for desmin [], myogenin, Melan-A [], PanCKAE1/AE3 [], U79 RCC marker [], S-100, vimentin, smooth muscle actin, and CD10. This tumor was graded as the French Federation of Cancer Centers Sarcoma Group grade 2. She was discharged on day three post-operatively and completed 50 Gy/25 fractions (2 Gy per fraction) of adjuvant radiotherapy. She is currently well with no signs of recurrence. |
A 42-year-old woman presented with a 2-year history of typical angina following the onset of premature menopause. At the time of presentation, she described angina consistent with Canadian Cardiovascular Society (CCS) Class II severity, with predictable chest tightness when walking up hills. She reported no symptoms at rest. The patient had been prescribed propranolol 10 mg three times daily for anxiety and an oestrogen patch for hormone replacement therapy. She was an ex-smoker and her past medical history included obesity, migraine, and depression. She underwent an ETT, exercising for 6 min and 23 s with reproduction of chest pain at 4 min but no ECG changes. The test was stopped due to dyspnoea and fatigue. Her attending cardiologist felt that CAD could not be excluded and referred her for a CTCA, which showed normal coronary arteries.\nInvasive CAG revealed myocardial bridging in the mid-LAD. Coronary flow reserve on coronary function testing was low at 1.7, with a borderline IMR of 24. On acetylcholine provocation testing, there was a transient reduction in antegrade flow in the LAD without evidence of epicardial vasospasm (). The patient experienced transient chest pain and the ECG disclosed ST elevation in leads I and aVL with reciprocal ST depression in leads II, III, and aVF. The angiographic appearance following acetylcholine, accompanied by the reproduction of her usual symptoms and ECG changes, was consistent with microvascular spasm, an endotype of microvascular angina.\nThe patient had been randomized to the standard, angiography-guided arm of the trial. She was diagnosed with probable non-cardiac chest pain. No changes to her medication were made.\nAt 12 months, the patient was unblinded. Her angina status remained consistent with CCS Class II severity and she was prescribed verapamil 240 mg daily and statin therapy. Her primary care physician had referred her to the cardiac rehabilitation service for supervised exercise. Four months later, she reported an improvement in her angina. Her exercise tolerance had improved and she only had symptoms when the weather was particularly cold. Her Seattle Angina Questionnaire—Physical Limitation (SAQ-PL) score had improved from 55 to 72 following the introduction of verapamil. |
A 51-year-old female with a history of stage 1 hypertension was admitted to the hospital post cardiac arrest. She had a significant family history of premature coronary artery disease. Her father had a coronary artery disease diagnosed in his 40’s, her mother had a pacemaker inserted, and she had two first degree relatives diagnosed with an early cardiac disease. She is a nonsmoker, nonalcoholic drinker and denied recreational drug use. She was in her usual state of health and functionally active until her husband noticed her moaning and eventually unresponsive in the middle of the night. Prior to this, the patient did not complain of any chest pain, dyspnea, dizziness, lightheadedness or any other constitutional symptoms. Her husband started cardiopulmonary resuscitation and was taken over by the emergency medical staff (EMS). She was intubated and defibrillated five times by EMS for ventricular fibrillation then was given a bolus of amiodarone. She eventually had a return of spontaneous circulation and was transported to the hospital in which she was noted to have decorticate posturing with no purposeful movements. Electrocardiogram (ECG) during this time showed sinus rhythm (Figure ). She was admitted to the coronary care unit and was placed on targeted temperature management and was maintained on amiodarone drip. Transthoracic echocardiogram showed no valvular abnormalities, normal left atrium and left ventricular cavity size but with borderline concentric left ventricular hypertrophy with an ejection fraction of 56% and note of subtle regional wall motion abnormalities. About 12 hours after initiating the targeted temperature management, the patient was in severe bradycardia in the mid 30’s and a decision was made to hold the amiodarone at that point. The patient completed the targeted temperature management protocol and eventually had a full neurologic recovery thereafter.\nShe was extubated and subsequently transferred to the floor. She was scheduled for cardiac catheterization; however, she had witnessed ventricular fibrillation arrest on the floor (Figure ). Advanced cardiopulmonary resuscitation was initiated and she was revived immediately after defibrillation. A total of three minutes of resuscitation was performed and she was following commands afterwards. She was also given an amiodarone bolus and transferred back to coronary care unit. ECG did not show any ST elevations at this time. Subsequently, she had 23 more episodes of ventricular fibrillation arrest (Figure ) over a six-hour period which were intervened by timely defibrillation and administration of antiarrhythmic medications and inotropic agents. The antiarrhythmic medications administered were amiodarone and lidocaine boluses and