Search

OBJECTIVE The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

New or preexisting atrial fibrillation (AF) is frequent in patients undergoing aortic valve replacement. We evaluated whether the presence of AF during transcatheter aortic valve implantation (TAVI) or surgical aortic valve replacement (SAVR) impacts the length of stay, healthcare adjusted costs, and inpatient mortality. The median length of stay in the patients with AF increased by 33.3% as compared with those without AF undergoing TAVI and SAVR (5 [3 to 8] days vs 3 [2 to 6] days, p

Calcineurin inhibitors (CNIs) are typically used to prevent organ rejection and their use has significantly improved allograft and survival rates with a marked reduction in rejection rates. However, CNIs have been associated with various side effects including nephrotoxicity, hypertension, gingival hyperplasia, hypertrichosis, hepatotoxicity, hyperkalemia, and neurotoxicity. Significant intra-patient and interpatient pharmacokinetic variability and narrow therapeutic indices make the therapy complicated. Although CNIs are essential in preventing organ rejection, higher doses could lead to toxicity, which can reduce patient tolerability and negatively affect long-term allograft survival and patient mortality. As individual patients respond differently to comparable drug levels, attaining the optimal drug level range does not ensure lack of drug toxicity or complete immunosuppressant viability. One to two adverse effects are commonly observed in patients using CNIs. However, no case about CNI-induced gingival hyperplasia, hypertrichosis, tremors, facial nerve palsy, and blepharospasm after kidney transplantation in a single patient has been reported. Our report describes the unusual case of a patient presenting with CNI-induced multiple adverse reactions.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

This review describes the clinical presentations and treatment options for commonly recognized epilepsy syndromes in the pediatric age group, based on the 2017 International League Against Epilepsy classification. Structural epilepsies that are amenable to surgical intervention are discussed. Lastly, emerging technologies are reviewed that are expanding our knowledge of underlying epilepsy pathologies and will guide future syndromic classification systems including genetic testing and tissue repositories.

Aim Total thyroidectomy, being a common surgical option, for multinodular goitre and selective cases of Graves’ disease, is associated with risks related to temporary hypocalcaemia and vocal quality dysfunction. Dexamethasone, as a steroid, has well-established anti-inflammatory and immune-modulating effects. As researchers continue to understand its pathophysiology on hypocalcaemia and voice quality, we aim to assess the effect of preoperative dexamethasone used to improve hypocalcaemia and vocal cord function/post-thyroidectomy voice disorder. Methods We conducted a single-centre, non-randomised, double-blinded placebo-controlled, parallel-group study with short follow-up in a public sector hospital from January 2014 to December 2019. Patients were equally distributed in two groups to compare the effect of dexamethasone with placebo on hypocalcaemia and vocal cord function/post-thyroidectomy voice disorder Results Out of 192 patients, 36 (18.75) male patients while 156 (81.25) were female. Age range was 18 to 60. Eighteen patients had both low calcium level at 24 hours after the operation and hypocalcemic symptoms, of which 4 remain to have hypocalcaemia on third postoperative day. Forty patients defined as having voice dysfunction at 24 hours after being scored using Voice scale. Of these 40 patients with voice dysfunction, 8 had low calcium level in blood at 24 hours while only 2 of these remained with low calcium level on third postoperative day. 6/8 patients had symptomatic hypocalcaemia on first postoperative day. Conclusions Single-dose dexamethasone, during the preoperative period, is as safe and as effective in improving transient postoperative hypocalcaemia and Temporary vocal quality dysfunction/post-thyroidectomy voice disorder.

Understanding how global brain networks are affected in epilepsy may elucidate the pathogenesis of seizures and its accompanying neurobehavioral comorbidities. We investigated functional changes within neural networks in temporal lobe epilepsy (TLE) using graph theory analysis of resting-state connectivity. Twenty-seven TLE presurgical patients (age 41.0 ± 12.3 years) and 85 age, gender, and handedness equivalent healthy controls (HCs; age 39.7 ± 16.9 years) were enrolled. Eyes-closed resting-state functional magnetic resonance image scans were analyzed to compare network properties and functional connectivity (FC) changes. TLE subjects showed significantly higher global efficiency, lower clustering coefficient ratio, and lower shortest path lengths ratio than HCs, as an indication of a more synchronized, yet less segregated network. A trend of functional reorganization with a shift of network hubs to the contralateral hemisphere was noted in TLE subjects. Support vector machine (SVM) with linear kernel was trained to separate between neural networks in TLE and HC subjects based on graph measurements. SVM analysis allowed separation between TLE and HC networks with 80.66% accuracy using eight features of graph measurements. Support vector regression (SVR) was used to predict neurocognitive performance from graph metrics. An SVR linear predictor showed discriminative prediction accuracy for four key neurocognitive variables in TLE (absolute R value range: 0.61–0.75). Despite TLE, our results showed both local and global network topology differences that reflect widespread alterations in FC in TLE. Network differences are discriminative between TLE and HCs using data-driven analysis and predicted severity of neurocognitive sequelae in our cohort.

An extraosseous intradural presentation for a sacral chordoma in the pediatric age group has not been reported to date. This is a report on an 11-year-old boy who presented with an extraosseous, intradural sacral chordoma. He underwent gross-total resection and received adjuvant proton beam therapy. Neoplastic transformation of the notochord is reviewed to illustrate the developmental basis for the surgical anatomy and pathogenesis of the classic chordoma variant. Clinical and pathological features are reviewed to differentiate this chordoma presentation from classic osseous chordomas and ecchordosis physaliphora, a related benign developmental notochordal lesion. Finally, the role of developmental signaling in the pathogenesis of chordomas from postembryonic notochordal tissue is discussed.

Highlights • Temporal encephalocele is a treatable cause of drug-resistant epilepsy. • Stereo-EEG can better localize seizure onset and identify potential post-op deficits. • We present a neurophysiologic illustration of an important anatomic relationship., Temporal lobe encephalocele has emerged as a potentially unrecognized cause of drug-resistant temporal lobe epilepsy (TLE) that can be effectively treated with epilepsy surgery. Here we present a case in which a 17-year-old male with drug-resistant epilepsy and left temporal encephalocele underwent workup for epilepsy surgery, and experienced unexpected pain with electrocortical stimulation. Stimulation of stereo-EEG electrodes in the left temporal pole resulted in severe, unilateral left-sided facial pain due to inadvertent stimulation of the trigeminal nerve. Stereo-EEG showed seizure onset adjacent to encephalocele with no involvement of mesial temporal structures. A temporal pole resection sparing the mesial temporal structures and repair of the sphenoid bone defect was performed. The patient experienced post-operative seizure freedom, with no loss of language function or sensory deficits in the distribution of the trigeminal nerve. This case highlights temporal encephalocele as a potentially overlooked cause of TLE and underscores the anatomical proximity of the trigeminal nerve to the temporal pole, an important consideration for surgical planning.

