A 28 year-old Turkish male, weighing 76 kg, was scheduled for unilateral varicocelectomy and hydrocelectomy under general anesthesia. The patient did not have an operation previously. Induction of anesthesia was achieved with 200 mg of propofol. Tracheal intubation was achieved with 15 mg of mivacurium. Isoflurane was used as the general anesthetic inhalation agent. The operation was completed within 30 minutes. After the surgery, the inhalation agent was discontinued and the patient received 100% oxygen. All vital signs were stable, oxygen saturation remained 100%, but PNS produced zero twitches. After an additional 10 minutes, there was suspicion of a PChE deficiency. Three milligrams of midazolam was administered intravenously for its sedative and amnestic effects. Fifty-five minutes later, spontaneous muscle twitching was noted. One hour and five minutes after the initial use of mivacurium, the patient had regained sufficient motor function to meet extubation requirements. Plasma cholinesterase was found to have low activity (961 IU/L). The patient was transferred to a hospital ward for the evening and discharged two days later.
*(Monitoring included electrocardiography (ECG), noninvasive arterial blood pressure (NIBP), oxygen saturation (SpO2), capnography (ETCO2), and peripheral nerve stimulator monitoring applied over the ulnar nerve in all four patients)


A 74-year-old white British lady attended the Accident and Emergency department after having fallen from a low height at home. She complained of pain around the right shoulder and had bruising extending from the shoulder to the elbow. There was no gross deformity of the shoulder. The fingers were cold to the touch with a delayed capillary refill. The nail beds were cyanosed. Further examination of the right upper limb revealed loss of pulsations in the brachial and radial arteries with preservation of sensations in the hand. A Doppler ultrasound also failed to detect distal pulsations. X-rays of the shoulder showed a minimally displaced fracture of the surgical neck of the humerus (Figure , Figure ). Vascular surgeons were consulted immediately and an angiogram performed showed kinking and compression of the axillary artery at the fracture site (Figure ) and diminished blood flow distal to the axillary artery (Figure ). However, the patient spontaneously recovered her pulse before any orthopaedic intervention was made; it was deemed unnecessary to fix the fracture because of its minimally displaced nature. The vascular surgeons also decided to treat the arterial injury conservatively.


A 37-year-old Hispanic male presented to the Loyola University Medical Center Burn Unit after being transferred from an outside hospital with concern for necrotizing fasciitis. This man suffered from chronic, painless lymphedema of his right lower extremity for the past 10 years. His lymphedema had accelerated in recent months, and his leg started to accumulate numerous nodules and verrucae. He began having intense pain in his affected leg and swelling in his scrotum. He reported having fevers, chills, diarrhea, and persistent nausea and vomiting for three days prior to being brought to an outside hospital. He presented to their emergency room delirious, hypotensive, febrile, tachycardic, and in respiratory distress meeting the requirements for systemic inflammatory response syndrome. Erythema encompassed his entire right leg and spread across his genitalia and to his left groin. Concern for Fournier's gangrene brought him to the OR for an exploratory scrotal operation. Fournier's was ruled out, and his incision was left open to heal by secondary intention. His condition continued to worsen, and was subsequently transferred to Loyola intubated and maintained on continuous norepinephrine.
On initial exam, he was hypotensive at 94/32 mmHg, tachycardic at 132 beats/min, saturating 95% O2 on SIMV, and with a fever of 40.1°C. Arterial blood gasses demonstrated an anion gap metabolic acidosis. His leg was erythematous and hot to the touch, with blistering and pustular exudates. His scrotum and penis were markedly swollen and erythematous, and serous drainage came from his midline scrotal incision (Figures , , ).
The working diagnosis was cellulitis superimposed on elephantiasis that led to a state of septic shock. His wounds were managed as per partial thickness burns care with twice daily debridement and application of silvadene and kerlix. His antibiotic regimen began with high dose penicillin drip, ciprofloxacin, and clindamycin for broad base coverage. His coverage was altered as cultures returned from both the outside hospital and Loyola. His leg wound cultures grew Proteus (pan-sensitive), MSSA, coagulase-negative Staphylococcus, and Candida parapsilosis. His scrotum wound cultures grew Group G Streptococcus, Peptostreptococcus magnus, and anaerobic gram negative rods that were not able to be identified (Bacteroides fragilis was excluded). His blood, urine, and BAL cultures had no growth. A microfilariae test for bloodborne parasites was negative. Given the anaerobes and yeast involved, his antibiotic regimen was changed to fluconazole, metronidazole, and ampicillin/sulbactam. He spiked fevers through the first week of his hospital stay, but this resolved and the erythema on his leg gradually improved with continual systemic and topical antibiotics.
He was maintained on norepinephrine for the first two days to keep his mean arterial pressure above 60 mmHg, then was successfully weaned and hemodynamically stable for the remainder of his hospitalization. The patient was successfully extubated on hospital day 7, and began breathing on room air with good oxygen saturation on day 8. His case was complicated by acute renal insufficiency. He presented oliguric with rising BUN and Creatinine. Fractional excretion of urinary sodium was 10%, and gross blood and small protein was seen in his urinalysis all factors leading to a diagnosis of acute tubular necrosis secondary to ischemia suffered in septic shock. His acidosis was treated with serial amps of bicarbonate plus maintenance fluids of 1 liter 5% dextrose with 80 ml Sodium Acetate. While his acidosis steadily resolved and his urine output rose, his BUN and Creatinine continue to rise reaching a level of 117 and 8.27, respectively. He received dialysis on Loyola hospital day 4, and received 3 additional dialysis treatments over the next 7 days. After these treatments his electrolyte levels stabilized, and he began having large urine output indicative of post-ATN diuresis.
Additional therapy included nutritional support with 1/2 strength Novasource renal tube feeds at 100 cc/hr, glucose control with insulin lispro sliding scale, and physical therapy. He was malnourished at presentation (Albumin 1.4 gm/dl, Transferrin 99 mg/dl), but improved with continual tube feeds and general diet. The patient was discharged on hospital day 16 with his cellulitis resolved, renal function and electrolyte levels normalized, ambulating independently with physical therapy, and tolerating a general diet. His lymphedema had actually improved somewhat since admission, but he maintained significant edema in the extremity. He subsequently followed up in the burn clinic 2 weeks later without any signs of infection but still with difficulty ambulating secondary to his condition.


As an outpatient female patient with 20 years old was admitted to our university endocrinology clinic with the complaining of thinning in her both wrists in 1997. In her past medical history, any disorder related to current disease was present in her parents. She had two brothers. One of her two brothers (second case) had DM, cataracts, genital hypoplasia, the other was normal. On her first examination, she had a short stature (143 cm) and her extremities were thin. Her nose had a bird-like appearance. After menarche she had menstruation regularly for 3 years but later on it was interrupted. She had bilateral cataracts. An abdominal ultrasonography showed genital hypoplasia. She had osteopenia revealed by DEXA (T-score: −1,55). FSH, LH, and estradiol levels were higher than normal ranges. She had gray hair. By 2004, an examination due to high-pitched voice revealed that right vocal cord was paralytic, and left vocal cord movement was normal. By 2007, a clinical evaluation disclosed that hepatosteatosis and grade 1 renal parenchymal disease. By 2008, she had an operation due to urethral obstruction and difficulty in voiding.


A 49-year-old Caucasian man developed a cough in early 2004. A chest X-ray revealed bilateral hilar lymphadenopathy, confirmed by thoracic computed tomography (CT) scan. He had no other symptoms. A diagnosis of sarcoidosis was considered, but his symptoms were thought insufficient to warrant treatment.
In July 2004, he developed numbness and pain behind the right knee which gradually spread to the lower back, right buttock and posterior thigh. Upon examination he had reduced sensation over the lateral border of the right foot, an absent right ankle tendon reflex and a positive Lasègue's sign at 70°. He also had a dusky discolouration of the skin of the right foot.
He was admitted to our hospital in September 2004 because of progressive worsening of the symptoms. Lumbrosacral spinal magnetic resonance imaging (MRI) showed an increased heterogeneous signal within the S1 nerve root and of the nerve root ganglion on T2 images, thought to be due to oedema, with right piriformis wasting. His cerebrospinal fluid (CSF) contained no white cells, 0.52 g/l protein and 0.36 g/l glucose. Nerve conduction studies and an electromyogram (EMG) revealed abnormalities in the S1 segment, consistent with an S1 radiculopathy. Serum angiotensin converting enzyme (sACE) was persistently normal but an isotope-labelled gallium scan showed increased bilateral lung hilar and lachrymal gland uptake. He developed skin nodules on his right thigh which, when biopsied, were confirmed as erythema nodosum. The diagnosis of sarcoidosis was considered overwhelmingly likely, and in the absence of compression, the involvement of the S1 root was thought most likely due to neurosarcoidosis. He started treatment with prednisone 30 mg/day but his pain persisted; intravenous steroids and then local steroid nerve root injection was tried with temporary benefit. In April 2005, he started methotrexate (up to 12.5 mg per week) because of persistent pain and the need to lower his steroid dose because of his elevated glucose levels.
In November 2005, he developed left peri-orbital and hemicranial headache, followed by diplopia on left gaze. He was found to have a partial left sixth nerve palsy and was re-admitted. MRI showed thickening and gadolinium enhancement in the left cavernous sinus with no parenchymal change. Repeat gallium scanning showed normal lung hilar and lachrymal gland uptake. Serum rheumatoid factor, plasma viscosity, C-reactive protein, urea and electrolytes, liver function, clotting, auto-immune profile, protein electrophoresis, acetylcholine receptor antibodies, anti-neuronal antibodies, anti-thyroid antibodies, creatine kinase and ACE were all normal. He was treated with a three-day course of intravenous methylprednisone and experienced significant improvement.
The following month, he developed headache and further diplopia and he was found to have a painful pupil-sparing left third nerve palsy. His CSF was again entirely normal, including negative oligoclonal band assay. Brain and orbit MRI scanning were normal. EMG and nerve conduction studies suggested improvement of S1 radiculopathy. He also complained of left facial pain with tearing of the left eye. There was a patchy decrease in sensation on the left side of the face and scalp. Corneal reflex was diminished. Blink reflex and facial nerve conduction studies showed an afferent defect on the left suggesting a left trigeminal ophthalmic division neuropathy. His prednisone was increased to 60 mg/day, and cyclophosphamide was started instead of methotrexate. However, after reducing his steroids to 40 mg/day, severe facial pain recurred.
In March 2006, he developed numbness and tingling on the left side of his face, and was found to have a left maxillary ophthalmic division Vth neuropathy. A month later he developed a lower motor neuron left seventh nerve palsy and numbness in the right shoulder and in the left thorax. His CSF was again normal and/or negative including cytological study, acid-fast bacilli staining, and fungal and Mycobacterium tuberculosis cultures and sACE was also still normal. His blood count showed mild lymphopenia and macrocytosis. Lactate dehydrogenase was normal. Borrelia, syphilis, cytomegalovirus, HIV and human T-lymphotropic virus Type 1 serology were negative. An ophthalmological examination was normal with no signs of granulomata.
At this stage, new neurological symptoms developed while the patient was on treatment with steroids and cyclophosphamide. Because no diagnosis emerged other than sarcoidosis, alternative immunosuppressive therapy was administered. Intravenous immunoglobulins, however, had no impact. Infliximab was added in June 2006. Despite this, the patient's right shoulder became weak, his headache persisted and he also developed unsteadiness of gait and significant weight loss. He now had bilateral seventh nerve palsies with weakness of the left palate, right serratus anterior, right triceps, left triceps and left finger abductors. All the upper limb deep tendon jerks were absent. A left vocal cord palsy was noted. EMG and nerve conduction studies showed abnormalities compatible with a pre-ganglionic lesion at C5 and C6 level (right), but no generalised neuropathy. Repeat brain MRI scanning showed enhancement of the fifth, sixth, seventh and eighth cranial nerves (Figure ). Spine MRI scanning was normal. MRI scanning of the upper brachial plexus was normal. A CT scan of the pelvis and abdomen were normal. A whole body fluorodeoxyglucose-positron emission tomography (FDG-PET) scan was normal. Urine thallium screening and lead level were negative. Upper gastrointestinal endoscopy was normal.
The patient then developed three subcutaneous nodules, one in the left outer inferior breast quadrant, without lymphadenopathy. Biopsy of the nodules showed lymphomatous change, and the final diagnosis was diffuse large B-cell lymphoma involving cranial and peripheral nerves (neurolymphomatosis). Bone marrow was normal.
He was transferred to the Oncology department and treated with chemotherapy including methotrexate. After chemotherapy he received total body irradiation and a bone marrow stem cell transplant. There was no significant improvement in his neurological condition.


