A 75-year-old man diagnosed with advanced pseudoexfoliative glaucoma presented for a routine follow-up consultation. He reported bilateral cataract surgery 4 years before, with implantation of a hydrophilic acrylic IOL (Akreos® Adapt; Bausch & Lomb) in the capsular bag. Also, 1 year later, he was submitted to pars plana vitrectomy in the oculus dexter (OD) due to a rhegmatogenous retinal detachment. His BCVA was light perception OD and hand movement oculus sinister (OS). Anterior segment examination revealed bilateral pupillary margin atrophy and XFM deposition on the anterior surface of the IOL in the left eye (). At that time, IOP was 14 mmHg OD (with brimonidine) and 20 mmHg OS (with travoprost, brimonidine, and a fixed combination of timolol and dorzolamide). The cup to disc (C/D) ratio was 0.9 in both eyes, and the optic discs were pale.


An 80-year-old woman was evaluated in a routine ophthalmological consultation. Bilateral cataract surgery had been performed 5 years earlier, and a hydrophobic acrylic IOL was implanted in the capsular bag (AcrySof®; Alcon Laboratories). The BCVA was 10/10 OU and slit lamp biomicroscopy revealed XFM on the IOL surface in the left eye (). The IOP was 12 mmHg OU, and ocular fundus was normal.


An 83-year-old man with known XFS presented for a routine consultation. He reported bilateral extracapsular cataract extraction surgery 19 years before, with implantation of an anterior chamber polymethylmethacrylate (PMMA) IOL in OD and posterior chamber PMMA IOL in the capsular bag OS. Left eye slit lamp examination revealed the presence of XFM on the anterior surface of the IOL (). The patient was being treated with a fixed combination of timolol and dorzolamide OU and IOP was 14 mmHg OU. Fundus examination revealed a C/D ratio of 0.5 OU.


A 65-year-old woman was referred to the glaucoma department due to OHT associated with XFS. She had previously undergone bilateral cataract surgery (9 years before in the right eye and 1 year before in the left), with implantation of a hydrophobic acrylic IOL in the capsular bag (AcrySof®; Alcon Laboratories). A neodymium-doped yttrium aluminum garnet (Nd:YAG) laser posterior capsulotomy was performed in her right eye 1 year after the cataract extraction. Her BCVA was 10/10 OU, and IOP was 12 mmHg OD and 18 mmHg OS (with travoprost OU). Slit lamp examination revealed XFM on the posterior surface of the IOL in OD (). Fundoscopy did not reveal any signs of glaucomatous neuropathy.


A 91-year-old woman with bilateral advanced pseudoexfoliative glaucoma presented for her follow-up consultation. She had been submitted to a bilateral extracapsular cataract extraction surgery 20 years before, with ciliary sulcus PMMA IOL implantation. A Nd:YAG laser posterior capsulotomy was performed in both eyes 9 years later. Her BCVA was 5/10 OD and 1/10 OS. Slit lamp examination revealed XFM on the posterior surface of the IOL in the left eye (). The IOP was 16 mmHg OU under latanoprost, brimonidine, and a fixed combination of timolol and dorzolamide OU. The C/D ratio was 0.9 bilaterally.


A 26-year-old male, unemployed person, presented to the Ophthalmology Clinic on 14 August, 2012 with a three-week history of Blurring, Soreness, Lacrimation, Redness, and Pain in the left eye. He had three similar attacks in the last three months, for which he was hospitalized. His presenting visual acuity was 6/9 OD, 6/24 OS. He had lid edema, cilliary injection, a severely hazy central disciform corneal stromal edema on an old corneal scar, with lipid corneal degeneration, and multiple nummular keratitis at around the 1 o’clock position of the left cornea. The anterior chamber of the left eye did not show any cell or flare reaction. The pupil was small in size and reactive. The red reflex was poor due to the corneal haze, with no visible details of the fundus. The right eye of the patient was relatively normal.
A diagnosis of secondary Viral Disciform and Nummular Keratitis was made and patient was put on oral and topical ointment of Acyclovir together with a stat dose of cyclopentolate and antibiotics. He was to be followed up in a week by which time a steroid was to be included in his therapy.
The patient did not show up until six weeks later when his visual acuity had deteriorated to Counting Finger in the left eye. He complied with his last medical prescription for only the last two weeks to the date of his presentation. A topical steroid was added to his other medications and he was to be reviewed in another one week.
At the scheduled appointment, he was subjectively fine and comfortable, his vision had improved to 6/24 in the left eye and the conjunctiva injection had subsided; stromal edema had subsided in the superior cornea, and the nummular keratitis had disappeared. He had a prominent feeder vessel into an area of the thick corneal scar in the inferior aspect of the left cornea [], with surrounding non-clearing, corneal edema and lipid keratopathy in the inferior-nasal quadrant bordering on the visual axis []. He had a deep anterior chamber, but the inferior iris and fundal details were not visible.
The relative quietness of the non-vascularized superior portion of the left cornea contrasted with the active edematous cornea area bordering on the feeder vessel in the inferior portion of the left cornea. It was, therefore, hypothesized that this could be explained by vascular leakage and the inflammatory mediators from the supplying feeder vessel into the adjacent cornea area. Therefore, an intervention of cauterization of the supplying feeder vessel was planned.
After the patient gave informed consent, he was put under a slit lamp microscope. The upper eyelid was manually retracted by the examiners gloved fingers after topical anesthesia with amethocaine. The cautery ballpoint was heated in a spirit lamp and the tip applied to about a 4 mm length of the sclera portion of the feeder vessel, to cauterize and separate the feeder vessel from the scarred and edematous portion of the inferonasal left cornea. The procedure lasted for about four minutes without any complication and was well-tolerated by the patient. The procedure was imaged pre- and post-intervention and the patient was given a stat dose of oral Ibuprofen–paracetamol combination. The immediate postoperative image is shown in .
Five days post intervention, the patient felt well with no more pain and his vision had improved. On examination, Visual Acuity OD remained 6/9 and OS had improved to 6/18, unaided. There were conjunctival petechial hemorrhages in the area of the cautery and the intra-cornea feeder vessel had regressed []. On slit lamp examination, the corneal edema had completely regressed. The corneal clearity had improved enough to allow easy visualization of the details of the inferior Iris and a normal fundus, with C:D = 0.3, a pink disc, normal macula, and a peripheral normal pink flat retina. These details were not visualizable prior to the cautery intervention five days earlier.
This patient had achieved rapid corneal edema reversal with significant functional improvement and has remained so, via phone conversation, a month later.


We report a patient with primary cerebellar neuroblastoma. A 2.5-year-old boy was admitted to our hospital because of nausea and vomiting in the morning, headache, ataxia, poor balance and torticulli. The preoperative routine complete blood count , clotting profiles , electrolytes , sequential multiple analysis for chemistry , and the urine analysis findings were all within the normal limits . A brain CT scan revealed a well defined hyperdense mass in cerebellar vermis compressing forth ventricle with obstructive hydrocephaly, compatible with medulloblastoma (). A microscopic suboccipital craniectomy was performed and after complete resection of the tumor, a ventriculoperitoneal shunt was inserted.
The pathologist reported a lesion composed of small, dense and round cells with hyperchromatic nucleus and scanty cytoplasm. The lesion arranged in sheets and prevascular accumulation numerous foci of small cells with few mitotic figures. There were necrosis and hemorrhagia. Also lobulated tumor cells were arranged in groups (islands separated by few fibrous stroma). Parallel patterns were seen in some foci of tumor cells. Also cells with vesicular nuclei were seen (). These findings established the diagnosis of neuroblastoma. Immuno-Histo-Chemistry (IHC) showed a strong positivity for Neuron-Specific Enolase (NSE).
During this hospital admission, postoperative systemic examination which included bone survey, bone marrow aspiration, abdomino-pelvic CT scan and MRI of the neuroaxis revealed no other evidence of tumor. Also cytological examination of cerebrospinal fluid was normal. After surgery we began chemotherapy with a combination of Cisplatin, Ara-c, Vincristine, Hydroxyurea, Lamustin, Procarbazin, Cyclophosphamide and Methyl prednisolon (8 at 1 protocol) every four weeks for 12 courses. Follow up examination revealed a profound delay in mental development but brain MRI performed 12 months after surgery revealed no evidence of tumor recurrence. The patient is presently 3.5 years old and his chemotherapy is finished and he is stable with no sign of relapse.


Case 1 was a 24-year-old man (No. 21 in ). He received hyperthermia treatment on December 26, 2007, and remained in good health during a long-term follow-up period. He died suddenly of grand mal epilepsy at 20 months after treatment. shows the changes in the CT and MRI images before and after treatment.


Case 2 was a 28-year-old man (No. 26 in ) who experienced the recurrence of astrocytoma after surgical removal and radiotherapy in 2002. He experienced headache and nausea in 2009, and underwent hyperthermia treatment in the same year. The patient remained healthy during an 18-month follow-up period. shows changes in the CT and MRI images before and after treatment.


A 14-year-old girl presented to us in 2008 with the complaints of headache, vomiting, and giddiness of 3 months duration. On examination, she had left hemiparesis and features of raised intracranial pressure. Magnetic resonance imaging (MRI) scan revealed an ill-defined nonenhancing, heterogeneous mass in the third ventricle measuring 4 cm × 3.5 cm × 3.5 cm. Both lateral ventricles were dilated, fourth ventricle was normal in size and the third ventricle not seen separately seen from mass. The sella and pituitary were normal.
A ventriculo-peritoneal (VP) shunt was placed as an emergency procedure. Serum and cerebrospinal fluid (CSF) alpha fetoprotein (AFP) were normal, however beta human chorionic gonadotrophin (HCG) levels were raised (13.0 mIU/mL in serum and 127 mIU/mL in CSF - normal <5 mIU/mL in serum and CSF). She received 2 cycles of chemotherapy using carboplatin (600 mg/m2 on day 1), etoposide (100 mg/m2 days 1-3 and days 22-24) and, ifosfamide (1800 mg/m2 on days 22-26), followed by radiotherapy, 5040 cGy in 28 fractions.
Serum beta HCG levels came down to <1.00 mIU/mL and remained normal throughout her treatment period. Computed tomography (CT) scan done 4 weeks after starting treatment showed marked reduction in tumor. At the end of the treatment, beta HCG was normal and CT scan showed an ill-defined 1.1 cm × 1.0 cm hypodense lesion in the right basal ganglia; the third ventricular mass had resolved. She was on regular follow-up after treatment completion and her clinical and biochemical evaluation remained normal.
Two and a half years after completion of treatment, she presented with progressive abdominal distension of 1-month duration. She was deeply icteric; a hard nodular mass occupied the entire right side of the abdomen. Liver function tests were deranged with total bilirubin-22.7 mg%, direct bilirubin-21.5 mg%, total protein-7.0 g%, albumin-3.1 g%, serum glutamic oxaloacetic transaminase-241U/L, serum glutamic-pyruvic transaminase - 44U/L, alkaline phosphatase-615U/L prothrombin time international normalized ratio 1.98, beta HCG-199.3 mIU/mL, AFP-1.04 IU/mL, hepatitis B and C screen was negative. CT scan of abdomen showed a large predominantly cystic lesion with foci of solid enhancing areas occupying the right lobe of liver (segments 5, 6 and 7) with extension to segment 4B of left lobe, measuring approximately 12 cm × 8 cm × 9 cm. The mass was infiltrating the primary hilar confluence with intrahepatic biliary radicular dilatation and was encasing the gall bladder. There was significant periportal adenopathy. There was only mild ascites and the bowel, mesentery and omentum were normal. VP shunt was in situ. MRI brain did not show any recurrence and MRI of spine was normal.
She received a total of five courses of chemotherapy using carboplatin 600 mg/m2 and bleomycin 15 mg/m2. Carboplatin alone was given in the first course in view of her poor clinical condition. Following this her general condition improved, jaundice reduced and beta HCG dropped to 15.7 mIU/mL. After the third course of chemotherapy beta HCG level normalized and has remained normal since then. She underwent right hepatectomy with excision of segment 4B after four courses of chemotherapy. The tumor was found to occupy the whole of the right lobe except a small portion of segment 8. There were two periportal lymph nodes which were also excised and the peritoneal end of the VP shunt was removed. No ascites or peritoneal deposits were seen. Histopathology revealed a well-circumscribed tumor with extensive necrosis, foci of recent and old hemorrhage, chronic inflammation and fibrosis with areas of hyalinization. Immunohistochemistry was positive for placental alkaline phosphatase and CD30 which was consistent with GCT. The postoperative period was uneventful. After she recovered from surgery, she received the fifth course of chemotherapy.
She has been on regular follow-up and is well 30 months after the completion of relapse treatment.


