A 53-year-old woman presented to the Cardiology clinic with a 1-month history of retrosternal chest tightness since the bereavement of a friend. Examination was unremarkable. The past medical history included hypertension and there was a family history of premature coronary artery disease. The pain radiated to shoulders, arms, jawline and was present at rest. The patient was, therefore, admitted to hospital for urgent cardiac investigations and inpatient coronary angiography. Post-admission, despite medical therapy, she continued to experience ongoing chest pain in the days preceding and during angiography.
Blood tests: Full blood count, renal function tests, and high-sensitivity troponin I levels were normal.
Echocardiogram: Biventricular size and systolic function normal with no regional wall abnormalities and no significant valvular disease.
Twelve-lead electrocardiogram (ECG): Sinus rhythm with T-wave inversion in the inferolateral leads.
The patient underwent coronary angiography via the right radial artery using a 6-Fr sheath. The left coronary artery was engaged using a Voda left 3.5 guide catheter and initial injections in the posteroanterior caudal view revealed TIMI 0 flow in the first obtuse marginal (OM1) branch (Figure ). The left anterior descending artery was free of disease and by the second contrast injection, OM1 flow had normalized (Figure ). Assessment of this vessel using optical coherence tomography (OCT) revealed no abnormalities (Figure ), suggesting that the temporary occlusion was due to vasospasm.
The right coronary artery (RCA) was engaged using a Judkins Right 4 catheter and selective angiography revealed a critical lesion in the mid-vessel (Figure ). In view of the history of stress-induced chest pain, the lesion was interrogated with OCT. This revealed a significant intramural haematoma within the mid-vessel causing a critical stenosis with a minimal luminal area of just 1 mm2 (Figure ).
These findings were consistent with Type 3 SCAD. In view of the patient’s ongoing ischaemia and ECG changes, the decision was made to treat this lesion. A 2.5-mm semi-compliant balloon was inflated to six atmospheres to gently pre-dilate the vessel. Repeat angiography and OCT imaging revealed improved appearances of the lesion (Figure ).
A 4 × 10 mm Flextome cutting balloon (Boston Scientific, MA, USA) was then gently inflated for 8 s to nominal pressure on three occasions (Figure ). This led to complete resolution of the intramural haematoma with only very minimal residual intimal dissection on OCT (Figure ). The maximal luminal area of the vessel by the end of the procedure increased to over 10 mm2.
Final angiographic appearances showed excellent resolution of the lesion and TIMI III flow (Figure ). The patient’s symptoms resolved and she was subsequently discharged 2 days later on a regimen of aspirin and clopidogrel for 3 months and aspirin alone thereafter. This dual antiplatelet regimen was prescribed due to disruption of the intimal layer by balloon angioplasty. The patient was followed-up in clinic and found to be asymptomatic with no further recurrences of angina. She will be investigated for associated conditions such as fibromuscular dysplasia.


A 30-year-old man with no remarkable medical history was admitted initially because of progressive bilateral leg edema and hydrocele testis, accompanied by a decreased serum albumin level. He had also noticed the symptoms of dry eyes and mouth. His serum albumin level was 1.5 g/dL. He received symptomatic treatments at another hospital previously; however, his albumin level further decreased to 1.2 g/dL in the following three months. Contrast-enhanced abdominal computed tomography (CT) revealed a thickened small intestinal wall with increased contrast enhancement in an extensive intestinal segment accompanied by multiple swollen mesenteric lymph nodes.
Four months after the clinical onset, he was referred to our hospital because of progressive hypoalbuminemia without clinical improvement. Upon admission, severe pitting edema in both legs and eye oil secretions were confirmed on physical examination. His appetite was normal with normal stool appearance. His body temperature was 36.8°C, blood pressure was 140/84 mm Hg, heart rate was 74 bpm, height was 174 cm, and body weight was 66.2 kg; the latter was significantly greater than that observed prior to the clinical onset. A blood test revealed normal liver and renal functions, total protein level of 4.0 g/dL (normal: 6.6-8.1), albumin level of 0.9 g/dL (4.1-5.1), prealbumin level of 11.3 mg/dL (22-40), C-reactive protein level of 0.21 mg/dL (0.00-0.14), erythrocyte sedimentation rate of 83 mm/1 h (2-10), d-dimer level of 7.1 μg/mL (0.0-1.0), fibrinogen level of 533 mg/dL (200-400), free light-chain κ/λ ratio of 2.10 (0.26-1.65), antinuclear antibody (ANA) result of 1:1280, anti-dsDNA level of 7.3 U/mL (0-12), anti-SS-A level >1200 U/mL (0-9.9), anti-SS-B level >1000 U/mL (0-9.9), rheumatoid factor level of 11.7 U/mL (0-15), MPO-ANCA level <1.0 U/mL (0-3.4), PR3-ANCA level of 3.8 IU/mL (0-3.4), sIL-2R level of 802 U/mL (122-496), C3 level of 63 mg/mL (73-138), C4 level of 19.6 mg/mL (11-31), and CH50 level of 23.4 U/mL (31.6-57.6). Urinalysis revealed urine protein (±), urine occult blood (−), and urine white blood cells (−). Pleural effusion revealed a protein level of 1.5 g/dL, LDH level of 58 U/L, and cell count of 225/μL. Serum protein electrophoresis showed a relative increase in the α2 and γ fractions without a monoclonal spike. Chest X-ray revealed unilateral pleural effusion on the right side. Upper and lower endoscopy revealed no abnormal findings except for suspected mild duodenitis. Histopathological study of the biopsied specimens from the colonic mucosa showed highly edematous stroma of the lamina propria mucosa with lymphocytic infiltration (Figure A left). Immunostaining was performed in the specimens biopsied from the duodenal mucosa, which revealed strong C1q, C3d, and immunoglobulin G (IgG) staining mainly around the vessels (Figure A middle).
Based on the findings of abnormally high levels of serum anti-SS-A and anti-SS-B antibodies, the presence of oral and ocular symptoms, and a decreased unstimulated whole-saliva flow rate of 0.046 mL/min, the patient was diagnosed with SS. Biopsy of the lower lip was performed, which revealed lymphocytic infiltration around the ducts, accompanied by atrophic acini and fibrillation of the ducts (Figure A right). The patient did not meet the diagnostic criteria for systemic lupus erythematosus developed by the Systemic Lupus Collaborating Clinics., At this stage, PLGE associated with SS was suspected, and technetium-99m (99mTc)-labeled human serum albumin (HSA) scintigraphy was performed, which revealed protein leakage from an extensive area of the small intestine (Figure B).
Immune suppression therapy with oral prednisolone (40 mg/d from day 3) and mycophenolate mofetil (500 mg/d from day 14) was initiated, instead of cyclophosphamide pulse therapy, which the patient refused because of its possible carcinogenicity. The treatments resulted in drastic improvements both in the hypoalbuminemia and in the systemic edema (Figure C). At present, after treatment for 12 months with prednisolone (8 mg/d) and mycophenolate mofetil (2000 mg/d), the serum albumin level has stabilized over 4.0 g/dL without any clinical symptoms.


A 3-month-old Saudi boy, who was in a good health before, presented to the emergency department of our maternity and children hospital with grayish-blue discoloration of the skin and respiratory distress that started 6 h before admission. There was no history of aspiration or abnormal movements. The mother had noticed some degree of abdominal distension without vomiting or diarrhea, but the infant had no fever. He was on breastfeeding plus formula milk, which was prepared using bottled water. In terms of family history, the patient had two brothers with glucose-6-phosphate dehydrogenase (G6PD) deficiency.
The initial physical examination included mottling of the skin with central and peripheral cyanosis. The patient was irritable, tachypneic with a respiratory rate fluctuating between 80 and 90 breaths/min. Furthermore, he was tachycardic and hypoxic, with an oxygen saturation of around 65% in room air and 76% with 10 L of oxygen with a nonbreathing mask. His initial blood pressure was 85/43 mmHg, which was normal for the age and gender. The patient was found to have poor peripheral perfusion, cold extremities with capillary refilling time exceeding 4 s. Other systemic survey was unremarkable.
Because of the persistent hypoxia and tachypnea, a decision of intubation was made and the patient was connected to mechanical ventilation (synchronized intermittent mandatory ventilation mode). The initial laboratory results included Hb (10.4 g/dl), hematocrit (32.9%), white blood cell count (≥24,000/mm) and platelet count (≥455,000/mm). The renal function tests and levels of electrolytes were within normal range. In addition, his chest X-ray was normal. The first venous blood gas showed very high methemoglobinemia (66.9%) with pH 7.137, pCO2 28.7 mmHg and HCO3 10.4, with a base defect of 18.0 matching the picture of metabolic acidosis with lethal methemoglobinemia. Based on this, the parents were asked if any medication or toxic material was given to the child before onset of the symptoms, when they revealed that he was given a gum resin of F. asafetida, which is locally known as heltit, as a treatment for infantile colic.
The toxicology center was contacted, and it was confirmed that this herbal medication can cause methemoglobinemia.[] To stabilize and manage the critical levels of methemoglobinemia, intravenous methylene blue is the preferred treatment option. However, because the patient had a positive family history of G6PD deficiency and the patient's deficiency status was unknown, methylene blue was not used as it could have caused hemolytic anemia and further aggravated methemoglobinemia. Ascorbic acid is another agent commonly used in the management of methemoglobinemia, but it was not available in our hospital. Therefore, the patient was kept on 100% oxygen plus other supportive care measures, and his oxygen saturation kept fluctuating around 94%. Meanwhile, the patient's blood samples were sent to determine the level of G6PD enzyme and reductase B enzyme. The level of methemoglobinemia dropped to 37.3% after 3 h and to 24.2% after 4 h of starting hyperoxia. While the patient was ventilated with a fraction of inspired oxygen of 1.0, arterial blood gas analysis showed an oxygen partial pressure of 305 mmHg and an oxygen saturation of 92%–94% on the pulse oximeter, which supported our diagnosis of methemoglobinemia. Furthermore, the level dropped to 7.4% after 10 h and FiO2 gradually decreased to 40%. The patient then stabilized and was kept on low ventilator settings. On the 3rd day of admission, he was extubated and discharged to the general pediatric ward. The level of methemoglobinemia gradually dropped to normal. The level of Met-Hb reductase B was 10.7 U/g Hb (reference range 6.6–13.3 U/g Hb), which excluded the possibility of congenital deficiency. The results of G6PD enzyme came as normal. The patient was discharged with no medications and was in good health when followed up in the outpatient department after 1 month.