drips. Intravenous magnesium was also given during the resuscitation course. Emergent cardiac catheterization showed minimal coronary artery disease. A transvenous pacemaker wire was placed due to bradycardia during a code in the catheterization laboratory, although the bradycardia did not initiate the arrest. An intra-aortic balloon pump was placed. Subsequently the balloon pump and temporary pacemaker wire were removed after she eventually became more hemodynamically stable. She once again had a full neurologic recovery and underwent an uneventful implantation of a dual-chamber implantable cardiac defibrillator (ICD). She was discharged from the hospital on amiodarone therapy and was closely followed up in the cardiology outpatient clinic. Cardiac magnetic resonance imaging was done which showed mild biventricular enlargement but with normal regional systolic function, no late gadolinium enhancement consistent with the absence of myocardial inflammation, infiltration or infarction and with no imaging features of ARVC. She eventually had a targeted genetic testing for idiopathic ventricular fibrillation and was found to have a mutation in the desmoglein-2 (DSG2) gene (c.338T > C, pVal1113Ala), which is a gene typically involved in ARVC.\nShe had been readmitted several times to the hospital due to palpitations, dizziness, lightheadedness, and defibrillator shocks. Her defibrillator had been interrogated as well as her antiarrhythmic medication had been adjusted which improved her symptoms and reduced defibrillator discharge. Because of intermittent episodes of defibrillator firing due to ventricular fibrillation despite antiarrhythmic medications and intracardiac device, a ventricular fibrillation radiofrequency catheter ablation was performed successfully. Thereafter, she had no repeated episodes of palpitations, dizziness, lightheadedness, and defibrillator shocks. |
A 57-year-old man, presented with acute abdominal pain, was admitted to the emergency room. The patient had a previous medical history of spontaneous celiac artery and of the superior mesenteric artery dissection. An abdominopelvic CT scan, obtained 3 months prior to admission, demonstrated a 7 mm-sized saccular aneurysm, with a mural hematoma along the celiac artery to the common hepatic artery and a dissection of the superior mesenteric artery with a mural hematoma (). Infiltration of the fat surrounding the celiac artery was noticed as a secondary sign of acute spontaneous celiac artery dissection. Other than the acute abdominal pain, the patient was healthy and had no risk factors associated with the occurrence of visceral artery dissection. He had been treated with an anticoagulant to prevent a thromboembolic complication for 3 months, because he had no major complication associated with the dissection of the celiac artery and superior mesenteric artery. The patient had no obvious abnormality on physical examination or in his laboratory findings. Vital signs were stable. Contrast enhanced CT revealed a progressive dissecting aneurysm along the celiac artery, which had a maximal inner diameter of 2 cm (). The aneurysm originated 2 cm distal from celiac artery orifice without compromised distal flow to the distal hepatic artery. In addition, the patient's previous superior mesenteric artery dissection with mural thrombus had improved completely without associated complications. The patient was transferred to an interventional suite for endovascular treatment, because the risk of rupture was high, due to the progressive enlargement of an aneurysm sac. Celiac angiography, obtained after an insertion of the 8 Fr guiding catheter (Cordis), showed a dissecting aneurysm, which obviously defined the celiac artery, proximal to splenic artery and left gastric artery orifice (). The involved vessel course had a relatively smooth curvature. We placed the 4-9 × 38 mm peripheral stent graft (Jostent, Abbott Vascular, Rangendingen. Germany), which was mounted over a 6 mm × 40 mm savvy balloon (Cordis) from the origin of celiac artery to the bifurcation of the common hepatic artery and the splenic artery. Post-procedure angiography showed complete exclusion of the dissecting aneurysm (). There was no noticeable compromise in the blood flow to the distal hepatic artery, gastroduodenal artery, and splenic artery. The postoperative course was uneventful and the patient's symptoms abated after the procedure. The follow-up CT scan, obtained 3 months after the procedure, showed a complete disappearance of the dissecting aneurysm. |
The patient is a forty four year old female who presented to a chiropractic office for evaluation and treatment of right upper extremity pain. She described the pain as starting in her neck and shoulder on the right and radiating down her right arm to her fingers. She also complained of tingling and numbness of her right lateral forearm and hand as well as loss of grip strength. She stated that the symptoms were insidious in onset several weeks prior with no history of trauma. The symptoms were constant and severe and getting worse in recent days. Working at her computer or using her right arm aggravated the condition, but she achieved some temporary relief with rest. She denied any prior upper extremity symptoms, but reported that she had experienced chronic frequent neck and upper trapezius pain on the right for years that was mild in nature and did not limit her activities of daily living or her job performance as a management information systems manager.