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This 54-year-old female was referred to the Neurofibromatosis outpatient clinic of the Oral and Craniomaxillofacial Surgery Department for treatment of numerous cutaneous tumors that preferentially covered her trunk and extremities. On admission, the patient described the tumors to be painless and disfiguring. Besides the cutaneous tumors the patient sought advice for treatment of a painful region of her left small finger (Figure 1 ). For some time the ulnar side of the distal phalanx of this finger had become very touch-sensitive. There was neither a tumor visible nor any other pathological alteration of the skin. However, the finger could not be examined by palpation. The suspected diagnosis was initially a glomus tumor [] or a non-palpable nodular PNF.\nMRI at 3T revealed numerous, popcorn-like, contrast-absorbing, hyperintense formations on T2-weighted images, up to 1.2 x 1.9 cm² in size located on the palmar side of the left hand (Figure 2 ). These small lesions mainly affected the metacarpal bones II and III. MRI also showed a sharply defined lesion of max. 1 cm in diameter in the subcutaneous layer at the level of the radiocarpal joint and distal to first digit that reached to the tendons of the extensor musculature. The bony structures showed a homogeneous signal. The findings were interpreted as disseminated subcutaneous and cutaneous neurofibromas of the entire left hand. Structures were found on the distal phalanx of the left small finger whose intensity pattern corresponded to that of the other lesions.\nDuring surgery for the excision and vaporization of numerous neurofibromas of the trunk and extremities, the pain sensitive finger region was also explored. After incision of the skin, no tumor was visible. Therefore, a circumscribed subcutaneous excision was taken as a tissue sample and examined histologically.\nThe tissue findings confirmed cutaneous neurofibromas for the numerous nodules excised from the trunk and extremities. Surprisingly, there was no evidence for a glomus tumor in the tissue sample of the finger. Smaller neurofibromas were differentiable in this tissue sample. Within the corium as well as in the subcutaneous adipose tissue there were some clustered Vater-Pacini (VP) corpuscles. Next to the corpuscles several small nerve fibers were present. Diagnosis of VP neuroma was made (Figure 3 ).\nThe nerve fibers were positive for S100 and neurofilament. S100 immunoreactivity was especially strong in the center of the corpuscle. The perineurium of the nerve fascicles and the lamellae of the corpuscles expressed epithelial membrane antigen (EMA).\nThe healing process of the wounds was inconspicuous. The patient noticed that the sensations of pain had diminished significantly in the area of the small finger soon after surgery. |
A 42-year-old male patient had history of pain abdomen which he attributed to a mass per abdomen noticed since 15 days. Pain was intermittent, dull aching variety with symptom-free intervals. He also complained of weight loss. On examination, a mass of size approximately 15 × 10 cm was felt occupying the epigastrium and left hypochondrium. All the blood tests were normal. Chest X ray revealed elevation of left hemidiaphragm and Ultrasonography revealed a large heterogenous mass lesion of size around 18 × 15 cm arising from the liver occupying the epigastrium, left hypochondrium extending up to splenic hilum. Liver function tests were normal. CT abdomen showed a large lobulated, well-defined, heterogeneously enhancing mass lesion of size 18 × 14 × 13 cm from the left lobe of liver with multiple cystic, necrotic areas and compressing the stomach and spleen (). Upper GI Endoscopy showed external compression on the anterior wall of stomach.\nDifferential diagnosis of a primary hepatic adenoma and carcinoma was made. There were no risk factors for malignancy. AFP levels were normal. FNAC was not attempted due to risk of tumor seeding and haemorrhage. Metastatic workup was done and no metastases was identified. CT Chest, pelvis was normal. During laparotomy, mass was arising from liver (). The left lobe of liver was thinned out because of the tumor pressure effect. The tumor was adherent to the left hemidiaphragm and the upper spleen.\nEn bloc resection of the tumor mass and extended left lateral hepatectomy with resection of the part of left hemi diaphragm and upper part of spleen was done. Diaphragmatic rent was closed with mesh and splenorrhaphy was done ().\nAn ICD was also put in the left pleural space. Specimen was sent for histopathological department for examination. Grossly, cut section of the tumor showed a soft fleshy, friable mass with extensive foci of haemorrhage and necrosis ().\nMicroscopy showed a tumor with sheets of large pleomorphic cells with vesicular hyperchromatic nuclei showing plenty of mitosis, occasional lipoblasts, and thick capsule with tumor infiltration suggestive of pleomorphic liposarcoma (). Diaphragmatic tissue showed tumor remnants on the undersurface with focal infiltration and splenic tissue was normal with capsule showing infiltration.\nImmunohistochemistry\nTest result-NEGATIVE for S 100, CK, and EMA. Postoperative course was uneventful. The patient tolerated normal activity and enteral feeds. Chest tube was removed and discharged and he was referred to oncology department. Clinical stage: Stage III [T2b N0 M0 G3] as per AJCC staging. And hence the patient was put on adjuvant chemoradiotherapy and now is free of recurrence. |
A 63-year-old male with a past medical history of non-ischemic cardiomyopathy (ejection fraction 10%-15%), hypertension, chronic kidney disease stage G3A, and severe mitral valve regurgitation status post valve repair initially presented for an upgradation of his biventricular pacemaker to biventricular implantable cardioverter defibrillator (BIV-ICD) and replacement of non-functional right atrial lead. The pacemaker pocket was exposed and identified and the atrial and ventricular leads were pulled back under fluoroscopic guidance. A new left coronary sinus lead was placed. Next, right atrial (RA) and right ventricular (RV) leads were placed and access was obtained via the left coronary sinus as the left subclavian vein was found to be occluded with formation of collaterals. The leads were sutured in fascial planes via silk suture. The pacing and sensing parameters were confirmed. BIV-ICD was placed in the pocket, and the pouch was closed via vicryl sutures. The patient had electrocardiogram (EKG) (Figure ) and chest X-ray (Figure ) performed after the procedure. He had developed small pneumothorax from the procedure, which resolved on its own.\nThe patient was asymptomatic for the next seven months and then he developed increasing shortness of breath. At that point, the EKG (Figure ) revealed loss of appropriate pacing function. It was discovered that the patient kept massaging his pacemaker pocket site in the last seven months but he denied manipulating the device itself. He had also been moving his arm up and down frequently, which would have led all his device leads to be pulled up towards the device in a ratchet-like fashion. The patient was scheduled again for revision of BIV-ICD and on incision of the generator pocket, the left ventricular lead was found coiled up in the pocket beside the generator (Figure ). A new left ventricular lead was placed in the coronary sinus and then access was obtained via the right subclavian vein for right-sided leads. His right atrial and right ventricular leads were also found pulled up. A decision was made to place new right atrial and right ventricular leads. It is to be noted that all his device leads were undamaged and his device was found to be in the pocket in normal orientation. After securing the leads via sutures, the pacing and sensing function was analyzed. The pocket was revised and closed with vicryl sutures and the device was sewn to the pectoral fascia. A chest X-ray (Figure ) and EKG (Figure ) were obtained after the procedure to ensure the absence of pneumothorax and to document the appropriate functioning of the device, respectively. |
A 36-year-old man who denied previous systemic disease had a history of drug abuse with ketamine for 6 to 7 years (at a frequency of 2–3 times per week, by nasal inhalation, and hence the dosage could not be measured), and had then ceased use for approximately 4 years.\nHe had suffered from dysuria, bladder pain, and a mild burning sensation during urination, especially over the urethral meatus and the perineal region, for approximately 1 month prior to admission. He ignored these symptoms initially, but the burning pain worsened, with concomitant urinary frequency and urgency. He visited another hospital for help, at which routine urine analysis revealed pyuria. Under the impression of acute prostatitis, oral antibiotic treatment with ciprofloxacin was initiated during an outpatient visit; however, his symptoms remained, with no improvement. Two days before admission to our hospital, the symptoms worsened, with a newly-developed decreased voiding amount (approximately 50 mL per void) and urgency with urge incontinence, accompanied by painful hematuria and blood clot formation, especially at the first urine void of the morning. The patient then presented to our Emergency Department. Urine analysis showed pyuria, over 100 white blood cells (WBCs)/high-power field (HPF), and significant tenderness and swelling of the prostate was noted upon digital rectal examination, but no pus-like urethral discharge was seen. Under the impression of acute prostatitis, for which oral antibiotic treatment had failed, the patient was then advised to undergo hospital admission for advanced antibiotic treatment.\nAfter admission, we consulted an infectious disease specialist for evaluation, and antibiotic treatment with ceftriaxone was started immediately. A blood test revealed WBC 4870/μL without predominance of neutrophils or eosinophils. No marked elevation of serum C-reactive protein (CRP) (0.25 mg/dL) was noted. The patient's temperature after admission had remained within the normal range, and there were no accompanying signs or symptoms of toxicity. Several blood and urine cultures were performed, including tuberculosis, but all results were negative. The symptoms of pyuria (which remained over 100 WBCs/HPF), urgency, and painful hematuria persisted with no improvement after one week of intravenous antibiotic treatment. However, a sonogram performed upon admission revealed suspected bladder wall thickening. Due to the persistent symptoms that failed to respond to advanced intravenous antibiotic treatment for 1 week, abdominal computed tomography (CT) with contrast was then arranged. The CT scan showed asymmetrical wall thickening (thickness of up to 1.2 cm) of the anterior aspect of the urinary bladder with a mural nodule, and mucosal enhancement with perivesical fatty stranding (Fig. ). According to the above findings and the clinical symptoms, bladder cancer was highly suspected, and we discussed cystoscopy with bladder biopsy with the patient and his wife, which was then performed the next day. Prior to hydrodistension, the bladder mucosa presented with hypervascularity, but there was no tumor over the anterior wall of the bladder as seen on the CT scan. The bladder mucosa of the anterior wall was erythematous, with multiple hump-like changes, and several biopsies were performed. After hydrodistension at a pressure of 90 cm H2O for 8 minutes, the bladder capacity was approximately 150 mL, and bleeding over multiple aspects of the bladder was seen, with glomerulation and ulcerative changes (Fig. A and B). Pathologic analysis of the bladder biopsies showed erosive cystitis, characterized by denuded urothelial cells, with prominent infiltration by eosinophils, lymphocytes, neutrophils, and plasma cells over the mucosa and submucosal layer. In addition, hypervascularity and submucosal granulation formation with fibrosis were observed (Fig. A and B).\nAfter the operation, the bladder capacity increased a little, to approximately 70 to 80 mL per urination, but the urgency, frequency, nocturia, and hematuria still persisted. We also prescribed an anticholinergic agent, a beta-3 agonist and a nonsteroidal anti-inflammatory drug (NSAID), and the patient was then discharged. During 2 weeks of outpatient treatment, his symptoms did not improve with medication. Thus, we discontinued medical treatment and performed hyaluronic acid (HA) instillation, once a week for a total of 10 times. After the treatment, the symptoms of urgency, frequency and nocturia improved, and the bladder capacity increased to 350 mL per urination according to the patient's own voiding diary; in addition, no morning hematuria or hematuria after holding back urine occurred. After the patient's symptoms had improved, we arranged follow-up MRI of the bladder and cystoscopy; on the images, no thickening of the bladder wall nor nodules were observed (Fig. ). Cystoscopy showed marked improvement of the previously-noted erythematous bladder mucosa, and there was neither active bleeding nor glomerulation seen during the whole procedure. Bladder biopsy near the previous biopsy site was performed, and the final pathologic analysis showed decreased inflammatory cell infiltration, regeneration of the urothelium, and less vascularity (Fig. A and B).\nWritten informed consent to publish this case report was provided by the patient, and the consent procedure was approved by the Ethics Committee of Tri-Service General Hospital. |
A 47-year-old man was admitted to the hospital with pain and immobilization of his right shoulder. The symptoms started suddenly and were accentuated over a few days with fever up to 39.0°C and redness and swelling above the SCJ. The C-reactive protein (CRP) was elevated 48 times (391 mg/L) above limit value at the admission. The patient was nondiabetic, was nonsmoking for five years, had no substance abuse, had no other infections, had no signs of immune deficiency, did not take immune suppressing medication, and was otherwise healthy. Blood cultures showed Staphylococcus aureus, and antibiotics were targeted. He was screened for endocarditis, urinary tract infection, and infectious skin lesions with negative results. A computed tomography (CT) scan showed inflammation in the joint and nearby tissue, and an abscess measuring 2,5 × 4 cm. Ten days after debut of symptoms he had surgical drainage and vacuum assisted closure therapy for 3 weeks (). The joint was saved and no permanent damage was seen. Relevant antibiotics were maintained for 4 weeks.\nThe second case was a 64-year-old man presenting with pain from his right shoulder, left knee, and right ankle. The SCJ was swelled and sore. The CRP was elevated 17 times (141 mg/L) above value limit, and the temperature was 38.3°C at admission. The signs were misinterpreted as an attack of gout, since the patient had suffered from previous episodes with gout in ankle and knee-joint. The patient was nondiabetic, was a heavy smoker, had no substance abuse, had no signs of other infections, had no signs of immune deficiency, and did not take immune suppressing medication. The pain worsened and CRP increased over the next week and fluctuation above the SCJ appeared. Fluid aspiration from the knee and a urine sample both revealed Staphylococcus aureus infection, and relevant intravenous antibiotics were prescribed. Despite two weeks of antibiotic treatment, the symptoms continued. A CT scan showed inflammation in the SCJ and a light swelling in the nearby tissue. A puncture of the joint was attempted but failed to aspirate any pus, and a reactive arthritis was suspected instead of a septic arthritis. A positron emission tomography- (PET-) computed tomography after 36 days disclosed an infectious focus with formation of an abscess in the SCJ (Figures and ). The abscess measured 6 × 5 cm. The joint and head of the clavicle were eroded and the intercostal muscle was destroyed. The patient had surgical drainage. The cavity was treated with vacuum assisted closure for two weeks, and Aquacel for another two weeks along with four weeks of antibiotics. The infection vanished and the wound healed, but the SCJ is destroyed. |
Mr C is a 39-year-old white man with a past psychiatric history of depressive disorder (not otherwise specified), opioid dependence, and benzodiazepine dependence who was admitted to an inpatient psychiatric facility after a suicide attempt. Psychiatric diagnoses were assigned by the treating psychiatrist according to the Diagnostic and Statistical Manual of Mental Disorders, 4th edition criteria. On admission, the urine drug screen was positive for barbiturates (prescribed phenobarbital for maintenance of seizure disorder), benzodiazepines (alprazolam abuse 2 days before admission), opioids (heroin use day before admission), and cocaine (last use the day before admission). He denied history of complicated withdrawal or withdrawal seizures. His past medical history was significant for nephrolithiasis as a young adult (last episode 20 years ago), chronic low back pain, and a baseline seizure disorder secondary to repeated concussions. On admission, Mr C reported taking gabapentin 600 mg orally 4 times daily, phenobarbital 162 mg orally twice a day, escitalopram 20 mg orally daily, and aripiprazole 10 mg orally daily. He reported medication nonadherence to escitalopram and aripiprazole for 4 months before admission.\nMr C was admitted and reinitiated on home medications. Escitalopram and aripiprazole were ordered for treatment of depressive symptoms but initiated at lower doses because of medication nonadherence. For benzodiazepine and heroin dependence, he was initiated on inpatient withdrawal assessment protocols on day 1 of admission and continued for 8 days on diazepam and 7 days on buprenorphine/naloxone, respectively. The patient denied any history of taking alternative antidepressants to treat chronic low back pain and depression, and expressed interest in this treatment approach. Therefore, escitalopram and aripiprazole were discontinued on day 5 of admission, and duloxetine was initiated on day 6 to target chronic low back pain and depression. The next day, Mr C reported pain in his scrotum and a persistent, painful erection that occurred within hours of his first dose of duloxetine 30 mg.\nPer patient report, the erection persisted for over 2.5 hours and subsequently subsided without treatment. He also reported that during this time he was unable to urinate and experienced testicular swelling and unilateral flank pain. The patient denied any history of priapism in the past, including while taking escitalopram as an outpatient. After the priapism resolved, physical examination by an internal medicine physician revealed no evidence of urethral discharge or lesions. He was noted to have marked scrotal erythema and warmth as well as tenderness to light palpation diffusely. Testes appeared to be normal size, and the penis was flaccid. There was presence of tender right-sided flank pain to deep palpation and percussion, and his range of motion was limited by pain in his back and scrotum. A urinalysis was done at the time of symptoms, which revealed 3+ urine blood, 1 squamous epithelial cell, and 540 red blood cells. Duloxetine was discontinued after 1 dose, and the patient had near complete resolution of adverse effect symptomatology by the following day, with the exception of unilateral flank pain, which decreased over the course of 3 days. Given the rapid improvement in GU symptoms after discontinuation of duloxetine, further workup to rule out nephrolithiasis was not performed. The patient was restarted on escitalopram and had no further complications during his hospitalization. |
A 24-year-old male patient referred to the department for opinion regarding radiating head and neck pain. Patient's complaints were on and off pain over neck region radiating to shoulders and arms for the past 5 years, headache and giddiness on the side to side neck movements, difficulty and pain while swallowing, and foreign body feeling in the throat. Previous history of any surgery and trauma was unremarkable. On examination, all third molars were completely erupted in proper occlusion and no abnormal finding was present on temporomandibular joint (TMJ) examination, but tenderness was present bilaterally in tonsillar fossae region on palpation. There was no evidence of any palpable mass in neck. Based on clinical findings, presumptive diagnosis of neuralgic pain and elongated SP (Eagle's syndrome) was made. Patient was preceded with a panoramic radiograph, which revealed bilateral elongated SP [] and TMJ morphology was also normal. After evaluation of panoramic radiograph, patient was subjected to computed tomography (CT) scan to obtain accurate information about SP including its length and direction as superimposition of several anatomical structures, distortion, and magnification are the drawbacks of conventional radiographs. Coronal section of CT scans showed bilateral elongated SP reaching upto the hyoid bone []. Right lateral view showed segmentations present along the elongated SP and left lateral view showed uninterrupted elongated SP [Figures and ]. 3D-CT reformatted image better defined anatomy and measurements were done using a software tool which gave length of 6.8 cm on left side and 6.7 cm on the right side []. Serum calcium and phosphorus levels were within normal limits. So after seeing imaging findings, confirmative diagnosis of Eagle's syndrome was made. Since the patient had persistent pain and giddiness, however, possible causative factor for giddiness could be impingement on carotid arteries, surgical removal of bilateral elongated SP was planned and was done through extra-oral approach on left side and intraoral approach on the right side after performing tonsillectomy. Approximately, 4.5-5.0 cm of the segment was removed on both the sides []. Patient was observed and was relieved of the symptoms after 1 week. Post-operative CT scan was advised after 40 days []. Patient was completely symptom free after 2 months and is under regular follow-up. |
A 34-year-old G6P2122 at 18 weeks of gestation with a history of 2 prior cesarean deliveries was transferred to the Rwanda Military Hospital (RMH) in Kigali, Rwanda, in hypovolemic shock. She had one prior antenatal visit but no ultrasound was done. Her history was also remarkable for pregnancy on IUD contraception. She was seen at a health center with a history of sudden onset severe abdominal pain followed by general body weakness and was referred to a district hospital where ultrasound showed miscarriage of an intrauterine twin pregnancy at 18 weeks of gestation and an additional intra-abdominal fetus dead as well. They suspected uterine rupture and transferred the patient to RMH after initiating IV fluids and transfusion. Travel time was approximately 3 hours, and she reached RMH 12 hours after the onset of symptoms in hypovolemic shock with tachypnea and tachycardia of 144 bpm. She was pale with a distended abdomen and rebound tenderness. Point of care ultrasound showed an intrauterine twin pregnancy with no cardiac activity for both fetuses. A third fetus was seen outside the uterus without cardiac activity and there was free fluid in the abdomen. Results of the full blood count (FBC) at admission came with Hb of 11.6 g/dL, WBC of 28.31 × 103/μL, and Plt of 280 × 103/μL. The patient was immediately taken to the operating room for explorative laparotomy, and consent for possible hysterectomy was signed. Intraoperatively, approximately 4 L of blood and clots was aspirated. A right side interstitial ruptured pregnancy with a dead male fetus in the abdomen with its placenta still attached to the ruptured site was found (). The uterus was not reparable because of the extent of the cornual rupture, and a subtotal hysterectomy was performed. Postoperatively, the uterus was opened and a monochorionic-diamniotic twin intrauterine pregnancy was noted (Figures and ). The patient received blood products intraoperatively and postoperatively. She fully recovered from anesthesia and was discharged from the hospital on her 4th day post operation. |
A 20-year-old female presented to our center with a telangiectatic osteosarcoma of the humerus. There was no history of malignancies within the family.\nMRI examination and open biopsy were performed by the previous physician. At the time the results were thought to be malignant lymphoma. After several diagnostic trials, the patient visited our center to seek advice about her telangiectatic osteosarcoma (A).\nWe observed a circumferential mass on the distal part of the right arm with a slight deformity of the arm, with marked venous engorgmnt and distal edema. The mass was warm and solid on palpation. Function of the right hand was still preserved. From the laboratory findings, there were marked elevation of the alkaline phosphatase and lactate dehydrogenase. From humerus X-ray, there was mixed lesions along the humerus with pathological fracture on the midshaft (A). T2-weighted MRI showed iso-hyperintense and expansile lesions along the humerus (A). For the metastatic workup, chest X-ray showed no coin lesions or metastatic characteristics. Patient also had a PET scan and the result was unremarkable. From the Clinico Pathological Conference (CPC) forum, it was concluded that the diagnosis was osteosarcoma of the right humerus stage IIB according to the Enneking classification.\nPatient took neoadjuvant chemotherapy regimen with Doxorubicin and Cisplatin for three cycles from January to March 2016. At the end of the third cycles, clinical and radiological evaluations were performed. Clinically the mass was not getting bigger compared to before chemotherapy (B). From x-ray, the mixed lesions became more marked compared to the previous x-ray (B). MRI showed that the mass was slightly became larger compared to the previous MRI with no involvement of neurovascular bundle (B).\nSix months after the initial complaint, the surgery was performed. We used extensive deltopectoral approach with anterolateral extension through the proximal part and curved backward to complete the posterior distal humerus and elbow approach. This approach was used because of the previous biopsy tract was in the posterior aspect of the distal humerus. The vascularity, rotator cuff tendons, biceps and triceps muscle, and majority of the nerves (musculocutaneous, radial, median, and ulnar nerves) were spared during the total resection of the humerus. Axillary nerve was sacrificed during the tumor resection. To reconstruct the humerus, both long shoulder hemiarthroplasty and total elbow prostheses were used. These two prostheses were joined using two long one-third tubular plate that worked as the long stem augmentation. Extension cerclage wire was used to make the implant as one unit (A). Finally, the stem was augmented with a bone cement from proximal through the distal. Prolene mesh then was sheathed to the bone cement, and then the preserved rotator cuff tendons and biceps and triceps muscle were sutured back with the Ethibond sutures (B). The resected tumor and humerus were then sent to the lab for histopathologic analysis (C).\nFrom the post-operative x-ray, the modified prosthesis sat well on the shoulder and elbow joint (). Function of the hand was excellent post-operatively. Patient also could immediately flex her elbow. Post-operative histopathological examination showed telangiectatic osteosarcoma with HUVOS IV (). After the wound healed without complication, patient underwent adjuvant chemotherapy. Two months after the surgery, patient could start writing with her right hand without marked difficulties. Further follow up of three years post-operatively, patient already came back to work and were able to perform daily activities without difficulties. Patient’s shoulder abduction and elbow flexion was shown on the pictures (). The MSTS functional score for the upper limb scored 83% which was excellent. There were no post-operative complications and the immunohistochemical workup used to rule out lymphoma (CD20, CD15, CD 30, and CK) would be planned. |
A 67-year-old Japanese woman complained of acute-onset abdominal pain and vomiting (Glasgow Coma Scale score = 14). Her vital signs were largely stable, apart from tachycardia with atrial fibrillation (Af) that had never been treated. The laboratory data revealed acidosis, inflammation, and elevated lactate and creatine phosphokinase (CPK) levels. The contrast-enhanced computed tomography (CT) imaging revealed that the SMA was occluded by a thromboembolism. Because of her pre-existing Af, she was diagnosed with embolic occlusion of the SMA and intestinal necrosis. She was treated with an emergency enterectomy (Figure ). The pulse of the SMA disappeared, and a relatively fresh thrombus was observed in the stump of the resected intestines. Anticoagulation therapy with heparin was initiated after the laparotomy.\nPost-operatively, she developed methicillin-resistant Staphylococcus aureus (MRSA) colitis and sepsis. Due to her respiratory insufficiency and shock conditions, she was treated with ventilator support. Although antibiotics resolved her MRSA colitis within 3 weeks of the laparotomy, she began to gradually develop gangrene in her right hand.\nThe three-dimensional CT images showed that her right brachial artery was occluded 5 cm proximal to the elbow joint (Figure B); however, we could detect arterial pulsation 15 cm proximal to the elbow joint. We diagnosed her with an acute brachial arterial occlusion associated with an SMA embolism, and 6 weeks after the emergency laparotomy, an amputation was performed 15 cm proximal to the elbow joint. We did not observe any severe atherosclerotic changes in the brachial artery. The pulsation of the brachial artery disappeared at the level of the bone transection, and the arterial lumen was filled with an organized embolus (Figure B).\nAn abdominal CT scan revealed multiple infarctions of the spleen, indicating a shower embolism. Anticoagulation therapy was re-started after the amputation to prevent recurrent or new emboli. She was able to ambulate independently 2 months after the amputation. She used a prosthetic upper limb for 12 months after the amputation, at which time she died of malnutrition resulting from the enterectomy. |
A 41-year-old female presented to the emergency department with a four-day history of signs and symptoms indicative of abdominal obstruction. Her past medical history included hypothyroidism due to autoimmune thyroiditis, managed with levothyroxine. In 2006 she underwent a right hemicolectomy for a myxoid liposarcoma grade 1-2 infiltrating the small and large bowels. The specimen weighed almost 3 kilograms with dimensions 25 × 22 × 11 cm. The removal was radical with free margins and an intact tumour capsule. Postoperatively, no adjuvant therapy was indicated and close followup was recommended.\nDuring the current admission she was dehydrated, haemodynamically stable, and apyrexial. Abdominal examination revealed a distended abdomen, tympanic to percussion, with no signs of peritonism or abdominal wall hernias. Blood investigations showed a raised urea (8.7 mmol/L) and WCC 14.2 × 109. Abdominal X-ray revealed grossly dilated small bowel loops. The initial treatment was conservative with nil by mouth, intravenous fluid resuscitation, nasogastric tube, and urinary catheter for fluid balance. After twenty-four hours the patient showed no signs of improvement and underwent an exploratory laparotomy. An adhesional band was found to be the cause of the small bowel obstruction and was divided. The entire small bowel was viable and no evidence of intra-abdominal or peritoneal metastasis was identified. However, a dark purple, smooth pelvic mass was found attached to the pelvic wall by a small stalk with similar macroscopic appearance to that of splenic tissue (). The mass was carefully detached off the pelvic wall and sent for final histological analysis.\nAfter the laparotomy the patient had an uncomplicated recovery and was discharged home on the eighth postoperative day. Histology confirmed a recurrence of the previous myxoid liposarcoma (). One month later a CT scan of the chest, abdomen, and pelvis showed a new well-defined oval hypodense mass in the right iliac fossa adjacent to the anastomotic surgical sutures site that was suspicious for recurrence (). In light of these findings, the patient has been referred to the regional sarcoma centre for further management. |
The patient was a 42-year-old man with a 7-year history of recurrent metastatic HPC. A CT scan during a physical examination in 2002 revealed a mass in the patient’s liver. He received a complete surgical resection of the mass that was pathologically and immunohistochemically confirmed as HPC (). No lymph node or distant metastasis was found, and the disease was labeled as stage I (G0TxN0M0).\nOne year later, the patient was admitted because of head injury in an accident. He was found with metastasis in the brain and subsequently underwent a resection of the metastasis as well as sequential radiotherapy.\nAfter that, residual tumors in the liver recurred twice, in 2004 and 2008, respectively, for which the patient received interventional therapy and radiation treatment.\nIn March 2009, a CT scan revealed a mass of 4.5 cm × 3.5 cm in the left kidney, multiple metastases in the lungs and both adrenal glands, and multiple small lymph nodes of the mediastinum. The patient underwent a complete excision of the recurrent tumor which was proved to be well-differentiated HPC. And then the patient was treated with a regimen of doxorubicin (ADM), dacarbazine (DTIC), vincristine (VCR), ginsenoside Rg3, and recombinant human endostatin (Rh endostatin) for four 21-day cycles. ADM and VCR were administered on day 1, DTIC on days 2 to 6, Rh endostatin on days 0 to 6, and ginsenoside Rg3 on days 7 to 20. The patient also received synchronous radiofrequency hyperthermia in both lung lobes during the course of treatment.\nA CT scan after 2 cycles showed reduction of masses in the left kidney as well as stable lesions in the right kidney, both lungs, and liver. A CT scan after 4 cycles revealed a partial response in the left kidney () and stable disease in the right kidney, both lungs, and the liver (). The disease was assessed as partially responding to the therapies.\nA special symptom was noticed during the course of the disease. The patient often felt hungry which would be relieved temporarily after eating. The patient experienced no conspicuous side effects, although biochemical tests showed hypoglycemia and pigmentation of the skin, especially in the hands and feet. The patient was at a good status as of this writing. |
A 39-year-old Caucasian man was referred to our emergency department due to a sudden loss of hot and cold sensation when taking a shower, followed by a progressive instability and weakness of his lower extremities. Our patient also complained of diplopia, dim vision and circumoral numbness. Our patient reported an upper respiratory tract infection for which he was being treated with an antibiotic (macrolide). Neurologic symptomatology began a few days later. His medical history was unremarkable apart from seasonal asthma. He used to smoke 20 cigarettes per day and drink alcohol only on social occasions. He was the father of two children. No high-risk sexual behavior was reported.\nOn physical examination, he had hot and cold temperature sensory disturbance under the T4 vertebrae level, symmetrically diminished muscle power mainly to his lower limbs, blurred vision and a loss of taste. Further neurological examination revealed paresis and diminished reflexes of his lower limbs; mainly the jerk ankle and patellar reflex. Cerebral pathology results following an MRI scan were negative. However, our patient reported a loss of balance, a failure to stand or walk and numbness. In summary, our patient showed progressive weakness of his lower limbs due to neuropathy, areflexia, sensory involvement and cerebella ataxia, and with the duration of the disease being less than four weeks, the required and some of the supportive diagnostic criteria for GBS were met. Other physical examinations were normal.\nTwo lumbar punctures were performed, the first on admittance and the second 14 days following commencement of the symptoms (Table ). The diagnostic tests are summarized chronologically in Table . Serology tests for neurotropic viruses were requested for differential diagnosis purposes. Hepatitis, human immunodeficiency virus (HIV) and varicella zoster virus (VZV) test results were negative, as were results for West Nile virus (WNV) with indirect enzyme-linked immunosorbent assay (ELISA) immunoglobulin G (IgG) and M antibody capture (MAC)-ELISA (IgM). However, CMV, herpes simplex virus (HSV) and EBV IgM antibody test results were positive. Blood CMV antigen and polymerase chain reaction (PCR) tests for CMV were negative, as was the PCR test for CMV from the cerebrospinal fluid (CSF). Blood, urine and CSF cultures were sterile. A tuberculin skin test and CSF culture for tuberculosis (TBC) were negative. Serology tests were repeated one month after hospital admittance and at a six month follow-up appointment. The comparative results are summarized in Table .\nResults of a chest X-ray were negative. A brain MRI scan showed no abnormalities of the cerebral parenchyma, but a slight inflammation of the ethmoid and right mastoid sinus was noted. In view of the neurological findings, his jugular and lumbar spinal column were scanned. No abnormalities except degenerative lesions and slightly slipped disks were noted, but these findings were irrelevant to the clinical symptoms. Brain, lung and abdominal computed tomography showed no abnormalities.\nElectromyography gave a mildly diminished activity of the examined muscles; the palpebra sphincter (right and left), first inter-osseus dorsalis muscle (right) and anterior tibialis (right). After infra-orbital nerve stimulation, no R1 responses were received bilaterally. Retarded R2 responses were received, while the R2 response of the left side was significantly lower. Electroneurography indicated absence of an f wave when his right median, ulnar and peroneal nerves were stimulated. Absence of an f wave was also observed when his left tibial nerve was stimulated, indicating a mixed demyelinative polyneuropathy with a conduction blockade in multiple nerves and mild axial damage.\nOur patient received 40 g intravenous Immunoglobulin for a duration of five days based on clinical suspicion. Notably, the CSF albumin became elevated (1 g/dL) only after the second lumbar puncture was performed two weeks after the onset of symptoms. Our patient received an additional 40 g of intravenous Immunoglobulin a few days later, for a duration of three days, in an attempt to ameliorate the final clinical outcome. Relapse can occur in patients who are treated early in the course of GBS and improve, and brief retreatment with the original therapy is usually effective in these cases [,]. On presentation with positive CMV IgM antibody test results, we applied an anti-CMV regime with ganciclovir intravenously 500 mg twice daily. The decision was taken to try to ameliorate our patient's neurological condition, which required him to remain resting in bed as any change in position caused inconvenience and a sense of falling. Intense physiotherapy was applied soon after diagnosis. Our patient markedly improved within two weeks. At that time, while still under anti-CMV treatment, he was able to sit up in bed and on a chair. Later on he was able to stand with support and also progressively take a few steps. After improvement of his clinical condition, our patient was discharged with appropriate follow-up. |
A 26-year-old man who presented with abdominal pain and vomiting was transferred to our hospital seven days after the onset of the symptoms. The patient had no history of abdominal surgery. On arrival, his vital signs were normal. Abdominal findings revealed distension and tenderness in the entire abdomen without any muscular defense signs. Laboratory examinations showed a white blood cell count of 7420/mm3, a C-reactive protein level of 0.3 mg/dL, and all other measured values within their normal limits.\nAn abdominal X-ray examination showed a dilatation and an air-fluid level of the small bowel. Abdominal computed tomography revealed an extensively dilated small bowel and a closed loop of small bowel in the mesosigmoid (). There was no blood flow obstruction at the closed loop of the small bowel. The patient was diagnosed with an intestinal obstruction due to an incarcerated internal hernia involving the mesosigmoid. An ileus tube was inserted to decompress the small bowel prior to an elective single-incision laparoscopic surgery that was performed six days after admission.\nThe operation was performed using a 2.0 cm transumbilical vertical incision. The fascia and peritoneum were then vertically opened, and three 5 mm trocars were inserted into the abdomen to introduce endoscopic instruments. Carbon dioxide was used to inflate the abdomen at 8 mmHg pressure. The operator used two 5 mm forceps, and the first assistant handled a 30 degree 5 mm laparoscope (Karl Storz, Tuttlingen, Germany). During the surgery, it was observed that the ileum had herniated into the intersigmoid fossa. Furthermore, there were mild adhesions of the herniated ileum at the hernial orifice (). The incarcerated small bowel was reduced after adhesiolysis. Based on the intraoperative findings, the patient was diagnosed with an intersigmoid hernia. The defect in the mesosigmoid was approximately 3 cm, and the length of the incarcerated small bowel was approximately 10 cm. We confirmed that incarcerated small bowel was viable (). The intersigmoid fossa was then closed with interrupted sutures (). The patient had an uneventful recovery and was discharged on postoperative day five. |
A 38-year-old African American female presented to the emergency department for chest pain. The patient's past medical history was significant for hypertension, for which she had not taken her lisinopril for about 2 years, and diabetes, controlled with diet. Additionally, the patient had a history of smoking for 20 years and had quit about 3 months prior to admission; however, 1 week prior to the onset of chest pain, the patient was started on oral bupropion 150 mg every morning for smoking cessation for tobacco cravings. In the emergency department, the patient received aspirin chewable 162 mg by mouth and 3 sublingual nitroglycerin tablets, which relieved the chest pain, and was admitted to the general medicine floor in order to rule out an acute coronary syndrome. The next day, prior to her stress test, the patient went to the cardiology clinic, where she became confused and began talking about demons and auras. The patient's husband noted that this behavior was strange, and the patient was escorted back to the medical unit. On the medical floor, the patient was requesting to be examined by a female because having a male examine her would be “inappropriate” because “together (they) are the body of Christ.”\nThe patient was evaluated by psychiatry, and the patient's husband provided further insight into the patient's bizarre behavior. According to the patient's husband, the patient started acting strange when she woke up the morning before coming to the emergency department. She was hyperverbal, religiously preoccupied, and distracted with flight of ideas. The husband also reported that the patient had similar manic symptoms about 5 years prior after using cannabis and was hospitalized for a brief period at a private hospital. Per the husband, the symptoms resolved, and the patient was discharged with a diagnosis of schizophrenia. The patient was treated with oxcarbazepine 300 mg per day, risperidone 1 mg twice daily, and fluoxetine 20 mg daily, and it appears that the patient took these medications for approximately 6 months following this previous hospitalization. Because of the current sudden changes in mood, the patient was admitted to the inpatient mental health floor for mood stabilization. While in the inpatient psychiatry unit, the patient continued to exhibit behaviors consistent with manic delirium, such as agitation, hypersexuality, and combativeness, requiring multiple doses of intramuscular haloperidol 5 mg and lorazepam 4 mg, given as needed. The patient was started on lithium 600 mg twice daily, and appropriately drawn lithium trough levels increased up to 1.74 mEq/L due to acute worsening of renal function. However, the patient was without side effects or any resolution of symptoms. The patient's mania and psychosis continued to worsen despite trials of haloperidol 10 mg twice daily and risperidone 1 mg at bedtime, and ultimately the patient required restraints as she was no longer responding to intramuscular haloperidol 10 mg, lorazepam 2 mg, or olanzapine 10 mg. After 10 days of admission on the mental health unit, lack of response, and dangerous behaviors, the psychiatry team resolved to perform emergency ECT. After the first ECT treatment, the patient remained agitated and delusional and continued to require restraints and as needed haloperidol and lorazepam. She received her second treatment of ECT the following day, which she tolerated.\nThe psychiatry team resolved to admit the patient to the intensive care unit (ICU) for sedation to avoid injury with a goal of 24 hours without restraints and intravenous medications before being transferred back to inpatient psychiatry. The patient was admitted and placed on a dexmedetomidine infusion with haloperidol and lorazepam as needed for agitation, which the patient continued to need during the initial ICU course. The patient's third ECT resulted in some improvement in her behaviors. The patient remained in the ICU for a total of 6 days, and during the last 2 days of the ICU stay, the patient was not sedated and did not require any medication or restraints. The patient was stable and not psychotic or manic, so she was discharged home after her fourth ECT with instructions to follow up with outpatient psychiatry. Postdischarge, the patient received 4 more ECT treatments. Three months postdischarge and 2 months post-ECT, the patient remained stable without any pharmacologic treatment and only psychotherapy. |