Background Inappropriate medication dosing can cause adverse drug reactions or ineffective therapy due to declined renal function in patients with renal insufficiency. This necessitates proper renal dose adjustment. This study was proposed to evaluate medication dosage adjustment in hospitalized chronic kidney disease (CKD) patients. Methods This study included all CKD patients hospitalized between May 1, 2019, and April 25, 2020, at the Institute of Kidney Disease, Peshawar, Pakistan. The estimated glomerular filtration rate was calculated using the Modification of Diet in Renal Disease formula, and dose appropriateness was established by evaluating practice with relevant reference books. Results Of the total 1,537 CKD patients, 231 (15.03%) had evidence of dosing error, which was considered for final analysis. Overall, 1,549 drugs were prescribed; 480 (30.99%) drugs required dose adjustment, of which 196 (40.42%) were adjusted properly and the remaining 286 (59.58%) were unadjusted. The most common unadjusted drugs were meropenem, cefepime, ciprofloxacin, and rosuvastatin, whereas captopril, aspirin, bisoprolol, pregabalin, and levofloxacin had the highest percentage of adjusted drugs. On multivariate logistic regression, the number of drugs requiring dosing adjustments and obstructive nephropathy were found to be statistically significant factors that increased the likelihood of the medication dosing errors: a unit increase in the number of drugs requiring dose adjustment increases 5.241 times the likelihood of dosing error. Similarly, the presence of obstructive nephropathy (OR: 0.383; 95% Cl: 0.153-0.960; p = 0.041) was found to be significantly associated with dosing error after adjustment for potential confounding factors. Conclusion The dosing of more than half of the prescribed drugs that required adjustment in CKD patients was not adjusted, which showed that medication dosing errors were high. This highlights the importance of medication prescription according to guidelines in these patients to improve the outcomes of pharmacotherapy.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

As obesity rates and life expectancies increase, diabetes mellitus continues to become more prevalent worldwide. Its complex pathophysiology is associated with micro- and macrovascular complications, with cardiovascular disease the leading cause of death in individuals with diabetes. Traditional anti-hyperglycaemic drugs have helped to improve glycaemic control without reducing cardiovascular complications, but novel agents such as sodium-glucose cotransporter (SGLT)-2 inhibitors and glucagon-like peptide (GLP)-1 receptor agonists have shown an effective reduction of adverse cardiovascular events. The management of diabetes focuses on reducing low-density lipoprotein-cholesterol, lowering blood pressure and keeping glycated haemoglobin within recommended limits. Pharmacological and lifestyle interventions should address all potential risk factors to improve cardiovascular outcomes in patients with diabetes.

BackgroundInappropriate medication dosing can develop adverse drug reactions (ADRs) or ineffective therapy due to declined renal function in patients with renal insufficiency. This necessitates proper renal dose adjustment. Using a retrospective analysis of medical records, this study was proposed to evaluate medication dosage adjustment in hospitalized chronic kidney disease (CKD) patients.MethodsThis retrospective review of medical records was conducted at the Institute of Kidney Disease (IKD), Peshawar. It included all CKD patients hospitalized between May 01, 2019 and April 25, 2020. Glomerular filtration rate was calculated using Modification of Diet in Renal Disease (MDRD) formula and dose appropriateness was established by evaluating practice with relevant reference books.ResultsOf the total 1537 CKD patients, 231(15.03%) had evidence of dosing error, which were considered for final analysis. Overall, 1549 drugs were prescribed, 480(30.99%) drugs required dose adjustment of which 196(40.42%) were adjusted properly and the remaining 286(59.58%) were unadjusted. The most common unadjusted drugs were meropenem, cefepime, ciprofloxacin and rosuvastatin, whereas captopril, aspirin, bisoprolol, pregabalin and levofloxacin had the highest percentage of adjusted drugs. On multivariate logistic regression, the number of drugs requiring dosing adjustments and obstructive nephropathy were found to be statistically significant factors that increased the likelihood of the medication dosing errors: A unit increase in the number of drugs requiring dose adjustment increases 5.241 times the likelihood of dosing error. Similarly the presence of obstructive nephropathy (OR 0.383, 95% Cl [0.153-0.960] P= 0.041) was found to be significantly associated with dosing error after adjustment for potential confounding factors.ConclusionThe dosing of more than half of the prescribed drugs that required adjustment in CKD patients were not adjusted which showed that medication dosing errors were high. This highlights the importance of medication prescription according to guidelines in these patients to improve the outcomes of pharmacotherapy and patients’ quality of life.

Purpose In patients with medically refractory epilepsy and normal magnetic resonance imaging (MRI), high-resolution dedicated MRI may identify cryptic lesions. The aim of this study was to assess improvement in lesion detection and its impact on clinical management, using additional high-resolution dedicated 3T MRI in children with medically refractory epilepsy who had normal 3T epilepsy protocol MRI. Materials and methods Children who had resective epilepsy surgery and suspected focal cortical dysplasia (FCD) or normal 3T epilepsy protocol MRI were included. Those with other diagnosis on MRI including tumor and hippocampal sclerosis were excluded. Patients who had normal MRI on 3T epilepsy protocol underwent dedicated high-resolution 3T MRI through the epileptogenic zone, guided by video EEG, Magnetoencephalography and FDG-PET data. Results 101 patients with at least 1 year follow-up were included. Twenty-nine of 44 (66%) patients who had normal epilepsy protocol MRI had a lesion identified on dedicated high-resolution MRI. The addition of dedicated high-resolution MRI to standard epilepsy protocol increased sensitivity from 53.1% (95%CI: 40%–66%) to 85.9% (95%CI: 75%–93%). Identified lesions were concordant to surgical resection in all patients and guided depth/strip electrode insertion in 20/25 (80%) patients who underwent staged resection. Dedicated MRI detected small deep seated lesions in 10/20 (50%), and guided depth electrodes placement, without which it would not be feasible, as the lobar location of epileptogenic zone from other non-invasive tests were not sufficiently precise. Conclusion Patients with non-lesional epilepsy on standard epilepsy protocol MR may benefit from high-resolution dedicated MRI to aid identification of an underlying lesion, which could impact surgical management and improve seizure control.