A 26-year-old man, with a known case of hepatitis B, was referred to our hospital from the ENT clinic after a severe respiratory distress and hoarseness, which had occurred after an episode of upper respiratory tract infection (URTI). The condition was started 2 weeks earlier. His symptoms were started and exacerbated following URTI. He was treated with Cefixime, Theophyline and Salbutamule (inhaler) but the symptoms did not resolve. He did not have dysphagia or odynophagia, nor did carry any history of asthma, allergy, smoking or illegal substance abuse. His father had died of end stage stomach cancer about a year ago and his brother had vasculitis (who is under the treatment with corticosteroids).
Physical examination revealed fever (38.5°c). Examination of his mouth and oropharynx showed erythema and tonsilar hypertropthy without exudates in his oropharynx. He had an obvious inspiratory and expiratory stridor and overt respiratory distress. Auscultation of lung revealed diffused wheezing in his lungs with a 3/6 systolic murmur in his left sternal border and pulmonar area. There was no evidence of peripheral edema. Indirect laryngoscopic examination showed pappilomatose lesion in the 1/3 posterior portion of true vocal cords. There were maculopopular rashes on his chest wall, upper back, neck, face and extremities ().
Chest x-ray showed an increase in bronchovascular markings in middle and upper lobes of right lung. Lateral head and neck radiography revealed a mass in his subglottic area (-). Chest CT–scan showed tubular bronchectatic changes at right lobe associated with pulmonary inflammation. Fibrotic band was seen at basal portion of both lungs with no pleural effusion. There was an aneurismal dilatation of aorta with maximum diameter of 50×48 mm in the ascending part and about 49×45 mm in the descending part. The ultrasound echocardiography confirmed the aortic aneurysm. Veneral disease research laboratory (VDRL), fluorescent treponemal antibody- absorption test (FT-ABS), antinuclear antibody (ANA), Cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA) and Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA) were negative and angiotensin-converting enzyme (ACE) levels fallen within normal range.His rheumatoid factor was 3+ and C reactive protein 1+. His ESR level in the first hour was 54 mm/hr. Emergency trachestomy and mass debulking was done for emergency air way management. Direct laryngoscopy showed a 1.5 cm mass in subglottic area. Specimens were taken for culture and pathologic diagnosis. The throat cultures were negative for Mycobacterium tuberculosis and fungus. The specimen also contained multiple pieces of creamy-brownish soft tissue. The microscopic evaluation showed tissue composed of large cells with hyperchromatic round to ovaloid nuclei, prominent nuclei and irregular border and eosinophilic cytoplasm arranged as sheets. The immunohistochemistry report was positive for leukocyte common antigen (LCA), antigen KI-67 (60% of tumoral nuclei) and negative for CD3, CD20, CD43, CD30. The pathologic diagnosis was diffuse large cell lymphoma (High grade) and immunohistochemistry test for CD79 was positive which determined the B cell type. He had no symptom afterward.


An 8-year-old boy was admitted to The Catholic University of Korea, Seoul St. Mary's Hospital after experiencing one day of severe abdominal pain. He was diagnosed with ALL (L3) 4 years ago. After first relapse, the patient received a bone marrow transplant 3 years ago, but second relapse occurred unfortunately. It had been three weeks since his last chemotherapy, which included a high dose of methotraxate. He was no vaccinated against chickenpox following the bone marrow transplant.
His body temperature was normal. He showed hepatosplenomegaly and tenderness in the periumbilical area. A complete blood count showed hemoglobin of 12.1 g/dL, white blood cells (WBCs) count of 3,490/mm3 with segmented neutrophils of 80.0%, and platelet count of 135,000/mm3. Liver function tests showed slightly increased levels of aspartate aminotransferase (AST, 78 U/L) and alanine aminotransferase (ALT, 135 U/L) (). Chest, abdomen x-rays and abdomen and pelvis computed tomography scans appeared normal.
The next day, the patient was running a fever of up to 39.0℃, so empirical intravenous antibiotics were started. His abdominal pain remained constant. On his third day, multiple erythematous vesicles formed on his trunk. Consequently, 1,500 mg/m2 of acyclovir divided into three doses per day was administered.
On hospital day 5, the vesicles developed across the whole body and he complained of dyspnea. A chest x-ray () showed numerous small nodules in the entire both lungs, which lead to a diagnosis of varicella pneumonia. Laboratory tests revealed WBCs of 5,090/mm3, platelet count of 32,000/mm3, AST of 931 U/L, ALT of 788 U/L, lactate dehydrogenase (LDH) of 3,196 U/L (). DIC profile was also positive. We continued administration of intravenous acyclovir, changed the intravenous antibiotics, and added intravenous immunoglobulin (IVIG, 500 mg/kg/day), single donor platelets, fresh frozen plasma, and antithrombin III. The patient was transferred to the intensive care unit (ICU).
The next evening, the patient began to show drowsy mental state and dyspnea was aggravated. A chest x-ray showed total haziness and ARDS. He required intubation and mechanical respiratory support. We increased the dose of IVIG to 1 g/kg/day.
On hospital day 7, a chest x-ray showed marked improvements in the lung, but cardiomegaly was noted. Platelet count was 69,000/mm3, and AST and ALT were 224 U/L and 280 U/L (). The patient was successfully recovered from DIC. We used IVIG for a total of four days, however fever persisted and the vesicular skin lesions showed no change. On echocardiography, cardiac function was normal.
On hospital day 9, the patient's blood pressure (BP) began to increase up to systolic pressure of 150 mm/Hg and diastrolic pressure of 125 mm/Hg. We continuously administered an intravenous labetarol, however no definite effect was observed. Using by real-time polymerase chain reaction (PCR), VZV was detected in patient serum that had had been sampled at the onset of skin lesions. We decided to administer IVIG for two more days.
On the morning of hospital day 11, the patient experienced a generalized tonic-clonic seizure that lasted 30 minutes which we thought was a sign of hypertensive crisis or VZV involvement in central nerve system. We added intravenous nitroprusside and conducted cerebrospinal fluid (CSF) tapping. The results were normal, and VZV antibody and PCR in CSF were negative. We increased the antihypertensives dose and added intravenous phenytoin. No more seizures were occurred thereafter.
By hospital day 13 and the ninth day after intubation, the patient's pulmonary condition was stable. He was successfully taken off the ventilator. He no longer had a fever, and all skin lesions reached the scarring stage.
On hospital day 17, the patient was transferred from the ICU to the general ward. His vital signs were stable. All laboratory data showed improvements. Lymphocyte subset showed CD3 of 87.0% (normal range [NR], 61%-85%), CD4 of 14.8% (NR, 23%-58%), CD8 of 70.6% (NR, 19%-48%), CD19 of 9.6% (NR, 7%-23%), and CD56 of 3.0%. (NR, 6%-29%). These results suggested that the patient's cellular immunity was not yet recovered. Due to the patient's history of seizure, we performed brain magnetic resonance imaging (MRI) and electroencephalogram (EEG). The brain MRI () showed multiple patchy high-signal-intensity lesions in the subcortical regions of the right frontal, parietal and occipital lobes as a results of VZV-induced encephalitis. The EEG looked normal, so phenytoin was stopped.
We ceased administration of intravenous antibiotics and acyclovir on hospital day 19. The patient complained of no other symptoms. He was discharged on hospital day as he had been restored to a healthy appearance and most laboratory data showed improvement (). The clinical course of patient was as follows (). One month later, chemotherapy was resumed. And the follow-up brain MRI was done three months later, multiple high-signal-intensity lesions due to VZV-induced encephalitis were disappeared.


A 65-year-old male patient presented with complaints of bone pain at multiple sites and bilateral exophthalmos. The patient underwent serum calcium and alkaline phosphatase level evaluation, which was 8.7 mg/dl and 80 IU/l (normal value 20-140 IU/l), respectively. The patient underwent a 99mtechnetium-methylene diphosphonate (99mTc-MDP) three phase skeletal scintigraphy, which revealed increased activity in bilateral orbital region (right > left) in flow and blood pool phases, whereas diffusely increased tracer uptake in the skeleton was noted with high bone to soft tissue contrast and faintly visualized kidneys. Along with this, intense tracer uptake was noted in bilateral orbits, nasal bones, and medial part of bilateral maxillae []. Axial section through the orbit in a non-contrast CT showed bilateral retrobulbar soft tissue density masses []. A non-contrast CT done elsewhere, through bilateral upper femora and pelvis showed multiple osteosclerotic foci. Fine needle aspiration cytology performed from the skeletal lesion revealed xanthogranulomatous infiltration by foamy histiocytes surrounded by fibrosis. A provisional diagnosis of histiocytosis was made. Further immunohistochemistry to characterize the type returned positive for CD68 and negative for CD1a and S-100. Based on the clinical, scintigraphic, and histopathological findings, a diagnosis of ECD disease was made. Because ECD often involves the kidneys, the patient was evaluated for the same, which revealed urea and creatinine levels of 36 and 0.8 mg/dl, respectively.