A 65 year-old male patient, current smoker (50 pack years) presented with complaints of dry cough and breathlessness for the past two months. He was also on treatment for hypertension for the last 3 years. Physical examination revealed no pallor, clubbing or lymphadenopathy. Examination of the respiratory system was not remarkable. The complete blood counts, electrocardiograph, and liver and kidney function tests (biochemistry) were within normal limits. The chest radiograph postero-anterior (PA) view taken one month apart showed a progressing mass lesion in the left paratracheal and parahilar region. Contrast enhanced computed tomography scan of the chest revealed a mass lesion with peripheral enhancement in the left hilum compressing the left main bronchus and encroaching on the wall of the descending aorta (). Subcarnial lymph nodes were enlarged. Spirometry showed moderately severe airflow limitation with FEV1/FVC of 68%, forced expiratory volume in one second (FEV1) of 56% predicted and forced vital capacity (FVC) of 67% predicted. Change after bronchodilator was not significant. Fibreoptic bronchoscopy under local anesthesia revealed extramural compression superomedially in the left main bronchus. A smooth surfaced growth was seen just beyond the compression. The overlying mucosa was hyperemic and the growth bled on touch. Biopsy was taken.
Bronchial aspirate did not show any atypical cells on microscopic examination However, post-bronchoscopy sputum showed a cluster of small atypical cells with dark blue hyperchromatic nuclei, nuclear overlapping and scant cytoplasm. Occasional singly arranged atypical keratinized squamous cells having irregular, angulated cytoplasmic margins and pyknotic nuclei were also seen (). The picture suggested the presence of two malignant cell populations — small cell and squamous.
Histopathologic examination of the transbronchial lung biopsy specimen showed two cell populations with atypia (). Superficial well-differentiated squamous cell carcinoma component with squamous epithelial pearl formation was identified. In deeper tissue an additional component of small cell carcinoma comprising of lymphoblast-like cells with large hyperchromatic nuclei, nuclear pleomorphism, nuclear overlap, increased atypical mitosis and scant amount of indistinct cytoplasm was seen. The alternative diagnosis for this component was Lymphoma.
To confirm the diagnosis, immunohistochemistry was performed using a panel of antibodies (Dako, Carpinteria, CA, USA) to Chromogranin A, Synaptophysin and Common Leucocyte Antigen CD-45 (Clone: 2B11 & PD7/26). The slides were stained using streptavidin – biotin kit (DakoCytomation LSAB2 system -HRP). Positivity for Synaptophysin and CLA negativity were diagnostic for small cell carcinoma and ruled out the possibility of lymphoma ().
A diagnosis of combined small cell lung carcinoma was thus made. The patient was explained the prognosis and referred for chemotherapy as surgical resection was ruled out due to the central location of the tumour and its close proximity to the aorta.


A 72-year-old woman presented to emergency with recurrent abdominal pain and vomiting from 4 months and acute abdomen for 1 week. She complained of non-bilious, non-projectile vomiting containing food particles. Visible peristalsis was appreciated, which appeared aggravated after taking food. She did not complain of any history of trauma or breathlessness. On clinical examination, her vitals were stable as she had been resuscitated in emergency and she was evaluated with radiographs, endoscopy and computed tomographic (CT) examinations. Plain radiograph performed on the day of admission, 1 week from the onset of acute symptoms, showed opacification of the entire lower part of the right hemithorax with significant air lucency within []. The opacification was silhouetting the right cardiac border, confirming its anterior location. The cardia and mediastinum were shifted to the left. The appearance of air lucencies within the lesion confirmed the presence of the bowel in the right hemithorax, which confirmed the diagnosis of diaphgrammatic hernia. Because the patient had also complained of hematemesis in the last week, endoscopy was performed on the same morning, which showed the esophagus filled with fluid residue. The gastric lumen appeared twisted at around 40 cm from the incisor level. The scope was negotiated beyond this point with mild difficulty and a lateral radiograph of the chest was taken. It showed the scope to enter the air-fluid level within the right hemithorax [], suggesting right diaphragmatic hernia and herniation of the stomach into the right hemithorax. CT scan was performed with oral contrast, rectal contrast and intravenous contrast immediately on the day of admission. CT scan showed herniation of the stomach, pylorus, ascending and transverse colon with the fatty omentum in the right hemithorax []. The oral contrast had predominantly accumulated in the bowel loop in the right hemithorax with very little contrast in the distal small bowel. The transverse colon opacified with rectal contrast was also noted in the right hemithorax on the CT scan of the chest and upper abdomen. On surgery, a small defect between the sternal and costal fibers of the diaphragm was noted in a paramedian location. The herniated contents were noted in the right hemithorax with a partially collapsed right lung []. The volvulus of the stomach that was suspected pre-operatively was absent. The gastric outlet obstruction was due to the herniation of the distal stomach and pylorus into the narrow defect. Because there were adhesions to be released, a right thoracolaporotomy was performed. The hernia was reduced after releasing the adhesions and the diaphragmatic defect was repaired.


A 16-year-old girl presented with acute onset left sided hemiparesis without loss of consciousness. General physical examination was unremarkable. Neurological examination revealed findings consistent with left-sided hemiparesis. A similar episode occurred three years back. No precipitating factors such as chronic drug intake were present. Family history was negative for vascular events or other predisposing factors for stroke.
CT head [] revealed a wedge shaped acute infarct in right middle cerebral artery territory alongwith areas of enchephalomalacia and gliosis in right fronto-temporal and temporo-parietal lobe and in paraventricular white matter with ex-vacuo-dilation of frontal horn of lateral ventricle, suggestive of chronic infarct of middle cerebral artery territory. Magnetic resonance angiography (MRA) showed narrow lumen and caliber of right middle cerebral artery alongwith absent flow in right supraclinoid internal carotid artery. Routine hematological examination along with lipid profile, coagulation profile, echocardiography, and duplex scanning were unremarkable. Vasculitis profile was negative. Cerebrospinal fluid examination did not reveal any abnormality.
Workup for thrombophilias revealed reduced protein S function (15% of normal) alongwith protein C; whereas, antithrombin III, anticardiolipin antibodies, and lupus anticoagulant were within normal limits. A diagnosis of protein S deficiency was kept and the patient was managed with intravenous heparin followed by oral anticoagulants. Neurological functions improved and patient was discharged on oral anticoagulants. Repeat thrombophilic profile after three months revealed protein S functional activity 42% of the normal with patient showing remarkable recovery.


A 17-day-old male baby was brought to the out-patient section of the pediatrics department, with a history of refusal to feed, sluggish activity and yellow discoloration of the body. There was no history of Rh-incompatibility. On physical examination, the neonate appeared ill and was icteric. His laboratory investigations were serum bilirubin - 17.6 mg%, serum calcium - 8.2 mg%, blood sugar (Random) - 72 mg%, haemoglobin-16.8 gm%, TLC - 5,800/cmm and DLC was polymorphs 82% and lymphocyte 18%. Blood culture was positive for MRSA. He was treated as a case of septicemia with Icterus neonatorum, in the neonatal intensive care unit (NICU).


A 5-day-old preterm female baby was brought to the out-patients section of the pediatric department with a history of refusal to feed, sluggish activity, yellow discoloration of the body, respiratory distress and seizure. There was no history of Rh-incompati-bility. On physical examination, the neonate appeared ill and icteric, with hypothermia, weak cry, tachycardia, tachypnea and poor motor activity. Her laboratory investigations were blood sugar (Random) - 112 mg%, serum bilirubin - 11.3 mg%, and serum calcium-3.9 mg%. Blood culture was positive for MRSA. She was treated in the NICU as a case of birth asphyxia with septicemia with Icterus neonatorum.
Blood culture and sensitivity was done as per standard protocol, in the Department of Microbiology, M.L.N. Medical College, Allahabad. The samples were collected in Brain heart infusion broth and periodically sub-cultured on MacConkey agar and blood agar. The colonies that grew were identified to the species level by a battery of biochemical tests. These isolates were tested by Kirby-Bauer disc diffusion method, against a panel of relevant antibiotics, using commercially available antibiotic discs.[] The antibiotics tested included ampicillin, amoxycillin-clavulanic acid, amikacin, cephalexin, cephazolin, co-trimoxazole, clindamycin, ciprofloxacin, erythromycin, gentamycin, linezolid, oxacillin, pristinamycin (dalfopristin/quinupristin), tetracycline, teicoplanin and vancomycin. The MIC of pristinamycin was determined by E-test strips (Hicomb strips from Hi Media Laboratories, Pvt. Ltd., India).