\nPhysical examination demonstrated normal cervical ranges of motion. Upper extremity symptoms were increased with upper limb tension tests[]. Valsalva test and neutral cervical compression were negative, but Spurling's test was positive on the right. Arm abduction provided relief of upper extremity symptoms. Manual muscle testing and deep tendon reflexes of the upper and lower extremities were normal bilaterally. Grip strength dynamometry revealed the following: 40/38/40 left and 60/58/60 right. The patient is right hand dominant. Palpation of the wrist extensors caused increased numbness of the first three digits of the right hand. Spinal palpation revealed segmental joint dysfunction at multiple levels in the cervical and thoracic spine with grade II tenderness at C4–5–6 on the right (tenderness ratings per American College of Rheumatology Pain Scale). Hypertonicity and grade I tenderness of the levator scapulae, anterior scalene and subocciptal musculature was noted on the right.\nA radiographic examination of the cervical spine was also performed. The films were read by a radiologist and revealed a block vertebra at C2–3, a markedly reduced cervical lordosis, advanced discogenic spondylosis at C5–6 and moderate to advanced uncovertebral arthrosis at C5–6 which he noted could be associated with foraminal encroachment and C6 radiculopathy.\nBased on the patient's history and the results of the physical and radiographic examinations a working diagnosis of cervical radiculopathy was formed. Differential diagnoses also included thoracic outlet syndrome and brachial neuritis associated with postural faults and segmental joint dysfunction.\nTreatment included spinal manipulation to the restricted segments, post-isometric relaxation to the hypertonic musculature and manual long axis traction of the cervical spine above the level of the suspected nerve root involvement. As the radiculitis lessened and the severity of the patient's symptoms decreased she was also instructed on neuromobilization techniques to decrease possible nerve root adhesions[,].\nThe patient was treated eighteen times over a seven week period during which time she experienced progressive relief. At the end of that time the patient's upper extremity symptoms had resolved but she continued to experience mild neck and upper back pain and stiffness that she described as tolerable. She expressed satisfaction with her outcome and was released from rehabilitative care. During the next year she was seen periodically on a supportive basis for mild flare-ups of neck pain and stiffness and upper extremity parasthesias that were quickly resolved using the same therapies.\nApproximately eighteen months after her initial symptoms the patient again experienced similar severe right upper extremity symptoms. Subsequently, the patient's primary medical physician referred her for an MRI of the cervical spine. The radiology report noted a disc osteophyte complex at C5–6 encroaching upon the subarachnoid space and right neural foramina. Consequently the patient was referred by her medical physician for a surgical consultation. However, the patient was resistant and wished to pursue conservative treatment and again presented to the chiropractic office.\nAt that time the patient had not been treated in the chiropractic office for almost five months. She complained of upper back and shoulder pain on the right as well as pain and numbness of the lateral forearm and hand that was persisting for several months. However, she noted that the intensity of the symptoms was not quite as severe as when she initially experienced the condition two years earlier. In intervening months she experienced occasional numbness of the distal right upper extremity, but did not report any pain.\nPhysical examination demonstrated normal cervical ranges of motion. Neutral cervical compression and Spurling's test were negative. Cervical distraction provided modest relief of the cervical spine symptoms, but had no effect on the upper extremity symptoms. The upper limb tension test produced anterior forearm pain, but did not reproduce the current chief complaint. However, digital palpation of a trigger point in the right infraspinatus muscle did exacerbate the chief complaint of shoulder pain and parasthesias of the lateral forearm and hand.\nBased on the physical examination the cause of the patient's current complaint appeared to be myofascial referred pain from an active trigger point. Treatment focused on manual trigger point therapy, including both ischemic compression and post-isometric relaxation, as well as functional postural correction. Due to the chronic nature of the condition active rehabilitation included cervical retraction and mid/lower trapezius strengthening exercises. Cervical and thoracic spinal manipulation was also used to address segmental joint dysfunction. She was treated three times during a two week period and her upper extremity symptoms resolved. During the next six months the patient did not experience any upper extremity pain or parasthesia, though she reported intermittent cervico-thoracic pain and stiffness associated with sitting at her computer and work-related stress. |