A 61-year-old man complaining of syncope and chest pain came to our hospital. Ten days prior to hospitalization, he had had a fever, chills, and body ache. He had taken some medication from a local clinic for these symptoms, which were supposedly caused by a simple upper respiratory infection. Despite the medication, the patient had developed another symptom of syncope 7 days earlier and was transferred to the Neurology Department for evaluation. On arrival, he had mild fever (37.5°C) with chills, sweating, and dyspnea. The blood pressure was 110/40 mmHg, pulse rate 90/min, and respiratory rate 20/min. On the chest auscultation, a diastolic murmur was heard at the left upper sternal border. The laboratory tests showed leukocytosis (16,900 leukocytes/μL). The chest X-ray revealed cardiomegaly, and the electrocardiogram demonstrated a complete AV block. In the brain magnetic resonance imaging, there were multiple infarcts, which were thought to be caused by septic emboli. Echocardiography showed moderate aortic regurgitation with a retracted bicuspid aortic valve and periannular abscess. Therefore, we decided to perform an emergency operation. After the median sternotomy, ascending aortic and bicaval venous cannulation were done. Cardiopulmonary bypass was initiated, and the ascending aorta was then cross-clamped. After infusing the histidine-tryptophan-ketoglutarate solution through the retrograde cardioplegic catheter and local cooling with ice slush, transverse aortotomy was performed. The aortic valve was thickened and retracted; this was consistent with a rheumatic aortic stenosis. There was a commissural fusion between the noncoronary cusp and the right coronary cusp, which also had fibrous thickening with calcification and abscess (). The periannular abscess involved the aortic root of the base of noncoronary sinus () and invaded down to the central fibrous body, whole membranous septum, and mitral and tricuspid valves (TVs) (). The aortic valve was excised carefully, and then, the infected tissue and the pus were removed meticulously from the abscess pocket down to the membranous septum, central fibrous body, annulus of the mitral valve (MV), and anteroseptal commissure of the TV. We also made an extended transseptal incision extending from the right atrial auricle to the TV to expose the lesion and achieve complete debridement. The septal and anterior leaflets of the TV were also partially resected due to vegetations. After the complete debridement of the infected area, we found a large defect including the left ventricular outflow tract (LVOT), which contained the membranous septum, central fibrous body extending to the TV, and mitral annulus. The MV itself was intact and had no vegetation. The reconstruction of the LVOT was performed with half-folded elliptical bovine pericardium (Peri-Guard; Synovis Surgical Innovation, St. Paul, MN, USA) (). The bovine pericardium was trimmed into an elliptical shape and a half-fold was made; then, its base was secured by interrupted suturing to the annulus of the MV by using reinforcement with a Teflon felt, followed by LVOT reconstruction using a leaflet of the half-folded elliptical bovine patch with aortic valve implantation concomitantly using a 21-mm mechanical valve (SJM Masters Series Valve; St. Jude Medical Inc., St. Paul, MN, USA) (). After closing the aortotomy site, tricuspid annuloplasty was done: partial excision and re-suspension of the septal and the anterior leaflet and anteroseptal commissuroplasty. The other half of the half-folded elliptical bovine patch was used for repairing the right atrial wall with a defect and TV. After weaning the patient from cardiopulmonary bypass, we conducted transesophageal echocardiography, which revealed neither an intracardiac shunt nor TV and MV regurgitation. Because the complete AV block was observed, we began temporary pacing at the rate of 80 beats/min after placing permanent pacing leads on the right atrial and ventricular walls. The total cardiopulmonary bypass and aortic cross clamping times were 303 minutes and 238 minutes, respectively. On postoperative day 1, we were able to wean the patient from mechanical ventilation and extubate an endotracheal tube. On postoperative day 6, we inserted the pacemaker generator. Microbiologic examination of the excised tissues showed no organisms on the Gram stain. Cultures of the aortic root abscess contents and vegetation were all negative. Intravenous antibiotics with ampicillin, nafcillin, and gentamicin were continued for approximately 6 more weeks. The echocardiography on postoperative day 51 revealed normal LV contractility, no intracardiac shunt, and a well-functioning prosthetic mechanical aortic valve without para-valvular leakage and tricuspid regurgitation. On postoperative day 56, the patient was discharged with oral anticoagulation.


A 40-year-old woman presented with a history of a painless lump in the left breast for 1-year. On examination, a soft nodule measuring 1 cm × 1 cm was palpable in the left infraareolar region. The lump was subjected to fine-needle aspiration with a 22 G needle and a 20 mL syringe. Two attempts were made as the yield of the material was scanty. Air-dried smears were stained with May-Grünwald-Giemsa (MGG) stain and the ethanol fixed smears with Papanicolaou stain.
On microscopic examination, the smears showed cohesive clusters and a few branching fragments of benign ductal epithelial cells intimately mixed with many spherical, acellular homogeneous hyaline globules []. Many detached globules were also noted and a few globules showed laminar concentric layers lined by a single layer of uniform, small, oval or comma shaped cells []. A few dispersed bare, bipolar cells were also seen in the background with occasional fragments of adipose tissue. The color of these spherules was green in PAP stained smears and purplish-pink in MGG stained smears []. Periodic acid Schiff (PAS) stain showed strong positivity for these globules.
A diagnosis of collagenous spherulosis associated with benign proliferative breast disease was rendered. The case has not yet been biopsied, but no signs of progression have been observed on close clinical and mammographic follow-up of 1-year.


This is a case report of a 30-year-old female patient who presented to us at 34 weeks gestation in her second pregnancy with 3 days duration of tingling sensations over her fingers and toes bilaterally. There was an associated weakness of the left side of the face. There was no history suggestive of bulbar, respiratory or autonomic disturbances. At 10 days prior to the onset of neurological symptoms she had an episode of gastro-enteritis that had lasted 2 days. She had received immunization for tetanus in view of pregnancy but otherwise her progress during this pregnancy has been unremarkable.
Neurological examination revealed normal eye movements and optic fundi. She had left lower motor neuron type of facial palsy, but no bulbar weakness. Tone was reduced over all four limbs. Symmetric mild proximal weakness was seen over the lower limbs and she required mild support to get up from the supine position. Sensory system was normal. Stretch reflexes were bilaterally absent and plantars were flexors on both sides. Respiratory examination was within the normal limits.
Serum chemistry, hemogram and urinalysis were normal. No atypical cells were seen in the peripheral smear. Erythrocyte sedimentation rate was 65 mm/h. Antinuclear antibodies, rheumatoid factor, C-reactive protein, venereal disease research laboratory, hepatitis B surface antigen, hepatitis C virus, human immunodeficiency virus, anti-neutrophil cytoplasmic antibodies and antiphospholipid antibodies (IgG and IgM) and lupus anticoagulant test were negative. Toxoplasma, rubella, cytomegalovirus and herpes simplex virus antibodies were negative. Thyroid function tests were within normal limits. Cerebral spinal fluid examination could not be done due to lack of consent. Her electrophysiological findings are presented in the , Study 1.
Weakness evolved and by day 2 of admission (day 5 of illness). She had worsening of proximal weakness requiring significant support for walking and worsening of difficulty in getting up. Intravenous immunoglobulin (IVIG) was instituted at a dose of 0.4 g kg/day over next 5 days. Weakness further evolved and by day 3 of admission (day 6 of illness) right facial and neck flexor weakness also developed. By day 4 of admission she also had requirement of assistive device (walker) for walking. By day 5 of admission (day 8 of illness) she however started improving with no further progression and mild improvement in neck flexion. No drop in single breath count was ever noticed during her hospital stay. Blood pressure (BP) during hospital stay was detected to be high (systolic 130-150 mm Hg, diastolic 90-100 mm Hg). Alpha-methyldopa was started and carefully titrated to avoid precipitous fall of BP considering the dysautonomia of GBS. Limb power gradually improved. By day 9 of admission (day 12 of illness) neck flexor weakness had improved significantly to normal power. She could walk unsupported though with mild unsteadiness, when she was discharged.
She remained neurologically stable subsequently and was admitted in her 36th week of gestation with labor pain. She underwent cesarean section under spinal anesthesia. Indication for cesarean section was floating head despite labor pain in the 36th week of gestation. Neurological examination a day prior to delivery (day 17 after the onset of the first symptom of GBS) did not reveal any limb weakness. There was only residual left facial weakness. No significant limb weakness was observed during her stay at the hospital following delivery and she was discharged on the seventh postpartum day (25th day after the onset of the first symptom of GBS).
She developed recurrence of weakness of limbs postpartum, noticed since the 29th day of onset of the first symptom of GBS. She was readmitted a day later with rapidly worsening weakness and requirement of support for walking. Neurological examination revealed bilateral symmetrical weakness over all 4 limbs. She had diffuse areflexia and normal sensory examination. Results of repeat electrophysiological studies are presented in the , Study II.
A repeat course of IVIG (0.4 Kg/day for 5 days) was administered. She gradually improved in her motor power and could walk unsupported after 7 days when she was discharged. She remained neurologically asymptomatic subsequently. Repeat electrophysiological studies done at 1 year follow-up showed significant improvement [, Study III].


A 57-year-old man with poorly controlled diabetes mellitus diagnosed 1 year ago (HbA1c 8.5% at admission) visited the local emergency room complaining of sudden left arm weakness. He denied any recent immunizations, preceding trauma (including procedures, such as acupuncture or apitoxin), or infection. A laboratory study and shoulder X-ray showed no evidence of abnormalities. Left shoulder pain and swelling developed after several hours and rapidly extended to the left hand. The patient visited another hospital and magnetic resonance imaging (MRI) revealed the formation of a subdeltoid abscess along with septic arthritis in the left shoulder joint (). He was transferred to the department of orthopedic surgery of our hospital for surgery 9 days after the onset of symptoms. His vital signs at admission were stable without fever. The laboratory data showed abnormal values, including white blood cell count of 18,210/µL (normal range, 4,0000-10,000/µL), C-reactive protein of 31.42 mg/dL (normal range, 0-0.3 mg/dL), and erythrocyte sedimentation rate of 101 mm/hr (normal range, 0-15 mm/hr). A physical examination revealed painful limitation of motion, tenderness, swelling, and a heat sensation in the left shoulder area. Intravenous antibiotics (cefazolin and clindamycin) were started, and open irrigation and debridement were performed through arthrotomy on the next day. S. agalactiae was identified by culture, and it was susceptible to most antibiotics; thus, cefazolin and clindamycin were changed to amoxicillin.
The left upper extremity weakness and hypesthesia continued after surgery. An electrodiagnostic examination was performed about 4 weeks after the onset of initial symptoms. The manual muscle test revealed severe weakness of the left supraspinatus, infraspinatus, and deltoid muscles (Medical Research Council [MRC] grade, 1/5) with mild weakness of the left biceps and triceps muscles (MRC, 4/5). Wrist and hand power was almost normal. A sensory examination showed paresthesia in the left axillary nerve distribution. Moderate to severe atrophy of the left supraspinatus, infraspinatus, and deltoid muscles was noticed, and mild atrophy of the left biceps and triceps muscles was also found (). Electromyography and nerve conduction studies (NCS) showed left upper and middle trunk brachial plexopathy but involving the upper trunk more severely. The axillary, musculocutaneous, and suprascapular nerves were absent of compound muscle action potentials (CMAPs) when recorded from the deltoid, biceps, and supraspinatus muscles, respectively (). The left supraspinatus, infraspinatus, deltoid, biceps, triceps, and flexor carpi radialis muscles showed discrete interference patterns and abnormal spontaneous activities on electromyography (). Motor unit action potentials (MUAPs) of all sampled muscles were unremarkable.
The patient received physical therapy after discharge. Nine weeks after symptom onset, motor power had improved in the following muscles: left supraspinatus and deltoid was MRC 2/5, left infraspinatus was MRC 3/5, and left biceps and triceps was MRC 5/5. Paresthesia of the left axillary nerve distribution continued. Follow-up electromyography and NCS were performed about 9 weeks after the onset of initial symptoms and showed improved signs of reinnervation. The axillary, musculocutaneous and suprascapular nerves that were absent of CMAPs during the first NCS examination began to show CMAPs, although amplitude was small compared to that of the right side (). The MUAPs were polyphasic in the left deltoid and triceps muscles on volition. Interference patterns had improved except that the triceps muscle showed full interference patterns and the supraspinatus, infraspinatus, deltoid, biceps, flexor carpi radialis muscles showed reduced interference patterns. Positive sharp waves had decreased and were observed only in the left infraspinatus muscle ().


