Encephaloduroateriosynangiosis (EDAS) in the management of Moyamoya syndrome in children with sickle cell disease.

BACKGROUND: Encephalo-duro-arterio-synangiosis (EDAS) in Moyamoya syndrome (MMS) treatment has been well described in the literature, however in MMS caused by sickle cell anaemia (SCA), EDAS use remains controversial with poor long-term follow-up. We present a case-series of SCA patients who have undergone EDAS for SCA-related MMS and describe their post-operative course as well as provide a literature review of the role of EDAS in the treatment of sickle cell anaemia. METHODS: A retrospective review of all the paediatric EDAS procedures conducted in our institution for SCA from 2007 to 2015. Two patients underwent behavioural screening. RESULTS: A total of eight patients with MMS secondary to SCA underwent EDAS. Unilateral EDAS was performed without complication in seven patients. One patient underwent bilateral EDAS but with a two-year gap in between procedures. Follow-up magnetic resonance angiography demonstrated no progression of Moyamoya collaterals or further ischaemic events with regression of collaterals clearly visible in one patient. All patients have demonstrated a return to normal school activities. CONCLUSIONS: EDAS is a well-tolerated revascularisation procedure for children with MMS. The prevention of further infarcts in our group with sickle cell disease has allowed these children to resume normal school activities.

Neonatal post-haemorrhagic hydrocephalus in the UK: a survey of current practice.

OBJECTIVES: The management of neonatal post-haemorrhagic hydrocephalus (nPHH) varies considerably between centres and even between neurosurgeons in the same centre. We performed a nationwide survey to evaluate current practice in the UK and reviewed the evidence for each intervention. METHODS: An online semi-structured questionnaire was emailed to all units registered on the British Paediatric Neurosurgery Group Mailing List. The survey consisted of 18 questions designed to establish the status quo in the management of nPHH, including preferred temporising procedure, factors guiding the timing of intervention and willingness to participate in a prospective study. RESULTS: A total of 24 consultants responded to the survey. Ventricular access device (VAD), trans-fontanelle tap, ventriculosubgaleal shunt (VSGS) and lumbar puncture (LP) were used by 33, 25, 17 and 17%, respectively, as the first temporising measure. Almost all reported that the timing of this intervention was guided by increasing head circumference and tense fontanelle. If the first temporising procedure fails, VAD, external ventricular drainage (EVD), trans-fontanelle tap, VSGS and other procedures (including ventriculoperitoneal (VP) shunting and endoscopic third ventriculostomy (ETV)) were performed by 42, 17, 13, 8 and 21%, respectively. Almost all respondents reported that VP shunting would be their preferred method of definitive cerebrospinal fluid (CSF) diversion. Almost all responded that they would be willing to participate in a prospective study in the future to determine best practice. CONCLUSION: As expected there was a significant heterogeneity between respondents in the initial management of nPHH in the UK. VAD was the most popular first and second choice temporising measure. On reviewing the literature, it is clear that more work still needs to be done to establish which temporising measure is best. There is willingness in the UK to participate in a study to help determine this.

A review of spontaneous intracranial extradural hematoma in sickle-cell disease.

Sickle-cell disease is common among patients of Afro-Caribbean origin. Though it can precipitate neurological conditions, it only rarely causes neurosurgical problems, with very few reported cases. We describe the case of a 7-year-old girl with a background of sickle-cell disease (SCD) brought into an acute neurosurgical unit in extremis, signs of a raised ICP, and with no history of recent trauma. Following further investigations, an acute drop in the hemoglobin and hematocrit levels were noted, with the cause of her presentation being attributed to a sickling crisis causing skull convexity infarction and resulting in spontaneous bilateral extradural hematomas requiring emergency evacuation. We review the current literature and propose the pathophysiological mechanism behind this phenomenon.

Antibiotic resistant infections with antibiotic-impregnated Bactiseal catheters for ventriculoperitoneal shunts.

