Complete third nerve palsy as a presenting feature of an interpeduncular lipoma.

BACKGROUND: Intracranial lipomas are rare, benign lesions, of congenital origin commonly found incidentally on imaging studies. METHODS: We describe a case of interpeduncular lipoma in an 18 year-old female presenting with a painful left complete oculomotor (IIIrd) nerve palsy and associated headache, which to the best of our knowledge has not been reported in an adult patient. RESULTS: Following eye patching and steroid treatment, at 6-week follow-up the patient's pain had significantly improved however the complete IIIrd nerve palsy remained. CONCLUSIONS: Evidence suggests improvement will occur regardless with conservative management independent of radiological changes to the lipoma.

Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancy-how serious is it?

PURPOSE: We test the hypothesis that ventriculoperitoneal (VP) shunt insertion significantly increases contralateral positional plagiocephaly. METHODS: We reviewed 339 children who had a VP shunt inserted at Birmingham Children's Hospital between 2006 and 2013, noting laterality of shunt insertion and frontal or occipital position. We ascertained the presence of post-operative positional plagiocephaly using the cranial vault asymmetry index. Multinomial logistic regression modelling was used to examine relationships between plagiocephaly, shunt position, gender and age. Adjusted odds and risk ratios for effect of variables on plagiocephaly were calculated. RESULTS: Children with occipital VP shunts are at significant risk of developing contralateral positional plagiocephaly, particularly in the first 12 months of life. CONCLUSIONS: We recommend careful follow-up and advice regarding head positioning following surgery. There should be consideration for active monitoring to avoid plagiocephaly, including physiotherapy and health visitor interventions. Endoscopic third ventriculostomy in selected cases or anterior shunt placement could be considered. A larger national study would be of interest to evaluate the extent of an otherwise correctable problem.

Improved survival in UK combat casualties from Iraq and Afghanistan: 2003-2012.

BACKGROUND: The United Kingdom was at war in Iraq and Afghanistan for more than a decade. Despite assertions regarding advances in military trauma care during these wars, thus far, no studies have examined survival in UK troops during this sustained period of combat. The aims of this study were to examine temporal changes of injury patterns defined by body region and survival in a population of UK Military casualties between 2003 and 2012 in Iraq and Afghanistan. METHODS: The UK Military Joint Theatre Trauma Registry was searched for all UK Military casualties (survivors and fatalities) sustained on operations between January 1, 2003, and December 31, 2012. The New Injury Severity Score (NISS) was used to stratify injury severity. RESULTS: There were 2,792 UK Military casualties sustaining 14,252 separate injuries during the study period. There were 608 fatalities (22% of all casualties). Approximately 70% of casualties injured in hostile action resulted from explosive munitions. The extremities were the most commonly injured body region, involved in 43% of all injuries. The NISS associated with a 50% chance of survival rose each year from 32 in 2003 to 60 in 2012. CONCLUSION: An improvement in survival during the 10-year period is demonstrated. A majority of wounds are a result of explosive munitions, and the extremities are the most commonly affected body region. The authors recommend the development of more sophisticated techniques for the measuring of the performance of combat casualty care systems to include measures of morbidity and functional recovery as well as survival. LEVEL OF EVIDENCE: Epidemiologic study, level III.

Coronary artery problems and disease in adults with congenital heart disease: how to evaluate, how to prevent, how to treat.

There are a wide variety of coronary artery anomalies and disease in adults with congenital heart disease (CHD). In fact, the increasing burden of acquired coronary artery disease (CAD) has to be considered in addition to congenital abnormalities of the coronary arteries, isolated or associated to other congenital diseases. This is largely a consequence of the increasing number of patients reaching older age. Due to complex underlying cardiac anatomy, previous surgery and comorbidities, treatment can be challenging. Individualized and multidisciplinary management involving congenital heart cardiologists, cardiac surgeons, coronary interventionists and imaging specialists is essential. This review gives an overview of coronary artery involvement in adults with CHD, summarizes the current literature and focuses on prevention, diagnosis and treatment. The potential role of cardiovascular risk factors for CAD is also discussed.

Post-radiotherapy radionecrosis of the temporal bone resulting in delayed CSF otorrhoea: a case report.