A 9-year-old girl presented to the pediatric outpatient department of the JSS Hospital, Mysore, with a swelling in the right cheek of 4 months duration. There was no history of trauma. This was a painless swelling and did not bleed. She did not have fever. On oral examination, there was a firm, globular swelling measuring about 2 cm × 2 cm in the posterior part of the buccal mucosa opposite the 2nd right maxillary molar tooth. The swelling was non-tender and mobile and had a smooth surface. Her oral hygiene was good and there was no dental caries. The cervical lymph nodes were not enlarged. The tumor was just underneath the mucosa and away from the parotid gland. The tumor was removed totally along with a margin of normal tissue with wide excision by the intraoral route under general anesthesia. Macroscopically, it was a grey white globular tissue mass measuring about 2 cm × 2 cm []. Microscopic analysis showed a tumor with ductal cells arranged in sheets, glandular and acinar pattern in a background of abundant fibromyxoid areas — features consistent with the features of PA []. The patient was followed-up for 1 year without any problems.


A 17-year-old man presented with pain and swelling of his left forearm with his wrist and fingers held in a flexed position (Fig. ). Prior to this, there was a 3-day history of progressive pain in the forearm, but no history of trauma. The patient had no previous medical history, was on no regular medications and had no history of previous infections. His observations showed pyrexia of 39°C, but all others were within normal limits. A clinical diagnosis of compartment syndrome was made and the patient was planned for urgent fasciotomies. Routine blood examination demonstrated pancytopenia with a haemoglobin level of 3.9 g/dl, white cell count of 0.9 × 109/l and platelet count of 4 × 109/l. In addition, an urgent ultrasound scan was performed to rule out haematoma and possible tamponade of an arterial bleed. The scan demonstrated a collection within the ulna volar forearm. After transfusion of three units of cross-matched blood, two units of fresh frozen plasma and two units of platelets, an urgent fasciotomy and drainage of the collection under general anaesthesia was performed with a concurrent iliac crest bone marrow biopsy.
A modified Henry's approach and carpal tunnel decompression was performed and revealed a collection of pus was superficial and ulna to flexor digitorum superficialis (FDS) muscle. The distal muscle of FDS was ischaemic and swollen preventing finger extension due to the tight wrist and carpal tunnel fascias. The ischaemic muscle was debrided and a dusky flexor carpi ulnaris (FCU) was decompressed and left in situ for a review at second look surgery (Fig. ).
Deep tissue and pus samples grew gram-positive cocci—Staphylococcus aureus, sensitive to flucloxacillin, on which he was commenced after samples were taken. No further focus of infection was identified. In the following 12 days, the patient underwent four further surgical debridements, topical negative pressure wound therapy and delayed primary closure of the wound. Only partial resection of FDS and FCU was necessary; therefore, all functional units were maintained (Fig. ).
The bone marrow biopsy confirmed a diagnosis of aplastic anaemia. An immediate family member was found to be a match for a bone marrow transplant which has been carried out successfully.
At latest review, 6 months following initial presentation, the patient had regained full extension of wrist and digits, full wrist flexion and full finger flexion apart from a mildly limited middle finger which comes within 1 cm of his palm. His median and ulna nerve sensation has resolved after being slightly reduced compared with the opposite side in the initial postoperative period.


An 18-month-old male child presented with 3 days history of fever, rigors and pyuria, 5 months prior to admission. He was born to non-consanguineous parents. The child is appropriately immunized for his age. He weighed 11 kg and had normal height for age also. Urine microscopy showed plenty of pus cells. Ultrasonogram (USG) showed a swollen left kidney. There was no obstruction or perinephric collection. Right kidney was normal. Urine culture grew Escherichia coli. He was treated with ceftriaxone for a week and the child recovered. The child had two similar episodes in the next 4 months. He was treated with oral cefixime and oral ciprofloxacin for a week each time by the pediatrician. He was referred to us for recurrence of fever and pyuria. At admission, the child was toxic with a temperature of 39°C, heart rate 142/min and respiratory rate 38/min and had a palpable, enlarged left kidney. Pertinent laboratory data included hemoglobin 13.8 g/dl; white blood cell count 14,900/mm3 (neutrophils 11,622 and lymphocyte 2980); serum creatinine 0.8 mg/dl; fasting blood glucose 79 mg/dl; and human immunodeficiency virus-negative. Urinalysis had a pH of 6.5, plenty of white blood cells and red blood cells. Urine and blood bacterial culture was sterile. Intravenous urogram and 99mTc ethylene dicysteine renal scintigraphy showed a non-functioning left kidney. USG continued to show swollen left kidney with perinephric stranding with suggestion of intra renal abscess formation along with a perinephric collection of size 4 cm × 3 cm. The child was not responding to piperacillin/tazobactum and amikacin. As the child continued to be toxic, surgical drainage of abscess was considered. During exploration, the left kidney was found be necrotic and hence left nephrectomy was done. The child continued to have fever in the postoperative period.
Histopathology report, received on the 3rd postoperative day, showed extensive necrosis and suppuration bordered by epitheloid cell granuloma along with interstitial inflammation by lymphocytes, plasma cells and neutrophils. The necrotic area shows numerous fungal elements which are broad non-septate with irregular branching consistent with mucormycosis []. A diagnosis of isolated left kidney mucormycosis was made. He was started on conventional amphotericin B at a dose of 1 mg/kg/d from the fourth postoperative day. The child tolerated amphotericin B well and became afebrile within the next 3 days. He was continued on amphotericin B for 4 weeks. He continues to thrive well and has normal weight for age. He did not have any recurrence of symptoms or any other opportunistic infection.


A 74-year-old woman (147 cm in height, weighing 37 kg) was scheduled for a reoperation of left total hip replacement surgery. She had received several hip surgeries since 1981 because of a pedestrian traffic accident. She had a previous history of Hashimoto's thyroiditis, diagnosed in 2002. She had been on synthetic thyroxine medication, which was stopped in 2004 because of elevated f T4, and propranolol 40 mg had been administered daily, but the patient was not on medication upon admission. She had not been on medications such as prophylthiouracil (PTU) or amiodarone.
Upon preoperative evaluation, thyroid function test (TFT) showed that thyroid stimulating hormone (TSH) was 6.02 µIU/ml (normal: 0.27-2.40 µIU/ml), f T4 was > 7.77 ng/dl (normal: 0.93-1.70 ng/dl), and T3 was 93 ng/dl (normal: 80-200 ng/dl). Total T4 or free T3 level was not measured. The measured antithyroid antibodies were as follows: anti-thyroglobulin, antimicrosome, anti-TSH receptor and thyroid stimulating antibodies were 3094.2 U/ml (normal < 60 U/ml), > 4000 U/ml (normal < 60 U/ml), 0.1 IU/l (< 1.5 IU/l) and negative, respectively. A huge goiter was observed on computed tomography (CT). Ultrasound examination showed a goiterous change with a diffuse heterogeneous parenchyma, and a thyroid scan showed no demonstrable cold or hot nodule. Surgery was delayed and she was referred to an endocrinologist for evaluation of the underlying causes of the abnormal TFT. On the primary evaluation, a brain magnetic resonance imaging scan (MRI) was obtained which revealed no evidence of a pituitary tumor. Thyrotropin-releasing hormone (TRH) stimulation was carried out which resulted in an exaggerated elevation of the TSH level (baseline TSH 7.76 to peak TSH 79.79). Thereafter, a TRH stimulation test was conducted with T3 suppression by administration of physiologic T3 medication, and the result was suppression of the TSH secretion. Therefore, the possibility of a TSH secreting pituitary tumor and resistance to thyroid hormone (RTH) was ruled out of her diagnosis, and inhibition of T4 to T3 conversion by 5'-deiodinase disorder was suspected. Her basal cortisol level was 9.9 µg/dl (normal: 7-21 µg/dl). Her basal adrenocorticotropic hormone (ACTH) level was 32.99 pg/ml (normal: 5-60 pg/ml). Thyroxine binding globulin (TBG) was 26.9 µg/ml (normal: 15.8-26.4 ug/ml) and albumin level was 3.3 g/dl (normal: 3.2-5.2 g/dl).
In the meantime, she did not manifest symptoms of thyrotoxicosis such as palpitation, hypertension, diaphoresis or arrhythmia, and she was considered to be in a clinically euthyroid state despite a significant elevation of serum fT4 level. Cardiac evaluation showed an ejection fraction (EF) of 66% and mild diastolic dysfunction without regional wall motion abnormalities (RWMA) on echocardiography examination and a normal sinus rhythm on electrocardiogram. However, she was pretreated with methimazole 10 mg for 15 days prior to surgery and Lugol's solution was also administered once for prophylaxis against perioperative thyroid storm. TFT results one day before surgery showed that the TSH was 5. 55 µIU/ml, f T4 was > 7.77 ng/dl and T3 was 78 ng/dl.
Premedication was not given. On admission to the operating room, her blood pressure was 150/60 mmHg, heart rate was 64 beats per minute (bpm). General anesthesia was induced with thiopental sodium 5 mg/kg and 8 vol% sevoflurane mask ventilation. After administration of vecuronium 1 mg/kg, despite the huge sized goiter, the trachea was easily intubated on the first attempt and anesthesia was maintained with desflurane 6 vol% at 0.5 fraction of oxygen. The arterial blood pressure and central venous pressure were continuously monitored. She did not show signs of excess catecholamine stimulation, and on the contrary, she was hypotensive and bradycardiac throughout the surgery (systolic blood pressure < 90 mmHg, HR < 50 bpm). After several injections of ephedrine 5-10 mg, norepinephrine was administered continuously at 0.1-0.15 µg/kg/min during the surgery. Blood gas analyses were performed at regular intervals. Initial hemoglobin (Hb) was 9.2 g/dl. Although the estimated blood loss was 800-1,000 ml, packed red blood cells were transfused 6 units to correct the preexisting dehydrated state and maintain the Hb level > 10 g/dl. Hb was 10.5 g/dl in the immediate postoperative period. 2 units of fresh frozen plasma was administered. The intravenously administered fluids were crystalloid 1,300 ml, 5% HES 500 ml for the initial 30 minutes of surgery, and the total administered fluid was crystalloid 1,300 ml and colloid 500 ml. Urinary output was maintained at 120 ml/h per hour. The esophageal temperature was measured throughout the surgery. The patient was heated with a warm blanket to maintain the temperature > 36.0℃. At the end of surgery, she was weaned from norepinephrine, was successfully extubated and was transferred to the surgical intensive care unit (SICU).
Twelve hours after admission to the SICU, she became hypotensive, and dopamine was infused at 10 µg/kg/min. Her echocardiogram during the postoperative follow up showed an EF of 65% without RWMA. The input and output was balanced to maintain an euvolemic state. After elimination of putative causes for hypotension, postoperative TFT was performed on postoperative day (POD) 2, which showed TSH at 0.9 µIU/ml, fT4 > 7.77 ng/dl and T3 57 µg/dl. Liothyronine sodium (T3) was orally administered at a starting dose of 10 ug/d and the dose was then elevated to 40 ug/d. On POD 6, TFT result showed serum TSH 0. 91 µIU/ml, free T4 > 7.77 ng/dL and T3 161 ng/dL. With the administration of T3, the blood pressure became elevated, but dopamine was tapered slowly until POD 6 after confirming TFT results of the elevated T3 level. She was transferred to the general ward on POD 6. On POD 15, she was taken off T3 medication because T3 became 254 ng/dl on the follow-up tests, but the T3 medication was later resumed because T3 dropped to 62 ng/dl on POD 27. On POD 43, her serum TFT showed TSH of 14.48 µIU/ml, free T4 > 7.77 ng/dl and T3 87 ng/dl, and she was discharged with a T3 of 20 µg/d. Three weeks after discharge, she visited the endocrinology clinic, and the T3 was discontinued with a normalized T3 level (TSH 0.34 µIU/ml, free T4 > 7.77 ng/dl and T3 153 ng/dl). Perioperative TFT results are listed in .