Hepatorenal syndrome and variceal bleeding are two serious complications of chronic liver disease. Terlipressin is commonly used for reversal of these conditions. Besides its clinical advantages, it often causes serious ischemic complications. These complications are seen in patients at risk. Ischemic events, typically involve peripheries, but gastrointestinal mucosa, coronary arteries, trunk, and scrotum are also rarely involved. We observed extensive skin necrosis of right hand and forearm of a 50-year female patient treated with terlipressin for hepatorenal syndrome. Since this event was never noticed earlier in tertiary care hospitals of our province; to the best of authors' knowledge, this is the first case in medical literature from this part of the country.

Introduction The diagnosis and surgical management of pediatric patients with insular epilepsy is challenging due to variable and indistinct seizure semiology arising within the insular cortex. In addition, surgical approaches are associated with potential morbidity given the regional neurosurgical and vascular anatomy. Our institutional experience in evaluation and surgical management of insular epilepsy patients is presented. Diagnostic evaluation, surgical treatment and seizures outcomes are highlighted. Methods The institutional database for all pediatric surgical epilepsy patients was reviewed over a period of 15 years (2000–2015). Study subjects were defined as patients who had undergone surgical resection of the epileptogenic zone involving the insular cortex. Diagnostic imaging, electrophysiological studies and surgical records were assessed. Results Six (n = 6) patients with insular epilepsy were identified with a mean follow up of 2.8 years (range: 0.8–6.8 years). Mean age at surgical resection was 9.5 ± 2.5 years (range: 2.5–16 years). Majority of patients (n = 4/6, 67%) underwent more than 1 surgical procedure. Magnetoencephalography (MEG) identified insular cortex involvement presurgically in all cases. MEG cluster localization was useful in guiding invasive EEG monitoring in 5 patients and was concordant with identification of epileptogenic zone through intracranial monitoring in all 5 patients. Surgical resection of the epileptogenic zone, as delineated through MEG spike sources and invasive EEG monitoring, was associated with favorable seizure outcome in 4 of 6 patients (67%; Engel Class I). Cortical dysgenesis was identified on histopathology in 4 cases. Conclusion Diagnostic evaluation through invasive and noninvasive electrophysiological studies is critical to identify pediatric patients with insular epilepsy. Our findings suggest that MEG may help with identifying the epileptogenic zone within the insular cortex, which could be confirmed with invasive intracranial monitoring.

We present a case of a 60-year-old female patient who has significant medical history of renal cell carcinoma diagnosed 2 years back and had undergone right nephrectomy and chemotherapy. She presented to the hospital with complaints of abdominal pain and jaundice of 2 weeks duration and was found to have periampullary mass lesion causing compression of distal common bile duct on imaging with computed tomography of abdomen. Endoscopic retrograde cholangiography and endoscopic ultrasound showed ampullary mass lesion causing biliary obstruction along with abdominal lymphadenopathy. A temporary plastic stent was placed to relieve obstruction. Fine needle aspiration cytology of the periampullary mass along with immunohistochemical staining confirmed the diagnosis of metastatic renal cell carcinoma.

A 72-year-old gentleman with significant cardiac history and a pacemaker in situ initially presented to the emergency department 5 days after he had his pacemaker-unit batteries changed. He had deranged vital signs, productive cough and fever. His chest plain radiograph did not show evidence of infection; however, he had right basal crackles on auscultation, which suggested a lower respiratory tract infection. He was treated with intravenous co-amoxiclav and supportive therapy, which led to his improvement. The patient was discharged but had to be readmitted a total of four times over the span of 4 months due to recurrent fever and associated symptoms. Transthoracic and transoesophageal echocardiograms and CT of the neck/thorax/abdomen/pelvis were done to look for endocarditis, pacemaker-unit infection and other sources of infection. However, these did not show any evidence of infection. He did have persistent raised inflammatory markers and two blood cultures growingEnterobacter cloacae.A fluorodeoxyglucose positron emission tomography scan was done, which showed evidence of pacemaker lead infection. His pacemaker unit was removed, which led to cessation of his symptoms and normalisation of his inflammatory markers. He had no further hospital admissions to date and has been regularly followed up in an outpatient cardiology clinic.

BACKGROUND Chronic kidney disease (CKD)-associated pruritus (CKD-aP) contributes to poor quality of life, including reduced sleep quality and poor sleep quality is a source of patient stress and is linked to lower health-related quality of life. This study aimed to investigate the effectiveness of zolpidem 10 mg and acupressure therapy on foot acupoints to improve the sleep quality and overall quality of life among hemodialysis patients suffering from CKD-aP. METHOD A multicenter, prospective, randomized, parallel-design, open label interventional study to estimate the effectiveness of zolpidem (10 mg) oral tablets versus acupressure on sleep quality and quality of life in patients with CKD-aP on hemodialysis. A total of 58 hemodialysis patients having sleep disturbance due to CKD-aP completed the entire 8-week follow-up. The patients were divided into a control (acupressure) group of 28 patients and an intervention (zolpidem) group of 30 patients. RESULTS A total of 58 patients having CKD-aP and sleep disturbance were recruited. In the control group there was a reduction in the PSQI score with a mean ± SD from 12.28 ± 3.59 to 9.25 ± 3.99, while in the intervention group the reduction in PSQI score with a mean ± SD was from 14.73 ± 4.14 to 10.03 ± 4.04 from baseline to endpoint. However, the EQ5D index score and EQ-visual analogue scale (VAS) at baseline for the control group with a mean ± SD was 0.49 ± 0.30 and 50.17 ± 8.65, respectively, while for the intervention group the values were 0.62 ± 0.26 and 47.17 ± 5.82, respectively. The mean EQ5D index score in the control group improved from 0.49 ± 0.30 to 0.53 ± 0.30, but in the intervention group there was no statistical improvement in mean EQ5D index score from 0.62 ± 0.26 to 0.62 ± 0.27 from baseline to week 8. The EQ 5D improved in both groups and the EQ-VAS score was 2.67 points higher at week 8 as compared to baseline in the control group, while in the intervention group the score was 3.33 points higher at week 8 as compared to baseline. Comparing with baseline, the PSQI scores were significantly reduced after week 4 and week 8 (P =

