A 55-year-old male patient suffering from left loin pain was admitted to the outpatient clinic. Following the required urological evaluation a left ureteral stone measuring 22×30 mm was detected. Non-enhanced, stone protocol, abdominopelvic spiral computed tomography (CT) was performed to evaluate the stone location and other anatomical structures. Plain X-ray KUB and IVU pictures were confirmed the left ureter stone []. Retroperitonoscopic ureterolithotomy was planned. The proper dissection was performed retroperitonocopically. Psoas major muscle, genitofemoral nerve, lower pole of left kidney, ureter were identified and dissected. At the level of mid-upper ureter, under the ureter, a nearly 2×2 cm bulging was observed and thought to be an anatomical variation of the ureter. The tactile sense of the bulging surface was hard and smooth. Therefore, a gentle aspiration was tried but not achieved. Mini-incision was performed with endoscalpel and there was sudden bleeding. Then, we converted the procedure to an open surgery because of an unexpected and uncontrolled bleeding. During open exploration, a common iliac artery aneurism and atherosclerotic structures were detected at the same level that was mimicking the ureter and stone. The incision on the aneurism was closed with 6/0 polyglicolic acid. The common iliac artery aneurism was located at the level of bifurcation and ureteral cross-side []. Aneurysmatic dilatation and calcified atherosclerosis of common iliac artery and external iliac artery mimicked the ureteral stone and caused the unusual complication as well []. The patient was discharged at the fourth day without postoperative complication.


A 40-year-old male presented with history of giddiness, gait ataxia and raised intracranial pressure (ICP) of 1 month duration. At presentation, his pulse rate was 66/minute and B/P was 110/70 mmHg. Electrocardiography (ECG) revealed sinus rhythm []. There was no history of any prior cardiac or other co-morbid conditions. Magnetic resonance imaging (MRI) of the brain revealed a midline posterior fossa lesion involving the dorsal brainstem, extending from the mid pons to the upper cervical spine [Figure –]. Anesthesia was induced with fentanyl 2 µg/kg and thiopentone 3 mg/kg. Muscle paralysis was achieved with pancuronium 0.1 mg/kg. Maintenance of anesthesia was carried with oxygen in nitrous oxide at a ratio of 50:50 and ventilated with volume controlled mode to mild hypocapneuic levels of pCO2 to 32 mmHg. The patient underwent a midline suboccipital craniotomy and decompression of the lesion. The tumor had a poor plane with the brainstem. Intraoperative bradycardia was recorded thrice. The first episode was related to tugging a small bit of tumor adherent to the floor of the fourth ventricle and the other two to placement of cottonoids, during hemostases, at the tumor bed. All three episodes ranged from a heart rate between 50 and 60 beats/minute, were transient (<1 minute) and recovered on discontinuing the offending stimulus.
Postoperatively, the patient was electively ventilated for 12 hours. A computed tomography (CT) scan of the brain [] done 6 hours post surgery revealed adequate decompression of the lesion but persisting hydrocephalus. A subsequent CT scan done 24 hours after surgery revealed a decrease in the size of the ventricles [].
The patient was electively ventilated and gradually weaned off during the first postoperative day. He demonstrated cardiovascular instability subsequent to weaning off with fluctuating pulse rate and predominant sinus bradycardia. The pulse rate at this point ranged from 50 to 65 beats/minute. The initial suspicion was related to the hydrocephalus and possible raised ICP. But during this time, the patient had been extubated, was conscious, alert and had no clinical features of raised intracranial tension. He developed a tachycardia-bradycardia syndrome on the second postoperative day. A formal cardiology evaluation was considered when an MRI of the brain [] revealed complete resolution of the hydrocephalus and thin bilateral subdural hygromas eliminating completely the possibility of hydrocephalus induced bradycardia. Initially, a 12-lead ECG and then a Holter recording [] revealed SSS with the typical tachycardia-bradycardia rhythm with a variable heart rate from 33 to 109 beats/minute. The ECG revealed a 2:1 block during the bradycardia phase [Figure and ]. In comparison to the preoperative ECG (sinus rhythm), the typical bradycardia-tachycardia syndrome and the 2:1 block were typical of the SSS. This patient responded well to initial doses of Atropine and subsequently was stabilized on Orciprenaline (Alupent®, German Remedies Mumbai, India) 10 mg t.i.d. Cardiac pacing was not deemed essential according to the AHA guidelines.[] His heart rate stabilized between 65 and 70 beats/minute at discharge. A timeline graph of the perioperative cardiovascular events has been delineated in .


We report a case of 15-year-old young man otherwise healthy who complains for 4 months of right upper quadrant pain accompanied with high fever and productive cough. Chest X-ray revealed an elevation of the right diaphragmatic dome (). Abdominal ultrasonography showed a hypoechoic and heterogeneous mass, measuring 8 cm in diameter, localized in the hepatic dome (). At his admission, the physical examination was normal. White blood cell count was 7500; c-reactive protein was normal too. Hepatic function test and tumor markers were within normal range (AFP and CEA).
Abdominal tomography revealed a subcapsular lesion occupying segments VII and VIII and measuring 4 cm in diameter spontaneously hypodense and having slight peripheral enhancement at delayed stage ().
A second tomography done one month later described the same lesion with the same characteristics but with a dramatic decrease in size which is evaluated to be 2 cm in diameter (). This spontaneous regression in size associated with radiological characteristics of a partially fibrous lesion permitted us to make the diagnosis of IPL and to abstain from any therapeutics. The patient is doing well and another radiologic control is foreseen in 6 months.


A weak and febrile 47 years old diabetic male presented to Tata Memorial Hospital (TMH) on 04.09.95 with hemoglobin 5.6 gm% and total count 17,100/cu mm. Investigation of the case at TMH confirmed diagnosis of AML M3. His Bone Marrow Aspiration (BMA) cytology indicated diluted cellularity; myeloid series – promyelocytes 96%; lymphocytic series and megakaryocytes were absent. Cytochemistry: a myeloperoxidase test was 100% positive. The patient was started with ATRA for 90 days from 14.09.95, after which he developed bronchospasm, high grade fever and odynophagia. He was treated with blood components and antibiotics conservatively. Bone marrow analysis carried out on 13.11.95 indicated that cellularity, erythropoesis and myelopoesis were normal. However, 1% lymphopoesis blasts were present. His induction chemotherapy was given from 12.01.96 to 18.01.96. Post chemotherapy the patient had fever and vomiting; these problems were managed conservatively. The patient then received 3 cycles of consolidation chemotherapy with Daunorubicin and Cytarabine combination from 12.01.96 to 14.05.96. The patient achieved clinical remission; however, cytogenetically the disease persisted. At initial diagnosis the patient was 100% t(15;17) positive and after (ATRA + 4 cycle chemo) he was 50% t(15;17) positive. For the next seven months the patient was relatively event free. In January 97 the patient again presented to THM for checkup. Investigations done at TMH indicated relapse of the disease. BMA indicated cellularity – hypocellular; erythropoesis – hyperplasia; myelopoesis – blast 2%, abnormal promyelocytes 75%, myelocytes 10% (NEC blast was seen), mature myelocytes 3%, band 10%. The patient then underwent two cycles of chemotherapy with Idarubicine and Etoposide. The first and the second cycle were given from 18.02.97 and 18.05.97, respectively. The patient tolerated the chemotherapy with some side effects. For the next 6 months after the completion of chemotherapy the patient was in remission. The Bone marrow investigation on 12/12/97 indicated a second relapse of the disease. Blast cell 14%, abnormal promyelocytes 85% (Faggot cells present) and 1% basophil were seen. A cytochemistry report found a myeloperoxidase test to be 100% positive. The patient was again advised to undergo another round of chemotherapy with a combination of Mitoxantrone, Cytarabine and Etoposide. However, he refused further chemotherapy and volunteered for a pilot study conducted by the Central Council for Research in Ayurveda and Siddha (CCRAS),[] and try an Ayurvedic therapy advocated by the first author. The patient’s bone marrow slide from TMH was reviewed at Institute of Rotary Cancer Hospital (AIIMS), New Delhi, and he was enrolled for the study on 22.12.97.
When the Ayurvedic therapy started, the patient was very weak, anemic, anorexic, febrile, had a lung infection and a big rectal abscess. His hemoglobin was 7.0 gm%, total count was 1050/cu mm, neutrophil to lymphocyte ratio was 5:95. The patient was treated conservatively with antibiotics (oral, I.V. and local application), along with Ayurvedic therapy. He was also given blood transfusion and human granulocyte colony-stimulating factor to improve his WBC count. Strict isolation was maintained, calorie rich diet devoid of Pittavardhak aahar and salt was prescribed. After about 15 days of alternative therapy the patient’s general condition started to improve, his lung infections subsided and he started to maintain normal body temperature. After 1 month of therapy his differential counts started to show a normal picture. As the patient continued with the therapy his weakness gradually subsided. The frequency of blood transfusion was also reduced. Before the start of Ayurvedic therapy he had received 269 units of blood components viz. 20 units of fresh frozen plasma; 32 units of packed cells; 31 units of whole blood; 165 units of platelets concentrate and 21 units of plasma concentrate from 07.09.95 to 18.12.95. After the start of Ayurvedic therapy the patients received 16 units of whole blood; 1 unit of packed cell and 6 units of plasma concentrate in the first two months of the therapy. No further blood transfusion was required. The Ayurvedic therapy was continued for one year and stopped. BMA studies done after 90 days Ayurvedic treatment on 01.04.98 indicated the disease was in remission. Though there was mild erythroid hyperplasia, granulocytic series appeared to be normal in morphology and maturation. No abnormal cells were seen. For the subsequent 5 years till 2003 the patient received three months Ayurvedic treatment every year. Subsequent, BMA performed on 19.01.2000 and 14.11.2000, respectively from Bangalore Institute of Oncology, Bangalore and Sir Ganga Ram Hospital, New Delhi detected no abnormality. Details of the hematological parameters investigated during follow up are given in . During the treatment period, and later during follow up, the patient was constantly monitored for liver and kidney function. The Ayurvedic treatment did not produce any hepato or renal toxicity. In June 2002 the patient had purpuric lesions on both legs with itching and burning sensation. His problems were managed conservatively. At present the patient has completed 7 years of relapse free survival after the stoppage of Ayurvedic therapy. He is still in follow up and doing well along with conventional management for his diabetes.