A 63-year-old male patient with type 2 diabetes mellitus was referred to us under the impression of proliferative diabetic retinopathy (PDR) with DME following panretinal photocoagulation (PRP) and intravitreal injection of bevacizumab in both eyes. He then received an intravitreal injection of Ozurdex in the left eye due to persistent, recurrent macular edema (VA 2/60; central retinal thickness = 513 μm) after another eight intravitreal injections of ranibizumab (IVIR) (4 months after the last IVIR). Massive vitreous hemorrhage developed several days after the injection, and he received pars plana vitrectomy and PRP (The Alcon CONSTELLATION® Vision System) due to persistent vitreous hemorrhage 2 months later. During operation, the neovascular membrane along the vascular arcade with a taut posterior hyaloid and the absence of PVD was observed. The implant was preserved during vitrectomy. The first postvitrectomy optical coherence tomography (OCT) images showed a marked reduction of the central retinal thickness (259 μm 1 month after vitrectomy, 3 months after DEX). There was no recurrence of vitreous hemorrhage or macular edema through the end of the follow-up (9 months after DEX) []. No adjunctive DEX or anti-VEGF was used through the end of the follow-up.


A 29-year-old female with type 2 diabetes mellitus presented with severe non-PDR in the right eye and PDR in the left eye, as well as severe DME in both eyes. Because of recurrent DME after IVIR (three instances), she received an intravitreal injection of Ozurdex in the left eye then in the right eye. The interval between DEX and the last treatment with ranibizumab was 2 months. Both eyes responded well, and the central retinal thickness of the left eye decreased from 1014 to 291 μm; however, vitreous hemorrhage developed in the left eye 1.5 months after DEX []. Pars plana vitrectomy (The Alcon CONSTELLATION® Vision System), PVD creating, internal limiting and neovascular membrane peeling, and PRP were performed 2 months later due to increased vitreous hemorrhage in the left eye. A neovascular membrane along the vascular arcade with taut posterior hyaloids and the absence of PVD were noted intraoperatively. The implant was preserved. The postoperative OCT showed no recurrence of macular edema through 3 months after operation, which was 6 months after DEX injection.


A 30-year-old male patient had poorly controlled hypertension and type 2 diabetes mellitus for 1 year. He came to our outpatient clinic for the treatment of blurred vision in the right eye, and PDR with DME, as well as hypertensive retinopathy in both eyes, were noted. He was first referred to an internist to achieve systemic control of his diabetes mellitus. Limited PRP was administered in the left eye owing to vitreous hemorrhage, and an intravitreal injection of Ozurdex was administered in the right eye due to the progression of DME (VA 6/20; 717 μm). Vitreous hemorrhage occurred 1 week later, as well as secondary glaucoma (21–23 mmHg), which was controlled with brimonidine tartrate ophthalmic solution (Alphagan P, Allergan). OCT showed a reduction in macular edema (198 μm). Limited PRP was performed in the right eye 2 weeks after vitreous hemorrhage. Pars plana vitrectomy ([The Alcon CONSTELLATION® Vision System]) and fluid-gas exchange with 20% octafluoropropane (C3F8) was performed due to persistent vitreous hemorrhage for 2 months. A neovascular membrane along the vascular arcade with taut posterior hyaloids and the absence of PVD was noted intraoperatively. The implant was preserved. Recurrent macular edema was noted 10 months after first DEX. There was no vitreous hemorrhage noted after repeated injection of DEX in the same eye [].


A 41-year-old man suffered a fracture of the left medial orbital wall, supraorbital rim, orbital roof, and anterior and posterior table of the frontal sinus from an injury inflicted by fallen laminate wood. Open reduction using an absorbable mesh plate was performed. After 8 months, the patient visited our hospital for the correction of post-traumatic enophthalmos. A 3D-CT evaluation revealed left-sided enophthalmos (6 mm lower than the normal side). Surgery was performed through a subciliary incision. Iliac bone was harvested (3.0×2.5×0.6 cm) and carved into an anatomically suitable shape (2.0×1.0×0.6 cm) to fit the defect size. Postoperative complications were not observed. The patient was discharged at 10 days postoperatively without specific complications. The preoperative difference on exophthalmometry was 6 mm, which improved to 3 mm postoperatively. The correction of enophthalmos and the patient’s esthetic appearance were also satisfactory (, ).


A 45-year-old man suffered a right orbital floor and infraorbital rim fracture due to being hit by a baseball. At 1.5 weeks after the injury, the orbital fractures were repaired with Synpor and open reduction using absorbable plates and screws. After 11 months, the patient wanted correction for post-traumatic enophthalmos. A 3D-CT evaluation revealed right-side enophthalmos (5 mm lower than the normal side). Surgery was performed through a subciliary incision. Iliac bone was harvested as needed (3.2×2.3×1.0 cm) and the harvested iliac bone was carved and trimmed to fit the defect size (3.1×2.0×1.0 cm). Postoperative complications were not observed. The patient was discharged 10 days after the operation without specific complications. The preoperative difference on exophthalmometry was 5 mm, which improved to 3 mm postoperatively. The correction of enophthalmos and the patient’s esthetic appearance were satisfactory (, ).


An 84-year-old male patient with heavy smoking history presented in June 2012 at our department with squamous cell carcinoma on the right lateral border of the tongue. Tumor stage was cT2N0M0 according to the 2007 UICC classification system. After rejecting glossectomy as well as definitive external-beam radiotherapy (EBRT), he was offered HDR-ISBT as monotherapy (). Treatment consisted of 54.0 Gy delivered in nine fractional HDR doses of 6.0 Gy over 7 days. The volumetrically calculated GTV and CTV were 5.3 cc and 17 cc, respectively. The generated D90 (GTV) was 6.6 Gy and D100 (GTV) 6.0 Gy. The respective D90 (CTV) was 6.3 Gy and D100 (CTV) 5.0 Gy with D2cc (mandible) at 3.4 Gy.
At 60 months after ISBT the patient remains free from disease without local or systemic progression. However, at 32 months after treatment he presented osteoradionecrosis of the mandible which requires pain management with oxycodone hydrochloride but no intervention or hospitalization. Against medical advice, he did not quit smoking despite experiencing symptomatic osteonecrosis [].


A 38-year-old female patient presented in August 2012 at our department with squamous cell carcinoma on the left lateral border of the tongue. Tumor stage was cT2N0M0 according to the 2007 UICC classification system. After rejecting glossectomy, she was offered HDR-ISBT as monotherapy (). Treatment consisted of 54.0 Gy delivered in nine fractional HDR doses of 6.0 Gy over 7 days. The volumetrically calculated GTV and CTV were 1.3 cc and 6.1 cc, respectively. The generated D90 (GTV) was 6.6 Gy and D100 (GTV) 6.3 Gy. The respective D90 (CTV) was 6.6 Gy and D100 (CTV) 6.1 Gy with D2cc (mandible) at 2.6 Gy.
At 56 months after ISBT the patient remains free from local disease recurrence. However, 3 months after treatment she was diagnosed with cervical lymph node metastasis and received radical neck dissection with postoperative EBRT up to 60 Gy in 30 fractions. At present, she remains free from local or further systemic progression. No mandible complication was observed.


A 12-year-old male child weighing 20 kg suffering from thalassemia major and immune thrombocytopenia presented with features of pancytopenia. His bone marrow examination showed red cell aplasia. The child was given steroid therapy in the form of oral prednisolone 1 mg/kg/day for 4 weeks.
Child's haemoglobin was 9 g/dl and his platelet count was 30,000/mm3 despite steroid therapy. Ultrasound of the abdomen showed hepatosplenomegaly. The child had pancytopenia, hypersplenism and was posted for splenectomy. Splenectomy was done under general anaesthesia with endotracheal intubation (ETT). Stress dose of steroid hydrocortisone 25 mg was given perioperatively. Before extubation, ultrasound-guided bilateral single shot erector spinae block with 15 ml of 0.2% ropivacaine was given at T-8 level. The trachea was successfully extubated and the child was shifted to the post anaesthesia care unit (PACU).
The child was pain-free in the immediate postoperative period with a visual analog score (VAS) score of 1/10. The patient was discharged from PACU. On the second post-operative night, the child complained of severe pain at the surgical site with a VAS score of 8/10. Intravenous paracetamol 15 mg/kg was given for pain control but the pain was not relieved. The nonsteroidal anti-inflammatory drug was not given due to the risk of platelet dysfunction. The child had a heart rate of 120 beats/min and non-invasive blood pressure of 170/94 mmHg. The patient had continued pain and at midnight and he complained of a bilateral diffuse occipital headache followed by the blurring of vision and visual hallucinations. The symptoms worsened rapidly. Two hours later, the child had a generalised tonic-clonic seizure lasting for 2 min. Seizure episode was managed by intravenous (IV) midazolam 4 mg. A loading dose of IV phenytoin 20 mg/kg was given by slow infusion followed by 100 mg intravenous per day. The seizure was followed by postictal confusion. During this episode, blood pressure increased up to 180/96 mmHg, and the child was shifted to the intensive care unit (ICU).
On admission to the ICU, his Glasgow Coma Scale was 9/15 (motor 4, eyes 2, verbal 3). His blood pressure (bp) was 180/100 mmHg, heart rate 106 beats/min, body temperature 36.7°C and pupils were 2.5 mm in diameter, equal and reactive. The fundus examination was unremarkable. Blood glucose, arterial blood gases, serum osmolality and serum electrolytes were normal. The child maintained oxygen saturation throughout the whole event.
The patient had a recurrence of seizure after ICU admission, following which levetiracetam 20 mg/kg intravenous infusion was given. MRI brain showed T2-weighted-fluid-attenuated inversion recovery (T2 FLAIR) hyperintensities in the bilateral parieto-occipital region and bilateral cerebellar hemispheres with underlying cortical swelling [Figures and ]. Magnetic resonance venography (MRV) was also done to rule out venous sinus thrombosis. All clinical and radiological features were consistent with PRES.
Blood-pressure monitoring showed wide fluctuations in reading hence left radial intra-arterial blood pressure monitoring was started in ICU. The systolic blood pressure of child ranged between 120–190 mm of Hg, whereas diastolic blood pressure ranged between 70–100 mm of Hg. The hypertension was managed with intravenous labetalol 0.4–1 mg/kg/hr intravenous infusion. Within 24 h, the blood pressure fluctuations resolved and oral prazosin tablet 0.5 mg once daily was started for maintenance. Oral phenytoin 150 mg and levetiracetam 400 mg were continued once daily. For analgesia paracetamol, 15 mg/kg was given intravenously every 6 h and intravenous fentanyl 1 μ/kg bolus as per requirement. The dose of steroid was tapered to prednisolone 6 mg once daily. Headache and visual symptoms resolved gradually over 2 days. He did not have any recurrence of seizure and phenytoin was stopped while oral levetiracetam was continued. The patient was discharged on day 15.


