A 46-year old female was admitted to the hospital because of low-grade fever, sore throat and fatigue for four days; she was on clarithromycin 500 mg twice daily, as she was considered to suffer from upper respiratory tract infection by her general practitioner.
The patient had a history of SLE for 24 years, antiphospholipid syndrome and autoimmune hypothyroidism. SLE was diagnosed in the background of immune thrombocytopenic purpura (ITP), starting at the age of 8. At the age of 12, splenectomy was performed to control refractory thrombocytopenia. Currently, SLE was adequately controlled (Systemic Lupus Erythematosus Disease Activity Index, SLEDAI = 0, anti-dsDNA antibodies negative, C3 and C4 levels normal); medication included methylprednisolone 8 mg/day, azathioprine 50 mg/day, aspirin 50 mg/day and levothyroxine 150 μg/day. The patient had been vaccinated against seasonal influenza and Streptococcus pneumoniae a month before, but not against A/H1N1 virus.
On admission, she was in severe cardiovascular instability with hypotension (BP = 60/40 mmHg), tachycardia (HR = 130/min), tachypnea (RR = 30/min), hypothermia (< 35.5°C), along with oliguria and altered mental status. Oxygenation fraction PaO2/FiO2 was over 350. No obvious site of infection could be identified. The initial chest X-ray was normal, as well as computed tomography of the thorax (Figure ). Heart ultrasound revealed mild diastolic dysfunction with preserved ejection fraction and no valvular disease. Concerning other organ involvement, there was mild prerenal azotemia (urea 94 mg/dl, creatinine 1.5 mg/dl) and severe liver impairment (alanine aminotransferase 2837 U/L, aspartate aminotransferase 3165 U/L).
The patient was considered to suffer from systemic inflammatory response syndrome (SIRS) and was treated with vigorous fluid resuscitation (crystalloids and colloids), oseltamivir 150 mg/day and moxifloxacin (400 mg/day, after appropriate cultures were obtained). A few hours later, she was intubated and carried to the ICU, because of refractory shock, where vasopressor therapy (noradrenaline up to 2 μg/kg/min) was administered to maintain mean arterial pressure ≥65 mmHg.
Arterial pressure wave form analysis (FloTrac/Vigileo System), continuous ScVO2 and CVP monitoring were used to estimate patient's hemodynamics. After initial fluid resuscitation, cardiac index, ScVO2 and CVP measurements were 2.7 L/min/m2, 75% and 13 mmHg, respectively (mean values). Blood lactate levels were 4.8 mmol/L. The ventilatory support consisted of controlled MV with tidal volume 7 ml/kg and respiratory rate 15/min. Initial plateau pressure was 16 cmH2O with respiratory system compliance 58 ml/cmH2O.
The patient responded after 24 hours; noradrenaline was tapered to 0.25 μg/kg/min and hydrocortisone (300 mg/day) was added as adjunctive therapy. Additional antibiotics included piperacillin/tazobactam (18 g/day) and linezolide (1.2 g/day). Blood, urine and bronco-alveolar lavage (BAL) cultures were negative. Real-time PCR for influenza A/H1N1 virus (BAL specimen) was positive and oseltamivir was administered at 300 mg/day.
In ICU, the clinical course was complicated by bacterial co-infections 10 days after admission; septicaemia due to carbapenem-resistant Klebsiella pneumoniae and ventilator-associated pneumonia due to multi-drug resistant Acinetobacter baumanii. Gentamicin and colistin were administered, according to strain sensitivity, leading to complete resolution of the lesions. She was extubated after 19 days and transferred to the ward.
Concerning H1N1 virus, BAL rt-PCR was persistently positive for 21 days. Oseltamivir was administered in high doses (300 mg/day) for 28 days and was discontinued after negative PCR.
The patient experienced a lupus flare (SLEDAI = 4, C3 = 65 mg/dl, C4 = 7.4 mg/dl), soon after extubation with thrombocytopenia and severe haemolytic anemia. The flare was successfully managed with steroids and intravenous immunoglobulins (IVIGs). Her clinical condition was complicated by severe critical illness polyneuropathy/myopathy (CIP/CIM), managed with long term physiotherapy. In four months, previous therapeutic regimen was re-established and satisfactory performance status was maintained.


We present the case of a 32-year-old Caucasian man who initially presented five years previously with right hip pain and was found at that time to have a large multilobulated mass arising from his right ilium and extending to his right sacrum with involvement of his gluteal musculature (Figure ). A biopsy showed a grade 1 chondrosarcoma. Subsequently, a right hemipelvectomy was performed, and the diagnosis of grade 1 chondrosarcoma arising from a pre-existing osteochondroma was confirmed (Figure ). Additionally, a minor component of clear cell chondrosarcoma was identified at the origin of the osteochondroma within the medullary cavity of the underlying ileum (Figure ). Recurrence in the sacral stump and an area adjacent to his paraspinous muscles was noted two years later after our patient experienced a fall. He underwent proton beam radiotherapy and resection, complicated by a postsurgical abscess.
Approximately two-and-a-half years later, our patient presented with significant pain in his right pelvis, difficulty in ambulating and a decreased performance status due to pain. A bone scan showed uptake in his left temporal bone and an excisional biopsy confirmed recurrence of the clear cell chondrosarcoma (Figure ). A positron emission tomography/computed tomography (PET/CT) scan showed uptake to an standardized uptake value (SUV) maximum of 9.0 in the right sacral lesion with two other areas of uptake in his pelvis (Figure ). Two non-hypermetabolic pulmonary nodules were also noted. Stereotactic radiosurgery was applied to the temporal bone lesion. Our patient was started on sunitinib 37.5 mg daily, which was initially tolerated well, with only mild fatigue. His pain greatly improved. A PET/CT scan obtained two months after the initiation of sunitinib showed improvement in the fluorine-18-fluorodeoxyglucose (FDG)-avidity of the lesions, with a right sacral lesion showing an SUV of 6.8 (Figure ). He continued to take sunitinib at the same dose and was referred for proton beam radiation to his right pelvis. He received twenty fractions of radiation concurrent with sunitinib 37.5 mg daily and experienced some diarrhea and worsening of an acneiform rash, but our patient wished to continue on the same dose given his excellent response with regards to the pain. A total of six months after initiating sunitinib, our patient continued to show improvement on imaging, with a PET/CT scan revealing stable non-hypermetabolic pulmonary nodules and improvement in the right sacral lesion with an SUV of 3.4 (Figure ). He did require a reduction in the dose 25 mg daily, however, due to the worsening acneiform rash and diarrhea.


A 58-year-old Caucasian female visited the U.S. Environmental Protection Agency’s Human Studies Facility in Chapel Hill, North Carolina, to participate in a study requiring sequential exposures to filtered air and concentrated ambient particles (CAPs). Protocols and consent forms were approved by the University of North Carolina (UNC) School of Medicine Committee on the Protection of the Rights of Human Subjects, and the subject provided informed consent. Two years previously, she had participated in an identical exposure protocol without any complication. At that time, two 24-hr Holter examinations obtained during the exposures to filtered air and CAPs demonstrated 29 and 54 episodes of supraventricular ectopy, respectively.
On the day of exposure to CAPs, the volunteer had no symptoms. The participant had a history of osteoarthritis and hypertension, and she was being treated with an angiotensin-converting enzyme inhibitor and a diuretic (10 mg lisinopril and 12.5 mg hydrochlorothiazide, respectively) for the hypertension. Previous surgeries included a hernia repair, a cholecystectomy, and a total left knee arthroplasty. Her family history was significant due to the death of her father at 57 years of age from a myocardial infarction. The volunteer was a lifetime nonsmoker. On physical examination, she was 173 cm tall and weighed 104.4 kg (body mass index, 34.9; waist, 45 inches). Her pulse was regular at 66/min, and her blood pressure was 144/61 mmHg. The baseline electrocardiogram (ECG) showed normal sinus rhythm (). A Holter monitor demonstrated evidence of increased supraventricular ectopy, with 157 ± 34 premature atrial contractions per hour during the 3 hr immediately preceding the exposure to CAPs.
Twenty-three minutes into the exposure to CAPs (filter weight, 112 μg/m3; particle number, 563,912/cc), the telemetry monitor revealed that the subject had nonsustained atrial fibrillation that quickly organized into atrial flutter. She was immediately removed from the exposure chamber. The volunteer reported no symptoms, and there was no change in the physical examination. The 12-lead ECG verified that she remained in atrial flutter (). Her serum electrolytes, blood urea nitrogen, creatinine, glucose, and complete blood count were all normal. Creatine kinase and the MB fraction were also normal. During the transfer to the UNC Medical Center (~ 2 hr after the onset of the arrhythmia), she spontaneously reverted to a normal sinus rhythm.
The patient was admitted to the hospital overnight for observation and telemetry. The next morning, the ECG documented normal sinus rhythm. Her serum electrolytes, blood urea nitrogen, creatinine, glucose, creatine kinase, and the MB fraction were again normal, and her complete blood count was normal except for a hematocrit of 35.7% (the lower limit of normal is 36.0%). Resting transthoracic echocardiography demonstrated normal right ventricular contraction with an ejection fraction of 55–60%, aortic sclerosis, and diastolic left ventricular dysfunction. The left atrium was considered mildly dilated; all other chambers of the heart were normal in size. She was discharged and was not prescribed a new medication. Approximately 6 weeks later, she underwent electrophysiology study, which did not provoke atrial fibrillation or significant atrial ectopy. The study did indicate a reentrant circuit of the cavotricuspid isthmus, which was ablated to prevent potential future episodes of atrial flutter.


A 15-year-old male who complained of a swelling inside his mouth was referred to the Oral Diagnosis Service of the School of Dentistry of the University Tiradentes (Aracaju/SE, Brazil) in March of 2005. Intraoral examination revealed an asymptomatic swelling on the right side of the hard palate. It was approximately 3.5 cm in size and according to the patient had been present for the past 1 year. The overlying mucosa was intact and normal in color and appearance. The teeth involved were all vital and no phlogistic signs were observed (Figure ). His past medical history and the family history were analyzed in detail but were noncontributory. Computed tomography of the lesion was done and showed a large hypodense tumoral image in the right side of the palate, where it had provoked a slight erosion of the maxillary cortical bone (Figure ).
Incisional biopsy was performed and the surgical specimen was sent for histopathological analysis. Histological sections of the specimen revealed a salivary gland neoplasm whose parenchyma consisted of plasmacytoid cells with eccentric round nuclei and eosinophilic (hyaline) cytoplasm, predominantly arranged in islands and sheets of tumoral cells. Less commonly, the tumoral cells were organized in anastomosing strings mimicking a pseudo-cribriform arrangement. Foci of hemorrhage and hemosiderin pigmentation were also found. The stroma showed strong hyalinization of the connective tissue with focal areas of myxoid changes (Figure ). Immunohistochemically, the cytoplasm of the plasmacytoid cells was positive for S-100 protein and vimentin (Figures and ) and negative for smooth muscle actin (α-SMA) (Figure ) and cytokeratin 14 (CK14). Focal reactivity for glial fibrillary acidic protein (GFAP) was observed in some areas (Figure ). The overall picture suggested the diagnosis of PM. The definitive treatment in this situation was surgical excision, extending down to the periosteum and including the overlying mucosa. The patient continues to be under rigorous follow-up and no recurrence has been detected so far.