A 17-year-old Caucasian female presented to local hospital with a history of acute alcohol intoxication and ingestion of large quantities of acetaminophen. The patient complained of nausea for 2 days and subjective increase in thirst and urine output for a day. She did not notice any dysuria, abdominal pain or change in the colour of her urine. She did however report a remarkable increase in lower back pain that she had attributed to strenuous exercise.
Two days prior to her presentation to the emergency department, the young lady had attended a party where she consumed over 300 mL of vodka and ingested twenty 500 mg tablets of acetaminophen later that night.
Past medical history revealed that she had experienced an episode of rash after having taken liquid acetaminophen as a child. She had not taken acetaminophen since infancy and also denied regular use of any other analgesic. Family history was non-contributory for relevant physiological and psychological illness. The patient reported feeling stressed due to a recent slip in school grades, however denied any suicidal ideation.
Physical examination in the emergency department revealed blood pressure of 140/92 mmHg, pulse 80 bpm, temperature 36.9°C, respiratory rate of 16 breaths per minute and oxygen saturation of 100% at room air. Height and weight were recorded at 170 cm and 63 kg. Physical examination was otherwise unremarkable. No rash, joint swelling or sign of dehydration was detected.
Initial renal function tests at the local hospital revealed serum creatinine and BUN levels of 1.6 mg/dL and 19.3 mg/dL, respectively. Other laboratory results included: glucose 112 mg/dL, sodium 141 mEq/L, potassium 4.3 mEq/L, chloride 104 mEq/L, bicarbonate 29 mEq/L, albumin 43 g/L, aspartate transaminase 20 IU/L, alkaline phosphate 66 IU/L and total bilirubin 1.11 mg/dL. Her INR was reported to be normal at 0.9. Toxicity screens on admission showed acetaminophen level at 9.06 μg/mL. Urine dipstick showed trace amount of blood and 2 plus protein. Microscopy revealed 25 red blood cells per high power field and was otherwise non-contributory. Abdominal ultrasound revealed increased hepatic echogenicity and the kidneys were normal in appearance.
At the outside hospital, she was given morphine, ondansetron and lansoprazole for her back pain and gastrointestinal symptoms. Acetylcysteine was not administered as the patient presented more than 24 hours after the ingestion of acetaminophen.
Renal function tests were repeated later that evening and monitored frequently thereafter. Over the course of 36 hours, the patient's serum creatinine and BUN levels elevated from 1.6 mg/dL and 19.3 mg/dL to 5.7 mg/dL and 49.6 mg/dL, respectively. Urine output was reported to be 2.7 mL/kg/hour. At this time, the patient was transferred to our Children's Hospital for rapidly progressive renal insufficiency.
Physical examination upon admission showed normal vital signs: blood pressure 115/74 mmHg, pulse 60 bpm, temperature 36.6°C, respiratory rate 16 breaths per minute and 100% oxygen saturation at room air. Serum creatinine and BUN levels were measured at 6.3 mg/dL and 52.4 mg/dL, respectively. Furthermore, her electrolytes were recorded as follows: random glucose 86 mg/dL, sodium 134 mEq/L, potassium 5.7 mEq/L, chloride 104 mEq/L and bicarbonate 22 mEq/L. Coagulation study revealed an INR of 1.0 and an APTT of 28 seconds. Other investigations including blood counts and liver functions tests were within normal range. The patient's daily urine output was 3.3 mL/kg/hour at this time. Urinalysis revealed small amounts of protein and blood in her urine. Staining for eosinophils on the random urine sample was positive.
Renal biopsy was performed to further delineate the underlying cause of rapidly progressive renal insufficiency. Figure depicts the histological findings. The biopsy showed no evidence of glomerulonephritis. The interstitium was mildly edematous with small aggregates of inflammatory cells, mainly lymphocytes and eosinophils. Immunofluorescence and electron microscopy were both negative for immune deposits. The histological findings were compatible with acute interstitial nephritis.
The patient was managed supportively by careful monitoring of fluid intake and output. Corticosteroids therapy was considered if no improvement of her renal function was observed. However, her serum creatinine and BUN levels peaked at 7.0 mg/dL and 60 mg/dL two days after admission then began to improve without further medical intervention. Her serum creatinine and BUN levels upon discharge were 4.7 mg/dL and 48 mg/dL respectively. Outpatient follow-up at two weeks after discharge showed that she had completely recovered; her creatinine was 1 mg/dL and BUN was 18 mg/dL.


A 48-year-old man with several years history of abdominal pain and transit disturbances was admitted to our hospital for further investigations. Physical examination and laboratory data were within normal limits. Colonoscopy revealed a sessile, dimpled 20 mm cecal polyp () covered with macroscopically normal colonic mucosa. The appendiceal orifice in the cecum was not seen. Biopsies were performed, and, 12 hours after, the patient experienced fever and shivering. Clinical examination of the abdomen and plain abdominal roentgenogram were normal. Complete recovery was obtained after broad spectrum antibiotherapy. On histology, the polyp was found to be normal inverted appendiceal wall. Abdominal ultrasound and CT scan showed a partially invaginated appendix into the cecal cavity without any evidence of tumoral process. Regular ultrasound examinations during two years showed an unchanged aspect of the inverted appendix


A 65-year-old man was admitted to our hospital with a six-month history of melena with transfusion requirement. Hemogram showed hypochromic microcytic anemia (hemoglobin 8 g dL-1). Gastroscopy was normal but colonoscopy showed an actively bleeding, sessile, cecal polyp located in the usual site of the appendiceal orifice (). There were three angiodysplastic lesions in the rest of the cecum, without active bleeding. An abdominal CT scan showed an invaginated appendix. The exploration of the small bowel using video capsule endoscopy did not reveal any other source of bleeding. During surgery, the appendix was found to be partially invaginated into the cecal cavity, with bleeding through the appendiceal orifice. Ileocecal resection was performed. On pathological examination, the resected specimen was found to contain angiodysplastic foci without any malignant lesions. The patient's recovery from surgery was uneventful. No recurrence of the lower-gastrointestinal-tract bleeding was observed during followup, and anemia was corrected after oral iron therapy.


A 48-year-old man came to our hospital for sudden effort dyspnea. The patient underwent seven years ago to aortic valve replacement (AVR) with 25 mm On-X (On-X Life Technology Inc., Austin, TX, USA) mechanical prosthesis for severe aortic valve regurgitation and ascending aorta replacement with a tube graft n 28 for ascending aorta aneurysm. The AVR was performed using three semicontinuous (2-0 Prolene, Ethicon, Somerville, NJ, USA) suture technique. The surgeon used three similar sutures with knots tied among them, hence the term semicontinuous. According to a "parachute technique" the sutures were suspended (left loose) to enable an easier suturing of the aortic annulus and sewing ring of the artificial valve prior to lower the prosthesis. Then a gentle alternate traction on the three semicontinuous sutures, usually with the help of a surgical nerve hook, is applied to pull up the redundant suture. This could weaken the suture and lead to its failure at a later date.
A yearly transthoracic echocardiography (TTE) follow-up didn't show any pathological findings. The TTE, performed as soon as the patient was admitted to Emergency Room (ER), revealed a severe aortic regurgitation due to a PVL. The ejection fraction was normal but the left ventricle was dilated: indexed end diastolic volume (iEDV) 107 ml, indexed end systolic volume (iESV) 45 ml. The transesophageal echocardiogram (TEE) confirmed the severe aortic regurgitation, showing an aortic non coronary sinus PVL with a partial detachment of aortic prosthesis. Figure clearly documents the PVL.
Preoperative 3 D, 24 slices, Computed Tomography Scan (CT-scan) showed perfect patency of coronary arteries and severe retrosternal adhesions.
After the sternum reopening and adhesions dissection, the ascending aorta tube graft was opened. The surgical findings confirmed a partial prosthesis detachment at the level of the non coronary aortic sinus, due to the semicontinuous suture breaking (Figure ). The other two sutures of the left and right coronary sinuses were intact. After the prosthesis explant, a new aortic mechanical prosthesis was implanted (23 mm S. Jude, S. Jude Medical, St. Paul, MN, USA), using interrupted suture technique with 2-0 Ethibond (Ethicon) polytetrafluoroethylene (Teflon)-buttressed stitches. The Teflon buttresses were positioned in the ventricular aspect of the annulus. The postoperative TEE showed normal position and function of the prosthesis The postoperative course was uneventful and the patient was shortly discharged.


A 71-year-old lady had an incidental finding of an opacity in her left lower lobe on a chest radiograph. Thoracic CT confirmed the presence of a 1.0 cm solitary pulmonary nodule but did not identify any lymphadenopathy or evidence of distant metastatic disease.
The patient was referred for an F18 FDG-PET study () for investigation of the pulmonary nodule []. The PET study confirmed a small retrocardiac focus of increased FDG uptake within the left lower lobe nodule (thin arrow) suggestive of a malignant process. In addition, the study demonstrated abnormal increased activity at the gastro-oesophageal junction (thick arrow). It was proposed that metastasis at this site from a primary lung malignancy would be unusual and that two malignancies (i.e. lung and gastric) were possible []. Lung metastasis from a gastric neoplasm was also possible, however, isolated pulmonary metastasis from gastric cancer is rare. Thus, further investigation was recommended.
The patient underwent upper gastrointestinal endoscopy. Biopsy of the gastric wall during endoscopy revealed non-Hodgkin’s lymphoma (NHL). The patient proceeded to thoracotomy for investigation of her lung lesion to definitively ascertain the nature of this lesion. Pathology again confirmed lung NHL. Therefore, the patient was treated with systemic chemotherapy for lymphoma.
A follow-up post therapy FDG-PET study () showed a complete metabolic response to treatment both in the stomach and the left lung when compared to the initial study ().


A 17-year-old male presented with a few days history of severe headache, visual disturbances, and a few episodes of vomiting. There was no history of fever, altered consciousness, seizures, or upper or lower limb weakness. The past medical history was unremarkable. At the time of examination, his BP was 240/130 mm Hg. The fundus examination revealed bilateral papilledema with grade 4 hypertensive changes. The neurological examination revealed normal grade 5 power in all four limbs with normally elicited deep tendon reflexes. The sensory examination was unremarkable with normal flexor plantar response bilaterally. Serum electrolytes were normal; kidney function tests were deranged (blood urea 86 mg/dL, serum creatinine 3.7 mg/dL). A renal biopsy revealed features of IgA nephropathy. MRI of the brain revealed a hyperintense signal of medulla and cervical cord on T2 and FLAIR images (Figures ); small hyperintense foci were also noted in the parieto-occipital regions and deep cerebellar white matter. The lesions were also hyperintense on diffusion-weighted imaging (because of T2 shine-through); there was no evidence of restricted diffusion on apparent diffusion co-efficient maps. As there was no clinical or lab evidence to support the diagnosis of encephalomyelitis or acute disseminating encephalomyelitis (ADEM) as suggested initially by the radiologist, an atypical variant of PRES was considered as the most plausible diagnostic possibility. Further investigations including CSF studies were not considered necessary in view of the strongly suggestive clinical and imaging features. The patient rapidly improved with antihypertensive treatment and a repeat MRI done 1 month afterward showed resolution of the earlier imaging findings.