Highlights • Riche–Cannieu anastomosis (RCA) in patients with CTS needs to be considered. • Misdiagnosis as severe CTS can lead to unnecessary surgical intervention. • Evaluation for CTS should always include an EMG., Introduction Anomalous anastomoses between the nerves of the hand are not as rare as thought to be. Amongst these, the Riche–Cannieu anastomosis (RCA) is a connection between the ulnar nerve and median motor nerve in the palm. Presence of this can sometimes be misdiagnosed as a severe median mononeuropathy at the wrist/carpal tunnel syndrome (CTS). We describe two cases that were referred for evaluation of CTS and were found to have incidental RCA. Results Electrophysiological studies showed typical findings consistent with RCA and coexistent mild CTS. Patients in whom median sensory and motor latency prolongations suggest CTS, the presence of an anastomosis should be suspected if the median motor amplitudes are worse than the median sensory amplitudes. Conclusion The electrophysiological study should be extended to avoid erroneous interpretation of low median motor amplitudes as severe CTS and to prevent unnecessary surgical intervention.

Objectives: To determine the frequency of different clinical features of myastheniagravis in patients presenting to a tertiary care hospital, Karachi. Study Design: Cross sectionalstudy. Setting: Neurology ward, JPMC, Karachi. Period: 23rd January 2013 to 22nd July 2013.Patients and Methods: A total of 71 diagnosed patients of Myasthenia Gravis (MG) betweenthe age 15 and 70 years were recruited. Structured questionnaire was used to collect the dataregarding most common clinical manifestations of MG. Data were entered and analyzed inSPSS version 17. Chi-Square test was used as test of significance. Results: Mean age + S.Dof patients was 34.11 ± 10.42 years. The mean ± SD of duration of symptoms among thesepatients was 5.23 ± 3.52 months. Most of the patients (21%) belong to age between 21 to30 years. Regarding clinical features in these patients of myasthenia gravis it was noted thatptosis and diplopia were most common symptoms, 62% and 54.9%, respectively. Conclusion:Myasthenia gravis, a chronic neuromuscular disorder leads to various degrees of neurologicdysfunction which manifest as different clinical features. The current study found that ocularsymptoms are commonest presenting features.

Identification of the epileptogenic zone is a critical determinant of seizure control outcomes following surgical resection in medically refractory seizures. There has been increasing recognition of the contributive value of Magnetoencephalography (MEG) studies in the diagnostic evaluation of epilepsy. Biomagnetic signals recorded by MEG studies in combination with source modeling techniques can yield diagnostic information regarding the underlying epileptogenic zone. MEG evaluation is particularly useful in evaluation of non lesional neocortical epilepsy. In these cases, MEG spike sources can guide presurgical and perioperative invasive monitoring. The incorporation of MEG-based diagnostic information into the surgical evaluation and management plan has consistently been shown to be associated with improved seizure control outcomes.

Introduction Pyrexia of unknown origin (PUO) is defined as a temperature greater than 38.3°C on several occasions, accompanied by three weeks of illness and a failure to reach a diagnosis after one week of inpatient investigations.1 We describe a case of a 72-year-old man with a significant cardiac history and a pacemaker in-situ presented to the emergency department of a district general hospital with sepsis five days after he had his pacemaker unit batteries changed. He had deranged vital signs, productive cough and pyrexia. He had a normal chest plain radiograph (CXR). However, with right basal crackles on auscultation, he was empirically treated for a lower respiratory tract infection (LRTI) with intravenous antibiotics. The patient was discharged but had to be readmitted four times over the span of 4 months due to recurrent pyrexia. Repeated Trans-thoracic (TTE) and Trans-oesophageal (TOE) echocardiograms, and CT neck/thorax/abdomen/pelvis were done however no evidence of infection was found on the scans. A FDG-PET scan [figure 1] was eventually done which showed evidence of pacemaker-lead infection. His pacemaker unit was removed and replaced with a REVEAL device. He improved clinically with no further hospital admission to date. Methods Routine blood test only showed raised WCC and CRP. Connective tissue disease screen, viral and tumour markers, syphilis serology, and hepatitis screen were negative. Two out of six blood cultures (on separate admissions) done grew Enterobacter cloacae. Repeated CXRs, CT scans and Echocardiograms showed no evidence of collection/infection. A FDG-PET scan was carried out which detected a focus point of increased activity around the pacemaker leads. Results Enterobacter cloacae is a nosocomial, gram-negative, facultatively anaerobic, rod-shaped bacterium from the Enterobacteriaceae family. It is usually found in the gut normal flora and skin of humans. It can colonise surgical equipment, operative cleaning solutions and heparin solution used to irrigate certain intravascular devices.2 The surgical equipment used in this patient’s pacemaker battery change may have been colonised with the bacteria which could explain why it was present in the blood cultures. Another explanation would be that it was a skin commensal for the patient. However, to our knowledge, no other cases from the hospitals involved have been identified or reported recently. Unfortunately, when the pacemaker was removed in our patient, the pacemaker leads were not sent for culture and were discarded. The type of PET scan used in this case was the FDG- PET. Hybrid F18- FDG-PET/CT has high sensitivity and relative non-specificity for malignancy, infection and inflammation.3 4 The subsequent removal of the pacemaker led to the improvement and resolution of symptoms. Conclusions This case has highlighted the importance to explore previous hospital admissions, building a timeline of events, which could be leading to the patients’ current presentation. Moreover, patients presenting with pyrexia after a recent prosthetic implantation should be investigated for prosthesis-led-infections, despite negative investigation results. Therefore, the approach to investigate and treat known source of infections and pyrexia of unknown origin needs to be done systematically according to set hospital protocols and evidence-based medicine. References Petersdorf RG, Beeson PB. Fever of unexplained origin: Report on 100 cases. Medicine (Baltimore) 1961;40:1–30. Mezzatesta ML, Gona F, Stefani S. Enterobacter cloacae complex: Clinical impact and emerging antibiotic resistance. Future Microbiol 2012 July;7(7):887–902. Hao R, Yuan L, Kan Y, et al. Diagnostic performance of 18F-FDG PET/CT in patients with fever of unknown origin: A meta-analysis. Nucl Med Commun 2013;34(7):682–8. Balink H, Verberne HJ, Bennink RJ, et al. A rationale for the use of F18-FDG PET/CT in fever and inflammation of unknown origin. Int J Mol Imaging 2012;2012:165080. doi:10.1155/2012/165080