The authors report a case of temporal glioblastoma multiforme, who received post-operative radiotherapy. The patient presented to neurosurgery 8 months post-radiotherapy with CSF otorrhoea. This was shown to be from a defect of the right tegmen tympani secondary to radionecrosis of the petrous temporal bone. This was successfully repaired via craniotomy and repair of the middle cranial fossa floor. The authors have been unable to find previously published examples of isolated radio-necrosis of the temporal bone following radiotherapy for glioblastoma multiforme.

Results of screening for intracranial aneurysms in patients with coarctation of the aorta.

BACKGROUND AND PURPOSE: IAs are found in 2.3% of adults; the mean age at detection is 52 years. Prevalence is <0.5% in young adults. Early studies suggest that 10%-50% of patients with aortic coarctation have IAs. Screening recommendations are variable. We sought to examine the prevalence of IAs through screening with MRA. MATERIALS AND METHODS: Consecutive patients older than 16 years of age with coarctation undergoing brain MRA between May 1999 and October 2007 were included. MRA was performed by using a 1.5T scanner with a 3D time-of-flight protocol; simultaneous MR imaging was performed of the heart and aorta. Cerebral MRAs were double-reported by a neuroradiologist. Statistics are described as mean ± SD and median ± range. Continuous variables were compared by using Student t tests and Mann-Whitney U tests (categoric variables, by using the Fisher exact test). RESULTS: One hundred seventeen MRAs were double-reported. The median age was 29 ± 11 years (range, 16-59 years). IAs were found in 12 patients (10.3%). The mean diameter of IAs was 3.9 mm (range, 2.0-8.0 mm). Patients with aneurysms were older (median, 37 years; range, 16-50 years) than those without (median, 23 years; range, 16-59 years; Z = -2.01, P = .04). Hypertension was more common in those with IAs (IA 83% versus no IA 43%, P = .01). There was no association between ascending aortopathy, bicuspid aortic valves, and IAs. CONCLUSIONS: Patients with coarctation have a higher prevalence of IAs, occurring at an earlier age than in population studies. Whether routine screening is appropriate for this group of patients is unclear. Hypertension is likely to be an important pathophysiologic factor.

Deep brain stimulation of the subthalamic nucleus for advanced Parkinson disease using general anesthesia: long-term results.

OBJECT: The authors analyze long-term outcome in a substantial number of patients who underwent subthalamic nucleus (STN) deep brain stimulation (DBS) surgery under general anesthesia. METHODS: Eighty-two patients underwent bilateral placement of DBS electrodes under general anesthesia for advanced Parkinson disease; the STN was the target in all cases. All patients underwent intraoperative microelectrode recording of the STN. No intraoperative macrostimulation was performed. Unified Parkinson's Disease Rating Scale (UPDRS) data were recorded in 28 patients. Assessment of outcome was performed using the UPDRS (in 28 cases), the electrophysiological recordings (in all 82 cases), medication reduction (in 78 cases), and complications (in 82 cases). RESULTS: There was improvement in UPDRS scores across all measures following surgery. The total UPDRS score, off medication, improved from 68.78 (geometrical mean, 95% CI 61.76-76.60) preoperatively to 45.89 (geometrical mean, 95% CI 34.86-60.41) at 1 year postoperatively (p = 0.003, data available in 26 patients). Improvements were obtained in UPDRS Part II (Activities of Daily Living) off medication (p = 0.001) and also UPDRS Part III (Motor Examination) off medication (p < 0.001). Results for the on-medication and on-stimulation states also showed a statistically significant improvement for UPDRS Part III (p = 0.047). Good microelectrode recording of the STN was obtained under general anesthesia; the median first-track length was 4.0 mm, and the median number of tracks passed per patient was 3.0. The median reduction in levodopa medication was 58.1% (interquartile range 42.9%-73.3%). One patient had an intracerebral hemorrhage in the track of 1 electrode but did not require surgical evacuation. One patient had generalized convulsive seizures 24 hours postoperatively and was intubated for seizure control. Unified Parkinson's Disease Rating Scale scores were obtained in 26 patients at 1 year, 28 patients at 3 years, 17 at 5 years, and 7 at 7 years postoperatively. Up to 7 years postoperatively, there was sustained improvement in the total UPDRS score. The results in these patients showed minimal deterioration in the motor section of the UPDRS over time, up to 7 years following the operation. The authors found no evidence that the UPDRS Part II scores changed significantly over the period of 1-7 years after surgery (p = 0.671, comparison of mean scores at 1 and 7 years using generalized estimating equations). CONCLUSIONS: Long-term outcomes confirm that it is both safe and effective to perform STN DBS under general anesthesia. As part of patient choice, this option should be offered to all DBS candidates with advanced Parkinson disease to enable more of these patients to undergo this beneficial surgery.