A 42-year-old Caucasian woman was referred to our hospital for pain management by a neurologist specializing in the treatment of daily headaches. She had experienced daily intractable headaches since age 18 years. She also had chronic bilateral occipital neuralgia on the basis of the diagnostic criteria outlined in the second edition of The International Classification of Headache Disorders []. Her occipital neuralgia persisted for more than 15 days monthly and was distributed throughout the greater occipital nerves, beginning in the occipital region and radiating upward to the top of the head. When the occipital neuralgia occurred, her occipital area became very tender to palpation. Complete alleviation of her pain had been achieved for a limited time with diagnostic bilateral greater occipital nerve blocks.
Her medical history included NF-1, which was first diagnosed in childhood. Several neurofibromas had been removed from her sacrum 10 years previously, as well as many from her upper extremities. She also had had problems with depression, anxiety, alcohol consumption, and smoking. She has been a housewife throughout her adult life. With regard to her family medical history, her mother had died at 68 years of age as a result of heart disease, and her father was alive at 72 years of age with a history of cancer. An older sister has rheumatoid arthritis but not NF-1.
Before her referral to our service, she had undergone extensive medical management with biofeedback training, physical therapy, massage, acupuncture, and pharmacological management with narcotic and non-narcotic pain medications. Her medications included sustained-release morphine (30 mg every 12 hours), hydrocodone and acetaminophen (10 mg and 325 mg, respectively, every four to six hours), and pregabaline (75 mg every eight hours). More recently, she had undergone three greater occipital nerve blocks that resulted in complete pain resolution that lasted from two to three days. Because she required an ever-increasing dose of morphine for pain relief, and because she had responded to the occipital nerve blocks, she was considered to be a good candidate for OPNS.
At her baseline office visit, the patient underwent a disability and quality-of-life assessment by completing a series of questionnaires (see "Quality-of-life assessment" section below) and was found to be a suitable candidate for a trial of OPNS. After the risks and benefits of the procedure were discussed with the patient and her informed consent was obtained, the trial of OPNS was carried out in October 2008 by using two percutaneous eight-contact leads (Octrode; St Jude Medical Neuromodulation Division, Plano, TX, USA). After a week-long successful trial with more than 80% symptom improvement, the patient was deemed a suitable candidate for permanent implantation and she underwent implantation of two permanent percutaneous eight-contact leads (Octrode) and a conventional implantable pulse generator (IPG) (Genesis; St Jude Medical Neuromodulation Division).
On the day of the procedure, which was carried out in an operating room, a slow intravenous infusion of 2 g of cefazolin was started, and the patient was placed in a prone position with pillows under her chest to augment neck flexion. Monitored anesthesia was administered by using intravenous fentanyl and midazolam at a level that allowed the patient to be comfortable but able to interact with medical personnel throughout the procedure. The patient's hair was shaved below a line connecting the external occipital protuberance to the mastoid processes, and her skin was treated with chlorhexidine. A sterilely draped C-arm was introduced to obtain a true anteroposterior image of the cervical spine at the C1-C2 interspace, and the overlying skin was marked with a sterile marker. Thereafter a portable ultrasound with a sterile linear array transducer of 5 MHz to 13 MHz frequency was placed to obtain images of the bilateral occipital fossae and the bilateral greater occipital nerves and arteries. The ultrasound probe was first placed at the midline just below the external occipital protuberance (Figure ). The probe was slowly advanced laterally at the same level until the greater occipital artery and nerve were visualized as two distinct structures: the artery as a hypoechogenic oval structure and the nerve as a hyperechogenic structure (Figures and ). The nerve could be traced from its exiting trunk into two distinct divisions within the substance of the trapezious muscle. The artery was identified by using Doppler ultrasound (Figure ). The locations of the nerve and the artery were marked bilaterally on the skin with a sterile marker. The depths of both the artery and the nerve were found to be consistent at 1.0 cm to 1.2 cm from the skin surface. The skin overlying the greater occipital protuberance was injected with 2 ml or 3 ml of 1% lidocaine as a local anesthetic, and the stimulating electrodes were introduced through a 14-gauge introducer needle (0.5 cm to 0.7 cm below the skin surface) in a mediolateral position. Positioning was guided by the skin markings and was verified by fluoroscopy to complement the ultrasonographic images (Figure ). The electrodes were tested intra-operatively by confirming adequate dermatomal paresthesia within the occipital nerve distribution. Implantation of the electrodes was performed by creating a mid-line subcutaneous pocket at the site of needle insertion. The implantable, programmable, rechargeable generator was permanently implanted in a subcutaneous pocket area in the left buttock. For the implantation, a local anesthetic (0.25% bupivacaine with epinephrine 1:200,000 to a total of 20 ml) was used for skin and tissue infiltration.
Two months after the implantation procedure, she developed an infection over the occipital implantation area, and the leads and the IPG were removed (Figure ). Bacterial cultures were not obtained during the removal procedure. Her recovery from the procedure was uneventful. After the infection resolved with antibiotic treatment, she underwent re-implantation of two permanent octapolar leads and a new IPG (Eon Mini; St Jude Medical Neuromodulation Division) in March 2009 without additional complications.
The patient's pain level and quality of life were assessed at baseline and again at one, three, and six months after implantation. The questionnaires used in these assessments were the short form McGill Pain Questionnaire [], the Visual Analogue Scale (VAS) [], the Oswestry Disability Questionnaire [], and the SF-36 Health Survey []. The data gathered from these questionnaires were plotted for comparison. Qualitative data were also collected from the patient's medical record.
After the trial implantation, the patient experienced nearly an 80% reduction in headache severity. Although the initial implantation procedure was complicated by infection in the implantation site and the patient underwent reimplantation, she has experienced sustained benefit from the treatment and remains infection-free. Over time, the severity, frequency, and duration of her headaches have improved by more than 60%. She continued to use hydrocodone and pregabaline, but was able to discontinue use of morphine. All outcome measures of pain and quality of life were positively affected by the treatment. She reported improvement in her quality of life, which she characterized as "being more active and enjoying life," being able to exercise, working for longer hours, and having improved mood.


A 60-year-old woman presented to our clinic with a two-week history of a focal patch of alopecia on her scalp. She had a history of hypothyroidism and hypercholesterolemia, treated with eltroxin and simvastatin, correspondingly. The patient’s daughter was reported to suffer from vitiligo. On physical examination, she had a regularly shaped, well-defined alopecic patch involving the vertex of her scalp. In addition, a depigmented patch was localized almost exactly to the same area as the alopecia []. She had no other areas of alopecia or depigmentation. A skin biopsy specimen revealed the presence of an anagen hair follicle with a peribulbar lymphocytic infiltrate, confirming the diagnosis of AA []. In addition, using MART-1 immunostaining, almost total loss of melanocytes was demonstrated in the basal layer, with only one or two melanocytes identified in the epidermis, confirming the coexistence of vitiligo in the same region [].


A 72-year-old man presented with high-grade fever with chills and dysuria for last 10 days, decreased urine output, and worsening breathlessness since 1 day. He denied any history of hematuria, pain in abdomen, skin rashes, or joint pains. He was a diabetic and hypertensive on regular treatment since 5 years. Physical examination revealed a middle aged man in acute respiratory distress with a respiratory rate of 32 beats/min. His blood pressure was 150/90 mmHg and his temperature was 100° F. The jugular venous pressure was elevated, he had bilateral pitting edema of the legs, and sinus tachycardia was present (108/min with an S3 gallop). Auscultation of the lungs revealed pulmonary congestion.
His laboratory investigations were as follows: hemoglobin 9.1 g/dL, total leukocyte count 23,200/cmm, platelet count 592,000/cmm, BUN 65 mg/dL, creatinine 1.8 mg/dL, sodium 139 mEq/L, potassium 4.3 mEq/L, chloride 95.3 mEq/L, bicarbonate 13.8 mEq/L, total bilirubin 1.3 mg/dL, direct bilirubin 0.3 mg/dL, SGOT 32 IU/L, SGPT 25 IU/L, ALP 210 IU/L, total proteins 7.7 g/dL, albumin 3.1 g/dL, C-reactive protein 142, amylase 71, lipase 82, procalcitonin 1.4. His urine microscopy revealed trace proteins with entire field full of pus cells. A chest radiograph confirmed pulmonary edema. His abdominal sonography was unremarkable except for moderate ascites. CT-abdomen and pelvis revealed acute pyelonephritis with perinephric fat stranding.
The patient was transferred to the intensive care unit. Diuretic therapy was intensified (frusemide infusion), he was put on noninvasive ventilation, and broad-spectrum intravenous antibiotics were administered (meropenem, teicoplanin, aztreonem). His urine culture came positive for Escherichia coli, whereas blood culture was sterile. He continued to have high-grade fever and went into septic shock, with the acute physiology and chronic health evaluation (APACHE) II score of 21 and the sepsis-related organ failure assessment (SOFA) score of 9. He was intubated, put on ionotropic support, and antibiotics were changed according to sensitivity reports (tigecycline, colistin, vancomycin, clindamycin). His blood pressure stabilized at 100/50 mmHg on dopamine infusion at 20 μg/kg/min, noradrenaline at 15 μg/kg/min, adrenaline at 0.1 μg/kg/min, and vasopressin at 0.02 U/min. He was initiated on sustained low-efficiency hemodialysis (SLED), following anuria, worsening azotemia, and severe metabolic acidosis. In view of on-going sepsis and unstable hemodynamics, decision was taken to initiate him on direct hemoperfusion using PMX-DHP.
After priming the cartridge and blood lines, 2 hrs of direct hemoperfusion was performed using a blood flow rate of 100 mL/min and heparin anticoagulation. Twelve hours after the initiation of PMX-DHP therapy, vasopressin and adrenaline infusions were tapered, and there was a noticeable improvement in his urine output. 24 hr after initiation of PMX-DHP, vasopressin and adrenaline infusions were stopped. After another 24 hours, the dopamine and noradrenaline doses were decreased, and the urine output increased to approximately 0.7 mL/kg/hr. Under these conditions, the heart rate was maintained at 110 bpm; blood pressure 135/60 mmHg with a mean arterial pressure (MAP) of 85 mmHg []. The SOFA score improved from 9 to 6, and the APACHE II score improved from 21 to 11 (since the patient was under sedation, the score for the central nervous system was excluded). He required four more days of SLED, and his urine output increased to greater than 1 mL/kg/hr and he was taken off dialysis. He was discharged 60 days after the hemoperfusion therapy, with a stable creatinine of 2 mg/dL and on empirical antitubercular therapy.