OBJECTIVEPediatric rolandic arteriovenous malformations (AVMs) present a treatment challenge given the lifetime risk of hemorrhage, rehemorrhage, and associated long-term morbidity. Microsurgical resection has been recommended as the optimal treatment for AVMs in general, but there is no dedicated literature on the outcomes of resection of pediatric rolandic AVMs. Here, the study objective was to review the outcomes of microsurgical resection of pediatric rolandic AVMs in the modern era, together with the utilization of surgical adjuncts including navigation, intraoperative angiography, and neurophysiological monitoring.METHODSThe authors performed a retrospective review of patients 18 years of age and younger with cerebral AVMs microsurgically treated between January 2000 and May 2016 at The Hospital for Sick Children. Only those patients with an AVM whose nidus was located within the rolandic region were analyzed. A descriptive analysis was performed to identify patient demographics, preoperative AVM characteristics, and postoperative obliteration rates and neurological complications.RESULTSA total of 279 AVMs were evaluated in the study period. Twenty-three of these AVMs were rolandic, and the median age in the 11 microsurgically treated cases was 11 years (range 1–17 years). AVM hemorrhage was the most common presentation, occurring in 8 patients (73%). Lesions were either Spetzler-Martin grade II (n = 8, 73%) or grade III (n = 3, 27%). The postoperative obliteration rate of AVMs was 100%. The mean imaging follow-up duration was 33 months (range 5–164 months). There was no documented recurrence of an AVM during follow-up. One patient developed a transient postoperative hemiparesis, while another patient developed right fingertip hyperesthesia.CONCLUSIONSMicrosurgical resection of rolandic pediatric AVMs yields excellent AVM obliteration with minimal neurological morbidity in selected patients. The incorporation of surgical adjuncts, including neurophysiological monitoring and neuronavigation, allows accurate demarcation of functional cortex and enables effective resection.

Objectives: To determine the frequency of various neurological manifestationsof Wilson’s disease in patients presenting to tertiary care facility. Study design: Cross sectionalstudy. Setting: Neurology ward, JPMC, Karachi. Period: 14-03-2012 to 13-09-2012. Patientsand Methods: A total of 150 diagnosed patients of Wilson’s disease between the age 15 and55 years were recruited. Structured questionnaire was used to collect the data regardingneurological manifestations of Wilson’s disease along with slit lamp examination for kayser-Fleischer rings. Data were entered and analyzed in SPSS version 17. Chi-Square test was usedas test of significance. Results: Mean age + S.D of patients was 38.7 + 8.23 years. Most of thepatients (42%) belongs to age between 41 to 50 years. Male to female ratio was 2:1. Tremorwas the most common neurological manifestation (88.7%) followed by dysarthria in 77.3%,dystonia was present in 59.3%, chorea in 36%, and Parkinsonism in 28.7%. All patients hadthe Kayser-Fleischer ring positive. Upon stratification of gender, tremor & dysarthria were morecommon in males as compared to females. Only dysarthria was significantly noted to increasewith increasing age up to 5th decade of life and decreased after age of 51 years. (P value< 0.001). Conclusion: Neurologic manifestations are very common in patients of Wilson’sdisease, tremor being the most common with male predominance.

Clinical presentation of craniovertebral junction disorders may range from acute catastrophic neurological deficits to insidious signs and symptoms that may mask the underlying etiology. Prompt recognition and treatment is essential to avert long-term neurological morbidity. Proatlas segmentation disorders are a rare group of developmental disorders involving the craniocervical junction. Abnormal bony segmentation leads to malformed bony structures that can in turn lead to neurological deficits through bony compression of the cervicomedullary junction. This report details a proatlas segmentation defect presenting as palatal myoclonus, a rare movement disorder. The clinical presentation, surgical management, and neuroanatomical basis for the disorder is presented. This report highlights the myriad clinical presentations of craniovertebral disorders and emphasizes a rare but treatable etiology for palatal myoclonus.

Background. The standard use of vaccinations against pathogens has resulted in a decreased incidence of musculoskeletal infections caused by these previously common bacterial pathogens. Consequently, the incidence of infections caused by atypical bacteria is rising. This report presents a case of septic arthritis caused by non-type bH. influenzaein a pediatric patient.Methods. We report a case of an infant with polyarticular septic arthritis caused byH. influenzaeserotype f. A literature review was conducted with the inclusion criteria of case reports and studies published between 2004 and 2013 addressing musculoskeletalH. influenzaeinfections.Results. An 8-month-old female presented with pain and swelling in her right ankle and left elbow. The patient was diagnosed with septic arthritis and underwent incision and drainage. Wound and blood cultures were positive forHaemophilus influenzaeserotype f. In addition to treatment with IV antibiotics, the patient underwent immunocompetency studies, which were normal. Subsequent follow-up revealed eradication of the infection.Conclusions.Haemophilus influenzaenon-type b may cause serious invasive infections such as sepsis or septic arthritis in children with or without predisposing factors such as immunodeficiency or asplenia. Optimal treatment includes surgical management, culture driven IV antibiotics, and an immunologic workup.