A 16-year-old male complained of inability to flex his left elbow since 1 year prior to admission. One and a half year before, he fell down and hit his elbow during football practice. He felt pain and there was swelling on his elbow. However, he didn't seek for medical treatment. He had his elbow massaged every week for 5 months but there was no improvement. His elbow became fixed in extended position. A month later, he went to an orthopaedic surgeon and underwent x-ray examination which revealed a fracture and dislocation on his left elbow. He was then referred to our institution for further treatment.\nFrom clinical examination, range of flexion-extension of the elbow was 300-00 with normal pronation-supination. There was no neurological deficit (). From radiological examination, there was a malunion of medial epicondyle with subluxation of left proximal ulna (). From 3D CT reconstruction, there was a deformity and malunion fracture in humeral capitellum with radial and ulnar postero-superior dislocation (). The patient was diagnosed with extension contracture of the left elbow due to malunion of left capitellum, neglected dislocation of the radiohumeral joint, and neglected dislocation of the ulnohumeral joint. The patient was scheduled to have a contracture release, open reduction and internal fixation, and ulnar interposition.\nIntraoperatively, we did a posterior approach to the elbow. The ulnar nerve was identified and preserved. The fibrotic tissues and heterotopic ossification were excised. We did a contracture release and open reduction and internal fixation using K-Wire. The flexion and extension of the elbow were evaluated and we managed to get 300 - 130° of flexion-extension ROM. Afterwards, ulnar interposition was performed to prevent ulnar impingement. The wound was closed and a single drain was placed. The elbow was immobilized with back-slab in 900 flexion position for two weeks.\nAfter 1 week, the patient went back to our hospital for follow-up examination. In the 1st evaluation, we tried to remove the back slab and moved the elbow passively. The movement is restricted due to pain and the patient went back home with the back slab on. In the 2nd week follow-up, we permanently remove the back slab and the stitches. At that time, the pain still persisted and the patient was planned to have physical rehabilitation.\nOn the 4th week after surgery, the surgical wound was infected. We performed debridement, implant removal, and manipulation under general anesthesia. Two weeks later, patient came back to our hospital. We removed the stitches and started rehabilitation. Later on, he continued his rehabilitation in his previous hospital.\nAfter 6 months, he visited our outpatient clinic for medical checkup. From physical examination, the elbow flexion-extension ROM was 1100 - 300 (). The patient is able to do normal daily activities (). |
A 4-year 7-month-old boy and his parents were referred to the Pediatric Dentistry Postgraduate Program Clinic in June 2016, requesting dental treatment due to multiple dental caries cavities, local infectious processes, and associated pain. Two years previously, the patient had been diagnosed with early infantile GS, confirmed on the analysis of the beta-GAL both in peripheral blood leucocytes and in cultured skin fibroblasts (sequencing of the CTSA gene was not carried out). Previously, the child was insufficiently treated by a pediatric dentist, due to the child's very poor level of cooperation. Only the upper right anterior segment was treated: a pulpectomy procedure on the lateral incisor and extraction of the root remnant of the central incisor. However, the patient did not continue the treatment.\nMedical and dental history revealed that when the child was 1 year of age, his parents noticed the existence of a mild soft outpouching swelling in his lower abdomen, which progressively increased in size. The patient was evaluated at a local public hospital, and the condition was diagnosed as peritoneal ascites, together with three abdominal hernias, due to enlarged liver and spleen.\nAt the moment of the patient's first dental visit, the presence was evident of a huge abdominal growth due to ascites (). According to the treating medical team, this anomaly was unable to be surgically repaired. Because of the significant swelling, the patient had difficulty in maintaining a straightened body posture, and he could not be adequately positioned on the dental chair. In addition, the patient manifested mild mental retardation, language delay, severe bilateral hypoacusia, hepatic damage, and bilateral hydrocele (swelling in the scrotum).\nThe patient's head exhibited a squared form, coarse face, and short neck. The facial profile was markedly convex with an increased lower third, retrusive chin, protruding maxilla, closed nasolabial angle, and manifested lip incompetence (mouth permanently open) (). Intraorally, the examination showed both arches with interdental spacing, carious cavities in all primary molars, a root remnant of the upper left lateral incisor with related abscess fistula and gingival swelling, and macroglossia associated with an evident anterior open bite (). Oral hygiene was very poor, and halitosis was significant.\nThe programmed treatment plan consisted of the placement of composite restorations, pulpotomies and preformed metallic crowns, and extraction of the root remnant. Due to the greatly reduced level of cooperation exhibited by the patient (rated as Frankl's scale level I, definitely negative), it was not possible to obtain X-rays. The patient was very fearful, with clear evidence of treatment refusal, forceful crying, and extreme negativism. Therefore, it was decided to start the treatment with an oral examination, dental prophylaxis, topical fluoride-varnish applications, and the teaching of tooth brushing. Traditional behavioral management techniques, such as conditioning, desensitization, “tell-show-do,” and positive reinforcement, were persistently employed. On the other hand, the patient was unable to maintain a supine or horizontal position on the dental chair due to pain caused by the abdominal hernia. Thus, the patient was approached when he was in a 90-degree seated position, with the aid of his mother. However, all these efforts were unsuccessful. Then, it was decided to treat the patient under general anesthesia, in agreement with the parents, who signed a special informed consent document.\nThe patient was managed according to the American Academy of Pediatric Dentistry (AAPD) guidelines on sedation and general anesthesia. First, the child was sent to the pediatric anesthesiologist for a physical examination and a presurgical health and risk evaluation; respiratory, cardiovascular, and gastrointestinal systems were exhaustively assessed, and blood and urine laboratory tests were indicated; only coagulation times appeared slightly increased. The patient was classified as American Society of Anesthesiologists (ASA) physical status classification III, with lower pulmonary capacity, limited open aperture, macroglossia, and challenging airway access due to decreased diameter. The parents were instructed, through printed guidance, regarding their child's eating and drinking on the day prior to the intervention.\nThe surgical intervention was carried out in August 2016 at the university hospital. After placing routine monitors, according to the American Society of Anesthesiologists standards, general anesthesia was induced via facemask with inhaled fentanyl, lidocaine, propofol, rocuronium bromide, and sevoflurane. Supplemental local anesthesia was also provided at the site of the root-remnant extraction. The extraction site was fully sutured with fine absorbable 6-0 Dexon in order to prevent a potential hemorrhagic episode. The whole surgical procedure lasted approximately 2 hours and ensued without complications. However, the extubation procedure was not possible due to respiratory restriction, and the patient was subsequently transferred to the pediatric intensive care unit (PICU). After 4 days under pharmacological management (dexamethasone, metamizole, ephedrine, clindamycin, and midazolam), together with assisted mechanical ventilation, the extubation could finally be performed. The patient was remitted to the pediatric area, where he was maintained with oxygen nebulization; the case proceeded uneventfully thereafter. He was discharged from the hospital 2 days later.\nThe patient was evaluated at our clinic 15 days after the intervention conducted under general anesthesia. Restorations were found to be in place adequately, and the cicatrization process at the extraction site was uneventful. Then, an individualized oral preventive program was initiated, including dental hygiene practice with a fluoridated paste (1,450 ppm), topical fluoride varnish, MI Paste Plus® applications, and diet counseling. Since then, the patient has been reviewed closely, every month; at each of the visits, the previously mentioned behavior modification techniques were applied in depth. The last control appointment took place in mid-November 2017, during which an excellent oral condition was observed. Currently, the patient is considered a poor candidate for treatment with orthodontic appliances, particularly for treating his anterior open bite. In the meanwhile, the eruption process and occlusal development will be continuously assessed. |
A 69-year-old woman with a chief complaint of hematochezia and anorexia that had been present for one month was admitted to the hospital. Physical examination and blood test results revealed nothing of note except for an elevated carcinoembryonic antigen of 24.7 ng/mL. Colonoscopy revealed that a type 1 tumor, i.e., a polypoid-type tumor, was located in the sigmoid colon, and an elevated lesion with redness was located in the appendiceal orifice (). The tumor was biopsied and diagnosed with well-differentiated tubular adenocarcinoma, which was suspicious of primary sigmoid colon cancer. The elevated lesion in the appendiceal orifice had redness on its top surface, but biopsy was not performed there because it was not suspected to be malignant due to the apparently normal mucosa. A computed tomography (CT) scan showed, however, an appendiceal mass that involved the sigmoid colon () and was located adjacent to the right ureter, suggesting an appendiceal cancer invading the sigmoid colon. The preoperative diagnosis was appendiceal cancer with a clinical stage of T4bN0M0, stage IIC, according to the TNM classification, the Union for International Cancer Control (UICC) 8th edition.\nIleocecal resection with extended lymphadenectomy and en bloc resection of the sigmoid colon was performed through a lower abdominal midline incision after the insertion of a ureteral stent. The appendiceal tumor involved the sigmoid colon and the terminal ileum. The ileocecal part was mobilized from the retroperitoneum, keeping the right ureter intact. The ileum was divided proximal to the involved part with a linear stapler. The ileocolic vessels were ligated at their origins, and the extended D3 lymph node dissection was performed. The ascending colon and then the sigmoid colon proximal to the invaded part were divided with a linear stapler. The ileocecal part which included the tumor and the involved sigmoid colon was resected in total after the rectum was divided. The pericolic lymph nodes were dissected in the resected area of the sigmoid mesocolon. The sigmoid colon and the rectum were anastomosed in a side-to-end fashion with a circular stapler, and the ileum and the ascending colon were anastomosed in a functional end-to-end fashion. The incision was closed after the placement of Blake™ silicone drains in the Douglas pouch and in the right lateral abdomen.\nMacroscopic findings showed that the tumor measured 60 × 40 mm and invaded and made a fistula with the sigmoid colon (). Pathological examination revealed that the tumor was a well-differentiated tubular adenocarcinoma according to the Japanese classification [] and invaded the sigmoid colon (). The tumor invaded in the broad area of the appendiceal mucosa and the largest part of the tumor existed in the appendix, whereas only a small part of the tumor was exposed in the mucosa of the sigmoid colon. In addition, the tumor appeared to invade from the peritoneal side toward the sigmoid colon lumen. These findings showed that the tumor originated from the appendix and did not originate from the sigmoid colon. All 41 resected lymph nodes, including lymph node No. 201, 202, and 203, were devoid of metastasis []. The pericolic lymph nodes in the sigmoid mesocolon (No. 241) were not explored for pathological examination. The final pathological stage was T4bN0M0, stage IIC, according to the TNM classification, UICC 8th edition.\nThe patient was discharged from the hospital uneventfully. Adjuvant chemotherapy was not performed, and she was alive without cancer relapse after a 20-month follow-up. |
A four-year-old girl presented to the emergency service with painful left hip and fever. There was no previous relevant medical history. There were no other local or systemic symptoms, except for a cervical adenopathy. On physical examination, she walked with a limp, and movements of the left hip were painful (mainly external rotation), but not restricted. Blood exam revealed anemia (Hb 8.7 gr/dL), normal WBC, ESB of 123 mm, and reactive C protein of 149.7 mg/L. An initial X-ray to the pelvis revealed no changes. An ultrasound of the left hip was performed revealing small infusion and synovitis. Guided puncture was then performed being macroscopically compatible with reactive arthritis, and general and bacteriological tests were demanded. Because of the unusual characteristics of the pain, a CT scan to the abdomen and pelvis was performed revealing a left adrenal mass and retroperitoneal adenopathies in the celiac trunk and superior mesenteric artery ().\nDespite the painful complaints of the patient, no bone or articular involvement was found in the CT scan. No further alterations were reported in the thoracic CT scan or in peripheral blood smears. Bacteriological examination of the hip effusion was negative. MRI was also performed. The direct myelogram was compatible with infiltration from neuroblastoma. Bone marrow biopsy and cervical adenopathy specimens were collected to perform histological diagnosis. Skeletal scintigraphy demonstrated numerous points of osteoblastic activity compatible with metastatic activity, and the 12 iodine-123 metaiodobenzylguanidine scintigraphy concluded the following: “Abdominal mass with low expression of noradrenergic transporters. Diffuse bone metastasization with high expression of noradrenergic transporters. No other soft tissue involvement was detected.” In the histological report of the cervical adenopathy, the diagnosis of neuroblastoma NOS was performed. Immunohistochemistry revealed extensive expression for synaptophysin and CD56 (NCAM) and absence of expression of myogenin. ().\nBone marrow biopsy revealed extensive metastatic involvement. The patient started chemotherapy two weeks after admission, with 8 cycles of rapid COJEC protocol. After six months of follow-up, the primary tumor was still without criteria for resection, despite a decrease in the metastatic involvement. Given the chemotherapy-related renal toxicity, it was decided to proceed with irinotecan in combination with temozolomide (TEMIRI). After thirteen months of follow-up, no significant regression of the primary tumor occurred, so surgery was contraindicated and the patient was proposed for stem cell treatment. |
A 40-year-old female, with 3-month history of nasal obstruction and tinnitus was admitted in August 2012. Nasopharyngeal endoscopy and biopsy already had been performed in another hospital, showing nonkeratinizing undifferentiated NPC. This histopathologic diagnosis was confirmed in our center. Magnetic resonance imaging (MRI) of the nasopharynx and neck revealed the tumor was confined to the nasopharynx and the bilateral locoregional cervical lymph nodes enlarged with its greatest dimension of 2 cm. Chest computed tomography (CT) scan, ultrasound of abdomen, and whole-body bone scan ruled out distant metastases. So clinical staging was determined to be T1N2M0, IIIA according to American Joint Committee on Cancer TNM Staging System for NPC (7th ed, 2010).\nThe patient was treated with definitive IMRT to 7050 cGy for primary tumor and 6600 cGy for infiltrated regional lymph nodes. Concurrent chemotherapy based on cisplatin and 5-flurorouracil was administrated for 2 cycles and then 2-cycle chemotherapy was given subsequently to consolidate the efficiency with the same regimen. At the end of therapy, she obtained clinical complete response by nasopharynx and neck MRI.\nIn the initial therapy, the patient had undergone abdominal ultrasonography for 4 times, and no hepatic lesions were noted during this period. Nevertheless, when she came to our hospital for 3-month conventional follow-up in April 2013, abdominal sonogram revealed a liver cystic lesion with thin wall and smooth margin of 18 × 16 mm in the right liver lobe, and the lesion was interpreted as a simple liver cyst (Fig. ). Therefore, intense follow-up was suggested. Five months later, the cystic lesion enlarged to be 59 × 46 mm, with thick wall, but no signal of blood flow. Further CT presented a low density and heterogeneous lesion taking irregular wall and incomplete septa with strong contrast enhancement, indicating liver abscess (Fig. ). However, the patient was asymptomatic, with no fever, no right up abdominal pain, and no palpable mass by physical examination. On the contrary, laboratory findings were negative, liver function was normal, the white blood cell count, C-reactive protein, and cancer-related antigen including α-fetoprotein were within the normal limits. Afterward the patient was transferred to another hospital for abscess drainage, and fluid culture was negative, but fine-needle aspiration was not performed. After drainage, the mass shrank significantly to one-third of the original size. However, it returned to previous size by CT 2 months later. Thus, the liver lesion was suspected to be malignant. Work-up examination including chest CT, pelvic MRI, emission CT for bones, gastroscopy, colonoscopy, and brain MRI excluded other lesion that may account for another primary tumor or extra-hepatic distance metastases from NPC. In addition, nasopharynx MRI showed no evidence of local relapse. On November 21, 2013, surgical resection was administrated both for histologic diagnosis and treatment, and the surgical margin was negative. Histopathologic examination definitely confirmed that the metastasis originated from NPC, since the cells of the surgical segment were similar to primary NPC on the morphology and they were positive in Epstein–Barr virus (EBV) encoded RNAs (EBERs) (Fig. ). No adjuvant chemotherapy was done after resection of the liver metastasis.\nHowever, relapse-free survival time lasted only for 8 months. In July 2014, 2 small cystic lesions were found on abdominal CT scan again, which were extremely similar to simple cysts (Fig. ). Further contrast-enhanced ultrasonography provided no sign of “fast in and fast out,” that is, a characteristic appearance in malignant carcinoma. As a result of multidiscipline team discussion, “watch and wait” strategy was recommended. However, the number of hepatic lesions increased to 4, and the size of previous 2 cysts enlarged to be 3 cm within 3 months. The multidiscipline team members reached a consensus that the cystic lesions were metastases from NPC and suggested a palliative systematic chemotherapy. Then the patient received chemotherapy with paclitaxel and cisplatin for 6 cycles. During the first 3 cycles, partial lesions diminished in size, but stayed stable within the later 3 cycles. Nevertheless, the intraliver metastases exhibited significant progress soon after the chemotherapy. Subsequently, she underwent chemotherapy with gemcitabine plus targeted therapy with nimotuzumab, then single navelbine both for 3 cycles, but neither protocols showed notable effects on hepatic lesions. In addition, during the phase of chemotherapy with navelbine, the patient complained of chest pain, an irregular and fixed lump was found on her chest wall, approximately 3 cm in diameter. After local surgical resection was performed, the lump histologically was demonstrated to be an extra-hepatic metastasis from NPC. Considering the patient's good performance status score and grade A liver function according to the Child-pugh grading system, transcatheter hepatic artery chemoembolization (TACE) was administrated for 5 times from December 2015 to April 2016. After the TACE treatment, all the lesions were obviously decreased in size, with the largest metastasis decreasing from 64 × 53 to 33 × 32 cm (Fig. ). Meanwhile, carbohydrate antigen 125 (CA125) decreased from 125 to 60.8 U/mL, and squamous cell carcinoma antigen decreased from 2.8 to 0.9 ng/mL. Then 4 cycles of gemcitabine plus cisplatin were offered to consolidate the clinical effects. In the next 6 months, her liver metastases maintained stable, but lung metastases were noted. In October 2016, the CT showed the liver metastases progressed. At last, she died of liver failure in March 2017. |
A 31-year-old man noted an increasingly painful mass in his left calf. Imaging found a tumor together with lesions suspicious for metastases in the distal left tibia and the distal left femur. Biopsy revealed ASPS. Chest CT showed multiple lesions interpreted to be metastases. Brain MRI showed no lesions. He embarked on palliative treatment with sunitinib at 37.5 mg daily. Three months later, his lung lesions were stable but his leg tumor continued to grow and became more painful. Sunitinib was held and he received a palliative course of radiation to the leg (30 Gy/10 fractions). Within a month of completing radiation, his tumor ulcerated and became infected and foul smelling. We hoped that this might be related to acute radiation toxicity, but over the ensuing 3 months, his leg worsened. Nine months after presentation, he underwent below-the-knee amputation with pathology confirming ASPS as well as tibial metastases. Two months postoperatively, it was clear that the metastasis in his distal femur was compromising his recovery and he underwent a palliative intralesional resection and plate stabilization of this lesion. He has had no further trouble from his leg or other bones and now walks with a prosthesis.\nChest imaging done 1 year after presentation described slow growth of pulmonary nodules as compared to prior imaging. It was thought this progression might be due to the several interruptions of sunitinib for surgery and radiation, but despite staying on sunitinib over the ensuing 16 months, the lung metastases continued to slowly grow. Sunitinib was discontinued and bevacizumab 10 mg/kg/14 days begun. Chest imaging 2 and again 5 months later described progression (fig ).\nBevacizumab was stopped, and 2 years after his initial presentation, he began pazopanib at 800 mg daily. This was complicated by hypertension controlled with labetalol, diarrhea necessitating dose reduction to 600 mg, and hypopigmentation, which made him ‘look like Santa Claus’. Imaging 2 months after pazopanib initiation showed a clear response of his many lung lesions followed by stability on subsequent CT scans. Ten months after initiation, he continues on pazopanib with stable disease and good quality of life. |
A 39-year-old man was admitted to our hospital because of a sudden onset of hemiparesis on his left side with dysarthria. He had a history of a SVA rupture that was surgically treated at the age of 24 (fig. ). During the surgery, there were no specific findings of cystic medial necrosis or crystalline lens subluxation due to the Marfan syndrome. In his infancy, there were no developmental abnormalities. In his school records, the subject had poor grades during childhood and adolescence. His family history over 3 generations was unremarkable in terms of neurological, psychological, and cardiac diseases. During the patient's examination, his body temperature was 37.0°C, blood pressure was 184/104 mm Hg, pulse was 83 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 97% (while he was breathing ambient air). At the time of hospitalization, the patient presented with an acute stroke and the symptoms included left-sided weakness and dysarthria; these neurological symptoms lasted for a week after admission. We assessed the risk of juvenile ischemic stroke in a laboratory study, but high-risk factors that are linked to juvenile cerebral ischemia, such as collagen diseases and vasculitides, were not found. An electrocardiogram revealed a sinus rhythm within the normal range. Although we performed magnetic resonance imaging (MRI) with a 1.5-tesla unit on the first day and with a 3-tesla unit 2 weeks later, acute cerebral infarction was not detected (fig. ). Routine sonographic evaluation of the carotid arteries demonstrated a diffuse narrowing of the left ICA and a low-lying carotid bifurcation. Thereby, cerebral angiography was conducted, and it showed hypoplasty of the left ICA and a low-lying carotid bifurcation at the level of the C6 vertebra (fig. ). The A1 segment of the bilateral anterior cerebral arteries (ACAs) was united and formed an azygos type (fig. ). The common ACA duct and left posterior cerebral artery were mainly supplied by the right ICA. Aortography and 4 vessel studies (bilateral common carotid arteries and vertebral arteries) were conducted, and there was no embolic source, such as ulceration of the arterial surface or arterial dissection, or significant partial arterial stenosis. No right-to-left shunt was found in transesophageal echocardiography.123 I iofetamine single-photon emission computed tomography brain imaging was performed, and no regional reductions or abnormal accumulations of tracer uptake into the brain were observed. The patient's intelligence quotient was evaluated with the Wechsler Adult Intelligence Scale-III []; his mental faculties were found to be at a significantly low level of functioning (table ). Facial manifestations were unremarkable, but he showed symptoms of nasal speech and nasal air emission while talking. In the otorhinolaryngological examinations, velopharyngeal insufficiency was revealed by laryngoscopy. The patient was discharged after 3 weeks; MRI could not detect any significant abnormal findings such as infarction, hemorrhage, or malformation. We decided that the patient should continue with an antithrombotic therapy after assessing normal treatment for cerebral ischemia. The patient is now taking warfarin; the target range for the prothrombin time/international normalized ratio is 2.0–3.0. |
A 53-year old female was admitted to the Intensive Care Unit of Kingston General Hospital in Kingston, Ontario, Canada with diabetic ketoacidosis (DKA). She had a past medical history of type 1 diabetes with recurrent DKA and end stage renal disease secondary to diabetic nephropathy on intermittent hemodialysis. Prior to admission, she was being dialyzed through a left arm arteriovenous fistula.\nShortly after admission, her left arm fistula thrombosed, and a right internal jugular (IJ) tunneled double-lumen catheter was inserted for dialysis along with a peripherally inserted central catheter (PICC) via right basilic vein for intravenous access. The patient had a previous failed right arm AV fistula so the left arm was avoided in hopes of future recovery of the left AV fistula.\nTwo weeks later during the course of her admission, she continued to have episodes of DKA, and inadvertently her PICC line was dislodged, requiring removal. The patient had poor peripheral intravenous (IV) access and multiple attempts to insert peripheral IVs under ultrasound guidance were unsuccessful. Ultrasound examination of the patient's left internal jugular showed significant narrowing. The patient declined an attempt femoral venous access because she had previous lower extremity venous grafting for her AV fistula and she was told to avoid cannulization of any of those vessels. A 7 French, 16cm triple-lumen CVC was placed into the right internal jugular vein with sonographic guidance without difficulty. The puncture site for the CVC insertion was significantly distal to the tunneled dialysis catheter. Post-procedure, all three lumens were able to draw blood and flush saline. A chest X-ray confirmed placement of the catheter adjacent to the tunneled dialysis catheter () close to the cavoatrial junction. The patient did not have any immediate complaints or discomfort. Approximately six hours later, the on-call resident was called by nursing staff to assess the patient for new neck discomfort and jaw pain which had begun two hours prior.\nAt the time of assessment, the patient's heart rate was 95 beats per minute and regular. Her blood pressure was 188/84, respiratory rate was 18 and oxygen saturation was 99% on room air. She was afebrile. On examination, the patient had distended neck veins, pronounced facial edema and plethora (). She also had bilateral upper-extremity edema primarily manifesting in her digits. The rest of her cardiorespiratory examination was unremarkable.\nAll three lumens of the CVC were withdrawing blood and flushing without difficulty.\nPoint of care ultrasound of the right internal jugular vein revealed no thrombus at the site of CVC entry or distal to it. The SVC was not visualized directly. The CVC was removed promptly. There was no visible clot on the catheter, and no clot was pulled along with the catheter. Within an hour, the patient's facial swelling and plethora began to resolve, and her neck pain began to improve. The next morning, pain and swelling had resolved completely ().\nA retrospective chart review revealed a fistulogram performed months prior that showed significant stenosis at the left subclavian vein and mild narrowing at the junction where the two brachiocephalic veins merge to form the SVC (). At that time, angiography was attempted to alleviate the stenosis. However, the patient did not tolerate this, and the procedure did not lead to a significant change in the degree of stenosis.\nThe events surrounding the SVC syndrome and the previously identified SVC stenosis were disclosed to the patient. In order to prevent a similar event from occurring in the future, a note was made in the chart that would be more visible should the need for further CVCs arise. |
A 22-years-old woman of southeast-asian origin with no prior significant ophthalmological or family history presented to the ophthalmological emergencies complaining of acute, painless, unilateral, visual loss of the left eye. A week before visual symptoms commence, she reported transient unilateral hearing loss that resolved without treatment.\nOn initial examination the right eye had a visual acuity of 20/20 and no pathological findings. The left eye had visual acuity of light perception and a mild relative afferent pupillary defect (RAPD). Ocular motility was normal with no sign of strabismus or nystagmus. Slit-lamp examination showed a clear cornea and a quiet and deep anterior chamber in both eyes. Fundus examination of the left eye revealed an elevated optic disc with superior indistinct margins (). The patient was admitted in our clinic for further ophthalmological and neurological investigation.\nFluorescence (FA) - and Indocyanine Green Angiography were performed. Both examinations were unremarkable in the right eye. In the left eye however FA showed staining of the lower margin of the optic disc (). Visual field examination confirmed significant amaurosis on the left eye ().\nA thorough workup was ordered in order to identify the cause of visual loss. Chest and hip x-rays were negative, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels were normal and a further series of negative diagnostics excluded all possible immunological-inflammatory or infectious causes. Neurological examination showed no acute pathology. Brain CT imaging was negative for hemorrhage and revealed a left, small herniation of the cerebellar tonsils characterized as “of no clinical significance”. Intracranial pressure (ICP) was normal at the level of 10 mmHg.\nAfter a neurosurgical evaluation including a brain MRI () which confirmed the CT findings the decision for an immediate surgical treatment was made. Under endotracheal anesthesia, the patient was positioned supine. The head was clumped in the Mayfield. A midline incision from the inion to the level of C5, a posterior fossa decompression and laminotomy of C1 was performed. The dura was opened in Y fashion revealing the arachnoid membrane which was dissected revealing the cisterna magna. Afterwards, a cerebellar tonsillectomy was performed preserving both posterior inferior cerebellar artery (PICA) (). The patient showed a quick and unremarkable postoperative recovery.\nAn impressive improvement was noted during the one-month post-op examination. Visual acuity was 20/20 sc in both eyes and the optic disc at the left eye showed a minimal residual marginal haziness (). Retinal nerve fibre layer analysis (RNFL) more was also noticeably improved. |
Woman 52 year old with severe grade III APLD came in consultation. She was also aware of her familiar condition. Liver transplantation was offered to her in another center but she refused. Previous MRI from another center showed multiple giants cysts compromising the whole abdominal cavity. Cysts reached hypogastric area and both inferior quadrants. Higher segments of the liver seemed to be respected. Globulous deformation of the abdomen was clearly observed. Despite aesthetic and mild abdominal pain symptoms were not as significant as they were in her two sister so she did not want to underwent liver resection too. However she kept coming to consultation every six months.\nTwo years later she came back presenting jaundice with serum levels of FAL>1700 mg/dl. Bilirrubin 9,8/7,9 mg/dl. New MRI showed cyst compression of the hepatic pedicle including common bile duct and portal vein ( A and 3B)\nSurgery was soon programed and approach was discussed. We decided laparoscopic approach but being aware room will be very reduced even after pneumoperitoneum. We decided to start by performing aspiration and evacuation of the big lower and anterior cysts to intend reducing the mass and make new space for the laparoscopic procedure. By performing this gesture followed by several fenestration and partial cysts resections space was increased within the abdominal cavity so laparoscopic liver resection could be then performed. Once gallbladder was located, we then identified two cysts that seemed to be responsible for hepatic pedicle compression. Fenestration was done and, then, intra-operative cholangiogram showed no further compression of the common bile duct with adequate passage of contrast to duodenum. Standard cholecystectomy was completed and later laparoscopic left lateral sectionectomy was done in association with several more cyst fenestration. Post-operative course was un-eventful and patient was discharged at 6th day with decreasing levels of FAL and bilirubin. Late follow up imaging control are still to come. |
The patient is a 71-year-old woman with a past medical history of hypertension, hyperlipidemia, type 2 diabetes mellitus, and severe aortic stenosis who underwent SAVR in August 2015. A preoperative coronary angiogram revealed normal coronary arteries without evidence of ostial obstruction. SAVR was successfully performed using a 23 mm diameter Magna bioprosthetic valve. During the surgery, antegrade cardioplegia was first administered to achieve electromechanical arrest. From this point, a dose of cold blood retrograde cardioplegia was administered to the coronary sinus every 15 minutes. The patient had an uncomplicated postoperative course and had returned to her usual lifestyle. A postoperative echocardiogram demonstrated a normal ejection fraction and a normally functioning bioprosthetic aortic valve with a mean gradient of 8–10 mmHg across the valve. The patient had been compliant with medications, including aspirin, statin, and beta-blocker. In June 2017, the patient presented with pressure-like exertional chest pain. An electrocardiogram demonstrated 1 mm ST segment depression in leads I, aVL, V2, and V3 (). Laboratory investigations revealed elevation of the serum troponin I level to 2.3 ng/ml (normal value: less than 0.032 ng/ml). Guideline-directed medical therapy for non-ST elevation myocardial infarction (NSTEMI) was begun, and the patient was assigned to an invasive strategy. Diagnostic coronary angiography demonstrated isolated severe ostial stenosis (99%) of the right coronary artery with significant catheter pressure waveform dampening but no evidence of thrombus (). It showed dramatically tapered appearance from what appeared to be normal arterial segment to a critical ostial stenosis. The lesion was less likely a catheter-induced spasm because (1) there was no direct catheter engagement or deep intubation of the vessel and (2) it persisted despite bolus administration of intracoronary nitroglycerin as well as continuous intravenous infusion of nitroglycerin prior to and during the procedure. Intravascular ultrasound (IVUS) could not be considered due to severity of the stenosis. The lesion was successfully treated using balloon dilations and a single 4.5 mm × 13 mm bare-metal stent (BMS) with excellent angiographic result (). The patient was discharged from the hospital the next day without acute complications and was asymptomatic at 4 months of follow-up after the percutaneous coronary intervention (PCI). |
This is a case of a 70-year-old Caucasian female with no personal psychiatric history prior to a CVA but a positive family history for mostly unspecified psychiatric disorders in her immediate relatives and also her son who had “serious substance-abuse issues” and was recently diagnosed with schizophrenia. Her medical history included essential hypertension, hyperlipidemia, type 2 diabetes mellitus, paroxysmal atrial fibrillation, coronary artery disease status post-coronary artery bypass graft, and stroke.\nShe was admitted to the hospital for observation of chest and left upper extremity pain and dyspnea with telemetry monitoring, serial troponins, and a cardiology consultation. Psychiatric consultation was requested after the patient reported hearing voices. Upon interview, she stated that she had been suffering from auditory hallucinations and paranoid delusions for the past five years, which she said began after a CVA. She initially did not want to seek help in fear of being labeled as “crazy.” She described the auditory hallucinations as constant indistinguishable voices that were making her anxious and confused and were negatively affecting her concentration and quality of sleep. Her main complaint was the increasingly bothersome insomnia. The patient also expressed concerns that her food was being poisoned and that someone else besides the nursing staff was following her. She denied any drug use, admitting to only an occasional use of alcohol but none recently. She reported no prior sexual or physical abuse. She did not have any feelings of depression or suicidal ideation and exhibited no episodes of aggression. She was not cognitively impaired and denied any symptoms suggestive of mania or hypomania. The patient also mentioned experiencing continual residual effects of numbness and discomfort in the right upper and lower extremity from the associated CVA.\nElectrocardiography (ECG, Figure ) was interpreted as rate-controlled atrial fibrillation. Serial troponins were negative throughout. All labs were within normal limits except for glucose and magnesium. The patient’s glucose was recorded at 254 mg/dL, while magnesium level was mildly decreased at 1.7 mg/dL at time of admission. A non-contrast MRI of the brain (Figures -) done during this hospital stay for a focal neural deficit with a history of CVA suggested the presence of an old infarct in the left parietal-temporal-occipital lobe and smaller infarcts in the inferior cerebellar hemispheres bilaterally. A non-contrast CT scan of the head (Figure ) from three months prior was interpreted similarly. No acute infarcts or acute pathologic processes pertaining to the brain were noted on these imaging modalities. The left upper extremity pain our patient presented with during this hospital stay was concluded to be likely secondary to a radicular neuropathy.\nThe patient was diagnosed with an unspecified residual psychosis disorder due to stroke and was subsequently started on quetiapine 12.5 mg nightly. Upon discharge from the hospital after reconciliation of the chest pain and dyspnea two days later, she was referred for follow-up with outpatient cardiology and psychiatry. |