A 22-year-old Asian American male presented for symptoms of dyspnea for six months that were being treated as asthma. This progressed to solid food dysphagia and a 20-pound weight loss over two years. The remainder of his history was noncontributory, and the physical exam was only positive for audible wheezing. A chest X-ray revealed a large mediastinal mass, with subsequent computed tomography (CT) scan demonstrating an upper esophageal mass that adhered to the posterior wall of the esophagus. The trachea, significantly narrowed, was seen to be bowing anteriorly (Figures and ).
Flexible esophagoscopy identified the mass at 19 cm from the incisors and with attachment to the left anterolateral esophageal wall. The following week, he was taken to the operating room for excision of the mass.
A right posterolateral thoracotomy was performed. The tumor was found in the submucosal layer along the wall. The azygos vein was visualized inferior to the mass and doubly ligated. The vagus nerve was free from involvement. The esophagus was opened from below the thoracic inlet to the level of the azygos vein. The mass was dissected and easily shelled out. Areas of the posterior wall were resected for tumor margins, and the esophagotomy was closed in two layers.
Gross examination of the esophageal mass revealed a smooth, multilobulated, but well-circumscribed mass measuring 8.5 cm in greatest dimension (). On cut section, the mass was uniform and tan with no discrete areas of hemorrhage or necrosis. Histologic examination of the mass revealed palisading spindled cells with scattered atypia, dense chromatin, and rare mitoses () in a background of prominent hyalinized vessels and a lymphoplasmacytic infiltrate. Immunohistochemical studies revealed the tumor to be positive for the S100 protein, confirming nerve sheath origin; the tumor was negative for actin, desmin, CD117 (ckit), and alk-1.
The patient tolerated the procedure well. His postoperative course was complicated by a contained leak at the esophagotomy repair site that was managed conservatively and postoperative pneumonia. He remained afebrile and hemodynamically stable. He was discharged postoperative day eight with a regular diet. At the three month followup visit, he was eating normally and had no symptoms.


We first diagnosed a 21-year-old Caucasian male suffering from CRC in January of 2009. The patient complained of having recurrent rectal bleeding for three months. He was otherwise a healthy non-smoker and in good condition appropriate for his age. His medical history was uneventful. Evaluation of family history revealed five relatives afflicted with malignant tumors at a young age. Among them were his mother, who died at the age of thirty-five from endometrial cancer, and the mother's brother, who passed away at the age of forty from CRC. The patient did not report significant weight loss, fever or night sweats. Physical examination was unremarkable. Carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were within normal range. Clinical staging diagnostics revealed a partially stenosing rectal adenocarcinoma (uT4, uN+) but no potentially metastatic lesions in the liver or lung at that time. There was no clinical evidence of FAP or Gardner syndrome. Li-Fraumeni syndrome was subsequently ruled out by sequencing of multiple TP53-exons (3-9) after PCR amplification of genomic DNA.
With respect to locally advanced tumor growth, the patient underwent neoadjuvant 5-fluorouracil-based chemoradiotherapy (5-fluorouracil/folinic-acid, 50.4 Gy) followed by low anterior resection including total mesorectal excision in the spring of 2009. Intraoperative sonography of the liver showed a small lesion in segment VII, but, due to the locally advanced tumor stage (pT4, pN2 (6/9), uM1 (hep), V1, L1, G2, R0), we decided in favor of non-simultaneous resection of the hepatic lesion []. According to revised Bethesda guidelines [], microsatellite instability (MSI) testing was performed by DNA isolation and subsequent PCR amplification from tissue of the primary rectal carcinoma resulting in detection of significant instability in microsatellites BAT25, BAT26, D17S250 and D2S123. This finding shaped up as high level of MSI (MSI-H). Moreover, sequencing of the protooncogenes KRAS and BRAF showed no mutation (wildtype). This raised the strong suspicion of a Lynch syndrome particularly with regard to the patient's family history, his age and the fulfillment of the Amsterdam criteria [,]. MSI in CRC of patients under the age of forty are estimated to be due to an underlying germline mutation in 85.7% of the cases, a probability, which is elevated by the presence of a BRAF-wildtype. The latter can be used to distinguish sporadic MSI CRC from MSI tumors that arise in the setting of Lynch syndrome []. Consecutively, the patient underwent human genetic counseling followed by testing for germline mutations in mismatch repair (MMR) genes by sequencing of their cDNA emanating from PAX-RNA and total RNA isolated from short-term lymphocyte culture. Thereby a mutation was detected in MMR-gene PMS2 (exon 11). Altogether, the diagnosis of Lynch syndrome was made.
Early restaging was performed during intermittent FOLFOX chemotherapy and the patient was found to have hepatic (Figure ) and pulmonary lesions suspicious for metastases. Thoracic computed tomography showed a well-circumscribed 6 mm lesion in the left lower lobe of the lung (Figure ) with homogenous contrast media enhancement as well as two smaller lesions in the right upper lobe. There were neither signs of infiltration of the adjacent tissue nor signs of pathologically enlarged lymph nodes. We decided to first perform a partial hepatectomy (segment VII), which confirmed hepatic spread of the tumor. In the light of the patient's young age, his early recovery and his good general state of health, we proceeded to remove the left-sided pulmonary lesion four weeks later. Therefore, he underwent atypical resection of the left lower lobe through a left anterolateral thoracotomy followed by a systematic mediastinal and hilar lymphadenectomy []. The patient's postoperative course remained uncomplicated and he again recovered well. Gross examination of the specimen, however, showed a well-circumscribed solid pulmonary tumor, 7 mm in diameter. Histological evaluation revealed a mixed papillary, hemorrhagic and sclerotic growth pattern of cuboidal surface cells and polygonal stromal cells. Cuboidal surface cells were immunopositive for thyroid transcription factor-1 (TTF-1), epithelial membrane antigen (EMA) and pan-cytokeratin, whereas polygonal stromal cells were immunopositive for neuron-specific enolase (NSE) and S-100 protein as well as EMA. These findings are consistent with a sclerosing hemangioma of the lung (Figure ). Ki-67 index was less than 5%. Both significant MSI evaluated by PCR amplification and loss of expression of MMR-proteins MLH1, MSH2, MSH6 and PMS2 determined by immunohistochemistry could not be detected in the pulmonary SH. Moreover, all lymph nodes sampled were free of metastases.
By thoracic computed tomography, the pulmonary lesions in the right upper lobe remained unchanged after 3 months. According to interdisciplinary tumor board recommendations and oncological guidelines [-] we decided not to suggest further chemotherapy or restorative proctocolectomy but to perform careful aftercare with monitoring of the pulmonary lesions at close intervals as well as attentive follow-up via abdominal ultrasound and colonoscopy.
Furthermore, the patient's family members were referred to cancer genetics specialists for counseling interviews and recommended germline mutation analysis. During regular follow up visits CEA and CA 19-9 were within normal range. Accurate colonoscopy and diagnostic imaging of liver and lungs were unremarkable, in particular pulmonary lesions of the right upper lobe both were not identifiable any more.


A 4 year-old, previously healthy boy was admitted to the surgical unit of the Colombo South Teaching Hospital, Sri Lanka with an abscess in the left big toe. No fever or local lymphadenopathy was present at initial presentation. The abscess was drained, treated with antibiotics and the child was discharged. Two weeks later the child was re-admitted with an infection at the site of original abscess and left sided inguinal lymphadenopathy. Full blood count (FBC) revealed an eosinophilia of 12.5% (WBC count 19,800, N 40.8%, L 35.9%). Blood picture showed moderate eosinophilia with reactive changes suggestive of either a parasitic infection, or an allergic/drug reaction. A blood sample was sent for the detection of Toxoplasma and Toxocara antibodies to the Medical Research Institute (MRI), Colombo. Empirical treatment was commenced with Diethyl Carbamazine 6 mg/kg/body weight for 14 days and Mebendazole 50 mg twice a day for 3 days (based on body weight of 13 kg) together with intravenous antibiotics. The lymph node enlargement which persisted during the wound infection resolved with treatment and the child was discharged 14 days after admission.
Three months later the child was referred to the Paediatric Unit of the same hospital with reports of the blood sample taken at the time of previous admission indicating positive serology for both Toxoplasma gondii and Toxocara spp (Toxoplasma antibody IgG Negative, IgM Positive and Toxocara antibody IgG Positive). Examination of the child at this instance revealed bilateral cervical (2 cm) and left side inguinal (3 cm) lymph nodes, and non tender hepatomegaly approximately 2 cm from the costal margin. No splenomegaly was noted. There was no history of fever, cough, wheezing or recurrent infections. No weight loss, night sweats or chronic cough suggestive of tuberculosis was recorded. There was no contact history of tuberculosis though intensive questioning of the parents revealed a history of lymphadenopathy due to tuberculosis in the elder sibling, approximately one year before this child was born. The sibling had been treated with the full course of anti-tuberculosis treatment based on the WHO recommendations [] and was healthy thereafter. There was no association with cats or ingestion of undercooked meat though there was a history of close contact with dogs which were not de-wormed.
The patient was re-admitted to the Paediatric Unit. Full blood count and blood picture was repeated and FBC showed a total count of 16,500 with 18.5% eosinophils (N 29%, L 46% M 5.4%). The blood picture was similar to the previous report. Ultrasound Scan abdomen confirmed mild hepatomegaly 3 cm from costal margin but no splenomegaly or para-aortic lymph node enlargement. As toxoplasmosis is generally a self- limiting disease in this age group, the child was treated with high doses of Albendazole (50 mg/kg per day in two divided doses to a maximum dose of 400 mg daily for 5 days) for toxocariasis.
Mantoux test was positive, suggesting co-existing tuberculosis in this child. Chest x- ray did not show any lesions suggestive of pulmonary tuberculosis. An inguinal lymph node biopsy was taken for histology on the 5th day of treatment with Albendazole. A repeat FBC indicated that the blood counts were within normal range (Total count 10,800, N 47%, L 51%, M 1%, E 1%). However, the lymph node enlargement persisted.
The biopsy report of the left inguinal lymph node which was received two weeks later, indicated central necrosis with numerous tuberculoid type granulomata. Granulomata consisted epithelioid histiocytes. Langerhans type giant cells seen in central caseous necrosis. The appearance was compatible with tuberculous lymphadenitis with no evidence of Toxoplasma or Toxocara in the lymph node sample.
The parents were requested to take the child to the national chest clinic for anti tuberculosis treatment with instructions to report back to the Paediatric clinic with results of antibody tests for toxoplasmosis and toxocariasis both in the mother and child and an HIV Profile of the child. Acute Toxoplasma and Toxocara infection was confirmed in the child with positive anti Toxoplasma IgM and IgG antibodies and a four-fold rise in the IgG titre for toxocariasis as compared to the results taken 3 months previously justifying the treatment for toxocariasis. The HIV screening was negative. All test results were negative in the mother. The parents were requested to repeat the tests 6 months after completion of treatment and advice given to prevent re-infection of zoonotic parasitic diseases. They were also educated about tuberculosis and the importance of completing the full course of treatment.