BACKGROUND: Shunts remain the commonest means by which hydrocephalus is treated. Despite the changes in valve and catheter technology, shunt infection and blockage are still a cause of great headache for the patient as well as the neurosurgeon. Antibiotic-laced catheters were proposed as a means by which to reduce shunt colonization and infection. METHODS: We present our experience of 52 months of Bactiseal catheters in all consecutive patients who underwent a ventriculoperitoneal (VP) shunt from July 2004 to November 2008, under the care of one neurosurgeon. This was a prospective study with outcome measures of infection, blockage, intra and postoperative complications and revision surgery. RESULTS: One hundred and twenty-five patients underwent VP shunting with Bactiseal catheters, with a combination of NSC, Strata and Burr Hole valves. The age range of the patients was from 1 week premature to 64-years old. Forty-two of the patients were paediatric. The aetiology for hydrocephalus included posterior fossa tumoursto intra-ventricular haemorrhage, post-meningitic hydrocephalus and aqueduct stenosis. The overall complication rate was 12%. The following complications occurred: blockage in two cases; haemorrhage and blockage in two cases; CSF leak in one case; infection in four cases; other in three cases (peritoneal adhesions, wound erosion and postoperative peritonitis). There were four infections in total (3.2%). All of these occurred within six months of implantation. All infections were caused by rifampicin resistant Staphylococcus epidermidis. CONCLUSION: In an era of increasing methicillin-resistant Staphylococcus aureus (MRSA) resistance and 'superbugs', is the use of antibiotic-laced catheters adding to the pool of resistant bacteria which may be harder to treat? Vigilance is required, as rare and resistant staphylococci strains occasionally can emerge as causative agents for VP shunt infections, in both adults and children, and their treatment can be difficult.

Intrathecal baclofen trials: complications and positive yield in a pediatric cohort.

OBJECT Intrathecal baclofen (ITB) is an effective management option for childhood hypertonia. Given the potential complications of implanted ITB pumps, trials of ITB are usually performed as part of the workup for ITB pumps. Two methods are used for ITB trials, lumbar puncture (LP) and catheter insertion into the intrathecal space. Little has been written to date on the number of positive trials and complications in trials. This study aimed to report the outcomes and complications in ITB trials for childhood hypertonia (dystonia, spastic, or mixed). METHODS A retrospective case notes review was conducted of all patients who underwent ITB trials at the Evelina London Children's Hospital between 2005 and 2012 (inclusive). Positive trials were defined as a reduction in Modified Ashworth Scale by a minimum of 1 point in at least 2 muscle groups and improvement reported by the caregivers in the areas of goals agreed upon between professionals and the families. RESULTS Our patient group comprised children with dystonia (n = 7), mixed spasticity/dystonia (n = 29), spasticity (n = 4), and pain (n = 1). A total of 47 trials were attempted in 41 children. Forty trials were successfully completed, with 39 being positive. Thirty-three were catheter trials, and 14 were LPs. The overall complication rate in the 47 attempted trials was 53%: 61% in catheter trials, and 36% in LP trials. This difference was not statistically significant. The most common complications were vomiting (n = 9) and CSF leak (n = 4). The most serious complication was meningitis (n = 1) in a catheter trial. No patients experienced a permanent injury. CONCLUSIONS There is a high risk of minor self-limiting complications with ITB trials, which needs to be factored into the decision process of progression to trials. The rate of positive trials in this study was 98%, of which 21% did not progress to pump implantation. While the authors would still advocate for ITB trials prior to ITB pump insertion to aid parental decision-making, this figure suggests that with good patient selection, ITB pumps could be placed without a preceding trial.

Malignant transformation in craniopharyngiomas.

BACKGROUND: Craniopharyngiomas are successfully managed with surgery and/or adjuvant chemoradiotherapy but have been documented to undergo malignant transformation (MT), albeit very rarely, with only 23 reported cases. The exact cause and pathogenesis of this MT are unknown, although the literature has suggested a possible correlation with radiotherapy. OBJECTIVE: To review the reported cases of malignancy, in particular looking at the incidence, tumor characteristics, previous treatment modalities, and median survival. METHODS: We conducted a PUBMED, SCOPUS, OVID SP, and INFORMA search with a combination of key words: craniopharyngioma, malignancy, transformation, neoplasm, radiation therapy, and anaplastic. We identified 23 cases relevant to our study. RESULTS: Median age at the time of diagnosis of malignant craniopharyngiomas was 31 years (range, 10-66 years); 52.6% of the patients were female. Histologically, the most common tumor types were squamous cell carcinoma (80.96%), with adamantinomatous cell type being the most common morphology (89.47%). We found that 21.7% of the cases were diagnosed as malignant craniopharyngioma at first biopsy. Of the rest, the median time from initial benign diagnosis to MT was 8.5 years (range, 3-55 years). Median overall survival after MT was 6 months (range, 2 weeks-5 years). Using the Spearman rank correlation, we found no correlation between the use of radiation therapy (correlation coefficient, -0.25; P < .05) or its dosage (correlation coefficient, -0.26; P < .05) and MT. CONCLUSION: Malignant craniopharyngiomas are rare and are associated with a poor prognosis. MTs occur years after the initial benign craniopharyngioma diagnosis and are associated with multiple benign craniopharyngioma recurrence. Results also show that, contrary to widespread belief, there is a poor correlation between radiotherapy and MT.