An 8-year-old girl presented to our hospital with 1-month history of pain in the mid back, paresthesias over both lower limbs and urinary incontinence. The cognitive and language developmental milestones were normal. She was the second offspring of nonconsanguineous parents, her two male siblings were normal and there was no history of similar deformities in any of her family members. At birth, she had an abdominal wall defect below the left subcostal region which closed spontaneously over a period of 6 months.
Physical examination revealed thoracic cage asymmetry, thoracic kyphoscoliosis and discoloration of the skin in the left subcostal region. There were no other dysmorphic features or obvious external malformations. Her anthropometric measurements were as follows: height 124 cm (25th centile), arm span 124 cm, upper segment: lower segment ratio 0.88:1 and head circumference 51 cm (50th centile). On neurological examination, power was 4 ± 5 in both the lower limbs, deep tendon reflexes were sluggish, plantar response was equivocal on the right side and extensor on the left and superficial sensations were decreased below the D11 level by 20%. Her higher mental functions, cranial nerve examination and upper limb motor and sensory functions were normal.
Thoracic and lumbar radiographs showed thoracic scoliosis with convexity toward the right, multiple vertebral segmentation anomalies at the D8-D12 level including hemivertebra at D8 and butterfly vertebra at D10, multiple levels of spina bifida at D8-L2 and L3-S1 levels and rib anomalies including irregular fusion of the lower ribs with absent ribs on the left side []. Magnetic resonance imaging of the dorsolumbar spine with screening of the whole spine showed diplomyelia from D12 to L3 without any bony spur. The two hemicords were asymmetric and were surrounded by a single dural tube. There was evidence of low lying cord extending up to L3 level and multiple levels of spina bifida involving the lower dorsal and lumbosacral region [].
The patient underwent D11 to L3 laminectomy. Intraoperatively, two hemicords with a single dural tube were noted and no bony or fibrous septum was found. Thick arachnoidal adhesions were found between the two hemicords, which were released. Primary dural closure was performed, and sectioning of thick fatty filum terminale was done. Post operatively, the patient did not have new neurological deficits, her scoliosis showed mild improvement and she is on follow-up.


A 17-year-old boy presented with increasing asymmetric spastic quadriparesis for 1 year associated with sphincteric involvement of 10 months duration. He had past history of TBM diagnosed 6 months before the recent complaints. He was, since then, on four drugs ATT (isoniazid, rifampicin, pyrazinamide, and ethambutol). He also had a history of undergoing ventriculoperitoneal (VP) shunt on two occasions for postmeningitic hydrocephalus which was detected around 6 weeks after TBM was diagnosed. The patient was investigated outside for spastic quadriparesis and on the basis of magnetic resonance imaging (MRI), a diagnosis of cervical intramedullary tuberculoma (C3-C6) with a concurrent cerebellar tuberculoma was made and further drugs were added to the on-going ATTs. The neurological status however continued to deteriorate prompting the patient to attend our centre.
On examination, he was conscious and oriented. He had hypertonia involving all the four limbs and deep tendon reflexes were diminished in bilateral upper limbs and exaggerated in both the lower limbs. Power was 0/5 on the right upper and lower limbs while it was 1-2/5 in left upper and lower limbs. Bilateral babinski sign was positive and graded sensory loss was present below C4 level.
His hematological and biochemical parameters, including liver functions were normal. Chest X-ray was normal, and HIV and hepatitis-B serologies were negative.
Magnetic resonance imaging of the cervical spine and brain was repeated in our institute. It showed a well-defined T1-hypo and T2-iso to hypointense mass situated within the substance of the spinal cord from C3 to C6 with conglomerate peripheral ring like contrast enhancement. There were no vertebral body or paravertebral soft tissue changes. MRI brain revealed mildly dilated ventricular system without periventricular seepage with bilateral VP shunts in situ. There was a conglomerate ring-enhancing mass in the lower part of the right cerebellar hemisphere as well [Figure –].
Comparison with the previous radiology report (unfortunately films were not available) revealed mild reduction in the size of the cerebellar lesion; however the cervical SIT had slightly increased in size (from 2 cm × 2 cm to current 3.5 cm × 3 cm size).
In view of the lack of clinical improvement and increase in size of the SIT on continued ATT, we decided to operate him. C3-C6 laminectomy was done and after durotomy, the arachnoid was seen studded with small granulomas. Posterior midline myelotomy was done, and yellowish, soft, encapsulated mass was encountered within the cord substance. It was maximally decompressed after which the spinal cord was seen pulsating well.
Postoperatively, the patient was electively ventilated overnight and extubated uneventfully next day. His postoperative course was uneventful. At discharge, he had mild decrease in lower limb spasticity. Biopsy was suggestive of granulomatous inflammation with caseation suggestive of tuberculoma, however no acid-fast bacilli (AFB) could be isolated from the lesion [Figure and ]. He was discharged on four first line anti-tubercular drugs.
At 6 week follow-up, the patient had slight improvement in neurological status (power improved to 3/5 in all four limbs). However, at 3 months follow-up, he had further deterioration in lower limb power to grade 1-2/5. Repeat MRI revealed lack of residual enhancement inside the cord suggesting a response to ATT but a syrinx was detected at C6-D1 level. The cerebellar tuberculoma had also disappeared [Figure –]. The patient was advised to continue ATT and regular follow-up.


An asymptomatic 68-year-old man was referred to our hospital due to the presence of numerous nodular hepatic lesions accidentally detected on ultrasound. There was a history of heavy drinking (alcohol intake around 70-90 g/d) and no prior history of medication or steroid intake. The other personal and family history was unremarkable. Physical examination upon admission revealed diffuse hepatomegaly, with no associated cardiac murmur or leg edema. Laboratory investigations showed a slight elevation of γ-glutamyl transpeptidase (76 UI/L; (normal value: 0-51 UI/L).
Abdominal ultrasound (US) showed multiple ill-defined sub-centimeter hyperechoic hepatic nodules, predominately in the right lobe, with no posterior acoustic shadowing or significant through transmission and absence of high vascularity on color Doppler [].
Abdominal MRI was performed for further evaluation. Countless predominantly sub-centimeter nodular lesions with low T1-weighted signal intensity (SI) and moderately high T2-weighted SI were depicted. Liver lesions showed moderately restricted diffusion, and post-contrast fat-suppressed T1-weighted images showed predominantly hypovascular features with retention of contrast in later phases. A discontinuous centripetal filling appearance was noted on larger lesions []. There was no evidence of extra-hepatic masses or lymphadenopathy. A US-guided biopsy using an automated gun with an 18-gauge needle was performed collecting three representative fragments.
Pathology revealed endothelial-lined sinusoidal proliferation with erythrocyte content, consistent with hepatic hemangiomatosis []. No sarcomatous changes were seen. No evidence for extra-hepatic hemangiomatosis was revealed on further imaging studies. Definitive diagnosis of DHH was established based on a combination of imaging and pathological findings and clinical/imaging follow-up. Imaging follow-up included annual abdominal MRI investigation. Two years following the diagnosis, the patient's hepatic function and imaging findings remain stable, with no relevant associated symptoms.


An 18-years-old Indian male with no past psychiatric or medical history presented with a history of repetitive distressing thoughts of dirt and contamination with repetitive washing behavior for one and half years. He was diagnosed as OCD, mixed obsessional thoughts, and acts as per International Classification of Diseases (ICD-10).[] Yale-Brown Obsessive Compulsive scale (Y-BOCS) was administered at the time of admission which indicated severe (obsessions = 13; compulsions = 15; total = 28) and started on sertraline 25 mg/day and hiked to 150 mg/day over 4 weeks. Patient showed minimal improvement after 4 weeks (obsessions = 12; compulsions = 15; total = 27). He started developing side-effects like headache, thus sertraline was not hiked further and lithium 900 mg was added as augmenting agent. Patient started reporting exacerbation of repetitive thoughts (obsessions = 15; compulsions = 18; total = 33 indicating extreme) and fine tremors on fourth day after starting lithium. Serum lithium level was done at 900 mg/day (Serum lithium-1.2 mmol/L) and hence lithium was tapered to 600 mg/day and serum lithium level decreased to 0.65 mmol/L. There was improvement in Y-BOCS score within a week after reducing the dose of lithium (obsessions = 11; compulsion = 14; total = 25 indicating severe) and the dose was maintained on the same dose. Four weeks later, there was further improvement on Y-BOCS (obsessions = 9; compulsions = 12; total = 21 indicating moderate) score. Patient was discharged on sertraline (150 mg/day) and lithium (600 mg/day).