Object Spinal deformity in pediatric patients with intramedullary spinal cord tumors (IMSCTs) may be either due to neurogenic disability or due to secondary effects of spinal decompression. It is associated with functional decline and impairment in health-related quality-of-life measures. The authors sought to identify the long-term incidence of spinal deformity in individuals who had undergone surgery for IMSCTs as pediatric patients and the risk factors and overall outcomes in this population. Methods Treatment records for pediatric patients (age < 21 years) who underwent surgical treatment for histology-proven primary IMSCTs between 1975 and 2010 were reviewed. All patients were evaluated in consultation with the pediatric orthopedics service. Clinical records were reviewed for baseline and follow-up imaging studies, surgical fusion treatment, and long-term skeletal and disease outcomes. Results The authors identified 55 patients (30 males and 25 females) who were treated for pediatric IMSCTs between January 1975 and January 2010. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2–37.2 years). Preoperative skeletal deformity was diagnosed in 11 (20%) of the 55 patients, and new-onset postoperative deformity was noted in 9 (16%). Conservative management with observation or external bracing was sufficient in 8 (40%) of these 20 cases. Surgical fusion was necessary in 11 (55%). Posterior surgical fusion was sufficient in 6 (55%) of these 11 cases, while combined anterior and posterior fusion was undertaken in 5 (45%). Univariate and multivariate analysis of clinical and surgical treatment variables indicated that preoperative kyphoscoliosis (p = 0.0032) and laminectomy/laminoplasty at more than 4 levels (p = 0.05) were independently associated with development of spinal deformity that necessitated surgical fusion. Functional scores and 10-year disease survival outcomes were similar between the 2 groups. Conclusions Long-term follow-up is essential to monitor for delayed development of spinal deformity, and regular surveillance imaging is recommended for patients with underlying deformity. The authors' extended follow-up highlights the risk factors associated with development of spinal deformity in patients treated for pediatric IMSCTs. Surgical fusion allows patients who develop progressive deformity to achieve long-term functional and survival outcomes comparable to those of patients who do not develop progressive deformity.

Cardiovascular disease continues to be the leading cause of death in the western world. Due to advancements in diagnosis, prevention, and treatment, cardiovascular mortality has fallen in recent years. Previous studies have evaluated the impact of traditional risk factors such as hypercholesterolemia and smoking. However, limited studies have been conducted to evaluate sex discrepancies among patients with cardiovascular disease. Pre-eclampsia is a multisystem placentally mediated disease, which usually arises after 32 weeks of gestation and classically presents with hypertension and proteinuria. Pre-eclampsia affects 2% to 8% of all pregnancies worldwide and is often complicated by fetal growth restriction. Women with a history of pre-eclampsia are at increased risk of future cardiovascular complications. Therefore, this topic is of significance to the cardiovascular health of over 300 million women worldwide. The goal of this review is to determine the association of pre-eclampsia and future cardiovascular risk and to explore the potential management options for these high-risk women.

Object Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up. Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010. Results Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2–18 years). Chordomas received proton or LINAC irradiation, and as of 4–15 years of follow-up, no recurrence has been noted. Conclusions Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

Background. A discoid meniscus is a thickened variant of the normal C-shaped meniscus prone to injury. Discoid medial meniscal tears have rarely been reported within families and may suggest familial or developmental origins.Methods. We report the cases of two Caucasian brothers with symptomatic discoid medial meniscus tears. A literature review was conducted addressing discoid medial meniscus and cases of familial meniscus tears.Case Presentation. Physically active brothers presented with progressively worsening knee pain. MRI revealed medial meniscus tears in both brothers. The family history of medial meniscus tears in their mother and the discoid medial meniscus injuries found on arthroscopy suggested evidence for familial discoid medial meniscus tears.Conclusions. Discoid medial meniscus tears within a family have not been previously reported. Two cases of families with discoid lateral meniscus tears have been reported. Discoid medial meniscus is rare relative to the discoid lateral meniscus and predisposes children to symptomatic tears.

Objective Skull base chordomas (SBC) are characterized by persistent progression. Conventional radiation following resection increases 5- and 10-year local control (LC) to 36 and 23 %, respectively. Patients treated with protons result in 10-year LC of 50 %. Brainstem dose constraints have inhibited higher prescription doses with photons. We reviewed our experience using high-dose fractionated stereotactic photon radiation therapy (HD-FSRT) to 81 Gy for SBC.

SummaryObjective There is some suggestion that microscopic infarct could be associated with invasive monitoring, but it is unclear if the microscopic infarct is also visible on imaging and associated with neurologic deficits. The aims of this study were to assess the rates of microscopic and macroscopic infarct and other major complications of pediatric epilepsy surgery, and to determine if these complications were higher following invasive monitoring. Methods We reviewed the epilepsy surgery data from a tertiary pediatric center, and collected data on microscopic infarct on histology and macroscopic infarct on postoperative computed tomography (CT) or magnetic resonance imaging (MRI) done one day after surgery and major complications. Results Three hundred fifty-two patients underwent surgical resection and there was one death. Forty-two percent had invasive monitoring. Thirty patients (9%) had microscopic infarct. Univariable analyses showed that microscopic infarct was higher among patients with invasive monitoring relative to no invasive monitoring (20% vs. 0.5%, respectively, p < 0.001). Eighteen patients (5%) had macroscopic infarct on CT or MRI. Univariable analysis showed no significant difference in macroscopic infarct between invasive monitoring and no invasive monitoring (8% vs. 3%, respectively, p = 0.085). One patient with microscopic infarct had transient right hemiparesis, and two with both macroscopic and microscopic infarct had unexpected persistent neurologic deficits. Thirty-two major complications (9.1%) were reported, with no difference in major complications between invasive monitoring and no invasive monitoring (10% vs. 7%, p = 0.446). In the multivariable analysis, invasive monitoring increased the odds of microscopic infarct (odds ratio [OR] 15.87, p = 0.009), but not macroscopic infarct (OR 2.6, p = 0.173) or major complications (OR 1.4, p = 0.500), after adjusting for age at surgery, sex, age at seizure onset, operative type, and operative location. Significance Microscopic infarct was associated with invasive monitoring, and none of the patients had permanent neurologic deficits. Macroscopic infarct was not associated with invasive monitoring, and two patients with macroscopic infarct had persistent neurologic deficits.