Clinical and microbiologic features guiding treatment recommendations for brain abscesses in children.

BACKGROUND: There are no guidelines for the management of brain abscesses in children, and there is a paucity of recent data describing clinical and microbiologic features. We aimed to identify factors affecting outcome to inform antibiotic recommendations. METHODS: From 1999 to 2009, 118 children presented with brain abscesses to 4 neurosurgical centers in the United Kingdom. Clinical, microbiologic and treatment data were collected. RESULTS: The commonest preceding infection was sinusitis, with 59% of all children receiving antibiotics before diagnosis. Nonspecific symptoms were common, with only 13% having the triad of fever, headache and focal neurological deficit. Time between symptom onset and diagnosis varied widely (median, 10 days; range, 0-44). Magnetic resonance imaging was more frequently diagnostic than computed tomography. The most frequent organisms were Streptococcus milleri (38%), except after penetrating head injury or neurosurgery, for which Staphylococcus aureus was most common. The commonest empiric antibiotics were ceftriaxone/cefotaxime and metronidazole, which offered effective antimicrobial therapy in up to 83% of cases. Metronidazole added benefit in a maximum of 7% of cases, with ceftriaxone/cefotaxime alone sufficient in at least 76% and in all cases with cyanotic congenital heart disease or meningitis. A carbapenem would have been effective in 90%. The case fatality rate was 6% (33% in the immunocompromised). Long-term neurological sequelae affected 35%. Age younger than 5 years and a Glasgow Coma Scale score ≤8 were associated with poor outcome at 6 months. CONCLUSIONS: We recommend ceftriaxone/cefotaxime and metronidazole as empiric treatment, although metronidazole may be unnecessary in many cases, with antistaphylococcal cover in cases of head trauma. Meropenem potentially would be a better choice in the immunocompromised. A prospective study of intravenous and oral treatment guided by clinical improvement is required beause 1-2 weeks of intravenous antibiotics during a total of 6 weeks may be sufficient in children.

Extracranial displacement of a ventricular catheter following trauma.

We report a case of complete extracranial displacement of the proximal part of a ventriculo-peritoneal shunt following trauma. The possible mechanism of the delayed displacement of the shunt is discussed. The importance of early recognition of this unusual cause of shunt dysfunction is considered.

Germinoma with synchronous lesions in the pineal and suprasellar regions.

BACKGROUND: Germinomas represent the most frequent tumour type in the pineal region and the synchronous involvement of the suprasellar region, frequently termed bifocal disease, is increasingly recognised. The incidence, pathogenesis, histomorphological, radiological, clinical features, and management strategies are discussed. CASE REPORT: A 6-year-old girl presented with clinical features of hypothalamic-pituitary axis dysfunction. MRI showed two distinct lesions in the pineal and suprasellar regions. Tumour markers for germ cell tumours from blood and cerebrospinal fluid were within the normal range. The suprasellar lesion was biopsied and the histology revealed a germinoma. She received a radical course of radiotherapy covering the entire craniospinal axis (24 Gy/15#) followed by focal boost to the sites of primary disease (16 Gy/10#). Three months after the completion of treatment, the patient achieved a complete clinical and radiological response, which was maintained during the 13-month follow-up period. CONCLUSIONS: The optimum management of primary intracranial pineal and suprasellar (bifocal) germinomas still remains controversial. The definition as either localised or disseminated disease has major implications on required treatment and its associated late morbidity. Prospective studies with large cooperative groups worldwide and consensus meetings may reduce uncertainties regarding available management options.