A male patient, 63-year-old, with no known comorbidities and a cocaine user, presented with over 1 year of classic triad of dementia, urinary incontinence, and ataxia march. The diagnosis of NPH was confirmed by CT scan, with Evans index of 0.62, as shown in . Therapeutic test (tap test) was positive for improvement of symptoms after 50 mL drain of CSF by lumbar puncture. He was submitted to ventriculoperitoneal shunt uneventfully. In our hospital, we routinely perform CT scans of the skull and abdomen as postoperative control, and both showed the entire system to be positioned properly [Figures and ]. However, on the second postoperative day, the patient presented with clinical worsening, with recurrence of early symptoms, and an abdominal collection was identified by superficial palpation. New abdomen CT identified the distal catheter protruding from the peritoneal cavity and housed beneath the subcutaneous tissue, producing a collection of CSF, as shown in . During further surgery to reposition the catheter, it was identified immediately below the skin after the incision, and no other pathological findings were noteworthy [Figures and ]. The shunt was otherwise functioning, however, there was a subcutaneous collection filled with a turbid CSF. New control CT showed the catheter well positioned in the peritoneal cavity [] and the patient symptoms improved. However, again 2 days after the last surgery, a superficial collection in the abdomen was identified, similar to the previous event. Another CT scan suggested the same event, with prolapsed and allocated catheter in abdominal subcutaneous and a collection of CSF, as illustrated in . New surgical repositioning of the entire DVP system was performed uneventfully, with appropriate control scans [Figures and ].\nThe patient was discharged on the fifth postoperative day of the second surgical shunt revision. No further problems were noted during regular follow-ups at the outpatient office, with significant clinical improvement in gait and bladder control, although with no marked cognitive improvement. CT scan showed reduction of brain ventricles globally. He had clean and dry operative wounds, functioning valve to palpation; and the abdomen flat, flaccid, and not painful on palpation. |
A 31-year-old woman with no significant past medical history presented to our emergency department complaining of a constant headache for the previous 4 days. The headache had begun approximately 6 h after receiving epidural anesthesia for labor. The documentation from the anesthesia service that day reported the use of a 17-gauge Touhy needle to enter the subdural space in the lower lumbar spine and the placement of a 19-gauge epidural catheter. No complications were reported with the procedure, and specifically, there was no mention of inadvertent dural puncture. The patient had an unremarkable delivery of a healthy infant at 38-weeks gestation later that day.\nThe patient described the headache as constant and occipital with some radiation to the frontal area. The headache was worse when upright and partially relieved in the supine position. She reported taking acetaminophen/butalbital/caffeine and ibuprofen with little relief. She had no associated vomiting, fever, or changes in her hearing or vision. She denied any photophobia or focal weakness or numbness. She was afebrile on physical exam, with pulse and blood pressure within the normal range. Her exam was notable for a normal neurologic exam including cranial nerves and no neck stiffness. The patient was tentatively diagnosed with a PDPH. After evaluation by the anesthesia service, she was admitted for pain control and possible placement of an epidural blood patch the next day. A computed tomography (CT) scan of her head was obtained prior to admission to evaluate for other possible causes of the headache (Fig. ). This CT identified bilateral parafalcine subdural hematomas measuring 7 mm in thickness on the left and 3 mm thickness on the right. There was no associated mass effect.\nThe patient was admitted to the intensive care unit and started on levetiracetam for seizure prophylaxis. Neurosurgical consultation advised observation, and a repeat CT scan of the head the next day showed no significant change in the hematomas. The patient also received an epidural blood patch the next day with no improvement in the headache. A head CT performed on hospital day 3 showed a decrease in the size of the hematomas, and the patient was discharged on levetiracetam for seizure prophylaxis for 1 week.\nISH occurring after dural puncture is extremely rare. Only sporadic case reports and a few small case series have described this condition [–]. Any procedure that results in spinal dural puncture will theoretically predispose to the development of an ISH. ISH has been described following epidural and spinal anesthesia, as well as lumbar puncture, myelography, epidural steroid injection, and after implantation of an intrathecal drug delivery device and a spinal cord stimulator [–]. The incidence of ISH specifically caused by epidural anesthesia used in obstetric practice has been estimated to be 1:500,000 [].