A 25-year-old primigravida was admitted in the hospital as pregnancy with chronic hypertension (not on any antihypertensives), at 36 completed weeks in active labor. She was a known case of active TA on treatment (taking prednisolone 2 mg, aspirin 150 mg, and clopidogrel 75 mg OD.) She was booked and supervised throughout her pregnancy at the same hospital, in liaison with cardiologists. Her past and personal histories were thoroughly reviewed in outpatient department; she had been a bidi smoker since teenage, one-two per day. She had surgical correction of complete stenosis of right common carotid artery (CCA) and right vertebral and subclavian artery by percutaneous stenting of right brachiocephalic (size 7 × 39 mm) and right CCA (size 9 × 30 mm) in some peripheral hospital. She had no history of other comorbidities like IBD and sinusitis. She had an uneventful antenatal and intrapartum period and delivered a healthy neonate weighing 2.8 kg. During her postpartum period, all necessary investigations were done and consultations were taken, and she was discharged on drugs after one week in stable condition, with the advice for regular follow-up and abstinence from smoking. During her subsequent visits, her MRA scan showed markedly attenuated flow in left CCA, for which she was conservatively managed by cardiologist.\nOne year later, she had an accidental second conception but could not get herself booked for antenatal care anywhere till the third trimester, due to social and financial issues. At her first visit at 34 weeks in the institute, she was admitted for safe confinement for severe fetal growth restriction and neurological sequelae of aortoarteritis in the form of tonic-clonic convulsions (three episodes in last 24 hours before admission). She gave history of myalgias, arthralgias, and fever off and on throughout her pregnancy, despite continued glucorticoid treatment. On admission, her BP was 200/110 in ankle and pulse was 100/min. There was no albuminuria. Per-abdomen examination showed a fundal height corresponding to 26 weeks with faintly audible fetal heart sound. She was put on strict fetomaternal surveillance. After thorough history taking, cardiology, and CTVS references were taken, her anticoagulants were stopped in v/o anticipated termination of pregnancy, and she was started on labetalol 300 mg in divided doses and continuous BP monitoring (noninvasive). A neurology consultation was done, and levetiracetam 1000 mg was started to take care of seizures. All routine antenatal and specific blood investigations (INR, PT, and APTT) were normal. Though CRP was also normal, it was on a higher side of normal range (3 mg/dL). ESR was found to be raised being equal to 33 mm/1st hour. Echocardiography was performed which revealed mild concentric LVH, moderate AR (38 mm diameter), mildly thickened aortic valve leaflets, and ejection fraction 63% (). Carotid and vertebral Doppler reported mild stenosis of poststent segment, with 65–70% stenosis of left CCA and ICA, which was suggestive of progressive active TA in the patient (). A renal Doppler was also done that was normal. Her obstetric ultrasound with Doppler velocimetry showed a single live growth restricted fetus with oligohydramnios (AFI-5CMS) and severely deranged diastolic flow in umbilical artery (). The couple were counselled adequately about fetomaternal prognosis and after their informed consent, she was given two doses of betamethasone for lung maturity, following which induction of labor was done with cervigel at 35 weeks two days. Her labor progressed well and she delivered live boy baby weighing 1.2 kgs, who was transferred to nursery, being very low birth weight baby, and discharged after one month. Patient's intrapartum and postpartum period were uneventful and she was discharged on higher dose of prednisolone, amlodac 7.5 mg, and levetiracetam. At the time of this writing, she is convalescing well, with both mother and baby doing fine, and following up periodically with cardiologists, rheumatologists, and gynecologists. |
A 73-year-old man was referred with an enlarging right groin swelling and urinary incontinence. An ultrasound scan of the scrotum 9 months earlier had demonstrated a right hydrocoele which was being managed conservatively. He had worn a truss for a right inguinal hernia in the early 1990s; however, on further investigation at that stage, a hernia could not be demonstrated and therefore no treatment was given.\nOn examination, a large mass was palpable in the right groin extending into the scrotum. The penis was buried within the scrotum and on retracting the foreskin and applying pressure to the right groin mass, urine was voided.\nA urethral catheter was inserted draining a residual of 1000 ml clear urine. After catheterization, the right groin mass had significantly decreased in size. We suspected the right groin swelling represented a herniated bladder and this was confirmed by a cystogram []. Ultrasound scan of the renal tract demonstrated bilateral hydronephrosis; therefore, bilateral nephrostomies were inserted which were eventually converted to ureteric stents. He subsequently underwent laparoscopic bladder hernia repair.\nThe procedure was performed under general anesthesia with 1.5 g intravenous cefuroxime and 500 mg metronidazole prophylaxis. An 18 Charriere silver-coated urethral catheter was inserted at the start of the procedure. Four ports (1 × 12 mm, 1 × 10 mm, and 2 × 5 mm Excel bladeless trochars) were inserted after establishing pneumoperitoneum via Hassan's technique. Following incision of the peritoneum and right inguinal dissection, herniotomy was achieved via retraction and external pressure. A 12 × 15 cm Dacron mesh (Cousin Biotech) was tacked over the inguinal defect and a drain was not inserted as the bladder had not been opened. Total operative time was 120 min.\nThe patient had an uneventful post-operative recovery and was discharged after 3 days following a successful trial of void. His stents were removed 6 weeks after surgery and his serum creatinine was stable at 168 umol/L. He remained well and was discharged after 5 months. |
A 66-year-old man presented with bilateral breast bulging. His breasts had slowly become enlarged over a number of years and this had led to huge breast bulging; the patient reported that he was ashamed of his breast disfigurement when in public baths and swimming pools. He had undergone an excisional biopsy of a mass in the central upper back region 1 year earlier, which revealed a lipoma.\nHe reported more than 25 years of alcohol abuse, and confirmed that he consumed alcohol 5 times a week. Furthermore, he had a history of cigarette use, but had stopped smoking 4 years previously. He also had a 10-year history of hypertension and diabetes mellitus. The patient's father had a history of multiple large disfiguring masses in the neck and upper body.\nPhysical examination of the patient revealed multiple large, disfiguring masses in the occipital region as well as on the breasts, upper arms, shoulders, neck, and upper trunk. These appeared to be symmetrical on the breasts, upper arms, and shoulders. Palpation revealed that the masses had an elastic consistency, could be moved without causing the patient any pain, and had no definite margins ().\nLaboratory examinations revealed normal liver and thyroid function, and a hormone assay confirmed that the results were within the normal ranges. A mammography was performed which revealed that the patient's breasts were comprised almost entirely of fat. Breast ultrasonography revealed excessively fatty breasts without any definite glandular tissue (). Computed tomography and magnetic resonance imaging showed diffuse and marked fatty deposits in the superficial soft tissue layers of the neck, chest, abdomen, and shoulder wall with marked bulging contours of both breasts ().\nThe patient underwent bilateral nipple-sparing mastectomy with nipple interposition. Pathology revealed soft tissue lipomas measuring 30×17×5 cm and 27×16×5 cm, and both lipomas were homogenous without hemorrhage and necrosis (, ). No hidden malignancy was found. The patient did well postoperatively and was satisfied with the cosmetic result. |
A 41-year-old man was admitted with a 3-day history of severe sore throat and painful swallowing. According to him, it started as a mild sore throat but rapidly worsened despite taking oral penicillin for 3 days from his general practitioner (GP). There was no significant past medical history. On examination, his temperature was 38.3°C and he was tachycardic (102/min). His neck movements were markedly restricted by pain and he had a spiking fever. The skin on the front of his neck was red, hot and tender down to his clavicles, with no evidence of localised swelling or ischaemia/necrosis. Oral examination showed a markedly inflamed right tonsil covered with patchy grey exudate. There was no clinical evidence of peritonsillar abscess. The left tonsil appeared remarkably normal. Flexible nasendoscopy showed normal epiglottis and larynx.\nBlood tests revealed leukocytosis of 22.3 × 109 cells/L and a high C-reactive protein (CRP) of 421 mg/L.\nA lateral X-ray of his neck showed air and swelling in the pretracheal soft tissues and loss of normal cervical lordosis. These findings prompted an urgent CT scan of his neck and chest, which demonstrated air shadows and diffuse swelling and enhancement of cervical fascia extending from skull base to upper mediastinum. Similar changes were also seen in the pretracheal soft tissue (figures , and ).\nThroat swabs and blood cultures were sent for microbiology and intravenous (I.V.) therapy with broad spectrum antibiotics was commenced as it was decided to initially treat the condition medically. Surgical debridement was not carried out at this stage because there was no skin necrosis and no collection was shown by the CT scan.\nHis general condition showed improvement. His temperature came down and his oral intake improved but over the next 3 days a tender and fluctuant swelling appeared in the suprasternal region with diffuse margins.\nA repeat CT scan of the neck and chest at this stage showed receding air shadows and the appearance of an abscess extending from the pretracheal region to the upper mediastinum. After discussions with a radiologist it was decided to carry out a limited surgery in the form of incision and drainage. About 200 ml of pus was drained via a small transverse suprasternal incision and midline split of the abscess wall. All the loculi were broken down with a finger. Cultures of the pus did not reveal any organisms.\nAfter surgery, the patient's condition dramatically improved. Drains from the wound were removed on the fifth postoperative day and the wound was allowed to heal by secondary intention. Inflammatory markers progressively came back to normal and the patient was discharged on the 18th day after admission. He was followed up after 2 weeks with a repeat CT scan which showed complete resolution. He underwent "interval tonsillectomy" in our department and the tonsils were sent for histopathology which did not reveal any abnormal findings. |
A 55-year-old woman presented three days after a sudden onset of right-sided chest pain, pleuritic and positional in nature, associated with an acute onset of shortness of breath. She had gone to her primary care physician, who performed a chest X-ray and urged her to come to the hospital. Upon presentation at the emergency department, her oxygen saturation was above 95% on room air, and she was not in any respiratory distress, but her exam was significant for decreased breath sound on the right. A chest X-ray confirmed a large right-sided pneumothorax with small pleural effusion. A chest tube was inserted on the right side for the resolution of the pneumothorax, and subsequent computed tomography (CT) scan of the chest revealed bilateral diffuse bullous disease of the lung with multiple cysts (Figure -). The patient underwent video-assisted thoracoscopic surgery for right thoracoscopic wedge resection of a lung bleb and talc pleurodesis. Gross examination of the specimen revealed several dilated air-like spaces ranging from 0.2 cm to 0.4 cm in size. The hospital course was complicated by postsurgical pneumonia, but she recovered fully and was discharged to home with only minimal symptoms of dyspnea on exertion. Upon further investigations, she was found to have multiple small lesions of angiomyolipoma on the right kidney with diffuse retroperitoneal lymphadenopathy. One of the lymph nodes was biopsied, and pathology revealed predominantly spindle cells positive for HHF35 and smooth muscle actin, consistent with the diagnosis of leiomyoma. At the eight-month follow-up at the pulmonology clinic, her pulmonary function test (PFT) showed normal vital capacity and forced expiratory volume in one second (FEV1), but moderately reduced diffusion capacity, which may also be related to LAM. At her 12-month and 24-month follow-up visits, her PFT results showed improvements in peak flow and diffusion capacity, and the patient continues to report no symptoms other than minimal dyspnea on exertion. |
A 72-year-old female presented 14 months after a two-part left proximal humerus diaphyseal fracture (OTA11 B2). Her past medical history was significant for osteoporosis. Physical examination revealed gross deformity, tenderness and motion at the fracture site. No neurologic deficit was found. Radiographic evaluation revealed an oblique fracture of the left proximal humerus diaphysis with lateral and anterior angulation of the distal fracture fragment []. There was minimal osseus bridging between the proximal and distal fracture fragments with associated callus formation. She was indicated for operative intervention.\nThe patient was administered regional anesthesia and positioned in a sloppy lateral position. An extended anterolateral approach was utilized as described by Gardner et al.[–] The skin incision began at the anterolateral tip of the acromion and was extended to approximately 5 cm proximal to the lateral epicondyle. Proximally, the subcutaneous tissue was divided to expose the raphe between the anterior and the middle heads of the deltoid. The raphe was carefully divided in the “safe zone” above the location of the axillary nerve, 6 cm below the anterolateral border of the acromion.[–] The muscle was separated using blunt dissection proximally. The axillary nerve was then palpated and found to be scarred to the humeral head. Neurolysis was performed and the nerve and surrounding soft tissues were protected with a vessel loop. The deltoid was further divided proximal and distal to the nerve and the insertion site was tunneled under for future plate placement. The lateral border of the biceps was then identified and the muscle retracted medially to expose the brachioradialis and brachialis muscles. The faschia between the muscles was then incised in line with the intermuscular plane and the radial nerve was identified. The radial nerve was found to be scarred down to the inferior aspect of the fracture, and was mobilized. The fracture site was then exposed and debrided. A proximal humerus Locking Compression Plate (LCP) (Synthes, Paoli, PA, USA) was passed under the axillary nerve and down the lateral aspect of the humerus. The humerus was transfixed distally using two compression screws. An indirect reduction was obtained proximally. The obliquity of the fracture was clamped and compressed using a standard reduction clamp and locking screws were then placed proximally. An interfragmentary screw was then passed across the obliquity of the fracture from the lateral to the medial side. Anteriorly, a six-hole recon plate was placed with two screws proximally and two screws distally to reinforce the fixation. The glenohumeral joint was then examined and found to have significant bursal scarring, which was excised producing a full range of motion at the shoulder. Recombinant human bone morphogenetic protein-2 (Infuse Bone graft, Medtronic Inc., Minneapolis, MN, USA) was then packed around the fracture site. Aggressive occupational therapy for active and passive range of motion was implemented 1 week after surgery. At the patient’s 3-month follow-up, healing was confirmed radiographically and the fracture site was nontender []. Range of motion for forward flexion, abduction and internal and external rotation at her 12 th-month visit was 90, 90, L2 and 40 degrees, respectively []. |
A 69-year-old female visited the emergency room with fever that had persisted for 2 weeks. Computed tomography (CT) on the same day revealed multiple lymphadenopathy in the abdominal cavity. A blood examination revealed the exacerbation of renal and liver function marker levels within several days, and she was admitted to hospital. Time course of her laboratory data after admission were noted in . Although antibiotics (ceftriaxone to meropenem) were administered during the first week after admission, her inflammatory markers remained elevated and her fever persisted. Anasarca with fluid retention (i.e., pleural effusion and ascites) showed extreme progression after admission and a blood examination revealed thrombocytopenia.\nAs her condition progressed, she satisfied the three major diagnostic criteria (anasarca with fluid retention, thrombocytopenia, and fever with high inflammatory marker levels) and two minor diagnostic criteria (multiple lymphadenopathy and renal dysfunction) for TAFRO syndrome.\nDiuretics (intravenous furosemide and oral administration of tolvaptan) had no effect on her fluid retention. Steroid pulse therapy followed by steroid maintenance therapy was performed to treat TAFRO syndrome, and hemodialysis was performed in an attempt to improve her fluid retention (). However, these strategies were ineffective, and her anasarca with fluid retention worsened. Finally, she was admitted to the intensive care unit (ICU) on the 24th day after admission. Mechanical ventilation support was required after endotracheal intubation due to respiratory failure, and continuous renal replacement therapy (CRRT) was performed due to renal failure. Since her liver function had not recovered after these treatments, her inflammatory marker levels remained elevated and her fever persisted, we decided to perform PE (albumin replacement for 2 days and fibrinogen replacement for 4 days) as a treatment for TAFRO syndrome. The rapid initiation of PE was very effective. Her fever quickly declined, her inflammatory reaction decreased, and her anasarca with fluid retention showed a dramatic improvement. After the beneficial effects of PE, rituximab (every week) was initiated.\nRespiratory support was smoothly withdrawn and the patient was transferred to the general ward on the 48th day after admission (, ). Rituximab treatment was performed for 1 month (four times every 1 week), and her good general condition was maintained. After 3 months of multidisciplinary treatment she made a complete recovery and was discharged to a rehabilitation hospital. We are planning to use the tocilizumab if she required the re-admission by the recurrence of TAFRO syndrome in future. |
A 62-year-old Caucasian man with advanced hepatic fibrosis secondary to hepatitis C viral infection was scheduled for a four-phase CT to screen for HCC, but was denied insurance pre-approval for the imaging study. He was diagnosed with hepatitis C viral infection a decade earlier, which was subsequently treated and cleared with interferon and ribavirin. Cirrhosis was confirmed via biopsy at that time, and stage III fibrosis diagnosed via biopsy four year later. At the time of his screening request, he had no additional medical problems or sequela of advanced liver disease. His physical exam was essentially normal—including vital signs and for stigmata of chronic liver disease. His alpha-fetoprotein (AFP) was 3.0 ng/mL with normal liver function tests, albumin, and platelets.\nRoutine US showed a heterogeneous, coarsened liver parenchyma with a suspected 2.2 cm hypoechoic structure in the right lobe on a background of geographic hepatic steatosis (). Contrast was then administered intravenously with targeted imaging of the lesion to determine if it was focal fatty sparing or tumor. The lesion markedly enhanced () and began washout rapidly, and on delayed imaging, showed intense washout. It was deemed a LI-RADS M because of its early enhancement and for its marked washout (). Given the LI-RADS M designation and for treatment planning, a four-phase CT was then approved by his health insurance company. Despite its size, the lesion was poorly visualized on the well-timed late arterial phase (). It was equally subtle on the portal venous and 3 min phases. Knowing where the lesion was on CEUS allowed the radiologist to confirm the findings on CT, where it was deemed a LI-RADS 5 lesion. However, without the pre-existing knowledge of its presence in that location, the lesion was initially missed. The late arterial phase on the CT was bolus-triggered, which resulted in scanning occurring at 35 s post injection. Interestingly, on the CEUS, the lesion was already washing out and was iso-intense to liver by 35 s. This may explain why it was poorly seen on CT despite its intense, avid enhancement on US.\nGiven the LI-RADS 5 designation on CT, the lesion was not biopsied prior to treatment, as may be indicated by an initial LI-RADS M diagnosis. Instead, it was treated as HCC with several transcatheter arterial chemoembolizations (TACEs). Despite two treatments with TACE, persistent and progression of tumor was identified on follow-up CTs over the next 8 months. |
A 29-year-old woman with systemic lupus erythematosus (SLE) diagnosed at age 12 years with severe idiopathic thrombocytopaenic purpura (ITP), presented acutely while visiting her baby in the Neonatal Intensive Care Unit with sudden onset chest pain and shortness of breath. Her SLE was complicated by spontaneous deep vein thrombosis, triple positive antiphospholipid syndrome (APLS), and Class IV lupus nephritis. This triggered an emergency call to the neonatal ward, with examination notable for tachycardia, but normotensive and normal oxygen saturation initially. Her chest was clear with dual heart sounds and no oedema. Her medications at presentation were enoxaparin 100 mg and aspirin 100 mg daily, transition to warfarin was planned at 2 weeks’ postpartum. Her immunosuppression regime was hydroxychloroquine 400 mg daily, azathioprine 150 mg daily, and prednisone 5 mg daily, other medications included calcitriol 0.25 μg daily and calcium carbonate 1.25 g daily.\nElectrocardiogram (ECG) showed sinus tachycardia with no ischaemic changes. Initial blood results shown in . Chest X-ray showed cardiomegaly with increased interstitial markings (). She deteriorated rapidly over the next hour with climbing oxygen requirements and hypotension and was investigated for pulmonary embolism (PE) and treated with morphine and enoxaparin. She was escorted to radiology from the neonatal ward for the CT pulmonary angiography (CTPA) which showed pulmonary oedema and no PE and was admitted to cardiovascular intensive care (CVICU). On arrival, she was intubated for respiratory distress and, as no transthoracic echocardiogram had been performed, she had a transoesophageal echocardiogram. This demonstrated the posterolateral papillary muscle rupture and flail anterior leaflet with torrential MR and preserved left ventricular (LV) function (see ).\nShe proceeded to emergency mitral valve (MV) replacement, on transfer to the operating table she had a pulseless electrical activity (PEA) arrest, resuscitated with adrenaline and CPR before return of circulation and the start of operative intervention. The posterolateral papillary muscle was pale and sheared directly off the ventricular wall. The MV was replaced by a 29-mm St Jude’s Mechanical valve. At the end of the procedure, VA ECMO was instituted due to persistent severe pulmonary oedema and biventricular failure despite maximal ionotropic support.\nThe histology of the valve revealed preserved architecture with scattered fibrinous deposits and a focal larger fibrinous vegetation with occasional inflammatory cells, with no organisms, consistent with chronic Libman -Sacks endocarditis (). The papillary muscle showed extensive central necrosis with a surrounding rim of acute inflammation consistent with infarction several days old, thrombi were seen within the intramyocardial arteries (). The endocardial surface had mixed inflammatory infiltrates and focally fibrinous material with granuloma-like appearance, in keeping with Libman–Sacks.\nECMO was required for 2 days, on decannulation and desedation, she had some abnormal movements so magnetic resonance imaging (MRI) brain was performed which showed widespread hypoxic injury. She had paroxysmal atrial fibrillation and mild LV impairment so was commenced on metoprolol CR 166.25mg and cilazapril 1 mg daily and transitioned from heparin back to warfarin and aspirin 100 mg daily. She underwent inpatient rehabilitation after 4 weeks in CVICU before discharge. At this point, her neurological function was normal.\nShe continued the same immunosuppression regime from admission on discharge as well as the medications mentioned above. She was planned to continue both aspirin and warfarin given the recurrent thrombosis previously. |
The patient was a 14-year-old girl whose major complaint was a preauricular swelling that had developed 3 years previously. At first, she went to the local doctor and MRI scan was done, but there were no significant findings. Two months before consultation, she had pain around preauricular swelling and visited our hospital. Past history was unremarkable. On initial examination, a massive lesion was observed in the preauricular region with rubor. No facial paralysis was evident. Computed tomography (CT) revealed a 45 × 40 mm tumor in the right parotid gland that had infiltrated surrounding tissues including the skin, anterior wall of the external auditory meatus, masseter muscle, and temporomandibular joint (). A CT finding of swellings in multiple lymph nodes in the right neck led us to suspect metastasis into neck lymph nodes, but positron emission tomography- (PET-) CT showed no evidence of metastasis into neck lymph nodes or other parts. Fine needle aspiration revealed large numbers of cell clumps comprising atypical epithelial cells with large, stained ovoid nuclei, and class V high-grade tumor was diagnosed. In view of the above findings, the lesion was considered to be a T4N0 M0 stage IV parotid gland carcinoma. As radical therapy, surgery was performed to resect the skin, including the pinna (). Temporal craniotomy and skull base surgery were performed to resect the temporomandibular joint capsule and external auditory meatus en bloc and mandibular dissection was carried out (). Dura was retained and facial nerves were resected at the same time. Left neck dissection from level I to level V was also performed (). A latissimus dorsi myocutaneous flap was used for reconstruction. Since dynamic reconstruction of facial nerves by means of neuroanastomosis was difficult, static reconstruction was performed. No postoperative complications were encountered. The permanent pathology diagnosis was high-grade mucoepidermoid carcinoma with a low-grade component, in view of the presence of high-grade malignancy with strong heteromorphism and a large number of solid foci () together with low-grade malignancy consisting of clearly visible ducts formed from goblet cells (). The resection stump was negative and no metastases to neck lymph nodes were observed. As this was a high-grade mucoepidermoid carcinoma, postoperative radiotherapy at 50 Gy was conducted. In postoperative week 6, the patient was started on a liquid diet, and in week 8 she was able to consume light food. By postoperative week 12, the patient was able to eat normal food again. No recurrence or metastasis has been seen in 4 years of follow-up. Facial deformity, healing loss of the right ear, and occlusion of teeth have remained; however, she was able to eat any foods anywhere with satisfaction. She has enjoyed the university life. |
Our patient is a 67-year-old male with a history of hypertension, type 2 diabetes, and hyperlipidemia, who went through the Inova Movement Disorders Program due to a history of fluctuating, progressing uncontrollable movements of the left side of his body. He reports these movements had been present for around 10 years, worsening over time.\nAt baseline, he always felt that his left arm and leg were moving, at times exhibiting large amplitude movements which could knock things down or affect his walking, making him fall. He described it as abnormal, uncontrollable movements that made his arm ‘reach around or fling out’ His leg was also described to ‘dance on its own.’ While at baseline, these were present but minimal stress or anxiety would exacerbate the movements. There were no movement issues on his right side, nor any facial movements noted.\nHis exam showed episodic, mild with some interspersed moderate-amplitude quick movements of the left hand, forearm, proximal arm muscles, as well as movement of the left leg. These were not suppressible, brought out more by distraction or with anxiety, such as discussing an MRI scan as he was claustrophobic. He also demonstrated some lateralizing movements of the head to the left, but no facial or tongue movements. His right side showed no such signs, and his neurological exam was otherwise unremarkable.\nHe was sent for a CT scan of the head initially due to a fear of MRI scanners, and was found to have asymmetric calcification of the right caudate nucleus and anterior putamen (Figure ). The calcification spared the anterior limb of the internal capsule. Laboratory work for parenchymal calcification was then sent and was normal. The MRI of the brain with contrast demonstrated both hypointense gradient echo (GRE) signal in the right caudate nucleus and putamen, as well as an underlying DVA (Figures -).\nGiven the lateralizing of his deficits and correlation with opposite hemispheric parenchymal calcification, he was diagnosed with unilateral hyperkinetic movements as a product of putamen and caudate calcification from an underlying DVA. Consideration was made for focal hemorrhage, but presentation and imaging were not thought to reflect this pathology. He was then treated with 0.25 mg clonazepam 2x daily with significant improvement in his hyperkinetic movements. |
A 39-year-old man was involved in a high-velocity motor vehicle accident. He sustained an open fracture of the right elbow, with significant loss of the external humeral condyle and partial loss of the olecranon. This fracture was classified as a Gustillo type IIIA injury. There was no neurovascular compromise. The patient was treated in a community center, close to the accident, where he received surgical care (debridement and partial excision of the olecranon). The wound was fully closed. The upper arm was then immobilized in a splint (). IV antibiotics (cefazolin-gentamicin) were started for 5 days.\nThe day following his elbow surgery, the patient fell in a staircase and sustained a C7-C8 and C8-T1 fracture-dislocation. This injury caused neurologic damage (quadriparesis), and his right arm became his only functional limb. Following this injury, the patient was moved to our tertiary center to get spinal fusion. During the spinal surgery, the elbow was tested under fluoroscopy. The patient's elbow showed varus instability and a positive pivot shift test. A CT scan of the elbow was obtained the following day and showed bony loss from the external humeral condyle and subluxation of the radial head ().\nWe decided to treat the patient's elbow surgically. The surgery underwent nine days after the initial trauma (after transfer from the community center, spine procedure and elbow imaging). A posterior approach to the elbow was used along with an extensive elbow debridement. A tricortical iliac crest graft was then collected from the patient's right side to replace the humeral condyle bone loss. A tendinous graft was collected from his third and fourth extensor digitorum longus tendons to reconstruct the lateral collateral ligament. The tendinous graft was fixed to the bone graft through two tunnels (anterior to posterior and lateral to medial). The iliac crest graft was then fixed to the humerus with a cancellous screw. A five-hole one-third tubular plate was used as buttress. The graft was left prominent on the lateral side to improve lateral stability of the elbow. The lateral collateral ligament was then reconstructed by fixing the tendinous graft to the proximal ulna with two bundles. We took care to preserve our reconstructed lateral collateral ligament's isometry during flexion and extension movements. Reduction and stability of the elbow joint were verified under fluoroscopy. The wound was closed with staples, and a posterior splint was applied for three weeks. 14 days postoperative X-rays are presented on . At three weeks following the surgery, passive and active ranges of motion were initiated.\nWhile wearing the splint, the patient developed a pressure wound on the lateral side of his elbow (at the site of the reconstruction). This wound later complicated into a Nocardia septic arthritis, which required surgical debridement. Four months after the reconstruction, range of motion was 30-120 degrees with full pronation and supination, without any evidence of instability.\nTwo months later, instrumentation was removed and a rotational flap was done to increase soft tissue coverage of the articulation. Five months after that procedure, the patient developed a Nocardia osteomyelitis of the humerus and was treated with 6 weeks of IV antibiotics (penicillin) and a surgical debridement. The patient responded well to our treatment. Eleven months after the initial intervention, range of motion of the joint ranged from 30 to 120 degrees of flexion, without clinical instability. Osteosynthesis was achieved according to X-rays of the elbow (). |
A male in his 30s was seen as an outpatient at a large metropolitan hospital with a seven month history of a 20 kg weight loss, pallor and lethargy. He had developed chronic diarrhea. Further investigations confirmed the presence of severe anemia (Hb 40 g/l, nr >125) and profound hypoalbuminemia (23 g/l, nr >40). On initial presentation he minimized his medical history and noted he had conceived a child with in-vitro fertilization 2 years prior to his symptoms.\nGastroscopy showed duodenal atrophy consistent with celiac disease. Duodenal ulceration was also noted (Figure ). The biopsies showed villous atrophy but absence of a lymphocyte infiltrate. A diagnosis of presumptive celiac disease was made in spite of the absence serological markers. He was placed on a gluten free diet. His anemia was thought to be due to iron malabsorption and he was treated with a blood transfusion and an infusion of parenteral iron. He was taking nonsteroidal anti-inflammatory drugs (NSAIDS) for a knee injury and was advised to discontinue these.\nA colonoscopy was normal. A CT scan of the abdomen did not reveal malignancy. He had a markedly elevated fecal calprotectin (>500 μg/g feces, nr <50) indicating ongoing small intestine inflammation. His ESR and CRP were elevated. There was no improvement in his clinical state with ongoing anemia and lethargy despite a gluten free diet and reporting no further NSAID use.\nCapsule endoscopy was undertaken which showed extensive, severe ulceration of the small intestine (Figure ). He was thought to have concomitant Crohn’s disease. His ASCA antibodies were negative. In the context of clinical decline despite gluten free diet, the patient’s confirmation of NSAID withdrawal and extensive small bowel ulceration on capsule study, he was treated with high dose oral prednisone, with temporary improvement of his symptoms. Azathioprine was added as a steroid sparing agent.\nHis immunoglobulins were subsequently measured, revealing profound hypogammaglobulinemia. His IgG was 1.5 g/l (nr> 8) with reduced IgA of 0.4 g/l (nr> 0.8) but IgM just in the normal range at 0.4 g/l (nr> 0.4). He was referred to the clinical immunology service at Auckland Hospital with suspected Common Variable Immune Deficiency (CVID) with predominant gut involvement. His fecal alpha 1-antitrypsin level was elevated consistent with protein losing enteropathy. The absence of serological markers of his gut disease was thought to be due to the humoral immune defect even though vaccine responses to H. influenzae type B (0.44 μg/ml, protective range >0.15) and tetanus toxoid (0.16 IU/ml protective range >0.1) were preserved. He had low levels of diphtheria antibodies and pneumococcal antibodies, which may have reflected absence of recent immunization. Vaccine challenge responses were not undertaken prior to IVIG treatment, because of the severity of the immune defect. The major immunology results are shown in Table .\nPersonal history and review of computerized medical records revealed a benign infectious history prior to the current presentation. Repeat testing confirmed persistent severe hypogammaglobulinemia. Immunophenotyping showed normal numbers of B cells (12% of total lymphocytes) and the presence isotype switched memory B cells. He was thought to have protein losing enteropathy secondary to CVID. He was commenced on high dose IVIG but his serum IgG failed to increase to the normal range (Figure ). In spite of his immunosuppression his albumin remained at 23 g/l. The fecal calprotectin levels remained markedly elevated indicating ongoing bowel inflammation. The clinical immunology service considered the use of Infliximab for the refractory CVID-associated enteritis.\nThe gastroenterology team became increasingly suspicious about the atypical presentation and lack of response to treatment. A bleeding time was prolonged raising the possibility of NSAID abuse. Careful review of gastric biopsies revealed micro ulcerations. These findings were discussed but both the patient and his wife offered repeated assertions that he had not taken any NSAIDs or similar agents for several months. Subsequent to that appointment however, his family discovered numerous concealed packets of an Ibuprofen/codeine combination and brought this to the attention of the clinical staff.\nWhen confronted with this information, the patient admitted surreptitious use of NSAIDS throughout the preceding year. It was felt by both the gastroenterology and immunology teams that all his symptoms were due to small bowel enteropathy caused by NSAID abuse. His immunosuppression and immunoglobulin infusions were discontinued and per his family, his symptoms abated.\nAlthough the patient declined psychiatric evaluation or outpatient therapy, collateral information elicited a complicated history. His family psychiatric history included bipolar disorder and addiction. In terms of his social history, the patient had undergone more than 6 career changes, including biochemistry and chemistry training as he had indicated at one point that he planned to pursue a career in medicine. According to his family, he frequently read medical texts and at times he described himself as a “pre-med” or a medical student although he had no formal medical training. He also reportedly was prone to self-medication that extended to significant use of recreational drugs such as marijuana, peyote and other hallucinogens that he felt “sharpened his understanding”.\nIn addition to illegal drugs, family reported that he used caffeine pills and energy drinks on a daily basis. His social history also included ongoing issues with financial stressors. His personal psychiatric history included sexual abuse as a child and a history of at least one known suicide attempt. While he was noted to be impulsive, he was not known to have received a diagnosis or treatment for bipolar disorder. He had been diagnosed and treated for depression. He has not returned for follow up. |
A 60-year-old man presented with a 1-month history of progressively worsening discomfort during swallowing. His medical history included treated gastric cancer (5 years earlier) and emphysema. The patient reported that he had smoked approximately 20 cigarettes per day since the age of 20. Laboratory tests did not detect hepatic disorders or renal problems. Upper gastrointestinal endoscopy revealed an ulcerative lesion with elevated distinct borders in the lower esophagus, and endoscopic ultrasound detected serosal invasion. The lesion was diagnosed as a squamous cell carcinoma from a biopsy. A positron emission tomography (PET) examination confirmed lung metastasis. As a result, the patient was clinically staged as cT3N1M1 and was treated with 5-FU and cisplatin. However, his obstructive swallowing problems continued to worsen. We next administered concurrent radiotherapy as a palliative treatment. The treatment regimen (FP regimen) consisted of 5-FU at a dose of 800 mg/m2 on days 1–5 and cisplatin at a dose of 80 mg/m2 on day 1 and was repeated every 28 days. The patient did not exhibit specific adverse effects during the first course of treatment. After the completion of that, a second course of the same regimen was started. However, the patient fell unconscious 72 hours after the initiation of treatment.\nOn physical examination, he was unconscious (Glasgow Coma Scale: E1V3M5) and afebrile and had a pulse rate of 69 bpm and a blood pressure of 111/61 mmHg. There were no signs of mucositis. A neurological examination did not detect paralysis or abnormal reflexes. The patient's laboratory data revealed hyperammonemia, mild hyponatremia, and a high blood urea nitrogen (BUN) level. Other findings are shown in . Radiological assessments including computed tomography (CT) and magnetic resonance imaging (MRI) scans of the patient's head did not detect any apparent cause of the patient's condition. On the following day, his condition normalized with only normal saline hydration, and he did not exhibit sequelae. We subsequently diagnosed the patient with 5-FU-related hyperammonemia.\nAt the onset of hyperammonemia, the patient's serum 5-FU concentration during the unconscious state was significantly lower (13 ng/mL) than the normal range (500–600 ng/mL). In 5-FU metabolism, approximately 80% of infused 5-FU is degraded by DPD, the initial and rate-limiting enzyme in the catabolism of pyrimidine bases, and this process produces ammonia as the end product. Due to the rapid clearance of 5-FU, we evaluated the patient's DPD activity using the urinary dihydrouracil to uracil ratio (DHU/U). shows the patient's DPD activity and a high DHU/U ratio, which indicated that his 5-FU metabolism had not been suppressed.\nWe concluded that continuing with the FP regimen would be harmful to the patient. He was subsequently treated with taxane-based treatment, which resulted in PD. He died approximately six months after being diagnosed due to cancer progression. |