A 48-year-old Saudi man presented with repeated vomiting and epigastric discomfort for a duration of ten days. Vomiting occurred one hour after eating, and was projectile, yellowish in color, and contained undigested food with no mucus or blood. It was associated with mild epigastric discomfort, which was of gradual onset, intermittent, dull and aching in nature, and occasionally felt in the back and both hypochondria. There was no history of peptic ulcer disease or drug ingestion. The patient denied any history of fever, cough, weight loss, or trauma. He was diagnosed with hypertension 10 months before presentation for which he was taking fosinopril 10 mg once daily. The family history was negative for aneurysms and malignancies. The patient was retired from the military services. He drank alcohol occasionally and had a 10 pack-year smoking history.
Clinical examination revealed a middle-aged man who was fully conscious, afebrile, and hemodynamically stable. Systemic examination revealed no abnormality, but abdominal examination revealed mild tenderness in the epigastric area. The rectal examination was unremarkable. Blood tests were unremarkable () Esophagogastroduodenoscopy revealed hiatus hernia with mild gastritis not consistent with the clinical condition of the patient. A gastric biopsy was negative for malignancy. A CT scan of the abdomen demonstrated a rounded structure in the posterior aspect of the pancreas, anterior to the third part of duodenum, which measured 2.5 cm and compressed the duodenum, causing dilatation in the stomach, and the first and second part of the duodenum, which suggested an aneurysm in PDA or gastroduodenal (hepatic) artery (). A CT angiography suggested by the radiologist confirmed the diagnosis of PDA aneurysm causing gastric outlet obstruction (). The patient received supportive treatment and was referred to a tertiary center for embolization of the aneurysm.


An 18-year-old, previously healthy, unmarried Sinhalese woman from a nearby city (20 km away from Colombo) was referred to a tertiary care hospital in Colombo for treatment of metabolic acidosis after an overdose of 35 tablets of metformin (17.5 g) and a quarter bottle (nearly 200 mL) of kerosene oil. She was admitted to a local hospital a few hours after ingestion and was transferred to a teaching hospital in Ragama, where she developed a generalized tonic clonic seizure that lasted five minutes. When she was admitted to the teaching hospital, her capillary blood sugar level had been 42 mg/dL and was treated with a bolus intravenous dextrose and 10% dextrose infusion. Her arterial blood gas (ABG) revealed a severe metabolic acidosis (Table ). She was treated with two doses of 75 mL of 8.4% sodium bicarbonate (NaHCO3) (75 mmol) but had persisting acidosis three hours later. At that stage, she was given 50 mL of NaHCO3 (50 mmol), 500 mg ampicillin, and 50 mg ranitidine (all intravenously) and was transferred to a Tertiary care hospital, Colombo (THC) for hemodialysis.
At THC, she complained of burning epigastric pain and shortness of breath. She also had several episodes of vomiting. Her peripheries were warm. She had Kussmaul breathing and a respiratory rate of 44/minute, a pulse rate of 116/minute, and a blood pressure of 90/60 mm Hg. Except for the mild epigastric tenderness, the results of the rest of a physical examination were normal. Her oxygen saturation was 92% with 5 L/minute oxygen via a face mask. The ABG at 15 hours after ingestion showed continuing acidosis with a high anion gap (Table ). Her serum lactate level was elevated at 59.96 mg/dL.
Eighteen hours after ingestion, she was started on intermittent hemodialysis with bicarbonate buffer (which contains both solutions A and B) (Additional file ). The initial dialysis was continued only for two and a half hours with a blood flow rate of 140 mL/minute and a dialysate flow rate of 500 mL/minute. However, she continued to be acidotic four hours after the dialysis (that is, 28 hours after ingestion). She was treated again with 8.4% NaHCO3 (100 mL), and dialysis was repeated on the following day. The ABG after the second dialysis (that is, 48 hours following ingestion) showed compensated metabolic acidosis (Table ). The serum creatinine level was 279 μmol/L on admission and started to increase rapidly two days after admission (Figure ) and was accompanied by a declining urine output. Subsequently, she was dialyzed on days 3, 10, and 14. All of the subsequent dialyses were continued for 4 hours with a blood flow rate of 200 mL/minute and a dialysate flow rate of 500 mL/minute. Her urine output and renal functions improved gradually, and after three weeks she was discharged with normalized renal functions.


A 56-year-old male was suffering from right thigh pain, numbness, and gait disturbance for 1 month before presentation.
Neurological examination showed that his muscle power was normal in lower extremities and his right patellar tendon reflex was absent. Sensory disturbance was detected in the right thigh. The patient was diagnosed with right L3 radiculopathy. All laboratory findings were within normal limits.
Lumbar spine MRI showed a mass compressing the dura that caudally migrated at the L2-L3 level on T2-weighted sagittal image (). The mass continued to the disc and was assumed to be a lumbar disc herniation. The ventral dural line was divided into two near the disc herniation such that the herniation existed between these lines. The cauda equina was compressed by the disc herniation on T2-weighted axial images (). Intradural extra-arachnoid disc herniation was diagnosed.
The lumbar spine was explored through a straight posterior midline approach. Partial laminectomy at L2-L3 revealed no disc herniation in the epidural space. We identified a disc herniation in the dura with an ultrasonography (). Durotomy revealed fragments covered with the arachnoid (). After removing the fragments, we found a hole in the ventral aspect of the dura; this hole was connected to the disc space. We did not suture the ventral dural defect to avoid the risk that threads irritate cauda equina.
The patient's leg symptom disappeared soon after surgery. One year after surgery, he has returned to a normal daily life with moderate low back pain.


A 23-year-old male presented in the emergency department of a district general hospital with an acute onset of left upper quadrant abdominal pain and a syncopal episode one hour prior to his admittance. Abdominal pain had a sudden outburst while the patient was resting at home, and there was no indication of trauma history. The patient reported one-week history of fever up to 40°C, sore throat, and malaise, as well as oral consumption of 5 to 8 tablets of 500 mg of acetylsalicylic acid within the two previous days. On initial examination he was in shock, pale, tachycardic with a heart rate of 110 beats per minute, tachypnoeic and hypotensive with a blood pressure of 90/60 mm Hg, and a fever of 38.5 °C. Physical examination revealed bilateral nontender cervical lymphadenopathy and diffuse abdominal guarding with rebound tenderness. Kehr's sign was also positive. Admission laboratory tests revealed a WBC count of 17.000 cells/μL with 53% atypical lymphocytes while the value of Hb was 9 g/dL. The patient's platelet count was normal (170.000/μL), prothrombin time (PT) was 13.8 sec (reference time: 13 sec), international normalized ratio (INR) was 1.06, and activated partial thromboplastin (aPTT) time was 30.6 sec (normal: 28–40 sec). Serum transaminases were slightly elevated with an ALT 152 U/L and AST 138 U/L. The patient's history of fever, lymphadenopathy, sore throat, atypical lymphocytosis combined with his clinical presentation of peritonitis, and hypotension in the absence of a trauma incidence was suggestive of a possible diagnosis of spontaneous splenic rupture on the basis of the underlying IM.
After initial resuscitation with 3 liters of intravenous crystalloids, an emergency abdominal CT was carried out to confirm the diagnosis. CT showed a large splenic hematoma and large amounts of fluid in all four abdominal quadrants and in the pelvis (). An exploratory laparotomy was undertaken. During laparotomy, there was a notable amount of fresh and clotted blood in the abdomen while the spleen was large and congested. It had a 5 cm capsular tear near the hilum and a large subcapsular hematoma. The enlarged, friable spleen was almost completely devoid of capsule. We performed an uncomplicated splenectomy, and after a thorough lavage of the peritoneal cavity, no other abnormal findings were revealed. The patient returned to the surgical ward hemodynamically stable, and no blood transfusion was necessary. Pathologic examination revealed an enlarged spleen 15 × 7 × 5 cm, weighing 430 gr. Microscopic examination accented an expanded red pulp and moderate follicular hyperplasia. The diagnosis of IM was later confirmed by the presence of IgM antibodies to Epstein-Bar virus capsid antigen (VCA). The patient recovered uneventfully and was discharged on the 7th postoperative day.


A 34-year-old male was referred to our hospital with a history of decreased vision as a result of recurrent bilateral posterior uveitis. He had repeated recurrences in spite of systemic corticosteroid therapy. At presentation to us, his best corrected visual acuity (BCVA) was 6/36, N36 in the right eye and 6/9, N6 in the left eye. Slit lamp examination revealed a normal anterior chamber and complicated cataract in the right eye. On fundus evaluation, both eyes revealed bilateral disc edema and vitritis and exudates in the macula in the right eye. He was not a known diabetic. Laboratory investigations for tuberculosis, collagen vascular diseases, and other infectious etiologies including human immunodeficiency virus were within normal limits except serum angiotension converting enzyme values which were borderline high. Treatment was initiated with MMF (2 g/day) [] along with tapering doses of oral prednisolone (1 mg/kg/day) in view of the recurrent nature of the uveitis. The lesions resolved completely with treatment. He underwent cataract surgery in both eyes 6 months later under cover of MMF (1 g/day) and systemic corticosteroids. Postoperative period was uneventful. Four months later, he had a recurrence of fundus lesions and was started on oral prednisolone (60 mg/day) and his MMF dose was doubled to 2 g/day by his local ophthalmologist. He came to us within a month with extensive skin lesions over his right neck and upper thoracic region which was clinically diagnosed as herpes zoster (Fig. ). MMF was stopped and oral acyclovir (400 mg five times per day) was started. With treatment, the herpetic lesions resolved and his ocular status was stable with BCVA 6/12, N12 in the right eye and 6/6, N6 in the left eye.