Diagnosis and management of elite young athletes undergoing arrhythmia intervention.

BACKGROUND: Sudden cardiac death is the most common cause of mortality in young athletes. In some of these, the final pathway is arrhythmia. The authors aimed to identify the incidence, diagnosis and management of athletes undergoing investigation and intervention for cardiac arrhythmias. METHODS: Retrospective analysis of all patients between 10 and 17 years presenting to a supra-regional paediatric cardiac unit for investigation and intervention for a cardiac arrhythmia. Elite athletes (county and national level) were identified from the departmental clinical and arrhythmia databases (October 1997-2007). Patients with significant congenital heart disease were excluded. RESULTS: From 657 patients undergoing 680 interventions, 324 were excluded. From the remaining 333 we identified 11 elite athletes - football (n=3), martial arts (n=2), rugby (n=2), triple jump, netball, canoeing, and motor sport (n=1). Presenting symptoms included palpitations (n=8) and syncope (n=1). Two were asymptomatic and investigated following routine screening. Diagnoses included atrioventricular (AV) re-entry tachycardia (n=3), AV node re-entry tachycardia (n=4), complete heart block (n=1), sinus node dysfunction (n=1), vasovagal syncope (n=1) and pre-excited atrial fibrillation (n=1). Arrhythmia interventions included implantable loop recorder (n=2), diagnostic electrophysiology study (n=9), including radiofrequency ablation (n=5), cryoablation (n=2) and pacemaker implantation (n=2). Following intervention, 10 children returned to competitive sport. There were no deaths. No child required long-term medication post-intervention. CONCLUSION: Of the young competitive athletes identified from the authors' study, there was a high incidence of significant arrhythmias. Intervention is usually successful and most athletes return to elite sport without the need for long-term medication.

Severe pulmonary hypertension: a frequent complication of stem cell transplantation for malignant infantile osteopetrosis.

This report describes eight infants who developed acute severe pulmonary arterial hypertension (PAH) at days -2 to +89 after allogeneic stem cell transplantation (SCT) for malignant infantile osteopetrosis (MIOP). They were taken from a total of 28 children (frequency 29%) transplanted for this disease at three institutions between 1996 and 2002. Typical presentations were acute dyspnoea, hypoxia and brady/tachycardia usually in the absence of fever, crepitations or other evidence of infection. Six patients (75%) required assisted ventilation and five (62%) died. There was clinical or pathological evidence of veno-occlusive disease (VOD) in three children, but absence of VOD in the remaining five suggests that a separate disease process may be responsible for the PAH. Responses to nitric oxide (NO), defibrotide (DF), nicardipine and steroids in varying combinations were disappointing. Three children showed sustained improvement after administration of epoprostenol (EP, prostacyclin) in conjunction with NO and/or DF and remain well and free of PAH 25, 31 and 32 months post-transplant. PAH must therefore be excluded in any child who becomes acutely breathless after SCT for osteopetrosis.

Glucose export from the brain in man: evidence for a role for astrocytic glycogen as a reservoir of glucose for neural metabolism.

We report transients of net export of glucose from brain with jugular venous glucose content exceeding arterial by up to 5.3 mmol.l-1 in 19 children undergoing cardiopulmonary bypass surgery. The magnitude of the release implies the presence of a glucose reservoir in brain, the most likely candidate being astrocytic glycogen. These data provide evidence that astrocytic glycogen can be exported as glucose, permitting spatial and temporal modulation of glucose delivery to neurons.

Cytogenetic abnormalities in a disseminated medulloblastoma.

A 3-year-old girl developed central nervous system, bone and bone marrow metastases, and hypercalcaemia shortly after presentation with medulloblastoma. Cytogenetic studies of the involved bone marrow showed multiple abnormalities including iso(17q). This chromosome rearrangement has been reported in other cases of recurrent or disseminated medulloblastoma. More studies are required relating the karyotypes of medulloblastomas to long-term outcome to determine if the presence of iso(17q) is a prognostic factor in this malignancy.