Our case is about a 39-year-old male, who suffered an electrical burn with high tension wires that caused an intraparenchimal bleeding. The patient was working on the ceiling of a house when he received an electrical discharge on his head. A high voltage wire touched his head, after which, he lost awareness without recovery. He received primary care by paramedic personal, who found the patient with 6 points on the Glasgow Coma Scale (GCS). He was sedated with advanced airway manage and was sent to our medical center. He arrived under the effect of sedation, with the Ramsay scale of 6, therefore, he continued under sedation and with mechanical-assisted ventilation.\nOn physical examination, he presented an electrical burn in the parietal area (entry zone) with a diameter of approximately 8 cm [], along with an electrical burn in the left forearm (exit zone) []. He was isometric with 3-mm sized pupils. In the laboratory analysis, we found high CPK levels (CPK 12307), secondary to rhabdomyolysis.\nThe head tomography scan revealed an intraparenchimatous bleeding in the left parietal area, under the entry zone, with gas density inside the hemorrhage []; the calculated volume was about 60 cm3 with deviation of the midline elements. He underwent surgical drainage through enlarged single burr-hole.[]\nThe control tomography scan showed the subtotal evacuation of the hemorrhage and the recovery of midline structures [].\nAfter surgery, he stayed in the intensive care unit (ICU) for 5 days where he presented renal failure secondary to rhabdomyolysis. During the ICU monitoring, he had satisfactory evolution. He was extubated and discharged to our hospitalization service, where he stayed for 5 days to complete antibiotic treatment. He was discharged from our hospital with 15 points in the GCS and the skin lesions in epithelialization phase.\nThe clinical effects of electric injury may be classified into immediate and late manifestations. The immediate manifestations include cardiac and respiratory arrest, loss of consciousness, motor and sensory disturbances, amnesia, and confusion. The late manifestations may be divided into focal and nonfocal deficits and may occur days to months after the electric injury. Focal deficits include cerebral (hemiplegia, aphasia), spinal (transverse myelitis, progressive muscular atrophy, amyotrophic lateral sclerosis), and peripheral nerve (neuropathies, radiculopathies) manifestations. Nonfocal symptoms such as psychoneurotic behavior, personality changes, confusion, amnesia, and headache are common, and their occurrence is not restricted to cases in which the brain lies in the electric current pathway. Abnormal electroencephalograms (EEGs) have been documented in cases in which the brain is outside the current pathway.[]\nAlthough the exact mechanism of nerve injury has not been explained, both direct injury by electrical current and/or a vascular cause receive the most attention.[]\nFour theories have been considered to explain the histopathological findings of electric injury. (1) The electrostatic theory suggests that, if the victim is not grounded, electric charges build up in the body, producing electrostatic effects. Particles with similar charges repel each other, resulting in a sudden expansive force of the body tissues that raises waves of decompression under the skin. This may account for the tissue rupture seen in autopsy, however, it does not explain the unilateral predominance of clinical effects or abnormalities beyond the current pathway. (2) The vascular theory suggests vasoconstriction as the mechanism of the acute manifestations. Initial injuries and subsequent vascular thrombosis may account for the delayed manifestations. Animal studies have shown that pial vessels constrict when exposed to electric stimulation. (3) The mechanical theory suggests that the injury is caused by a violent jarring of the tissue because of the electrical current. (4) The heat theory suggests thermal injury to the brain tissue as the mechanism of the pathological findings. Cerebrospinal fluid temperatures as high as 145°F have been recorded 5 hours after legal electrocution.[]\nElectrical injury results in direct damage of the tissue because of the electric current and subsequent heat generation. The amount of direct damage depends primarily on the intensity of the current. Ohm’s law states that this current is proportional to the voltage and inversely proportional to the resistance of the tissue. Although we would expect the current density to be higher in nerves and blood vessels because of the low resistance, the tissues act as a volume conductor, with the composite resistance of all the tissue components.\nThe heat that is generated by the passage of the current can be quantified with Joule’s law, which states that heat is proportional to square of the voltage and inversely proportional to the resistance of the conductor.\nAlthough the resistance and voltage are important for determining the current and the heat generated, other factors that determine the clinical injury include the duration of contact with the conductor and the pathway of the current’s flow. Heat generation due to the passing current causes direct damage to the tissues. Robson et al. demonstrated that electrical injuries result in elevated levels of thromboxane A2. The high thromboxane A2 levels contribute to progressive tissue necrosis by causing vasoconstriction and thrombosis in the microcirculation. Partial or complete vessel occlusion may occur at the time of the injury, especially in smaller nutrient arteries, and delayed arterial thrombosis may also occur. Electrical injury to blood vessels causes varying degrees of damage to different layers of the vessel. Wang et al. examined aorta and pulmonary artery endothelial cells from electrocution victims and found that cell membrane perforations were present within 24 hours of the electrical injury, whereas after 24 hours, the endothelium disintegrated. Jaffe et al. found that electrical injury caused a complete loss of endothelial cells. The muscle fibers of the media were more sensitive to electric current, whereas the adventitia showed little change. The vessels lost their elasticity, and fusiform aneurysms were common.[]\nHuman body can transport electric current, but in an electric shock situation, similar to the case presented here, current distribution through the body depends specifically on each person as well as on the circumstances of the electric shock. Although dielectric properties of tissue have been described, particular circumstances play a major role in electrical injury, i.e., skin resistance, voltage breakdown of skin, contact area with source voltage, distance between points of contact, and of course source voltage. According to our patient’s set of images, we can try to study the current distribution and possibly the tissue injury in the first centimeters depth, from the contact point in his head. First, let us consider the dielectric properties of tissue shown in .\nIn a macroscopic approach, these properties can be used to analyze the different tissue behavior as a resistive element of current transport; hence, we can present a biological system injury which will have circuit properties of both serial and parallel circuits.\nTherefore, the electric power (P = I2 × R, from Ohm’s law) is a helpful measurement to compare the role of each resistive element in this biological circuit system, taking into account specific dielectric and geometrical properties we can estimate power deposition.\nAccording to power values shown in , most heat generation would be expected to occur in tissue with the highest resistive value if we use Joule’s law, H = I2 × R × t, current I is constant in every node of the circuit by conservation of electric charge. This is in skin and blood vessel tissue.[]\nIn our case, the injury must be secondary to mixed sources, mainly the heat generation hypothesis, i.e. the electric current passing through different tissue resistance damages the vascular tissue, that was the bleeding source, and the heat generation was capable to disrupt the chemical interaction between the oxygen and the hemoglobin. The high temperature leaves the accumulation of gas density in to the hematoma [].\nIn this case, the electric route was the scalp, cranial bone, blood vessels and brain, upper limb muscle, and skin. The damage was different according to the dielectric property of each tissue. The injury was in the scalp, cerebral blood vessel, skeletal muscle, and upper limb skin.\nThe main damage was in the brain’s blood vessels because of the dielectric and geometric features, leading to bleeding, high temperature, and gas delivering (maybe oxygen or carbon dioxide, delivered from the hemoglobin). It is remarkable to mention that although the blood vessel and brain tissue had similar calorific capacity, the blood vessel has higher resistance and it leads to higher temperature than the brain tissue. |
A 51-year-old female admitted on March, due to large amount of vaginal bleeding. On transvaginal ultrasound, uterine was woman's feast sized, globular 3 cm sized myoma were identified in anterior lower uterine and both ovaries were normal. Biopsy under the hysteroscopy was done and showed uterine AS with histological high grade with stromal overgrowth, 30–40 brisk mitotic figures in 10 high power fields, no heterologous component, microscopically tumor cell necrosis present. Clinical staging work up was performed including CT scan and Positron Emission Tomography CT and the disease was confined to the pelvis.\nShe received total abdominal hysterectomy, bilateral salpingo-oophorectomy and pelvic lymph node dissection on explorative laparotomy. On gross examination, the uterus is distorted due to bulging intramural mass. There was no evidence of peritoneal seeding at exploration or any lymph node involvement from resection. Pathologic diagnosis was ASSO, high grade (). Vascular invasion and lymph nodes involvement were not showed. The patient had received six cycles of paclitaxel and ifosfomide chemotherapy as adjuvant treatment and no evidence of disease was noted on follow-up imaging.\nAbout 4-year later, she presented to the emergency room with right-sided weakness for 2 hours. On physical examination the patient appeared acutely ill and vital sign was normal range. Motor grade in the right upper grade was IV, right lower grade was III and left side was V. sensory is intact, her cranial nerve examination was normal.\nBrain MRI revealed newly appeared well-enhancing lobulated mass with extensive surrounding edema in left frontal lobe measuring 3.8×2.8 cm and midline shifting to right side (). This lesion was metastasis more likely. CT scan of chest and abdomen/pelvis did not show any evidence of systemic disease. Dexamethasone treatment was started with an improvement of neurologic symptoms. Craniotomy with radical tumor excision in left medial frontal lobe was done. Tissue from the tumor was sampled, and an intraoperative frozen-section diagnosis of metastatic carcinoma was rendered. Pathologic diagnosis was metastatic sarcoma, consistent with uterine AS. Sarcomatous component was dominant in brain metastatic AS lesion compared to primary tumor (). Postoperative brain MRI demonstrated a gross total resection with no evidence of residual enhancing mass and her right upper and lower limb motor were recovered to grade V and the patient was discharged. Although postsurgical additional radiation therapy to brain was recommend, our case that underwent brain surgery refused subsequent radiation and chose close observation with no immediate additional active treatment. At a follow-up 12 months, the patient is still alive in good clinical conditions except mild neurologic deficit after resection and there was no evidence of tumor recurrence. |
A 6-year-old boy presented to the emergency department with a 2-week history of abdominal pain, vomiting, fever, and diarrhea. His physical examinations showed abdominal tenderness, his laboratory tests were of no significance except for a fecal occult blood, and the ultrasound revealed a prominent thickening of the descending and sigmoid colon wall. As a result, colonoscopy was performed revealing the emergence of an extremely edematous reddish mucosa and ulcerations covered by exudates lining the sigmoid and the ascending colon (). Endoscopic colonic biopsies revealed chronic inflammation with granulation tissue suggesting an inflammatory bowel disease (IBD) or an infection. Consequently, he was put on antibiotics, Prednisolone and Mesalazine.\nIn the following months, the patient did not show enough improvement, while conversely, he developed a persistent abdominal distention and pain in the left iliac fossa. Since our hospital does not own an MR Enterography; another colonoscopy was scheduled. Unfortunately, the colon was extremely friable it perforated, and the kid underwent surgery. At surgery, an old perforated necrotic area was spotted necessitating resection of the whole sigmoid (). The resected specimen was then sent to the pathology department for further examination. Grossly, the colon had multiple perforations, a stricture, and an inflamed and thickened area to the point where it almost closed the lumen (). Histologically, the H&E-stained sections of the sigmoid colon demonstrated extensive erosion of the overlying mucosa, interlacing fascicles of spindle-shaped cells that had Schwannian features, as well as ganglion cells in the muscularis propria and the submucosa of the sigmoid (). No signs of atypia were detected. Immunohistochemical stains were positive for S100 and synaptophysin ( and ), confirming the diagnosis of diffuse colonic ganglioneuromatosis.\nHowever, after the surgery he was admitted to the ICU, but few hours later, unfortunately, the kid developed cardiac bradycardia with respiratory depression due to excessive blood loss. He did not respond to resuscitation, and eventually died.\nThe child’s family history is unremarkable, and he did not display any characteristic clinical hallmarks of systematic disorders such as, marfanoid habitus in MEN2B, cafe’-au-lait spots and cutaneous neurofibromas in NF1. Also, he is not known for any congenital or genetic disease. |
We studied the case of a 76-year-old male patient who had left hip and leg pains for 4 weeks. He had a transforaminal lumbar interbody fusion on the L4–5 segments for both leg and back pains under a diagnosis of stenosis and instability of the same segments 6 months ago. Computed tomography revealed a displacement of the interbody cage into the left subarticular zone of the spinal canal. There was also subsidence around the interbody cage at the 4th and 5th vertebral bodies, and periscrew loosening (identified by a halo effect) was observed in all transpedicular screws. Magnetic resonance imaging demonstrated a severe spinal canal stenosis on L4–5 with compression of the left L5 nerve root by the migrated interbody cage ().\nFirst, the rods connected to the loosened pedicle screws on L4 and L5 were removed in posterior revision to create a sufficient interbody space by retraction with the lateral approach.\nOnce the lateral disc space was reached via the lateral approach, we followed a procedure for detaching the interbody cage from the L4 and L5 endplates using a Cobb retractor and osteotome. We made a gap between the interbody cage and endplates of the 4th and 5th vertebral bodies to make them movable and loose. After the interbody cage was completely loosened with additional curettages for surrounding scar tissues, we attempted to remove the interbody cage with a hook or pituitary force.\nHowever, the presence of subsidence entrapped the interbody cage into the body, and the interbody cage was difficult to remove from the intervertebral space. In addition, when the interbody cage started to rotate or move away during the catching and holding process, the situation became unfavorable, requiring additional time to complete the retrieval of the interbody cage. We also experienced these unfavorable situations several times before.\nTo avoid these situations, we designed and added new steps into the abovementioned process. A taphole (1-mm diameter) was first made on the surface of the interbody cage before loosening and moving the interbody cage. Thereafter, a long stick with a screw thread (with the bigger diameter size than that of the taphole, we used 2-mm diameter in this patient) at the tip was fixed on the interbody cage via the ready-made taphole (, ). Subsequently, a complete loosening of the interbody cage from the endplates and surrounding connective tissues was achieved; the cage was then removed from the intervertebral space.\nThe endplates of the disc space were prepared as a fusion bed, a new cage was inserted, and a lateral interbody fusion was performed. A posterior pedicle screw fixation was then added from L3 to S1, and pedicle screws with bigger diameters were used on the previous screw holes on L4 and L5. |
Case 3 presented the most complex clinical course. A 66-year-old female was referred to our hospital for SAH of WFNS grade I (E4V5M6) []. She underwent endovascular coiling for the aneurysm at left P2 segment of posterior cerebral artery []. However, on the 7th day after initial SAH, the patient revealed recurrent SAH with severe headache and slight drowsiness. Her catheter angiography demonstrated complete obliteration of left P2 aneurysm and abnormal aneurysm-like dilatation on ACoA []. Compared with CT angiography obtained at the first SAH [], this aneurysmal dilatation had been growing in 7 days, suggesting that unruptured posterior cerebral artery aneurysm was treated and the ACoA aneurysm ruptured twice. Endovascular treatment of this ACoA aneurysm was not feasible and microsurgical clipping through anterior interhemispheric approach was planned.\nOperation commenced with bifrontal craniotomy and the interhemispheric fissure was widely opened from genu of corpus callosum to rectus gyrus. There was no clippable component at the aneurysm neck because entire aneurysmal wall was disintegrated and the virtual neck of the aneurysm was only covered with a fragile fibrin cap []. Because aneurysmal neck clipping with the goal of ACoA preservation seemed difficult and right A1 segment was aplastic on imaging [], left superficial temporal artery (STA)-right ACA bypass was performed before dissecting the aneurysm in the event that aneurysm trapping was necessary []. Left STA was selected as a graft because it was larger than right STA. The parietal branch of left STA was harvested as an interposition graft. Both ends of the free graft were anastomosed with the frontal branch of the left STA and callosomarginal artery, in end-to-end and end-to-side fashion, respectively []. However, dissection of the aneurysm revealed that trapping of the longitudinal rent on ACoA contained a risk of sacrificing blood flow to the hypothalamic artery, the origin of which is adjacent to the aneurysm. Finally to spare the hypothalamic artery, an aneurysm clip was placed parallel to the rent on ACoA with slight incorporation of the arterial wall []. The intraoperative indocyanine green videoangiography confirmed blood flow both in ACoA and hypothalamic artery []. In summary, right A2 was reconstructed for potential trapping, but, in fact, neck clipping was performed with physiological flow of ACoA reserved.\nThe patient recovered well, although she was slightly confused due to symptomatic vasospasm. However, on the 7th day after the onset of the second SAH, the patient suddenly became comatose. Head CT scan demonstrated the third ictus of SAH with intracerebral and intraventricular hemorrhage []. Catheter angiography revealed irregular bulging of ACoA underneath the aneurysm clip, suggesting clip displacement and rerupture []. After confirming the left STA-right ACA bypass was still patent on the angiography [], reoperation was performed by the interhemispheric approach and the aneurysm was trapped with two aneurysm clips. The proximal clip was applied obliquely and the clip blade was placed just distal to the origin of the hypothalamic artery. Although blood flow of the hypothalamic artery looked diminished by the Doppler ultrasonography and the indocyanine green videoangiography, the clips were not replaced to prioritize complete trapping [].\nHer recovery of consciousness from comatose was excellent. Postoperative MRI revealed no cerebral infarction related to the surgical procedure []. She underwent ventriculoperitoneal shunt in the chronic stage. For a few months after operation, she manifested a wide range of cognitive dysfunction as frontal lobe syndromes, including memory disturbance and impaired comprehension, initiation, and motivation. She was able to walk independently, but most of her daily life was dependent in terms of cognitive function. However, after intensive rehabilitation, she was discharged home and was able to look after her own affairs without assistance. Her hypophyseal function was normal through her clinical course. Her mRS at 12 months was 2. |
A 47-year-old male patient was admitted to the pain department due to cancer pain in the right lower abdomen and inguinal area. He was diagnosed as having hepatobiliary adenocarcinoma with retroperitoneal metastasis. He had received multiple anti-tumor treatments during the past 3 years, and had had a pain in the right side of the abdomen for 8 months. Imaging revealed enhanced soft tissue thickening around the right paraspinal site at T11–12 (Fig. A). Thus, the patient underwent paravalvular palliative radiotherapy at a dose of 25 Gy. However, the analgesic effect of radiotherapy was negative. He complained about a persistent dull, burning pain and soreness accompanied by paresthesia in the T11–12 dermatome, with an average VAS score of 6 points (8 points at its worst, 3 points at its least), and an average DN4 of 6 points. The patient received oxycodone (120 mg/day) and pregabalin (300 mg/day), but his pain scores were not reduced as a result of the treatment. In addition, his sleep quality was severely affected and he could not even lie on his right side. According to the diagnostic criteria of the expert consensus, we diagnosed the patient as having refractory cancer pain. Then, this case was introduced through our multidisciplinary channel, and further analgesic strategy for this patient was developed by multidisciplinary decision making and a comprehensive informed consent. We decided that the neuraxial neurolysis could be one of the best next analgesic approaches for him because the metastasis was indenting the thecal sac at the T12 level, which is a potential contraindication for intrathecal pumps. In addition, further palliative radio- and chemotherapy or surgery were infeasible according to the experts’ decisions. However, we are still concerned about the outcome of the neurolytic blocks because the tumor invades around the block target. Therefore, an ultrasound-guided paravertebral block with 1% lidocaine in 5 mL was first performed. Fortunately, the patient responded well to the test block; hence, he was further administered a CT-guided paravertebral doxorubicin injection.\nTherefore, we obtained the approval from the ethics committee and the patient's informed consent, and performed a minimally invasive operation on the T11 and T12 nerve root segments. We determined the operation target based on CT scan images. We advanced 2 needles toward the T11 and T12 intervertebral foramina and tested a 3-mL analgesic dose (1% lidocaine). After 10 minutes, the patient reported pain relief and we proceeded to slowly inject 1 mL of 0.5% doxorubicin into the 2 sites (Fig. ).\nOn the first day after the operation, the patient reported significant relief from the right lower back pain and approximately 50% pain relief in the lower abdomen and groin. In addition, he could now sleep on his right side, and turning over was not aggravating the pain. One month later, the pain was relieved completely, and we detected hypoesthesia at the T11 and T12 dermatomes (DN4, 2 points). The pregabalin was reduced to 150 mg/day, and the use of oxycodone was stopped. In the second month after the operation, the patient received a course of chemotherapy for the cervical lymph node metastasis. The patient felt pain again (VAS, 3 points; DN4, 4 points) in the right lateral position at the 4th month, and we increased the pregabalin to 300 mg/day, which effectively controlled the pain. |
A 64-year-old man was referred to the ophthalmology department of Pusan National University Hospital for evaluation of tearing, nasal obstruction, and trifling epistaxis of the left side that had arisen 12 months earlier. His medical history was free of allergies, chronic sinusitis, and rhinologic pathologies. Ophthalmologic examination confirmed mild swelling in the left medial canthal area and obstruction in the area of the left lacrimal duct (). Visual acuity, intraocular pressure, ocular motility, and the anterior segment were within normal limits. There was no exophthalmos. The patient was referred to the otolaryngology department, and nasal endoscopies revealed the presence of a diffuse, smooth, and flush swelling mass in the inferior meatus. Computed tomography (CT) revealed that the tumor extended from inferior meatus to the entire length of the left nasolacrimal duct and sac, with involvement of the medial wall of the maxillary sinus (). We observed neither regional lymph node involvement nor metastasis to other organs. We performed external dacryocystectomy to remove the lacrimal sac and proximal nasolacrimal duct and endoscopic inferior medial maxillectomy, including medial wall of maxillary sinus, inferior turbinate, and lateral wall of nasal cavity.\nMicroscopically, the tumor demonstrated a destructive, infiltrative growth pattern and was composed of numerous irregular small nests of pleomorphic oncocytic epithelial cells with abundant eosinophilic cytoplasm. It was positive for pancytokeratin (). Adjuvant radiotherapy was recommended to the patient after surgery, but the patient refused further treatment. Nine months after surgery, the patient complained of purulent discharge with tearing, swelling, and tenderness in the medial canthal area of the left eye. An orbital CT revealed a mass in the medial orbital wall ().\nThe orbital mass was removed through an inferior transconjunctival approach, and histological examination confirmed oncocytic carcinoma recurrence (). The patient was referred for adjuvant radiotherapy to eradicate any residual tumor. He received computer controlled radiation therapy as 60 Gy in 33 fractions over 8 weeks. The patient remains free of tumor recurrence after 1 year. |
A 39-year-old man was involved in a high-velocity motor vehicle accident. He sustained an open fracture of the right elbow, with significant loss of the external humeral condyle and partial loss of the olecranon. This fracture was classified as a Gustillo type IIIA injury. There was no neurovascular compromise. The patient was treated in a community center, close to the accident, where he received surgical care (debridement and partial excision of the olecranon). The wound was fully closed. The upper arm was then immobilized in a splint (). IV antibiotics (cefazolin-gentamicin) were started for 5 days.\nThe day following his elbow surgery, the patient fell in a staircase and sustained a C7-C8 and C8-T1 fracture-dislocation. This injury caused neurologic damage (quadriparesis), and his right arm became his only functional limb. Following this injury, the patient was moved to our tertiary center to get spinal fusion. During the spinal surgery, the elbow was tested under fluoroscopy. The patient's elbow showed varus instability and a positive pivot shift test. A CT scan of the elbow was obtained the following day and showed bony loss from the external humeral condyle and subluxation of the radial head ().\nWe decided to treat the patient's elbow surgically. The surgery underwent nine days after the initial trauma (after transfer from the community center, spine procedure and elbow imaging). A posterior approach to the elbow was used along with an extensive elbow debridement. A tricortical iliac crest graft was then collected from the patient's right side to replace the humeral condyle bone loss. A tendinous graft was collected from his third and fourth extensor digitorum longus tendons to reconstruct the lateral collateral ligament. The tendinous graft was fixed to the bone graft through two tunnels (anterior to posterior and lateral to medial). The iliac crest graft was then fixed to the humerus with a cancellous screw. A five-hole one-third tubular plate was used as buttress. The graft was left prominent on the lateral side to improve lateral stability of the elbow. The lateral collateral ligament was then reconstructed by fixing the tendinous graft to the proximal ulna with two bundles. We took care to preserve our reconstructed lateral collateral ligament's isometry during flexion and extension movements. Reduction and stability of the elbow joint were verified under fluoroscopy. The wound was closed with staples, and a posterior splint was applied for three weeks. 14 days postoperative X-rays are presented on . At three weeks following the surgery, passive and active ranges of motion were initiated.\nWhile wearing the splint, the patient developed a pressure wound on the lateral side of his elbow (at the site of the reconstruction). This wound later complicated into a Nocardia septic arthritis, which required surgical debridement. Four months after the reconstruction, range of motion was 30-120 degrees with full pronation and supination, without any evidence of instability.\nTwo months later, instrumentation was removed and a rotational flap was done to increase soft tissue coverage of the articulation. Five months after that procedure, the patient developed a Nocardia osteomyelitis of the humerus and was treated with 6 weeks of IV antibiotics (penicillin) and a surgical debridement. The patient responded well to our treatment. Eleven months after the initial intervention, range of motion of the joint ranged from 30 to 120 degrees of flexion, without clinical instability. Osteosynthesis was achieved according to X-rays of the elbow (). |
A 40-year-old woman (gravida 4, para 4) was evaluated at our specialized center for rapidly progressive symptoms of heart failure in the first week postpartum. The patient’s medical background and first three pregnancies were unremarkable. The last pregnancy was uneventful except for a caesarian section for nuchal cord during a term spontaneous labor. She was prescribed antibiotics on day five postpartum for new onset dyspnea and dry coughing. She rapidly deteriorated with dyspnea at rest, orthopnea, and paroxysmal nocturnal dyspnea. Echocardiography showed a left ventricular ejection fraction (LVEF) of 10%, severe left ventricular dilatation with left ventricular end-systolic diameter (LVESD) of 58 mm and moderate functional mitral regurgitation. The electrocardiogram showed sinus rhythm and new onset left bundle branch block (LBBB) with a QRS width of 158 ms. Dobutamine perfusion was initiated for low cardiac output signs along with intravenous Furosemide. The coronary angiogram was normal and the Swan–Ganz catheterization showed a cardiac index of 1.71 L/min on inotropic support with normal pulmonary artery and capillary wedge pressures. The cardiac MRI showed no sign of inflammation or fibrosis. At this point, the differential diagnosis included peripartum cardiomyopathy and idiopathic dilated cardiomyopathy. However, the latter seemed less likely because of the absence of previous symptoms and the acute onset of symptoms in the early postpartum period, which is characteristic of peripartum cardiomyopathy. Valsartan 40 mg PO twice a day and Eplerenone 25 mg PO daily was initiated. Slow weaning of Dobutamine was completed after five days and small dose Metoprolol was eventually added. Despite uptitration of therapy, the patient did not improve. Bromocriptine was discussed with the patient considering some evidence to support its use but was not initiated because of the initial preference of the patient to breastfeed. However, the inherent difficulties of the long hospitalization led the patient to abandon breastfeeding []. At this point, complete pre-transplant assessment was performed. Considering the typical LBBB and enlarged QRS > 150 ms, we decided to implant CRT. An endovenous dual chamber pacemaker and defibrillator was implanted but unfortunately, due to unfavorable anatomy and ostial stenosis of the two posterolateral branches, the left ventricular pacing lead implantation failed. Considering the possible benefits and absence of significant clinical improvement, a minimally invasive thoracotomy was performed to implant a left ventricular pacing lead ().\nAfter the intervention, the patient improved to a NYHA functional class II. Control echocardiography showed resolution of mitral regurgitation and a mild increase in LVEF of 15%. The patient was followed at our specialized heart failure outpatient clinic and the titration of the optimal medical therapy was done including Sacubitril-Valsartan up to 49–51 mg PO twice a day, Bisoprolol 10 mg PO daily, and Aldactone 25 mg PO daily. The echocardiography follow-up showed increase in LVEF to 35% and 50% after seven and thirteen months respectively (). |
A 67-year-old Caucasian man presented to the emergency room because of a 4 days’ history of abdominal pain, with one episode of vomiting.\nThe patient’s past medical history was significant for colonic diverticulosis and an episode of gastrointestinal bleeding one year before. The event had been investigated by two different gastroscopies, a colonoscopy and a MDCT, which produced inconclusive results. Ten months later he was newly admitted because of abdominal pain and fever at 38.0 C°, with valid urination and defecation. A CT of the abdomen was performed, which confirmed the colonic diverticulosis and revealed the presence of multiple diverticula of the small intestine, fat stranding, signs of inflammation as well as a small amount of free liquid in the abdomen. The patient was hence diagnosed with jejunal diverticulitis and managed conservatively with intra-venous antibiotics, with an apparent complete recovery.\nHe re-presented to the emergency department two months later with acute abdominal pain. The pain was described as severe and constant, localized mainly in the lower abdomen with clinical signs of peritonitis. No change in bowel habits nor urinary symptoms were complained. His vital signs were stable, with a temperature of 37.2 C°; he appeared fully oriented and not in any acute distress. Laboratory examination reported a hemoglobin of 121 g/dL, a WBC count of 12.2 × 10E9/L and a CRP of 249 mg/L. Other laboratory data were within normal limit. An abdominal and pelvic contrast-enhanced computed tomography, with administration of oral contrast, was performed. Jejunum and ileum showed several diverticula as well as an inflammatory thick-walled mass involving different loops of the intestine. In addition, free fluid in the abdomen and a small amount of subdiaphragmatic air were reported (, ). On the basis of these findings, the diagnosis of perforated diverticulitis was hereby proposed.\nThe patient underwent a diagnostic laparoscopy which revealed plenty of purulent yellowish liquid collected in the right abdomen and a conglomerate of intestinal inflamed loops. We hence decided to convert immediately the procedure to laparotomy. Large multiple diverticula were found covering a section of small intestine approximately 2.5 m long, without signs of obvious macro perforation. Among the middle distal tract of the jejunum and the middle distal tract of ileum, strong adhesions were identified (). The involved segments of jejunum and ileum were connected by an intestinal loop free of signs of diverticulosis (). There were no signs of bowel ischemia. Adhesiolisis was partially carried out, however, because of difficulties associated with the procedure, we opt to perform a double enterectomy, removing only those segments involved in the intestinal conglomerate and deeply affected by the pathology. Roughly 25 cm of ileum and 80 cm of jejunum were resected. Bowel continuity was restored with an ileo-ileal and a jejuno-jejunal anastomosis. Almost 700 cl of pus were drained and the peritoneal cavity was washed with 10 L of saline solution. The postoperative recovery was uneventful and the patient was discharged 8 days later. There were no signs of malignancy in the resected intestine. |
A 74-year-old female was referred to the surgical oncology clinic following an incidental finding of a right retroperitoneal mass initially identified on transthoracic echocardiogram. As part of the work up for this mass, a computerized tomography scan of the abdomen and pelvis was obtained. The CT scan revealed an 18 cm × 14 cm mass in the right retroperitoneum. The mass was noted to displace the right kidney inferiorly and medially (Fig. ). An additional 6.1 cm × 4.2 cm ovoid solid lesion was also seen posterosuperior to the larger retroperitoneal mass and reported as a possible right adrenal mass or exophytic hepatic mass (Fig. ).\nA full biochemical panel for adrenal incidentalomas was done and the tumor was found to be non-functioning. Subsequent to this, a CT-guided biopsy was performed for the masses. The larger, right retroperitoneal mass returned a diagnosis of neurilemmoma (schwannoma). The posterosuperior mass was also biopsied and the findings were reported as patchy hepatocyte atypia which was inconclusive of a definitive diagnosis.\nIntra-operatively, significant findings included a huge right retroperitoneal mass which displaced the right kidney inferomedially. This mass was adherent to the right lateral aspect of the IVC and the right renal vein coursed on its anteromedial surface. The mass was carefully mobilized away from the IVC without any violation of its capsule. The right renal vessels were also salvaged thereby ensuring preservation of kidney. The mass was successfully removed with its capsule intact. A completely separate and encapsulated mass was identified posterosuperior to the larger mass. This mass had vessels directly communicating with the IVC.\nPathologic analysis revealed that the large right retroperitoneal mass measured 22 cm × 15 cm × 7 cm and weighed 2289 g (Fig. ). Histologically, the tumor showed spindle cells with focal nuclear palisading, nests of foamy histiocytes, patchy chronic inflammation and rare enlarged polymorphic nuclei. No mitosis was identified (Fig. ). With immunohistochemical staining, the tumor cells were positive for S-100 (Fig. ) and vimentin but negative for CD34, CD117, SMA, desmin and pancytokeratin. Both morphology and immunostains were consistent with neurilemmoma (schwannoma).\nThe smaller right retroperitoneal tumor weighed 177 grams and measured 10 cm × 8 cm × 5 cm (Fig. ). Histopathology showed an oncocytic neoplasm with focal zellballen growth pattern, abscess formation and recent hemorrhage (Fig. ). The tumor cells were strongly positive for NSE, focally positive for S-100 but were negative for inhibin, Hepatic Specific Antigen (HSA), pancytokeratin, PAX8, EMA, chromogranin-A and synaptophysin. Ki-67 stained ~5% of tumor nuclei (Fig. ). The final diagnosis was an oncocytic variant of adrenal cortical adenoma (oncocytoma). |
A 59-year old male pedestrian was hit by a vehicle on an expressway. He was brought to the Emergency Department and immediately intubated due to a low Glasgow Coma Scale Score. He underwent resuscitation according to ATLS protocol and underwent fluid and blood product resuscitation. Venous access was poor, therefore, intraosseous lines were inserted into both proximal tibiae for infusions. Into his left tibia intra-osseous line, two units of packed red cells (approximately 500 mls in volume) and 1000 mls of crystalloid were infused over a period of two hours. The right tibia line was not used.\nAfter initial computer tomography scans from head to pelvis the following injuries were identified: Subarachnoid haemorrhage with frontal lobe haemorrhagic contusions; multiple facial bone fractures; left pulmonary contusion, pneumothorax with left flail chest; left iliac wing pelvic open fracture with sacro-iliac joint disruption and an overlying degloving injury and left clavicle fracture.\nAfter urgent angioembolisation of the distal branches of his left superior gluteal artery and distal branches of his iliolumbar arteries, he underwent insertion of an intracranial pressure monitor and venous cut down for vascular access to substitute the intra-osseous lines.\nAt the end the surgery the patient’s left leg was notably tense and swollen. The orthopaedic team reviewed him in the operation theatre and clinical examination revealed a tense and cool leg, non palpable distal pulses, with no external clinical evidence of fractures or soft tissue contusion. Compartment pressure was measured at 199mmHg for the deep posterior, anterior and lateral compartments and 140mmHg for the superficial posterior compartment.\nA standard two-incision fasciotomy with four compartment decompression was performed immediately revealing approximately 500ml of blood clots and bulging calf muscles which were contractile and viable. There was no obvious source of active bleeding that needed hemostatic control. Distal pulses returned following the fasciotomy. A plain radiograph of his left tibia showed a non-displaced fibula shaft fracture which was treated non-operatively. The clinical picture prior to initial fasciotomy and the subsequent radiograph are shown. ()\nAfter two further debridements, his leg fasciotomy wounds were closed without skin grafting. During his two-month inpatient stay, he achieved full neurological recovery. However, due to the pelvic injury, he still required walking aids six months after the accident. There was no long term complication directly linked to his compartment syndrome or its treatment. |
A 57-year-old African-American male presented to the emergency department via emergency medicine service (EMS) with witnessed cardiac arrest. Patient reportedly was at work talking to a coworker when he collapsed and received immediate cardiopulmonary resuscitation (CPR) on scene. Patient was found in ventricular tachycardia arrest by EMS and received advanced cardiac life support (ACLS) protocol treatment with ROSC en route to the emergency department. Upon department arrival, the patient arrived with supraglottic device in place and remained unresponsive. No obvious signs of trauma were noted; however, the patient was noted to have leftward gaze with bilateral pupils responsive to light. The patient then went into ventricular tachycardia arrest upon being placed on department bed. CPR and ACLS protocol were initiated and continued for approximately three rounds when ROSC was achieved. Epinephrine drip was started and definitive airway was established. Soon after, the patient was noted to have spontaneous movements with opening eyes and was placed on Diprivan drip for sedation. While preparing for central line placement to the right internal jugular, it was noted that the patient’s left eye became significantly proptotic and chemotic appearing without pupil reactivity to light. IOPs were then immediately measured with tonometer device demonstrating pressure of 35 mmHg to the left eye and approximate pressure of 8 mmHg on the right. Decision to perform cantholysis was made given these findings. There was concern of retrobulbar hematoma likely in setting eye trauma upon patient collapsing prior to cardiac arrest. Using materials from laceration kit commonly found in the emergency department, hemostat pressure was applied to lateral canthus using Kelly clamp and then careful dissection of lateral canthus was made using tissue scissor. Upper and lower eyelids were retracted for better visibility of the superior and inferior crux, which were both cut given the extent of proptosis and chemosis (Figure ). Post canthotomy pressure was measured at 11 mmHg and the left pupil was noted to be reactive. After surgical decompression, the patient underwent CT imaging of the brain which demonstrated soft tissue swelling of the left orbital region without evidence of retro bulbar hematoma (Figure ). The patient was admitted to the medical intensive care unit for stabilization and later transferred to a tertiary care center for further ophthalmologic evaluation. |
A healthy 20-year-old female came to our clinic with a complaint of progressive, continuous, and long-standing history of bilateral nasal obstruction that was worse on the left side. She was having frequent thick mucoid nasal secretions, left-sided facial pain, and intermittent hyposmia, also it was associated with moderate to severe allergic rhinitis symptoms; she had no visual disturbances. The patient had already tried intranasal corticosteroids with no satisfactory improvement.\nA nasal endoscopic exam revealed a deviated nasal septum to the right side, a mildly hypertrophied right inferior turbinate, and a large mass occupying the left nasal cavity. This mass had displaced the nasal septum to the contralateral side and compressed the left inferior turbinate. Using the freer and fraiser suction, the mass was found to be hard but with normal overlying mucosa (Figure ).\nIt originated from the skull base on the endoscope, with small nasal polyps noted beside it, but a further passage to the nasal cavity was not possible. The rest of the clinical exam, including her cranial nerves and orbital exam, was unremarkable.\nThe patient came to our clinic for a second opinion, as she was concerned about the results of her medical imaging, which included CT scans and magnetic resonance imaging (MRI) of the paranasal sinuses. These reports commented on a bony nasal lesion measuring 1.7 × 3.7 × 3.0 cm and arising from the left skull base. The lesion was obstructing all ipsilateral paranasal sinuses with complete opacification and displacing the nasal septum to the contralateral side. Fortunately, there was no intracranial or orbital extension. No comments were made regarding heterogeneous opacification. These findings were thought to be an aneurysmal bone cyst, mucocele, mucopyocele, or other sinonasal tumors.\nHowever, upon careful study of the imaging, AFS was considered due to the heterogeneous nature of the content, the bony expansion of the lesion in the CT scans, the low signal intensity on T2-weighted images, and the heterogeneous high signals on T1-weighted images (Figures -).\nShe was then assured and counseled for surgery. The patient had a routine and unremarkable preoperative assessment and then underwent functional endoscopic sinus surgery, polypectomy, and septoturbinoplasty (resecting whole middle turbinate) under general anesthesia.\nIntraoperatively, the mass was found to be a huge CB of the left middle turbinate that was filled with debris. Biopsies were taken from its mucosa and debris for histopathological staining and regular culture. It was resected, and all ipsilateral sinuses were opened and drained. The skull base and orbital wall were intact, and the surgery was uneventful (Figure ).\nThe patient was given a tapered dose of oral steroids over 15 days and had an uneventful post-operative course.\nThe histopathologic examination revealed amorphous debris with focal calcification and inflammatory cells with no evidence of malignancy. The material sent for fungal stain was reported positive. The intraoperative nasal swab result showed no pathogenic bacteria isolated after 48 hours of incubation at 37°C. In the subsequent postoperative visits, the patient remained asymptomatic, and the endoscopic exam showed a patent nasal airway, healthy mucosa, and no signs of recurrence. |