A 38-year-old Japanese man with seronegative RA, diagnosed in the year 2004 by using the American College of Rheumatology (ACR) 1987 criteria, had received sulphasalazine and methotrexate (MTX) therapies before he visited our institute and was started on regular low-dose prednisolone therapy. Despite these therapies, his RA disease activity remained high (tender and swollen joints at the knees and ankles; patient global assessment score, 48/100 mm; C-reactive protein (CRP), 3.37 mg/dL; erythrocyte sedimentation rate (ESR), 48 mm/hour; matrix metalloproteinase-3, 1531 ng/mL; Disease Activity Score 28-ESR, 4.57). The patient was obese (175 cm, 95 kg, BMI (body mass index): 31.0); however, he neither had a history of other medical problems (no viral infection and a non-carrier) nor had he ever received surgical intervention. He did not require intra-articular steroid injection. IFX at a dose of 3 mg/kg (total dose, 285 mg) was introduced in addition to MTX (10 mg weekly), prednisolone (5 mg daily), and folic acid (5 mg weekly). He suffered no immediate adverse effects and experienced excellent pain relief in his knees and ankles the day after his first infusion of IFX, but six days after infusion he presented with a fever of 40°C, chills, and polyarthralgia including the knees and ankles that persisted for two days, and he was admitted to hospital.
At admission (day 0, eight days after his first infusion), his body temperature was 40.6°C and he was tachycardic (107 beats/minute) with 104/68 mmHg blood pressure. His heart sounds were normal and he had no visible rash. The affected joints (knees, ankles, and right wrist) were swollen, painful, and warm. There was no evidence of a primary source of infection. Laboratory data showed marked acute inflammation (CRP, 31.0 mg/dL; white blood cells, 19,200/mm3) and pre-disseminated intravascular coagulopathy and shock (prothrombin time (PT), 62.2%; activated partial thromboplastin time (APTT), 45.1%; fibrinogen, 804 mg/dL; serum fibrin/fibrinogen degradation products (FDP), 14.7 μg/mL). Findings from chest computed tomography (CT) (Figure ), urine smears, and cultures were all normal, and he had no symptoms involving the abdomen or pelvis. Fluid drawn from the knees was purulent and its smear revealed numerous gram-positive cocci arranged in chains, which were subsequently identified as S. pneumoniae at day five. He was clinically diagnosed with bacterial septic polyarthritis. Emergency surgical joint lavage and drainage was then performed at the knees, along with needle aspiration and lavage of the ankles and right wrist just after admission (day 0). He was treated with intravenous antibiotic therapy with cefazolin (2 g twice daily at days 0 and one) and vancomycin (1 g once daily at days one and two). At day two, a whole body CT showed auxocardia and hydrothorax, but no pneumonia or other abnormal findings (Figure ). Two blood cultures taken at day 0 revealed penicillin-sensitive S. pneumoniae (minimal inhibitory concentration of penicillin G ≤ 0.03 μg/mL) with no penicillin-resistant strains at day two. He was switched to ceftriaxone (2 g twice daily at day two and thereafter for a total of 29 days). He made a rapid and sustained recovery with intensive rehabilitation. He was discharged on day 37 with 10 mg weekly of MTX, when he returned to his preadmission state (Figure ).


A 59-year-old Caucasian male patient was referred to our department in March 2007 for mandibular evaluation. In April 2006, when the patient was completely healthy, he underwent extraction of first left mandibular premolar with failed endodontic treatment () and two intraosseous implants placement in the left mandibular molar region one month after. Dental implants were placed using a two-step process, and, six months after (October 2006), were uncovered and restored, uneventfully ().
The patient received the diagnosis of MM in February 2007 (IgA lambda, Durie and Salmon's stage III A, ISS 3). When MM was diagnosed, the patient showed widely skeletal affection, including cranial calotte, thoracic and lumbar vertebral spine. Calcium, creatinine, and hemoglobin were in normal levels. The hematologist referred the patient to our department in March 2007 because of mandibular pain and swelling. Clinical examination revealed left mandibular cortical expansion without bone exposure or fistula in the affected area. No labial paresthesias were referred. A panoramic radiograph showed a large radiolucency adjacent to the left implant and second left mandibular molar (). A diagnosis of mandibular involvement of MM was proposed. As induction therapy, the treatment included six alternating cycles of vincristine, carmustine, melphalan, cyclophosphamide, prednisone (VBMCP) and vincristine, carmustine, doxorubicin and dexamethasone (VBAD) (each five/four weeks, resp.), ending in July 2007. The patient developed a very good partial response, without plasmatic cells in bone marrow and monoclonal pick in electrophoresis, but with positive immunofixation in serum. Consolidation therapy consisted in melphalan, alpha interpheron 2b, and autologous stem cell transplantation, carried out in October 2007. The patient also received 17 doses of Zoledronic Acid (4 mg/dose, once per month). A new panoramic radiograph performed seven months after starting therapy revealed a complete regression of the mandibular lesion (), supporting previous diagnosis of mandibular involvement of MM.
The patient presented again in September 2008 complaining of discomfort in the left mandibular molar area. Left inferior labial paresthesia was referred. Intraoral examination revealed that the alveolar bone adjacent to the left implant was exposed with a purulent discharge (). The panoramic radiography provided poor diagnostic information, showing only low-diffusion radiolucency in the affected area (). Computerized tomography revealed low- and high-density areas in the alveolar process involving left mandibular implant and giving an aspect of necrotic bone (). Treatment consisted of simultaneous amoxicillin (4 gr/day) and clavulanate (250 mg/day) with chlorhexidine mouthwash. A biopsy of the lesion performed shortly thereafter showed bone necrosis with superficial contamination with oral organisms. Electrophoresis was absolutely normal. No evidence of MM was detected. A diagnosis of BRONJ was made. After 3 months, the pain did not disappear and bone exposure had not reduced (). Then, the mobile fragment of exposed necrotic bone including left implant was removed under local anesthesia (). The specimen was submitted for microscopic evaluation, and diagnosis was bone sequestrum with microbial colonies of Actinomyces.
After 1 month, the pain had almost completely disappeared. The lesion was asymptomatic and soft tissues healed completely. At present, the patient is still under antineoplastic therapy (bortezomib and dexamethasone) for refractary vertebral MM, but zoledronate therapy has been discontinued.


A 29-year-old woman (gravidity, 1; parity, 0) was referred to our hospital at 28 weeks of gestation because of KTS complicating her pregnancy. She had been diagnosed with KTS at the age of 3 and had not received any treatment because of the absence of symptoms. Her right leg was swollen and hypertrophic and had multiple venous varicosities. She did not have a history of thrombosis or hemorrhage. Although her pregnancy had been uncomplicated until 28 weeks of gestation, she noticed prominent right vulvovaginal varicosities and new varicosities in the right lower abdomen.
On hospital admission at 35 weeks of gestation for planned delivery, physical examination showed prominent hypertrophy and multiple venous varicosities of the right leg as well as massive (15 × 10 cm) vulvovaginal varicosities (). The circumferences of the thigh, calf, ankle, and knee were 2, 15, 11, and 10 cm, respectively, larger on the right side than on the left side. Laboratory studies revealed a normal coagulation profile (PT, 11.6 sec; APTT, 31.7 sec). The ultrasound study revealed no obvious AVMs and varicosities around the uterus.
At 35 weeks of gestation, systematic MRA was performed to detect unusual AVMs. Abdominal and pelvic MRAs revealed hemangiomas and varicosities in the right leg, communications among the varicosities in the right lower abdomen, and abnormal vessels near the anterior superior iliac spine as well as unusual varicosities in the pubic region (Figures –). In her left lower abdomen, the hemangioma associated with the inferior epigastric vein communicated with the lesions near the left anterior superior iliac spine (). MRA of the bronchial tube and spine showed no obvious AVMs. Although obvious AVMs and varicosities were not noted on the uterine surface, the possibility of spread of the venous lesions to Retzius space and the area near the rectovaginal septum was considered.
On the basis of these findings, we decided to deliver her baby by cesarean section. For prophylaxis against thrombosis, she wore elastic stockings, and heparin calcium injection was planned after the operation. At 37 weeks of gestation, the patient underwent a cesarean section under general anesthesia by a Pfannenstiel incision to avoid rupturing any vascular anomalies at the surface of the right lower abdomen and upper pubic region. No abnormal vessels were noted around the uterine incision during the operation. A female baby weighing 2865 g was delivered; her Apgar scores were 5 and 6 at 1 and 5 min, respectively. No abnormal finding indicative of neonatal KTS was seen. The estimated blood loss including amniotic fluid was 1276 g and no blood transfusion was required.
The patient's postoperative course was unremarkable. To prevent thromboembolic disease, low-dose heparin (10,000 U/day) injection was administered for 3 postoperative days. The patient was discharged with her baby in good health. The prominent vulvovaginal varicosities shrunk considerably one month later (), although the vascular anomalies in her right leg showed no changes.


A 38-year-old woman with an eating disorder and depression became light-headed and fell, spilling boiling water from a kettle on herself at home. She was treated for 3 days at a local medical clinic and was then referred to the Plastic and Aesthetic Surgery Department, Kitasato University Hospital, 4 days postburn. The patient sustained partial and full thickness burns over 5% of her total body surface area: the left buttock () and the right posterior thigh and calf (). The patient was 158 cm tall with a body weight of 33 kg; thus, her BMI (body mass index) was 14. Examination of other body systems revealed that they were normal. Laboratory data results were within normal limits except hemoglobin 9.9 g/dL, total protein 6.2 g/dL, and albumin 3.0 g/dL in her blood serum. Surgical treatment and alimentation with hospitalization was planned, but consent could not be obtained. We, therefore, chose to treat her at our outpatient clinic. Consent for hospitalization was obtained 24 days postburn, and the patient was admitted. But the patient strongly desired to leave the hospital as soon as possible, so she was discharged on postburn-day 39. Burn wound infection occurred on around day 20, but the infection was controlled with silver sulfadiazine and povidone-iodine gel. On day 53, she experienced dyspnea and lower-limb edema and returned to the hospital, whereupon, pleural effusion was observed on the chest X-ray (). Laboratory data results were: hemoglobin 7.9 g/dL, total protein 4.8 g/dL, and albumin 2.7 g/dL. The pleural effusion was treated with a colloid solution and a diuretic agent with hospitalization (). The operation for the burn wound was performed using a sequential excision and split-thickness skin graft from the right buttock on postburn-day 76 and lasted 2 hours. The burn covered 5% of the patient's body surface area. But because of the skin graft on the left buttock and posterior right thigh, the patient required bed rest and splinting her right leg for 7 days; afterwards, the skin graft stabilized.
The burn wounds were successfully covered with skin grafts and treated with petroleum jelly. Rehabilitation was given for 1 week thereafter, and the patient was discharged on postburn-day 95. By postoperative-month 8, the patient was satisfied with the burn wound scars, skin grafts, and donor sites ().