Following a motor vehicle accident, a 51-year-old female who presented neurologically intact with cervical magnetic resonance/computed tomography (CT) documented bilateral avulsion fractures of the occipital condyles (type III) and fracture of the inferior clivus [-]. The cervical magnetic resonance imaging (MRI) further showed a diffuse T2 signal involving the ligaments of the CCJ and AOJ with a focal tear of the tectorial membrane []. Due to instability of the CCJ, the patient underwent an O-C2 posterior cervical instrumented arthrodesis using a modular (multiple piece) OC plate-rod construct [ and ]. Postoperatively, the patient remained intact and the cervical X-rays 3 months later confirmed adequate fusion [].


A 27-year-old man from Chhattisgarh presented with painless swelling over the right side of the forehead since 6 months and multiple swellings on the right leg since 2 months. He had undergone excision of a nasal mass 5 years back in a private hospital and histopathology from the excised lesion was not done. He was a farmer and a regular user of the village pond for bathing which was also shared by cattle.
Physical examination revealed two well-defined hard, nontender subcutaneous swellings over the right side of forehead just above the eyebrow varying from 5 × 5 to 2 × 2 cm in size []. There was central crackling sensation on one of the swellings. There were three lesions on the right leg. The two were well-defined subcutaneous nodules − 3 × 4 and 2 × 3 cm on the medial aspect and one was diffuse cystic swelling on the lateral aspect []. History of shared pond bathing and surgery for nasal mass excision in the past raised the suspicion of disseminated rhinosporidiosis. Fine needle aspiration of all the swelling was suggestive of rhinosporidiosis []. Roentgenography of the skull and lower limbs appeared normal. Computed tomography (CT) scan of the head showed erosion of the frontal bone at two corresponding sites [–].
The final diagnosis of disseminated cutaneous rhinosporidiosis with frontal bone involvement was made. Tablet dapsone 100 mg daily was started, but there was no change after 2 months of therapy. Infact, the size of his forehead lesion has gradually increased to cover the upper half of the orbit and eye opening was restricted. Surgical management was planned. The two subcutaneous nodules of leg were subjected to excisional biopsy along with electrocoagulation of the margins. Aspiration of the diffuse swelling on the lateral aspect of right leg yielded serosanguinous fluid and was left after decompression for further observation.
On exploration of the forehead lesion, there was fragile granulation tissue with serosanguinous fluid over the eroded area of the frontal bone. Fluid was drained and the granulation tissue was curetted out. The content was sent for histopathological examination which confirmed rhinosporidiosis. Both outer and inner table of the frontal bone was deficient in the bigger lesion whereas inner table was intact in the smaller one []. Dura was not breached in either cases. One centimeter margin of the bone was excised all around and subjected for histopathological examination. Histology confirmed the involvement of bone with the presence of sporangia in the marrow space []. Postoperative recovery was uneventful and he was discharged on tablet dapsone 100 mg once daily. After 1 year of follow-up, there are no signs of recurrence [].


A 44-year-old female, gravida 8 para 5 plus 2 at 37 weeks' gestation admitted electively for cesarean section and tubal ligation. She is a known case of DCM with an ejection fraction of 30% for a few years. Her symptoms were well-controlled on treatment with oral frusemide, Carvedilol, Hydralazine, and Iso dinitrate. The patient is New York Heart Association (NYHA) class 3, following up with a cardiologist who increased frusemide from 40 to 60 per day, 2 months before the procedure, and she has a regular follow-up appointment. In addition, she is on a diet plan for a well-controlled gestational diabetes mellitus.
In the preoperative evaluation, the patient was doing well, with no active complaint and good fetal movement with no history of vaginal leakage. She has a history of shortness of breath on mild to moderate physical exercise, unable to climb a flight of stairs without becoming breathless. On examination, she was 165 cm tall and weighed 90.3 kg with body mass index of 33.2, her heart rate (HR) was 90/min and blood pressure (BP) was 127/71 mmHg, and her SpO2 was 99% while breathing room air. There were no features suggestive of congestive heart failure. Routine laboratory investigations were within normal limits with a hemoglobin level of 8.6 g/dL. 12-lead electrocardiography (ECG) showed sinus rhythm with occasional premature ventricular complexes, otherwise normal ECG. Chest X-ray was unremarkable.
Echocardiogram (Echo) showed severely dilated left ventricle with moderately to severely reduced systolic function due to global hypokinesis and indeterminate filling pressures, and no thrombi were present. Other findings included normal right ventricle size, systolic function, and filling pressures with moderately dilated left atria. Moreover, the mitral valve had a restricted bileaflet closure resulting in moderate regurgitation. No other significant valvular malfunction was noted. There was no indeterminate pulmonary artery pressure. There was no pericardial effusion.
The last formal ultrasound (US) on 36 weeks showed an estimated fetal weight of 2.8 kg, cephalic with normal amniotic fluid, and normal Doppler. The epidural anesthesia technique was explained to the patient, and the consent was signed.
The plan was to admit her for cesarean section after cardiology clearance. So, the cardiologist suggested to keep her on frusemide 60 mg BID and input/output charting and daily weight after the procedure (due to high risk of developing postoperative heart failure decompensation). After admitting the patient, the anesthesia plan is to proceed with epidural anesthesia with a backup plan of general anesthesia.
Routine noninvasive monitoring was established, including noninvasive blood pressure, hr, pulse oximetry (SpO2), and ECG. Her SpO2 was 99% on face mask 5 L/min, and ECG results showed normal sinus rhythm. An awake arterial line was inserted first followed by epidural in the sitting position, followed by central venous catheter insertion under US guidance and confirmed with X-ray. Two large intravenous accesses were inserted, and the chest pads were attached preoperatively. The crash cart was brought in the operative room, and inotropes and vasopressors were on pumps ready to go.
The patient was very anxious; midazolam 1 mg was administered before epidural, another 1 mg around 20 min later and a third dose 30 min later; the nephrologist was informed after placing in the sitting position and then 4 mL of 2% lidocaine was used for local infiltration anesthesia at L3–4 space. The epidural space was located with an 18-G Tuohy needle at the first attempt. After confirming the space by loss of resistance, bupivacaine 0.25% 20 mL + 40 μg fentanyl was given into the epidural space. The depth of the epidural space was 9.5 cm, while the depth of the catheter was 5.5 cm. The epidural was uneventful, and we changed the patient position into a supine position.
Intravenous fluids were maintained at 60–80 mL/h. Dexmedetomidine was administered at a bolus of 0.5 μg/kg over 10 min followed by a second bolus intravenously; norepinephrine infusion was initiated with a total dose of 0.30 mg throughout the procedure; her BP decreased to 125/63 mmHg and HR to 87/min. There were no complaints from the patient, and a baby girl was delivered. The surgeon was asked to massage the uterus for a long time to avoid the administration of uterotonic medications, with estimated blood loss of around 700 mL. The surgery lasted for 90 min. The Apgar score for the newborn infant was 9 points. The cesarean section went smoothly with no complications. The patient was admitted to the intensive care unit (ICU) for observation as she is in a high risk for having decompensated heart failure post operation. She was stable in the ICU, and they transferred her back to the ward with a stable condition after 24 h. No perioperative or anesthetic complications occurred. The patient was discharged home 2 weeks later.


An 8-year-old female neutered Whippet was presented to the Royal (Dick) School of Veterinary Studies (University of Edinburgh) for investigation of a 4-week history of chronic intermittent macroscopic hematuria. The dog had no other lower urinary tract signs and the urine appeared discolored during all the micturition with occasional visible blood clots. The dog received regular flea treatment; vaccination and deworming were up to date. There was no previous history of a bleeding predisposition or trauma. No history of traveling overseas nor history of tick exposure was reported. The dog had been treated by the referring veterinarian surgeon (RVS) with a short course of non-steroidal anti-inflammatories and potentiated amoxycillin with no improvement of the clinical signs. Extensive investigations prior to referral were performed by the RVS and included the following: urinalysis revealed urine specific gravity (USG) of 1.055, pH 6, blood 3+, and protein 3+; urine sediment revealed a large amount of red blood cells. The bacteriologic culture of the urine resulted negative. Hematology and serum biochemistry revealed a mild increase in urea (8.2 mmol/l, reference 1.7–7.40 mmol/l), mildly increased hemoglobin at 19.7 g/dl (12.00–18.00 g/dl), and hematocrit at 58.0% (37%–55%) suspected to be breed-related. Platelet count was normal. Plain abdominal radiographs of the abdomen, pneumocystogram, and double-contrast cystography had revealed no abnormalities other than an intrapelvic location of the urinary bladder silhouette of equivocal clinical significance. On presentation to our referral center, physical examination was unremarkable, but signs of discomfort were elicited upon palpation of the dorsocaudal abdomen. Differential diagnoses considered for hematuria included a clotting disorder, urinary tract infection or pyelonephritis, neoplasia, trauma, urolithiasis, or IRH. Repeat complete blood count and serum biochemistry were within normal limits. Prothrombin time, activated partial thromboplastin time, D-dimers, and fibrinogen were within the reference intervals. Buccal mucosal bleeding time was less than 4 minutes. Systolic blood pressure was mildly increased (165 mmHg) but not considered high enough to be the cause of bleeding. Repeated urinalysis from both a free catch and a cystocentesis urine sample showed similar findings: macroscopic hematuria, USG of 1.040, pH 7, protein 3+, and blood 3+. Sediment showed large amounts of erythrocytes with no signs of crystals, casts, or inflammatory cells. Hemoglobinuria or myoglobinuria was excluded by centrifugation. Repeat urinary culture resulted in no bacterial growth. Thoracic radiographs, performed to rule out neoplastic/metastatic changes, were unremarkable. B-mode full abdominal ultrasound and CEUS study of the urinary system were performed using a multifrequency microconvex transducer (CA 123 Esaote, Genova, Italy). On CEUS, the mechanical index was set at minimum (0.08) and only a single focal zone was positioned, directly below each kidney and urinary bladder. A second-generation contrast agent (SonoVue© Bracco, Milano, Italy) was injected as a bolus into the cephalic vein through an angiocatheter, to evaluate the entire urinary tract. Two different boluses of 0.06 ml/kg were administered at 10 minutes intervals, followed by injection of a bolus of 3 ml of 0.9% sodium chloride flush. Images were stored digitally and subsequently analyzed. Moderate amount of suspended echogenic material was visible in the urinary bladder lumen in B-mode (). During pre-contrast B-mode imaging, the jets from the UVJs were not clearly seen. On CEUS, an intermittent jet of echogenic material from the right-UVJ was noted in the fundamental mode. This finding was more conspicuous with harmonic imaging where intermittent excretion of markedly enhancing mobile material was visible from the right-UVJ (). No evidence of hyperechoic fountain-like material emerging from the left ureteric orifice was detected. Normal enhancement of the urinary bladder wall was detected with hyperenhancing submucosal layer, serosal layers, and hypoenhancing muscular layer. On B-mode and CEUS ureters, urethra and urinary bladder wall were unremarkable. Kidneys showed a normal architecture, size, and echogenicity, apart for a single left renal cortical cyst (2 mm) that was considered an incidental finding. Contrast study of both kidneys was also unremarkable with a progressive cortical, corticomedullary, and washout phase. The ultrasonographic findings were indicative of urinary bladder sedimentation most likely consistent with hematuria, likely secondary to active upper urinary tract bleeding from the right nephro-ureteral tract. The most likely differential diagnosis considered was idiopathic renal/ureteral hematuria. Infectious diseases affecting the urogenital tract were excluded due to the negative microbiology exam of the urine. The presence of a neoplastic disease or occult urolithiasis was considered unlikely due to the absence of crystalluria, normal abdominal radiography and normal B-mode, and CEUS studies of the urinary system.
A diagnosis of IRH was made, most likely originating from the right kidney. Cystoscopy with direct visualization of the UVJ, and eventual angio-CT (to rule out subtle vascular lesions of the renal parenchyma or pelvis including arteriovenous malformation, telangiectasias, and aneurysms) were recommended for further assessment but were declined by the owner. Periodical monitoring for signs of anemia and/or signs consistent with lower urinary tract disease (stranguria, dysuria, and urinary obstruction) was recommended. One month later, the dog still had mild intermittent hematuria with no signs of anemia or iron deficiency on complete blood count. At 1-year follow-up, the patient is alive with no current episodes of macroscopic hematuria reported.