A 6 year old girl presented at a local hospital with an acute onset of abdominal pain, nausea and bilious vomiting. A history of constipation, recurrent vomiting associated with high fever in the preceding 12 months was elicited. Clinical examination was reported to be normal at the time. Complete blood counts revealed haemoglobin (Hb) 94 g/l, WBC 18.4 × 09/l (Absolute PMN 15.6 × 109/l, Lymph 2.0 × 109 /l) and platelets 663 × 109 /l. Her ESR was elevated at 54 mm/hr [1-15 mm/hr) and CRP 84 mg/l (<10 mg/l). Ultrasonography of the abdomen revealed diffuse, mesenteric lymphadenopathy. A barium meal with follow through suggested a small bowel obstruction. A laparotomy showed extensive intraperitoneal adhesions, mesenteric lymphadenopathy and a tight jejunal band with obstruction. The adhesions were divided, the obstruction was relieved and the patient's bowel function recovered steadily. Tissue cultures were negative. Histopathological analysis of the peritoneal tissue revealed fibro-fatty tissue with extensive fibrosis throughout, infiltrated by many scattered lymphocytes, plasma cells and occasional neutrophils but no malignant cells. The single lymph node examined revealed dilated sub-capsular sinuses with an expanded para-cortex with mixed histiocytes and occasional immunoblast with no granulomas, Reed Sternberg cells or any evidence of lymphoma. Ziehl-Neelsen stains were negative for tuberculosis organisms as was subsequent culture. The clinical condition improved slowly over the subsequent 2 weeks and she was discharged on oral iron supplements. No evidence of mono-clonality was seen on flow cytometry for immuno-phenotyping of lymph node tissue.
Three months later, she moved interstate and presented to the gastroenterology clinic at our hospital with continued weight loss and an exacerbation of abdominal pain and vomiting of fecal matter. Upper and lower endoscopies were normal. MRI of the abdomen revealed apparent thickening of a loop of proximal jejunum in the left mid abdomen with enlarged mesenteric lymph nodes. CT scan revealed evidence of a subtle attenuation in the mesentery with diffuse lymphadenopathy (Figure ). The symptoms of obstruction settled with conservative management. At one month follow up, her haemoglobin remained depressed, 87 g/l, WBC 15.0 × 109/l with thrombocytosis. She had lost 7 kgs in weight (from the first presentation), had recurrent abdominal pain and required a second admission for a partial obstruction which resolved with conservative management.
A rheumatology consultation was made. Review of the case revealed multiple episodes of subacute small bowel obstruction with chronic anaemia, weight loss, and a chronic inflammatory process with fibrosis on mesenteric biopsy and elevated acute phase reactants. Further investigations revealed a borderline positive ANA of 1:40 (speckled pattern), a negative dSDNA and rheumatoid factor (RF). Total serum proteins were 82 g/l, albumin 40 g/l (32-48 g/l), globulins 42 g/l (23-35 g/l), and serum IgG 15.7 g/l (6-12.3 g/L) indicating mild hypergammaglobulinemia. IgG subclasses IgG1 11.1(4.0-10.8 g/L), IgG2 4.3 (0.8-4.1 g/L), IgG 30.4 (0.1-1.4 g/L), IgG4 1.47 (<1.9 g/L) were unremarkable. An abdominal angiogram was done to screen for major vessel vasculitis such as polyarteritis nodosa, and was negative.
A diagnosis of idiopathic sclerosing mesenteritis was made and the child was treated with 3 daily pulses of methyl prednisolone therapy repeated monthly for 3 months. She made a remarkable and rapid clinical recovery with marked reduction in fatigue, abdominal pain and resolution of fever within 2 weeks. Four weeks into treatment, laboratory investigations revealed significant improvement; Hb 118 g/l, WBC 10.1 × 109/l platelets 490 × 109/l and ESR 3 mm/hr. Subcutaneous methotrexate (10 mg/m2 ), total dose 10 mg once a week was started for long term immunomodulation and steroid sparing effects. At 6 months follow - up from the onset of treatment, she had gained 3 kgs, was completely asymptomatic and her laboratory investigations remained normal. Her repeat CT scan this time had revealed resolution of the lymphadenopathy with normal mesentery (Figure ). Currently at 18 months follow up, she is under regular 3 monthly follow-up, off steroids and only on subcutaneous methotrexate at (10 mg/m2) now 12.5 mg. Figure illustrates the impressive and sustained response to treatment evidenced by her haemoglobin and ESR results over time.


A 36-year-old, previously healthy Hispanic female with no significant past medical history and no prior history of hypertension presented to the emergency room with sharp retrosternal chest pain radiating to the back. She was 165 centimeter tall and weighed 70 kilogram. She was a housewife and mother of two living children. She smoked one pack of cigarettes a day for more than ten years. For 3 months prior to presentation, she had been taking 18-20 tablets of caffeine-based weight loss pills daily, which was higher than the recommended daily dose. Each tablet contains 60-100 mg of caffeine and she was taking 1080 mg to 2000 mg of caffeine daily. She denied the use of other medications, stimulants, herbal, alcohol and illicit drugs. She said that she had lost approximately 20-25 kilograms in last 3 months. No family history of hypertension, diabetes or coronary artery disease. She denied palpitation, chest pain, insomnia, headache, abdominal pain or increase in urination.
On presentation, her blood pressure was found to be 220/110 mm Hg in right arm and 230/118 mm Hg in left arm. Her initial work-up included an electrocardiogram which showed T-wave inversion in leads III, aVF and V6. A complete blood count showed hemoglobin 12.9 g/dl (normal: 12.0-16.0 g/dl), white blood cell count 15.1 k/ul (normal: 4.0-11.0 k/ul) and platelet count 231 k/ul (normal: 150-450 k/ul). Serum basic metabolic panel showed sodium 132 mmol/L (normal: 135-145 mmol/L), potassium 3.3 mmol/L (normal: 3.5-5.3 mmol/L), chloride 103 mmol/L (normal: 95/105 mmol/L), bicarbonate 23 mmol/L (normal: 22-31 mmol/L), creatinine 1.3 mg/dl (normal: 0.6-1.3 mg/dl), calcium 8.3 mg/dl (normal: 8.6-10.3 mg/dl), and anion gap 6 mmol/L (normal: 7-17 mmol/L). Liver function test showed normal aspartate transaminase (15 U/L; normal: 0-55 U/L), alanine transaminase (23 U/L; normal: <45 U/L), alkaline phosphatase (55 U/L; normal: 34-104 U/L) and albumin (3.5 g/dl,normal:3.0-5.1 g/dl). Urinalysis showed only elevated specific gravity of 1.031(normal: 1.005-1.03). There were no crystals, protein and glucose in the urinalysis. Troponin I level was less than 0.030 ng/ml (normal: 0.0-0.049 ng/ml). Urine and serum toxicology screens were negative for cocaine, amphetamine, marijuana, benzodiazepines, barbiturates and phencyclidine. A chest X-ray did not show any infiltrate, consolidation, effusion or mediastinal widening. A computed tomography aortogram showed aortic dissection involving the posterior aspect of the arch extending into the common iliac arteries and right proximal external iliac artery. The dissection extended into the left subclavian and axillary arteries. There was narrowing of the origin of the right renal artery due to dissection (Stanford type B dissection) (Figure ,Figure , & Figure .)
She was started on intravenous esmolol and nitroprusside for blood pressure control and was admitted to the medical intensive care unit. During hospitalization, her blood pressure remained well controlled with amlodipine. All other investigations for a secondary cause of hypertension were negative.Patient was discharged home and upon follow-up 2 week later, her blood pressure remained well controlled with low dose amlodipine.


This case is a 34-year-old Caucasian female nonsmoker who presented with recurrent pneumonias of her right middle lobe. She experienced recurrent fevers, cough, and occasional pleuritic pain for approximately one year. She had no gynecologic issues and her pelvic exam was normal. Chest radiograph showed a bandlike density in the area of the right middle lobe. Computerized tomography (CT) of the chest showed pleural thickening of the inferior half of the right middle fissure with adjacent subsegmental atelectasis of the lateral segment of the right middle lobe with some air bronchograms and bronchiectasis seen within it (Figure ). Bronchoscopy with biopsies revealed an obstructing lesion of the right middle lobe bronchus with fragments of tumor with interlacing fascicles of smooth muscle cells with spindled nuclei lacking atypia and mitotic activity consistent with a primary endobronchial leiomyoma.
She was taken to the operating room for a right middle lobectomy that required opening of the right middle lobe bronchus with hand-sewn bronchial closure and mediastinal lymph node sampling. The resected specimen contained a well-circumscribed mass contained within the bronchus attached by a stalk, deep to the actual bronchial margin. Pathology revealed a leiomyoma with acute and chronic inflammation and interstitial fibrosis in the remaining lung tissue (Figure ). The tumor was strongly positive for smooth muscle actin (SMA) immunostain and negative for CD117 and S100 immunostains. The lymph nodes were all described as reactive. On 6-month follow-up, the patient's symptoms have resolved and she was doing well. CT of the chest at 3 months post-resection shows no residual disease.


A 57-year-old French Canadian male presented with gross hematuria and worsening lower urinary tract symptoms. He was known for a history of recurrent superficial TCC refractory to two induction courses of BCG. He had a 35-pack year history of smoking and no family history of genitourinary malignancy. Cystoscopy revealed abnormally 'edematous appearing' mucosa in the posterior wall of the bladder. Urine sent for cytology was negative. Metastatic workup including CT chest/abdomen/pelvis and colonoscopy were negative and was subsequently scheduled for a radical cystectomy. Exploratory laparotomy revealed multiple metastatic lesions involving the GI tract and mesentery. Frozen sections were sent which were consistent with metastatic poorly differentiated TCC. Since patient was significantly symptomatic, he underwent a palliative cystectomy with ileal conduit diversion. Final pathology revealed muscle-invasive high-grade urothelial carcinoma of the bladder with plasmacytoid features penetrating through the entire bladder wall and into the serosa (Figure ). Immunostaining was positive for cytokeratin, confirming plasmacytoid urothelial carcinoma (Figure ). The metastatic lesions were also positive for plasmacytoid features (Figure ). The pathological stage of urothelial cancer was pT3a, N0, M1. The patient had an uneventful postoperative recovery. He was discharged 10 days post operatively. He received postoperative systemic chemotherapy (gemcitabine/cisplatin); however, he passed away within 6 months due to rapid progression of disease.