A 35-year-old male patient with a 10-year history of HIV presented to the emergency room complaining of 3 months of progressive dyspnea and 2 hours of sudden chest pain and profuse diaphoresis. His most recent CD4 count on record was 154 cells/mL with a HIV viral load of less than 40 copies/mL. He had been taking zidovudine-lamivudine and efavirenz for the past 8 years. On physical examination, he was afebrile, anicteric, and without rash. His arterial pressure was 70/40 with a heart rate of 120 beats per minute. There was no prominent lymphadenopathy. The lungs were clear, but on cardiac auscultation a grade IV systolic murmur was auscultated at the left sternal border. On abdominal examination, a pulsatile epigastric mass was palpated. Cardiac enzymes were not elevated. The EKG was consistent with left ventricular hypertrophy without signs of cardiac ischemia. A chest radiograph demonstrated mediastinal widening () and prompted a 3D computed tomography of the aorta (Figures and ), which revealed an aortic aneurism extending from the ascending aorta to the iliac vessels. Further laboratory studies were notable for a strongly positive Venereal Disease Research Laboratory (VDRL) titer of 1 : 128 and a positive fluorescent treponemal antibody absorption (FTA-ABS) test. The patient denied any knowledge of prior syphilis infection or testing. The patient's aortic aneurysm was presumed to be secondary to syphilis. Waiting for cardiovascular surgery consultation, during the following 24 hours, the patient was started on aqueous crystalline penicillin and volume resuscitated with a crystalloid infusion. During the second hospital day, the patient developed sudden abdominal pain and hypovolemic shock. He died 30 minutes later despite resuscitation. An autopsy was performed on the following day, and 3500 cc of blood was encountered within the peritoneum as well as a ruptured abdominal aneurysm. On histopathological examination of the aorta, the adventitia showed focal cellular infiltrates with a predominance of lymphocytes and the vasa vasorum demonstrated thickening of the intima () strongly suggesting the diagnosis of a syphilitic aortic aneurysm.


A 38-year-old Caucasian woman presented to our hospital with two months of epigastric abdominal pain associated with bloating. She denied nausea or vomiting, history of acid reflux, constipation, or diarrhea. She had no known trauma. She affirmed complaints of fatigue and decreased appetite but denied fevers, chills, night sweats, or weight loss. She denied a history of liver or blood disorders.
The initial evaluation revealed a jaundiced young woman with abdominal ascites and trace lower-extremity edema. Laboratory studies revealed elevated levels of gamma glutamyl transferase (GGT), total bilirubin, and CA-125. A computed tomography (CT) scan with oral and intravenous contrast of the abdomen and pelvis was performed, demonstrating a heterogeneous appearance of the liver and caudate lobe hypertrophy, ascites, and a cystic mass in the right ovary. The liver abnormalities described were thought to be consistent with diffuse metastatic involvement of the liver, and the ovaries were thought to be a possible primary source. Our patient then underwent laparoscopic oopherectomy and liver biopsy to evaluate for underlying malignancy. No evidence of either ovarian or hepatic malignancy was found, but the liver biopsy revealed fibrous obliteration of the central hepatic veins and patchy areas of sinusoidal dilatation diagnostic of hepatic vein obstruction, or BCS. A triphasic CT scan of the liver with three-dimensional reconstruction confirmed occlusion of the hepatic veins and associated ascites, splenomegaly, and abdominal varices.
Our patient's medical history was notable for one episode of unexplained syncope one year prior and migraine with aura. Medications included warfarin and aspirin. Our patient was married and lived with her husband. She worked as a busy executive, frequently taking international flights. She had never smoked and she drank one or two glasses of wine per week. The only family history of a thrombosis was in a paternal aunt with a deep vein thrombosis in the setting of a long car ride. Our patient had no family history of cancer or other blood disorders.
Laboratory findings revealed a white blood cell count of 3640 K/μL, hemoglobin of 11.1 g/dL, mean corpuscular volume (MCV) of 80fL, and a platelet count of 252,000 K/μL. Erythropoietin level was 22 mIU/mL (normal is 4.1 to 19.5), international normalized ratio was 1.4 on warfarin therapy, ammonia 45 Umol/L (10 to 47 Umol/L), total bilirubin 3.1 mg/dL, direct bilirubin 0.6, albumin 3.7 g/dL, and alkaline phosphate 158 IU/L. Alanine aminotransferase and aspartate aminotransferase were normal. GGT was elevated to 120 U/L (12 to 48 U/L). CA-125 was 55 U/mL (normal is less than 35 U/mL). Liver biopsy results showed central hepatic veins with pericentral fibrosis and mild portal fibrosis and patchy areas of moderate sinusoidal dilatation consistent with chronic BCS.
Our patient was evaluated for a hypercoaguable state, including tests for lupus anticoagulant and anti-phospholipid antibodies, factor V Leiden, and prothombin 20210 mutation. The results of these tests were negative. Levels of anti-thrombin and protein C and S were normal. The result of one test was found from the hypercoaguable workup to be positive: JAK2 V617F mutation with an allele percentage of 36%. Complete blood counts obtained in the five years prior to her diagnosis were notable for thrombocytosis (Table ).


A 48-year-old female patient called to the Cornea Department prior to her appointment complaining of decreased visual acuity (VA) in her left eye for five days. She underwent left penetrating keratoplasty for nonspecific corneal leucoma one year earlier. Approximately 7 years earlier, she underwent left trabeculectomy twice, at a different institution. Data on the use of adjunctive antimetabolites was not available.
Visual acuity was measured using a Snellen chart. Intraocular pressure (IOP) was measured by a Goldmann applanation tonometer. Ocular examination was performed using a slit lamp biomicroscopy. Bleb leak was checked under cobalt blue slit-lamp illumination after staining with flourescein.
Over the last three months, VA decreased in her left eye from “counting fingers at one meter” to “hand motions,” the IOP decreased from 17 mmHg to 3 mmHg. The nuclear sclerosis (grade III) and posterior subcapsular cataract progressed to a mature cataract (). Additionally, three sclerotomies (superonasally, superiorly, and superotemporally) and two iridectomies (superonasally and superotemporally) were noted. The conjunctiva covering the superotemporal sclerotomy was avascular, flat, and partially lost (). The corneal graft was clear and secured with 16 interrupted 10-0 nylon sutures. B-scan ultrasonographic evaluation of the posterior segment was normal. Axial length was 30.48 mm. After an unsuccessful first attempt to identify a bleb leak with 2% fluorescein, we were able to observe the Siedel positivity with a heavily painted bleb and we made the diagnosis of late-onset filtrating bleb leak () which was a point leak but not an oozing.
In her right eye, the penetrating corneal graft was clear (for 27 years), distant corrected visual acuity was 0.5 (in decimal notation), IOP was 12 mmHg (with dorzolamide and timolol maleate combination, (Cosopt, Merck & Co. Inc., NJ, USA)). Axial length was 30.20 mm. Posterior segment was consistent with degenerative myopia.
In order to eliminate the risk of infection and obtain adequate IOP, surgical intervention was scheduled to fix the leak. The surgical procedure was performed under topical and subconjunctival anesthesia. The avascular conjunctiva was excised together with 1 mm of healthy conjunctiva. An atrophic and irregular scleral flap was exposed. The surrounding relatively healthy conjunctiva was firmly attached to the underlying sclera, causing difficult subconjunctival dissection. As the scleral flap could not be fixed properly and adequate sclera for flap creation was not available due to the firm subconjunctival scarring and degenerative myopia-associated thin sclera, the decision was made to overlay the atrophic and partially absent scleral flap with a processed human pericardium patch graft (Tutopatch, Tutogen Medical GmbH, Neunkirchen am Brand, Germany) followed by conjunctival advancement. Four interrupted 10-0 nylon (Daclon, Smi, St. Vith, Belgium), sutures were used to secure the graft. The tightness was adjusted so as to allow aqueous drainage underneath the graft and not to seal it completely. After a large subconjunctival dissection, the conjunctiva was advanced and closed with multiple 10-0 nylon sutures. The limbal side was closed with running mattress suture, and watertight wound closure was obtained with minimal tension. Ofloxacin (Exocin, Allergan Pharmaceuticals Inc., County Mayo, Ireland) and dexamethasone sodium phosphate (Maxidex, Alcon Laboratories Inc., TX, USA) were used eight times per day postoperatively for one week and one month, respectively.
The IOP stabilized at 16 mmHg without medication, and a functioning filtrating bleb was formed (). After four months, phacoemulsification and a +7.00 D intraocular lens (Acrysof MA60AC, Alcon Laboratories Inc, TX, USA) implantation were performed. Visual acuity did not exceed 0.1 (in decimal notation) due to degenerative myopia. Although macular edema was not detected after the cataract surgery, hypotony-associated macular edema sequela could have been another cause for low final visual acuity. The corneal graft remained clear at her 6-month followup, after the phacoemulsification ().


A 63-year-old previously fit and healthy woman, presented to the Accident and Emergency Department with acute onset stridor and difficulty breathing. She had been feeling unwell for the previous two days and had put her symptoms down to a common cold. She had a background of hoarse voice for more than a year for which she never sought medical attention. On arrival to the department she was found to be stridulous and in respiratory distress. She received immediate airway management in the A&E with one millilitre of Adrenaline mixed with four millilitres of normal saline nebulised and 200 milligrams of Hydrocortisone intravenously. On examining her larynx with a flexible nasal endoscope, a limited view of the glottis was obtained secondary to significant supraglottic oedema. There were no signs of systemic sepsis although her initial haematological and biochemical investigations revealed nonspecific inflammation with elevated levels of C-reactive protein (25.6 mg/L) and erythrocyte sedimentation rate (48 mm/h). She did not respond to conservative treatment and was, thus, intubated with a size 6.0 Fr endotracheal tube with the help of the ENT team.
Direct laryngoscopy at the time of intubation revealed a mass over her right glottic area which was almost completely obscuring the laryngeal inlet. She was then transferred to the intensive care unit, where she remained sedated and ventilated. She was treated with intravenous antibiotics for a suspected supraglottitis given her clinical picture and the appearance of her larynx.
Over the next 48 hours, it became apparent that there was no demonstrable leak around her endotracheal tube cuff despite medical management. A decision was made to perform a microlaryngoscopy and to attempt extubation in theatre.
Suspension laryngoscopy was performed with supraglottic jet ventilation, and a bulging mass was then seen protruding over her right subglottic area and partially obstructing her laryngeal inlet (). She was reintubated, and her airway secured with a surgical tracheostomy. Her larynx was then revisited, and after careful examination, a diagnosis of a laryngopyocoele was made. The laryngopyocoele was de-roofed using long laryngeal biopsy forceps, and 10 millilitres of frank pus was immediately aspirated from the area. The excess mucosa comprising the remaining laryngocele was excised and sent to histology. Histological examination of the specimen revealed necrotic and infracted tissue with a very slight possibility of malignancy. The pus aspirated from the cavity grew Staphylococcus aureus sensitive to Penicillin and Flucloxacillin. The patient then returned to ITU and made a very swift recovery on intravenous antibiotics, leaving ITU the next day.
A CT scan was performed following the procedure revealed that the mass was adequately drained, and no external component was identified ().
The patient underwent a repeat microlaryngoscopy and biopsy of the area 14 days later, which showed good healing (). Further histological analysis revealed no evidence of malignancy. She was, therefore, decannulated and discharged home shortly after that. On reviewing her larynx in clinic four weeks later, the inflammation appeared to be resolving with only a small amount of granulation tissue in the supraglottis. There was also significant subjective improvement in the voice quality.