This was a case report of a 45-year-old male patient presented to the dermatology clinic complaining of a painless, slow-growing mass on his left forearm, which gradually developed over the course of 2 years []. Patient reported no allergies and did not smoke or take any specific medications. On physical examination, there was a solitary, firm, purple, mobile, non-tender nodule located in the distal part of his left dorsal forearm, which was 1.8 cm in diameter. There were no palpable regional lymph nodes. Laboratory studies, including complete blood count, erythrocyte sedimentation rate and liver function tests were within normal limits. With the possible diagnoses of pilomatricoma, dermatofibrosarcoma and adnexal tumors in mind, the tumor was surgically excised and sent for the histopathological evaluation.
Macroscopic examination showed a well-circumscribed tumor with an epithelial component in a myxoid, chondroid and hyalinized fibrous stroma []. The epithelial component was composed of clusters and solid cords of cells as well as ductal structures with occasional branching lined by two layers of cuboid cells. Solid islands of squamous epithelial cells as well as hyaline epithelial cells with plasmacytoid appearance were present [Figures and ]. The immunohistochemical study was positive for cytokeratin, vimentin and S-100 protein in the stroma. Results of biopsy and hematoxylin and eosin staining confirmed the diagnosis of CS and showed no evidence of malignancy.


A 59-year-old man was presented in our hospital because of respiratory distress since 3 days before admission, which had been gradually sever. He was admitted with impression of pulmonary edema. He had a history of uncontrolled diabetes mellitus, systemic hypertension and hyperlipidemia.
On physical examination, he was blind, with blood pressure of 210/140 mmHg, pulse rate about 150 beat/min and respiratory rate about 40 cycle/min. The pulses were equal in upper limbs. Both femoral pulses were equal but weak. There were diffuse moist rhales in both lungs and cardiac examination systolic ejection sound and murmur was audible in the aortic area and with less severity in apex and lower left sternal border.
Electrocardiography showed sinus tachycardia with complete left bundle branch block. After several hours of aggressive medical treatment, the patient’s condition became relatively stable.
Echocardiography showed sever aortic valve stenosis (mean pressure gradient = 60 mmHg) and sever LV systolic dysfunction (LV ejection fraction = 25%).
After initial stabilization with conservative treatment, coronary angiography was done and revealed three vessels coronary artery disease.
Cardiac catheterization from right femoral artery showed occlusion of the distal thoracic aorta to the left subclavian artery and angiography from right brachial artery proved interrupted aorta (type A). We were not able to pass through aortic valve to the left ventricle because of valvular stenosis, and it seems that left internal mammary artery plays role in collateralization but we did not engage through it ().
The descending thoracic aorta was supplied by extensive collateral vessels from the vertebrobasilar system down to the posterior chest wall and the spine (). Successful surgical correction including coronary artery bypass graft and aortic valve replacement and repair of interruption of the aorta was performed without any complication. Three weeks later the patient died due to gastrointestinal (GI) bleeding which was not controllable by aggressive treatment and hospital acquired pneumonia.


A 23-year-old primigravida diagnosed with TA type III along with bilateral subclavian and renal artery involvement presented to the obstetric clinic for safe confinement. She had renovascular hypertension and dilated cardiomyopathy secondary to TA.
Pre-anesthetic evaluation for elective cesarean section revealed a 68-kg, 164-cm-tall lady with a 38-weeks gestation-sized uterus. Her blood pressure was recorded as 160/96 mmHg and 164/94 mmHg in the upper and lower limbs, respectively, with a heart rate of 90/min. A detailed cardiovascular evaluation, including a review of the angiography recorded for unexplained tachycardia and high BP recording (following an attack of enteric fever 5 years back), revealed 70% and 100% occlusion of the left and right renal artery, respectively, along with bilateral subclavian artery stenosis. Records of 2D echo performed earlier revealed severe myocardial dysfunction, with ejection fraction ranging from 25% to 34%. The 2D echo performed after admission to the obstetric unit revealed an ejection fraction of 40%, dilated left ventricle, and type II diastolic dysfunction. She was on nifedipine 10 mg PO OD, digoxin 0.25 mg PO OD, levo-carnitine 500 mg PO OD, and prednisolone 30 mg PO OD. Serial abdominal ultrasound examinations at 18, 24, and 36 weeks showed normal fetal development. There were no features suggestive of cerebral involvement/ischemia with head and neck extension. All the laboratory and clinical parameters were within normal limits. Her airway was graded as Mallampatti class 2 and she was accepted for anesthesia in ASA class 2.
Because of the development of sudden fetal distress, she was taken up for emergency cesarean section. She was administered non-particulate antacid 30 ml of 0.3 M sodium citrate along with ranitidine 50 mg IV and metclopromide 10 mg IV before shifting her to the OR. A wide bore peripheral IV line was secured and hydrocortisone 25 mg was administered IV. The patient was preloaded with 500 ml of intravenous crystalloids. Under aseptic precaution, an 18 G epidural catheter was introduced through the 16 G Touhy needle via L2-L3 interspace using the loss of resistance to saline technique, followed by subarachnoid block with 1 ml of 0.5% hyperbaric bupivacaine injected intrathecally via the L3-L4 interspace through a 25 G Quincke's needle. The patient was placed in the left lateral position by keeping a wedge under the right hip and thigh. Five minutes later, 8 ml of normal saline was injected via an epidural catheter. After administration of 1.5 mg midazolam, right jugular canulation was performed using sterile precautions. Dermatome level of anesthesia was found to be T9, T7, and T4 at 3, 5, and 10 min after epidural saline injection, respectively.
Non-invasive blood pressure was monitored in both the upper and the lower limbs and the pulse oximeter probe was placed on the right upper limb. A healthy baby was delivered. Intraoperatively, central venous pressure was maintained between 10 and 12 cm of H2 O. The surgical time was 45 min and the blood loss was 600 ml. The intraoperative period was uneventful and the patient was shifted to the post-operative ward for further management.


We encountered an 8-years-old male child weighing 22 kg admitted for fracture both bone forearm surgery with history of fall from tree 15 days back. Bone was coming out of the wound. He was talking irrelevantly and having hallucinations. While examining, the patient started developing intermittent spasm of upper limbs. Ionized calcium done which was 9.1, total leukocyte count (TLC) 21,500, blood urea 58 mg%, and other routine investigations were normal. He received last tetanus toxoid at 5 year age. Patient had painful spasms, with no trismus. Deep tendon reflexes were brisk, clonus+, and planter was silent. He was given human tetanus immunoglobulin 500 U intramuscluar (i.m), intravenous (iv) diazepam 7.5 mg QID, phenobarbitone 500 mg in 100 ml normal saline (N.S.) slow infusion followed by 100 mg OD, metronidazole 250 mg TDS, dressing changed alternate day and wound condition improved. On 4th day of admission, patient was posted for surgery. Patient was conscious, oriented, with adequate mouth opening and Mallampatti grade II, heart rate 130/min and blood pressure was 126/74 mmHg. Premedication with inj. midazolam 1.5 mg and fentanyl 50 μg was given. After preoxygenation for 3 min, induction was done by propofol 60 mg, vecuronium 2.5 mg, ventilated with O2+ N2O 50:50 with sevoflurane 2% and tracheal intubation was done with 5.5 ID cuffed PVC endotracheal tube. Anesthesia was maintained with sevoflurane 2% and vecuronium intermittently when required. Intraop vitals were stable. On completion of surgery, reversal given and patient was extubated uneventfully and shifted to recovery room.


A 32-year-old male migrant worker from St Lucia presented to the emergency department with a one-week history of myalgias, headache and fever. He was previously healthy. He was visiting Canada temporarily and had arrived in Canada 11 days before his presentation. One day after arriving in Canada, he ate commercially prepared hamburger patties, which he reported as “off-tasting”. Five days later, he became fatigued and experienced a bilateral, intermittent headache associated with photophobia and visual aura. He noticed that his eyes were bloodshot. Three days later, he developed loose, nonwatery, non-bloody diarrhea, associated with fever and rigors, which resolved completely in two days. At presentation, he complained of lethargy, lower-extremity myalgias, tea-coloured urine and intermittent epigastric discomfort. On examination, his temperature was 37.2°C, heart rate 87 beats/min, respiratory rate 18 breaths/min and blood pressure 113/72 mmHg. He had scleral icterus with conjunctival edema (). He had no hepatosplenomegaly. His leukocyte count was 19.9×109/L, hemoglobin level 124 g/L and platelet count 24×109/L. His electrolyte levels, bicarbonate levels and anion gap were within normal limits; however, his creatinine level was 352 μmol/L and urea level was 18 mmol/L. His aspartate aminotransferase level was 310 U/L, alanine transaminase level 175 U/L, alkaline phosphatase level 79 U/L and total bilirubin level 191 μmol/L. His initial creatine kinase level was 2343 U/L and troponin I level was 1.6 μg/L. An initial electrocardiogram showed sinus rhythm with first-degree atrioventricular block and an incomplete right bundle branch block.
He was admitted with an initial, presumptive diagnosis of hemolytic uremic syndrome or thrombotic thrombocytopenic purpura, and received two units of fresh frozen plasma. A hemolysis work-up returned negative: serum lactate dehydrogenase level was 353 U/L, haptoglobin level 2.9 g/L, total bilirubin level 191 μmol/L, indirect bilirubin level 60 μmol/L and direct bilirubin level 131 μmol/L. On a blood film, fragments were only rarely observed. Abdominal ultra-sonography revealed hepatic periportal echogenicity, diffuse renal cortical echogenicity and no hydronephrosis. Echocardiography showed mild concentric left ventricular wall thickening and no regional wall motion abnormalities, with a preserved left ventricular ejection fraction (54%). Furthermore, serology for cytomegalovirus, hepatitis A virus, hepatitis B virus, hepatitis C virus and HIV were negative. Blood films failed to show intraerythrocytic trophozoites. Blood cultures were negative. Stool culture for Escherichia coli O157:H7 was also negative.
He remained clinically stable and had good urine output with improving kidney function. However, his leukocyte count increased to 37.6×109/L and his direct bilirubin level and erythrocyte sedimentation rate increased to 169 μmol/L and 110 mm/h, respectively, suggesting progression of his hepatopathy and ongoing systemic inflammation.
A diagnostic test was performed.