We report a case of spindle cell sarcoma of the breast in a 76-year-old man. He presented to the Daegu Catholic University Hospital with a lump in his left breast that had been present for the previous two months. He had been taking medication for hypertension and benign prostate hypertrophy and had not suffered trauma to his chest wall. Further, he had no family history of malignancy, including breast cancer.\nOn physical examination, the patient had a poorly demarcated, mobile, firm mass in his left breast. The mass was nontender, approximately 1 cm in diameter, and was detected in the subareolar area of the left breast. There was no clinical evidence of regional lymphadenopathy, and there were no abnormal findings in the right breast. Mammography revealed a dense lesion occupying the subareolar region; this lesion was consistent with prominent fibroglandular tissue and suggested asymmetric left gynecomastia (). Ultrasonography revealed a poorly demarcated and highly suspicious malignant lesion in the periareolar area of his left breast, and the lesion was categorized according to Breast Imaging Report and Data System (BIRADS) as BIRADS 4C (). He underwent ultrasound-guided core needle biopsy, which indicated the presence of atypical cells in the fibrous, proliferative lesion (). Preoperative examination consisted of a complete blood count, serum kidney and liver function test, thyroid function test, and tests for the levels of several hormones related to the development of gynecomastia, including estrogen, testosterone, prolactin, and gonadotrophic hormones. All results were within the normal limits.\nThe patient underwent wide excision of the lesion, including removal of normal breast tissue to provide a safety margin and he did not require subsequent axillary lymph node dissection. Gross examination of the specimen revealed a whitish, fibrotic nodular lesion, measuring 1.5×1 cm in size including surrounding adipose tissue. The specimen was fixed in 10% formalin. Paraffin sections were prepared and stained with haematoxylin and eosin (H&E). Microscopic examination of the sections from the specimen showed nodular proliferation of fibrous tissue with focal infiltrating margins (). There were no ductal components and epithelial tissues. The nodules were composed of plump to spindle-shaped fibroblasts, many lymphoplasma cells, eosinophilic infiltrate, and many keloid-like collagen bundles (). A few atypical multinuclear giant cells and pleomorphic cells were noted; however, abnormal mitosis was not identified. Immunohistochemical staining for desmin, smooth muscle actin (SMA), and S-100 protein was negative (). These diverse histological and immunohistochemical findings established the diagnosis of atypical spindle cell lesion with uncertain malignant potential, exhibiting features of a reactive fibroblastic lesion.\nThe patient's progress was monitored after the operation, and no specific adjuvant treatment was administered. One year later, he returned with a recurrent mass at the previous surgical site. On physical examination, a firm mass of about 2 cm in diameter was palpated in the wound from the previous surgery. Radiological studies, including computed tomography (CT) and positron emission tomography-CT (PET-CT), were conducted, and PET-CT showed faint fluorodeoxyglucose uptake in the area of the left breast that corresponded to the lesion seen on the CT scan (). There was no evidence of either regional lymphadenopathy or distant metastasis. The lesion was surgically removed using wide excision.\nA histological assay showed that the recurrent tumor was 3.8 cm sized in diameter and had an irregular margin. On sectioning, the cut surface showed fibrous bands and lobular adipose tissue with focal congestion without necrosis. The nodules were composed of plump to spindle-shaped fibroblasts, many lymphoplasma cells, eosinophilic infiltrate, and many keloid-like collagen bundles. Microscopic findings were similar to the previous histological findings (), but many atypical cells and abnormal mitotic activity ranging from 6 to 10 mitotic figures per 10 high power fields (hpf) were noted and the resected margins were involved with the tumor (). Based on the rapid recurrence of the mass, increase in tumor size, high degree of cellularity, presence of atypical cells, many abnormal mitotic figures, and infiltrating growth pattern, malignancy was indicated. The immunohistochemical staining for CD34 showed focal positivity; however, the cells were not reactive for other immunomarkers including cytokeratin (CK), epithelial membrane antigen (EMA), SMA, murine double-minute protein 2 (MDM2), desmin, S-100 protein, CD68 and anaplastic lymphoma kinase (ALK). These established histological features of malignant pleomorphic spindle cell tumor in addition to the immunohistochemical results demonstrated no line of differentiation and led to the diagnosis of undifferentiated pleomorphic sarcoma of the breast. Due to the involvement of the resected margin, the patient underwemt simple mastectomy. |
A 45-year-old Thai male presented with a six-month history of right buttock and hip pain. The patient could not recall any injuries or incidents that may have caused the pain. He had no medical problems nor was he taking any medication. The MRI of the lumbosacral spine from the initial consultation at another hospital was unremarkable. He was treated unsuccessfully with a non-steroid anti-inflammatory drug and acetaminophen. He was referred to our institution for consultation. Physical examination revealed a healthy-appearing adult with an ill-defined 12 cm × 20 cm nontender firm mass at frank and groin area. There was no overlying skin change. Both hips had a full range of movement. The neurovascular function in the field of lower extremity was found to be intact, except the power of extensor hallucis longus on the right side was grade 4/5. He had no palpable lymphadenopathy. Laboratory studies were within normal limits.\nA plain radiograph of the pelvis revealed a well-defined mineralized mass extending from the right pubic and periacetabular area that also extended to the left pubic bone. The right obturator foramen was obliterated by the tumor mass. There was a popcorn-like chondroid matrix at the soft-tissue part of the tumor, which also extended into the pelvic cavity (Figure ). Bone scintigraphy and computed tomography (CT) of the chest showed no distant metastasis except the abnormal uptake of the right pelvic and hip area. The CT scan of the pelvis showed a huge tumor mass in the pelvic cavity that involved the right sacroiliac joint and anterior border of the sacrum (Figure ). The initial diagnosis was a chondrosarcoma involving both pubic areas, the right periacetabulum and ilium.\nIncisional biopsy was performed over the right pubic area. Microscopic examination revealed small chondrocytes with dark nuclei and scant cytoplasm. They were arranged in clones and binucleated cells were present. No mitotic figures were found. The diagnosis was consistent with chondrosarcoma. The tumor was classified as Grade I (well-differentiated) (Figure ) according to the musculoskeletal tumor society staging system []. In this patient, the tumor resection was at PI-II-III, according to the Ennecking and Dunham classification [] for the resection of primary tumor involving the pelvic bone. An internal hemipelvectomy with wide margin tumor removal was performed in this patient using the modified technique described by Eilber et al. []. The incision was made from left inguinal area to right pubis and anterior superior iliac spine, then curved superoposterior to iliac crest. The previous biopsy scar was ellipsed out from the incision. The external iliac vessels, femoral and sciatic nerves were preserved and mobilized from the tumor. The muscles attached to the pelvic bone were dissected out from the affected pelvis such as rectus abdominis, abdominal muscles, iliacus, gluteal muscles, rectus femoris, adductors and hamstrings. Osteotomies were made through the sacral ala just lateral to the neural foramina and contralateral pubic rami and ischium. A polypropylene mesh (Parietene, Sofradim, Trevoux, France) was used as an anchoring mesh to maintain all dissected muscle from the pelvis and soft-tissue reconstruction (Figure ). All muscles were sutured along with mesh by prolene no. 0. They were kept attached with mesh at their optimal anatomical length to gain maximal muscle strength. Gluteal, lumbosacral and hamstrings were firstly sutured behind the mesh. Then, abdominal muscles, rectus abdominis, rectus femoris and adductor were sutured respectively by their anatomical layers. The final pathological report confirmed a well-differentiated chondrosarcoma with free margin. The radiograph of the pelvis following an internal hemipelvectomy showed upward migration of the head of the right femur (Figure ).\nPatient was placed in balanced skeletal traction at the tibial tuberosity with a weight of fifteen pounds for three weeks after surgery. Rehabilitation was uneventful. Progressive partial weight-bearing with axillary crutches was allowed after six to eight weeks. Regular strengthening exercises of the psoas, gluteus, quadriceps, and hamstring muscles were started and maintained to the highest level of tolerance during this period. At 7.5-year follow-up, the patient remained disease-free without local or distant relapse of the disease. No infection or wound complication occurred in this patient. Partial peroneal nerve palsy occurred, but was improved at the last visit. The patient had regained a range of motion of hip abduction of 15° and a range of hip flexion of 15°. The functional analysis at the final follow-up according to the Musculoskeletal Tumor Society system [] was 66.7 %. He had a 3 cm leg-length discrepancy, which could be compensated by simple shoe lifts. He was able to perform most activities of daily living without any assistance. He could resume his employment as an electrical engineer by using one Canadian crutch during walking and could participate in sports exercise such as swimming or bicycling (Figure ). |
A 30-year-old female presented in the Department of Periodontics, SRCDSR, with a localized gingival swelling in relation to interdental gingiva of mandibular left canine-first premolar region []. It was present since 1 year and had slowly grown to this size, causing displacement of both the teeth and creating a space between them. Patient gave a vague history of presence of similar growth in the same region around 11 years back which she got excised in a government hospital, but no records could be obtained regarding that either from the patient or from the hospital. On examination, the swelling was approximately 0.8 × 1 cm in size [], was found to be sessile, nonlobulated, sharply demarcated but otherwise of the same color as the surrounding mucosa. On palpation, it had a fibrotic consistency. Complete full mouth periodontal examination revealed the presence of generalized 3-4 mm deep pockets and subgingival flecks or complete bands of calculus. Patient had a very poor oral hygiene. A clinical diagnosis of generalized mild to moderate chronic periodontitis with focal fibrous hyperplasia/irritation fibroma was made. IOPA Intra oral periapical radiograph (IOPA) of the involved area was taken which revealed a horizontal bone loss []. Excisional biopsy was done along with open flap debridement as the patient was suffering from periodontitis. The growth was removed with an internal bevel incision and root surface was scaled and planed thoroughly. Periodontal pack was placed and the patient was recalled after 7 days for pack and suture removal. At follow-up, the area seemed to heal well. The patient is getting treated for periodontitis now and will remain on a regular follow-up of 3 months in the future. A 5-week follow-up photograph has been shown [].\nMicroscopically, the lesion showed partially circumscribed tumor mass exhibiting interlacing fascicles of collagen fibers and scattered abundant odontogenic nests and strands []. Intervening connective tissue also consisted of numerous localized areas of chronic inflammatory cells (mainly plasma cells) and few multinucleated giant cells. The overlying epithelium was hyperplastic, parakeratinized stratified squamous epithelium with thin, long rete ridges. |
A 51-year-old male with a history of nonischemic cardiomyopathy with a left ventricular assist device was admitted for expedited heart transplant evaluation. The evaluation included an elective colonoscopy in light of a family history of colorectal cancer in his mother who died at age 61 from the disease. The patient had his first screening colonoscopy at age 45 and was diagnosed with benign polyps, which were removed, and left-sided diverticulosis. The procedure was uncomplicated and he was advised to repeat a colonoscopy in five years.\nThe patient was without GI symptoms at the time of his colonoscopy. He denied tobacco, alcohol, or illicit drug use. His medications included amiodarone, aspirin, famotidine, levothyroxine, lisinopril, metoprolol, sildenafil, and intravenous heparin as well as torsemide, acetaminophen, docusate sodium, and melatonin as needed. On examination, he had a left ventricular assist device port entering at the upper abdomen, but otherwise the abdomen was soft and nontender to palpation with normal bowel sounds and no appreciable masses or ascites.\nThe patient underwent a standard bowel preparation which included a clear liquid diet the day prior to the procedure and 20mg of Dulcolax with 4 liters of polyethylene glycol the night prior to the procedure. Monitored anesthesia care sedation was administered with propofol. The colonoscopy was performed at night without difficulty with good bowel preparation. Abdominal pressure was briefly required to maneuver around the splenic flexure. The colonoscope was advanced to the cecum with identification of the appendiceal orifice and ileocecal valve. Findings included multiple sigmoid and descending colon diverticula and two, small (<5mm) sessile polyps that were removed using cold forceps.\nThe night of the procedure the patient had no pain or nausea and ate dinner and breakfast the following morning without incident. He then developed epigastric abdominal pain in the midmorning approximately 12-14 hours after the procedure and had one episode of nonbloody, nonbilious emesis following lunch. On physical examination, he was afebrile with a blood pressure of 104/89mmHg, heart rate of 68 beats per minute, and oxygen saturation of 100% on room air. Abdominal examination was notable for mild distension and moderate tenderness to palpation in the epigastric region without guarding or rebound tenderness and decreased bowel sounds. Laboratory examination revealed an elevated lipase of 2275 U/L and amylase of 1141 U/L. Additional abnormal laboratory findings included an elevated aspartate aminotransferase of 105 U/L, alanine aminotransferase of 94 U/L, and total bilirubin of 1.4 μmol/L (normal prior to the procedure). An abdominal X-ray did not reveal an obstructive bowel gas pattern or evidence of free air. A computed tomography (CT) scan of the abdomen/pelvis revealed diffuse edematous changes of the pancreas with surrounding inflammatory stranding in the bilateral paracolic gutters, extending superiorly to the perihepatic region and inferiorly to the pelvis (). The constellation of symptoms, labs, and imaging were suggestive of an episode of acute pancreatitis.\nThe patient was treated conservatively with bowel rest, intravenous fluids, and analgesics as needed. Over the next three days his symptoms and abdominal examination improved and his diet was advanced to a regular diet. The lipase normalized to 15 U/L. |
A 35-year-old, 70 kg, 180 cm, male patient with traumatic cervical spine injury was admitted to our intensive care unit with chief complaints of labored breathing and partial loss of power in all four limbs. After securing the airway with a tracheal tube, central venous cannulation was attempted using 7 F20 cm triple lumen catheter (Certofix, Braun, Germany). Under strict aseptic precautions landmarks were identified and right SCV was located infraclavicular at medial 2/3 and lateral 1/3 of the clavicle using introducer needle in single attempt. After confirming good backflow of the blood, guide wire (50 cm with j-tip), was introduced up to 15 cm mark without any resistance. After that tissue dilator was threaded over the guide wire followed by railroad of triple lumen catheter (up to 13 cm) over it, although the line was inserted in single pass but resistance was encountered during removal of guide wire. Gentle traction was applied several times to remove it but all in vain. Bed side chest roentgenogram revealed the guidewire going through right SCV towards SVC but after its entry it took sharp turn and entered IJV on the same side []. With bed side X-ray as a control we tried to remove the guide wire, pushing it caused the assembly to move inside the right atrium (arrhythmia elicitation) but attempts at removal resulted in decoiling at the site of insertion. This prevented the applied force from being transmitted to the stuck component. Further attempts at removal of the guidewire were deferred and patient was shifted to intervention radiology suite. The attempts at removal of guidewire by passing a tissue dilator to straighten the kinked portion were not met with success, so through percutaneous right transfemoral venous route a vascular snare was used to pull the guide wire out of IJV []. It was followed by successful railroad of long and large bore tissue dilator from the unraveled to the intact segment of guide wire from the insertion site. The incision at site of insertion was then deepened to remove the kinked portion []. Another guidewire was passed through the same dilator followed by insertion of triple lumen catheter. A post-procedural chest radiograph revealed no evidence of hemothorax or pneumothorax. |
A 48-year-old female Chinese patient was admitted to our department for discontinuation of bowel movements for 2 months. The patient reported the alternation of constipation and diarrhea after an abdominal mass was found 3 years earlier. No laxatives or illicit substances had been used. She had chest tightness and shortness of breath. However, there were no signs of intestinal obstruction, including vomiting or abdominal pain. Family history was unremarkable. On examination, the abdomen was grossly distended, and a hard, round mass was found in the right lower abdomen. No peritoneal irritation was found, and rectal examination revealed an empty rectum.\nThe carbohydrate antigen 125 level in the blood was 91 U/mL, and the hemoglobin was 103 g/L. The serologic test for syphilis was positive. Abdominal computed tomography (CT) revealed fecal accumulation and dilatation of the colon above the sigmoid colon and suspicious wall thickening of the sigmoid colon (Fig. ). Anorectal manometry revealed relaxation of the anal sphincter.\nConservative treatment was ineffective, and an urgent surgical intervention was planned. The laparotomy revealed a significantly dilated transverse and descending colon (Fig. A) and the sigmoid colon was redundant and narrow. The transverse colon was cut open, and a drainage tube was placed to flush intestinal feces. We excised the sigmoid colon partially and performed a transverse colostomy.\nThe postoperative period was uneventful. Histological examination of the sigmoid colon showed chronic inflammation of the intestinal mucosa and a decreased number and the vacuolar degeneration of ganglion cells in the myenteric plexuses, on average more than 4 cells/cm (Fig. B). Inflammatory cells had infiltrated the serosa, and the blood vessels were dilated and bruised. Immunohistochemistry showed the tissue was positive for soluble protein-100 (S-100), synaptophysin, and neuron-specific enolase. The patient did not have a history of chronic constipation or pseudo-obstruction, and was diagnosed as having acquired segmental HG. Since there was focal stenosis of the sigmoid colon, the condition was typed as type I HG.\nThree months after the surgery, a barium enema revealed a recovery in colorectal dilatation (Fig. ). Since her discharge following surgery, the feces at the colostomy were excreted unobstructed and the abdominal volume was significantly reduced. Chest tightness and shortness of breath had disappeared. |
A 68-year-old man with no history of homosexual activity presented with a painless ulcerated dark reddish nodule on the penile shaft noticed 3 months earlier. He had both legs amputated because of a bomb explosion during the Korean War. The patient had undergone three transurethral resections of bladder tumor (TURBTs) due to urothelial cell carcinoma since he visited us with a chief complaint of painless gross hematuria in September 2000. Also, the patient underwent total gastrectomy with splenectomy on account of advanced gastric carcinoma in May 2005 and six treatments of adjuvant fluorouracil (5-FU)/cisplatin chemotherapy from July 2005 to January 2006. On the physical examination, an approximately 1 cm sized nodule with pus-like discharge was evident on the outer layer of the dorsal prepuce and two black discolorations on the glans were obvious. There was no evidence of inguinal lymphadenopathy. The results of routine laboratory investigations and urine examinations were normal. Circumcision including the ulcerated nodule was performed. Purple discoloration and profuse bleeding were evident on the field excised nodule. By histologic examination, the nodule with ulceration was diagnosed as the nodular stage of KS. Histologic findings showed an infiltration composed of spindle cells scattered between collagen bundles and small vascular proliferation. Slit-like spaces containing red cells with marked nuclear pleomorphism and mitoses were observed. Immunohistochemical investigations revealed a vascular tumor because of reasonable positivity for vascular markers such as CD31 and CD34, and immunohistochemical staining confirmed KS by diffuse nuclear staining of human herpesvirus type 8 (HHV-8) latent nuclear antigen 1 (LNA-1) ().\nAt the repeat physical examination, we found no other skin lesions. We performed additional local excision of two black discolorations on the glans. However, they were reported as keratosis. Repeated HIV tests (enzyme-linked immunosorbent assay and Western blot) were negative. Computed tomography scanning of the chest, abdominal cavity, and pelvis failed to detect any visceral lesions. A 2-year follow-up did not show any disease progression or recurrence from May 2008 to June 2010. |
A 61-year-old woman fell over a barrier curb while walking and bruised her right shoulder. A local clinic examined the patient and diagnosed her with anterior dislocation of the shoulder with a glenoid rim fracture (). The right shoulder had been immobilized with a sling for a month after immediate reduction (). However, the active range of motion for the shoulder remained limited. An MRI scan two months after injury revealed rupturing of the supraspinatus tendon. The patient was referred to our hospital for further management. Clinical examination found stiffness in the right shoulder with muscular weakness. Respective ranges for active and passive right shoulder flexion were 30 degrees and 120 degrees. There was sensory disturbance over the lateral aspect of the shoulder. Radiographs and CT scanning revealed a large anteroinferior glenoid rim fracture, a type IA fracture under the Ideberg classification [, ]. The articular surface was displaced by 2.5 mm, and the width of the bony fragment was 30% of the glenoid length. Upon initial presentation at our hospital, the fracture had already achieved partial union (); therefore, we treated the glenoid rim fracture conservatively. An MRI scan showed a large tear approximately 3 cm long in the supraspinatus tendon, with atrophy and fatty degeneration of the supraspinatus muscle (). Electromyography and nerve conduction studies showed an incomplete lesion of the axillary nerve; therefore, we diagnosed axillary nerve palsy in addition to glenoid rim fracture and rotator cuff tear. The axillary nerve palsy was incomplete and recovering. Following improvement in manual muscle testing (MMT) of the deltoid from grade 4/5 to 5/5 and the disappearance of the sensory disturbance three months after injury, we performed an arthroscopic rotator cuff repair using a suture bridge technique (). Shoulder function recovered satisfactorily with approximately full ranges of motion one year after surgery. Constant score recovered from 38 points to 75 points. |
The patient was a 47 years old lady who came in to the emergency ward due to severe abdominal pain with a bending position like the typical presentation of acute appendicitis. The associated symptoms were nausea and non-bilious vomiting. There was no history for associated melena, diarrhea, constipation or obstipation. The patient has had several similar attacks during the last two years that referred to many physicians and has also had 3 times hospital admission without definite diagnosis. Previous workup including abdominal sonography, small bowel follow throw and abdominal CT scan were inconclusive. A gastroenterologist who has visited the patient during an acute abdominal pain attack and suspected a clinical diagnosis of jejunal volvulus, but after esophagogastroduodenoscopy (EGD) the patient had lost her follow up due to poor compliance. In new presentation surgical visit was done without definitive diagnosis so EGD and full colonoscopy and terminal ileal intubation were done with negative results. In the last episode of abdominal pain and with suspicion to internal hernia, we decided to do MRI enterography, which was in favor of left paraduodenal internal hernia (LPDIH). The patient was operated.\nDiagnosis of left paraduodenal internal hernia rarely can be made preoperatively. The presentation is indistinguishable from other causes of mechanical small bowel obstruction. Imaging plays an essential role in diagnosis of SBO and LPDIH. MRI enterography can be diagnostic in suspected cases and is a useful and safe technique due to high soft tissue contrast resolution, lack of radiation, detection of intraluminal and extraluminal as well as mural lesions. MRI enterographic findings in our patient included:\nA) Clusters of bowel loops within a hernia sac in abnormal duodenal location in left upper quadrant (LUQ) ().\nB) Small bowel folds thickening due to vascular cong-estion and ischemia ().\nC) Additional findings include engorgement, twisting and crowding of mesenteric vessels and bowel volvulus that named ‘whirlpools sign’.\nThe patient underwent midline laparotomy with impression of internal hernia. In the operation left paraduodenal (mesocolic) internal hernia was detected. All parts of small bowel trapped in this Landzert’s fossa were examined and were devoid of gross evidence of bowel ischemia (). Enlargement of the mesocolic space by mobilizing the left colon and opening the neck of the sac was done sufficiently so that the chance of future strangulation be minimized. The web was released totally in all parts. Incidental findings were two cystic lesions in the left ovary that were resected and sent for histopathologic evaluation.\nHistopathologic findings were in favor of congested fibroadipose tissue with mild infiltration of chronic inflammatory cells and no sign of malignancy in web sample and the simple ovarian cyst and the luteal cyst. |
A 7-year-old boy was referred to our pediatric neurology clinic for hyperactivity, speech delay, and behavioral problems that had lasted for 4 years. The patient is the third of healthy unrelated Turkish parents. He was born at term after uneventful gestational delivery. Furthermore, there was no family history of speech delay or mental retardation. He walked at 2 years and spoke his first words at 3 years. Later on, a more general retardation in psychomotor development with restlessness and impulsivity with hyperactivity became evident, for which he was referred to a child psychiatrist. On evaluation, he was considered to have attention deficit hyperactivity disorder. The treatment with antipsychotic drugs (risperidone) was started; however, improvements in neurologic status were not observed after 6 months. He also had a history of recurrent upper respiratory tract infections.\nOn physical examination, he had normal anthropometry and head circumference, mildly coarse facial features, slightly depressed nasal bridge, frontal bossing, and stocky hands with short fingers []. Organomegaly was not present.\nNeurological examination showed normal tone, power, and deep tendon reflexes with bilateral flexor plantar response. He was awake, alert, and anxious. He had a mild language disability.\nLaboratory investigations showed normal values of blood counts, chemistry, electrolytes. Urine organic acids, tandem mass, plasma lactate, pyruvate, thyroid function tests, and karyotype, and fragile X tests were normal.\nIn light of these findings, such as coarse facies and progressive hyperactivity, the diagnostic possibility of MPS was suspected, but subsequent laboratory examination revealed normal urine glycosaminoglycans (GAGs). Skeletal survey was not suggestive of the characteristic dysostosis complex. Magnetic resonance imaging (MRI) of the brain showed J-shaped sella turcica, mild widening of the lateral ventricles with thinning of the corpus callosum and small perivascular spaces in the bilateral parietal regions. In addition, there were cysts (isointense to cerebrospinal fluid and therefore likely representing dilated perivascular spaces) within the body of the corpus callosum [].\nEnzyme analysis for MPS IIIA showed null activity of the enzyme sulfamidase activity in leukocytes (normal range 3.2–20.4 nmol/17 h), confirming the diagnosis. He was started on rehabilitation and behavior therapy for hyperactivity. |
Our patient was a female infant who was born to a 27-year-old Japanese nulliparous woman. The mother had been diagnosed with infantile epilepsy at 1 year of age. Her antiepileptic therapy was discontinued at the age of 7 years because she had no symptoms, but she had an epileptic seizure at the age of 17 years, and treatment with the antiepileptic drugs was resumed. She married at the age of 25 years; however, she did not receive preconceptional consultation. Because she had slight mental retardation, she did not report her wish for a pregnancy to her physician. At the age of 27 years, she was referred to our hospital from a primary obstetrical clinic at 13 weeks of gestation. She was obese, with a body mass index of 30 kg/m2. The antiepileptic drugs she was taking included 1400 mg/day of VPA, 140 mg/day of phenobarbital (PB), and 1200 mg/day of carbamazepine (CBZ). The blood concentrations of the drugs were 80 μg/ml VPA (effective blood range is from 40 to 100 μg/ml), 26.1 μg/ml PB (10–30 μg/ml), and 10.5 μg/ml CBZ (4–12 μg/ml). Despite the mother’s use of three antiepileptic drugs, she had convulsions about three times per week. Therefore, reduction or alteration of the drugs was difficult. She was informed of her epileptic status and the teratogenic effects of VPA, but her wish to have a baby was so strong that she continued her pregnancy with oral folic acid of 5 mg/day.\nAlthough second-trimester transabdominal ultrasonography was performed at 20 weeks of gestation, fetal malformation was not detected because of a poor ultrasound image due to maternal obesity. At 25 weeks of gestation, contracture of the fetal right wrist joint suggestive of a radial ray defect was detected by ultrasonography (Fig. , ). The fetus demonstrated growth retardation starting from 32 weeks of gestation. After that, fetal ultrasonography was performed weekly until delivery. At 34 weeks of gestation, the estimated fetal weight was 1660 g (−2.5 SD: standard deviation), the biparietal diameter was 74.9 mm (−2.8 SD), mother’s abdominal circumference was 261.7 mm (−1.5 SD), and the femur length was 58 mm (−1.3 SD). The right ulna was shorter than the left ulna; the right was 35 mm (−8.4 SD), and the left was 53 mm (−1.1 SD). The right radial ray was not detected. At 37 weeks of gestation, saddle nose as a facial anomaly was observed on a three-dimensional ultrasound (Fig. ). Therefore, right radial ray defect and saddle nose caused by FVS were suspected.\nThe serum levels of antiepileptic drugs were within effective ranges; however, multiple syncopes occurred. Fetal growth retardation was not improved, but growth arrest was not observed. The mother went into active labor at 39 weeks and 2 days of gestation and delivered a female infant weighing 2056 g with Apgar scores of 8 and 8 at 1 and 5 minutes, respectively. The neonate showed slight muscular hypotonia of the whole body and respiratory distress. The neonate’s physical examination revealed bend and contracture of the right wrist joint (Fig. ), right radial ray defect (Fig. ), a ventricular septal defect (VSD), an atrial septal defect (ASD), and a patent ductus arteriosus (PDA) in addition to characteristic facial configurations such as euryopia, broad nasal root, saddle nose, shallow philtrum, and low-set ears. The neonate’s features were compatible with FVS. PDA ligation was performed when she was 13 days old, and cast immobilization of the right forward arm was started when she was 89 days old. A follow-up examination at age 12 months demonstrated developmental and growth retardation. |
A 16-year-old female patient reported to the Department of Conservative Dentistry and Endodontics with a history of an automobile accident and injury to the upper front teeth. The patient had reported to the department within half an hour after injury. Initial examination revealed an oblique fracture on permanent maxillary right central incisor (MRCI) and permanent maxillary left lateral incisor (MLLI) involving three-fourths of the clinical crown with associated exposure of the pulp []. Diagnosis of Ellis's class 3 fractures on both tooth were made. Maxillary left central incisor (MLCI) had a 3-year-old composite restoration. On clinical examination, the fracture line extended from labial to lingual in an apical direction. Radiographic examination revealed an oblique fracture with MRCI and MLLI []. While MRCI exhibited fracture line extending 2 mm above the bony crest, the MLCI exhibited some periapical changes. The treatment options presented to the patient and to her legal guardians were: (1) Crown build-up restoration with a resin based composite, (2) reattachment of the tooth fragment (3) root canal therapy, post and core and lastly, the least desired (4) extraction. After some deliberation about the advantages, disadvantages, prognosis, and cost of each treatment option, the patient opted to have the tooth fragment reattached. It is important to note that the reattachment option was presented only after confirming that the fragment was in good condition and that it fit reasonably well on the fractured tooth.\nThe fragments of MRCI and MLLI were still attached by a soft-tissue junction at the palatal aspect []. After local anesthesia, the coronal fragments were separated with minimum force [Figure and ] and was stored in saline to prevent dehydration. Since the pulpal exposure was fresh, the exposed site was disinfected with chlorhexidine and saline and direct pulp capping was done with mineral trioxide aggregate (MTA) (Dentsply, Tulsa Dental, Tulsa, OK, USA) [Figure and ]. Glass ionomer cement (Fuji IX GC Corporation Tokyo, Japan) was placed as the final restoration. After 2 weeks orthodontic extrusion was planned with MRCI to expose the fractured site supragingivally, using a custom-made appliance []. Vitality test was performed to check for the vitality of the teeth after 6 weeks. Both MRCI and MLLI tested positive for vitality, but MLCI showed delayed response. The procedure of reattachment of fragment was carried out.\nThe pulp chamber of the respective fragments (MRCI and MLLI), which were stored in saline and anti-fungal agent thymol for 8 weeks, were debrided of pulp and a groove was made with a long flat ended tapered fissure bur to act as a retentive area (Mani, Japan) []. Etching (37% phosphoric acid gel, Prime Dent, New Delhi, India) and bonding (Bond I, Pentron Technologies LLC, Wallingford, CT, US) of the MRCI and MLLI and the respective fragments were done. A small pre-measured piece (2 mm) of ribbond (Ribbond Inc., Seattle, WA, USA) was selected, bonding agent applied (Bond I, Pentron Technologies LLC) and cured for 20 s. The ribbond was placed vertically in the groove prepared in the fractured fragments along with panavia F dual cure cement (Kuraray, Osaka, Japan) []. The fragments were then verified for a fit with the teeth surface to ensure proper adaptation. Excess cement was removed and light cured from both buccal and palatal side.\nThe occlusion was carefully adjusted and the patient was instructed to avoid exerting heavy function on the reattached teeth. Patient was recalled after 6 months, vitality test was done with electric and cold test. The MRCI and MLLI responded positively but MLCI showed no response. Root canal therapy was done with MLCI. An 18-month recall showed good esthetics and function of the reattached teeth and vitality of both MRCI and MLLI was confirmed [Figure and ]. |
Patient 10 (Tables and ), a 76-year-old female, was referred to the department of neurology in July 2012 because of progressing cognitive decline over the last 12 months, loss of weight, nausea, gait disturbance and tremor. She was seen on May 2011 for the first time by a neurologist with a 3-month history of dull holocephalic headache who ordered a cranial magnetic resonance imaging (MRI) and diagnosed a tension-type headache and a depressive disorder. Treatment with an antidepressant (duloxetine) was started. The patient experienced no improvement and a second examination by another neurologist was undertaken 2 months later. Again no focal neurological signs could be detected. Due to the weight loss, an occult neoplasm was suspected but not detected during an extensive inpatient investigation at a medical department during February 2012; however, the MRI showed bilateral white matter lesions (WML) and an old lacunar lesion located at the left striatum, the latter was not seen in the previous MRI from May 2011. Since the patient also suffered from mild hypertension, vascular encephalopathy was thought to be the cause of the progressive cognitive decline. Extensive neurocognitive testing was carried out in a rehabilitation centre in May 2012 and disclosed a severe decline of attention, memory and executive functions corresponding to subcortical dementia (Fig. ). When the patient was seen for a further diagnostic work-up at the SMZ-Ost-Donauspital in July 2012, the weight was 47 kg and a weight loss of 20 kg was reported over the past year. The gait was insecure with postural instability and with a tendency to fall when turning around. Frontal signs were positive, the voice was quiet, the tonus was mildly elevated and showed a slight hesitancy (“Gegenhalten”), tendon reflexes were brisk, paresis and pyramidal signs missing. There were no signs of ataxia, but a mild bradykinesia. Action tremor was more distinct than a mild resting tremor. Again, neurocognitive testing and gait disturbances were consistent with subcortical dementia (Figs. and ). Regarding the mild signs of parkinsonism, dementia with Lewy bodies (DLB) was also suspected but excluded by a dopamine transporter (DAT) scan. Fluorodeoxyglucose positron emission tomography (FDG-PET) demonstrated hypometabolism in the left striatum and in the left frontotemporal cortex (Fig. ). Cerebrospinal fluid (CSF) showed signs of a chronic lymphocytic inflammation. The CSF markers for dementia, total tau protein and phosphor-tau were within the normal range, while beta-amyloid 1-42 and the Innotest-amyloid-tau index (IATI) were found to be below the reference values (beta-amyloid 1-42: 290 pg/ml, reference value > 500 pg/ml; IATI 0.6, reference values > 1). Finally, LNB was diagnosed when further CSF examinations disclosed a highly elevated Bb-specific-AI indicating local intrathecal Bb-specific antibody synthesis (Table ). The patient was treated with 2 g ceftriaxone daily for 3 weeks.\nNeurological symptoms and impaired cognitive functions, although persistent for a year, recovered rapidly within a few weeks (Figs. , and ) and so did the pathological CSF findings (Table ). A follow-up FDG-PET examination showed the left frontotemporal hypometabolism in remission, while this was not the case for the cystic lacunar lesion in the left striatum. A new and clinically silent small right thalamic lesion was detected that was not present in the pretreatment MRI (Fig. ). The Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) test battery at the last follow-up in April 2014 scored within the age-dependent normal range with the exception of verbal learning and semantic verbal fluency (Fig. ). In a telephone call in February 2018 at the age of 82 years, the patient reported no gait problems or cognitive impairment and had just returned from a trip to Cuba. |
History and physical
A 67-year-old male former smoker with a history of prior occupational asbestos exposure and recurrent bronchitis presented with progressive dyspnea and thoracic pain to the point that he could not lie down in bed. A computed tomography (CT) scan of the chest was performed, which was interpreted as right-sided pneumonia with right parapneumonic effusion. He was sent to his local emergency department, where he was admitted for antibiotics and thoracentesis, the latter which demonstrated the presence of atypical mesothelial cells with inflammatory cells. He was readmitted two weeks later for progressive thoracic pain, was found to have a recurrent right-sided pleural effusion, and was managed with partial right pleurectomy with pleural biopsy, and talc pleurodesis. Right pleural pathology demonstrated atypical mesothelial proliferation at the pleural surface, without true invasion or definitive pathologic evidence of malignancy. Following surgery, he felt substantially better, such that he could sleep in the bed again, and he was able to return to his baseline activity levels. He underwent repeat chest CT five months later, which showed right pleural thickening and a small loculated pleural effusion, favored to represent a combination of calcification, pleurodesis, and atelectasis. He remained clinically well for another five months until he presented with cough and sinus congestion unrelieved by guaifenesin, dextromethorphan, and antibiotics. He underwent repeat chest CT that showed extensive mass-like pleural thickening completely encasing the right lung, with prominent involvement of the mediastinal pleura, and probable mediastinal extension into the right paratracheal and precarinal space, with pericardial effusion and probable pericardial metastases. There was no definite invasion into the right chest wall and no evidence of disease outside of the thorax.