A 46-year-old female presented with a chief complaint of swelling in the left lower jaw toward the posterior aspect for 3 months, and the swelling was gradually increased in size to the present size with intermittent pain. On extraoral examination, a diffuse swelling was present on the left side of the face extending from the angle of mandible posteriorly to ramus superiorly and posterior submandibular region inferiorly measuring about 5 cm × 4 cm in size. Overlying skin was normal with no evidence of discharge []. On palpation, swelling was firm, mild tender, nonpulsatile, and noncompressible. Intraoral examination revealed a diffuse swelling in the left retromolar region and buccal vestibule in 33–38 region []. There was absence of any significant cervical lymph node on palpation. The patient had a history of swelling in the same location 17 years back for which she underwent surgical excision and diagnosed as plexiform ameloblastoma on histopathological examination [Figure –].
Radiograph of the mandible revealed expansion of buccal and lingual cortical plates with multilocular, soap bubble appearance []. Noncontrast computed tomography (CT) and three-dimensional CT scan revealed an expansile lytic multicystic lesion of size 5.5 cm × 3.0 cm × 2.7 cm with well-demarcated margin in the body of the left hemimandible with air foci within it and the lesion not crossing the midline [Figure and ]. The mass was abutting the left margin of the tongue, left mylohyoid, and buccinator muscle. With the above radiological features, the patient was suspected as a case of recurrent ameloblastoma.
Fine-needle aspiration cytology from the lesion showed blood-tinged, straw-colored fluid with inconclusive diagnosis. Hemimandibulectomy was performed with sufficient, safe margins under the general anesthesia. Multicystic appearance of the lesion was found on intraoperative finding [].
Histopathological examination of the operated specimen revealed delicate collagenous stroma with numerous odontogenic follicles comprised peripheral ameloblast like-cells and central stellate reticulum-like cells. Multiple follicles were showing squamous metaplasia of stellate reticulum-like cells, some of which were transferred to keratin pearls. Many follicles also showed evidence of cystic degeneration. Diffusely arranged mixed chronic inflammatory cells were present in vascular stroma. With these features, the final diagnosis of acanthomatous ameloblastoma was confirmed [Figure –]. The patient was doing well without any recurrence during 6 months of follow-up.


A 67-year-old male with known case of hypertension for 10 years on Losartan and recent onset type 2 diabetes mellitus on Metformin with reasonable blood pressure and sugar control presented with history of loss of appetite, slowness of movements, decreased memory, frequent falls, and swaying to either side while walking for the last 3 months. He also gave a history of loss of appetite associated with nausea, occasional vomiting, and easy fatiguability. For his symptoms, he was taking over the counter indigenous medication for the last 6 months. He was evaluated by a neurologist, who did not notice any significant findings on examination. His cardiovascular, respiratory, and abdominal examination was noncontributory. Neurological examination revealed the presence of ataxia, dysmetria, dysdiadokokinesia, tremors, bradycardia, and mild rigidity. A neurological diagnosis of early Parkinsonson's disease was made and a magnetic resonance imaging (MRI) of the brain was done, which was suggestive of a encepaholapathy. There was no evidence of ischemic changes secondary to age, hypertension, and diabetes mellitus in the MRI []. His paraneoplastic panel and autoimmune markers were not contributory. (autoimmune encephalitis mosaic antibodies and paraneoplastic neuronal antibodies were all negative.)
His initial laboratory investigations showed normal hematological parameters, normal liver function tests, normocalcemia, normal electrolytes, and normal ammonia levels. His serum creatinine was high (2.2 mg/dl). The serum creatinine done 6 months back before starting the over the counter medications was normal at 1.4 mg/dl (normal range for the lab 0.9–1.5 mg/dl). Because of his worsening renal function he was evaluated by a nephrologist also. His urine examination was normal and his protein creatinine ratio was within normal range (0.12). His ultrasound of the abdomen revealed normal sized kidneys. He underwent a renal biopsy for unexplained renal dysfunction, which showed normal glomeruli with dense interstitial infiltrate consisting of mononuclear cells and eosinophils []. The electron microscopic examination revealed toxic vacuolation of the proximal tubules []. A diagnosis of interstitial nephritis with toxic proximal tubular injury was made and as the MRI was suggestive of a toxic/metabolic encepahalopathy, investigations were done to find a putative cause. As the basic metabolic evaluation did not reveal any plausible cause, a toxicology screen was asked for. The aluminum levels were above normal in the toxicology screen []. Treatment with desferroxime chelation was advised and the indigenous medicine was stopped. One month later the aluminum levels had reduced to 29.4 μg/l and the chelation was continued for another 4 weeks. A course of 6 weeks of low dose steroids (0.5 mg/kg/day) was also given for the interstitial inflammation. Currently, the patient has a normal serum creatinine (1.3 mg/dl) with complete resolution of the neurological symptoms. The cause of cerebral symptoms were believed to be because of aluminum toxicity as the symptoms resolved completely with aluminum chelation and it is speculated that the toxic proximal tubular injury can also be attributed to aluminum.
This patient developed neurological manifestations after 3 months of starting over the counter medications. His creatinine was only checked after 6 months of continuation of the medications. The complete resolution of his neurological and kidney injury simultaneously with the reduced aluminum levels (temporal association) suggests a putative role of aluminum in this patient's illness.


A 51-year-old woman was transferred to our hospital from a local clinic. Bone marrow biopsy had confirmed the presence of B-cell precursor lymphoblastic leukemia in the local hospital. At our hospital, bone marrow cytology showed that lymphoblasts and prolymphocytes accounted for 87% of bone marrow cells (). Flow cytometry revealed that blasts were positive for CD34, TdT, CD19, CD10. Few cells showed weakly positive and scattered expression of cytoplasmic CD79a, and MPO, Lyso, CD3 and CD138, but no expression of cytoplasmic CD56. Initial complete blood counts were as follows: a hemoglobin level of 56 g/L, a platelet count of 30*109/L and a white blood cell count of 1.5*109/L, 10.3% segmented neutrophils, 85.6% lymphocytes, 2.7% monocytes. PET/CT revealed that multiple lymphadenopathy, hepatosplenomegaly, and extensive bone marrow infiltration with high FDG. The 24 hrs unstimulated culture of bone marrow aspirates showed that all the metaphase cells, though not massive, were normal karyotypes (). Fluorescence in situ hybridization (FISH) signals using Ph-like B-ALL probes, including IGH/CRLF2, PDGFRB, JAK2, BCR/ABL1, ABL1 and EPOR revealed that of the 100 interphase cells analyzed, 36 (36%) showed ABL1 gene deletion but no rearrangement, and the rest showed no abnormality. The FISH analysis using BCR/ABL1 and ABL1 probes confirmed the presence of nuc ish (BCR×2, ABL1×1)[35/100], (ABL1×1)[36/100] (). The signals indicating BCR-ABL fusion did not appear.
Before beginning therapy, the second round of FISH analysis using BCR/ABL1 probe identified ABL1 deletion in up to 80% of the cells. Then, we proceeded copy number variations (CNV) analysis based on next-generation sequencing (NGS), belong to CNV-Seq, which covered the targeted region of 118 sequenced genes with a total length of 215kb. CNV analysis found the reduction in the copies of ABL1 gene on the long arm of chromosome 9, EVT6 gene on the short arm of chromosome 12 and DLEU2 gene on the long arm of chromosome 13; the increase in the copies of RET, PTEN, NT5C2 and SMC3 on the long arm of chromosome 10 (). The further analysis of CNV according to the sequencing depth of NGS indicated about 90% deletion of ABL1 gene and combined with FISH considerations, ABL1 deletion is loss of heterozygosity. It is worth mentioning that the deletions of exons are 2–10 (corresponding transcript: NM_005157), that is, all exons of ABL1 involved in this panel.
Subsequently, the patient received one cycle of IVCLP chemotherapy (Idarubicin, Vindesine, Cyclophosphamide, Pegaspargase and Dexamethasone), achieving obvious clinical remission and negative results in bone marrow biopsy (). After six regimens of chemotherapy, the patient has been in continuous remission till now.


A 71-year-old man presented to the emergency department after falling off his bike. He was diagnosed with a head injury, nasal fracture and lacerations on his right forehead and right lower eyelid. The head injury and nasal fracture were managed conservatively. Due to his right pupil being unreactive and rapidly declining vision, an emergency canthotomy and cantholysis was performed. His lacerations were sutured, and he had weekly follow-ups for 3 weeks with ophthalmology department. About 2 months later, the patient presented with scar contracture and outward rotation of his right lower eyelid. This was diagnosed as cicatricial ectropion ( and ). This was treated with scar revision, canthopexy and upper to lower lid switch flap ( and ). The patient was evidently anxious as his left eye was already blind from a previous ocular injury.


An 86-year-old woman, with a 20-year history of type 2 diabetes mellitus (DMT2) came to the ER due to motor agitation with involuntary movements of the left upper extremity with 3 days of evolution. She was apyretic, hypotensive and dehydrated. Involuntary, irregular and non-suppressible movements of the left superior member with involvement of the ipsilateral face were noted. The patient was found to be hyperglycaemic with a serum glucose of 1,045 mg/dl and also had hyperosmolar hyponatraemia, anaemia, leucocytosis with neutrophilia, acute renal injury and elevated troponin I, with non-supraST waves on the ECG. In the urine summary, the findings were of leucocyturia without ketonuria. Hyperglycaemic hyperosmolar syndrome and hyperglycaemic hemichorea were diagnosed. A CT scan was carried out () with the identification of a hyperdensity at the level of the right striatum, which corroborated the neurological findings. Infusion with insulin therapy was started, and fluid therapy, as well as other supportive care directed towards the anaemia, acute cystitis and for the acute myocardial infarction type 2, was initiated. Antipsychotic medication was also needed. At the end of 3 days of hospitalization, the patient no longer presented involuntary movements and was discharged 6 days later. The glycated haemoglobin (HbA1c) result, however, was 19.9%.