A Male patient aged 24 years presented to the Department of Oral and Maxillofacial Surgery, Postgraduate Institute of Dental Sciences, Rohtak, with pain in the right preauricular region, which, exaggerated during chewing. The patient further felt shifting of the lower jaw towards opposite side. On clinical examination there was obvious facial asymmetry with deviation of chin to the contralateral side and associated angulation of labial commissure and lateral tilting of mandibular incisor teeth, which accentuated the nasolabial fold over the unaffected side []. Further, there was slight but conspicuous posterior open bite on the affected side and associated anterior cross bite over the uninvolved side [].


A male aged 20 years presented with symptom of facial disfigurement. There was deviation of chin toward the left side with flattening of face along with shifting of facial midline and associated malocclusion [Figures and ].
Being a BDS student, the patient was very conscious of facial asymmetry.


This was a case report of a 22-year-old female with an unremarkable medical and family history who presented to us with a painless swelling in the left parotid region of 2 years duration, examination revealed a well-circumscribed 3 cm × 3 cm swelling in the left parotid region []. The facial nerve function was preserved and there was insignificant cervical lymphadenopathy. A magnetic resonance imaging scan of the parotid region confirmed the nature and extent of the swelling in the left parotid gland []. Aspiration cytology from the cyst revealed an acellular proteinaceous material with occasional squamoid cells, degenerating lymphoid cells and histocytes. The cyst reappeared in a few days time; she was hence taken up for surgery. The intra-operative finding showed the cystic lesion within the substance of the superficial lobe of the parotid gland. A left superficial parotidectomy preserving all the branches of the facial nerve was successfully done, no fistulous tract was identified. The histopathology showed a cystic lesion measuring 3.5 cm × 2.5 cm × 2.5 cm surrounded by normal parotid tissue all around. The cyst was lined by ulcerated stratified squamous epithelium; the cyst wall showed lymphoid tissue with deep seated investigations of the squamous cells, the lumen was filled with necrotic debris, features suggestive of a brachial cleft cyst []. She is disease free and continues to be on follow-up for over 2 years.


A 69-year-old man had a decades-long history of chronic alcohol consumption, consuming approximately 500–550 mL of Japanese sake daily (standard alcohol content, 15% v/v; about 60–66 g ethanol/day). Up to about 1 year before admission, although he often experienced falls, he was independent in activities of daily living. Four days prior to admission, he had difficulty walking and stopped drinking. Three days prior to admission, the level of activity deteriorated, and he ceased speaking at home. On the day preceding admission, he was unable to walk or stand.
On admission, the patient showed normal vital signs. In terms of the level of consciousness, he showed stupor, confabulation, delusive talking, agraphia, and ideomotor apraxia. Hyperreflexia of deep tendons, bilateral positive Babinski reflex, and muscle hypertonia (spasticity) were observed on neurological examination with brachybasia and instability by walking. He scored 15/30 on the Mini Mental State Examination (MMSE). Laboratory tests, including blood count, biochemical tests, blood sugar, ammonia, thyroid function tests, and levels of vitamins B1, B2, and B12, folic acid, and soluble interleukin-2 receptor, were all within normal limits. Cerebrospinal fluid examination also showed normal results.
Head MRI performed on admission revealed hyperintensity on fluid-attenuated inversion recovery (FLAIR) imaging in the corpus callosum and frontal white matter (), but DWI did not reveal any abnormal signals (). Based on the characteristic MRI findings and clinical history, MBD was diagnosed and treatment was started.
The patient was able to eat and drink, so he consumed regular meals, stopped drinking alcohol, and began medical rehabilitation. He was given thiamine 75 mg/day beginning the day after admission and nicotinamide 1.5 g/day beginning 5 days after admission.
Follow-up head MRI was performed 12 days after admission. The hyperintensity in the total area of corpus callosum remained the same on FLAIR imaging (), but the white-matter lesion in the left frontal area was decreased in size (). The callosal lesion showed hyperintensity on DWI (). Part of this lesion appeared to have a reduced apparent diffusion coefficient (ADC) ().
Another follow-up MRI was performed 39 days after admission, showing cavitary changes in the corpus callosum ( and ). After treatment, motor functions gradually recovered, but mild agraphia and apraxia remained. He was independent for activities of daily living and scored 29/30 on MMSE 67 days after admission.


In September 2008, a 37-year-old woman underwent surgery for breast cancer in our institution. The postoperative clinical stage was T2N0M0. She underwent postoperative chemotherapy and endocrine therapy. In January 2009, multiple osteoblastic bone tumors, which had been causing severe back pain, were found on contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI). Bone lesions were found in the whole spine, but as she complained of severe back pain, 40 Gy external radiotherapy was given from the 5th to the 9th thoracic vertebrae. After external radiotherapy, systemic chemotherapy was continued. In August 2010, multiple liver metastases were found on contrast-enhanced CT and the patient suffered from severe left hip pain. A bone scan showed increased uptake of Tc-99m hydroxymethylene diphosphonate (HMDP) in an area consistent with the left iliac bone. After 40Gy external radiotherapy of the left iliac bone, systemic chemotherapy was resumed. Despite continuing treatment, the multiple bone metastases and liver metastases increased in size. Serum carcinoembryonic antigen (CEA) and cancer antigen 15–3 (CA 15–3) were increased. Bone scan showed multiple uptake of Tc-99m HMDP consistent with multiple bone metastases (), and on contrast-enhanced CT, the right 9th rib was destroyed by a huge bone tumor (). The multiple bone metastases were more progressive in size than the liver metastases and the severe pain could not be controlled with the use of opioid analgesics. After written informed consent was obtained, 89SrCl2 therapy was planned in order to control the pain.
In December 2011, 89SrCl2 (Metastron; GE Healthcare UK Ltd., Little Chalfont, UK) was administered at a dose of 124.0 MBq (2 MBq/kg). Pre-treatment blood tests showed white blood cell count of 3.2 × 103/µL, hemoglobin 11.2g/dl, and platelet count 16.4 × 103/µL. No major toxicity was found that required care related to the 89SrCl2 injection. The patient did not receive concurrent and adjuvant systemic chemotherapy. According to the Common Terminology Criteria for Adverse Events (CTCAE), Grade 2 anemia, Grade 1 leucopoenia, and Grade 1 thrombocytopenia were found at 3–8 weeks after administration of 89SrCl2, but resolved without treatment. The patient reported pain relief 2 weeks after the 89SrCl2 injection. Over time, the pain was further controlled.
Five months after the 89SrCl2 injection, a bone scan showed decreased uptake of Tc-99m HMDP consistent with multiple bone lesions in regression (), and contrast-enhanced CT revealed bone tumor regression of the right 9th rib (). In consistence with the shrinking of multiple bone metastases, the CEA and CA 15–3 levels decreased (). However, as the patient did not receive any systemic chemotherapy during this period, the liver metastases increased in size (). In May 2012, pain control was satisfactory, but a second injection of 89SrCl2 was administered at the request of the patient, because lower back pain from bone metastases remained. One month after the 89SrCl2 injection, transcatheter hepatic arterial chemotherapeutic infusion (TAI) was conducted for multiple liver metastases. After the second 89SrCl2 injection and TAI, the multiple bone metastases and liver metastases were well controlled. Currently, the patient is being treated on an outpatient basis without progression of symptoms.


A 74-year-old woman, non-smoker, who underwent bilateral TKA for the treatment of osteoarthritis, presented with mild dyspnea and a small amount of hemosputum 2 days after surgery. She was given 2,000 IU of enoxaparin twice daily to prevent deep vein thrombosis from 24 hour after surgery. Her respiratory failure developed on day 4. We suspected that she might have congestive heart failure because 1250 ml of fluids were given during surgery, then followed by 1000 ml in the next 12 hours, and 400 ml of autologous blood was given on day 3. In addition, a chest radiograph (Figure A) and computed tomography (Figure A, B) scans showed not only ground-glass opacity, but also pleural effusion, in both lungs. The patient's N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level was 1,212 pg/ml on day 5 after surgery. At that time, we presumed that her hemosputum and respiratory failure were associated with heart failure. Prothrombin time and international normalized ratio (PT-INR) was 0.95 and activated partial thromboplastin time (APTT) was 32.4 second at that time.
Six days after surgery, respiratory failure developed despite treatment of heart failure with a diuretic. We performed bronchofiberoscopy to identify the cause of her respiratory failure. We presumed the patient might have DAH, because her bronchoalveolar lavage fluid (BALF) was red in color and thickened during 3 sequential lavages. Approximately 12% of hemosiderin phagocytic macrophages were found in the BALF. No bleeding focus was remarkable in the upper airway, or bronchus.
DAH is caused by a high incidence of infection, autoimmune vasculitis, or malignant neoplasm in the lung or airway. However, no autoantibody, including ANCA, was detectable in the blood; no pathogen was detectable in the cultured BALF; and no new medication was started other than enoxaparin. Therefore, we assumed enoxaparin might be a major cause of DAH and discontinued it.
The chest radiograph abnormalities and respiration improved by the next day after stopping enoxaparin. At that point, a slightly ground-glass opacity remained on the chest radiograph, but gradually became clear after 2 weeks from surgery and after a week from stopping enoxaparin (Figure B). Echocardiography performed before and after surgery showed normal left ventricular function.


A 14-year-old male patient visited our hospital with headache, nausea, vomiting, dysarthria and right hemiparesis grade I as the main symptoms. Dark brown colored large melanocytic nevus that covered about 1/2 of the trunk and various sized nevus ranging from several millimetre to several centimetre were observed throughout the whole body of the patient ().
When the patient was 6 months old, in the dermatology department of our hospital, histologic examination of nevus sections showed nevus cell infiltration with pigmentation in the reticular dermis. No evidence of malignant transformation was found (). The patient suffered an onset of seizure when he was two years old and received treatment with anticonvulsant medication at the pediatric department of our hospital. Under NCM impression at the time, serial follow-ups for clinical symptoms were done for 12 years by the pediatrics department. During that time, there was no manifestation of abnormal neurological symptoms. The patient visited due to motor weakness. The computed tomography (CT) imaging scans showed intracerebral and intraventricular hemorrhage throughout left posterior frontal cortical area and left lateral ventricle. Under magnetic resonance image (MRI) gadolinum-enhanced T1 weighted image, partially high signal was detected in the cortical area of frontal lobe. Small inhomogenous high signal was also observed on the pons, temporal horn, and anterior portion of lateral ventricle through gadolinum-enhanced T1 weighted image. MRI scans also revealed a cyst in the posterior fossa and hypoplasia of the cerebellar vermis with dilatation of the entire ventricular system. Longitudinal diffuse enhancement lesion was observed along the posterior dura at the thoracic level in the whole spine MRI scans carried out for the purpose of screening but nodular lesion or focal mass lesion was not observed in other areas (). Although surgical treatment under NCM impression accompanies by DWC was recommended, we could not operate on him due to refusal by the guardian. After the conservative care during one month, the patient was discharged with improvement of neurological symptoms. However, the patient was hospitalized again with headache and right hemiparesis grade I after 4 months. Large mass, which is a marked enlarged state of left frontoparietal area, was observed under the MRI scans. The surgical treatment was performed. Tumor along with diffuse dark colored leptomeninges and with soft, friable, vascularized and poorly defined borders of margin was removed gross totally (). Histopathologic examination showed melanin-pigment laden neoplastic cells, necrotic change and pleomorphic malignant cell, which could be diagnosed the malignant melanoma (). No pathologic deposits of the skin area were seen in the positron emission tomography with 18-FDG (). It was possible to definitively diagnose NCM with DWC. After 2 month following first brain surgery, the patient was discharged with symptom improvement. Three months after discharge, the patient was hospitalized again with observation of increased intracranial pressure symptoms such as decreased mentality, headache and nausea. Re-grown large mass on the left frontoparietal area and new mass on left medial temporal area were observed under MRI scans (). The mass on the left frontoparietal area was removed gross totally once again through surgery with same pathological findings. On the 3rd week following the second surgical treatment, the patient complained of back pain, voiding difficulty, left side motor weakness grade III and respiratory difficulty. So spinal MRI scans was done, and mass lesion with high signal at the T2 weighted image and inhomogenous high signal at gadolinum-enhanced T1 weighted image along the C5-T3 area were observed, and bilateral laminectomy with partial removal of the mass was performed. The intradural mass showed a soft, friable, highly vascularized characteristics and infiltrated into the leptomenignes with extremely poorly defined border (). Although performing aggressive treatment, decreased of consciousness, respiratory difficulty and motor weakness continued in the patient, and progressive hydrocephalus was observed on CT scan. The guardian did not wish further treatment. The patient died on the 3rd week following the spine surgery.