He then established care at our institution’s mesothelioma and pleural disease multi-disciplinary program. Pathology review of the previously biopsied pleural tumor revealed that the pleural tumor cells were positive for Wilms' tumor-1 and calretinin, and negative |
A 71-year-old male patient was referred to the Department of Otolaryngology-Head and Neck Surgery, Kobe University Hospital, for the evaluation of a 6-month-old, large neck mass that had gradually increased in size. Written informed consent was obtained for surgery including neck dissection. Upon examination, the mass presented as a swelling of approximately 4×5 cm in size, located in the left posterolateral side of the lower neck. It was non-tender, hard, and attached to deep neck structures. A contrast enhanced computed tomography scan revealed a large soft tissue mass in the left posterior neck that involved both the anterior and medius scalene muscles (, ). Magnetic resonance imaging (MRI) revealed an iso-intense mass on the T1 weighted image and a low-intensity mass on the T2 weighted image (). On the anterior side, the mass extended up to the sternocleidomastoid muscle (SCM), with splaying of the left internal jugular vein. No pathological finding was detected in the upper aerodigestive tract by endoscopic examination. After an inconclusive fine needle aspiration, surgical extirpation of the tumor was performed. We did not perform preoperative incisional biopsy because incisional biopsy of neck masses before radical treatment is associated with poor prognosis. The negative influence of preoperative incisional biopsy on prognosis is possibly because of tumor cell dissemination or changes in lymph flow. We were ready to perform a neck dissection procedure if the tumor was malignant. A preoperative incisional biopsy was performed at the beginning of the surgery. The pathological diagnosis of the biopsy was fibromatosis. In response to the clinical findings, a wide excision of the tumor was performed. We encountered difficulty because of local infiltration of the surrounding tissues. Parts of the scalenus medius and SCM were also resected because of tumor invasion. The carotid artery, internal jugular vein, the remainder of the SCM, lower cranial nerves, phrenic nerve, and brachial plexus were successfully preserved. Frozen sections for surgical margins were obtained perioperatively, and the result was negative. On the basis of the intraoperative pathological diagnosis, no additional neck dissection was performed.\nHistopathologically, the lesion showed an increase in well-differentiated fibroblasts within a collagenous stroma with infiltration of nearby skeletal muscle and adipose tissues. The excision margins were negative, except for the area adjacent to the scalenus medius muscle. The cells did not show nuclear atypia or hyperchromasia (). Immunohistochemically, the tumor was negative for S-100, beta-catenin, and CD34 but focally positive for alpha-smooth muscle actin (). Based on the clinical behavior and histomorphological features, a definitive diagnosis of AF was made.\nThe postoperative period was uneventful, and the patient recovered completely. Unfortunately, at his 3-month postoperative MRI follow-up exam, a small residual tumor was detected in the scalenus medius muscle (). The recurrent lesion was treated by radiotherapy with a total dose of 70 Gy/35 Fr, resulting in significant regression. The patient has been on close, regular follow-up for the last 7 years at our clinic. Radiographic studies have shown no evidence of tumor recurrence (). |
The blind and painful right eye of a 38-year-old man was eviscerated in September 2016. The patient stated that his right eye had been blind since early childhood due to a unilateral congenital anomaly complicated by secondary glaucoma. He received the diagnosis of SO in January 2017 after he experienced visual loss in his only seeing (left) eye. At the time of diagnosis, the patient was admitted to the hospital and meticulously investigated for possible infectious and noninfectious causes to rule out other uveitic entities, but without any positive findings. At that time, his best-corrected visual acuity was 6/10. Slit-lamp examination yielded some vitreous cells in the left eye. Fundoscopy showed a few scattered pigmented chorioretinal scars and discrete yellowish round choroidal lesions throughout the left fundus (). Fluorescein angiogram delineated the active lesions as early hypofluorescent () with late staining. Left macular contour was normal on optical coherence tomography (OCT) examination (). He was started on oral prednisolone (64 mg) for 2 weeks with gradual tapering of 8 mg per week. Despite initial visual improvement, he experienced another episode of visual decline while taking 32 mg of prednisolone. His best-corrected visual acuity decreased to 2/10 and he had grade 4 vitreous haze according to the Miami grading. Fundus examination showed marked yellowish-white discoloration of the macula with some evidence of intraretinal hemorrhage ( and ). He was hospitalized and treated with pulse methylprednisolone 1 g (250 mg 4 times daily) for 3 days. Following pulse therapy, 64 mg oral prednisolone and 150 mg (50 mg 3 times daily) azathioprine were co-administered. Two weeks after the completion of pulse therapy, his visual acuity was still 2/10 despite a significant reduction in vitreous haze. Fluorescein angiogram and OCT demonstrated type 2 choroidal neovascularization (). Five intravitreal 2 mg aflibercept injections were given within a period of 8 months. His final visual acuity was 6/10 with a stable-looking macula ( and ) and he was continued on a treatment regimen of 150 mg azathioprine and 8 mg prednisolone daily. |
A 6-day-old (46 kg) male Belgian Blue-Holstein crossbred calf was submitted for clinical investigation because of ulcerations of the skin and nasal planum since short time after birth. The animal was delivered after a gestation period of 287 days.\nThe cutaneous lesions were characterized by widespread irregular erosions and ulcerations of various sizes on most parts of the body (Figure ), but in particular on the limbs (Figure ) and over bony prominences. Upon handling, the epidermis easily separated leaving a blister with a black colored, nonhemorrhagic base indicating a separation superficial to the stratum basale. Peracute blister were easily induced by rubbing the skin by an eraser after having cut the hair locally. Older lesions consisted of ulcerations covered by crusts and occasional acute hemorrhage. On the nasal platum, lips and nares extensive ulcerations were present; the calf also showed a purulent nasal discharge (Figure ). Moreover, the animal seemed to be in pain when walking on a hard surface. The aspect resembled EB and therefore was further referred to the Danish surveillance program for genetic diseases in cattle for further examination. Because of the poor prognosis and the painful situation, the calf was euthanized for welfare reasons by IV administration of an overdose of pentobarbital. In addition to the skin lesions, gross pathologic examination revealed erosions in the oral cavity, pharynx, proximal esophagus, and rumen. The epithelium on the dorsal surface of the tongue was thickened and with furrows (Figure ). The incisor teeth were disorganized and not completely erupted and the surrounding parts of the mandibles appeared thickened and cystic. The hoofs seemed intact, yet when sawed longitudinally, the capsule was partly separated from the dermal lamella with hemorrhage in the interface.\nImmediately before euthanasia, the skin covering the dorsal part of the pelvis was gently scrubbed with an eraser with blister formation. Skin biopsies from this area and from other representative cutaneous lesions were taken immediately after euthanasia for histological analysis, whereas the necropsy was completed at the university a few hours later. Additional specimens for histological analysis were then collected, including the oral mucosa, pharynx, rumen, reticulum, and major internal organs. All collected samples were fixed in 10% neutral buffered formalin, trimmed, processed, embedded in paraffin wax, sectioned at 4 to 5 μm, and stained by hematoxylin and eosin (HE). Histologically, the peracute lesions induced by rubbing before euthanasia displayed a very striking, multifocal to coalescing dermal-epidermal separation at the level of the basal layer, which extended into the wall of the hair follicle infundibula (Figure ). The spontaneously occurring, chronic lesions present in the nasal planum and in the distal limbs displayed a multifocal to coalescing epithelial loss with consequent severe ulceration and underlying neutrophilic infiltration, replacement of the papillary dermis by granulation tissue, and re-epithelialization. A multifocal dermal-epidermal separation at the level of the basal cell layer with multifocal underlying accumulation of free erythrocytes and fibrin exudation was occasionally visible at the border of the ulcerated areas.\nIn the tongue, the mucosa of the dorsal surface showed a marked parakeratotic hyperkeratosis. At the lateral borders, where the epithelium had a normal thickness, areas with complete loss of mucosa were observed. The superficial layer of the submucosal connective tissue had a necrotic surface, intense hyperemia, and infiltration with neutrophils. Mucosa cleavage in the adjacent areas was not observed, but the height of the epithelium gradually decreased. The stratum spinosum showed ballooning degeneration and in these areas, the stratum corneum was not present. In the pharyngeal lining, multiple intensely inflamed ulcers covered by a debris of fibrin, degenerated neutrophils, erythrocytes, bacterial colonies were present.\nThe epithelium of the rumen and reticulum was normally developed but an acute suppurative multifocal rumenitis was present. Separation of the epithelium from the underlying submucosa was seen in some areas but considered as a post mortem artifact. Additional findings included suppurative periportal hepatitis and multifocal intense pulmonary hyperemia associated with fibrin in the alveoli. Other tissues were unremarkable. The histopathological findings in the skin and pharyngeal linings resembled EBS.\nAdditionally, WGS using the Illumina NovaSeq6000 was performed on DNA extracted from ethylenediaminetetraacetic acid (EDTA) blood of the calf. The sequenced reads were mapped to the ARS-UCD1.2 reference genome resulting in an average read depth of approximately 19×, and single-nucleotide variants and small indel variants were called. The applied software and steps to process fastq files into binary alignment map and genomic variant call format files were in accordance with the 1000 Bull Genomes Project processing guidelines of run 7 (), except for the trimming, which was performed using fastp. Further preparation of the genomic data had been done according to Häfliger et al. In order to find private variants, we compared the genotypes of the affected calf with 493 cattle genomes of various breeds that had been sequenced in the course of other ongoing studies and that are publicly available (Table ) in the European Nucleotide Archive (SAMEA6528898 is the sample accession number of the case; ). Integrative Genomics Viewer (IGV) software was used for visual inspection of candidate variants. A total of 115 private protein-changing variants with a moderate or high predicted impact on the encoded protein, located within 108 different genes or loci, were identified. These variants were further checked for their occurrence in a global control cohort of 3103 genomes of a variety of breeds (1000 Bull Genomes Project run 7; ), which revealed 26 protein-changing variants exclusively present heterozygous in the genome of the EBS-affected calf. These 26 variants located within 25 different genes or loci (Table ) were subsequently visually inspected using IGV software confirming all as true variants. Of all these 26 remaining private variants, only 1 occurred in a candidate for EB: keratin 5 (KRT5). The variant was a heterozygous disruptive in-frame deletion variant on chromosome 5: 27367604delCAA (NM_001008663.1:c.534_536delCAA), leading to a loss of an asparagine amino acid at residue 178 of the encoded KRT5 protein (NP_001008663.1:p.Asn178del). This variant affecting an EB candidate gene was further investigated as likely causal mutation for the observed phenotype.\nTo confirm that the c.534_536delCAA variant in KRT5 was a de novo mutation, the affected genomic region was amplified by polymerase chain reaction (PCR) and Sanger sequenced in the affected calf, its Belgian Blue sire and Holstein dam based on DNA extracted from EDTA blood of the dam, and from both EDTA blood and semen of the sire. PCR products were amplified using flanking primers for the KRT5 exon 1 deletion with 5′-AGGCATCCAAGAGGTCACCG-3′ (forward primer) and 5′-TAGCACATATCCCACACTCATGG-3′ (reverse primer). Sequence data were analyzed using Sequencher 5.1 (GeneCodes). Analyzing the sequencing data, we concluded that only the EBS-affected calf was heterozygous for the KRT5 variant and the dam and sire were both homozygous for the wild type allele in all analyzed samples including both semen and blood of the sire (Figure ). |
A 26-year-old man of Bangladeshi origin presented with a two-day history of generalised abdominal pain, loss of appetite, and nausea. His past medical history includes hypertension, type 1 diabetes mellitus, and autosomal dominant polycystic kidney disease. He is the product of a consanguineous marriage and suffered from intrauterine growth retardation and failure to thrive with persistent short stature and microcephaly. Previous investigations for chromosomal disorders at Great Ormond Street Hospital were negative, and the karyotype was confirmed as 46XY.\nOn examination, he was afebrile and physiologically stable with tenderness in the lower abdomen and a BMI of 14 (height 135.5 cm and weight 25.56 kg). Inflammatory markers were mildly raised (white cell count 13.2, neutrophils 8.4, and C-reactive protein 87). He was admitted for observation, intravenous fluids and antibiotics. A computed tomography scan of the abdomen and pelvis revealed mild thickening of the descending colon but no focal inflammatory changes, free air, or free fluid.\nThe patient deteriorated clinically and underwent a diagnostic laparoscopy, which identified a thickened inflamed loop bowel adherent to the anterior abdominal wall. A laparotomy was performed. There was extensive diverticular disease and an associated inflammation of the entire colon with an inflammatory mass around the transverse colon adherent to the anterior abdominal wall. The diverticula were large and broad based, resembling jejunal diverticulosis macroscopically. Malignancy could not be excluded, and the tissues were extremely friable, so a subtotal colectomy and end ileostomy with abdominal washout were performed. Our patient's postoperative course was complicated by an episode of aspiration pneumonia from which he made a full recovery.\nHistopathological examination supported the diagnosis of true pancolonic diverticulosis. The entire specimen showed numerous large diverticula and associated inflammation/abscess formation. The majority of the diverticula had a colonic type muscularis propria and can therefore be considered as true diverticula resulting from congenital malformation (). Lymph nodes showed reactive changes only. Subsequent elastin immunohistochemical staining was within normal limits.\nHe was discharged 13 days postoperatively, with a view to restoration of intestinal continuity in 6 months. |
The patient was a 79-year-old Caucasian male with locally advanced adenocarcinoma of the pancreas encasing the superior mesenteric artery and celiac ganglion. His past medical history includes a hiatal hernia, varicosities in the legs, and mild hypertension. Current medications include pancrelipase, omeprazole, and lisinopril. He smoked about one pack of cigarettes per day for 40 years but stopped at the age of 55 years and denied alcohol abuse. At the initial oncologic evaluation, his physical examination did not reveal any abnormality except varicose veins on the left leg more than right. Neurological examination was also normal. He was able to ambulate without any assistance and was able to do all activities of daily living (ADLs).\nThe patient started chemoradiation therapy consisting of capecitabine (1600 mg/m2 in two divided doses = 3200 mg/m2 per day Monday to Friday with weekends off), with concurrent radiation (50.4 Gy in 28 fractions over 5.5 weeks). He tolerated the combination therapy extremely well for the first three weeks without any major toxicity except grade one nausea, grade one vomiting, grade one diarrhea and grade one hand-foot syndrome. On day three of the 4th week, he presented to the oncology clinic with new onset of gait abnormality noticed by his son that morning. The son noticed that the patient was dragging the front of his right foot on the ground when he walked. The patient further confirmed that he developed a new difficulty lifting the front part of his foot that morning when he wanted to walk to the restroom. No gross abnormal findings were elucidated on neurological examination, including cranial nerves, except unsteady gait with right-sided foot-dragging, impaired heel-toe walking, diminished sensation to pinprick in the lower extremities, and decreased distal motor strength. A magnetic resonance imaging scan of the brain and thoracolumbar spines was unremarkable. Laboratory studies including complete blood count, chemistry, liver function tests, vitamin B12, thyroid stimulating hormone (TSH) test, rheumatoid factor, thiamine levels, antibodies against double-stranded DNA phospholipid and cardiolipin were collected and all came within normal limits. The patient was not receiving any other potentially neurotoxic medications. Nerve conduction studies and electromyogram revealed acute, axonal sensorimotor polyneuropathy with secondary demyelinative features, with an increasing proximal to distal neuropathic gradient (Figure ).\nWe immediately halted administration of capecitabine, consulted neurology as well as physical therapy. Neurology agreed with our assessment and suggested not to resume the drug as no other etiology was found. They suggested to rule out cancer progression as sometimes these neurological abnormalities might be manifesting paraneoplastic syndrome and the repeat CT imaging of scan of the abdomen, pelvis, and chest showed a slight improvement in tumor size in the pancreas and no distant metastasis.\nIn addition to above tests, we also performed genetic studies related to capecitabine including dihydropyrimidine dehydrogenase (DPD) deficiency and thymidylate synthase (TYMS) polymorphism. DPD gene mutation analysis (Quest Diagnostics Nichols Institute-San Juan Capistrano, CA) was negative for the IVS14+1G>A mutation in the DPD gene, which accounts for 50% of the DPD deficiency alleles. However, the patient was found to have two mutations (2R) of a 28 base-pair tandem repeat in the 5’ promoter enhancer region (5’-TSER) on both alleles (2R/2R) of TYMS gene. This 2R/2R genotype predicts low TYMS expression.\nThe patient’s symptoms slightly improved within the next four weeks after holding capecitabine and initiation of physical therapy. However, after one week of delay, he finished the remaining fractions of radiation therapy, impressed by the CT scan findings showing shrinkage of the tumor. After a short break of two weeks, he came back to the oncology clinic for further discussion on treatment. After extensive discussion, the decision was made to proceed with a different chemotherapy and hence single agent gemcitabine 1000 mg/m2 weekly x three out of four weeks was administered starting after another two weeks delay. At that time, he had gradual improvement in his gait (using a cane), balance and coordination. He received a total of six cycles (four weeks = one cycle) of gemcitabine with no new neurological deficit. Physical examination of seven months after discontinuing revealed residual grade two sensory loss in the feet and grade one motor loss with dorsiflexion. Repeat electromyogram (EMG) test at that time (Figure ) showed chronic peripheral nerve degeneration, despite the improvement of symptoms. |
We describe a case of a 36-year-old black African woman with two previous live births by cesarean section and two previous miscarriages who was referred in her fifth pregnancy after 6 weeks of amenorrhea. Her serum quantitative β-human chorionic gonadotropin (bHCG) was 16,124 mIU/ml. However, an intrauterine or extrauterine pregnancy could not be located on a transabdominal ultrasound scan.\nA copper intrauterine contraceptive device had been removed 2 months prior to her last menstrual period. She reported using one cycle of clomiphene 50 mg with the hope of achieving a twin pregnancy.\nShe had delivered twice by cesarean section for failure to progress. Her last two pregnancies had been first-trimester miscarriages; one was managed expectantly, and the other was surgically evacuated, though the actual procedure was unknown to the patient. She did not have any chronic medical illness and was not receiving any medication prior to this presentation. She stayed in a city suburb that was well serviced. She was a school principal in her second marriage with no children in the current relationship. She did not smoke or drink alcohol.\nOn examination, she had a normal blood pressure of 113/70 mmHg and a pulse rate of 98 beats/min. Her body temperature was 37.5 °C. On examination, her cardiorespiratory and neurological systems were normal. Her abdomen was soft and not tender. The result of her pelvic examination was normal. TVUS showed a gestational sac of 13 mm with irregular margins and a visible yolk sac located on the anterior isthmic portion of the uterus, raising suspicion of a cesarean section scar ectopic pregnancy. She declined a Doppler ultrasound evaluation scheduled for the next day. She was scheduled to have serial bHCG evaluations every 48 h.\nA repeat serum quantitative bHCG done 48 h after the initial test revealed a level of 21,521 mIU/ml, a 33% rise. She defaulted follow-up until 1 week later, when she presented with pelvic pain of increasing intensity for 5 days. An urgent transvaginal scan was performed. A fetal pole with active cardiac activity (crown-rump length 0.9 cm) in a gestational sac was located in the anterior low myometrium. The sac traversed the full width of the anterior myometrium, with the posterior margin of the sac abutting the anterior margin of endometrium and the anterior margin of the sac extending to a subserosal location in a fairly exophytic fashion. There was evidence of trophoblastic circulation on Doppler examination. There was no endometrial fluid or free pelvic fluid (Fig. ).\nShe was immediately admitted for a diagnostic laparoscopy/hysteroscopy and possible excision of the scar pregnancy if confirmed. A preoperative complete blood count showed hemoglobin 13.4 g/dl, white blood cells 7.2 × 103/μl, and platelet count 243 × 103/μl. The patient’s kidney function was normal with sodium 135 mmol/L, potassium 4.9 mmol/L, urea 3.5 mmol/L, and creatinine 65 μmol/L. The patient’s liver function test results were also normal. She had a negative result in a blood test for human immunodeficiency virus. Urinalysis did not show abnormalities. The patient’s random blood sugar was 5.6 mmol at admission. At laparoscopy, the bladder was adherent high on the anterior uterine wall, and the ectopic pregnancy was not visualized (Fig. ). At hysteroscopy, there were extensive adhesions within the lower endometrial cavity, which obscured visibility. There was no active intracavitary bleeding ruling out a threatened or inevitable miscarriage. We could not visualize any obvious bulge in the cervical canal suggestive of a cervical ectopic pregnancy. Because of the uncertainty of the location of the pregnancy due to adhesions, excision was postponed. Postoperatively, the patient became unstable with low blood pressure, systolic pressure range of 82 to 95 mmHg and diastolic pressure range of 40 to 55 mmHg, and a pulse rate range of 64 to 73 beats/min, but without active vaginal bleeding or use of medications inducing persistent hypotension. Anesthesia had been induced with etomidate 16 mg and suxamethonium 100 mg, and maintenance was initiated with isoflurane 0.8–1.5%. Intra- and perioperative analgesia was induced with fentanyl 200 mg intravenously (IV), indomethacin 100 mg rectally, and paracetamol 1 g IV. Antibiotic prophylaxis was with ceftriaxone 1 g IV and metronidazole 500 mg IV. This prompted us to order an urgent MRI scan to map the location of the pregnancy in the immediate postoperative period. MRI confirmed the TVUS findings of a cesarean section ectopic scar extending to the serosa (Fig. ).\nAn emergency laparotomy was then performed on the same day. The abdomen was entered through a Pfannenstiel incision along the old skin scar. A transverse incision was made in the upper uterine segment just above the adherent bladder. The products of conception were removed with forceps, and the gap in the anterior myometrium at the old scar was seen and felt. There was massive bleeding from the implantation site. Twenty milliliters of vasopressin (20 U diluted in 100 ml) in normal saline was administered into the bleeding myometrium edges. The edges were apposed in layers with VICRYL suture (Ethicon, Somerville, NJ, USA) to repair the defect. Estimated blood loss was 2000 ml. The patient was transfused with 1 U of packed cells intraoperatively. She was continued on the same intravenous antibiotics and analgesia that had been commenced after the laparoscopy. Her hemoglobin count on day 1 postoperatively was 8 g/dl, and she declined any further transfusion. Oral iron and folic acid supplementation was commenced.\nThe patient’s postoperative recovery was uneventful, and she was discharged on day 4 after surgery. Histology confirmed the presence of decidua and chorionic villi. The patient wanted a child because she was in a new relationship, but she was no longer sure of her future fertility plans after the ectopic pregnancy. A levonorgestrel implant was inserted 2 weeks postoperatively. The patient last attended physical review at 6 weeks, and she was well with no problems related to the surgery at a telephone review at 3 months postoperatively. |
62-year-old woman with medical history significant for aortic stenosis and chronic atrial fibrillation presented to the emergency department with fatigue and progressively worsening shortness of breath with minimal exertion. She was asymptomatic at rest and denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, presyncope, or syncope. She was incidentally found to have a systolic murmur during her pregnancy 20 years prior to this presentation. Notably, she had been offered aortic valve replacement in the past but declined.\nVital signs were normal with blood pressure of 110/60 mmHg, pulse rate of 79/min, temperature of 97.2°F, and respiratory rate of 16 breaths/min with normal oxygen saturation of 100% on ambient air. Physical examination was significant for irregularly irregular heart rhythm, and ejection systolic murmur was loudest in the aortic area with radiation to the carotids. Her lungs were clear to auscultation, and no pedal edema was noted. Electrocardiogram revealed atrial fibrillation with voltage criteria for left ventricular hypertrophy. Her most recent transthoracic echocardiogram revealed a thickened calcified aortic valve with decreased excursion with 4.6 m/s velocity suggesting a peak of 86 mmHg and mean of 36 mmHg suggestive of severe aortic stenosis. No other significant valvular abnormalities noted. Cardiac catheterization revealed widely patent coronary arteries.\nBased on the presence of worsening symptoms and the risk of sudden cardiac death, the decision was made to proceed with surgical aortic valve replacement. Given her history of chronic atrial fibrillation, she was also planned for left atrial appendage exclusion and Cox Maze IV procedure simultaneously with the aortic valve replacement. Intraoperative transesophageal echocardiogram revealed quadricuspid aortic valves confirmed during surgical exploration (). The native stenotic quadricuspid aortic valve leaflets were excised, and a 21 mm Saint Jude Medical Trifecta valve was implanted. The left atrial appendage was excised, and Cox Maze IV procedure was performed. Postoperatively, she remained in junctional rhythm and underwent uneventful placement of the dual chamber pacemaker on postoperative day 3. She recovered without further complications and was discharged on the eight postoperative day. |
A 39-year-old man was involved in a high-velocity motor vehicle accident. He sustained an open fracture of the right elbow, with significant loss of the external humeral condyle and partial loss of the olecranon. This fracture was classified as a Gustillo type IIIA injury. There was no neurovascular compromise. The patient was treated in a community center, close to the accident, where he received surgical care (debridement and partial excision of the olecranon). The wound was fully closed. The upper arm was then immobilized in a splint (). IV antibiotics (cefazolin-gentamicin) were started for 5 days.\nThe day following his elbow surgery, the patient fell in a staircase and sustained a C7-C8 and C8-T1 fracture-dislocation. This injury caused neurologic damage (quadriparesis), and his right arm became his only functional limb. Following this injury, the patient was moved to our tertiary center to get spinal fusion. During the spinal surgery, the elbow was tested under fluoroscopy. The patient's elbow showed varus instability and a positive pivot shift test. A CT scan of the elbow was obtained the following day and showed bony loss from the external humeral condyle and subluxation of the radial head ().\nWe decided to treat the patient's elbow surgically. The surgery underwent nine days after the initial trauma (after transfer from the community center, spine procedure and elbow imaging). A posterior approach to the elbow was used along with an extensive elbow debridement. A tricortical iliac crest graft was then collected from the patient's right side to replace the humeral condyle bone loss. A tendinous graft was collected from his third and fourth extensor digitorum longus tendons to reconstruct the lateral collateral ligament. The tendinous graft was fixed to the bone graft through two tunnels (anterior to posterior and lateral to medial). The iliac crest graft was then fixed to the humerus with a cancellous screw. A five-hole one-third tubular plate was used as buttress. The graft was left prominent on the lateral side to improve lateral stability of the elbow. The lateral collateral ligament was then reconstructed by fixing the tendinous graft to the proximal ulna with two bundles. We took care to preserve our reconstructed lateral collateral ligament's isometry during flexion and extension movements. Reduction and stability of the elbow joint were verified under fluoroscopy. The wound was closed with staples, and a posterior splint was applied for three weeks. 14 days postoperative X-rays are presented on . At three weeks following the surgery, passive and active ranges of motion were initiated.\nWhile wearing the splint, the patient developed a pressure wound on the lateral side of his elbow (at the site of the reconstruction). This wound later complicated into a Nocardia septic arthritis, which required surgical debridement. Four months after the reconstruction, range of motion was 30-120 degrees with full pronation and supination, without any evidence of instability.\nTwo months later, instrumentation was removed and a rotational flap was done to increase soft tissue coverage of the articulation. Five months after that procedure, the patient developed a Nocardia osteomyelitis of the humerus and was treated with 6 weeks of IV antibiotics (penicillin) and a surgical debridement. The patient responded well to our treatment. Eleven months after the initial intervention, range of motion of the joint ranged from 30 to 120 degrees of flexion, without clinical instability. Osteosynthesis was achieved according to X-rays of the elbow (). |
An 89-year-old male with a history of stroke, atrial fibrillation, heart failure, esophageal adenocarcinoma status post radiation, cholecystitis one year prior to presentation (treated with percutaneous cholecystostomy tube that had subsequently been removed), presented to the emergency room with left lower quadrant abdominal pain of two-day duration. He denied nausea, vomiting, diarrhea, constipation, or blood in the stool. He did not have any sick contacts or recent travel. In the emergency room, his temperature was 98.1°F, pulse 80 beats per minute, respiratory rate 16 breaths per minute, and blood pressure 106/67 mmHg. The laboratory blood tests are described in Table .\nA CT of the abdomen and pelvis revealed a fistula between the gallbladder and the large bowel at the hepatic flexure and air in the gallbladder. Marked diverticulosis with wall thickening and fat stranding was seen at the level of the mid to distal descending colon. A large lamellated stone (3 cm x 2.6 cm) was present in the distal descending colon (Figure ). A gastrografin enema revealed sigmoid colon diverticular stricturing disease (Figure ).\nGiven the patient’s several comorbidities surgery was deferred, the patient was kept nil per mouth and conservative treatment with an aggressive bowel regimen including mineral oil enemas as well as IV hydration was started. However, after four days he did not have any bowel movement and for this reason, total parenteral nutrition was initiated and an endoscopic approach to remove the stone was planned. On the fifth day of hospitalization, a colonoscopy was performed which showed a large black pigmented stone that was completely obstructing the lumen of the sigmoid colon, the surrounding mucosa appeared ulcerated (Figure ).\nAttempts to pass a guidewire proximal to the stone to pursue balloon-assisted dislodgment of the stone or mechanical lithotripsy as well as attempts to capture the stone in a retrieval net were unsuccessful. Consequently, an exploratory laparotomy with partial left colectomy was successfully done, however, the cholecystocolonic fistula was left intact. Later, the pathology results revealed a 3.4 cm x 2.7 cm black stone and a 4.5 cm x 3 cm transmural defect at the colonic wall where the stone was impacted. The patient’s postoperative period was complicated, he did not tolerate oral intake and his nutrition deteriorated substantially despite total parenteral nutrition (TPN). At the request of the patient and the family he opted for inpatient hospice care. Unfortunately, he died 15 days after the surgery. |
A 24-day-old female neonate was referred to our hospital with swelling in the left leg. She was born by spontaneous vaginal delivery at 40 weeks of gestation with a birth weight of 3392 g. The maternal vaginal culture was positive for group B Streptococcus (GBS; Streptococcus agalactiae) in the third trimester, and her mother had received a prophylactic antibiotic at delivery. There were no perinatal complications, including birth trauma or antecedent infections. She did not receive any invasive medical interventions, such as catheterization or parenteral nutrition. Her umbilical cord fell off on day 15 after birth. She had no significant family history of recurrent or intractable infections. On day 21 after birth, her parents noticed that she cried during diaper change. On day 23 after birth, her left leg was swollen and she was referred to our hospital. On admission, she had mild fever with left leg swelling and erythema. She had no history of trauma. Laboratory data showed an elevation of inflammatory markers, including blood leukocyte count 22 140/µL, absolute neutrophil count 15 276/µL, C-reactive protein 4.5 mg/dL, and erythrocyte sedimentation rate 31 mm/h. A lower leg plain radiograph revealed osteolytic cortex and hyperlucency at the left proximal tibia. Osteomyelitis in the left proximal tibia was diagnosed, and intravenous cefotaxime was initiated. On hospital day 4, magnetic resonance imaging (MRI) indicated an intramedullary ring-enhanced lesion in her left tibia. On day 5, surgical debridement of necrotic bone and pus drainage were performed. The pus culture was positive for methicillin-susceptible Staphylococcus aureus, whereas three sets of blood cultures on admission were all negative. Cefotaxime was switched to cefazolin based on the culture result. Her clinical symptoms and inflammation markers improved gradually. On day 52, follow-up MRI showed no enhanced intramedullary lesions, and she was discharged on day 57 of admission without complications. Antibiotic treatment was completed with 6 weeks of cefazolin, followed by 5 months of oral cephalexin. No adverse event, including neutropenia, renal, or liver dysfunction, was observed during the treatment. The time from onset to detection of osteomyelitis was short in this case; however, we clinically diagnosed chronic osteomyelitis because sequestrum was observed intraoperatively and the pathological findings of the scraped tissues showed necrotic bone trabeculae. We decided to administer a 6-month treatment for chronic osteomyelitis. Permanent disabilities, such as growth arrest or limb length discrepancy, have not been observed during 1.5 years of observation.\nImmunological studies exhibited no abnormal findings in lymphocyte subsets, neutrophil reactive oxygen species, immunoglobulin levels, or complement components. Normal thymus and spleen were confirmed by chest radiograph and abdominal ultrasonogram. We retrospectively reviewed neonatal patients with osteomyelitis at our institution between 2002 and 2019 and found two additional cases. All three patients received immunological assessments, but no apparent immunological abnormality was detected (Table ). |