Background: Temporomandibular disorder (TMD) is a collective term that encompasses many clinical problems involving the masticatory muscles, temporomandibular joints (TMJ) and associated structures and it has high prevalence among populations. Aims and objectives: To determine the prevalence of TMD and its relationship to age, gender, occlusion and psychological factors and to evaluate which age group, gender, malocclusion parameters and psychological factors contribute to the prevalence of TMD. Materials and methods: The study includes a sample of 250 young adults (134 girls and 116 boys) with age ranging from 18 to 25 years, the presence and severity of TMD was determined using a self-administered anamnestic questionnaire composed of 10 questions regarding common TMD symptoms. Morphologic occlusion was evaluated according to Angle's classification (molar classes I, II, III) and to evaluate the psychological factors, The hospital anxiety and depression scale (HADS) developed by Zigmond and Snaith was used. Results: Data were computerized and the SPSS package (version 11.2) was used and nonparametric test of Chi-square for data analysis and unpaired t-test was also used for statistical data analysis. Anamnestic index (AI) showed that the percentage of women (5522%) had higher degree of TMD symptoms than compared with men (50.86%), comparing the age of men and women free TMD and with TMD, the statistical difference was not significant as (t = 1.35, p > 0.5), distribution of the cases among Angle's class I, II, III occlusion the difference was not statistically significant as (p > 0.5), the degree of anxiety and TMD degree among men and women patients, women had higher anxiety levels as compared to men and no statistical difference was found between the cases of men and women in the depression levels. Conclusion: Prevalence of TMD symptoms in our sample of 250 patients was high for women- Morphologic occlusion was not associated with the presence of TMD symptoms. By considering the psychological factors we found anxiety but not depression associated with TMD symptoms.

Object Hyperbaric oxygen therapy (HBO) is used as primary and/or adjunctive therapy in the treatment of various clinical conditions complicated by local hypoxia. It may have therapeutic potential in the treatment of neurosurgical infections such as spinal osteomyelitis that are associated with significant morbidity rates. The purpose of this study was to evaluate the efficacy of HBO therapy in the treatment of spinal osteomyelitis. Methods The clinical records of patients diagnosed with spinal osteomyelitis who received HBO therapy during their treatment at the authors' institution over the past 10 years were retrospectively reviewed. Six adult patients were identified. Four patients had recently undergone spinal surgery and secondary spinal osteomyelitis had developed. These patients received adjunctive HBO therapy due to significant comorbidities and risk factors for poor healing. Results All patients remained symptom and infection free over the subsequent follow-up period. Two patients had primary spinal osteomyelitis that had recurred despite a full course of appropriate antimicrobial therapy. Infection control was achieved after HBO therapy in 1 patient. The mean follow-up period for the study group was 2.9 years (range 5 months to 5 years). Conclusions Hyperbaric oxygen therapy enabled infection cure in 5 of 6 patients with spinal osteomyelitis complicated by medical comorbidities or the failure of primary therapy. These results show that HBO may be a useful adjunctive therapeutic modality in the treatment of spinal osteomyelitis, particularly when there are medical comorbidities that increase the risk of poor healing. Hyperbaric oxygen therapy may also be beneficial in patients with relapsing primary spinal osteomyelitis after standard therapy has failed.

The surgical management of craniovertebral junction instability in pediatric patients has unique challenges. While the indications for internal fixation in children are similar to those of adults, the data concerning techniques, complications, and outcomes of spinal instrumentation comes from experience with adult patients. Diminutive osseous and ligamentous structures and anatomical variations associated with syndromic craniovertebral abnormalities frequently complicates the approaches and limits the use of internal fixation in children. Cervical arthrodesis in the pediatric age group has the potential for limiting growth potential and causing secondary deformity. Recent advances in image analysis have enabled preoperative planning which is critical to evaluate the size of instrumentation and its relation to the patient’s anatomy. Newer techniques have recently evolved and have been incorporated in the management of pediatric patients with requirement for craniocervical stabilization. Over 750 craniovertebral junction fusions have been reviewed in children. The indications for atlantoaxial arthrodesis were: (a) absent odontoid process, dystopic os odontoideum, absent posterior arch of C1; (b) Morquio’s syndrome, Goldenhar’s syndrome, Conradi’s syndrome, and spondyloepiphyseal dysplasia. The acquired abnormalities of trauma, postinfectious instability, and Down’s syndrome completed the indication in children. The indications for occipitocervical fusion were: (a) anterior and posterior bifid C1 arches with instability, absent occipital condyles; b) severe reducible basilar invagination, unstable dystopic os odontoideum, and unilateral atlas assimilation; (c) acquired phenomenon with traumatic occipitocervical dislocation, complex craniovertebral junction fractures of C1 and C2, after transoral craniovertebral junction decompression, cranial settling in Down’s syndrome and inflammatory disease such as Grisel’s syndrome. Instability was seen in children with clivus chordoma and osteoblastoma. Atlantoaxial fusions were performed mainly with interlaminar rib graft fusion and more recently with the transarticular screw fixation in the older patient. In the teenager, lateral mass screws at C1 and rod fixation were made; C2 pars interarticular screw fixation and C2 pedicle screw fixation. A C2 translaminar screw fixation is described. Occipitocervical fusions were made utilizing rib grafts below the age of 6. A contoured loop fixation was made in children above the age of 7, and recently, rod and screw fixation was also utilized. Abnormal cervical spine growth was not seen in children who underwent craniocervical stabilization below the age of 5. The authors have reserved rigid instrumentation for children above the age of 10 years and dependent on the anatomy.

An abdominal aortic aneurysm (AAA) is a focal full thickness dilatation of the abdominal aorta, greater than 1.5 times its normal diameter. Although some patients with AAA experience back or abdominal pain, most remain asymptomatic until rupture. The prognosis after AAA rupture is poor. Management strategies for patients with asymptomatic AAAs include risk factor reduction, such as smoking cessation, optimizing antihypertensive treatment, and treating dyslipidemia, as well as surveillance by ultrasound. Currently, aneurysm diameter alone is often used to assess risk of rupture. Once the aneurysm diameter reaches 5.5 cm, the risk of rupture is considered greater than the risk of intervention and elective aneurysm repair is undertaken. There is increasing interest in detecting AAAs early, and national screening programs are now in place. Furthermore, there is increasing research interest in biomarkers, genetics, and functional imaging to improve detection of AAAs at risk of progression and rupture. In this review, we discuss risk factors for AAA rupture, which should be considered during the management process, to advance current deficiencies in management pathways.