Our patient, a 22-year-old male with cystic fibrosis, was admitted as an inpatient for scheduled intravenous antibiotic therapy of chronic pseudomonas airway infection. He was in good general health with 66 kg body weight, 184 cm height (BMI 19.5 kg/m2), and a constant Chrispin-Norman score (CN score = 7). Patient history included well-managed lung and gastrointestinal manifestations of CF, exocrine pancreatic insufficiency, impaired glucose tolerance, allergic rhinopathy, and nasal polyps. Occasional spare time sports activities were reported, although recurring air-way infections frequently prevented exercise. The treatment plan during the hospital stay included physiotherapy and supervised physical exercise.
On day 6 in hospital, the patient suddenly complained about bilateral pain and swelling of upper arm muscles. Laboratory analysis revealed a marked elevation of previously normal CK, LDH, and CK-MB levels (). Serum creatine (<0.9 mg/dL) and glomerular filtration rate (150 to 181 mL/min/1.7m2) remained normal; C-reactive protein was negative (<0.10 mg/dL). The slightly elevated leukocyte count (<12.060/μL) was clinically attributed to the presence of chronic airway infection and sinusitis.
As clinical symptoms persisted, an ultrasound examination was performed which revealed a diffuse bilateral increase in echogenicity limited to the biceps brachii, brachialis, and brachioradialis muscles (). Normal echogenicity was seen in the pronator teres, flexor carpi radialis, triceps brachii, and deltoid muscles. Doppler ultrasound showed no signs of hyperperfusion in the hyperechogenic tissue. Deep vein thrombosis and thrombophlebitis were ruled out sonographically. MRI of the right elbow joint performed the next day confirmed the ultrasonographic diagnosis of edema confined to the described muscles with marked homogeneous signal increase on T2 TSE in the absence of other pathological signal alterations (). The affected muscles showed normal signal intensity on T1-weighted images. Whole-body MRI scanning with coronal T2 TIRM and T1 TSE sequences did not reveal any other abnormal findings apart from a previously diagnosed pansinusitis (not shown).
Upon interrogation, the patient admitted nonsupervised dumbbell training for more than four hours in his room one day prior to the onset of clinical symptoms, particularly exercising his “biceps” bilaterally. With immobilization and rest, clinical symptoms and laboratory findings normalized within days, so that no further imaging studies were performed. After completion of antibiotic infusion therapy, the patient was discharged in good health. Normal LDH and CK serum levels were seen on followup 4 months later (), and, with moderate levels of physical exercise, no recurrence of symptoms occurred.


A 69-year-old man was admitted to a hospital in the South of Brazil, with weight loss, weakness, and difficulty to swallow. In the last 4 months, he had anorexia, asthenia, and difficulty to swallow any food. When he tried to eat, he felt nausea and sometimes vomited. He had lost 7 kg. He denied fever or other symptoms. He was an active smoker that had smoked 100 pack-years. He did not abuse alcohol or other drugs. He had arterial hypertension, type 2 diabetes mellitus with HbA1c: 6.5% one month earlier and mild chronic obstructive pulmonary disease. He had presented three no disabling ischemic strokes. He was taking hydrochlorothiazide 25 mg, simvastatin 20 mg, and acetylsalicylic acid 100 mg, once a day; and captopril 50 mg TID. His father died of sudden death at 83 years old, and his mother died of stroke at 80 years old. His brother died of esophageal cancer with 55 years old. He lived with his wife. His physical examination revealed an alert, oriented man, with blood pressure: 120/70 mmHg; heart rate: 72 bpm; respiratory rate: 20 rpm; axillary temperature: 36°C; pulse oximetry: 95%; weight: 33.5 kg; height: 1.53 m; BMI: 14.3 kg/m2. There were several lymph nodes the cervical region, the larger one with 1.5 cm, and digital clubbing. Other aspects of his physical examination were normal. Two months before admission, chest radiography showed signs of chronic obstructive pulmonary disease, micronodules and linear opacities in the upper lobes, and a small consolidation in the lower left lobe. The initial laboratory evaluation revealed hemoglobin = 8.6 g/dL (normal range: 13–14.2 g/dL); VCM = 88.9 fL (80–100 fL); leukocytes = 7220/μL (4000–10000/μL); neutrophils = 2758/μL (1800–7500/μL); eosinophils = 606/μL (40–500/μL); basophils = 87/μL (<100/μL); monocytes = 477/μL (120–1000/μL); lymphocytes = 3292/μL (1000–4000/μL); platelets: 409000/μL (150–400 × 103/μL); serum sodium: 134 mEq/L (135–145 mEq/L); potassium: 5.2 mEq/L (3.5–5.1 mEq/L); creatinine: 0.64 mg/dL (0.5–1.2 mg/dL); ureic nitrogen: 21 mg/dL (5–20 mg/dL); calcium: 7.8 mg/dL (8.5–10.4 mg/dL); albumin: 3.0 g/dL (3.5–5.0 g/dL); ALT: 17 U/L (<31U/L); glucose: 94/mg/dL (70–99 mg/dL). An upper gastrointestinal endoscopy was normal, and a nasojejunal tube was inserted. The patient remained with the same symptoms, receiving food and liquids through a nasojejunal tube. In the sixth day after admission, after being submitted to chest and abdomen computerized tomography, he developed fever (39.5°C), confusion, blood pressure 160/110 mmHg, heart rate: 120 bpm, pulse oximetry: 95%. He received intravenous dipyrone. A few moments later, his blood pressure dropped to 80/40 mmHg, and intravenous saline solution was infused. A blood sample to measure serum ACTH and cortisol was drawn. In the next two days, he had no fever, and two samples of blood culture were negative. In the following days, the patient developed severe hyponatremia (sodium = 122 mEq/L and 119 mEq/L), and postural hypotension was identified (supine resting = 122/60 mmHg and standing = 100/60 mmHg). The results of computed tomography of chest and abdomen are shown in Figures and . Serum cortisol and ACTH were, respectively, 1.03 μg/dL (4.3–22.4 μg/dL) and 626 pg/mL (12–46 pg/mL), and serum TSH was 8.06 μU/mL (0.35–5.5 μU/mL). The patient received intravenous hydrocortisone, and hydrochlorothiazide was discontinued. Electrolyte abnormalities, anorexia, and difficulty to swallow are resolved. A biopsy of the left adrenal gland showed signet-ring cell adenocarcinoma with areas of necrosis. In the immunohistochemical study, the cells were positive for CK7, TTF-1, and CEA, and negative for CK20, vimentin, CA19.9, CD10, prostatic specific antigen, and thyroglobulin. Therefore, the diagnosis of adenocarcinoma of pulmonary origin was made. Bone and brain metastases were not evident in bone scintigraphy and brain computerized tomography. A few days later, the patient was switched to 5 mg oral prednisone and 0.1 mg of fludrocortisone and was discharged to his home. One month after initiating glucocorticoids, he came to the outpatient oncology division. He was eating normally, his weight increased to 40.1 kg, but he refused the palliative chemotherapy offered.


A 69-year-old man with CTEPH was scheduled for PTPA; a new treatement strategy originally reported in 2001 [] in USA and now developing in Japan []. On medical checkup at 52 years of age, he was pointed out to have cardiomegaly. At 54, he noticed exercisintolerance and dyspnea and was diagnosed to have pulmonary hypertension with cardiac catheterization and multiple stenoses and occlusions of his peripheral pulmonary arteries by pulmonary angiography. Pulmonary artery endarterectomy was not indicated because most of the lesions were not located at the proximal pulmonary arteries. He started to take warfarin, ticlopidine hydrochloride, and nifedipine. At 59, he had dyspnea at rest and was diagnosed to have protein S deficiency and CTEPH. His symptom developed gradually, and amblatory oxygen inhalation therapy (3 L/min) and oral intake of sildenafil citrate were started at 68 year, and PTPA was scheduled at 69.
On admission to our hospital, the patient's New York Heart Association functional status was class III with recent worsening of dyspnea and apparent leg edema. His SpO2 was 94% with 3 L/min of oxygen inhalation, cardiothoracic ratio on chest X-ray was 60%, and his brain natriuretic peptide (BNP) level was 390.3 pg/dL. Transthoracic echocardiography revealed right ventricular enlargement with mean pulmonary artery pressure of 42 mmHg. The diameter of the inferior vena cava (IVC) was 20/18 mm.
Initial PTPA was performed for his left pulmonary artery. Seven peripheral regions of his left pulmonary artery was dilated by the balloon. At the end of the procedure, his pulmonary artery pressure was 91/27 mmHg, and he was admitted to the intensive care unit. On admission to the intensive care unit (day 0), his SpO2 was 95% with 40% of oxygen inhalation with no complain of dyspnea. On day 1, his chest X-ray and computed tomography revealed localized consolidation on his left lower lobe (). On day 2, consolidation on chest X-ray developed in accordance with his complication of dyspnea. Because his SpO2 decreased to 70% with 10 L/min of oxygen inhalation using reservoir mask, NPPV was applied.
The initial setting of NPPV was CPAP mode with 10 mmHg inhaling 100% of oxygen. Applying NPPV, SpO2 increased up to 90%. CPAP was gradually decreased and was 5 mmHg on day 5. NPPV was finally discontinued on day 16. Although mean pulmonary artery pressure was unchanged as assessed by postoperative cardiac catheterization, his brain natriuretic peptide level decreased from preoperative 390.3 to postoperative 44.3 pg/dL. In addition, total pulmonary resistance decreased from preoperative 18 to postoperative 9.6 wood unit·m2. The patient was discharged on day 25 with SpO2 of 95% on 5 L/min of oxygen inhalation and NYHA class II status.