A 54-year-old diabetic male patient referred to surgical outpatient department with complaints of urinary urgency and increased frequency. Serum prostate-specific antigen (PSA) level was mildly elevated, while other haematological and biochemical parameters were within normal limits. Digital rectal examination and pelvic ultrasound revealed gross prostatic enlargement, measuring approximately 195.6 gm by weight with median lobe projecting into the base of the bladder. Based on clinico-radiological findings, he was first diagnosed as benign prostatic hyperplasia with cystitis and was treated with tablet Tamsulosin and antibiotics for almost one month. However, on follow-up, no improvement of symptoms was observed. Further, the patient complained of exacerbation of symptoms and supplementary radiological investigations stated progressive increase in prostate size. Therefore, in suspicion of malignancy, open prostatectomy was undertaken and a large prostatic mass was excised, which was sent for histopathological analysis.
On gross examination, the specimen was composed of three nodular masses, each measuring 5x4x3 cm, 4x4x3 cm, and 4x3x2 cm respectively. External surface was unremarkable. Cut section showed solid grey-white whorled areas with no necrosis or haemorrhage (). Microscopic examination reveals a benign neoplasm composed of unencapsulated and circumscribed nodules; comprising of haphazardly arranged ovoid and spindle cells with indistinct cell borders and relatively uniform nuclei, embedded in a fibrocollagenous stroma with peripherally compressed benign prostatic glands (). Multiple hemangiopericytoma-like/staghorn blood vessels () were interspersed within the lesion, while neither necrosis nor increased mitotic activity was noted. Immunostaining showed strong positivity for vimentin, CD34, CD99 and BCL-2 () and immuno-negativity for pan-cytokeratin, epithelial membrane antigen (EMA), actin, desmin, myogenin, progesterone receptor (PR) and CD117 among the neoplastic cells. In accordance with the histomorphological and immunohistochemical (IHC) findings, diagnosis of a solitary fibrous tumor of the prostate was rendered. Currently, the patient is on regular follow-up and asymptomatic.


A 23-year-old woman with no medical antecedents was admitted to our service because of a sudden headache, vomiting, and disturbance of consciousness while she was working.
Initial physical examination revealed disturbance of consciousness (Glasgow Coma Scale E3V4M5), without any deficits of brain stem function. A cranial CT revealed hemorrhage in all cerebral ventricles, acute hydrocephalus, and a hyperdense mass in the cisterna magna (fig. ). Emergent angiography revealed tumor staining and several feeding vessels originating from the left posterior inferior cerebellar artery. According to these radiological findings, HB presenting as intraventricular hemorrhage was presumed as a preoperative diagnosis. Because her consciousness rapidly became worse (Glasgow Coma Scale E1V2M3), she underwent emergency surgical intervention immediately after an angiography.
An urgent surgical removal of the tumor and external ventricular drainage were performed. The patient was placed in the prone position. After suboccipital midline incision, suboccipital craniotomy was performed. The reddish tumor measuring 3 × 2.5 × 2.5 cm was located in the dorsal medulla oblongata. The tumor feeders were carefully selected and sectioned, avoiding injury of pial passing arteries, and finally the tumor was removed in en bloc fashion. During the operation, a large amount of secretions were suctioned through the endotracheal tube and refractory hypotension was recorded.
The tumor was composed of a large number of microvascular dilated vessels and a rich population of stromal cells. The nuclei were hyperchromatic, but no prominent pleomorphism, nucleoli, or other atypical features were evident. Histologically, the tumor was diagnosed as HB.
After surgery, the patient was treated in the intensive care unit because of cardiovascular/pulmonary system dysfunction. She woke up from anesthesia 12 h after surgery with a complete recovery of consciousness and no motor deficit.
The patient showed severe cardiovascular/pulmonary system dysfunction immediately after surgery, and high-dose norepinephrine and dobutamine were administrated to maintain a systolic blood pressure. After surgery, rapid testing for cardiac troponin T was positive and the serum C-reactive protein value was 1.36 mg/dl. An electrocardiogram showed ST-segment elevation in the high lateral leads and ST-segment depression in V3–V6. An echocardiogram showed global hypokinesia of the left ventricular wall with severe mitral insufficiency and an estimated ejection fraction of 24%. The condition was thus taken to be suggestive of TTC. Three days after the operation, the brain natriuretic peptide (BNP) level was 2,098 pg/ml (normal range: 0.5–30 pg/ml) and increased up to 3,333 pg/ml on postoperative day (POD) 9.
The patient required hANP and furosemide for the treatment of heart failure. X-ray after operation showed a typical finding of NPE. After continuing the positive pressure ventilation, she was extubated on POD 6. She required noninvasive positive pressure ventilation for 4 days after extubation. Left ventricular function and BNP level had gradually normalized, and NPE was cured on POD 40.
Postoperative neurological examination showed dysphagia and ataxia from disturbance of deep sensibility derived from brain stem damage. After rehabilitation for dysphagia, ataxia, orthostatic hypotension and aglutition, she returned to normal status 12 weeks after surgery. Postoperative MRI showed no residual tumor (fig. ).
She had no family history of VHL disease, and a gadolinium MRI study of the whole neuraxis ruled out any other HBs. Abdominal CT showed no mass in the kidney, and ophthalmological examination denied retinal hemangiomas.


A six-year-old male, who was fully immunized and his vaccinations were current, was treated for stage II TBM for one month, with good response, developed profound diminution of vision. He was on oral 4-drug ATT, that comprised of isoniazid (5 mg/kg/day), rifampicin (10 mg/kg/day), pyrazinamide (25 mg/kg/day) and ethambutol (15 mg/kg/day), and systemic steroids (1 mg/kg/day) on a tapering dose. The visual acuity was no light perception in both eyes. The anterior segment examination was unremarkable except for semi-dilated pupils, which were not reactive to light. The fundus examination showed slight disc hyperemia with well-defined margins bilaterally. Brain magnetic resonance imaging (MRI) showed extensive enhancing basal exudates along with dilatation of lateral and third ventricles and obstruction at the aqueductal level []. A diagnosis of paradoxical tuberculous OCA was made on the basis of MRI findings, cerebrospinal fluid (CSF) analysis (cells 910/cu mm, protein 230 mg/dL, glucose 30 mg/dL, Pandy test positive), and clinical findings. Under cover of ATT, intravenous methylprednisolone (IVMP), 30 mg/kg body weight/day, was administered. After three doses the visual acuity increased to light perception in both eyes. IVMP was continued for two more days and then the medication was changed to oral prednisolone tablets (2 mg/kg body weight/day in single dose) on a tapering schedule for a month. The vision slowly improved over a period of 12 weeks. Seven months after the visual loss, brain MRI showed decreased inflammation of the meningeal sheaths around the optic nerves and mild hydrocephalus []. ATT was stopped after 12 months. At the 18 months follow-up visit, the best corrected vision was 6/18 in right eye and 6/12 in the left. The pupils in both eyes were approximately 4 mm in diameter round and briskly reactive to torch light. Fundus examination showed optic disc pallor bilaterally [].


A four-year-old male child, fully immunized and his vaccinations were current, had stage I TBM with hydrocephalus, was on oral ATT for three weeks. He underwent surgery for ventriculoperitoneal shunt (VP shunt) under general anesthesia after his systemic condition stabilized. Preoperative ophthalmoscopic examination showed a normal fundus. Six days postoperatively, the child was referred back to us by the pediatric surgeon with complaints of bilateral loss of vision. The child was on 4-drug ATT (isoniazid 5 mg/kg/day, rifampicin 10 mg/kg/day, pyrazinamide 25 mg/kg/day and ethambutol 15 mg/kg/day) but not on systemic steroids. The visual acuity at this visit was no fixation or following of light. There was no light perception in both eyes with absence of pupillary reaction to light. Fundus examination by indirect ophthalmoscope and 78 D slit-lamp ophthalmoscopy showed normal fundi bilaterally. Brain MRI showed enhancing lesions at the level of optic chiasm involving both the optic nerves. A diagnosis of paradoxical OCA was made on the basis of the clinical picture, CSF analysis (cells 120/cu mm, 90% lymphocytes, protein 20 mg/dL) and neuroimaging findings. The child was started on 30 mg/kg body weight IVMP for five days and then placed on systemic prednisolone (2 mg/kg body weight/day) on tapering dose along with systemic ATT. Two weeks after starting systemic corticosteroids, the child started to perceive light on vision testing. ATT was stopped after a year and the best corrected visual acuity at that time was 6/18 in both eyes. The pupils were round, semi-dilated with brisk reaction to light. Fundus examination showed prominent temporal pallor of the optic nerve head in both eyes []. Magnetic resonance imaging of the brain showed VP shunt in situ with no evidence of exudate in the optochiasmatic region.


A four-month old male patient, born from non-consanguineous parents, coming from the
countryside of São Paulo, was referred to our clinic due to chronic diarrhea,
protein-energy malnutrition and skin lesions. At admission, he presented exulcerations
covered with honey-colored hematic crusts on the anterior chest, upper limbs, dorsal
area, face and especially on the scalp. The patient also had a cleft palate, deformity
of the ears, decreased amount of eyelashes and eyebrows, besides erythematous papules on
lower limbs, micropenis, dystrophy on all nails and personal history of surgery for
ankyloblepharon (-). Skin biopsy result suggested epidermolysis bullosa.
Given the clinical manifestations we diagnosed Hay-Wells Syndrome. Foam dressings
(Mepilex ®) on the scalp and topical antibiotics in areas with exulcerations and
exudation were instituted.