A 70 year old lady was referred urgently to the colorectal clinic with a palpable abdominal mass. She presented to her General Practitioner (GP) with left sided abdominal pain, diarrhoea and weight loss. Her GP found a suspicious left sided abdominal mass on examination and referred her urgently under the two week colorectal cancer rule. Her past medical history included hypertension, chronic obstructive airways disease and appendicectomy. She also had a previous total abdominal hysterectomy and bilateral salpingo-oopherectomy for large uterine fibroids.\nOn examination in the outpatient clinic a 10 × 7 cm firm, fixed lump was found in the left iliac fossa area of the abdomen. Urgent colonoscopic examination revealed mild sigmoid diverticular disease with no evidence of colonic malignancy. Computer Tomography was preformed (Figure ) and showed the mass to be arising from the anterior abdominal wall muscles, in particular the internal oblique and transversus abdominis. There was no evidence of distant metastatic disease to the liver or lungs. The clinical suspicion was of a malignant abdominal wall sarcoma. Fine needle aspiration or percutaneous biopsy was not performed. En-bloc surgical resection of the tumour was performed via a left flank incision (Figure ). At surgical resection the tumour mass involved the internal oblique, transversus abdominis and there was a small area of peritoneal ulceration. No distant disease was found at surgery. The tumour was excised en-bloc with a surrounding margin of healthy tissue (Figure ). Part of the external oblique aponeurosis was preserved to allow adequate closure. The large abdominal wall defect was closed using a sheet of Permacol® mesh (Tissue Science Laboratories plc, Hampshire, England). The Permacol® mesh was sutured to the posterior leaf of the rectus sheath medially and the internal oblique laterally using a slow absorbing polydioxanone suture. The remaining external oblique muscle was closed over the mesh and the subcutaneous tissue and skin were closed in a standard fashion.\nThe surgical specimen measured 11 × 7 × 4 cm. In the centre of the specimen, there was a 4 cm whitish solid tumour. The tumour consisted of relatively large cells with granular eosinophilic cytoplasm and small pleomorphic nuclei with occasional nucleoli (Figure ). No conspicuous mitotic activity was noted. The tumour was completely resected within generous margins of normal tissue. The tumour cells showed strong positive reaction with S100 (Figure ) and were negative with GFAP. The appearances were therefore consistent with a GCT.\nThis patient has been reviewed in the outpatient clinic and is currently alive and well five months after surgery. |
Patient 1 was a 38 years-old male. In May 2010, this patient was diagnosed with glioma soon after an episode of seizures. MRI showed intra-axial expansive and infiltrative lesions that were cortical and subcortical, and which affected the anterior half of the right temporal lobe and extending from the pole to the Sylvian fissure superiorly and to the right parahippocampal gyrus, posteriorly, and medially. Partial surgical resection was performed in August 2010 and the first pathologic diagnosis was astrocytoma grade II. He underwent chemotherapy with TMZ at a dose of 2,000 mg with cycles every 28 days for 5 days in the years 2011–2013, with no tumor regrowth until the beginning of 2015. At this time, he underwent MRI, which was used to compare the discrete extension of the signal alteration areas, especially the subinsular regions. In March 2015, he resumed chemotherapy with TMZ at a dose of 100 mg/day and the patient then lost 12 kg of body weight, which was associated with anorexia, insomnia, and depression. In May 2015, he suffered a seizure requiring hospitalization. In June 2015, the patient resumed the old chemotherapy regimen with TMZ (2,000 mg every 28 days for 5 days), and a follow-up with MRI; however, the tumor size continued to increase. In January 2016, the neuro-oncology team decided to discontinue treatment with TMZ considering the risk/benefit and planned a surgical re-approach. This was followed by chemoradiation and lasting 6 cycles of PCV associated with CBD. The CBD dosage was ranging from 300 to 450 mg/day.\nDuring chemoradiation, the patient had an excellent clinical performance, practiced sports and had few symptoms of fatigue and/or nausea.\nAt 1 month after the end of chemoradiation, control MRI (Figure ) was characterized by exacerbation and the ultra-precocious phenomenon of PSD with increased edema and inflammatory disease characterized by extensive areas of contrast enhancement associated with tissue hypoperfusion (not shown). MRI controls demonstrated the progressive reduction of these findings.\nThe result of a pathological study after the first surgery was astrocytoma grade II with Ki67 staining of 5%. After the second surgery, he progressed to GBM grade IV (Figure ), related to increased cellularity, frequent mitosis, presence of micronecrosis, microvascular proliferation/endothelial, Ki67 staining of 30%, and loss of ATRX expression. Biomolecular marker analysis indicated IDH-1 mutated and MGMT methylated. |
A 53-year-old female presented with chief complaints of intermittent palpitations and chest tightness for 6 years, aggravated for 3 days. Six years ago, the patient got palpitation, chest tightness, and pulse acceleration (self-measured and the specific data was not clear) with no obvious causes. Five years ago, the patient had irregular uterine bleeding. When the hemorrhagic amount increased, the patient was prone to palpitations and chest tightness. She visited a local hospital for treatment and the coronary angiography showed no abnormalities. Three days prior to the admission, the symptoms of palpitations and chest tightness aggravated after activities, and nocturnal paroxysmal dyspnea appeared, accompanied by intermittent abdominal pain. Thus, she went to the local hospital again, electrocardiogram showed III degree atrioventricular block. The local diagnosis was coronary atherosclerotic heart disease, arrhythmia, and she received appropriate treatment, however, the symptoms did not improve significantly. Therefore, on the fourth day, she visited outpatient clinics and was admitted to the department of cardiology with the diagnosis of coronary atherosclerotic heart disease in our hospital. Physical examination revealed the body temperature of 35.7°C, heart rate of 78 beats per minute, respiratory rate of 18 breaths per minute, and blood pressure of 114/80 mm Hg. The patient was alert and cooperative. There was no cyanosis on the lips and no dilatation of bilateral jugular vein. Moist rales, without dry rales, can be heard in the left lung. The heart rate was 78 beats per minute with regular rhythm and no murmur was heard in the area of each valve auscultation. The abdomen was soft without tenderness or rebound tenderness. Liver and spleen were not palpable. Mobile dullness was negative. There was no lower extremity edema. Preliminary diagnosis was arrhythmia, III degree atrioventricular block, heart failure, irregular uterine bleeding cause to be investigated. After admission, the electrocardiogram demonstrated that sinus rhythm and I degree atrioventricular block. Cardiac color doppler ultrasound showed dilatated left atrium and right heart, a small amount of mitral valve regurgitation, a large amount of tricuspid valve regurgitation, and the increased pulmonary artery pressure with a small amount of pericardial effusion. Chest radiography showed increased heart shadow and a small amount of pleural effusion on the left. On the day of admission, the patient appeared restlessness with eyes gazing to the right, and felt weakened in the left limbs. Cranial computed tomography (CT) showed no abnormalities. The abdominal CT showed a small amount of pleural, peritoneal and pericardial effusion. Then the patient was transferred to the department of neurology. Neurologic examination: the patient was sleepy and could answer simple questions correctly. The eyes were fixed to the right. Pupils were 4 mm and briskly reactive to light. The forehead wrinkles and nasolabial folds were symmetric with normal eye closure. Tongue was middle with normal movement. The left upper limb muscle strength was grade 1, and the left lower limb was grade 3. Muscle tone was increased in the left limb. Motor examination of the right limb were normal. Bilateral tendon reflexes were symmetrical. Deep and superficial sensation were intact. Babinski sign was positive on the left side. Meningeal irritation sign was negative. Cranial magnetic resonance imaging (MRI) showed that right frontotemporal, right insula, bilateral basal ganglia, and left parietal lobe were presented with acute multiple cerebral infarction. Right internal carotid artery was not clear shown, and the right internal carotid artery and middle cerebral artery were not shown clearly (Figs. A and B). D-dimer was 3292 g/L. The level of troponin was 2.58 g/mL. The electrocardiogram was considered as an acute myocardial infarction. Blood routine examination showed white blood cell count was 12.53 x 109/L and neutrophil ratio was 91.3%. Biochemistry showed total protein of 57.2 g/L, albumin of 29.6 g/L, aspartate aminotransferase of 95 U/L, lactate dehydrogenase of 703 U/L, hydroxybutyrate dehydrogenase of 708 U/L, sodium of 133.0 mmol/L, and creatine kinase of 190 U/L. Thyroid stimulating hormone level was 4.400 U/mL. Carbohydrate antigen 125 was elevated slightly. Review of myocardial enzyme showed elevated aspartate aminotransferase (155 U/L), lactate dehydrogenase (1385 U/L), and hydroxybutyrate dehydrogenase (1355U/L). Brain natriuretic peptide was 10733.00 g/mL. The level of troponin and D-dimer was also elevated to 2.12 g/mL and 747 μg/L separately. Diagnosis showed acute cerebral infarction, acute myocardial infarction, heart insufficiency, heart failure. We gave the patient atorvastatin, cilostazol, edaravone, oxiracetam, butylphthalide, compound dextran in order to dilate vascular, nurture nerves, clear free radical, and improve microcirculation.\nThe patient felt repeated abdominal pain with no positive abdominal sign 1 week later. We considered the possible cause of mesenteric artery embolization, however, the abdominal pain relieved after 3 days. The left lung could be heard moist rales in the next week. The patient could follow the commands and her speech was clear and fluent. Both eyes were able to move coordinately in all directions. The left muscle strength increased to grade 3 of upper limb, and to grade 3+ of lower limb. We reviewed the laboratory tests 10 days later. The troponin was significantly lower than before (0.105 g/mL); myocardial enzyme also decreased, including aspartate aminotransferase (89 U/L), lactate dehydrogenase (374 U/L), hydroxybutyrate dehydrogenase (335 U/L), creatine kinase (24 U/L). Blood routine examination showed that neutrophil ratio was 85.7%; brain natriuretic peptide was 2678 pg/mL.\nThe patientʼs condition was stable than before. In order to search the cause of the disease, we checked cardiac color ultrasound again and found PFO (Fig. ). The dynamic electrocardiogram was used to evaluate the degree of atrioventricular block. Combined with troponin, myocardial enzyme, and electrocardiogram, we diagnosed acute myocardial infarction. Combined with physical examination and MRI, acute cerebral infarction was also clearly diagnosed. Discharge diagnosis was acute cerebral infarction, acute myocardial infarction, cardiac insufficiency, heart failure, arrhythmia with I degree atrioventricular block, and PFO. The study protocol was approved by the Ethics Committee of the Hebei General Hospital and the patient gave consent for publication of this case report. |
This 56-year-old-man, elder brother of the index case, was bed ridden when first presented to the author. He had a history of having had poliomyelitis at the age of 4 years and was left with a slightly wasted left leg but was in no way disabled. At the age of 35 years, he first noticed weakness in both legs with progressive difficulty in walking. Some years later he also noticed weakness in both upper limbs and had difficulty in raising his arms above shoulder. Over the years his disability increased but he never developed any form of abnormal movements. A CT scan of his brain was normal. His EMG (done on 3 occasions) was suggestive of a diffuse anterior horn cell disease with spontaneous activity at rest and poor recruitment with neuropathic potentials on volition. Nerve conduction study showed reduced CMAP amplitude, prolonged distal latency and slow conduction velocity in lower limbs and upper limbs suggestive of axonal degeneration. Sensory conductions were absent in upper limbs. Over the years, his clinical diagnosis varied from post-polio syndrome to Motor neuron disease and he was treated with Riluzole for a while.\nWhen examined in February 2009, he was bed ridden with slurred speech and dysphagia. His MMSE score was 28/30. His external ocular and sphincteric functions were intact. There were no abnormal involuntary movements anywhere. The tongue was wasted and showed fibrillations. There was gross weakness and wasting of all four limbs with occasional fasciculations in the upper limbs. He had total tendon areflexia.\nHis serum CK was normal (166 units) and a freshly drawn blood smear showed plenty (>50%) acanthocytes (Leishman stain) (DB). Electrophysiology was repeated. EMG features suggested evidence of chronic partial denervation with re-innervation and nerve conduction studies were suggestive of axonal neuropathy. Consent for immunohematology, molecular genetic, and muscle histology studies was not obtained. ECG, Holter monitoring, and echocardiography were normal. |
A 39-year old Caucasian female with known NF1 presented herself in the emergency department of an outside hospital with acute symptoms of thoracic pain and dyspnea. Her history was otherwise significant for an extensive thoracic myelomeningocele and status post-surgical correction of a thoracic scoliosis using a tibia bone graft 27 years earlier.\nOn physical examination at the outside hospital, she was awake, afebrile, blood pressure of 80/40 mmHg and a heart rate of 100 beats per minute. The initial haemoglobin was 12.5 g/dl, quickly deteriorating to 8.4 g/dl in the following hour. Chest x-ray (Figure ) was performed 40 mins after admission and revealed an extensive mediastinal shift. Subsequent computer tomography (Figure ) was performed 1h later at the outside hospital and revealed a massive hemothorax on the left side. The patient was intubated orotracheally because of hemorrhagic shock and imminent cardiopulmonary arrest and received seven packed red blood cell concentrates. A thoracic drainage was inserted prior to transfer. 4 h 10 min after admission to the outside hospital the patient was transferred to our institution and arrived there 1h later.\nOn admission, she was highly catecholamine-dependent. We found one left-thoracic suction drainage installed using Monaldi technique with 50 ml of blood in the drainage bottle. During placement of venous and arterial lines she suffered a cardiocirculatory arrest. Resuscitation was immediately initiated and we proceeded with performing an emergency exploratory left antero-lateral thoracotomy in the intensive care unit. Thereupon 2 liters of blood exsanguinated spontaneously. Despite massive blood transfusions (16 packed red blood cells, 2 thrombocyte concentrates, and 4 fresh frozen plasmas) and intravenous fluid administration no sufficient circulation could be established. Due to massive bleeding and very difficult operative conditions it was not possible to perform either an exploration of the thorax or an open cardiac massage via the same approach, which forced us to take a second approach through a median sternotomy for cardiac massage. Upon exploration of the thoracic cavity through the antero-lateral approach a large tumor, dorsal on the left side next to the vertebral column with massive bleeding, was detected. Despite several sutures and massive transfusions the bleeding could not be stopped and no sufficient blood pressure could be established. 7 h 30 mins after her first admission to the outside hospital and three hours after arriving at our institution the patient succumbed to the bleeding.\nOn autopsy multiple neurofibromas and several café-au-lait spots were scattered over the entire body surface. In the area of the tibia bone graft in the upper thoracic spine a protrusion of the dura mater of 8 × 10 cm was found. This meningocele showed a 1.8 cm perforation, which was probably due to the surgical procedures. Unfortunately, the autopsy could not reveal a distinct source of bleeding, but with a high probability, given the proximity to the oozing point, the bleeding must have originated from an intercostal artery. |
A 44-year-old female with no past medical history was referred to the gynecology department of our hospital because of progressive lower abdominal pain more left than right in the last 2 days. The patient had no other complaints except for a subfebrile temperature of 38°C. Cervical motion tenderness was present. Transvaginal echography showed a simple cyst of the left adnexa and a minimal amount of free fluid in the pouch of Douglas. No gynecological cause for the complaints could be identified. Surgery was consulted. We observed a painful looking patient. Vital signs were normal. Physical examination of the abdomen revealed hypoactive bowel sounds and tenderness of the whole abdomen, mostly in the lower abdomen more left than right. Laboratory studies showed leukocytosis (14,300 cells/μL) and an elevated C-reactive protein (59 mg/L). Abdominal ultrasound revealed a circumferential thickening of the wall of the sigmoid colon with possible stranding of the pericolic fat. Since no complete overview of the pelvis could be obtained, an abdominal CT was performed. CT showed fat stranding around the sigmoid colon and extraluminal air nearby a solitary diverticulum (Fig. ). The radiological diagnosis of acute diverticulitis with a free perforation was made.\nEmergency laparoscopy was performed which showed acute diverticulitis of the sigmoid colon with a substantial amount of pus in the pelvis and signs of an intramural perforation (incomplete perforation where not all layers of the colonic wall are perforated). After extensive lavage, the perforation was closed using the epiploic appendages and a Vicryl® suture. A drain was left behind in the pouch of Douglas. Postoperatively, the patient was treated with cefuroxime and metronidazole intravenously. The drain was not productive anymore after 4 days and was removed. After 5 days of intravenous antibiotic treatment, the patient was discharged in good clinical condition. Follow-up in the outpatient clinic has been uneventful up till now (10 months after surgery). |
The patient was a 59-year-old woman who had been treated 18 months previously for serous adenocarcinoma of the ovary. Treatment at that time consisted of total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic lymphadenectomy, para-aortic lymphadenectomy, and low anterior resection for direct invasion of the rectum. An anastomotic leak between the colon and rectum occurred after surgery and a diverting ileostomy was created. After adjuvant chemotherapy, the stoma was closed 8 months postoperatively. At 18 months after surgery, recurrence of the serous adenocarcinoma at the vaginal stump was detected on gynecologic examination. Radiological examination revealed no evidence of recurrence at any other site. Preoperative colonoscopy findings showed no evidence of the tumor at the prior anastomosis, which was located 5 cm from the anal verge. The patient strongly desired SPS and gave consent for treatment by this new method after a full explanation was provided.\nConventional laparotomy and adhesiolysis were conducted in the lithotomy position with the legs lowered. The tumor was located around the bladder, vaginal stump, and the prior rectal anastomosis, but no invasion of the bladder was observed. Dissection between the bladder and proximal vagina was performed without difficulty, and the ureters and superior vesical arteries were preserved. The perirectal tissues, particularly around the prior anastomosis, were dense and offered no dissection plane to secure the pelvic plexus. Dissection along the anterior surface of the sacrum was advanced as much as possible using electrocautery toward the deepest part of the pelvis, from which horizontal advancement was limited (Fig. , arrow 1).\nThe patient was subsequently placed in the lithotomy position with the legs elevated. A full-thickness circumlinear incision of the rectal wall was created inside the anal canal 3 cm from the anal verge, and the proximal stump was closed with a purse-string suture. After irrigation with physiological saline, a multiple access port (GelPOINT Path®, Applied Medical, Inc., Rancho Santa Margarita, CA, USA) was placed into the anus. An AirSeal System® (Conmed, Utica, NY, USA) was used to establish pneumoperitoneum with CO2 insufflation and smoke evacuation. An anterior dissection was performed from the anterior rectal wall to the distal two thirds of the vagina using an ordinary flexible laparoscope and a laparoscopic grasper. Care was taken to ensure that the dissection did not advance as far upwards as the tumor. This procedure resulted in a space behind the vagina we termed the “terminal” space, as its creation marks the final goal and end of the combined procedure (Fig. ). A dorsolateral dissection was made between the internal and external sphincter muscles and advanced as far as possible through the loose connective tissue between the sphincters (Fig. ). The separation unavoidably included scar tissue from the prior surgery on the levator muscle and sacrum. We secured hemostasis under endoscopic guidance (Fig. ). The posterior dissection was advanced into the scar tissue over the tip of the coccyx (Fig. , arrow 2). The communication to the abdominal dissection could not be completed using the TEA procedure. The lateral dissection was performed to preserve the sacral nerve.\nReturning to the abdomen, electrocautery was used to advance farther along the sacral parietal fascia to finally connect to the TEA dissection (Fig. , arrow 3), allowing for the creation of a “guidance port” for further dissection. An index finger was inserted into the connection to identify the plane between the rectal fascia and pelvic plexus. After completing the posterior dissection, the anterior and then posterior vaginal wall were sectioned with sufficient margin from the tumor to complete the terminal space behind the vagina made using TEA (Fig. , arrow 4). Scar tissue in the deepest part of the pelvis was dissected and the specimen was extracted. Bowel continuity was achieved by hand-sewn coloanal anastomosis, protected by a temporary loop ileostomy. Operation time was 301 min and total blood loss was 441 mL. Pathological examination revealed no involvement of the surgical margins (Fig. ). The postoperative course was uneventful and the patient was discharged on postoperative day 18. The diverting ileostomy was taken down 8 months later after adjuvant chemotherapy and following confirmation that there was no evidence of recurrence. After stoma reversal, the patient had frequent bowel passage, which is gradually improving with time, and has not noticed fecal soiling or mucus from the anus. |
In April 2013, in Northern Norway, a 24 year-old Caucasian healthy male soldier participated in a qualifying ski march to become a combat officer in the Norwegian Defence Forces. He was wearing full winter protection gear including wind protectors over his gloves. Earlier that day the weather conditions were fine with an air temperature around −5°C and a light southerly breeze. During the ski march, he felt warm and took of his gloves. The weather deteriorated rapidly in the afternoon. Air temperatures dropped to around −15°C and a forceful wind was now blowing towards his right side. Although he noticed a chilling of his hands from the wind, he still felt subjectively warm. After approximately 90 minutes, he decided to put on his gloves again and noticed that the skin of his hands had turned a blue-grey colour. His fingers felt numb with loss of finger dexterity. Having put on his gloves, he continued marching. After 2 hours, he felt a burning pain in his hands and noticed that the fingers were swollen and that blisters had developed on his fingers, especially on the right hand side. Strongly motivated to pass the test, he did not seek medical attention and continued the march. His fingers became more painful and he felt loss of sensation. After the exercise, he was diagnosed with second-degree frostbite injuries to his hands and was treated conservatively. The blisters were covered with paraffin gauze and a dry bulky dressing was applied. The wound dressings were continued until the wounds had healed. Due to cold hypersensitivity and pain when exposed to even a mild cold challenge, he was assigned to indoor duties.\nIn 2014, he wanted to qualify for a higher rank in a combat group. The training for this takes place in Northern Norway with more than 6 months of winter during which temperatures may reach −40°C. In the autumn of 2014 he reported that his fingers still became rapidly cold, pale and totally numb as soon they were exposed to cold, even at a temperature of +5°C. He felt a constant pain and loss of sensation in his fingers resulting in reduced finger dexterity. He was deemed unfit for outdoor military service and had to leave the combat unit.\nIn May 2015, he was referred to the outpatient clinic for Plastic Surgery at the University Hospital North Norway. He was diagnosed with sequelae from frostbite. To exclude other pathological conditions further investigations were carried out. Angiography of the upper extremities showed open arteries on the lower arms, but all digital arteries were described as very thin with no dilatation after heat stimulation or hand/finger movements. No other pathology was found.\nDynamic Infrared Thermography (DIRT) [] was performed to test the patient’s response to a cold challenge. This involved measuring the skin temperature of the dorsal aspect of both hands before, immediately after and for 3 minutes following a mild cold challenge (1 min. immersion of the hand in water at 20°C). During the immersion, each hand was covered by a thin plastic bag to avoid it becoming wet. The skin temperatures were measured with a high definition infrared camera. The patient was wearing outdoor winter clothing to stimulate peripheral vasodilation. Prior to the cold challenge, the patient reported that he felt warm. Additional obtained facial thermographic images showed the nose was vaso-dilated, presumably due to open arterio-venous anastomoses. DIRT showed a rapid onset of cooling and slow rewarming of the fingers after the cold challenge, similar to the slow warming subjects reported by Brandstrom []. However, in our patient we performed a mild cold challenge, resulting in a clear difference between the right and left hand, the right hand being cooler.\nQuantitative Sensory Testing (QST) [] indicated a loss of function with regard to non-painful stimuli in the mechanical and thermal domain (cold-, mechanical-, and vibration detection threshold). These findings corresponded well with the patients’ symptoms, namely loss of sensation in his fingers and reduced finger dexterity.\nThe soldier was interested in receiving treatment. He was informed on the off label use of BTX-A to treat vasospastic disorders and nociceptive/neuropathic pain disorders. He consented to a treatment with BTX-A. Each 100 unit vial of BTX-A (Allergan, Inc. Irvine, CA) was prepared in a standard manner with a concentration of 40 units/ml. The injections were administered at the neurovascular bundles in the palm of each hand at the level of the metacarpophalangeal joints (60 Units BTX-A per hand). This resulted in a dose of 12 units of BTX-A just at or proximal to the annular pulley A1 of the flexor tendon sheath of each finger.\nAt 3 weeks follow up the patient reported that he had less pain, warmer hands and improved sensory function, based on his own subjective evaluation. In November 2015, during the winter period, the patient reported improved cold tolerance and was able to perform normal outdoor activities. Angiography, DIRT and QST were repeated and the results were compared to the pre-treatment results.\nAngiography showed that the digital arteries were more dilated and more clearly visible (). DIRT showed rapid and improved rewarming of all fingers compared to the pre-treatment examination although the rewarming of the right hand was still a bit slower compared to the left hand at all time points during the examinations (). QST showed a normalisation of sensory function apart from a marginal reduction of the ability to detect vibration (). Based on the encouraging results it was agreed to repeat the treatment. The same procedure with the same dose of BTX-A of 60 U (40U/per ml) per hand was used as during the first treatment.\nAt 6 weeks follow-up in January 2016, the patient reported even further improvement. The only side effect of both BTX-A treatments was a temporary weakness of the intrinsic muscles of each hand, the lumbrical and interosseous muscles to the 2–5 finger and the flexor pollicis breves, the opponents and adductor muscles of the thumb. This transitional weakness lasted about 3 weeks. |
A 13-year-old girl was admitted with dyspnea due to severe pneumonia and respiratory failure. On past medical history, she developed bronchiolitis at the early age of 1 month for the first time. Since that time, pneumonia and bronchiolitis recurred several times a year up to school age. The medical history of her family was unremarkable. When she was 10-years-old, she was referred to our department for the evaluation of abnormal infiltration of both lung fields visible on a chest X-ray. At that time surgical removal of nasal polyps due to chronic sinusitis was decided and a chest X-ray was checked as one of preoperative evaluations. However, she had no respiratory symptoms or signs and her breathing sound on auscultation was clear. Several evaluations to find the causes of this abnormal infiltration were performed, especially by tuberculosis tests. But AFB stain, PCR and culture of sputum and urine were all negative and a Mantoux test was also negative. So, she was diagnosed with simple infectious bronchiolitis with bronchitis. After that, she was followed up irregularly during the next 3 years. Several months before admission, she had been also diagnosed with hypothyroidism and gastritis at another hospital.\nOn physical examination, she had lip cyanosis and clubbing fingers. A computed tomography (CT) scan showed multifocal bronchiectasis and extensive bronchiolitis (). In the course of the admission, she was supported by mechanical ventilation for nearly one month and by the parenteral treatment of antibiotics for pseudomonas infection proven by culture of sputum. She has received chest physiotherapy to improve mucus clearance with mucolytic drug. Systemic and inhaled corticosteroids were also applied for short-term period. On admission, the laboratory findings of this girl were as follows: pH 7.37 and pCO2 35 mmHg on arterial blood gas analysis, white blood cell count 22,860/mm3, hemoglobin 9.3 g/dL, platelet count 757,000/mm3, erythrocyte sedimentation rate 83 mm/hour, C-reactive protein 11.18 mg/dL, blood urea nitrogen (BUN) 4.4 mg/dL, serum creatinine 0.42 mg/dL, total protein 4.8 g/dL, and albumin 1.8 g/dL. Cold agglutinin and antibody to mycoplasma were negative. Urinalysis showed protein (++), occult blood (+) and urine protein during 24 hours was 2,090 mg/m2. To elucidate the cause of bronchiectasis, several additional tests were also performed. Immunologic studies showed normal levels of immunoglobulin (Ig) G, IgA, IgM and α1-antitrypsin except for a mild elevation of IgE. Several weeks later a sweat test performed as one of the evaluations of CF was not specific and there was no mutation of the CF transmembrane conductance regulator (CFTR) gene. After confirming non-CF related bronchiectasis, a mucosal biopsy of the nasal cavity was performed to rule out primary ciliary dyskinesia and the result was normal on an electron microscopy (EM). Six months later after an improvement of her respiratory condition, an ultrasonogram-guided renal biopsy was performed to reveal the cause of proteinuria. On light microscopy, there were many segmental homogeneous deposits of amyloid with positive Congo red staining in the glomeruli and interstitium (). Immunohistochemistry revealed positive staining of the AA amyloid and immunofluorescent microscopy showed negative staining of IgG, IgA, IgM, C3 and fibrinogen. EM showed relatively straight, non-branching, randomly arranged amyloid fibrils in the mesangium of the glomeruli and these fibrils were approximately 10 nm in diameter (), compatible with secondary amyloidosis. At that time, serum amyloid A was remarkably elevated (62.0 mg/L, normal range: <8.0 mg/L).\nAt recent follow-up (22 months after admission), BUN and serum creatinine were mildly elevated (34 mg/dL and 1.2 mg/dL, respectively) and proteinuria was improved with the results of the protein (+) in dipstick and decreased spot urine protein-creatinine ratio. The repeated chest film and CT scans showed no remarkable interval change and serum amyloid A slightly decreased than before (26.9 mg/L). |
A previously healthy 38-year-old man, who had emigrated from Honduras 5 years ago, presented with a several weeks history of progressive abdominal pain and dyspnea. Physical examination revealed respiratory distress, an irregularly irregular tachycardia at 140 beats per minute, and a blood pressure of 126/72 mmHg. The cardiac exam was remarkable for a displaced and diffuse apical beat, and a 2/6 mitral regurgitation murmur heard at the apex. His ECG demonstrated atrial fibrillation (AF) with a rapid ventricular response. There was no electrocardiographic evidence of right ventricular overload. Chest X-ray showed cardiomegaly and pulmonary edema. Transthoracic echocardiography (TTE) demonstrated a markedly dilated left atrium in the four-chamber view (51 mm) divided into 2 chambers by a membrane (). Continuous wave Doppler showed flow across the membrane with a peak diastolic gradient of 6 mmHg, peak systolic gradient of 2 mmHg, and a mean of 4 mmHg (). The left ventricle (LV) was dilated in the parasternal long-axis view (65 mm) with an ejection fraction of 20–30%. There was moderate-to-severe functional mitral regurgitation. The right ventricular systolic pressure (RVSP) was 70 mmHg. Transesophageal echocardiography demonstrated a membrane within the left atrium (LA) with a 7 mm gap in its midportion and measured a peak gradient of 15 mmHg. The interatrial septum was intact. Of note, the patient had a previous TTE 5 years ago when first immigrating to Canada, which identified a dilated left atrium and the cor triatriatum defect which had a peak gradient of 9 mmHg, which was not that much different than the more current study. The LV was normal in size and systolic function at that time. The patient and his primary care provider were unaware of this prior established diagnosis.\nThe patient was admitted and diuresed. He spontaneously converted to sinus rhythm and remained free of AF at discharge. Given the coexistence of AF and cor triatriatum sinister, the decision was made to initiate anticoagulation with heparin with subsequent conversion to warfarin. The workup of alternative causes of his dilated cardiomyopathy (including serology for Chagas and autoimmune disease) proved to be negative. In addition, there was no history of significant alcohol intake. At 3 months follow-up, he remained in sinus rhythm and had returned to functional class 1 despite lack of improvement in LV systolic function on focused cardiac ultrasound evaluation. |