On July 15, 2005, a 58-year-old man presented with sudden onset of severe pain and blurred vision in his right eye, without significant irritation, of one day's duration. The eye pain was alleviated after half an hour; however, visual acuity was significantly decreased. On examination, visual acuity in the right eye was HM/60 cm with accurate light projection in every direction; best-corrected visual acuity in the left eye was 0.6 (+10.00/−3.50 × 45). Intraocular pressure (IOP) was 7.8 mmHg in the right eye and 15.0 mmHg in the left eye. Examination of the right eye showed conjunctival congestion, a brown protruding bulge occupying the entire nasal quadrant with a clear boundary seen at the outer edge of the corneoscleral limbus. The cornea was transparent, the anterior chamber was shallow and about 1/3CK, the pupil shifted up and dwindled with an apparent discoria, the light reaction disappeared, and the fundus was not clear (). The right eye vitreous had an obvious opacity with posterior vitreous detachment (PVD) () by B-scan. Examination of the left eye showed a pupil shifted up in a lip-like shape, with a defect at the top of the iris and a flocculent vitreous opacity. The boundary of the optic disc was clear. There was mild sclerosis of the fundus artery and no macular reflex, and the retina was procumbent. In 1967, the patient had undergone cataract surgery in a local hospital due to congenital cataracts in both eyes. He has been wearing spectacles as treatment after the operation without discomfort. There was no past history of trauma in either eye. Apart from a 10-year history of hypertension, he was otherwise fit. A diagnosis was made of anterior sclera staphyloma and vitreous hemorrhage in the right eye.
On July 19, 2005, the patient underwent anterior scleral staphyloma resection and repair with sutures placed, plus vitrectomy, coreoplasty, and endophotocoagulation under general anesthesia. The bulbar conjunctiva around the anterior staphyloma on the superior nasal quadrant was separated; the prolapsed uveal tissues and vitreous body were pruned during operation (). We observed an apparent thinning and perforated sclera at the 12:00–2:30 position and iridoleptynsis on the superior nasal quadrant. Subsequently, we placed an apposition suture and covered the wound using the anadesma and conjunctiva (). We performed a vitrectomy with three incisions at the 7:00, 10:00, and 11:00 o'clock positions (normally at 8:00, 10:00, and 2:00 o'clock positions) of 3.5 mm behind the corneoscleral limbus and cut the portion of the shifted-up iris. We noticed a circular pupil, the hemorrhagic vitreous opacity, and the discus opticus with normal size and shape (). In addition to the inferior nasal quadrant, scattered patchy hemorrhage points were seen in the remaining parts of the retina. The patient was then subjected to laser photocoagulation followed by local conventional anti-inflammatory and mydriasis treatment after the operation. The removed specimens were confirmed as anterior staphyloma on histological examination (). Reexamination three months after surgery showed a corrected visual acuity of 0.3 (+10.00/−4.50 × 60) and IOP of 14.0 mmHg in the right eye. The right cornea was transparent, the stitches at the corneoscleral junction on the superior nasal quadrant were eutopic, the anterior chamber was deep, and the pupil was circular (); the light reflex was slow, and the retina was procumbent by Panoramic 200 (). The patient's last visit was four years after surgery with the clinical findings identical to those at three months after surgery.


Patient 1 is an 11-year-old male who presented with a 3-year history of postprandial vomiting and abdominal pain, 1.5 kg weight loss, and plateau in height. Weight at presentation was 28.3 kg (7th percentile), height 144.5 cm (54th percentile), and BMI 13.5 (<5 percentile). On examination only asthenia was noted. The complete blood count, electrolytes, erythrocyte sedimentation rate, stool elastase, and 72-hour fecal fat were normal. Stool examinations for H. pylori antigen, hemoccult, bacteria, and viruses were negative.
An esophagogastroduodenoscopy (EGD) with a standard endoscope showed retained food and liquid in the stomach despite 18 hours of fasting. Visual examination showed thickened mucosal folds along the lesser curvature and stenosis of the pylorus with no visible ulcers. The patient was started on lansoprazole 30 mg daily.
Rapid urease test (Campylobacter-like organism, CLO test, Kimberly-Clark, Roswell, GA) of the gastric antrum and fundus was negative, upper gastrointestinal series (UGI) showed a narrowed pyloric channel, and computed tomography (CT) of the abdomen/pelvis and pyloric ultrasound were normal. The patient became asymptomatic, and the lansoprazole was stopped after 6 months of therapy.
Seven months later, the patients symptoms recurred, and lansoprazole, 30 mg/day, and nutritional supplementation were started. One month later, the patient lost 2.5 kg. Repeat EGD showed stenosis of the pyloric sphincter; lumen diameter estimated at 3 mm (), gastrin was 27 pg/mL (normal 0–100), and a repeat CLO was negative. Pathology from the pyloric channel showed necrotic tissue with neutrophilic and fibrinous exudates; adjacent gastric mucosa showed active inflammation. The patient was admitted for high-dose proton-pump inhibitor treatment (pantoprazole 40 mg IV bid) and parenteral nutrition. UGI showed a narrow pyloric channel with ulceration and cicatrization (). Barium remained in the stomach after 18 hours.
An EGD eight months after initial presentation showed a small pylorus that could not be traversed with a 6 mm endoscope (Pentax EG1870K, Pentax Medical, Montvale, NJ). Histology of the duodenum showed benign mucosa containing a lymphoid follicle and no active inflammation. The antrum showed mild active gastritis. Serum studies and colonoscopy were not suggestive of Crohn's disease. The patient was referred for surgical intervention. In the operating room a dilated stomach and a 1–1.5 cm thickened area of the pyloric channel were observed. A Jaboulay pyloroplasty was performed. The resected section showed an ulcer, with normal smooth muscle and hypertrophic nerves. The surface epithelium was replaced by granulation tissue. The lamina propria showed mild active chronic inflammation. No etiologic source for the hypertrophy was identified.
Three months following surgery, 10 months after initial presentation, the patient was asymptomatic; his weight was 41.2 kg (48th percentile) and height 148 cm (38th percentile).


Patient 2 is a 15-year-old female who presented with a 2-month history of vomiting and weight loss. Laboratory evaluation prior to referral included normal H. pylori IgG and abdominal ultrasound. A large pyloric channel ulcer was seen on UGI. Weight was 53.8 kg (50% tile), height 160 cm (35% tile), and physical exam unremarkable.
She was treated for H. pylori. The EGD demonstrated a narrowed pyloric channel () and mucosal abnormalities. The pyloric channel was dilated with CRE Wire-guided 6-7-8 180 cm pyloric balloon dilator (Boston Scientific, Natick, MA). Biopsies showed normal histology of the esophagus and antrum and no tissue from the duodenal bulb. Repeat ultrasound revealed GOO caused by thickening of the pylorus channel length 3.2 cm, thickness 1.2 cm. Biopsies were negative for H. pylori, and serum gastrin and thyroid levels were normal. Over the next 6 weeks the patient had a total of 7 diltations, each resulting in transient improvement in symptoms. Surgical referral was made following the second dilatation, and following a failed seventh dilatation she underwent a Billroth I gastrectomy with preservation of the vagal nerve. Pathology of the stenosed area showed muscular hypertrophy with no identifiable etiology. Following surgery all symptoms of GOO resolved, and follow-up EGD 18 months after surgery showed no ulcer and no scaring.


An 80-year-old woman with a previous history of breast cancer presented with an erythematous, indurated lesion on the right chest wall. She had previously undergone a radical mastectomy on the right breast 52 years earlier for breast carcinoma; additionally she was treated with radiation therapy at that time. The patient had undergone only placement of a split-thickness skin graft placed directly over her ribs as reconstruction after her initial resection. The patient has undergone routine mammographic examination of her contralateral breast, but no further imaging was performed on her ipsilateral mastectomy site since the time of her initial treatment. The patient presented to a dermatologist in August of 2007 after development of a 3 cm × 3 cm red, scaly lesion on the right chest wall at the medial aspect of the skin graft (). The patient noted that this lesion had become painful and friable with intermittent bleeding.
A shave biopsy of the skin graft was performed which revealed an invasive, moderately differentiated squamous cell carcinoma. Wide local excision of the erythematous lesion, including the underlying rib, was then performed. This specimen revealed a poorly differentiated adenocarcinoma consistent with locally recurrent breast cancer. The deep margin was positive, and the tumor was found by stains to be ER+/PR+ and Her2/neu+. A CT scan further revealed the presence of pulmonary metastases. The patient underwent local tissue rearrangement using the contralateral breast skin to close the defect, followed by chemotherapy for treatment of her pulmonary metastases ().


A sixty-seven-year-old male patient admitted to our clinic with complaints of headache and dizziness. Doppler ultrasound and digital subtraction angiography revealed 75% stenosis and kinking corresponding to the segment distal to the endarterectomy region. There was no severe stenosis in the contra lateral carotid artery. Minor stroke two months ago, the right carotid endarterectomy in 2002, and CABG in 1987 were reported in his medical history ().


A six-year-old girl from the Eastern Cape in South Africa presented with her mother to the Ophthalmology Unit at the Red Cross War Memorial Children's Hospital, with the complaint of painless, gradual loss of vision in her left eye (). This symptom began in the months subsequent to a lightning strike to her, while at home in Ngcobo, which tragically also caused the sudden death of a younger sibling and cousin. She was referred to us by the school's optometry service.
In the lightning incident, our patient sustained annular burns to the outer aspect of her right lower leg and left arm (Figures and ). Her mother described swelling across her daughter's face from right superior to left inferior, which healed without scarring. She had been admitted for the treatment of these burns for three weeks to the local All Saint's hospital. Ocular and systemic history was unremarkable. She had had a normal birth, at term, and had no prior history of serious illness, poor vision, ocular trauma, or red eye.
General examination revealed scars to the skin of the right leg and left arm, as well as a small area of hypopigmentation on the cheek below her left eyelid (). Otherwise, there were no systemic abnormalities.
Right unaided visual acuity was 20/20 with Snellen's numbers, and the left eye perceived light projection in all quadrants. There were normal ductions and versions of both eyes and a mild left esotropia, although this was estimated with poor fixation. The pupillary reactions were normal and brisk. The ocular adnexa, conjunctiva, cornea, and iris were normal. There was no evidence of blunt trauma to the eyes. The intraocular pressure in both eyes was 12 mmHg. The left lens was densely cataractous precluding fundal visualisation () and had marked anterior subcapsular vacuoles. The examination of the right eye was normal, including a clear lens. A B-scan of the left eye was normal. Potential acuity testing was not performed, as we have found it unreliable in eyes with vision worse than 20/200. TORCH serology screen was negative.
Arrangement was made for lens washout and intra-ocular lens implantation on the next available elective list. The surgery was uneventful, and a 28D intraocular lens (MA60-AC, Alcon Laboratories, Inc., Tex, USA) was implanted in the capsular bag.
On the first postoperative day, the vision was 20/20 without improvement despite preexisting corneal cylinder. The intraocular lens was well-centered in the bag and the cornea clear. Fundus examination was normal, with no posterior vitreous detachment, retinal scars, or embryological abnormalities. OCT done at one month showed no macular anatomical abnormalities. There was no residual esotropia, and the patient showed no evidence of amblyopia. Stereoacuity testing with the Stereo Fly test, as well as the Worth 4-dot testing, was also normal.