A 17-year old girl was referred with widespread EN (Figure A+B). She was otherwise healthy apart from a tendency to back pain. Her parents recalled the first appearance of the EN when she was 4 months old. The EN initially presented as hyperpigmented linear streaks which gradually increased in size and thickness, becoming more elevated and verrucous. The brown, papillomatous and velvety EN followed the lines of Blaschko, with streaks and whorls on her body stopping abruptly at the ventral midline. The EN extended to her neck, scalp and extremities and was present on her face (Figure ). She had intraoral mammilated lesions inside her lower lip (Figure ) and at the buccal mucosa close to her oral angles. Laterally at the hard palate she had cobblestone-like thickening of the mucosa. She did not show any dysmorphic features and her face, trunk and extremities appeared symmetric with normal proportions, although a radiologic examination of the spine revealed a minimal thoracic scoliosis of 5 degrees. An eye examination was unremarkable and neurological examination was normal.
After informed consent of the patient and her parents, a 4 mm punch biopsy was taken from the chest. On histological examination, the biopsy showed a slightly papillomatous surface with non-specific laminated hyperkeratosis and acanthosis, typical of EN.
The patient was diagnosed with a bilateral, systemic keratinocytic nevus of the non-epidermolytic subtype. Maceration in the intertriginous areas was troublesome, but repeated laser (carbondioxide and Nd:YAG) treatments were without great success, as the skin lesions either relapsed or formed disfiguring scars.


A 54-year-old ex-smoker presented with an abnormal chest radiograph. He was asymptomatic had a normal physical examination, and the radiograph was taken as part of a license application. CT scan revealed a well-circumscribed lesion in the right lower paratracheal region abutting the azgos vein. EBUS-TBNA was performed using the 7.5 MHz convex probe bronchoscope (BF-UC260F; Olympus Ltd, Tokyo, Japan). The lesion was identified as a round, anechoic structure and distinguished from surrounding blood vessels using color Doppler (). A dedicated 22-gauge needle (NA-202, Olympus Ltd, Tokyo, Japan) was used to puncture and aspirate the lesion under direct visual guidance. To ensure complete drainage of the lesion, the gradual shrinkage of the cyst was observed on ultrasound while 50 milliliters of serous fluid was aspirated (). Cytological examination of the aspirate yielded metaplastic squamous cells with negative microbiological cultures. He has remained clinically and radiologically stable on followup for 18 months.


An 18-year-old male presented with an abnormal chest radiograph that was performed before military enlistment. He was also asymptomatic and had a normal physical examination. CT scan revealed a mass in the right hilum. EBUS-TBNA was performed and ultrasound identified a round lesion with an echogenic centre and thickened, hyperechoic walls (). Ten milliliters of purulent aspirate was drained. Cytological examination identified numerous neutrophils, occasional squamous cells and an amorphous granular background. Bacterial cultures grew Haemophilus influenza. He was treated with antibiotics and referred for surgical resection. A lobulated mass at the root of the right upper lobe with a cystic cavity was found on right thoracotomy. Histopathological examination revealed a well-defined cystic space lined by inflamed respiratory epithelium consistent with the diagnosis of an intrapulmonary bronchogenic cyst.


A 54-year-old Arab woman presented with a four-month history of epigastralgia and anemia. The endoscopic findings of an examination of the patient's stomach revealed an ulcerative tumor of the fundus, from which the guided biopsy yielded a spindle cell neoplasm with positive immunohistochemical staining using monoclonal antibodies against CD117 (c-kit). The morphologic (Figure ) and immunohistochemical features of the tumor were consistent with GIST. The molecular biology examination was not done. The abdominal computed tomography scan revealed a heterogeneous mass of the gastric lesser curvature measuring 11 cm × 9 cm × 7.5 cm (Figure ). The tumor was unresectable; hence IM was indicated at the dose of 400 mg/day. The patient received six months of treatment with IM, which was well tolerated. A radiology-based major response was obtained, thus making the tumor resectable (Figure ). Fluorodeoxyglucose positron emission tomography (FDG-PET) has proved to be highly sensitive in the early assessment of tumor response. Unfortunately, the patient did not undergo FDG-PET, because it is not available in Morocco. The patient received a partial gastrectomy. The margins of the gastric resection were free of neoplasm.
No gastric cancer cells were detected in the resected specimen (Figure ). The patient received one year of IM therapy after the surgery. The patient was in complete remission nine months after the end of IM therapy.


A 20-year-old female patient reported to our institute with a swelling on the zygomatic area on the right side of the face. The swelling was noticed 10 years ago, which remained asymptomatic and had gradually increased to the present size of 6 cm in diameter causing asymmetry of the face []. Another bony hard swelling was also seen in relation to the left mandible extending laterally to the symphysis area []. In both swellings, the color of the surface of the skin was normal and not attached to underlying tissue. There was no local raise in temperature over the swelling.
The intraoral examination in relation to the maxillary right quadrant revealed a bony hard swelling involving the buccal [] and palatal aspects of the canine area, extending up to the maxillary tuberosity. The maxillary left quadrant revealed a bony hard swelling involving the palatal aspect of the premolar extending up to the molar region. The mandibular left quadrant revealed a hard swelling leading to obliteration of the vestibular sulcus from the canine to the first molar region [].
The orthopantomograph of the maxillary right quadrant revealed mixed, radiopaque [ black arrows] and radiolucent areas [ blue arrows] with diffuse, ill-defined margins obliterating the maxillary sinus, extending from the infraorbital region to the alveolar region. The medial aspect revealed opacity extending into the nasal cavity and laterally extending to the zygomatic arch and posteriorly up to the maxillary tuberosity.
The maxillary left quadrant revealed an impacted second premolar [ orange arrow] present at the apex of the first premolar and radiopacity [ black arrows] was seen surrounding the impacted premolar. The mandibular left quadrant showed radiopacity [ black arrows] in relation to apical areas of the mesial and distal roots of the retained deciduous second molar. In addition, the mandibular left quadrant demonstrated a unilocular radiolucent area with sclerotic margin in relation to the premolar and molar regions extending to the crown of the impacted second premolar at the lower border of the mandible [ orange arrows]. The mandibular right quadrant demonstrated unilocular radiolucency in relation to the molar area [ blue arrow]. The complete blood picture and serum alkaline phosphatase was performed and was found to be within the normal range.
On the basis of clinical and radiological findings, dentigerous cyst was suspected involving the impacted 35. Incisional biopsy was performed on the maxillary right quadrant and provisional diagnosis of cemento-ossifying fibroma was given. Surgery was planned and performed for all three quadrants under general anesthesia.
The surgical specimen received showed multiple bits of tissue that were white in color with a gritty surface. It was hard in consistency in relation to the maxillary right quadrant [], maxillary left quadrant [] and mandibular left quadrant []. In addition to this, an impacted permanent second premolar was seen in relation to the maxillary and mandibular left quadrant. The mandibular left quadrant contained soft tissue, which was firm in consistency, along with a retained deciduous second molar. In case of the retained deciduous molar, the radiographic picture of fused radiopacities at the mesial and distal root apex was not evident as the retained tooth was devoid of such attachments and the roots of the tooth were well defined, as shown in the inset of .
Microscopic features in relation to the maxillary right quadrant exhibited trabeculae of immature bone and irregular basophilic cemental masses in a moderately cellular fibrous connective tissue stroma []. The periphery of the section exhibits normal vital bone. There is no connective tissue separating the immature trabeculae from the normal bone. The cemental masses appear to be fusing with the trabeculae of the bone and with themselves to form larger masses [].
The decalcified tissue section in relation to the maxillary left quadrant showed trabeculae of immature bone and irregular basophilic cemental masses in a highly cellular fibrous connective tissue stroma exhibiting whorling around the cemental masses and the immature bone []. The immature bone appears to be curvilinear, resembling ginger roots. Cemental masses appear to be fusing with trabeculae of the bone and with themselves to form larger masses [].
The mandibular left quadrant showed a cyst-like area with irregular basophilic cemental masses in a background of less-cellular fibrous connective tissue []. The cystic lumen is lined by non-keratinized odontogenic epithelium resembling reduced enamel epithelium [inset; ]. The epithelium shows proliferation, with the underlying connective tissue being infiltrated with lymphocytes and plasma cells. Other areas showed fibrous connective tissue stroma with cemental masses [].


A 20-year-old Indian woman who was an unbooked primigravida presented as an emergency at 27 weeks of gestation with generalised tonic clonic seizures over a six hour period. She had been unconsciousness for one hour. She was referred by a general practitioner with a diagnosis of eclampsia. There was no past history of seizures and she was not on any medication. On examination, she was in a grade III coma and her pulse and blood pressure were 92 beats/min and 130/90 mmHg, respectively. Her urine albumin was +1 and the liver function, renal function and fundus examinations were normal. An ultrasound showed a live 26-week-size fetus with spina bifida. An emergency MRI of our patient's head showed a 4-5 mm spherical ring-enhancing lesion in the frontal region of her brain. A diagnosis of neurocysticercosis was made on the basis of the MRI finding, symptoms and living in an endemic area. She was put on intravenous phenytoin and the pregnancy was terminated in view of the fetus' spina bifida with misoprostol. She aborted after 16 hours and regained consciousness after 24 hours: she was discharged on albendazole and phenytoin.


A 24-year-old Indian woman who was a booked primigravida presented at 24 weeks of gestation with a history of one generalized tonic clonic seizure 30 minutes before. She had no past history of convulsion. On examination she was conscious, alert and had a blood pressure of 140/100 mmHg. There was +1 albuminuria. Fundus, renal and liver function tests were normal. She became normotensive after 24 hours. An MRI of her head reported a single cystic enhanced lesion in the parietal lobe of the brain (Figure ). A diagnosis of neurocysticercosis was made on the basis of the MRI, convulsion and living in an endemic area. She was put on carbamazepine and discharged from the hospital after two days. She was followed-up regularly throughout the pregnancy. The remainder of her antenatal period was uneventful but, at term, she had an emergency Cesarean section due to fetal distress. Her postnatal period was uneventful and she was discharged in good condition with a healthy baby.