The case was a 64-year-old lady with moderate cataract, primary angle closure glaucoma, essential hyper-tension, and ischemic heart disease. She was on three topical anti-glaucoma medications besides anti-platelet therapy (Clopidogrel and Aspirin, each 75 mg/day). Her vision was 6/18, N6 in both eyes. She had a functional laser peripheral iridotomy in both eyes. The applanation pressures measured 22 and 19 mm Hg in right and left eyes, respectively. The angles were open with patchy trabecular meshwork pigmentation. The right optic disc had a cup-to-disc ratio of 0.8:1 with inferior rim loss of two clock hours. The left optic disc had a cup-to-disc ratio of 0.7:1 with inferior rim excavation of one clock hour. Humphrey visual field (Humphrey perimeter, Carl Zeiss Meditec, Dublin, CA) revealed bilateral superior arcuate scotoma, worse in the right eye [].
She underwent single site phacotrabeculectomy (with intra-ocular lens implantation) + Mitomycin C under peri-bulbar anesthesia in the right eye. The anti-platelet agents were stopped 5 days prior to and were re-started 3 days post-surgery. The triangular scleral flap was secured with one releasable 10-0 nylon suture (GN Corporation Ltd., Japan) at the apex and two interrupted 10-0 nylon sutures at the base. The eye was unremarkable on the first post-operative day.
On eighth post-operative day, the intraocular pressure (IOP) in the right eye was 26 mm Hg. The bleb was shallow. The anterior chamber was deep. Gonioscopy ruled out internal obstruction of the sclerostomy. Digital ocular compression was ineffective. The releasable suture was removed. Subsequent digital ocular compression resulted in elevation of the bleb and reduction in IOP to 13 mm Hg. She was instructed to perform digital ocular compression. The technique was to look up and apply constant, moderate digital pressure to the inferior sclera through the lower eyelid. She was asked to perform three 5-10-second sets of ocular compressions, 10 seconds apart, three times daily.
On 15th post-operative day, she presented with rapid onset blurred vision, redness, and pain in the operated eye. A huge intra-bleb hematoma [] and hyphema [] were noted. The IOP was 40 mm Hg. Her bleeding time was 3 min 30 s (normal is 2-9 min), and clotting time was 5 min 20 s (normal is 5-15 min). Topical and systemic anti-glaucoma mediations were started. She was instructed to discontinue digital ocular compression.
On 19th post-operative day, the intra-bleb hematoma was unchanged, hyphema was almost resolved, and the IOP was 26 mm Hg. The intra-bleb hematoma was drained through a limited peritomy 6 mm posterior to the limbus. Needle revision of the scleral flap was done with a 27-gauge needle under direct visualization. The conjunctival incision was closed with an 8-0 polyglactin (Ethicon inc., Aurangabad, India) continuous interlocking suture. At the end, 5-Flurouracil (5-FU; 5.0 mg/0.1 mL) was injected in the sub-conjunctival space adjacent to the bleb. Anti-glaucoma medications were discontinued. Post-procedure 5-FU (5.0 mg/0.1 mL) was injected in the inferior fornix every alternate day for a week. Four weeks later, the IOP was 16 mm Hg. The bleb was shallow and diffuse. At the 3-month follow-up visit, the IOP was 18 mm Hg and the bleb was functional [].


A 42-year-old man was admitted to the hospital after experiencing fever and night sweating for 2 weeks. He had visited another hospital 1 month earlier because of pain on the left side of his chest. At this hospital, he underwent chest radiography and computed tomography (CT), which showed a left pleural effusion. Diagnostic thoracentesis was performed, yielding 200 mL of clear, yellow-colored pleural fluid. Analysis of the pleural fluid revealed an exudative pattern; a protein level of 5.3 g/dL (serum protein level, 7.5 g/dL), a lactate dehydrogenase (LDH) level of 273 IU/L (serum LDH level, 342 IU/L), a glucose level of 61 mg/dL, an albumin level of 3.3 g/dL, and a pH of 7.3. The pleural fluid cell count was 2,560 cells/mL, with 10% polymorphonuclear cells, 90% lymphocytes, and an adenosine deaminase (ADA) concentration of 82 IU/L. No malignant cells were observed on cytological analysis. Bacterial, fungal, and mycobacterial cultures of the pleural fluid were also negative. The patient was diagnosed with tuberculous pleurisy and was administered anti-tuberculosis medications. After treatment, the pleural effusion and chest pain disappeared and the patient was discharged from the hospital. However, 2 weeks later, he developed new symptoms of fever, chills, and night sweating, which caused worry because of the patient's past illness. Eight years ago, he was diagnosed with extranodal NK/T-cell lymphoma, nasal type. He had been successfully treated with treatment with a combined of chemotherapy and radiotherapy resulting in a complete remission without recurrence. Upon admission, the patient's vital signs were stable, except for the presence of fever. In addition, laboratory findings were within the normal range. A chest radiograph showed findings consistent with a small amount of pleural effusion (). A chest CT scan revealed an heterogeneously enhanced-mass to the left of the eight thoracic spines (). A subsequent 18F-fluorodeoxyglucose (18F-FDG)-positron emission tomography revealed FDG activity at the same site (). A biopsy using video-assisted thoracic surgery was performed. Subsequent analysis by microscopy showed that the pleural tissue was diffusely infiltrated with atypical lymphoid cells (). It was CD3 +, CD56 +, granzyme B +, and EBV + (). These histological features were corresponded with the previous biopsy from nasal cavity eight years ago (). This led us to diagnose the patient's condition as recurrent pleural recurrence of extranodal NK/T-cell lymphoma. He has been been undergoing sequential chemotherapy followed by autologous hematopoietic stem cell transplantation.


A 23-year-old healthy female was admitted to our hospital with a new-onset tender bulge in her left groin, associated with subjective fevers and chills. Four days prior to presentation, she noted a sudden protrusion in her left groin with associated pain. Given worsening pain and intermittent nausea, she presented to our emergency room. On admission, physical examination revealed a soft, non-distended abdomen that was tender to palpation in the left lower quadrant. A soft, mobile, exquisitely tender, and non-reducible mass was appreciated in her left groin, concerning for an inguinal hernia. She had an unremarkable medical and gynecologic history and no prior surgical history. Laboratory data were notable for a leukocyte count of 6.7, a hematocrit of 36.9 and a lactate of 1.4 in the setting of normal electrolytes. An outside ultrasound showed an avascular complex cystic lesion measuring 2.1 cm in the left groin. A subsequent computed tomography scan of the abdomen and pelvis revealed a tubular cystic structure along the left inguinal canal, coursing along the expected path of the round ligament, without surrounding fat stranding or herniation of bowel loops. Given her symptoms and the unclear etiology of the hernia, she was admitted to the hospital and taken to the operating room for exploration (Fig. ). In the operating room, a 6-cm dark purple cystic structure was visualized within the sac of an indirect inguinal hernia, travelling along the round ligament down toward the mons pubis (Fig. ). The cystic structure was dissected away from the round ligament and the cyst was opened and noted to contain clear liquid. Ultimately, the patient underwent left indirect inguinal hernia repair, with Gore Bio A absorbable mesh, and excision of a left inguinal hydrocele of the round ligament. Final pathology revealed fibromuscular tissue lined by mesothelium consistent with a hernia sac and a hydrocele with concordant endometriosis. The patient's post-operative course was uneventful and she was discharged home on post-operative Day 1.


A 27-year-old man presented with a history of painless swelling of the left testis mass for 7 months. Physical examination revealed nontender firm testicular mass. Scrotal ultrasound revealed a well-defined oval heterogenous hyperechoic hypervascularized mass in the left testis measuring 3.7 × 2.7 cm, associated with mild hydrocele []. The right testis appeared normal. Chest X-ray and CT scan of the abdomen were normal. Laboratory investigations showed: Quantitative beta-HCG <3.0 (normal <5.0) U/L, AFP 3.0 (normal <15.0) micro g/L. The patient underwent surgery for exploration and possible radical orchidectomy. Intraoperative consultation was considered and wedge biopsy was sent for frozen section examination which showed a round cell tumor with neuroendocrine features associated with extensive necrosis. Therefore, radical orchidectomy was performed.
Macroscopically, the tumor showed multiple yellow confluent nodules measuring up to 6.2 cm, associated with foci of necrosis. The epididymis and spermatic cord were unremarkable.
Microscopically, the tumor cells were arranged in nests and solid islands with rosettes formation. Extensive tumor necrosis was present []. Tumor cells were round with mild pleomorphism and had eosinophilic cytoplasmic granules []. The mitosis was rarely seen.
There were no invasion to tunica albuginea and no intratubular germ cell neoplasia. The tumor mass was submitted entirely, and showed no teratomatous elements.
On immunohistochemistry, the tumor was positive for chromogranin, synaptophysin, and neuron-specific enolase (NSE). MIB-1 proliferative index was less than 1%. Ultrastructural examination of the tumor cells by transmission electron microscope (TEM) revealed the multiple intracytoplasmic neurosecretory granules [].


A 47-year-old male patient presented to our hospital with a left-sided renal mass with a biopsy positive for malignancy. On evaluation, the bone scan carried out 1 month prior showed increased tracer uptake in the body of L2 vertebra suggestive of metastasis. The rest of the skeletal system was normal. The patient was recently diagnosed with hypertension, but was not on any medication. All other investigations (including platelet count and coagulation profile) were within normal limits. The patient was posted for a left-sided cytoreductive nephrectomy with retroperitoneal node dissection. The anesthetic plan was general anesthesia with perioperative epidural analgesia.
Before induction of general anesthesia, epidural catheter placement was attempted in the D8-D9 interspace. This led to a dural tap; the catheter was subsequently inserted one interspace above at the D7-D8 level. Following this, general anesthesia was administered.
The surgery was uneventful with blood loss of 1L and the patient was extubated postoperatively. An epidural infusion of 0.125% bupivacaine was given intra- and postoperatively. The patient was observed overnight in the recovery room and was shifted to the ward the next day. Pain relief was adequate and an epidural infusion of 0.05% bupivacaine was started through an elastomeric pump. Sub-cutaneous once-daily low molecular weight heparin (LMWH) -enoxaparin (60 mg in 0.6 ml) - was started from post-operative day 1 as pharmacological prophylaxis for deep vein thrombosis (DVT). The patient was ambulatory with good pain relief and the epidural infusion was continued for 6 days. The epidural catheter was removed on the 7th post-operative day on the morning rounds (18 hours after the last dose of LMWH) and oral analgesics were started.
On the night of the 8th post-operative day, the patient complained of urinary retention, for which he was catheterized. The next morning he could not move both his legs (power of 0/5-1/5 in both lower limbs) and had a complete loss of bladder sensation. At this stage, on direct questioning, he admitted that he had felt some weakness in his left leg the previous evening, which he had neglected, attributing it to fatigue. An urgent magnetic resonance imaging (MRI) of the spine showed cord compression at D7-D8 levels. Injection dexamethasone was started and the patient was taken up for an emergency decompressive laminectomy for evacuation of an epidural hematoma causing compressive myelopathy and paraplegia. D8 laminectomy was performed and the material was sent for histopathology. However, there was no improvement in motor function over the next 24 hours and a repeat MRI performed the next day showed some re-accumulated blood causing cord compression two spaces above the laminectomy segment. A repeat multi-segment laminectomy was carried out and the material was sent for histopathologic examination. The patient was given intravenous methylprednisolone and mechanical prophylaxis for DVT with sequential compression devices. Power continued to be grade 0/5-1/5 with no improvement in paraplegia. Histopathology report of the epidural decompression material revealed metastatic clear cell renal cell carcinoma with morphology similar to the primary renal tumor. A repeat MRI done a week later revealed post-laminectomy changes with resolving cord edema at D6-D9 regions and a new lesion in L2-L3 region compressing rootlets. Palliative high dose radiotherapy was given from L1 to L4 levels. The patient was discharged from the hospital after 3 weeks with no improvement in neurological status.