A 72-year-old Caucasian woman presented with squeezing chest pain. The pain had started 30 minutes previously and was associated with nausea, vertigo, shortness of breath and weakness. Her history was significant for hypertension and coronary artery disease. Two years before admission, she had undergone coronary angiography because of angina, which revealed three-vessel coronary disease. The examination was followed by repeated angioplasty with stent implantation. A drug-eluting stent had also been applied. The first intervention had been complicated by a haematoma at the femoral puncture site.
On physical examination, the patient had a blood pressure of 130/60 mmHg and a heart rate of 65 beats per minute. The lung and heart sounds were clear on auscultation. Her weight was 60 kg, her height was 156 cm and her body mass index was 24.65 kg/m2. An electrocardiogram showed normal sinus rhythm without signs of myocardial ischaemia. Laboratory testing was significant for haemoglobin at 11.4 g/dL, blood urea nitrogen at 10.45 mmol/L and creatinin at 131 μmol/L. The creatinin clearance calculated with the Cockroft-Gault equation was 26.47 mL per minute. There was no elevation of cardiac troponin-I and creatine phosphokinase-MB isoenzyme levels.
After admission, her symptoms soon subsided. Repeated electrocardiography did not show signs of ischaemia, and the troponin-I value remained in the normal range. A diagnosis of unstable angina was established. The patient received early conservative treatment with the following pharmacotherapy: 2 × 60 mg enoxaparin subcutaneously, 75 mg clopidogrel, 100 mg aspirin, 50 mg metoprolol, 20 mg atorvastatin, 0.2 mg per hour transdermal nitroglycerin, 80 mg valsartan and 12.5 mg hydrochlorothiazide.
On the fifth day of her hospital stay, an exercise stress test was performed on a bicycle ergometer where she reached 3.9 metabolic equivalent and no ischaemic response occurred. A few hours later, she experienced pain in the left side of her abdomen. On examination, a palpable, growing tender mass was detected in the lower left quadrant. An abdominal ultrasound scan demonstrated a rectus sheath haematoma with a depth of 20 mm and a width of 40 mm. The upper margin of the haematoma was at the umbilicus, the lower reached the pubic bone. As continued haemorrhage was suspected, the patient underwent surgical exploration with haematoma evacuation, and received a blood transfusion. She remained on a reduced dose of enoxaparin (1 × 60 mg). Since she was febrile and showed a decreasing haemoglobin level, on the 9th postoperative day a computed tomography (CT) scan was performed. This showed a recurrence of the rectus sheath haematoma (Figure ) and its extension in the abdominal wall. A second haematoma evacuation was performed. Subsequently, no recurrence of bleeding was observed, but the patient developed an ascending superficial thrombophlebitis on the left lower limb, which was treated with ligature. She was finally discharged in good health.


A 53-year-old man was admitted to our hospital for further evaluation of anemia (haemoglobin concentration 10,6 g/dl, RBC 2.970.000/mm3, HTC 31,4%) associated to positive fecal occult blood test. He had been treated for an autoimmune haemolytic anemia 4 years before, but immuno-haematological tests excluded signs of haemolysis. He also had an autoimmune thyroiditis. He had neither bleeding from rectum nor diarrhea or other gastrointestinal symptoms. Abdominal examination was negative. Rectal exploration did not reveal any pathological sign. A colonoscopy identified the presence of a spherical, sessile, lobulated polyp of the cecum, 4.2 cm in diameter, with a red and not bleeding but partially ulcerated surface (figure ). Because of the absence of a peduncle and its large size, the endoscopist decided to perform a biopsy of the polyp rather than a polypectomy. The histological examination of the specimen revealed the presence of inflammatory granulation tissue with lymphocytic and eosinophil infiltration associated to a fibrous stroma: it was diagnosed as inflammatory fibroid polyp []. Considering the chronic anemia and the polyp's features (absence of a peduncle and size), we decided to perform an ileocecal resection with a latero-lateral ileocolic anastomosis (figure ). The histological examination of the polyp revealed inflammatory granulation tissue and engorged capillaries in the lamina propria, hyperplastic glands with cystic dilatations, proliferation of smooth muscle and multiple erosions on the polyp surface (figure ). There was not superficial fibrin exudation. According to these features [] it was finally diagnosed as inflammatory myoglandular polyp (IMGP). There was also another little polyp next to the ileocecal valve, not revealed at the colonoscopy, 0.8 cm in diameter, diagnosed as tubulovillous adenoma with low grade dysplasia. The patient was discharged from our hospital four days after the operation without any postoperative complications. After two months the patient has good health and anemia was resolved.


An eighty one-year-old lady presented to the emergency department with a right groin swelling for more than two years which had become acutely painful. Clinical diagnosis of a femoral hernia was made. At operation, the sac contained a knuckle of non-viable omentum which was otherwise unremarkable. This was excised and was sent for histopathological examination. The histology revealed a metastatic papillary adenocarcinoma, moderate to poorly differentiated, with a probable ovarian primary. Postoperative CA125 was markedly elevated at 853.2 KU/L (normal value less than 30 KU/L). Subsequent ultrasound scan of her pelvis showed an 84 × 47 × 80 mm solid mass of probable ovarian origin in the pouch of Douglas. The patient recovered well from her surgery and was subsequently referred to the gynaecologists for further management.


A forty-year-old female had a routine excision of a sebaceous cyst from the anterior chest wall, as day case, under local anaesthesia. The specimen was sent for microscopic evaluation and was reported as a sebaceous adenoma. This rare skin neoplasm when combined with a family history of colorectal cancer suggests Muir-Torre syndrome (This is a rare autosomal dominant genodermatosis recognised as a subtype of LynchType II hereditary nonpolyposis colorectal cancer), which increases the risk of developing bowel, uterine and other visceral malignancies [].
As per recommendation by a geneticist she is under long-term follow-up by surgeons and gynaecologists []. As this is an autosomal dominant condition it has implications with regard to screening of her relatives [,].


A 44-year-old Caucasian woman was referred to the ophthalmology unit four hours after sudden painless loss of vision in her right eye. She was in remission following six months of chemotherapy with cyclophosphomide and fludarabine for chronic lymphocytic leukaemia. Two months after her chemotherapy ceased (9 months prior to this presentation) she had severe right sided herpes zoster ophthalmicus with uveitis that was treated with topical steroids. She was a smoker and on hormone replacement therapy.
Her visual acuity was count fingers at one metre in the right eye and 6/6 in the left eye. Pupils were equal and reactive with a right relative afferent pupillary defect. Dilated fundus examination at presentation was essentially normal showing healthy discs with some mild arteriolar attenuation in the right eye. Whilst the history was highly suggestive of a central retinal artery occlusion (CRAO) optic neuritis could not be excluded. Dual therapy was commenced with ocular massage and intravenous acetazolamide and high dose intravenous (IV) methylprednisolone (1 gram daily) and oral famciclovir (500 mg TDS) for a possible underlying vasculitic cause or optic neuritis. She continued the IV steroids for a total of three days and the oral famciclovir for three weeks.
Dilated fundus examination of the right eye the following day demonstrated a cherry red spot and retinal oedema, findings consistent with right CRAO (Figure ). The early and late phase fluorescein angiogram images showed reperfusion of the right central retinal artery with no signs of optic neuritis or retinal vasculitis.
An embolic screen for possible sources of emboli included routine blood tests, carotid doppler ultrasound and a transthoracic echocardiogram. Blood tests were normal with no active leukaemia and erythrocyte sedimentation rate of 19 and c-reactive protein < 3. The carotid doppler ultrasound revealed complete occlusion of the right ICA with a patent left ICA. CT angiogram the same day supported the finding of 100% right ICA occlusion with no signs of calcification and demonstrated a generalized diminished right intracranial circulation (Figure ).
Vascular surgery was not undertaken as the occlusion was total and stable and the right intracranial circulation was well collateralized. At one month follow up she was clinically stable with no visual recovery.


A 20-year-old Caucasian female with diagnosis of CN was referred by the Department of Immunology and Allergology, to the Department of Periodontology of the University of Naples Federico II, Italy. Anamnesis revealed that since the first year of life the patient has been suffering from repeated phases of fever and oral ulcerations, associated with pain and lymphadenopathy. During childhood and adolescence the patient had repeated admissions to the hospital, but CN was diagnosed only in September 2007. The haematological findings (complete laboratory work-up) showed the neutropenic phase recurred approximately every 21 days. The lowest neutrophil count was 0,20 × 103/μL. A light enlargement of submandibular and cervical lymph nodes was revealed by palpation and moreover fever (37,8°C) sinusitis and pharyngitis were recorded during the neutropenic phase. In addition she reported to brush her teeth three times a day but during the neutropenic crisis oral hygiene was hindered by pain due to oropharyngeal ulcerations (Figures , , and ). Full Mouth Plaque Score (FMPS) and Full Mouth Bleeding Score (FMBS), recorded on 6 sites per tooth, were 66.7% and 64.8%, respectively (). Periodontal examination revealed a generalized increased probing depths (). No furcations were involved and no gingival recessions were recorded. Tooth mobility was not present. An X-ray examination revealed bone loss on interproximal aspect of maxillary and mandibular teeth (). A diagnosis of moderate periodontitis as manifestation of systemic disease was made.
Since September 2007 up till February 2008 the patient received a systemic treatment with Prednisolone 10 mg/die o.s./7 days (Deltacortene, Bruno Farmaceutici) during the neutropenia phases. The above therapy had a minimum effect on severity of oral ulcerations and produced minimal improvement in ANC. At this regard pain and burning determined poor oral hygiene conditions (FMPS = 41.3% and FMBS = 4.2%).
In March 2008, the patient received a new treatment with GCSF administration consisting of subcutaneous injection of Pegfilgrastim 6 mg (Neupopeg, Dompè Biotec S.p.A., Italy) once a month.
Periodontal treatment including oral hygiene instructions, scaling, root planning, and polishing was meticulously carried out once a month when absolute neutrophil count (ANC) was >1500/μL and for this reason prophylactic antibiotic therapy was not prescribed. During neutropenic cycles the patient was advised to rinse her mouth twice a day with 0.2% chlorhexidine gluconate. A very high level of compliance was recorded. A complete laboratory work-up was performed every week. Therapy with G-CSF initially led to a “peak” of WBC and ANC and subsequently to a normalization for few days (). No side effects has been reported by the patient during the treatment and no adverse effects were noted when the counts were greater than 40 × 103/μL. A reduction of general and oral manifestations during the neutropenic cycles was constantly recorded. One week after neutropenic phase periodontal re-evaluation was carried out in order to assess the plaque control and the tissutal response to the treatment.
A considerable improvement of oral hygiene conditions was observed: FMPS and FMBS remained constantly ≤25% and a period general reduction of probing depth (PD ≤ 4 mm) during the successive 6-month follow-up was recorded (Figures and ). Microbiological analysis was performed. Microbiological sampling included selection of the deepest periodontal pocket in each quadrant of the dentition based on the probing depth measurements. Sample sites were isolated with cotton rolls and supragingival plaque was carefully removed with curettes and cotton pallets. Subsequently, two paper points were inserted to the depth of the pocket and left in place for 10 seconds.
The paper points were transferred to a vial to be send to the microbiological laboratory in order to perform Polymerase Chain Reaction (PCR, Lab Oral International, The Netherlands). Microbiological analysis revealed no residual pathogenic subgingival microflora but an elevated level of Fusobacterium nucleatum.