A 60-year-old male patient reported with a chief complaint of inability to swallow food since 6 months. He had an history of smoking tobacco”; 4th Line “h/o” as “history of since 20 years. Patient’s previous medical records disclosed h/o of nasal obstruction on and off and anosmia and histopathological diagnosis of previous biopsy was given as round cell tumor by a private lab and as tissue insufficient for reporting by TATA memorial hospital.
On general examination, patient’s health was poor, pallor was positive (++) and his body weight was 35 kgs (ht 155cms). He had trismus and on intraoral examination with tongue depressor, oral hygiene was poor and large defect was seen in the secondary hard and soft palate with massive destruction of the tissues. The palatal bone was denuded of its mucosal covering and was seen exposed on the left side of the cleft like defect. Ulcer was foul smelling and oroantral fistula draining pus was also present. Ryles tube feeding was started to maintain nutrition.
Routine blood and urine investigations were carried out. Erythrocyte sedimentation rate (by Westerngren method) was 20 mm/1 hr (normal 0-9 mm/1 hr), hemoglobin level was 8.7 g/dl (normal 13-17 g/dl), TLC and DLC and platelet count were within normal limits. In peripheral blood smear- RBCs showed mild anisocytosis with hypochromia, microcytes (positive), few macrocytes were seen, WBC series showed neutrophilia, toxic granules were seen in neutrophils, platelets were adequate and parasites were absent. Plasma glucose level and serum sodium, potassium, urea, creatinine were within normal limits. With a presumptive diagnosis of either an infective or neoplastic lesion biopsy was done and histopathologically evaluated. On microscopic examination biopsy specimen showed necrotic tissue only without infective organisms or neoplasia and deeper biopsy was advised.
VDRL/RPR test, HIV- Tridot test were nonreactive and Hbs Ag was negative. AFB/ZN staining for acid-fast bacilli was negative. Ultrasonographic findings of abdomen and pelvis and chest X-ray findings were within normal limit. Skin smears from right and left ear lobes and dorsal aspect of right middle finger showed no evidence of Hansen’s disease.
X-ray PNS showed mucosal thickening in right maxillary sinus and both the frontal sinuses appeared normal. X-ray nasopharynx showed anterior osteophytes in the cervical vertebrae suggestive of cervical spondylosis.
Negative microbiological findings ruled out infectious nature of the lesion. Syphilis was excluded by serology. Normal chest radiography and absence of renal involvement ruled out diagnosis of Wegener’s granulomatosis.
On microscopic examination, hematoxylin and eosin-stained sections from deeper biopsy specimen revealed stratified squamous epithelium, which was hyperplastic.
Underlying connective tissue stroma revealed lymphoproliferative process characterized by an extremely polymorphous picture; it largely consisted of a mixture of atypical large and small lymphoid cells, along with plasma cells, histiocytes and occasional eosinophils. The large lymphoid cell component was predominant. The large lymphoid cells had hyperchromatic nuclei. The neoplastic cells showed a tendency to be angiocentric with focal areas of vascular destruction [Figures and ]. Invasion of vascular walls and occlusion of lumina by lymphoid cells (angiodestructive) with varying degrees of cytologic atypia were also noted. The vascular occlusion was associated with prominent necrosis of both tumor cells and normal tissue []. No granulomatous lesions or giant cells were found.
Histological features of invasion of vascular walls of normal tissue by the neoplastic cells and widespread necrosis ruled out diagnosis of midline idiopathic destructive disease. Sections of tissue specimen were subjected for immunohistochemical evaluation. On immunohistochemical analysis tumor cells were strongly positive for leukocyte common antigen (LCA) and negative for CD20 and CD30 and CD3 was equivocal.
Based on clinical findings of localized destruction of the soft and hard tissues confined to the nose and paranasal sinuses, histological features of angiocentric distribution, angiodestructive nature of neoplastic cells and associated necrosis of the normal tissue and the tumor cells, the presence of immunoreactivity with markers indicative of a neoplasm of lymphoreticular origin (LCA, CD3) and absence of renal and pulmonary involvement the diagnosis of “Extranodal NHL -Nasal type” (angiocentricity) was established.
Patient was referred to the Oncology department for management. Bilateral superior deep cervical lymph nodes were palpable. Clinical staging of the lesion was done as T4 N1-2 M0. Since the destructive process was widespread; chemotherapy was planned for the patient. Treatment of the lesion was planned with six cycles CHOP (Cyclophosphomide, doxorubicin, vincristine and prednisone) chemotherapy.
As the patient was poor, treatment was started with Cyclophosphamide inj 500 mg i.v. slow, Vincristine inj 2 mg i.v. slow, weekly for 4 weeks, tab prednisone 120 mgday 1 to day 5(D1-D5). Whole cycle was to be repeated after 28 days. If general condition of the patient improves addition of inj. doxorubicin was planned. But after the first cycle of chemotherapy patient died of clinical complications.


An 87-year-old Caucasian British woman presented to our emergency department with a three-day history of shortness of breath, pyrexia and non-productive cough. Her only significant past medical history was well controlled hypertension. She was independent in her daily activities, did not smoke cigarettes and reported a good cardiorespiratory reserve prior to the onset of symptoms. The diagnosis of community-acquired multilobar pneumonia was made with a CURB-65 score of three. She was admitted to the high dependency unit with type 1 respiratory failure and a high alveolar-arterial oxygen gradient. She received intravenous antibiotics (piperacillin/tazobactam and erythromycin) and non-invasive high-flow continuous positive airway pressure (CPAP). By the fourth day, our patient had deteriorated, with a chest radiograph showing bilateral alveolar and interstitial infiltrates and a ratio of partial pressure of arterial oxygen to the fraction of inspired oxygen (PaO2/FiO2) of < 26.6 kPa, which required invasive ventilation. The criteria for ARDS were met and the cause was thought to be a combination of the direct pneumonic pulmonary injury and extrapulmonary severe sepsis. With protective lung ventilation, low dose methylprednisolone, antibiotic therapy and a negative fluid balance, gradual improvement was made; over the following five days respiratory parameters weaned sufficiently to allow an uncomplicated percutaneous tracheostomy to be performed. On day 10, a period of desaturation required recruitment maneuvers with a Mapleson C circuit that resulted in notable surgical emphysema. The cause was thought to be a tracheal injury sustained at the time of tracheostomy insertion. An adjustable flange tube was positioned under bronchscopic guidance just proximal to the carina in an attempt to limit any further tracking of air through the potential tracheal defect. Despite these measures, a high alveolar-arterial oxygen gradient and peak airway pressures persisted. A chest radiograph showed more homogenous central pulmonary alveolar shadowing. An upper airway bronchoscopy showed no obvious tracheal wall injury and computed tomography (CT) of her chest showed extensive surgical emphysema and a small anterior left sided pneumothorax. On further review of the CT scan, it was felt that the perivascular and peribronchial emphysema was consistent with a diagnosis of pulmonary interstitial emphysema (Figure ). Over the following days, despite protective ventilatory strategies and intercostal tube thoracostomy, lung compliance along with oxygenation deteriorated. By day 13, the deteriorating respiratory parameters along with inotropic requirements resulted in a decision to limit therapy and the patient died on day 14.


A 32-year-old man was admitted to our department complaining of left chest pain and cough. Chest radiography showed a large round opacity (10 × 10 cm) of the left hilum (Figure ). The electrocardiogram was normal. Computed tomography (CT) scan demonstrated a large cystic mass arising from the pericardium, adjacent to the left pulmonary pericardial sinus (Figure ); no other abnormalities were observed.
Resection of the cystic mass was effected by VATS, with three-port approach on the left side. At thoracoscopy a left upper pericardial defect (3 × 4 cm), oval-shaped, was found. A large cyst was identified, arising from the upper border of the PD. The cyst was adherent to the left main pulmonary artery and to the visceral pleura of the left lung upper lobe (Figure ). After needle aspiration of part of the dense fluid content of the cyst, the latter was dissected from adhesions to the lung and to the upper border of the pericardial defect, using the harmonic scalpel. The cyst was radically resected with minimal blood loss and without complications. The left atrial appendage was partly bulging from the pericardial defect, but without herniation. Therefore the defect was left untreated. Pathology of the resected specimen revealed a bronchogenic cyst (Figure ). The postoperative course was uneventful. Cardiac function was monitored postoperatively by transthoracic echocardiography, which demonstrated no cardiac herniation, and the patient was discharged on the 5th postoperative day. At 18-month follow-up the patient was asymptomatic; cardiac magnetic resonance imaging and transthoracic echocardiography demonstrated no cardiac herniation nor functional deficiency, also when the patient was examined in the left lateral decubitus position [].


An 89-year-old man presented with a history of episodes of a paroxysmal headache on the right occipital region, superior and posterior to the ear, over the past 40 years. He complained of abrupt onset, sharp, electric-like pain in the right occiput radiating toward the vertex accompanied by an intense and stabbing pain localized in the depth of the auditory canal. Palpation of the right greater occipital nerve elicited the pain. A single pain episode usually lasted for a few weeks. The pain in the depth of the right auditory canal did not appear during every episode but only when the occipital pain was severe. The pain attacks usually occurred at the time the patient felt fatigued or when he was afflicted by a cold with or without fever. During the latest episode, the occipital pain was accompanied by symptoms of a herpes zoster and/or herpes simplex reactivation in the territories of the right maxillary and auriculotemporal nerves. Specifically, the skin was erythematous and small vesicles appeared on the right side of the face, around the right mouth angle and the right external auditory canal. The viral smear was not obtained and the vesicles resolved spontaneously in weeks. The frequency of the pain attacks was increasing from once in every 1 or 2 years in the antecedent decade to two or three times per year in recent years. Neurological examination revealed normal findings with no hypesthesia in the affected area, no cranial autonomic features, and no occipital skin lesions. Pain was eased temporarily by local anesthetic nerve block with lidocaine. Magnetic resonance image (MRI) examination of the cervical spine and brain was normal. A diagnosis of ON in combination with NIN was established based on symptoms which fulfilled the International Headache Society (ICHD-II) criteria []. In the antecedent decade, the patient did not take any pain killer. In recent years, carbamazepine was used at a dosage of 0.3 g daily (divided into three times) during the pain attacks and the intensity and frequency of the pain attacks could be eased, but the episodic duration of pain attacks was not reduced.


A 48-year-old woman was admitted to our hospital with diagnosis of subacute thrombosis of the right external iliac and common femoral artery, following HILP for recurrence of right limb melanoma. HILP was performed 3 weeks prior: common right femoral artery and vein were isolated with open technique, heparin was given, and then the vessels were cannulated and connected to a perfusion circuit providing membrane oxygenation and a heat exchanger (perfusate temperature 38.5–40°C). Melphalan was given in a dose of 10 mg/L of tissue volume and human recombinant TNF-α in a dose of 2 mg. A duplex ultrasound of the right iliac and femoral vessels performed preoperatively to assess vessel size documented good quality vessels and, of note, the absence of peripheral vascular disease (a contraindication to HILP). Several days after, another duplex scan was obtained, as postprocedural routine, to document regular vessels patency but, unexpectedly, it showed a postocclusive demodulated duplex signal, with the femoro-popliteal-tibial axis reperfused by collateral circulation, being the patient asymptomatic for claudicatio or limb pain and with a slight clinical evidence of limb ischemia. We carried out a multidetector 64-slice CT angiography (collimation 64 mm × 0.625 mm; tube current 300 mA, tube voltage 120 Kv) after injection of two intravenous bolus of contrast dye (Iomeron 350 mg/mL; first bolus 60 mL volume, 4 mL/s flow; second bolus 40 mL volume, 2 mL/sec flow). The evaluation was completed by volume rendering and multiplanar reconstructions (MPR). As shown in , the external iliac artery was completely occluded by a thrombus and reperfusion flow was appreciated in the distal common femoral artery and in profunda femoris by collateral circulation. The patient underwent surgical intervention consisting in iliac artery-femoral artery bypass with a Dacron vascular graft 8 mm in caliper. Postprocedural hospital stay was uneventful, and she was discharged home on day 11.