A 27-year-old woman (height 156 cm, weight 44 kg) who was 16 weeks pregnant was scheduled for emergency dilation and curettage (D&C). She was diagnosed with missed abortion with a hydatidiform mole in a local clinic and transferred to our hospital for further treatment. She had no medical or surgical history, and her vital signs upon admission were 160/95 mmHg- 88 /min- 20 /min- 36.5℃. Her chest X-ray, electrocardiogram, and laboratory tests completed at the local clinic were normal, with the exception of the beta human chorionic gonadotropin (β-hCG) level of 1,046,900 mIU/ml (normal: 0.5-2.9 mIU/ml). After vaginal insertion of misoprostol (prostaglandin E1) for abortion, she developed chilling sensations and vital signs were 156/106 mmHg- 125 /min- 20 /min- 38.1℃. The obstetricians removed the misoprostol due to suspected side effects, but the chilling sensations continued. She showed nausea and agitation with a blood pressure (BP) of 183/107 mmHg and heart rate (HR) of 158 /min. The obstetricians suspected preeclampsia and magnesium sulfate and hydralazine were administered repeatedly, but they were not effective for controlling the BP and HR. The obstetricians considered septic complications from the hydatidiform mole and we decided to perform an emergency D&C under general anesthesia. This situation was considered critical, and we conducted the anesthesia before the results of laboratory test done in our clinic were known.
After arrival in the operating room, routine monitors were applied and vital signs were 169/85 mmHg- 180 /min- 40 /min- 38.1℃. Anesthesia was induced with pentotal sodium (200 mg) and rocuronium (20 mg). After intubation, esmolol (20 mg) and nicardipine (0.5 mg) were given to attenuate the hemodynamic response. Anesthesia was maintained with 1.5 vol% sevoflurane in medical air (FiO2 0.5) and remifentanil in 4.0-6.0 ng/ml by a target-controlled infusion (TCI, Orchestra® Base Primea, Fresenius Vial, Grenoble, France). Esmolol (10-30 mg) and nicardipine (0.5-1.0 mg) were administered intermittently, but hypertension and tachycardia persisted. Labetalol was infused continuously and her BP and HR decreased to 130-140/60-80 mmHg and 105-120, respectively. At the end of the 20-min operation, the recovery from the muscle relaxant was incomplete and she was sent to the recovery room in an intubated state. The estimated blood loss was 100 ml, and 500 ml crystalloid was infused.
After 20 min in the recovery room, the neuromuscular block was measured by the train-of-four ratio and reversed with 0.2 mg glycopyrrolate and 10 mg pyridostigmine. After extubation, persistent hypertension of 130-160/90-100 mmHg, tachycardia of around 130-140 /min, and hyperthermia of 37.9℃ were noted. No proteinuria or leukocytosis was found on preoperative laboratory tests done in our clinic, and preeclampsia and sepsis were ruled out. Physical examination and laboratory tests were repeated to find the cause of continuous tachycardia and hypertension. We found a small lump on her neck and suspected a thyroid storm. A thyroid function test (TFT) was done and she was transferred immediately to endocrinology. Hydrocortisone (200 mg) was administered empirically and labetalol was infused continuously to control her BP and HR. An ice bag and intravenous cold fluid were applied to reduce her fever.
Postoperative TFT showed elevated triiodothyronine (T3)/ thyroxine (T4) levels and a decreased thyroid-stimulating hormone (TSH) level (). An endocrinologist diagnosed her with secondary thyrotoxicosis due to molar pregnancy. One day after her operation, she was in a clinically euthyroid state and no further treatments were needed. The patient was discharged on the fourth day after the operation without complications. The TFT and β-hCG values returned to normal 3 months later ().


A 39-year-old male patient with a background of type II diabetes, spina bifida and meningocele hydrocephalus presented to the emergency ENT clinic with a 2-day history of a throat complaint. He described that while hyperextending his neck to finish his cup of coffee he felt a sudden crack in the middle and central portion of his throat. This crack produced a sound that was also heard by his partner on the other side of the room. The patient subsequently experienced a spontaneous coughing fit. During the history, he described pain over the thyroid cartilage, odynophagia, pain on moving his neck and a hoarse voice. He also described the sensation of having something flapping in his throat while breathing. He denied any recent history of throat trauma and had no other ENT complaints. On examination he had a tender thyroid cartilage with palpable crepitus of the thyroid cartilage when balloting it between the fingers of each hand. There was no palpable surgical crepitus of the soft tissues of the neck and no lymphadenopathy. Flexible fibre optic nasendoscopy allowed dynamic and real-time evaluation of the vocal cords and revealed significant bruising and swelling of the right arytenoid cartilage. Potential cord avulsion or paralysis was not identified. A computed tomography (CT) scan was subsequently arranged to further assess the laryngeal anatomy and was reported to show locules of free air in the soft tissues of the neck with generalized oedema of the paraglottic tissues (Fig. ). There was no fracture of the cartilage. The patient was admitted for strict airway observation, given a stat dose of IV steroid and started on IV antibiotic to cover supraglotittis. Blood biochemistry and haematology samples were normal, with the exception of the C-reactive protein (a marker of inflammation), which was 58 mg/l. Repeat nasendoscopic examination the following day showed that the bruising had migrated into the right vocal fold and a new fullness in the left pyriform fossa. By Day 3 following admission the nasendoscopic examination was improving to normal and the right arytenoid cartilage was seen to be less floppy. By this time the patient's pain was settling, although the hoarseness of voice was still present. He was discharged with 7 days of oral antibiotics and was reviewed in ENT clinic at 1 week where, although his voice was still hoarse, the nasendoscopic examination was normal. By 6 weeks, the voice had settled, the thyroid cartilage crepitus was gone and the larynx had returned to normal.


A 68-year-old male noticed headaches on July 8, 2012, and then right hemiparesis on July 19 of the same year. The primary care physician diagnosed stroke, and he was admitted to hospital. He had no medical history of head trauma, sinusitis, or diabetes, but he drank 1,800 mL of a distilled spirit (equivalent to 330 g of ethanol) with blue cheese every day, and had alcoholic liver cirrhosis (Child-Pugh grade B). He was febrile (39.0℃) and had become progressively paralyzed on the right side of his body. Brain magnetic resonance imaging (MRI) disclosed several ring-enhancing lesions in the brainstem and cerebral hemispheres. A cerebrospinal fluid (CSF) examination revealed pleocytosis, and a blood examination revealed an increased titer of Aspergillus antigen without inflammatory reaction; the (1→3)-β-D-glucan test was negative.
Bacterial cultures taken from two independent vessels grew L. monocytogenes, which led to a diagnosis of multiple brain abscesses due to L. monocytogenes infection. He was treated with ampicillin (ABPC; 2 g every 4 h i.v.) and voriconazole (200 mg/day), but he developed consciousness disturbance and complete right-sided hemiparesis. Follow-up brain MRI disclosed extended lesions, and the patient was transferred to our hospital. Upon admission he had a body temperature of 36.9℃, and a physical examination revealed neither superficial lymphadenopathy nor hepatosplenomegaly. A neurologic examination revealed that he was in a comatose state (score on the Japan Coma Scale of 30-200). Right blepharoptosis and anisocoria (3.0 mm on the right and 4.0 mm on the left) was observed. The left light reflex was attenuated, while that on the right was preserved. His oculocephalic reflex was positive. The right hemiparesis was severe but without muscular waste and fasciculation. The tendon reflexes were exaggerated in the right upper and lower limbs, but without pathologic reflexes. Neither neck stiffness nor Kernig's sign was detected. Laboratory data revealed no inflammatory reaction such as leukocytosis or elevation of C-reactive protein, but pleocytosis was evident in the CSF: 101 cells/mm3 (96.6% monomorphonuclear and 3.4% polymorphonuclear cells); protein, 201 mg/dL; and glucose, 58 mg/dL (plasma glucose, 78 mg/dL). No pathogenic bacteria grew in bacterial cultures of the CSF, blood, urine, and a nasopharyngeal swab.
Brain MRI disclosed several ring-enhancing lesions on the left cerebral hemisphere and brainstem (). High-intensity signals were present inside the mass lesions in diffusion-weighted imaging (). Susceptibility-weighted imaging disclosed a dual rim sign, defined as two concentric rims at the lesion margins, with the outer rim being hypointense and the inner rim hyperintense relative to the cavity contents (). MR spectroscopy revealed no elevation of choline in the lesions. Positron-emission tomography (PET) demonstrated no uptake of 2-[fluorine-18]-fluoro-2-deoxy-D-glucose, suggesting the absence of malignancy.
Treatment with ABPC (2 g every 4 h i.v.) and voriconazole (100 mg every 12 h i.v.) was continued. Diagnostic and therapeutic drainage for brainstem lesions is not favored at our neurosurgical department, and so the patient instead underwent HBO therapy (100% O2 at 196.1 kPa abs at 1 h/day, for 25 days) from August 17. After the treatment, his consciousness status and pleocytosis gradually improved. The anisocoria had resolved by August 20, and voluntary movements of the right upper and lower limbs were possible by September 5 (). Since the lesions were dramatically ameliorated on follow-up MRI, we terminated the voriconazole and ABPC therapy on October 3 and 10, respectively. The patient was transferred to another hospital for rehabilitation on October 17.