Case 1 was a 54 year old man who presented with respiratory failure and bulbar dysfunction. His first problem was dyspnoea whilst swimming. He was treated with non-invasive ventilation (NIV) from the time of diagnosis throughout his illness. There was no reported family history of neurological disease. On examination at the time of presentation, he had a moderately severe dysarthria and bilateral wasting, fasciculation and weakness of the tongue. He had wasting and proximal fasciculation in the upper and lower limbs, weakness of the ulnar and median innervated intrinsic hand muscles and the hip flexors. The deep tendon reflexes were normal and plantar responses were flexor, consistent with a pure lower motor neuron phenotype. His symptoms progressed rapidly over the course of 15 months, at which stage he died of respiratory failure with relatively preserved limb function.


Case 2 was a 64 year old female who presented with back pain and progressive weakness of the right leg. She underwent L4/5 spinal decompression to no avail and her symptoms continued to progress to affect both legs, with later development of upper limb, bulbar and respiratory muscle weakness. There was no significant family history. On examination she had wasting and predominantly distal weakness in the lower limbs with upper limb fasciculations. The reflexes were normal throughout, and the plantar reflexes were flexor. She died 66 months after symptom onset.


Case 3 was a 49 year old man who initially noticed a deterioration in his ability to play football, due to weakness affecting his left leg. His symptoms progressed steadily. Fifteen months after onset he had wasting and fasciculations in the upper limbs with preserved power. The lower limbs were wasted globally, most severely in the left anterior tibial compartment. Power in hip flexion and ankle dorsiflexion was reduced on the left and the ankle jerks were absent bilaterally. He developed bulbar symptoms 24 months after disease onset. No UMN signs were detectable clinically throughout the disease course. There was no family history of ALS, with healthy parents and 5 siblings, including one with developmental delay described as possible autism. He died of respiratory failure 29 months after his initial symptom onset.


Case 4 was a 73 year old male who presented with dysarthria, dysphagia and clumsiness of the hands. His symptoms progressed rapidly and 12 months after onset he had a wasted, fasciculating tongue, LMN weakness in the upper and lower limbs, depressed reflexes and flexor plantar responses. He died 14 months after disease onset. He had a cousin who had died from ALS several years earlier.


A 51 year-old female was admitted to the Emergency Department with a week's history of lethargy, asthenia, and vomiting. Her medical history did not reveal any pathology or pharmacological treatment.
At the time of admission, she was conscious and her laboratory investigations showed serum creatinine concentration = 3.14 mg/dL and glucose = 1044 mg/dL associated with severe polyuria, polydipsia, and leukocytosis (24 × 103/L). An immediate diagnosis of diabetes was postulated. Physical examination was unremarkable with the exception of a wide subcutaneous emphysema involving the whole abdominal surface and extending up to the lower thorax. A contrast-enhanced chest-abdomen CT scan showed extensive retroperitoneal and extraperitoneal air, with bubbly gas collected in the kidney and under the abdominal right flank muscles. Both the kidneys had a polycystic structure, the right one was larger than the left, and fluid material was detectable in all the cysts [].
As the patient's condition rapidly worsened, an emergency right nephrectomy was performed and broad-spectrum antibiotics (piperacillin-tazobactam) were empirically started. She was admitted immediately after surgery to the intensive care unit (ICU) for septic shock, requiring intensive resuscitation. The patient needed coupled plasma filtration adsorption (CPFA) for four days as well and was extubated after four days; vasopressors were stopped and the dialysis was suspended due to improvement in renal function. Urine and blood cultures were positive for the same E. coli, which was susceptible to all the tested antibiotics. shows the biochemical and hematological parameters monitored during the patient's ICU stay. The patient was transferred to the Nephrology Division on the sixth day and was discharged from the hospital without any further dialysis after 34 days.


A 41-year-old female was admitted to our hospital with shortness of breath for about 24 hours. There was no significant past medical or surgical history except for the fact that she experienced a painful hyperextension of the spine the previous morning, during routine physical exercise. A subsequent chest x-ray showed enlargement of the cardiac silhouette (Figure ).
A transthoracic echocardiogram demonstrated a large pericardial effusion, with right ventricular collapse consistent with cardiac tamponade physiology. An urgent therapeutic pericardiocentesis was performed and 1200 ml of milky fluid was removed and an 8 Fr drain was left in place. The laboratory results of the fluid revealed the following: triglycerides 550 mg/dl, cholesterol 110 mg/dl, total proteins 4.6 g/dl, glucose 85 mg/dl. The diagnosis of chylopericardium was established. Cytology stains and cultures were all unremarkable. Blood tests for rheumatologic, endocrinologic and autoimmune disorders were normal. Tests for bacterial, fungal, mycobacterial and viral infections were also conducted and found negative. Chest, abdomen and brain scans were normal. No evidence of lymphadenopathy was noted. Despite the absence of severe symptomatology concerning the spine injury a magnetic resonance imaging of the thoracic spine was ordered and was consistent with a disruption of the anterior longitudinal ligament and anterior protrusion of the intervertebral disc (Figure ).
A daily output of 350 ml of pericardial fluid led us to start total parenteral nutrition, subcutaneous octreotide and no oral feedings for 7 days. These conservative measures proved to be unsuccessful because the rate of the pericardial drainage did not decrease. The patient also underwent, a bipedal lymphangiography which showed no anatomic abnormalities of the thoracic duct and no leakage. Under these circumstances the patient was addressed for thoracic surgical evaluation. The patient finally underwent a video-assisted supradiaphragmatic mass ligation of the thoracic duct and creation of a pleuropericardial window through a low right mini thoracotomy. Ligation of the thoracic duct together with all the adjacent soft tissue between the esophagus, the azygos vein and the aorta was performed. The patient was recovered uneventfully for both spine injury and tamponade. There has been no recurrence of the pericardial effusion for 12 months.


A 42-year-old Caucasian Finnish woman was scheduled for laparoscopic cholecystectomy due to typical symptoms and radiological findings of gallstones. She was obese (89 kg, BMI = 33), even though she had managed to lose weight by 20 kg six months prior to presentation. She was using sibutramine and oral contraceptives as regular medication.
Surgery was uneventful, but on the second postoperative day, the general state of our patient started to deteriorate, resulting in anuria and difficulty of breathing, admission into the intensive care unit (ICU), endotracheal intubation, and mechanical ventilation. Endoscopic retrograde cholangiopancreatography (ERCP) was performed upon ICU admission because of suspected biliary tract leakage. However, no signs of leakage were observed. Computed tomography (CT) examination revealed fluid around her liver, while her pancreas could not be visualized. Her plasma amylase concentration was elevated, thus confirming the diagnosis of pancreatitis.
Due to decreased renal function, she was commenced on continuous hemodiafiltration therapy on the third day and continued until the 10th postoperative day. Propofol infusion for sedation, supplemented with intravenous oxycodone boluses, was started as part of our hospital's standard therapy in order to facilitate mechanical ventilation and other treatment procedures. Propofol sedation was continued until the 36th postoperative day. Upon the decision of weaning, her attending physician decided to add dexmedetomidine infusion into the sedation regimen 17 days after her surgery.
Weaning was not successful, and tracheostomy was performed on the 18th postoperative day. On the 19th postoperative day, esophagogastroduodenoscopy and explorative laparotomy were performed because the general condition of our patient again started to deteriorate. Hemorrhagic pancreatitis with severe inflammation of her abdominal cavity was discovered. This deterioration prevented further weaning. Our aim was to stop propofol infusion after starting her on dexmedetomidine, but in order to achieve the desired level of sedation (light to moderate, Sedation-Agitation Scale (SAS) levels 3 to 4 []), propofol infusion had to be continued despite the already high dose of dexmedetomidine she was receiving.
Our patient later required yet another laparotomy because of elevated abdominal pressure. This surgery did not reveal any additional findings, but this time her abdominal wall had to be left open, and sedation had to be continued. Vacuum-assisted closure therapy was established on the 22nd and continued until the 34th postoperative day. Weaning was started again on the 36th postoperative day. Clonidine infusion was started on the 40th postoperative day, while the dexmedetomidine infusion was discontinued on the following day, on the 24th day of its administration.
Our patient's abdominal wall was finally closed on the 47th day, and the clonidine infusion was stopped on the 51st postoperative day. The tracheostomy cannula was removed on the 54th day and she was admitted to a surgical ward on the 55th postoperative day for further recovery.
During dexmedetomidine sedation, her plasma albumin level was low (8.4 g/L to 11.6 g/L) and her creatinine level was slightly elevated (23 μmol/L to 126 μmol/L). Meanwhile, her bilirubin level and international normalized ratio (INR) were both normal at 5 μmol/L to 16 μmol/L and 1.1 to 1.4, respectively.
At 14 months later, her recovery is still incomplete. Already in the ICU she complained that her vision is impaired. According to the consulting ophthalmologist, this symptom is likely due to ischemic optic neuropathy. At present she can only see movement and light with her right eye. The vision of her left eye is also severely impaired, but she is able to read using special equipment for the visually impaired. Additionally, her everyday life is harmed by numbness and weakness of her extremities, which is caused by critical illness polyneuropathy. Despite these impairments, her aim is to return to work.
Samples for the measurement of dexmedetomidine concentrations in her plasma were drawn at 8-hour intervals as directed by the plan of the pharmacokinetic study she was recruited in. The concentrations were determined using reversed-phase high-performance liquid chromatography with tandem mass spectrometric detection (PE Sciex API4000 instrument; PE Sciex, Foster City, California, US) as described previously [].
The rates of the dexmedetomidine and propofol infusions, as well her plasma concentration results of dexmedetomidine, are presented in Figure . In calculating the dexmedetomidine dose, we used her preoperative weight of 89 kg.
Because the dose rate of dexmedetomidine remained constant for relatively long periods of time during three separate intervals, we calculated the plasma clearance of dexmedetomidine during these intervals by dividing the infusion rate by the plasma concentration. The calculated clearance was 55 L/h, 92 L/h and 87 L/h during the 2nd to 6th, 14th to 20th and 21st to 23rd day of the dexmedetomidine infusion, respectively. A list of drugs administered during her ICU stay is presented in Table .