\nThe same mechanism has been postulated for both PDPH and ISH []. The leakage of cerebral spinal fluid (CSF) from the dural puncture site may continue for several weeks, causing reduction in CSF volume []. This results in lower intraspinal and intracranial pressure, leading to relative ventricular collapse and caudal movement of the spinal cord and brain. As a consequence, the dura, pain-sensitive structures, cranial nerves, and subdural bridging veins are stretched. This may ultimately result in a tear of the bridging veins and consequently an ISH. Risk factors associated with ISH after dural puncture include excessive CSF leakage from multiple punctures in large needle use, pregnancy, coagulopathy, cerebral vascular abnormalities, dehydration, brain atrophy, and alcoholism [–].\nThe duration of time from dural puncture to the diagnosis of ISH ranges widely from 4 h to 29 weeks []. In one case series, 37% of cases were diagnosed within 1 week of dural puncture, and 85% were diagnosed within 1 month []. A headache, most often diagnosed as PDPH, is the main presenting symptom [, –]. Other reported symptoms and signs present at the time of diagnosis are listed in Table [, , ]. Reported rates of surgical intervention for ISH after dural puncture vary from 9 to 80% [, , ]. In general, surgical intervention for ISH is indicated if the hematoma thickness exceeds 10 mm, there is a midline shift of greater than 5 mm, or there is neurologic deterioration []. Furthermore, some have advocated for the use of epidural blood patching in the treatment of ISH caused by dural tears resulting in chronic CSF leaks [, ]. A full recovery is reported in over 80% of patients, with death reported in 7–10% of cases [–].\nHeadache in the postpartum period is common, occurring in 39% of women []. The majority of these headaches are benign primary headaches, such as migraine and tension type []. Secondary headaches in the postpartum period are typically due to obstetric or anesthetic complications, or the hypercoagulable state after delivery (Table ). Our patient was initially misdiagnosed as having PDPH, similar to many previous reports of this condition. PDPH is defined as a headache that develops within 5 days of dural puncture that significantly worsens soon after sitting upright or standing and improves after lying horizontally []. PDPH is more likely to occur in young women of low body mass as compared with other patients []. An epidural blood patch is considered the gold standard for treatment of PDPH, with a success rate of 70–90% []. Over 85% of patients report resolution of PDPH within 6 weeks regardless of treatment [].\nThe incidence of ISH after dural puncture is probably underreported since many of these patients are treated as PDPH with the eventual resolution of their symptoms. When to obtain brain imaging studies in the assessment of a likely PDPH is unclear. A reasonable approach would be to consider imaging in patients that (1) have a postural headache lasting more than 1 week, (2) do not improve or have worsening of their headache after an epidural blood patch, (3) report a change in the headache from postural to non-postural, or (4) develop other neurologic signs or symptoms with the headache []. |
A 45-year-old male with a previous history of tympanoplasty and functional endoscopic sinus surgery with septoplasty 10 years earlier presented to the ear, nose, and throat (ENT) clinic with several months of left moderate-to-severe otalgia and a sensation of ear blockage in his left ear accompanied by ipsilateral hearing loss. He gave a history of multiple failed ear wax removal in his left ear that had been performed at several ENT clinics, despite the use of alkaline ear drops.\nOn examination, the patient was comfortable and afebrile, and his vital signs were stable. Otoscopic examination of the left ear revealed impacted left ear wax covering the tympanic membrane, which could not be assessed. Otoscopic examination of the right ear also demonstrated mild ear wax, and the tympanic membrane was unremarkable. Oropharynx examination was unremarkable, the lymph nodes of the neck were not palpable, and all cranial nerves were intact upon examination. Nasal endoscopy revealed no pathologies. Ear wax removal under suction was attempted and failed. Another trial of removal after using alkaline ear drops for several days was also attempted but was unsuccessful. However, the surgeon became suspicious that the patient had KO rather than impacted ear wax because the wax was thick, had the appearance of keratin plugs, and was hard to remove after several attempts, despite the use of ear alkaline drops.\nBlood test results of the patient were within normal limits. The patient was planned for microscope-guided examination of the ears under general anesthesia. The examination revealed that the left ear was full of wax that was accumulating in the skin and contained a thick keratinous plug that had dilated the external auditory canal (EAC) with pockets and bone remodeling. Furthermore, the patient ear canal was circumferentially distended with a normal annulus. The tympanic membrane became visible and was intact. The keratinous plug was removed, and a diagnosis of KO was established (). An ear pack was draped with antibiotics and placed in the left ear. The patient was extubated, shifted to the ward without any complications, and discharged the same evening with the ear pack, which was removed after 3 weeks in the outpatient clinic. The patient was started on ciprofloxacin ear drops and analgesia for 1 week.\nIn the follow-up, the ear pack was removed, his hearing returned to normal level, and the pain disappeared. Pathological analysis of the removed plug revealed acellular lamellated keratin flakes and keratinous material (Figures and ), which confirmed our diagnosis. |