The patient is a 37-year-old right-handed Caucasian man who was in his usual state of health until December 2006 when he started to notice that his feet were cold, numb, and had a tingling sensation (described as pins and needles) from the ankles down. In a few weeks, the tingling sensation progressed up to the knees which remained stable for the next three months. In April 2007, he also noted the tingling sensation in the arms.
At time of his presentation to our pain clinic and neurology clinic in October 2007, he reported diffuse constant tingling sensation, mostly involving his arms, legs, and face, which was accompanied by sharp pains, mostly in the feet and distal legs. The pain was rated on average as an 8 on a numerical rating scale (NRS) (0 is no pain and 10 is most severe pain imaginable). He also reported episodic lower extremity allodynia to light touch and burning dysesthesia in his trunk and hands. These symptoms kept him up at night and disturbed his sleep. He began to experience symptoms of depression secondary to the relentless pain condition. He denies weakness. Physical examination at the time of presentation was significant for decreased light touch sensation and hyperalgesia to pinprick in a stocking-glove distribution. Motor strength, proprioception, and tendon reflexes were well preserved.
A nerve conduction study and electromyography was normal showing no evidence of a large fiber peripheral neuropathy or radiculopathy. We further conducted skin biopsies at the distal leg (DL), distal thigh (DT), and proximal thigh (PT) in the Cleveland Clinic Cutaneous Nerve Laboratory and carried out intraepidermal nerve fiber density (IENFD) analysis as previously described [, ]. Intraepidermal nerve fiber density was significantly reduced at the distal leg as compared to normal control (), which indicates a distal small fiber sensory neuropathy. The neuropathy etiology evaluation was significant for diabetes mellitus as revealed by oral glucose tolerance test with a 2-hour glucose level being 220 mg/dL. ESR, ANA, ENA, rheumatoid factor, ACE level, ANCAs, TSH, free T4, serum and urine immunofixation, folic acid, vitamin B12, RPR, Lyme serology, and heavy metal screen were all unremarkable.
He was diagnosed with painful diabetic small fiber sensory neuropathy and started on Glucophage. In terms of his neuropathic pain control, however, he had failed to respond to multiple pain medications, including Topiramate, Oxcarbazepine, Duloxetine, Amitriptyline, Darvocet, Tramadol, and Lidocaine transdermal patches. Gabapentin only offered minimal pain relief. Given the unsatisfactory outcomes of conservative treatment, we decided to try a lumbar sympathetic block for his pain and allodynia in the low extremities. We used the classic approach for bilateral lumbar sympathetic block at the level of L3 under fluoroscopic guidance and injected a mixture of 12 mL of 1% Lidocaine and 20 mg Triamcinolone on each side. The temperatures, monitored in the plantar surface of the big toes (LS 14000 Temperature Monitor and Skin Temperature Probe, NovaMed, NY, USA), increased significantly, and NRS pain scores decreased substantially after the procedure as shown in (initial block) and .
At his 2-month followup, the patient reported “excellent” relief of his foot pain bilaterally for over 6 weeks, but became more cognizant of his thoracic and hand pain. A decision was made to proceed with a repeat lumbar sympathetic block to further enhance the pain relief. Subsequent temperature and pain score changes were again noted as shown in (2 months) and . Management of his thoracic pain was further considered at the following office visit. At a follow-up visit 4 weeks later, the patient again reported “significant, sustained” relief of his bilateral foot pain. He felt “dramatic” improvement of the “coldness and tingling pain” with the first injection with sustained relief that was further enhanced with the second procedure.
At this point, his chief complaint became the “hypersensitive, pins and needles, burning” thoracic and abdominal pain that was described as occupying a discrete antero-/posteroarea of his thorax, roughly covering the T6 to the L1 dermatomes. Physical examination, as previously described, was significant only for bilateral lower extremity stocking-distribution decrease in sensation to light touch and pinprick. He returned 2 weeks later for a T8 bilateral paravertebral sympathetic nerve block, which was performed in the classic fashion, under fluoroscopic guidance. A solution of 1% Lidocaine 10 mL and 20 mg Triamcinolone was injected on each side. The patient was maintained on the same doses of Gabapentin and Tramadol. Upon his return to the clinic 4 weeks later, he reported “70%” relief of his thoracic pain following his T8 paravertebral block. By this point, his bilateral foot pain was 5 on the NRS three months following his second lumbar sympathetic block; therefore, a third bilateral lumbar sympathetic block was performed (, 4 months). Pain medications remained the same, and the patient was followed up in three months.
Over the next two years, the patient continued to have lower extremity neuropathic pain as described above with good, sustained pain relief after each of subsequent lumbar sympathetic blocks. The intervals of each block are shown in () with the corresponding temperature changes and the NRS pre- and postblock pain scores. He additionally underwent two thoracic paravertebral blocks at the T6 level in April and May of 2010 with good relief following each procedure. The remainder of his clinical course was uneventful.


A 31-year-old Asian woman who complained of 'loss of right side vision', headache, dizziness, nausea, paroxysmal eye pain, and blurred vision was seen in our clinic. Humphrey visual field testing with a 4-mm2 Goldmann size III stimulus (0.43°diameter) on a dim background (31.5apostilb) revealed a right homonymous inferior quadrantanopsia (Figure ). Her medical history was significant with dilatation and curettage for menolipsis and irregular vaginal bleeding two years ago. Pathological examination of her curettage specimen showed hydatidiform mole. Microscopic examination demonstrated that molar vesicles had penetrated deeply into the myometrium giving rise to extensive coagulation necrosis and residual degenerative chorionic villus (Figure ). On eye examination, the best corrected visual acuity was 20/20 OU. Pupillary responses were normal with no relative afferent pupillary defect. Extraocular eye movements were normal and she did not have any history of ocular diseases. A funduscopic examination showed no papilledema or retinal hemorrhage. Other cranial nerve functions were within normal limits. The remainder of the ophthalmologic examination was all negative.
On medical examination, an ultrasonic test of her pelvis showed 1.0 × 2.0 cm2 echogenic dots in the anterior wall of the lower uterine segment. An MRI of her brain revealed a low signal lesion at the left occipital lobe on a T1-weighted image (Figure ) and high signal mass on a T2-weighted image (Figure ). An MRI of her chest and abdomen demonstrated no evidence of systemic metastasis. Laboratory tests showed that her serum human chorionic gonadotropin (hCG) level was elevated to 78,187.5 mIU/mL. General gynecologic examination was negative. After hospitalization, this patient was treated with ten cycles of chemotherapy including intrathecal chemotherapy with methotrexate (MTX) and intravenous injection of vincristine, 5-fluorouracil (5-FU), cyclophosphamide and etoposide. After the first cycle of chemotherapy, her symptoms related to the increased intracranial pressure disappeared. Her vision field improved slightly after the second cycle of chemotherapy (Figure ), but showed no further improvement until the completion of ten cycles of the chemotherapy program. A repeat MRI of the brain demonstrated a reduction in the left occipital lobe metastasis and the associated edema. The patient was left with a permanent lesion (Figure ).


A 20-year-old man referred to surgery department of Faghihi hospital, affiliated to Shiraz University of medical science, due to painless left axillary mass for 2 months duration. His past medical history was unremarkable. On physical examination he had stable vital signs. A firm mass was palpated at the left axillary area measuring about 8 × 7 centimeter (cm). There was no evidence of hepatosplenomegaly and generalized lymphadenopathy.
Left axillary ultrasound examination showed a well-defined superficial mass with area of punctate calcification with no evidence of hemorrhage and necrosis, suggestive of an enlarged lymph node ().
The patient's lab data including complete blood count with differential and biochemical tests were within normal limits. Chest X-ray and abdominal ultrasound were normal. The left axillary mass was excised. On gross inspection, there was a firm creamy brown lymph node, measuring 8 × 7 × 5 cm with focal areas of white discoloration and hard consistency (). Histologic examination revealed follicular pattern with large nodules of mantle cells arranged concentrically around atrophic and vascularized germinal centers (Figures and ). There was also some benign-appearing bone trabeculae interspersed with lymphoid tissue ().
The diagnosis based on histologic examination was Castleman's disease, hyaline-vascular type with osseous metaplasia.


A 55-year-old Caucasian man was admitted to the Nephrology Department at our institution for ARF diagnosed in the emergency room along with left-sided, community-acquired pneumonia. He was on oral anti-diabetic treatment for uncomplicated type 2 diabetes and was a cigarette smoker. He reported no recent use of non-steroidal anti-inflammatory drugs or antibiotics.
Clinical examination revealed that his temperature was 38°C and his blood pressure was 120/60 mmHg. His urinary output was diminished and concentrated. Pulmonary examination revealed diffuse crackles of the left lung accompanied by a dry, irritative cough and exertional dyspnea. The rest of the patient's examination was normal.
Chest X-ray revealed alveolar opacities in the left lung. No sputum could be obtained for culture, but his test for Legionella antigenuria was positive. Antibiotic therapy with erythromycin and ofloxacin was initiated.
Blood tests revealed elevated serum creatinine (614 μmol/L; normal range, 62 to 106 μmol/L), blood urea nitrogen (28 mmol/L; normal range, 2.14 to 7.14 mmol/L) and C-reactive protein (360 mg/L; normal range, 0 to 3 mg/L) with leukocytosis (13 g/L; normal range, 4 to 11 g/L). No anemia or thrombocytopenia was noted, and the patient's liver function tests were normal. The patient had elevated levels of lactate dehydrogenase (408 IU/L; normal range, 135 to 225 IU/L) and creatine phosphokinase (CPK) (2000 IU/L; normal range, 47 to 171 IU/L). At room air, his arterial blood gas was pH 7.44 (normal range, 7.35 to 7.45), partial pressure of carbon dioxide was 29 mmHg (normal range, 35 to 45 mmHg) and partial pressure of oxygen was 65 mmHg (normal range, 80 to 100 mmHg) with HCO3 of 22 mmol/L (normal range, 20 to 25 mmol/L).
Analysis of the urinary sediment revealed aseptic leukocyturia (684/mm3; normal range, < 20/mm3) and hematuria (56/mm3; normal range, 0 to 10/mm3). The patient's urinary sodium was below 20 mmol/L, urinary urea was 13 g/L and proteinuria was 2.48 g/L (normal range, 0 to 0.3 g/L) with albuminuria of 0.4 g/L (normal range, < 0.03 g/L). His renal ultrasound was normal.
Although our patient's respiratory signs and chest X-ray revealed improvement with antibiotics, his ARF worsened despite saline solute infusion, and he became anuric. His serum creatinine level at day 3 was 1000 μmol/L. Hemodialysis was initiated with a central jugular catheter.
Percutaneous renal biopsy was performed at day 4, which showed acute TIN (Figures and ) with interstitial edema and inflammatory peritubular infiltrate composed of lymphocytes and plasma cells. No proliferation or deposit was noted on the 21 glomeruli examined. Under immunofluorescence, only immunoglobulin-secreting plasma cells were visible.
The patient's blood cultures were normal, his bacterial and viral serologies were negative (leptosirosis, human immunodeficiency virus (HIV), hepatitis B virus and hepatitis C virus), the search for tuberculosis and autoimmunity was negative (normal complement level, negative anti-nuclear antibodies and anti-SSA/SSB) and his eye examination was normal. Therefore, we attributed his acute TIN to LD.
Steroid treatment was initiated at 1 mg/kg/day. The patient's renal function rapidly improved, with appropriate diuresis allowing for withdrawal of hemodialysis after 2 days. There was no worsening of respiratory signs under steroid treatment. The patient was discharged at day 10, at which time his serum creatinine level was 110 μmol/L. One month later, after cessation of steroids and antibiotics, his serum creatinine level was 77 μmol/L. Investigation by Health Services did not find the source of Legionella contamination.