The patient was a healthy 14 year-old male. A delayed eruption of the maxillary right canine was noted upon intraoral and radiographic examination [Figures and ]. On palpation, the tooth showed an erupting position that was facial to the crest of the alveolar process and entirely within the alveolar mucosa. Apically positioned partial-thickness flap is the procedure of choice for soft tissue management.
A split-thickness flap was raised by sharp dissection using a number 15 scalpel blade along the tip of the unerupted canine.
Vertical releasing incisions were carried high enough into the vestibule to permit apical positioning of the flaps.
The flap was wide enough to maintain adequate vascularity.
The flap was raised to permit the exposure of impacted tooth. Osteoplasty was done to expose the crown portion for bracket placement.
The flap was apically positioned and stabilized with 4-0 silk sutures.
The patient was given oral hygiene instructions that included chlorhexidine rinsing of the mouth for seven days, but no tooth-brushing. One week later, sutures were removed and the area evaluated. Clinical examination revealed a favorable response in the absence of bleeding and inflammation. Mechanical tooth-brushing was reinstated one week after the surgery. The impacted tooth was cleaned and scaled to permit bonding. An orthodontic bracket or button was bonded to position []. After three weeks, the surgical site had revealed an adequate width of keratinized gingiva. The patient was followed up by his orthodontist to bring the maxillary canine into proper occlusion. The six month postoperative view is depicted in .


A 45-year-old woman visited our hospital for further evaluation of fecal occult blood in a yearly physical checkup. Colonoscopy revealed a red peduncular polyp, about 20 mm in diameter, in the sigmoid colon (). With conventional colonoscopy, the lesion did not show type III or IV pit pattern although magnifying colonoscopy was not performed. We speculated that this polyp was nonneoplastic. It was suspected to be an inflammatory polyp [IMGP or inflammatory fibroid polyp (IFP)] from endoscopic findings. Excluding the polyp, there was no lesion in the colorectum. Endoscopic polypectomy was performed. Histological examination of the specimen revealed inflammatory granulation tissue in the lamina propria, proliferation of smooth muscle, and hyperplastic glands with cystic change.


A 50-year-old man visited our hospital for further evaluation of fecal occult blood in a yearly physical checkup. Colonoscopy revealed a red, hard peduncular polyp, about 20 mm in diameter, in the descending colon (). Magnifying observation (EC-450ZH, Fujinon Toshiba ES Systems) showed type I or II pit pattern (). We speculated that this polyp was nonneoplastic. It was suspected to be an inflammatory polyp from endoscopic findings although it should be distinguished from a juvenile polyp. Endoscopic polypectomy was performed. Histological examination of the specimen revealed inflammatory granulation tissue in the lamina propria, proliferation of smooth muscle, and hyperplastic glands with cystic change (Figures and ).


A 49-year-old man consulted a physician for further evaluation of fecal occult blood in a yearly physical checkup. Colonoscopy revealed a red, hard, spherical peduncular polyp with erosion and mucous exudation, about 20 mm in diameter, in the sigmoid colon (). Magnifying observation (EC-450ZH, Fujinon Toshiba ES Systems) revealed a red, slightly rugged surface consisting of an aggregation of smooth nodules () with some normal round crypt openings (). We speculated that this polyp was nonneoplastic. It was suspected to be an inflammatory polyp from endoscopic findings although it should be distinguished from a juvenile polyp. An air contrast barium enema also revealed a pedunculated polyp like a snowman shape in the sigmoid colon (). Endoscopic polypectomy was performed. Histological examination of the specimen revealed inflammatory granulation tissue in the lamina propria, proliferation of smooth muscle, and hyperplastic glands with cystic change (Figures and ).


A 78-year-old man consulted a physician for further evaluation of fecal occult blood in a yearly physical checkup. Colonoscopy revealed a red, hard, conic peduncular polyp, about 20 mm in diameter, in the ascending colon (). With conventional colonoscopy, the lesion did not show type III or IV pit pattern. Magnifying observation (EC-450ZH, Fujinon Toshiba ES Systems) revealed a red, slightly rugged surface consisting of an aggregation of smooth nodules (). We speculated that this polyp was nonneoplastic. It was suspected to be an inflammatory polyp (IMGP or IFP) from endoscopic findings. Excluding the polyp, there was no lesion in the colorectum. Endoscopic polypectomy was performed. Histological examination of the specimen revealed inflammatory granulation tissue in the lamina propria, proliferation of smooth muscle, and hyperplastic glands with cystic change.


A 68-year-old male who had owned a bicycle shop for several decades visited our institute in March 2004 with a one-month history of swelling and pain of the right distal forearm. His dominant hand was right. On physical examination, an elastic soft mass, measuring 6 cm×4 cm, was observed on the palmar side of the right forearm. Motion of each finger was normal, but the range of motion of the wrist joint was slightly restricted. Skin color change and local heat were not observed around the mass. Laboratory data, including rheumatoid factor and C-reactive protein, were not contributory. He had no history of tuberculosis, and his tuberculin reaction on admission to the hospital was weakly positive. No hilar lymphoadenopathy was observed on a chest radiogram.
Plain radiograms of the right wrist and hand showed degenerative changes and Heberden's nodes in the distal interphalangeal joints, but no apparent calcification was observed in the mass (). On magnetic resonance (MR) imaging, T1-weighted images showed a low-intensity mass surrounding the flexor tendons (A). On T2-weighed images, the mass partially showed high-signal intensity (B). Sagittal T2-weighed images demonstrated an effusion in the sheaths of the flexor tendons and many small low-intensity bodies in the mass (C). The mass was moderately enhanced by administration of intravenous gadolinium (D), but the small bodies were not enhanced.
An incisional biopsy was performed in June 2004. The mass existed adjacent to the flexor tendons and was encapsulated by a yellow fibrous wall. Many rice bodies had erupted from a small hole in the wall of the mass (). Histological findings revealed hyperplastic tenosynovitis with fibrinous loose bodies (). During the surgery, we confirmed that the flexor tendons were intact. In July 2004, he complained of disability to flex in his ring and little fingers. After physical examinations, we made a diagnosis of spontaneous rupture of the flexor tendons. An open repair was performed in August 2004. During the surgery, we observed granulation tissues proliferating around the flexor tendons () and a rupture of the flexor tendons of the ring and little fingers. The granulation tissues were removed, and the superficial flexor tendon was transferred to the ring finger. Tendon graft of the palmaris longus to the little finger was also performed. Histologically, the granulation tissues showed chronic synovitis (), and acid-fast bacilli were negative. There was no apparent granuloma suggesting of sarcoidosis. The patient regained good function of the operated ring and little fingers with no evidence of recurrence at the latest follow-up in March 2009.


A case is presented for a 38-year-old woman with recurrent ductal carcinoma in situ (DCIS) and previous whole-breast irradiation who elected to undergo a total mastectomy with immediate reconstruction. She was an active runner and preferred a DIEP flap reconstruction. Her physical examination revealed only a moderate amount of infraumbilical soft tissue. Half of this abdominal pannus supplied by a single pedicle would not carry enough volume to match her large contralateral breast with significant projection (Fig ). A large-volume contralateral reduction was not preferable for this patient. A bipedicled, stacked DIEP flap would supply appropriate volume by utilizing all available abdominal donor tissue. Intraoperatively, 2 single perforators were chosen on each hemi-abdomen. The perforators were dissected down to their respective deep inferior epigastric pedicles. The abdominal tissue was transferred as a single unit, without dividing the skin in the midline. After harvesting the abdominal tissue, the flap was folded on itself and microsurgery was performed. One set of deep inferior epigastric vessels was anastomosed to the usual antegrade internal mammary vessels, whereas the other set of vessels was anastomosed to the retrograde internal mammary vessels. Good flows were observed in both recipient arteries. The folded flap was then inset in a stacked manner to maximize volume and projection. All buried skin was de-epithelialized. Keeping the dermis and fat intact between the 2 hemi-abdominal flaps enhanced cross-venous drainage, whereas folding the flap added projection and contour to the inferior pole (Fig ). Computed tomographic angiogram of the chest on postoperative day 5 was performed incidentally for respiratory concerns and revealed patent vascular anastomoses to both pedicles (Fig ). At 1-year follow-up, a satisfactory aesthetic outcome with a good size match was achieved without fat necrosis (Fig ).


A 16-year-old Hispanic male from Puerto Rico presented with fever and sore throat for 5 days. He was given oral penicillin by his primary care doctor. After a day, he developed an erythematous, nonpruritic rash which progressed proximally from both feet to thighs and upper extremities including palms and soles. Later the feet became swollen with moderately intense (7/10) burning pain, aggravated by ambulation. Review of systems is unremarkable except left ear pain. His significant comorbidities include asthma and gastrojejunostomy following a remote motor vehicle accident. On physical examination, there was pharyngeal erythema, petechiae on the soft palate, cervical lymphadenopathy, a nodular, and nontender, nonblanching purpuric rash involving both upper and lower extremities with nonpitting pedal edema (Figures and ). There was no truncal involvement. Two days later, the patient developed abdominal pain involving the right and left upper quadrant which was constant, colicky in nature, 8/10 in intensity, aggravated with meals, and associated with hematemesis and watery stools. Laboratory tests showed leukocytosis (WBC: 16,900/microL); Hb: 14 g/dL; Hct: 41.2%; BUN: 16 mg/dL; Serum Creatinine: 0.9 mg/dL; Urinalysis: no hematuria or proteinuria; ESR: 58 mm/Hr; CRP: 5.6 mg/dL; Antistreptolysin O titer: 823 IU/L; C3: 125 mg/dL; C4: 11 mg/dL; ANA: Negative; EBV-VCA IgM: Negative; Anti-HAV IgM: Negative; HbsAg: Negative; Anti-HBc IgG: Negative; Mono spot test: Negative; c-ANCA: 0.2 units; p-ANCA: 0.2 units; Stool for occult blood: positive. Esophagogastroduodenoscopy () showed multiple erosions in the duodenum, antrum, and the gastrojejunal anastomotic site. Histopathology (Figures and ) of the small bowel showed preserved villous architecture, and neutrophilic and eosinophilic infiltrates with leukocytoclastic vasculitis. Warthin-Starry stain was negative for Helicobacter pylori. There was no evidence of epithelioid granuloma. Colonoscopy showed erythema and inflammation in the terminal ileum and cecum. Cecal biopsy also showed leukocytoclastic vasculitis. Skin biopsy () showed pustular leukocytoclastic fibrinoid vasculitis with microabscess. The patient was diagnosed with Henoch-Schonlein purpura as per American College of Rheumatology and European League Against Rheumatism (EuLAR) and Pediatric Rheumatology Society (PReS) criteria. He was treated with intravenous fluids and kept nothing by mouth for 3 days with no enteral or parenteral nutrition. He was started on oral prednisone 20 mg twice a day, with resolution of his symptoms and a decrease in ESR and CRP. His diet was advanced to regular diet on the fourth hospital day.