A case of a 40-year-old man who accidentally ingested a piece of metal which was about 10 cm in length is reported. Intermittent right lower quadrant abdominal pain was experienced for 2 days by the patient as recorded in the emergency department. On conducting an abdominal X-ray, the piece of metal was found in the right lower quadrant of the abdomen (). Obvious gas or effusion was not found in the abdominal cavity. Taking into account the disease process and imaging report, surgical treatment was needed immediately.\nUnder general anesthesia, the patient was placed in the supine position on the surgical table. The surgeon stood on the left side of the patient with the assistant standing on the same side. A 1.5 cm long incision was made within the umbilicus. The Veress needle technique was used to establish pneumoperitoneum and to keep the intra-abdominal pressure at around 13 mm Hg. A 10 mm trocar was placed in the umbilical for camera access. The piece of metal was identified about 10 cm away from the ileocecal region under visualization. The incision within the umbilicus was thereafter enlarged to about 3 cm, cutting the anterior sheath of the rectus abdominis. Two other 5-mm trocars were implanted in the same incision (). Thereafter, under the guidance of the laparoscopic camera, an approximately 2 cm longitudinal incision was made on the ileocecal wall. The piece of metal was successfully removed through the 2 cm incision (). The part of the incised ileocecal was then pulled out of the abdomen and anastomosis was completed with absorbable sutures. There was a little bleeding but no injury recorded to other organs under inspection. Thereafter, the incision was closed by meticulously using the running suture technique to avoid future complications and obtain good cosmetic results ().\nThe total operation time was about an hour, and intestinal function gradually recovered. The following morning after the operation, the patient showed no symptoms of abdominal distention or abdominal pain after having an all-liquid diet and was therefore later discharged 2 days into the post-operative period without any complications. After 6-month follow-up, no complications occurred and the patient was considerably satisfied with the cosmetic result (). |
The patient reported is a 32-year-old man with a past medical history of seizure disorder and a known psychiatric history of bipolar disorder with multiple inpatient hospitalizations for manic episodes during which he exhibited agitated and violent behavior. The patient has a comorbid use of synthetic cannabinoids. He was admitted to the acute inpatient service for a recurrent episode of agitation and threatening behavior in the community in the context of medication nonadherence and continuous use of synthetic cannabinoids. In the course of index hospitalization, the patient self-reported his coprophagic behavior during his initial psychiatric assessment. The patient stated that he believed that the consumption of feces was preventing him from going to jail and facing the death penalty for a murder that he claimed he committed in the past. He was unable to describe any specifics for his murder but felt that he deserved unusually cruel and harsh punishment from law enforcement that could only be prevented by consuming feces. This feeling of guilt and continued belief that he was deserving of cruel and harsh punishment induced his coprophagic behavior. He did not report any associated command auditory hallucinations but expressed depressed mood as he discussed his reasons for feeling guilty. His affect was however incongruent with his stated depressed mood. He was oriented to person, place, and time. Laboratory work-up was within normal limits and a urine toxicology panel (which does not detect synthetic cannabinoids) was also negative for illicit substances. He declined brain imaging for unclear reasons.\nThe patient was started on a course of risperidone 2 mg twice daily, by mouth, and divalproex sodium 750 mg orally twice a day for mood stabilization. He continued to display mood instability with multiple periods of agitation and aggression, as well as coprophagic behavior. His expressed reasons for coprophagia remained the same. He declined any psychotherapeutic interventions to address his excessive guilt and automatic negative cognitions. Over the course of a few days, risperidone was titrated to 6 mg daily in divided doses, with augmentation of his treatment with 200 mg every eight hours orally for mood stabilization/impulsivity in addition to divalproex sodium. The patient's mood symptoms improved after a few days on the current regimen; he became less irritable and easily redirectable, exhibited less impulsivity, and expressed more logical thinking process. His activities of daily living also improved, as he was less malodorous and exhibited improved grooming and hygiene. The patient's coprophagic behavior, however, did not resolve. He continued to express the same guilt and desire of unusually cruel and harsh punishments for his previous transgressions. He was discharged on the twenty-sixth day of admission with resolution of his acute manic episode. His coprophagic behavior and feelings of guilt did not show any resolution. |
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