The second patient reported in this series was a 73-year-old female with a diagnosis of rectal adenocarcinoma 6 cm from the anal verge who underwent neoadjuvant therapy. In contrast to the first patient, this patient underwent a hybrid NOTES TME using a transanally inserted rigid, single port device. The single port access device has 3 channels for instrumentation, with 2 additional channels for CO2 insufflation. Using a 10 mm 45-degree laparoscopic camera, in lieu of a flexible colonoscope, the TME dissection was then carried out transanally with laparoscopic assistance as previously described. Operative time was 360 minutes. This patient also recovered uneventfully and was discharged home on postoperative day 6. Pathology revealed tumor-free margins and intact mesorectum with 2 of 11 lymph nodes positive for tumor (pT3N1).


The third case was reported by Tuech et al. in 2011 []. This report describes a 45-year-old woman with a reported T1sm3 rectal adenocarcinoma 3 cm above the dentate line. For this procedure a single port access device, endorec trocar (Aspide, France), was also used. This trocar consists of a rigid port with 40 mm outer diameter, three 5 mm, and one 10 mm access channel and an air inlet tube through which CO2 can be inflated. The extraperitoneal rectum was completely mobilized using this device. Once the lateral rectal attachments were divided, the rectovaginal peritoneal reflection was identified and perforated to gain access to the abdominal cavity. A second endorec trocar (Aspide, France) was then placed through the proposed ileostomy site and laparoscopic assistance with proximal colonic mobilization ensued. The procedure was performed successfully without complication. Operative time was 5 hours. The patient did well postoperatively without complication. Pathology revealed a pT1sm3N0 tumor. Fifteen lymph nodes were retrieved with the specimen.
While the principles of NOTES transanal rectal cancer resection remain the same, the methodology, particularly with respect to transanal dissection, varies between clinical trials. The consensus is that the majority of the rectal and mesorectal dissection can be achieved transanally while laparoscopic assistance is needed for proximal colon mobilization and tissue retraction. It is the preference of our group at this time to use the rigid TEO platform for transanal endoscopic rectal dissection rather than a flexible single port device. The TEO platform comes in 2 lengths, provides rigid stabilization for instrument manipulation, and is an established cost effective, reusable platform readily available at our institution. Nonetheless, the published reports thus far demonstrate that adequate hybrid NOTES TME can be achieved using flexible or rigid platforms and highlight the importance of continued work and development in this field.
As part of our effort to further this work, we are currently enrolling patients into an ongoing United States based Institutional Review Board (IRB) approved prospective clinical trial []. Patients selected for this approach include those with biopsy proven resectable adenocarcinoma of rectum located 4–12 cm from anal verge who are otherwise eligible to undergo standard open or laparoscopic low anterior resection with temporary diverting stoma. Tumors must be preoperatively staged as node negative, T1 (high risk features), T2 or T3 based on pelvic MRI with no evidence of metastasis on staging CT scans. For preoperatively staged T3N0 tumors, patients must have completed full-course neoadjuvant treatment. Procedures are performed following the same steps as described in cadavers, using an abdominal and perineal team working simultaneously. Transanal dissection is performed via the TEM platform with laparoscopic assistance through 1–4 abdominal trocars. The right lower quadrant trocar is later used as the ileostomy site. Following transanal specimen retrieval, a handsewn coloanal anastomosis with diverting ileostomy is performed. For this protocol, a diverting ileostomy is standard given performance of a low-lying anastomosis in patients who likely will require either neoadjuvant or adjuvant chemoradiation.


A 28-year-old man with no past medical history presented to the emergency department with a two-week history of severe “10-out-of-10” colicky epigastric pain with profound nausea, 15–20 episodes of vomiting daily, and decreased oral intake for fear of triggering these symptoms. He denied alcohol and tobacco use, but reported he had smoked two marijuana cigars (each containing approximately 1.5 grams marijuana bud) every day for the last ten years. As his symptoms of nausea, vomiting, and abdominal pain intensified, he self-medicated himself with increasing amounts of marijuana and his symptoms became increasingly intense. The patient reported that he initially got symptomatic relief with a hot shower, though as his symptoms intensified, he required increasingly longer bathing times that eventually progressed to the patient soaking himself in hot baths for hours each day. He stated that he had never experienced any of these symptoms in the past. In the emergency department, the patient's vital signs were within normal limits and his physical exam was significant only for minor tenderness to palpation in the epigastric region. The patient had no focal neurological deficits. The patient was admitted for intravenous fluid support, antiemetic therapy, and further evaluation.
The patient's medical evaluation revealed a complete blood count, basic metabolic profile, and hepatic profile that were within normal limits. An abdominal computerized tomography (CT) scan was negative for pathology, an EGD with biopsies showed only mild gastritis, and a gastric emptying study showed mildly delayed gastric emptying.
In this patient, initial symptomatic treatment with ondansetron and morphine was unable to keep the patient from having breakthrough episodes of nausea, vomiting, and epigastric pain. The patient was unable to tolerate even a clear liquid diet, and was extremely anxious about trying to increase or advance his oral intake given the painful episodes that food had triggered in the past. After administering 1 mg IV lorazepam, the patient improved at a remarkable pace; within 10 minutes of administration he no longer experienced nausea, abdominal, or food aversion. Over the next 12 hours, he transitioned to a regular diet, oral lorazepam (1 mg tablets), and was able to discontinue all other analgesic and antiemetic medications. The time from marijuana cessation to complete resolution of symptoms was approximately three weeks. The patient was discharged with a seven-day prescription for lorazepam (1 mg PO, twice daily). The patient was contacted at 3 and 6 months after discharge and he reported that, with sustained abstinence from marijuana, he had no return of his symptoms.


A twenty-five-year-old second gravida at 36 weeks of gestation was referred to our institution as a case of encephalocele with preeclampsia complicating pregnancy, for further management. The patient had regular antenatal visits and was diagnosed to have preeclampsia at 32 weeks of gestation. She was not on any medication. A target scan done at 19 weeks was said to be normal. On examination, her blood pressure was 140/90 mmHg. Systemic examination was normal. Obstetric examination revealed a single live fetus corresponding to thirty-six weeks in breech presentation. On ultrasound examination (Toshiba Nemio 10, curvilinear, 5 MHz probe), there was a fetus corresponding to 36 weeks in breech presentation with an amniotic fluid index of 14. There were two echogenic masses of 4 cm × 5 cm and 3 cm × 4 cm, arising from the placenta in close proximity with the occiput of the fetus (). One was cystic, and the other showed lobulation/septation. As there was no defect in the skull, encephalocele was considered to be less likely. Their attachment to other structures could not be traced. Gross fetal intracranial anatomy was normal. On Doppler ultrasound examination, there was minimal vascularity on the surface of the mass. Although magnetic resonance imagining (MRI) is the investigation of choice when an encephalocele is suspected, this investigation could not be done due to patients financial constraints. At thirty-eight weeks of gestation, the patient was posted for cesarean section for breech presentation, and a live male baby of 2.8 kg was delivered. There were no gross external deformities or anomalies in the neonate. The placenta measured 15 cm × 12 cm × 4 cm and weighed 250 grams. Maternal and fetal surfaces of the placenta were unremarkable. There were two masses attached to the membranes measuring 5.5 cm × 5 cm and 4 cm × 3 cm, respectively (). The first mass appeared lobulated. The second mass appeared cystic. Histopathological examination of the placenta was unremarkable. Histopathological examination of the masses showed lining of epidermis with attached adnexa of varying maturity. There were lobules of adipose tissue, stromal myxoid changes and islands of osteoid, and cartilage and smooth muscles tissues. The above findings were suggestive of benign teratoma of the placenta. The patient had uneventful postoperative period and was discharged on the seventh postoperative day. Neonate also did not have any problems.


A 27-year-old female reported with the chief complaint of a “fractured filling” in her left lower back tooth. History revealed intermittent pain localized to the same tooth during mastication. The tooth was previously restored with a silver amalgam restoration 7 years back. Clinical examination revealed a silver amalgam restoration with secondary caries in the left mandibular first molar (tooth 19) which was tender to percussion. Vitality tests elicited no response. Preoperative radiographs showed widening of periodontal ligament space in relation to the mesial root apex. In addition, radiographic apical contour of the tooth suggested that there might be two distal and two mesial roots (). From the clinical and radiographic findings a diagnosis of pulpal necrosis with symptomatic apical periodontitis was made and endodontic treatment was initiated.
Following endodontic access cavity preparation, two mesial and one buccally placed distal canal orifice was identified. Upon visual inspection of the floor of the pulp chamber using a dental operating microscope, a dark line was observed extending from the distal canal orifice towards the distolingual corner. At this corner, the overlying dentin was removed and a second distal canal orifice was detected. The conventional access was modified to improve access to the additional canals (). Root canal orifices were named as per the nomenclature proposed by Albuquerque et al. []. Working length was confirmed () and the canals were instrumented. Calcium hydroxide was placed as an intracanal medicament with a lentulospiral and the access cavity was sealed with Cavit G. The patient was asymptomatic at the next appointment, a week later, which allowed for root canal obturation and a coronal composite restoration ().