A 5-year-old boy had met with road traffic accident and was declared brain dead. Parents and other nearby relatives gave consent for use of organs for purpose of transplantation. The size of both kidneys on ultrasound examination was 7.8 × 2.5 cm. A 1.2 cm long grade 1 injury was found on the anterior surface of the right kidney. Kidneys were perfused in situ after procurement by clamping the suprarenal aorta using Ringer's lactate solution [] and packed en bloc for transport and transplantation. On bench, the laceration was repaired by Surgicel® bolster and upper end of aorta and vena cava were sutured by 4/0 and 5/0 polypropylene sutures respectively []. The lower end of donor aorta and vena cava were anastomosed to recipient's external iliac vessels and both kidneys were placed extraperitoneally in a 42-year-old female recipient who had renal failure due to chronic glomerulonephritis []. Both ureters were implanted by modified Lich-Gregoir method over 4.5 Fr size double ‘J’ stents. Drain tube was placed and wound was closed in layers.


A 75 year-old lady with long-standing hypertension was detected to have mild renal failure during evaluation for an upper respiratory infection. She did not give any history of colics, previous episodes of urinary tract infections, or trauma to the chest or abdomen. A subsequent abdominal ultrasonogram revealed mild right hydroureteronephrosis with slightly decreased parenchymal thickness.
In view of her altered renal functions, a noncontrast computed tomography scan was done which revealed a relatively small right kidney that was displaced cranially towards the diaphragm; the renal axis was abnormal with the pelvis facing posterosuperiorly []. Along with retroperitoneal fat, the dilated right ureter could be seen herniating into the ipsilateral thoracic cavity through a 2.6 cm wide defect in the posteromedial aspect of the right hemidiaphragm [], which was suggestive of a Bochdalek hernia. The lower chest sections showed the ascending and descending limbs of the herniated ureter in a loop configuration []. The descending ureter appeared to be compressed at the location of the diaphragmatic defect. A subsequent retrograde pyelogram confirmed the classical ureteral loop or knuckle, which has been described as the curlicue sign []. It also demonstrated prompt and complete emptying of the pelvicalyceal system, thus ruling out an obstructive cause for the renal failure. Hence, it was decided to manage the patient's renal failure with medical measures. Future surgical intervention has been planned only if there are chest symptoms due to the mass effect, or if renal failure can be definitely attributed to the anatomical anomaly.


An asymptomatic 29-year-old female with no medical history or history of trauma to the chest wall was admitted due to a left chest wall mass which was incidentally discovered on routine chest X-ray (). Computed tomography (CT) demonstrated a localized mass, measuring 4.5 cm, along the inner surface of the 7th left rib (). Osteosclerosis was present on the top of the lesion along with calcification in different places and thickening on the nearby parietal pleura. Routine hematologic and blood biochemistry results were normal. The patient underwent left lateral minithoracotomy in which a total excision of the rib was performed. The patient had an uneventful recovery and discharged on the third postoperative day. Histological examination of the resected lesion revealed a cavernous hemangioma of the rib while the margins were negative for tumour cells.


A 35-year-old woman with skin type III was referred to our department for the evaluation of whitish macules on trunk and limbs. They first appeared when she was 12 years old and increased slowly but steadily in size and number. After the first gravidity at age 29 and a second one at age 32 the patient observed a marked acceleration of this process. There is no history of itching or dysaesthesia. The patient denied recreational sun exposure for the past ten years. When she was exposed to sunlight the whitish macules did not change the colour whereas the nonaffected skin became more tanned, so that the contrast between the whitish macules and the nonaffected skin was remarkably intensified. She had not been on antibiotics, birth-control or hormone, containing pills, had no noteworthy history of dermatitis or eczema. Signs of atopy were absent. Her ancestors lived for many generations in Germany.
Skin findings consist of symmetrically distributed hypopigmented, nummular, partial confluent, nonscaly macules on trunk and less pronounced on proximal parts of arms and legs, as well as sparing head and neck (Figures , ). Distribution is neither segmental nor does it follow Blaschko-lines. The round or oval hypopigmentated macules have well-defined borders. No signs of inflammation are present in these macules. Uninvolved skin displays brownish coloration alike typical skin type III.
There was no evidence for fungi or other infectious agents in morphologic and cultural surveys on repeated attempts. A Biopsy from affected skin displayed a reduced amount of melanin in the basal layer compared to nonaffected skin.
Upon electron microscopic comparison of type and distribution patterns of melanosomes in affected () and nonaffected epidermis (), we found those in macules of PMH to be more aggregated and single melanosomes were smaller. They displayed filaments and looked tessellated (stage I-II). In contrast the melanosomes in nonaffected skin are usually larger and distributed more evenly with less discernible internal structures. In other words the affected skin is characterized by less developed melanosomes (stage I or II), whereas nonaffected epidermis displays mature melanosomes (stage III or IV) according to the natural skin type of the patient.


A 41-year-old white woman presented in July 2003 with two nodules in the upper quadrants of the left breast and underwent radical mastectomy according to Madden and removal of 18 axillary lymph nodes for a multicentric infiltrating moderately differentiated ductal carcinoma (2 cm maximum diameter); lymphovascular space invasion was positive, and 8 lymph nodes were shown to contain malignant cells.
The tumor was positive for both estrogen receptors (ER) (90%) and progesterone receptors (PR) (70%), negative for human epidermal growth factor receptor 2 (c-erbB-2), and positive for Ki-67 (15%). The tumor was pathologically staged as pT2N2M0.
Postoperatively, the patient received 8 cycles of adjuvant chemotherapy with Epirubicin (4 cycles) and Cyclophosphamide, Methotrexate, Fluorouracil (CMF) protocol (4 cycles) plus radiotherapy (44 Gy) to the left hemithorax and left supraclavicular fossa. Treatment continued with Leuprolide in a monthly dose of 3.75 mg for two years, and Tamoxifen in a daily dose of 20 mg.
In March 2007, an increase in CA15-3 levels (54.9 U/mL; reference level, < 30) was documented in the absence of any sign of recurrence as assessed by imaging. Thoracic computed tomographic (CT) scan demonstrated a solitary small soft tissue mass of approximately 10 mm of diameter located in the middle lobe of the right lung attached to the mediastinal pleura. A total body CT-PET was positive.
She underwent surgical resection of the nodule and of the visceral pleura of upper lobe. Histology examinations showed the presence of metastatic breast carcinoma both in the middle lobe and visceral pleura of upper lobe. The tumor was positive for both ER and PR, and negative for c-erbB-2. Postoperatively, she received 6 cycles of chemotherapy with Docetaxel and hormone therapy with Letrozole.
Follow-up was negative until March 2009, when a novel increase of CA 15.3 (59.8 U/mL) was demonstrated. Ultrasonography of the liver showed, at the level of pancreatic head, a cystic lesion approximately 14 × 13 mm in diameter, with internal septa with features suggestive of intraductal papillary mucinous neoplasm. Pancreatic magnetic resonance confirmed this suspicion. Even if CT guided biopsy and a total body CT-PET were negative, the patient was strongly motivated to take away the pancreatic lesion, and a month later pancreaticoduodenectomy was performed; the postoperative course was uneventful. Pathological examination revealed a mucinous cystoadenoma of the pancreatic (1 cm maximum diameter) with negative resection margins. Surprisingly and fortunately, a peripancreatic lymph node removed during surgery (0.7 cm maximum diameter) was shown to contain tumour cells of the same histotype of primitive breast cancer and showing both ER and PR positivity, apparently stronger membrane signal for HER-2 (Figure ), CK7 and 19 positivity (data not shown). Tumor cells resulted consistently negative for carcinoembryonic antigen (CEA) (data not shown) and for breast tissue marker gross cyctic disease fluid protein (GCDFP) (data not shown). Immunoreaction for CDX-2 antigen used to discriminate tumors of gastroenteric origin was negative (data not shown).
A re-joint evaluation of the slides was planned with two independent pathologists who confirmed the diagnosis.
Consequently, surgery tumour markers returned quickly within the reference range. After a careful counseling hormone therapy with Exemestane was begun which is currently ongoing. At 16 months of follow-up, the patient is free of disease.


A six-month-old female infant presented to us with bilateral polydactyly of hands and inward deviation of the right eye (OD). She was delivered normally at term, was the only daughter of a 28-year-old healthy father and 22-year-old healthy mother of first-degree consanguinity. No other family members had similar hand deformity or defect in the position of eyeball. Ocular examination revealed right esotropia of about 90 PD deviation with left eye fixating and cross fixation on side gaze []. She had no abduction deficit. Nystagmus was absent. Cycloplegic refraction in both eyes was +1.0 D. Direct Ophthalmoscopy of both eyes revealed no abnormality. On physical examination, her weight was 3.8 kg, height 48.5cm and head circumference 36 cm (all below 2SD). The chest circumference was 31 cm (below 2SD) and trunk of normal size. Each upper limb was 20 cm, while each lower limb was 16.5 cm (both below 2SD). The arm span was 43 cm (below 2SD). The arms and legs were short, clubby and shortened out of proportion to the trunk. The hands were wider than normal and feet were square shaped. Bilateral postaxial polydactyly of hands were present []. Her digits were proportionately short compared to her hands. Fingernails and toenails were markedly hypoplastic, thin and were set deeper. She had a fusion of the upper lip to the maxillary gingival margin that produced a V-notch in the middle of the lip and had a lip tie.
Oral examination revealed serrated alveolar margins without any erupted tooth. Cardiological evaluation including echo and electrocardiography revealed no abnormality. The biochemical, routine hematological and urine tests were within normal range. Radiological investigations showed postaxial polydactyly, short distal and middle phalanges compared to the proximal ones [], short iliac wing and horizontal acetabulum with medial spurs []. Carpal development was delayed. Abdominal, renal and lung ultrasonogram were normal. In our case, clinical and radiological features were suggestive of EVC with essential infantile esotropia OD.