A 26-year-old man was admitted to our department for sonographic evaluation of the scrotum due to infertility. On physical examination, both testes were intrascrotal, normal sized, there was no palpable lesion, and no clinical evidence of varicocele. A sperm count revealed an oligospermia (sperm count: 9400000/mL). FSH level was in the upper limit of normal. The α-fetoprotein and human chorionic gonadotropin levels were within normal limits. Gray-scale images showed a well circumscribed, 5.7 mm sized hypoechoic round lesion within the left testis (). Color Doppler US demonstrated that the lesion consisted almost entirely of multiple tortuous enlarged vessels (). There were 2 dilated vessels on both sides of the vascular mass. One of them was arterial in nature and displayed high flow velocities with a peak systolic velocity of 20.3 cm/s and end diastolic velocity of 13.6 cm/s (). The resistive index was 0.33 and pulsatility index was 0.40. The vessel on the other end showed pulsatile venous flow () on spectral Doppler US. Based on the gray-scale and color Doppler US examinations, an arteriovenous malformation of the testis was diagnosed. US findings were not correlated to the patient's fertility status. The patient refused surgery for the mass. Because of the small size of the vascular mass, invasiveness, and risk of radiation, no angiographic imaging and endovascular interventional procedure were performed. During the followup period, there were no changes in patient's clinical and radiological findings.


A previously healthy 66-year-old Caucasian woman presented with a 24-hour history of sudden onset painful right-sided groin swelling. On clinical examination, there was a fixed, round, tender mass about 5 × 3 cm in size in the right groin, above the inguinal crease. Her temperature was 38.7°C and she did not appear to be in distress. She did not have any bowel obstruction revealed by clinical examination or on the abdominal X-ray. Her past medical history was insignificant. Her laboratory findings were within normal limits except an increased WBC count (13.00 K/μL) with 80% neutrophils.
A presumptive diagnosis of a chronically incarcerated femoral or inguinal hernia versus a strangulated hernia or an inguinal abscess was made with plans for a right groin exploration using a more curved low inguinal incision under general anesthesia (Figure ). When the hernia sac was opened, an inflamed appendix was seen. The appendix was thickened and inflamed, but there was no perforation. Intraoperative findings were consistent with an inflamed and gangrenous appendix protruding through the femoral hernial sac (Figure ). Routine appendectomy was performed through the hernial sac. The mouth of the hernia was wide and the senior surgeon was even able to pass a finger through the hernia into the peritoneal cavity. The hernial sac was closed using a V-shaped polypropylene mesh. A broad-spectrum antibiotic cover was provided at induction. The postoperative course was uneventful and the patient was discharged home on the third day after the procedure. The histological examination was consistent with acute appendicitis.


A 36-year-old Caucasian man with a history of hypertension, depression, bipolar disorder and two past suicide attempts was brought to our emergency department two hours following voluntary ingestion of a total estimated amount of 10 g of atenolol in association with an unknown amount of sertraline, nifedipine, Lacidipine and fluoxetinee.
Upon arrival, he presented with a decreased level of consciousness, shortness of breath, hypoxemia (arterial oxygen saturation, 91%) and hypotension (blood pressure (BP), 80/40 mmHg; heart rate (HR), 80 beats/minute). A 12-lead electrocardiogram (ECG) showed normal sinus rhythm, PR interval 168 milliseconds, prolonged corrected QT time 448 milliseconds and QRS widening 133 milliseconds. The results of his routine laboratory tests, including glucose, an electrolyte panel, liver function tests and coagulation were normal, with the exception of mild renal impairment (blood urea nitrogen 46 mg/100 mL, creatinine 2.2 mg/100 mL).
Briefly afterward the patient's condition suddenly deteriorated, and he experienced cardiac arrest. He was resuscitated according to advanced cardiac life support (ACLS) protocols, and an effective cardiac rhythm was regained 30 minutes following the cardiac arrest. However, he had severe hemodynamic instability, persistent metabolic acidosis and shock (BP 70/40 mmHg, HR 80 beats/minute), despite adequate fluid resuscitation, the administration of 100 mEq sodium bicarbonate IV bolus, very high-dose dopamine (50 μg/kg/minute) and epinephrine (2.5 μg/kg/minute) infusion.
At the same time, we started specific medical management for BB and CCB intoxication, including gastric lavage followed by gastric administration of 40 g of activated charcoal, 40 ml IV bolus injection of calcium chloride, and 5 mg glucagon IV bolus injection performed. Two hours after admission to the emergency department, the patient was transferred to our intensive care unit (ICU).
Upon arrival to the ICU, the patient was unconscious (Glasgow Coma Scale score 3) without sedation and on controlled mechanical ventilation. He had persistent shock with multiple organ failure, refractory metabolic acidosis (pH 7.22, arterial partial pressure of oxygen (PaO2) 79 mmHg, arterial partial pressure of carbon dioxide 49 mmHg, base excess -7.4, lactate 9.3 mmol/L) and oligo-anuric renal failure.
The specific treatment of his drug overdose was performed as follows: (1) 40 g of activated charcoal administration through a nasogastric tube three times daily for 24 hours, (2) whole bowel irrigation with 2 L of polyethylene glycol, (3) calcium chloride continuous IV infusion to provide a stable slightly supra-normal serum ionized calcium level (2 mmol/L), (4) glucagon continuous IV infusion at a rate of 5 mg/hour for 20 hours following the first 5 mg loading dose, (5) high-dose insulin and glucose continuous IV infusions (0.7 to 1.5 IU/kg/hour and 50 to 100 g/hour, respectively) and (6) single plasma exchange treatment followed by three days of high-volume continuous veno-venous hemofiltration (HV-CVVH) at a 70 to 80 mL/kg/hour ultra-filtration rate.
His plasma levels of anti-psychotic medications and CCB were reduced by medical treatment and plasma exchange. His atenolol level continued to rise for six to eight hours after toxic ingestion and then started to decrease with the initiation of HV-CVVH. His atenolol plasma level normalized after 72 hours, at which point the ultra-filtration rate was reduced to 40 mL/kg/hour. The efficacy of atenolol removal by HV-CVVH can be assessed on the basis of the trends in plasma and ultra-filtrate levels outlined in Table .
The patient's cardiac insufficiency required the insertion of a pulmonary artery catheter to guide hemodynamic optimization, increasing the infusion of inotropes and vasopressors up to extremely high-dose dopamine (300 μg/kg/minute) and subsequent epinephrine (15 μg/kg/minute) and vasopressin (0.03 IU/minute) continuous infusion, which still did not achieve satisfactory hemodynamic stability or adequate peripheral tissue perfusion (BP 80/40 mmHg, HR85 beats/minute, cardiac output (CO) 4.2 L/minute, mixed venous oxygen saturation (SvO2) 61%, pH 7.18, and lactate 8 mmol/L). An echocardiogram confirmed global cardiac hypokinesis and depressed left ventricular function.
The patient's young age, relative lack of pre-existing co-morbidities, potassium levels and failure to accomplish satisfactory correction of tissue acidosis led us to consider extra-corporeal cardiopulmonary support. Two hours after admission extra-corporeal cardiopulmonary support (Jostra RotaFlow Centrifugal Pump; Maquet, Jostra Medizintechnik AG, Hirrlingen, Germany) was started through percutaneous femoral artery and vein cannulation. Extra-corporeal blood flow was initially set at 4 L/minute, and gas flow through an artificial lung was initiated at 2 L/minute of oxygen.
After starting ECMO, vasopressin was discontinued and both dopamine and epinephrine administration were quickly tapered (Table ). His clinical signs of tissue perfusion improved, minimal spontaneous diuresis resumed and lactic acidosis was corrected. After 24 hours of combined ECMO and HV-CVVH treatment, his lactate levels had decreased to near physiological levels (< 2 mmol/L). Specific β-agonist cathecolamines, such as isoproterenol and dobutamine, were added to epinephrine while dopamine was replaced with a more specific vasoconstrictor, such as norepinephrine.
Unexpectedly, the patient's severe myocardial impairment was never associated with rhythm disturbance. Serial 12-lead ECGs revealed moderate sinus bradycardia, but neither atrio-ventricular blocks nor QRS widening nor cQT interval prolongation was detected, except on the admission ECG. Therefore, there was no indication for cardiac pacing.
After 24 hours, a test of extra-corporeal blood flow reduction revealed persistent cardiac insufficiency complicated by pulmonary edema (BP 85/40 mmHg, CO 3.9 L/min, pulmonary capillary wedge pressure 21 mmHg, SvO2 63% and ratio of PaO2 to fraction of inspired O2 < 150).
The patient was weaned from ECMO 48 hours later after a successful flow reduction test. After by-pass interruption, inotropes and vasopressors were progressively tapered and discontinued completely on day 6, when his hemodynamic parameters were completely within normal range (Figure and Table ).
The second clinically relevant problem was the extremely low potassium plasma level on admission (< 0.8 mEq/L). Potassium was supplemented by high-dose potassium chloride continuous IV infusion at a rate of 30 to 35 mEq/hour without successful response. Once the by-pass was started, the effects of potential sudden cardiac death due to arrhythmia secondary to electrolyte derangement was reduced, thus the IV infusion was quickly tapered to 10 to 15 mEq/hour without cardiac rhythm disturbance. The plasma level of potassium normalized only about 12 hours later. Transient hyperkalemia was also noted with no ECG abnormalities.
We also observed progressive rhabdomyolysis (myoglobinuria, creatine phosphokinase 24,169 IU/mL and myoglobinemia 14,444 IU/mL) and some skin sub-ischemic injuries on the right gluteus and second and third left toes. The patient's rhabdomyolysis was managed with high fluid washout and to some extent by HF-CVVH. His external lesions were dressed daily. All complications resolved without sequelae.
Moderate neurological impairment progressively developed as a result of the initial anoxic insult. Basal brain CT and magnetic resonance imaging scans showed bilateral ischemic dyencephalic lesions. Serial electroencephalograms confirmed depressed cortical activity with predominant bilateral theta-delta waves.
An echocardiogram obtained three days before the patient's discharge from the ICU showed a light left ventricular hypertrophy, normokinesis and a preserved ejection fraction (58%). The patient was transferred from the ICU on day 28 without cardiac, renal or pulmonary sequelae.
One year after his discharge from the ICU the patient was not self-sufficient and therefore was living in a long-term care facility. Although his neurologic performance overall had improved over time, he still has reduced motor skills and impaired coordination, gait ataxia and mild aphasia.