A 53-year-old right-handed male visited outpatient clinic due to progressive headache for one month. He reported that his headache started after PEN performed at another institution for his back and leg pain one month ago. As the catheter was inserted through the coccyges and came up to the back side, he had immediate anxiety associated with back pain and sweating. He felt palpitation of heart, which had never experienced before. After the procedure, his back pain and leg pain improved but headache started. His headache was aggravated by standing and relieved with lying down, least in the morning and worst at night. He also had intermittent nausea and anxiety. His headache worsened despite analgesics.
On his first outpatient visit three weeks after PEN, his mental status and the rest of his neurological examination were normal. The past medical history was otherwise unremarkable. He did not have a history of chronic alcohol consumption or coagulopathy, nor had he been on a medication that could affect coagulation. Physical examination was also unremarkable except for mild neck stiffness. He could not recall any trauma or injury except for PEN. Due to new-onset progressively worsening headache, brain MRI was recommended but he refused at that time.
One week later when his headache worsened, even worse he agreed to brain MRI. It showed bilateral chronic subdural fluid collections compressing the brain (). The patient was taken to the operating room for bilateral burr hole evacuation of subdural fluid collection an placement of subdural catheter. Chronic subdural hematoma was confirmed in the operating room. He improved postoperatively with decreased headaches. We planned lumbar MRI to evaluate the site of neurolysis but patient refused because he was getting better.
Headache eventually resolved and brain computed tomography performed one month after burr hole trephination showed resolution of SDH ().


The patient was a 33-year-old man who presented with abdominal distension and edema of the lower limbs for approximately 20 days. Blood tests were negative for viral hepatitis and showed normal liver function, prothrombin time, and significantly increased alpha-fetoprotein (AFP) value (17,201 ng/mL). Doppler ultrosonography and enhanced computed tomography (CT) with contrast showed a tumor lesion 6.5 cm in diameter located in segments VI and VII with multiple intrahepatic metastases. The tumor had direct invasion into the inferior vena cava and presented with a tumor thrombus 2.0 cm in length. Slight ascites was also present. Needle biopsy confirmed the histologic diagnosis of HCC (pathologic grade III). The Child-Pugh score was 6 (class A). The Eastern Cooperative Oncology Group performance status (ECOG PS) was 1. The disease stage was determined to be T3bN0M1 according to the American Joint Committee on Cancer (AJCC) staging system and advanced stage (stage C) according to the BCLC staging system.
TACE was first performed to control the primary tumor, using 50 mg of epirubicin (Pharmorubicin, Pfizer, Wuxi, China) mixed with 10 mL of Lipiodol (Lipiodol Ultra-Fluide; Andre' Guerbet Laboratories, Aulnay-Sous-Bois, France) and absorbable gelatin sponge particles (Gelfoam; Hangzhou alc Ltd, China). Subsequently, the patient was treated with 400 mg of sorafenib twice daily. Tumor response was evaluated using the Response Evaluation Criteria in Solid Tumors (RECIST) 1.0 and modified RECIST (m-RECIST) every 2 months. The patient's best response was stable disease (SD) by RECIST and partial response (PR) by m-RECIST, respectively (). Progressive disease (PD) was found in the 14th month after the initial treatment. Then another TACE with the same formula was performed to enhance local control of the intrahepatic lesions, with sorafenib continued. Radiologic SD was once again achieved and lasted for another 18 months, even though the serum AFP level continued to rise during follow-up (). In the 33rd month after the initial treatment, the intrahepatic lesions had significant progression with liver function deteriorating to Child-Pugh class C. The inferior vena cava tumor thrombus remained stable until the last follow-up. Sorafenib was discontinued due to severely impaired liver function at that time, and the best supportive care was given as the only treatment. Finally, the patient died from progressive liver disease 44 months after the initial treatment.
The primary TACE-related adverse event was grade 3 post-embolization syndrome (transient fever, abdominal pain, and elevated transaminases). The sorafenib-related adverse events consisted of grade 3 hand-foot skin reaction, grade 2 alopecia, tinnitus, and diarrhea. Sorafenib had been interrupted for no more than 7 days due to grade 3 hand-foot skin reaction during the first 3 months of treatment. The adverse events of sorafenib declined and were well tolerated after 3 months.


The patient was a 60-year-old man who presented asympto-matically when hepatic lesions were found on regular ultrasound surveillance due to hepatitis B virus infection. Both spiral CT and magnetic resonance imaging (MRI) with contrast were performed to confirm the clinical diagnosis of HCC. Radiologic examination revealed multiple tumors with maximum 5.0 cm in diameter located in segments IV and VIII with direct invasion of the middle hepatic veins, and tumor embolus extending to the inferior vena cava for 4 cm. Other investigations showed compensated liver function and mildly increased AFP (73.83 ng/mL). The Child-Pugh score was 5 (class A) and the ECOG PS was 0. The disease was evaluated as T3bN0M1 according to the AJCC staging system and advanced stage (stage C) HCC in the BCLC staging system.
TACE was carried out as the initial treatment with the same formula as Case 1. Sorafenib (400 mg twice daily) was given orally 5 days later. TACE was repeated twice monthly to further ensure tumor destruction. Sorafenib was interrupted for 3 days during TACE and then continued. The patient was followed up with enhanced spiral CT scan and AFP test every 2 months. Intrahepatic lesions responded satisfactorily to treatment with the inferior vena cava tumor embolus remaining stable (SD according to RECIST criteria and PR by m-RECIST, respectively; ). AFP level had no significant elevated trend during follow-up (13.25-73.85 ng/mL, ). At the last follow-up in April 2013, the patient had no evidence of progressive disease and had survived for 35 months after the initial treatment with a good performance status (ECOG PS 1). Sorafenib was still continued at a dose of 400 mg twice daily.
Grade 2 post-embolization syndromes occurred in the first week post-TACE but were relieved after symptomatic treatment. Sorafenib-related adverse events consisted of grade 1 tinnitus and diarrhea. Grade 2 hypertension occurred after 2 months of sorafenib therapy and was thought to be sorafenib-related. Valsartan and nifedipine were prescribed to control hypertension. Entecavir was also prescribed for hepatitis B. No adverse event–related interruption or discontinuation of sorafenib occurred.


A 63-year-old woman presented sudden involuntary, dance-like movements of her right arm and leg. The patient had a 10-year history of diabetes mellitus and hypertension managed with medication. There was no history of prior use of dopamine agonist, antipsychotics and anticonvulsants. Her blood pressure was 130/80 mmHg and blood sugar was 236 mg/dL. Physical and neurological examinations were unremarkable, with the exception of the involuntary movements of the right limbs. Routine biochemical and hematologic tests such as antinuclear antibodies, serum complement level, rheumatoid factor, serum copper, ceruloplasmin, and thyroid function test were normal apart from hyperlipidemia. Psychiatric evaluation and Mini-Mental State Examination (MMSE) score did not reveal remarkable cognitive impairment (score 24/30) or other psychiatric manifestations. Brain MRI revealed acute infarction of the left subthalamic nucleus and left lateral midbrain (). The patient was placed on aspirin 100 mg daily, atorvastatin 40 mg daily for acute stroke medication. As a diagnosis of vascular chorea had been established, we tried on risperidone 1 mg bid to control the choreic movements for 3 days, but failed to improve and complained of somnolence. Then we tried use clonazepam 1.5 mg/day for 4 days but the involuntary movements continued. No optimal doses had been achieved because the patient complained of worsening symptoms and somnolence. At 8 days after the onset, administration of topiramate was started at 25 mg twice daily for 3 days and was increased in quantity up to 50 mg bid. The involuntary movements improved to mild degree within 1 week of starting topiramate, and the optimum hemichorea control occurred with the dose of 50 mg of topiramate twice daily. She experienced no side effects with topiramate. However, when she discontinued topiramate due to poor compliance after 1 month of treatment, her movements were aggravated. On the contrary, her hemichorea disappeared almost completely on final examination performed 1.5 months after restart of the medication which maintained at 100 mg per day.


A 71-year-old Korean male was presented with a dark brownish to violaceous discoloration with edema on the right eyelid. He had been diagnosed with angiosarcoma on the right forehead 3 years ago and was treated with a complete excision of the lesion with a negative margin and an adjuvant radiotherapy (66 Gy in total) at another university hospital. Several months after the treatment, a dark brownish to violaceous patch with edema arose from his right upper eyelid and gradually spread out to the lower eyelid (). Clinically, an ecchymosis caused by a trauma or other hemangioma was suspected more than a recurrent angiosarcoma, because the lesion was about 3 cm away from the surgical scar from the previous operation on the right forehead, and it presented as an edematous, purpuric patch such as the black eye. A skin biopsy taken from the upper eyelid showed numerous irregular anastomosing vascular channels lined by a single layer of somewhat enlarged atypical endothelial cells dissecting collagen bundles in the dermis (). Immunohistochemically, the tumor cells showed a positive reaction for CD31 and D2-40 (). A pathologic diagnosis of well differentiated angiosarcoma was made, and it was identical to that of primary angiosarcoma of the right forehead 3 years ago.
All routine laboratory tests, including a complete blood count, a urinalysis, the blood glucose level, the liver and the kidney function and the chemistry profile were within normal range. Positron emission tomography-computed tomography findings showed no evidence of distant metastasis. Because the tumor involved almost the entire right eyelid diffusely, a surgical excision was not an option. As the tumor gradually spread, he was referred to the radiooncology department for a radiotherapy. He underwent a radiation therapy with a total dose 66 Gy in 33 fractions over 2 months. After the 2 months' course of treatment, a moderate clinical improvement was shown (). One and a half months later, we performed a skin biopsy to find out the histological improvement. But, the histopathologic findings revealed atypical scattered tumor cells in the dermis suggestive of the remnant of an angiosarcoma (). An immunohistochemical staining was positive for the CD31 and negative for D2-40 (). Thus, we concluded that he responded to the radiotherapy with a partial regression of the tumor. We could not perform the next sessions of radiotherapy because we believed that a periorbital edema and the induration might be worsened and he might not be able to open his eye. After a discussion with the multidisciplinary team, it was decided that he will be treated with a paclitaxel chemotherapy. Now, he is under a treatment with a weekly paclitaxel and we are following-up on the treatment response.