Objective Chordomas carry significant morbidity due to their growth patterns and surgical constraints in resection. En bloc resection, when feasible, is the ideal treatment goal, but is associated with significant morbidity. We sought to elucidate the relationship between extent of surgery, location and radiotherapy in relation to overall disease and progression free survival (PFS). Methods We reviewed case records for all patients with a primary histopathological diagnosis of clival and spinal chordomas that was presented to our institution between 1978 and 2010. Results A total of 49 patients (location: n = 30, skull base/clival; n = 12 vertebral column; n = 7 sacrum) were identified with mean follow-up period of 6.3 years (range 0.25 months–33 years). Improved 5 year and 10 year survival rates were noted following gross total resection (n = 8, 5 year and 10 year survival = 88%) as compared to patients that underwent subtotal resection (n = 41, 55% and 31%, respectively), (p-value > 0.05, GTR versus STR). Adjuvant high-dose stereotactic fractionated radiotherapy (HS-FSRT) significantly improved 5 year PFS in craniocervical chordoma patients (70%, n = 13) as compared to standard dose radiation therapy (20%, n = 16; p-value = 0.03). Overall 10 year survival for craniocervical patients undergoing HD-FSRT (40%) was however not significantly different in comparison with conventional radiotherapy (45%). Sacral chordomas had the worst prognosis with 3 year survival of 28.6%. Conclusions GTR offers the best prognosis for improved long-term survival. Adjuvant HD FSRT for cranio-cervical/clival chordomas significantly improves disease free survival though the long-term benefits on survival have yet to be established. Sacral chordomas are associated with a worse prognosis and poor long-term survival.

In the present study, we review the indications, technical aspects, preliminary results, risks, and clinical implications of fetal endoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia (CDH) performed outside the United States and its potential future directions in this country and globally. Congenital diaphragmatic hernia occurs in approximately 1 in 2500 live births and results in high neonatal morbidity and mortality, largely associated with the severity of pulmonary hypoplasia and pulmonary arterial hypertension. With the advent of prenatal imaging, CDH can be diagnosed before birth, and in utero treatment is now available in some centers. The prognosis of CDH can be evaluated by assessing the fetal lung size, the degree of liver herniation, and the fetal pulmonary vasculature in isolated forms of CDH. These parameters help classify fetuses as having mild, moderate, severe, or extremely severe isolated CDH. Severe and extremely severe diaphragmatic hernias have poor outcomes and thus are candidates for innovative therapies such as FETO. Fetal endoscopic tracheal occlusion is usually performed between 26 and 30 weeks' gestation. In utero, an endoscope is passed through the fetal mouth and down to the carina; the balloon is deployed just above the carina. After the procedure, ultrasound surveillance every 2 weeks ensures the balloon's structural integrity and measures the fetal pulmonary response. At approximately 34 weeks' gestation, the balloon is deflated and removed. Fetal endoscopic tracheal occlusion is thought to improve outcomes by decreasing mortality and allowing more rapid neonatal stabilization. Ultimately, the goal of FETO is to minimize pulmonary hypoplasia and pulmonary arterial hypertension. Following delivery, neonates still require diaphragm repair.

Intracerebral hemorrhages (ICHs) comprise 5% to 30% of all strokes with a case fatality rate of 30% to 50%. With an increasing elderly population, nontraumatic ICH is likely to present as a recurring medical emergency in Pakistan. There is scarce documentation in Pakistan regarding the predictors for mortality in patients with ICH that can guide physicians in their management. We conducted a hospital-based study to determine the predictors of in-hospital mortality from ICH in patients admitted to a tertiary care facility.We reviewed case records of patients above 17 years admitted to The Aga Khan University Hospital, Karachi, Pakistan, between July 1995 and December 1997, with the primary diagnosis of ICH confirmed on computed tomography scanning. Clinical and risk factors were also evaluated.The data of 221 patients was analyzed. Seventy-one (32%) patients died during hospital stay, half of whom died during the first 2 days of hospitalization. Patients who died during hospital stay were more likely on admission to have intraventricular extension of the primary hemorrhage (odds ratio = 10.6, P.01), a Glasgow Coma Score less than 12 on admission (odds ratio = 4.8, P.01), and a systolic blood pressure greater than or equal to 180 mm Hg (odds ratio = 2.7, P = .03).Our study suggests that Glasgow Coma Score and intraventricular extension are predictors of acute mortality, consistent with published results. Elevated systolic blood pressure at admission, also found to be a predictor of acute mortality from ICH, may be explained alternatively by internal and external stroke-induced stress factors. The actual mechanisms for high admission systolic blood pressure remain unclear.

Traumatic brain injury (TBI) is a common cause of morbidity and mortality in people of all ages. Following the acute mechanical insult, TBI evolves over the ensuing minutes and days. Understanding the secondary factors that contribute to TBI might suggest therapeutic strategies to reduce the long-term consequences of brain trauma. To assess secondary factors that contribute to TBI, we studied a lateral fluid percussion injury (FPI) model in mice. Following FPI, the brain cortex became acidic, consistent with data from humans following brain trauma. Administering HCO3 (-) after FPI prevented the acidosis and reduced the extent of neurodegeneration. Because acidosis can activate acid sensing ion channels (ASICs), we also studied ASIC1a(-/-) mice and found reduced neurodegeneration after FPI. Both HCO3 (-) administration and loss of ASIC1a also reduced functional deficits caused by FPI. These results suggest that FPI induces cerebral acidosis that activates ASIC channels and contributes to secondary injury in TBI. They also suggest a therapeutic strategy to attenuate the adverse consequences of TBI.

Nephrogenic systemic fibrosis (NSF) is a rare and a debilitating disease noted uncommonly in patients with impaired renal function when exposed to low-stability gadolinium-based contrast agents (Gd-CAs). According to experimental studies, cytokines released by the stimulation of effector cells such as skin macrophages and peripheral blood monocytes activate circulating fibroblasts which play a major role in the development of NSF lesions. The presence of permissive factors, presumably, provides an environment conducive to facilitate the process of fibrosis. Multiple treatment modalities have been tried with variable success rates. More research is necessary to elucidate the underlying pathophysiological mechanisms which could potentially target the initial steps of fibrosis in these patients. This paper attempts to collate the inferences from the in vivo and in vitro experiments to the clinical observations to understand the pathogenesis of NSF. Schematic representations of receptor-mediated molecular pathways of activation of macrophages and fibroblasts by gadolinium and the final pathway to fibrosis are incorporated in the discussion.

Background: Prognosis of patients with spinal cord glioblastoma is poor, with an average survival of 18 months. There are reports in the literature describing cordectomy as a treatment option for patients with spinal cord tumors. Case Description: This is a case report of a patient with spinal cord glioblastoma who, in addition to radiation and chemotherapy, was treated with cordectomy. Outcome of treatment resulted in 12-year survival. Conclusion: Cordectomy in spinal cord glioblastoma can result in prolonged and meaningful survival.