A 54-year-old Caucasian man with a 14-year history of Parkinson's disease (PD) was scheduled for DBS of the sub-thalamic nucleus (STN) bilaterally. His anti-parkinsonian medication consisted of 600 mg/day levodopa, 125 mg/day carbidopa, 1200 mg/day entacapone, and 0.54 mg/day pramipexole. This regimen was discontinued 18 hours prior to the DBS procedure, according to our standard protocol, to avoid medication-induced dyskinesias during surgery and to allow for the patient to be in an off-state, thus maximizing the clinical information gained by intra-operative stimulation []. The procedure itself was uneventful, with implantation of the DBS electrodes in the STN bilaterally (Medtronic 3389 electrodes; Medtronic, Minneapolis, MN, USA). Three hours post-operatively the patient developed tremor, muscle rigidity, and high fever resistant to common anti-pyretic drugs (paracetamol 1 g). The tremor and rigidity were attributed to PD and 200 mg levodopa three times daily was administered through a nasogastric tube. Systemic and central nervous system infection were also considered in the differential diagnosis; however, cerebrospinal fluid analysis, a chest X-ray, and blood and urine cultures were all within normal limits. A subsequent brain magnetic resonance imaging scan depicted no intracranial pathology and confirmed optimal lead placement. Twelve hours postoperatively the patient's PD features worsened: He developed severe axial and appendicular rigidity, coarse resting tremor, and prolonged spasms of the extremities.
His temperature had risen to 40°C, and his blood pressure had increased to 165/94 mmHg. A new laboratory investigation showed leukocytosis (leukocyte count, 19.4 × 109/L with a shift to the left (neutrophil count, 167 × 109/l). His serum levels of creatine kinase (CK) were markedly elevated to 1500 U/l with a normal CK-MB fraction, and his cardiac troponin levels were normal, indicating that the CK elevation was not of cardiac origin. At that point, the diagnosis of NMS was established on the basis of the clinical examination and the laboratory findings. Malignant hyperthermia was excluded after a negative caffeine-halothane contracture test []. The patient was intubated and transferred to the intensive care unit (ICU). Treatment by intravenous administration of 3 mg/kg/day dantrolene, 600 mg/day levodopa, and 60 mg apomorphine was initiated. After copious ICU treatment, the patient was extubated on the ninth post-operative day. Fifteen days post-operatively he still appeared lethargic and confused. The DBS device was consequently activated on the 20th post-operative day, as it was considered that it could accelerate the patient's recovery. Indeed, after DBS activation, the patient showed a good recovery pace and was discharged from the hospital on the 32nd post-operative day. At his six-month follow-up examination, he demonstrated an overall improvement of 15% in the Unified Parkinson Disease Rating Scale.


A 16-year-old Indian girl presented to our hospital with swelling on the scalp. She was born with a black-pigmented triangular patch over the scalp covered with hair as well as multiple black hairy patches on her extremities, back, and most of her anterior trunk. The patch on the scalp had increased in size over a period of eight years after birth. Within the past three months, swelling had progressed from the hairline to just above the left brow, leading to unilateral mechanical ptosis. Upon applying pressure, there was a scanty, yellowish, non-foul-smelling discharge that was occasionally blood-stained and was associated with intense itching without any pain. Her sleep, appetite, bowel, and bladder were unaltered. Her menarche had begun at the age of 15 years, and her menstrual cycle was normal and regular. Her birth and developmental history were normal. The family history was negative for congenital nevi or melanoma.
The patient's clinical examination revealed her to be afebrile, with a pulse rate of 64 beats/minute, a respiratory rate of 16 breaths/minute, and blood pressure of 120/70 mmHg. The examination of her cardiovascular system was normal. Her cranial nerve functions were normal, and no neurological deficit was found in the limbs. The plantar response in both the limbs was flexor. There was no evidence of meningeal irritation.
A local examination revealed a single swelling of 10 cm × 8 cm on the left side of the scalp. The swelling extended anteroposteriorly from 3 cm in front of the left parietal eminence to just above the lateral part of the left supraorbital ridge. The transverse extension was from the line passing through the left parietal eminence to the right of the midline (Figure ). Her local temperature was not raised, and tenderness was absent. The surface was irregular, rough with multiple pits, and the margins were irregular with rounded edges. The swelling was firm in consistency and mobile. The anterior part of the swelling overlying the forehead could be lifted. The underlying skin appeared normal. It was non-pulsatile with no evidence of impulse on coughing. The regional lymph nodes were not palpable. Numerous hairy nevi were present over the scalp, trunk, and limbs.
The patient was referred to the Department of Neuro-ophthalmology and Otolaryngology for further assessment. Her fundoscopy and visual evoked potentials were normal. Her otorhinolaryngological evaluations were normal.
Routine blood investigations showed that her hemoglobin level was 11.6% and her total leukocyte count was 4200/mm3 with relative neutrophilia (80%). Her serum urea and electrolyte levels were normal. A lumbar puncture revealed clear cerebrospinal fluid with a normal cell count and biochemistry and no growth on culture. Her liver and kidney function tests were within normal limits. Her electrocardiogram did not show any abnormal features.
An X-ray of her skull (posteroanterior view) revealed a soft tissue swelling in the left temporofrontoparietal region. Ultrasonography of the swelling showed a large, homogeneous, hypoechoic, solid 9.4 cm × 6.8 cm × 1 cm lesion in her scalp in the left frontal region and partially extending into the parietal region on the left side. The underlying outer table of the skull and diploic spaces were normal with few hyper-reflective areas scattered throughout the lesion and distal shadowing raising clinical suspicions of tiny calcified foci. The interface between the lesion and the scalp was obscured. No significant color flow was noted on a color Doppler ultrasonogram. These findings were highly suggestive of a possible angiofibroma. Power Doppler ultrasonography showed a highly vascularized lesion.
MRI of her brain (both plain and contrast images) was done by using a multi-echo, multi-planar technique, which showed the presence of a 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp (subcutaneous plane within fat) in the left frontoparietal region. The signal intensity appeared isointense to hyperintense on T1-weighted MRI scans with few small tubers with cortical dysplasia in the left frontoparietal region and asymmetric dilatation of the left ventricle that were hypointense on T2-weighted MRI and fluid attenuated inversion recovery (FLAIR) images. Few foci were seen within the lesion, which appeared hyperintense on T2-weighted MRI and FLAIR images. The MRI impression revealed multiple intra-cranial lesions, which included a benign, homogeneously enhancing, subcutaneous scalp lesion in left frontoparietal region suggestive of angiofibroma; a few calcified subependymal nodules within the body of the lateral ventricles on the right side; a few small tubers with cortical dysplasia in the left frontoparietal region; and left maxillary sinusitis (Figure ). These radiological findings were highly suggestive of tuberous sclerosis. Magnetic resonance angiography revealed the absence of involvement of any underlying blood vessels.
Microscopic examination of the patient's scalp swelling revealed the presence of lining keratinized, stratified squamous epithelium and underlying dermis. The epidermis was thinned out with loss of rete pegs. Her dermis showed lobules and nests of nevi cells, hair follicles, sweat glands, and sebaceous glands. There was diffuse deposition of melanin pigment and the presence of melanocytes around and within the hair follicles and sebaceous glands extending up to deep subcutis and infiltrating the fat (Figure ). All of these findings were suggestive of congenital melanocytic nevi.
On correlating these imaging findings with the patient's clinical symptoms, surgery was considered. Excision with primary skin grafting was done (Figure ). The graft was taken from the medial aspect of her right thigh.


A 28 year old Caucasian woman was brought to our emergency department with a five hour history of progressive impairment in consciousness and slurred speech. Her past history was unremarkable, and she neither smoked nor drank alcohol. Her older brother said that she took no medications and she did not use illicit drugs as far as he knew. No history of head trauma was obtained. At the time of admission, her blood pressure was 140/70 mmHg with a pulse rate of 90 beats/minute, respiratory rate of 12 cycles/minute, and a temperature 37.1°C. Our patient was stuporous and there were no lateralizing signs or neck stiffness. Both planter reflexes were down. Our patient underwent a battery of investigations with the following results: hemoglobin 13.6 g/L; total white cell count 9100/mL3; platelets 270,000/mL3; mean corpuscular volume 84fL; mean corpuscular hemoglobin concentration 33 g/dL; erythrocyte sedimentation rate 19 mm/hour; blood urea 35 mg/dL; serum creatinine 0.9 mg/dL; serum sodium 139 mEq/L; serum potassium 4.1 mEq/L; serum calcium 8.9 mg/dL; serum total bilirubin 0.8 mg/dL; aspartate transaminase 21 u/L; alanine transaminase 19 u/L; alkaline phosphatase 190 u/L; serum total protein 7.3 g/dL; serum albumin 4.4 g/dL; thyroid stimulating hormone 2.9 u/L; serum total triiodothyronine 1.3 nmol/L; serum total thyroxin 89 nmol/L; serum total cholesterol 177 mg/dL; serum triglyceride 100 mg/dL; low density lipoprotein cholesterol 128 mg/dL; very low density lipoprotein cholesterol 20 mg/dl; high density lipoprotein cholesterol 38 mg/dl; prothrombin time 12 seconds; activated partial thromboplastin time 31 seconds; a serum Venereal Disease Research Laboratory test was negative; and general urine examination and microscopy were unremarkable. Blood and urinary screening for cocaine, opioids and amphetamines was negative. A 12 lead electrocardiogram (ECG) was normal. A non contrast brain computed tomography (CT) scan showed bilateral hypodensities in her corpus striatum (Figure ). In addition, there was a small hyper dense area at the anterior part of her right globus pallidus. The physician suspected encephalitis, and managed our patient accordingly. He ordered serology for toxoplasma and human immunodeficiency virus, and a lumber puncture was done: all of these tests turned out to be negative. At day four of admission, our patient became comatose, and our neurology department was consulted. The Glasgow coma scale was 3/15, no neck stiffness was detected, and both planter reflexes were up. On day five, a brain magnetic resonance imaging (MRI) scan with gadolinium revealed hemorrhagic infarctions involving both basal ganglia (Figure ). Brain magnetic resonance angiography and magnetic resonance venography (MRV) were normal. Serum anti nuclear and rheumatoid factors as well as anti phospholipid antibodies were negative. Transthoracic and transesophageal echocardiographic examinations were normal, as was the carotid Doppler study.
Our patient was managed as ischemic stroke with secondary hemorrhagic transformation. Anti hypertensives and a statin were prescribed. Anti platelets and anticoagulation were not given. Gradually over a period of three weeks, our patient's consciousness improved to a degree of mild drowsiness. As for her language assessment, comprehension was intact but there was no speech output; she uttered few sounds, however, but no comprehensible words. She had generalized rigidity and hypokinesia. No abnormal movements were found and dystonic posturing was absent.