A 39-year-old gentleman was being treated for paranoid schizophrenia since the onset of positive psychotic symptomatology in 1996 and had been on clozapine for 7 years prior to his presentation in our cancer hospital, in the dose of 300 mg (in daily divided doses). He was also on a combination of trifluoperazine 2.5 mg and trihexyphenidyl 1 mg 3 times a day. Though he did have occasional auditory hallucinations, overall he remained psychiatrically stable. He did not report any distressing side-effects of the medications except for excess salivation. He was diagnosed with cancer of the tongue T4N2bM0 moderately differentiated squamous carcinoma in August 2009. Detailed investigations showed that the tumor was borderline inoperable, which led to institution of platinum- and taxane-based neoadjuvant chemotherapy, each chemotherapy cycle lasting for 3 weeks.
As a formal nationally co-ordinated clozapine monitoring protocol in India is not present, previous annual complete blood count reports were lacking. However, there was no history suggestive of infection and the complete blood count done in the hospital prior to starting chemotherapy was essentially within normal limits (white blood cell count 10,400 per cubic millimetre (mm) and neutrophils 8,160 per cubic mm, hemoglobin of 13.3 g/dl, platelets 387,000 per cubic mm). The dose of clozapine was reduced by the institute-based liaison psychiatrist to 250 mg in daily divided doses before chemotherapy started in an attempt to reduce the hypersalivation, which was helpful and did not lead to any exacerbation of psychotic symptoms. A decision to continue on clozapine in this dose was taken following a detailed discussion between the liaison psychiatrist with patient and his brother, discussing the benefits and risks associated, with regular monitoring schedules fixed for mental state examination, complete blood counts and medications. Though the white blood cell count monitoring was advised 17 days following the start of the first cycle of chemotherapy, it was decided that it be done earlier than that, in view of the concurrent treatments.
A complete blood count done on the 10th day in a private laboratory following start of first chemotherapy cycle revealed a drop in the white blood cell count to 2300 per cubic mm, neutrophils were 79%, platelet count of 250, 000 per cubic mm and hemoglobin of 10.3 g/dl. The patient developed high grade fever and mucositis, for which he was assessed on the 11th day and daily subsequently for the next week by the medical oncologist. The white blood cell count repeated on the 12th day, this time in the hospital laboratory, revealed a further drop to 1000 per cubic mm. Patient was started on antibiotics and granulocyte colony stimulating factor, following which the white blood cell counts improved on the 17th day to 8910 per cubic mm. As the platelet count was low, patient received platelet transfusion.
In the course of these events in these 8 days from 10th to 17th day following start of chemotherapy, the liaison psychiatrist after discussing with patient and his brother had earlier reduced the dose of clozapine to 200 mg daily in divided doses on the 5th day, considering the possible synergistic effect of the drug, as reported in literature[] and a watch was kept on the mental state. But further discussion between the psychiatrist and the consultant medical oncologist and also between the former and the patient with his brother, led to the decision of maintaining patient in the same dose of clozapine, keeping in view the patient's psychiatrically stable condition and to prevent rebound psychosis leading from any further reduction, as this neutropenia seemed to be related to the chemotherapy.
By the 18th day, which was originally suggested as the day for monitoring post first chemotherapy cycle, when the complete blood count was repeated, the white blood cell count was 10,800 per cubic mm and neutrophil count 7680 per cubic mm, (essentially within normal limits), but still a low haemoglobin of 10.3 g/dl and a manual platelet count of 100,000 per cubic mm.
The pre-planned second cycle of chemotherapy was delayed by just 6 days following completion of the first cycle and a third cycle was added at the scheduled time after imaging and reassessment by the multidisciplinary Head and Neck Joint Clinic of the hospital. Growth factors were started on day 6 of each of these cycles as a preventive measure, in view of the experience of the first cycle. The white blood cell and neutrophil counts remained within normal limits after the second cycle but dropped to 2400 per cubic mm and 570 per cubic mm respectively after the third cycle. These stabilized to normal limits within the next 2 weeks. The dose of clozapine that the patient was on remained unchanged [].
At this stage, a further reassessment by the head and neck oncology group advised radiation therapy with a palliative intent in view of progressive disease. The psychiatry team liaised with the consultant radiation oncologist prior to scheduling of radiotherapy sessions, with continuation of weekly monitoring protocols for mental state examination and complete blood counts. The patient underwent palliative external radiotherapy to face and neck to a dose of 50 Gy in 20 fractions, which led to partial relief in pain and swallowing. During this period, his mental state remained stable and white blood cell counts were maintained within normal limits, continuing so during the 2 months following completion of radiation treatment. To palliate further, metronomic therapy was then advised, which the patient underwent, with regular psychiatric review and weekly complete blood counts over the next 2 months. There was no resurgence of psychotic symptoms. Unfortunately, he had a rapid progression of disease and developed local infection. The white blood cell count increased to 13,600 per cubic mm and later to15,600 per cubic mm. He passed away 2 weeks later, nearly 8 months following his initial diagnosis.


A 47-year-old woman presented with lumbago and severe left leg pain for 1 week. The patient had no trauma history or any experience of similar pain. The pain was aggravated by walking. The patient complained of severe radiating pain on left L3 sensory dermatome area and reported aggravation of leg pain at 20 degrees of hip flexion by SLRT. However, there was no motor weakness on neurological examination.
Plain films of the lumbar spine showed only loss of lordotic curvature. Computed tomography (CT) revealed no bony abnormality, such as osteolysis. Magnetic resonance imaging (MRI) demonstrated a plaque-appearing extradural mass which was located in the left and posterior spinal canal at L2-3 level. The lesion was homogeneous iso-SI on T1-WI, hypo-SI on T2-WI, and peripheral rim enhancement on a gadolinium enhanced MRI ().
She was not able to walk and sleep due to incapacitating pain. The pain was not relieved by intravenous analgesics. We decided to perform surgical treatment. Under microscopy, left partial laminectomy was done at L2-3 level. The yellow-reddish colored, and flat typed-mass was located between the dura mater and the anterior wall of the L3 lamina (). The mass was soft and easily separated from the dura, lamina, and pedicle. The mass was totally removed.
Histopathologic examination revealed that the tumor consisted of chondroma, which had mature hyaline cartilage with nests of benign-appearing cells and calcium deposits in lacunae ().
Postoperatively, the radiating pain was completely relieved. At 3 months after surgery, follow-up MRI showed no remnant mass or recurrence. The patient presented no radiating or back pain anymore.


A 4 year-old girl reported to the Department of Pedodontics and Preventive Dentistry at A. B. Shetty Memorial Institute of Dental Sciences, Mangalore, India with a swelling in the gums (limited to gingiva) on the lower left side of her jaw.
It had been present for a period of ten days. The swelling was associated with pain and uncontrollable bleeding. The bleeding began two days prior to the child’s visit to the dental operatory, for which a physician was consulted and tranexamic acid was administered immediately as nasal drops.
Her medical history revealed a subgaleal hematoma after trauma to the occipital region at the age of two, and uncontrolled bleeding for 3 consecutive days. Furthermore, an injury to the anterior faucial pillar of the tonsil at the age of three resulted in continuous bleeding and led to an increased suspicion of a bleeding disorder. There was no history of consanguineous marriages in the family. The child’s diet history revealed a cariogenic dietary pattern, with inadequate oral hygiene measures.
On general physical examination, ecchymosis on the trunk as well as the upper and lower limbs was observed (). Intraoral examination revealed a periapical abscess in the left lower primary molar () with a deep carious lesion in the contralateral tooth. Dentinal caries were present in the primary maxillary incisors () and molars along with primary mandibular second molars (). The right and left primary mandibular first molars had deep carious lesions causing coronal destruction, radicular resorption and furcation radio-lucencies (). Individual intraoral periapical radiographs were obtained with foam coated over the X ray holder to prevent injury to the mucosa. This was the child’s first dental experience.
The child belonged to the uncooperative group (Frankel’s behavior rating scale-definitely negative) [] and repeated attempts to achieve the desired behavior with usual nonpharmacological techniques failed. Hence, treatment under general anesthesia was considered the best option for this child.
In addition, the need for repeated lengthy dental appointments and her medical condition warranted transfusions; which further justified treatment under general anesthesia.
A detailed hematological investigation revealed the following values as shown in : Factor VIII (100%), IX (80%), XII (87%) and fibrinogen (277 mg %) were found to be within normal levels, with borderline low levels of factor IX. Normal platelet counts, prolonged bleeding times, and abnormal platelet aggregation along with clinical signs and history of frequent superficial bruises and prolonged mucocutaneous bleeding led to the diagnosis of Glanzmann’s thrombasthenia. To provide the best care, an interdisciplinary approach was pursued with a team consisting of pediatric dentists, pediatricians and anesthesiologists. Complete oral rehabilitation was planned under general anesthesia with all considerations required for an interdisciplinary approach.
The treatment was divided into 2 phases, namely, the medical phase and the dental phase. The bleeding time was recorded prior to the procedure and was not within the normal limits ().
Reckoning the patient’s past medical history, the pediatricians recommended 1 unit of platelet transfusion 2 hours preoperatively and 1 unit postoperatively as blood loss was anticipated.
Special caution was taken during nasal intubation to prevent bleeding from the end vessels present in the nasal epithelium. A smaller size intubation tube was slowly inserted by the anesthetist with utmost care.
The dental phase consisted of multiple esthetic restorations for the anterior teeth with strip crown and restorative procedures for posterior decayed teeth.
Glass ionomer cements (GC, Type IX) and posterior composite resin (3M, ESPE) were the materials of choice for the restorative procedures. The local anesthetic used for the surgical procedure was lignocaine 2% with adrenaline; which was administered via infiltration technique prior to the extractions of the primary mandibular first molars.
Local measures taken to prevent any inadvertent bleeding from the soft tissues included wrapping of the bite block in cotton, careful positioning of the mouth mirror and other instruments used, avoiding nerve blocks, using the infiltration technique with insulin syringes and prevention of hematoma.
During the procedure there was bleeding from the extraction sockets; which were brought under control with the help of gauze soaked in tranexamic acid solution and pastes made of tranexamic acid tablets mixed with water; which were placed repeatedly into the socket. A total of 4 tablets and 3 vials were used as per the recommendations of the pediatrician so as to not exceed the maximum permissible dose (recommended dose in children 50-100 mg/kg) []. The patient was advised to maintain “Nothing Per Oral” (NPO) for 24 hours to prevent any injury to the extraction socket.
Brushing and rinsing was not recommended for 24 hours, but gentle use of wet gauze was advised for hygiene. The patient was asked to refrain from consumption of hard and fibrous food, and have only pureed food/soups/tender coconut water for 2 days post-surgery.
To prevent dehydration, an IV line was maintained during the NPO status. An antibiotic prescription of Augmentin (amoxicillin + clavulanic acid, 30 mg/kg body weight) for 5 days and acetaminophen (15 mg/kg) were recommended. At 2 weeks post-procedure, the socket area was examined to confirm adequate healing, after which a bonded space maintainer was given for the first primary molar in the right quadrant, followed by the same in the left quadrant for the contralateral tooth the next week. At the end of 3- and 6-month follow ups, the space maintainers were intact in the oral cavity.