The patient was a 20-month-old male who was referred to Myelin Disorders Clinic due to motor milestones delay and neurologic regression which was first noted at 6 months of age by his parents. He was born at term at 39 weeks of gestation through normal vaginal delivery from consanguineous parents. His mother had taken good prenatal care; however, she experienced preeclampsia during her final pregnancy trimester. The birth weight and head circumference (HC) of the patient were 3350 g (Z-score 0SD) and 34 cm (Z-score 0SD), respectively. Shortly after birth, he was admitted to the neonatal intensive care unit (NICU) due to a low APGAR score at 5 min of age, not crying, and possible birth asphyxia for a short-term period without the requirement to assisted ventilation and finally was discharged with the good general condition after 2 days.\nAfter hospital discharge, limb jerking movements were noticed during sleep by his parents which were diagnosed with neonatal sleep myoclonus by their family physician. They were stopped within a few days without treatment. In addition, he was admitted again due to exaggerated neonatal jaundice with indirect bilirubin of 17 mg/dL at the end of the first week of life which was treated by phototherapy. Given his developmental milestones, he achieved neck holding at age 2 months, rolling over at age 4–5 months, and starting to crawl at age 6 months.\nThereafter, the patient’s motor development stopped and motor regression commenced. His parents recognized that he lost his ability to crawl and using his legs especially the left foot, gradually. He was not able to sit even with support at 8 months old. As a result, firstly, his parents visited a pediatric orthopedist, and then, they were referred to a child neurologist for further investigation. Moreover, his pelvic X-ray was normal and he had no contracture, joint deformity, and obvious scoliosis. On neurologist examination at 9 months of age, lower limb spasticity, hand fisting, increased deep tendon reflexes (DTR 3+) in lower limbs, and bilateral upward plantar reflex were detected. Ophthalmic examination included all directions’ gaze, fix and follow, reaction to light, and strabismus was normal. His weight and head circumference were 8 kg (Z-score −1SD) and 45 cm (Z-score 0SD), respectively. All biochemical and basic metabolic tests including serum creatine phosphokinase (CPK), thyroid, liver, and kidney function tests; uric acid level; plasma amino acid chromatography (HPLC); and alpha-1, 4 glucosidase enzyme activity level which was measured by dried blood spot (DBS) test were within normal limit. Occupational therapy was started and a follow-up visit was scheduled at 1 year old.\nOn review at age of 12 months, his motor ability had declined, significantly. He was not able to crawl or roll over anymore. Furthermore, his lower limbs had become more spastic. He had acceptable social interactions and no significant feeding or swallowing problems, but his speech and cognition skills progression were arrested according to his age. His weight, height, and head circumference were 8.5 kg (Z-score −1SD), 76 cm (Z-score 0SD), and 46 cm (Z-score 0SD), respectively. A brain and spinal MRI were done which were reported normal by a neuroradiologist (not shown).\nAdditional metabolic studies consisting of serum ammonia and lactate, metabolic screen (MS/MS), acylcarnitine, and urine organic acid profiles were all normal. Abdominal and pelvic ultrasonography revealed an ectopic right kidney in addition to horseshoe kidneys. Cardiologic and ophthalmologic consultations indicated no significant findings. Finally, whole-exome sequencing was done according to motor neurologic regression, speech, and cognitive delay; parents’ consanguinity; normal metabolic test results; and imaging study.\nThe second brain MRI which was done at 18 months old indicated delayed myelination of subcortical white matter in addition to abnormal signals of posterior periventricular white matter (Fig. (3A, C white arrows)). The T1-weighted sequence at the level of basal ganglia indicated no significant finding (Fig. (3B)). Sagittal T1-weighted image showed mild thinning of the corpus callosum and upper vermis (Fig. (3D)). Furthermore, electromyography and nerve conduction velocity studies (EMG-NCV) revealed generalized sensory polyneuropathy. |
Patient is a 43-year-old female with past medical history of a SG and an ‘anterior’ hiatal hernia repair at an outside facility 11 years ago for a body mass index (BMI) of 74.3 Kg/m2 at index SG. The reported hiatal hernia repair at index SG involved approximation of the right and left crura with one stitch anterior to the esophagus without a formal standard posterior or mediastinum esophageal dissection. She presented to our clinic with 3 years history of progressive dysphagia and heartburn. Preoperative upper gastrointestinal (UGI) series and computed tomography (CT) scan demonstrated the migration of almost all of the sleeved stomach into the thoracic cavity with a partial organoaxial volvulus ( A and B). UGI endoscopy showed 10 cm hiatal hernia with intrathoracic migration of the SG with esophagitis grade B (Los Angeles classification).\nThe patient underwent an elective laparoscopic surgical revision. Intraoperatively, majority of the SG had herniated into supradiaphragmatic position through the diaphragmatic esophageal hiatus and had a partial organoaxial volvulus (). Lysis of adhesion was carried with full mobilization of the SG and distal esophagus were achieved. The intrathoracic SG was reduced followed by the excision of the hernia sac with preservation of anterior and posterior vagus nerves and the left gastric artery (). The esophageal hiatus diaphragmatic defect was closed with four figure of eight stitches posterior to the esophagus using braided zero polyester (). A relative narrowing of the mid portion of the SG at the incisura and an excess of gastric fundus was present. A 4 cm long gastric pouch was created in a standard fashion and the remnant of the SG was excised ( and C). Then a standard jejuno-jejunostomy (J-J) was done with a biliopancreatic limb of 60 cm and alimentary limb of 100 cm and the gastro-jejunal anastomosis was created using a 25 mm/4.8 mm circular stapler and reinforced with Lambert sutures of 3–0 polyglactin (). The J-J and Peterson mesenteric defects were closed. Intraoperative leak test was negative.\nThe recovery was uneventful and the patient was discharged home on postoperative day 3. The patient was symptoms free upon 2 years follow-up with BMI of 33. CT scan 1 year postoperatively was performed for symptoms not related to surgery confirmed an intact repair (). |
25-year-old male patient presented with a complaint of black colored growth on the anterior maxilla since 3 months. Patient gave the history of extraction of loosened upper front tooth which was associated with a black tinted peanut sized growth. Following extraction the growth gradually increased and attained to the present size with change of colour to dark black. Growth was associated with difficulty in eating, speaking, loosening of adjacent teeth, mild pain, and frequent bleeding on provocation. Patient gave history of no such similar disorders in the family, and no adverse habits. The patient was moderately built and nourished, and vital signs were normal. General examination did not reveal any other similar findings. Extra oral examination shows incompetent lips with obliteration of nasolabial fold on right side (). Lymph nodes were nonpalpable. Intraoral examination showed grayish black colored exophytic growth on maxilla involving maxillary gingiva and hard palate (Figures and ) of approximately 7.5 × 5.0 cm2 size extending anteriorly on gingiva from 16 to 24 by crossing midline with displacement of 11, 21, and 22 and superiorly to depth of maxillary anterior vestibule, and posteriorly to soft palate, involving whole of the hard palate with protrusion into oral cavity. Tooth 12 was missing. Surface was irregular, shiny with teeth indentation at the borders. On palpation the lesion was slightly tender, soft in consistency, smooth textured, and fixed to underlying structures and bleeds on probing. Based on the clinical findings provisional diagnosis of oral malignant melanoma involving maxillary alveolus, palate was given. Differential diagnosis of any vascular related disorders was suspected.\nTo rule out the diagnosis, primary investigations such as aspiration, haematological and urine examinations, incisional biopsy, and CT were done.\nAspiration was negative. Haematological, urine examinations did not reveal any significant findings, which showed that the lesion was nonhematological. Histopathology revealed strips of squamous epithelium with proliferation of tumor cells arising from basal layer infiltrating deeply into the stoma. Individual cells are round to spindle with melanin pigment. Nuclei are binucleated and large mononucleated giant cells are seen, which is suggestive of malignant melanoma ().\nCT revealed a soft tissue density mass in the maxillary alveolus, hard palate protruding into oral cavity measuring 5.4 to 3.2 cms (Figures and ). The mass is eroding the alveolus in the midline and eroding free molars. And also causing displacement of anterior teeth. Mass does not involve an sinuses. Contrast CT revealed lymphadenopathy of right axillary and few subcentimeter lymph nodes of the neck.\nFNAC of cervical and axillary lymph node, chest radiograph, and CT of chest and abdomen were done to know the metastatic extent. FNAC revealed metastasis of malignant melanoma to right cervical and axillary lymph nodes. Chest radiograph, abdominal sonography, and CT of chest and abdomen did not show any significant metastatic lesions.\nSince the lesion was large with metastatic extension the patient was referred to higher oncology center for further treatment. |
A 30-year-old male presented to an outside facility with the complaint of increasing low back pain over the past month that radiated bilaterally to the level of his ankles. The pain was accompanied by progressive lower extremity weakness to the point where ambulation became impossible 3 days prior. He also complained of bilateral calf and anterior thigh numbness with paresthesias. He denied changes in normal bowel and bladder function. He had been evaluated 3 weeks prior at the same facility with similar but less severe complaints and diagnosed with bilateral sciatica. Workup during the more recent visit revealed an L2 mass invading the spinal canal for which the patient was transferred to our facility for neurosurgical evaluation [].\nThe patient had noted a left testicular mass 3 years ago and stated that it had been enlarging in size and was painful to touch. He denied having ever brought this complaint to the attention of a physician. He also admitted to poor appetite and significant weight loss over the prior month amounting to 25 pounds and attributed this to his major depression. He denied fevers, vision changes, night sweats, or any other palpable lesions or rashes.\nThe patient was a pale, anxious-appearing male who was alert and cooperative, tachycardic, and hypertensive. He exhibited tenderness to palpation in the midline lumbar spine. Upper extremity strength was normal. Right hip flexion, knee extension, dorsiflexion, and plantar flexion were grade 4/5. The same motor groups were only at anti-gravity strength (3/5) on the left side. Rectal sphincter function was normal. On sensory exam, saddle anesthesia was noted as was decreased sensation to light touch in a distribution that approximated the bilateral L2 and L3 dermatomes. His reflexes were grade 2+ throughout and no long-track signs were observed. His left testicle was enlarged and a mass was palpable that was non-tender at the time.\nA magnetic resonance image (MRI) of the spinal axis demonstrated a 12 cm left-sided retroperitoneal mass that extended into the spinal canal at the L2 and L3 levels through the left L2 and L3 neural foramen with severe thecal sac compression at the L2 level []. A scrotal ultrasound showed an echogenic region within the left testis with clustered microlithiasis suspicious for primary testicular neoplasm. Initial serum labs were remarkable showing the following levels: Sodium 131 mmol/l, alkaline phosphatase 291 IU/l, hemoglobin 6.7 g/dl with serum iron level being 13 μg/dl and ferritin level of 2294 ng/ml, albumin 2.3 g/dl, sedimentation rate 125 mm/h, and β-Human Chorionic Gonadotropin 248,379 IU/l.\nIn the first 24 h after admission, during his inpatient medical workup, the patient's lower extremity motor exam deteriorated rapidly to less than anti-gravity bilaterally. A decision was made to urgently decompress and stabilize the spine, as well as obtain tissue diagnosis before the full metastatic workup had been completed. Bilateral laminectomies were performed from L1 through L3. Soft tumor mass was removed extradurally from within the canal, resulting in thorough decompression of the conus and cauda equina. Surprisingly, blood loss was not particularly bad and transfusion was not needed. Frozen intraoperative pathology was reported as metastatic carcinoma. As the amount of bone and tumor removed was likely destabilizing and the bone quality found to be poor at these levels, a posterolateral pedicle screw fusion was performed from T12 through L4 [].\nFinal histopathologic analyses revealed abundant tissue necrosis with an estimated live tumor cell presence of only 5-10% []. Immunohistological analysis indicated a final diagnosis of metastatic choriocarcinoma.\nPostoperative metastatic disease burden workup revealed that the retroperitoneal mass was actually 13 × 14 × 26 cm and encased the aorta, splenic artery, the left renal artery, and vein. Also, there was extensive metastatic burden within both lungs and the liver. Free fluid was present within the pelvis. A contrasted MRI of the entire neuraxis revealed a hemorrhagic right occipital enhancing lesion, a non-enhancing subcortical left inferior frontal lobe lesion, and an enhancing C3 vertebral body lesion.\nWithin 24 h after decompression, the patient's bilateral lower extremity strength and sensation improved. He was maintained on dexamethasone for cerebral and spinal edema. Subcutaneous enoxaparin and sequential compression devices were used to prevent thromboembolism. Chemotherapy was initiated on postoperative day 9 and consisted of etoposide, ifosfamide, and cisplatin. On the same day that chemotherapy was started, his hospital course was complicated by acute respiratory failure secondary to a suspected pulmonary embolism. He was intubated and an inferior vena cava filter was placed to prevent additional emboli. He declined while intubated, requiring pressor support, and developed a large pericardial effusion which was drained via pericardial window. He was started on continuous renal replacement therapy, and his spouse elected to withdrawal care compassionately on postoperative day 21 (hospital day 22) which resulted in death on the same day. |
A 67-year-old female, with a typical presentation of carpal tunnel syndrome in the right hand, presented initially with numbness in digit IV and V a year later. A few months later, she developed numbness in all five fingers on the left side. Furthermore, the patient mentioned that she had had a burning sensation in both feet for a few years. Another few months later she complained of weakness in all four limbs and was sent for a neurological consultation with electromyography. Clinical examination revealed a Hoffmann-Trömner reflex on the left side, mild loss of strength in both hands, loss of vibratory sense and hypoesthesia in the distal end of all four limbs and loss of proprioception in both legs. The electromyography showed disturbed sensorimotor signals in the left hand and mildly disturbed sensorimotor signals in the right hand. The disturbed electromyography was attributed to carpal tunnel syndrome, which was presumably less severe on the right side due to treatment with long acting corticosteroid injections. The paresthesia in digit IV, digit V and both feet, however, could not be explained with the diagnosis of carpal tunnel syndrome. Therefore, magnetic resonance imaging (MRI) of the cervical spine was performed. Imaging showed an extra-dural soft tissue mass posterior to the odontoid process of the axis. The mass extruded through the transverse ligament of the atlas with severe compression of the myelum and myelomalacia at the level of C1 (Figures , and ).\nA plain radiograph of the cervical spine was performed to check for atlanto-axial instability. The radiograph during flexion shows a slightly widened atlantodental interval, measuring 4 mm (normal value: <3 mm) (Figure and ).\nPre-operative imaging of the cervical spine also revealed an anatomical variant of the craniocervical junction, atlanto-occipital assimilation of the massa lateralis bilaterally (Figure ).\nThe patient was treated with laminectomy of C1 to decompress the spinal canal followed by posterior fixation of C1-C2 for stability. |
A 16-year-old male complained of inability to flex his left elbow since 1 year prior to admission. One and a half year before, he fell down and hit his elbow during football practice. He felt pain and there was swelling on his elbow. However, he didn't seek for medical treatment. He had his elbow massaged every week for 5 months but there was no improvement. His elbow became fixed in extended position. A month later, he went to an orthopaedic surgeon and underwent x-ray examination which revealed a fracture and dislocation on his left elbow. He was then referred to our institution for further treatment.\nFrom clinical examination, range of flexion-extension of the elbow was 300-00 with normal pronation-supination. There was no neurological deficit (). From radiological examination, there was a malunion of medial epicondyle with subluxation of left proximal ulna (). From 3D CT reconstruction, there was a deformity and malunion fracture in humeral capitellum with radial and ulnar postero-superior dislocation (). The patient was diagnosed with extension contracture of the left elbow due to malunion of left capitellum, neglected dislocation of the radiohumeral joint, and neglected dislocation of the ulnohumeral joint. The patient was scheduled to have a contracture release, open reduction and internal fixation, and ulnar interposition.\nIntraoperatively, we did a posterior approach to the elbow. The ulnar nerve was identified and preserved. The fibrotic tissues and heterotopic ossification were excised. We did a contracture release and open reduction and internal fixation using K-Wire. The flexion and extension of the elbow were evaluated and we managed to get 300 - 130° of flexion-extension ROM. Afterwards, ulnar interposition was performed to prevent ulnar impingement. The wound was closed and a single drain was placed. The elbow was immobilized with back-slab in 900 flexion position for two weeks.\nAfter 1 week, the patient went back to our hospital for follow-up examination. In the 1st evaluation, we tried to remove the back slab and moved the elbow passively. The movement is restricted due to pain and the patient went back home with the back slab on. In the 2nd week follow-up, we permanently remove the back slab and the stitches. At that time, the pain still persisted and the patient was planned to have physical rehabilitation.\nOn the 4th week after surgery, the surgical wound was infected. We performed debridement, implant removal, and manipulation under general anesthesia. Two weeks later, patient came back to our hospital. We removed the stitches and started rehabilitation. Later on, he continued his rehabilitation in his previous hospital.\nAfter 6 months, he visited our outpatient clinic for medical checkup. From physical examination, the elbow flexion-extension ROM was 1100 - 300 (). The patient is able to do normal daily activities (). |
A 46-year-old female with symptoms of hoarseness and Horner's syndrome presented with a left cervical mass that was diagnosed to be undifferentiated carcinoma based on the findings of aspiration cytology (Figure ). The patient's chest computed tomography (CT) findings showed a mass measuring 5.0 cm in size spreading from the left upper mediastinum to the left supraclavicular area, which pressed against both the trachea and esophagus and it seemed to involve the left common carotid artery (Figure ). Based on these findings, and cytology findings a clinical diagnosis of stage IIIB (T4N3M0) non-small cell lung cancer (NSCLC) originating from the apex of the left lung involving both the mediastinum and the supraclavicular lymph nodes was made []. The patient received concurrent chemo-radiotherapy (cisplatin 80 mg/m2 for days 8 and 36 + UFT 400 mg/m2, both on days 1–14 and on days 29–42 plus radiotherapy, 2 Gy/day on days 1–20 for a total of 40 Gy) []. After this treatment regimen, the tumor size decreased by 35.0%. Thereafter, the patient underwent a surgical resection though a median sternotomy with a combined resection of the left clavicle. During the operation, an encapsulated tumor was detected in the mediastinum. Although the tumor was easily ablated from the left common carotid artery, it involved both the left vagus and the sympathetic nerves. As a result, both nerves had to be sacrificed in order to achieve a complete resection of the tumor. Grossly, the tumor was in continuity of the vagus nerve was whitish in color and oval shaped measuring 5 × 3 cm in diameter (Figure ). Both cytological and histological examinations revealed 1) Continuity between the vagus nerve and tumor was seen, while the no continuity between the tumor and the sympathetic nerve was found. 2) The findings of aspiration cytology of the tumor diagnosing it to be undifferentiated carcinoma before the treatment included an atypical spindle cell. (Figure ). ) The predominantly tumor consisted of necrotic tissue and a few viable atypical spindle cells (Figure ) which were positive for S-100 protein (Figure ). As a result, the tumor was considered to arise from the left vagus nerve while invading the left sympathetic ganglion, and was therefore diagnosed it to be a malignant schwannoma. At present, the patient has survived for about 2 years since operation without any recurrence. |
The patient was a previously healthy 28-year-old man who presented to the emergency room in the spring of 2008 with a one-day history of a headache after shoveling snow. It resolved but recurred the next day. His headache became very severe with subsequent nausea and vomiting. Computerized tomography (CT) scan at 15:10 hours showed a hemorrhagic tumor measuring 3.9 x 4.4 cm in the left cerebellar hemisphere, with cerebellar tonsillar herniation and midline shift. While in the emergency room, he developed a decreasing level of consciousness and apnea, requiring intubation and ventilation. The repeat CT at 23:20 hours demonstrated more bleeding and the tumor measured 6.3 cm. Therefore, at midnight he underwent an emergency frontal ventriculostomy, left suboccipital craniectomy, evacuation of hematoma, and tumor debulking. The next day, a repeat CT showed a residual tumor mass measuring 3.5 x 3.1 cm. Since the residual was more than 1.5 cc, the stage was considered to be a high-risk disease. Edema was present in the deep left cerebellar hemisphere, and there was slight compression and shift of the midline, including some compromise of the fourth ventricle. There was some persistent mass effect upon the left posterior brainstem and quadrigeminal plate cisterns were effaced.\nHe suffered a third episode of hemorrhage into the tumor bed with increased intracranial pressure on postoperative day (D) 4. He was re-operated to evacuate the hematoma. At that time, a portion of the cerebellum had become necrotic and swollen and was therefore evacuated. He underwent a third craniotomy on D12 to evacuate a new hemorrhage in the tumor bed, i.e. his fourth episode of bleeding. Pathology confirmed a Grade IV medulloblastoma.\nHis clinical course continued to be very unstable, necessitating that he remained in the intensive care unit with sepsis and fever, although cultures were negative. He had respiratory failure and was ventilated for 21 days. He required a tracheostomy. He was suffering from a persisting severe productive cough with excessive secretions, requiring frequent suctioning. His blood pressure was labile, with systolic varying between 100 to 180 mm Hg. He had a decreased level of consciousness, with fluctuating Glasgow coma scale of 8-10. Some days he did not respond to commands. He also developed agitation, seizures, and intolerance of tube feeding with continual vomiting. A repeat CT scan following surgery still showed persistent blood and edema in the cerebellar area. His severe weakness, the decreased level of consciousness, and poor general condition were most likely due to posterior fossa syndrome.\nOn D31 postoperatively, his level of consciousness improved after dexamethasone was increased in frequency from 4 mg thrice to four times a day. He could obey commands to open his eyes and move the toes. He had limited facial expression. He was on enoxaparin, 40 mg subcutaneously, to prevent deep vein thrombosis that, fortunately, did not provoke cranial rebleeding. The cerebellar hematoma was smaller on the MRI scan on D43 compared to that on D32.\nA prone CSI technique would have been impossible because of his moderate paroxysmal coughing spells, secretion from the tracheostomy requiring frequent suction, and oxygen. Our department designed a relatively simple supine CSI technique without skin gaps to facilitate treatment (Figure ).\nThere was no skin gap between the cranial and upper spinal fields since both had half-beam blocks. The half-beam block isocenter was just below the cranial planning target volume (PTV). It would have been more ideal to position it further inferiorly if the field size limitation (20 cm) allowed the entire head to be covered with margin. Therefore, the position of the neck junction depends on the size of patient’s head. For a smaller head, the junction can be in the lower neck. Imaging is done for brain/superior spine fields (which are mono-isocentric) and to ensure the patient is straight per length of the treatment area.\nBoth spinal fields have couch rotation to 270 degrees. The patient was shifted superior-inferiorly. The couch was “kicked” through to 270 degrees and the gantry was rotated to match the divergence of the superior border of the inferior spine field to the inferior border of the superior spine field (imaging could not be done once the couch was “kicked”). The inferior border of the superior spine field was marked on the underside of the couch (paper and marker) for checking. The inferior spine field can be treated with extended skin-source distance (SSD) if required to treat the remainder of the spine. Stepped control points were used to smooth the dose over the length of the spine. The plan contains four junction shifts at 0.5 cm to smooth out any chance of over/underlap where fields match. All junctions were shifted weekly, i.e. every 9 Gy. Compensators were used. A cranial aquaplast shell and body vac lok were used for immobilization.\nThis technique did not require intensity modulation radiotherapy, which simplified and expedited his treatment planning, and quality assurance was less complicated. Planning CT was done on D36 and treatment started on D38 postoperatively. With this technique, the amount of time required at the time of CT simulation was not prolonged and the patient could remain still for the duration of each fractional treatment. The total set-up and treatment time was 40-50 minutes because he required suctioning of sputum. The supine technique was well tolerated and more stable than the prone technique and, as a consequence, this results in a reduced daily treatment time.\nAt the conclusion of chemotherapy, magnetic resonance imaging (MRI) head showed complete remission of the residual tumor. At last follow-up in the fall of 2015, he finished all physical rehabilitation and could now walk with minimal support. His speech was initially muffled and now he is comprehensible on the phone. He has diplopia and limited facial expression. Due to mild ataxia on examination, past-pointing is worse with the left hand than the right hand. He frequently enjoys watching his favorite football, sitting in the stands at the stadium. MRI scan of the head has not shown any recurrence in the posterior fossa. Our department has stopped the prone technique since this case and adopted this technique as a standard set-up. |
A 4 year-old girl from Sarlahi district in Nepal was brought to our emergency room with a history of sudden onset of severe headache and generalized tonic clonic seizures. She had a history of a minor fall injury while playing at preschool. At that time there was no loss of consciousness, nausea or vomiting and she remained well for the following two days. Two days later at around 3 a.m., the child screamed out in her sleep and complained of severe headache followed by an episode of generalized tonic clonic seizure. The child was then rushed to the hospital. At presentation, her Glasgow Coma Score was 14/15 (E4/M6/V5). No anisocoria was present. She was hemiparetic on the right side with a power grade of 3/5. Computed tomography (CT) scan of the head showed focal intra cerebral hemorrhage in the medial basifrontal region and subarachnoid hemorrhage (SAH) in the inter-hemispheric and in the left sylvian fissure (\n). No significant past medical or surgical illnesses were elicited. She was managed conservatively in the neurosurgical intensive care unit. In repeated serial CT scans, the hematoma was found to be resolving and the child’s motor power in the right side had improved to 4+/5. The child was discharged with advice of regular follow up. The child was again brought to the emergency room one month following the initial hemorrhage with a history of headache and repeated episodes of vomiting. GCS was 15/15 with both pupils equal and reacting to light. CT scan showed re-bleed in the left medial basifrontal region with ventricular extension (Graeb score of 8/12) (\n). The child developed acute hydrocephalus with sudden drop in conscious level which was managed with emergent placement of external ventricular drain (EVD) from the right Kocher’s point. Conventional cerebral angiography showed delayed filling of a 9.6 mm × 6.8 mm aneurysm arising from the proximal part of the left anterior cerebral artery without a discrete neck (\n). Left ACA complex was not visualized except the aneurysmal sac. Both the distal anterior cerebral complex i.e. A2 segments were filled via the right anterior cerebral artery (\n). Left pterional craniotomy and trapping of the aneurysm was performed without any intraoperative complications. H complex was redefined and A1 was found to be blind which itself was a culprit for repeated rupture. Post-operatively the child remained irritable whenever the EVD drainage was clamped off and repeated CT scans revealed persistent hydrocephalus. She was therefore managed with a right ventriculo-peritoneal shunt. On the day of discharge, 23 days after admission, she was playful with grade 4+ power of right sided limbs which became normal in the 2 week follow up period. The child made excellent recovery during 1 month of clinical follow up with no focal neurological deficit and remained asymptomatic. Angiographic follow up after 3 months showed complete obliteration of the aneurysm (\n;\n). |
A 53-year-old Japanese man was transferred to our hospital with sudden onset of severe headache and disturbance of consciousness. On admission, his consciousness level was E4V5M6 on the Glasgow Coma Scale and he had no neurological deficits. Head computed tomography (CT) revealed a thick SAH with Sylvian hematoma (Fisher Group 4), and CT angiography (CTA) revealed a fusiform aneurysm of the right A1 segment of the ACA and a saccular aneurysm of the left vertebral artery (VA) []. The right A1 segment of the ACA was identified as the site of rupture based on the distribution of the hematoma, and the patient underwent right frontotemporal craniotomy. Intraoperatively, the right A1 segment of the ACA was dilated, fusiform, and resembled a blood blister-like aneurysm, indicating arterial dissection. The right A1 segment of the ACA aneurysm was trapped with cisternal drainage and external decompression to prevent involvement of the perforators. Postoperatively, the patient had no neurological deficits; therefore, routine maintenance infusion and pain medication were continued. The patient was not on any blood pressure medication, and his systolic blood pressure was <180 mmHg. However, on day 7 after SAH, he experienced right hemiparesis and aphasia. The left intracranial ICA was not clearly visible on CTA. Digital subtraction angiography showed ICA dissection at the cervical portion with delayed distal flow. Urgent carotid artery stenting (CAS) for symptomatic left ICA dissection was performed. Before the procedure, we administered 200 mg aspirin and 300 mg clopidogrel to prevent thrombotic complications. Complete coverage of the dissected segment of the left ICA was achieved using an 8 mm × 60 mm stent (Protégé RX; Covidien, Irvine, CA). Blood flow in the left ICA improved, and it was checked for restenosis and acute in-stent thrombosis. The patient’s symptoms disappeared postoperatively. Postoperative magnetic resonance imaging showed left cerebral infarction in the anterior watershed area; however, symptomatic cerebral vasospasm and secondary hydrocephalus did not occur.\nLaboratory results showed that the C-reactive protein level was not elevated, and immunological tests for antineutrophil cytoplasmic antibody and antinuclear antibody were negative. Abdominal CTA showed dissecting aneurysms of the celiac and superior mesenteric arteries []. SAM was strongly suspected because of the lack of features characteristic of other diseases and radiographic findings of multiple nonconsecutive dissecting lesions.\nThe patient underwent cranioplasty, and on day 46 after SAH, he was discharged with no neurological deficits. At present, he undergoes regular follow-up imaging to monitor the left VA, celiac artery, and superior mesenteric artery aneurysms. |
A 70-year-old woman with a past medical history of hypothyroidism, coronary artery disease, cerebrovascular accident with right-sided weakness, and hypertension presented to the emergency department for altered mental status. Earlier that day, she had an appointment for a screening colonoscopy with her gastroenterologist. During the procedure, she was given propofol at 50 mg for sedation. The colonoscopy was complicated by stool in the rectum, and it was subsequently aborted. It was noted that her mental status was not improving after cessation of propofol, and thus the patient was sent to the emergency department. Vitals on arrival were notable for tachycardia at 120 bpm, temperature of 96.8°F (36°C), and blood pressure of 146/83 mm Hg. On examination, the patient was awake with her eyes open, but she was not responding to verbal stimuli. She reacted verbally and withdrew her extremities in response to pain, and her extremities were held in flexion. She scored an 11 on the NIH Stroke Scale. An ECG in the emergency department demonstrated normal sinus rhythm and a right bundle branch block. Laboratory studies on arrival revealed hyponatremia, hypokalemia, an elevated blood urea nitrogen (BUN) and creatinine, elevated glucose (298), elevated lactic acid (3.1), and an elevated thyroid stimulating hormone (TSH) at 175.0 mIU/mL. A CT of the head revealed no overt hemorrhage. An echocardiogram and magnetic resonance angiography study were performed to rule out a possible ischemic event, both of which resulted in no significant abnormalities. The patient was given an electroencephalogram (EEG), during which she developed left arm myoclonus. The EEG showed paroxysmal bursts of discharges suspect for complex partial seizures and wave complexes near the end of recording, suggesting nonconvulsive status epilepticus (). The patient's son reported that she has no previous history of seizure activity. The patient received lorazepam 3 mg and placed on levetiracetam 1,500 mg and valproate 500 mg.\nThe patient was transferred to the intensive care unit and intubated the following day for airway protection after initiation of a midazolam drip. Due to the uncertain nature of her condition, we examined a broader differential diagnosis before we were confident with myxedema coma. The consulting endocrinologist initially began treatment with levothyroxine 25 mcg IV and hydrocortisone 100 mg three times daily. The midazolam drip was discontinued to assess the mental status of the patient, but the patient remained unarousable despite not being on any sedation medication. The nonconvulsive status epilepticus was treated with fosphenytoin 500 mg every 12 hours, lacosamide 200 mg twice a day, and levetiracetam 2000 mg twice a day. Approximately one week later, the patient's status epilepticus terminated and was noted with EEG. To assess for adrenal gland functionality, ACTH and AM cortisol levels were measured. The patient's ACTH level was found to be 49.9 pg/mL and her AM cortisol level was noted at 27.1 mcg/dL, not suggestive of any adrenal gland dysfunction. The patient remained intubated in the ICU for two weeks in a comatose state with occasional left sided myoclonus and bilateral upper and lower extremity nonpitting edema. She was unresponsive to verbal or painful stimuli. The patient's TSH and free thyroxine (T4) levels as measured in the ICU initially trended down but began to rise again after two weeks ().\nThe diagnosis of myxedema coma was made based on the patient's clinical presentation and a score of 65 on the diagnostic scoring system published in 2014 by Popoveniuc et al. []. The typical characteristics of myxedema coma, including bradycardia, hypotension, and hypothermia, were absent in this case. However, a score of >60 using these criteria is reported to be diagnostic of myxedema coma with a sensitivity of 100%. The patient's dose of levothyroxine was increased to 50 mcg IV, 25 mcg IM, and 25 mcg of liothyronine (T3) in the ICU and subsequently levothyroxine was increased to 50 mcg bid IV and 50 mcg of liothyronine due to the later increase in the patient's TSH levels.\nA repeat CT of the head was performed after 14 days in the ICU, which revealed a large, nonhemorrhagic middle cerebral artery (MCA) infarct that was not present on admission (). On the same hospital day, the patient became arousable, and she was able to follow basic verbal commands. She had marked weakness of the left lower extremity and absent motor function of the left upper extremity. A tracheostomy was performed due to depressed respiratory function and inability to wean off ventilator support. The patient was scheduled to undergo a percutaneous endoscopic gastrostomy (PEG) tube insertion and subsequent transfer to a long-term rehabilitation facility to help improve her motor function as well as her respiratory function. |
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