A 64-year-old Japanese male, who had no past medical history or present systemic disease, noticed a mass in the right knee 10 years ago. He had no history of a pre-ceding trauma in the lesion. The mass gradually grew larger and harder in the last 5 years, accompanied by sense of tension in a sitting position. Physical examination revealed a smooth, elastic hard, and mobile mass of 3 cm in the largest diameter in the medial posterior side of his knee, which was painless and caused no neurological abnormality. Difficulty in physical activities such as deep knee bend and square-sitting was observed. Computed tomography scan revealed a mass lesion of heterogeneous density with a cystic area, enhanced not in the early arterial phase but in the delayed phase. Magnetic resonance imaging demonstrated a mass lesion embedded in the subcutaneous tissue with low and high signal intensity at T1- and T2-weigthed images, respectively (Fig. ). Thallium-201 chloride (Tl-201) scintigraphy showed an uptake of Tl-201 into the mass at early scan at 10 minutes after injection, which slightly decreased but still accumulated at 2 hours later (Fig. ). The patient underwent a resection of tumor for a tentative diagnosis of neurilemoma of degenerative type ("ancient Schwannoma"). Surgeons found a tumor encapsulated by a thin fibrous capsule in the subcutis and marginally excised it. The patient was uneventfully discharged from the hospital and free from locally recurrent or metastatic disease two years after surgery.
Grossly, the resected specimen was a white-colored, elastic hard mass measuring 3.0 × 2.0 × 1.5 cm, and on cut-surfaces, consisted of grayish solid area and whitish gelatinous component with a cystic space containing a little amount of serous fluid. Microscopically, uniform-appearing epithelioid cells without cytoplasmic vacuolation were arranged in nests, cords, and ductules, and spindle-shaped myoepithelial cells embedded in abundant chondromyxoid matrix, accompanied by osseous metaplasia (Fig. ). Neither vascular proliferation nor blood pools were recognized. Mitotic figures and nuclear pleomorphism were absent.
Comprehensive immunohistochemical findings are demonstrated in Figure . The epithelial component of the tumor was diffusely stained with cytokeratin (CK) AE1/AE3 and CAM5.2, and partially with CK 34βE12. Epithelial membrane antigen (EMA) was strongly expressed on the luminal surface of glandular structures. Mesenchymal tumor cells were totally negative for these epithelial markers except scattered cells positive for CAM5.2. Desmin and α-smooth muscle actin were not expressed within the tumor. Glandular component was positive for anti-p63, indicating the basal myoepithelial characteristics of the cells. Many mesenchymal tumor cells were stained with anti-S-100 protein. Vascular structures indicated by CD31 and CD34 were scant in the stroma. Bcl2 was strongly expressed in the epithelial component with a weaker intensity in the mesenchymal component. Epithelial tumor cells were faintly stained with anti-p16 in the cytoplasm. Ki-67 labeling index was less than 1% and p53-positive cells were rarely seen in both epithelial and mesenchymal lesion, indicating the low proliferation rate and the absence of disruption of p53 tumor-suppressor pathway. These findings indicated that the tumor to be composed of epithelial and mesenchymal lesions, and the epithelial component having myoepithelial characteristics. The pathological diagnosis was a benign mixed tumor arising in the subcutis of the knee. All the primary antibodies used for the differential diagnosis were from Dako A/S (Glostrup, Denmark) except CAM5.2 (Becton Dickinson, Flanklin Lakes, NJ, USA) and anti-p16 (Novocastra Laboratories Ltd., Newcastle upon Tyne, UK).
Immunohistochemical analysis using anti-mouse monoclonal antibody against Na+/K+-ATPase α (M7-PB-E9, Affinity BioReagents Inc., Golden, CO, USA) demonstrated an expression of Na+/K+-ATPase in cell membrane and cytoplasm of tumor cells (Fig. ).


A 65-year-old man was admitted to hospital with acute abdominal pain and hypovolemic shock. He was a cigarette smoker for more than 20 years, had arterial hypertension and a medical history of cholecystectomy and appendectomy. Abdominal CT imaging showed a huge retroperitoneal hematoma of 7 × 23 × 25 cm (Fig. ) and an aneurysm of the IMA about 2 cm from its origin resulting in concurrent occlusion of superior mesenteric and celiac arteries (Fig. ). The IMA also was very wound from its origin. The patient underwent midline laparotomy with a transperitoneal approach to the IMA by a multidisciplinary team of vascular and colorectal surgeons. Severe bowel adherences were observed which possibly helped to limit the massive hemorrhage limited to the retroperitoneum. The aneurysm was completely excised. The normal proximal and distal ends of the IMA were then mobilized. The artery was finally reconnected in an end-to-end manner to re-establish blood flow. Neither a graft nor a shunt was used, with only a short period of ischemia during the running suture. Heparin was rinsed only locally, due to the massive hemorrhage and the need for transfusion. Macroscopic examination of the aneurysm sac demonstrated typical appearances of atherosclerotic aneurismal tissue. There was no history of trauma or any clinical sign of sepsis that led us to suppose that another cause might be responsible for this lesion. The postoperative course was uneventful, and no symptoms of intestinal ischemia were noted.
Worldwide, there have been only eleven reports of IMA aneurysms associated with tight stenosis of the SMA and celiac trunk [,]. Other publications have presented cases with abdominal angina and weight loss [] or even rupture []. Previous authors have conjectured that this situation may arise because of a ''jet disorder'' phenomenon. Concurrent occlusion of the superior mesenteric and celiac arteries leads to greatly increased and possibly turbulent blood flow in the IMA []. This produces localized areas with high arterial pressure that in an unadapted vessel can lead to aneurysm formation and subsequently thus to rupture, as was the case in our patient. The marginal artery of Drummond was also greatly increased in size (Fig. ) and supplied the entire gastrointestinal tract. Today, abdominal ultrasound, computed tomography, magnetic resonance imaging, and arteriography facilitate an early diagnosis. Although these patients are generally asymptomatic, it is generally acknowledged in literature, to treat VAA because of the risk of rupture or ischemia. Either surgical or endovascular therapeutic procedures can be performed in the treatment of this lesion and are well described in literature []. Percutaneous transcatheter coil embolization techniques are also used with increasing frequency in the treatment of VAA []. Nevertheless, a high incidence of aneurysmatic sac reperfusion and a relatively high morbidity are associated with this procedure. Other percutaneous techniques such as permanent liquid embolic material and the use of a covered stent have been proposed, but the results must yet be verified. In our case, because of the hypovolemic shock an emergency surgical approach was indicated. The IMA being very tortuous, thus implying a difficult endovascular approach, we decided to perform a direct anastomosis between both ends of the inferior mesenteric artery instead, which seemed more feasible in our case. In the same instance we could take advantage of this type of approach to also evacuate the symptomatic retroperitoneal hematoma mentioned above. The mesenteric circulation being not compromised, revascularisation of the SMA and celiac trunk was not undertaken.


A 68-year-old Caucasian female with a history of hypertension and hysterectomy (approximately 20 years ago) presented to the hospital with dehydration and right upper quadrant pain. A computed tomography (CT) scan of our patient obtained as an out-patient showed irregular thickening of the gallbladder wall associated with stones, but there was no evidence of cholecystitis. Both the intrahepatic and extrahepatic biliary ducts were dilated approximately to the level of the CBD, but indicated no choledocolithiasis. A follow-up endoscopic retrograde cholangiopancreatography (ERCP), performed to evaluate for possible cholangiocarcinoma, revealed a CBD stone with marked intrahepatic and extrahepatic duct dilatation. ERCP indicated no evidence of a cholangiocarcinoma. After papillotomy, the endoscopist determined the stone was beyond the scope of endoscopic removal, thus open cholecystectomy with CBD exploration was indicated.
Two days later, our patient underwent an open cholecystectomy and CBD choledochoscopy with an Olympus VRF Type P2™ flexible fiberoptic choledocoscope. A prophylactic antibiotic, 1 g cefazolin, was given on induction of anesthesia. The choledocolith was removed by choledocotomy performed on the palpable stone. A CBD T-tube was placed, brought out through the lateral abdominal wall, and attached to the bile drainage bag. No other stones remained in the CBD after the completion of surgery, which was confirmed by intra-operative cholangiogram. The stone was assumed to have been present in the CBD for many years since it was significantly large. The possibility of it being a primary CBD stone or a stone that was passed into the CBD years ago and continued to grow also had to be entertained.
Five weeks post-operatively, our patient was readmitted with dehydration and poor oral intake. The patient had remained afebrile with white blood cell count of 15.1×103k/mcL with no shift in white blood cell morphology. A CT-scan was obtained and indicated a fluid collection suggesting an abscess of the right flank (figure ). Our patient was taken to the operating room where a retroperitoneal abscess was found that tracked into the right paracolic gutter and into the right flank. There was no subhepatic fluid collection. The CBD repair was intact. Multiple drains were placed in the abscess cavity. Cultures of the collected fluid revealed H. parainfluenza. An Infectious Disease specialist was consulted, and the patient was placed on intravenous ceftriaxone (which was susceptible to the organism) for 6 weeks. Follow-up appointments and CT-scans at 6 weeks showed a decrease in the fluid collection, and she denied any further symptoms.


A ten-year-old East Asian boy presented to the Pediatric Out-Patients Department with abdominal distension of five years' duration and having suffered shortness of breath on and off for the same period. Abdominal distension, without abdominal pain, had been gradual and progressive since he was five. There was also a history of joint pain on and off during the past two to three years. There was no history of constipation, diarrhea, vomiting, bleeding, jaundice, seizure, weight loss or loss of appetite or of consciousness. His bladder habit was normal. He started school at seven years of age and was below average in his studies.
On examination his head was acrocephalic in shape, with a circumference of 54.5 cm. He had a depressed nasal bridge, a short neck, coarse facial features, small stubby fingers with flexion of the distal interphalangeal joint, and a low arched palate (Figure ). Anthropometric examination showed him to be severely stunted without wasting. His fundus appeared normal. His abdomen was soft and slightly distended with a protruding umbilicus. His liver was 11 cm below the right costal margin in the mid-clavicular line, with a firm, sharp margin and a smooth surface with a span of 12 cm. His spleen was 2 cm below the left costal margin in the mid-clavicular line. His heart had a grade 2/6 non-radiating systolic murmur in the mitral area. Suspecting MPS, we performed a skeletal survey. Anteroposterior and lateral X-rays of the skull showed an enlarged and J-shaped sella turcica (Figure ). The bones of the skull and sutures appeared normal for his age. Anteroposterior and lateral X-rays of the dorsolumbar spine showed anterior beaking (Figure ). Vertebral bodies appeared ovoid due to convexity of the superior and inferior surfaces. Spinal curvature was normal and vertebral body height also appeared normal. X-rays of both hands showed his phalanges and metacarpals to be widened with proximal tapering of the metacarpals (Figure ). The remaining bones and joints under view appeared normal. An anteroposterior X-ray showed his ribs as wide with tapered posterior ends (a paddle and/or spatulated appearance) (Figure ). No parenchymal lesions were seen in visualized lung fluids.
Although his radiological features were suggestive of MPS, without determining the type of MPS, from his history and clinical examination we have made a diagnosis of MPS type II (Hunter syndrome). However in our patient there was atypical presentation such as an acrocephalic head, mild mental retardation, and no corneal clouding, which are important features of MPS type II. Unfortunately we could not perform any measurement of GAG, keratan and heparan sulphates, in his urine because of the lack of test kits. Enzyme assay for iduronate sulfatase is not carried out in our laboratory therefore it was not performed either. Our diagnosis of MPS was confirmed from his history, clinical examination and skeletal survey.