Graham Fieggen

Congenital malformations of the brain--a neurosurgical perspective at the close of the twentieth century.

Abstract Current classifications, indications for surgery, operative options and outcome statistics available to neurosurgeons for the management of congenital encephaloceles, arachnoid cysts and the Dandy-Walker complex are reviewed.

SIOP PODC adapted treatment recommendations for standard-risk medulloblastoma in low and middle income settings

Effective treatment of children with medulloblastoma requires a functioning multi‐disciplinary team with adequate neurosurgical, neuroradiological, pathological, radiotherapy and chemotherapy facilities and personnel. In addition the treating centre should have the capacity to effectively screen and manage any tumour and treatment‐associated complications. These requirements have made it difficult for many low and middle‐income countries (LMIC) centres to offer curative treatment. This article provides management recommendations for children with standard‐risk medulloblastoma (localised tumours in children over the age of 3–5 years) according to the level of facilities available. Pediatr Blood Cancer 2015;62:553–564.

Spinal cord involvement in pygopagus conjoined twins: case report and review of the literature

Introduction.Pygopagus conjoined twins are joined at the rump and have union of the gastrointestinal and genitourinary systems as well occasional involvement of neural elements.Case report.We report a case where the terminal spinal cords were fused, despite which there was only a minor motor deficit in the lower limbs after separation. The pre-operative and intra-operative findings are described and issues of long-term follow-up discussed.Discussion.Seven other cases reported in the literature with varying degrees of involvement of the spinal cord and cauda equina are reviewed.

Transition from child- to adult-orientated care for children with long-term health conditions: A process, not an event

Long-term health conditions in childhood include both congenital conditions and acquired diseases. Children with long-term health conditions face issues and potential secondary problems that are different from those of adults with chronic diseases. Transition to adult-orientated care for such children and adolescents is a major challenge. Transition needs to be prepared for and planned. A variety of possible transition models exists, depending on circumstances.

Clinical characteristics and neurodevelopmental outcomes of children with tuberculous meningitis and hydrocephalus

Tuberculous meningitis (TBM) is a lethal and commonly occurring form of extra‐pulmonary tuberculosis in children, often complicated by hydrocephalus which worsens outcome. Despite high mortality and morbidity, little data on the impact on neurodevelopment exists. We examined the clinical characteristics, and clinical and neurodevelopmental outcomes of TBM and hydrocephalus.

Pulsatile Dynamics of the Optic Nerve Sheath and Intracranial Pressure: An Exploratory In Vivo Investigation.

BACKGROUND: Raised intracranial pressure (ICP) may lead to increased stiffness of the optic nerve sheath (ONS). OBJECTIVE: To develop a method for analyzing ONS dynamics from transorbital ultrasound and investigate a potential difference between patients with raised ICP vs normal ICP. METHODS: We retrospectively analyzed data from 16 patients (≤12 years old) for whom ultrasound image sequences of the ONS had been acquired from both eyes just before invasive measurement of ICP. Eight patients had an ICP ≥20 mm Hg. The transverse motion on each side of the ONS was estimated from ultrasound, and Fourier analysis was used to extract the magnitude of the displacement corresponding to the heart rate. By calculating the normalized absolute difference between the displacements on each side of the ONS, a measure of ONS deformation was obtained. This parameter was referred to as the deformability index. According to our hypothesis, because deformability is inversely related to stiffness, we expected this parameter to be lower for ICP ≥20 mm Hg compared with ICP <20 mm Hg. The one-sided Mann-Whitney U test was used for statistical comparison. RESULTS: The deformability index was significantly lower in the group with ICP ≥20 mm Hg (median value 0.11 vs 0.24; P = .002). CONCLUSION: We present a method for assessment of ONS pulsatile dynamics using transorbital ultrasound imaging. A significant difference was noted between the patient groups, indicating that deformability of the ONS may be relevant as a noninvasive marker of raised ICP. The clinical implications are promising and should be investigated in future clinical studies. ABBREVIATIONS: AUC, area under curve ICP, intracranial pressure ONS, optic nerve sheath ONSD, optic nerve sheath diameter ROC, receiver operating characteristic

Spina bifida: a multidisciplinary perspective on a many-faceted condition.

Open spina bifida or myelomeningocele (SBM) is the most common birth defect involving the central nervous system, second only in incidence to congenital cardiac disease. Outcomes in this disorder were poor until the mid-20th century, when modern neurosurgical techniques (closing the lesion and treating hydrocephalus) and treatment for the neuropathic bladder addressed the major causes of mortality, although SBM may still be poorly treated in the developing world. Initial management - or mismanagement - has a profound impact on survival and long-term quality of life.

Hydatid disease of the spine in South African children.

We present three patients with neurologic complications of the spine from hydatid disease. The first was a 6-year-old girl with lower limb paralysis evolving over 2 weeks. Neuroimaging revealed a cystic mass compressing the spinal cord at the level of T8 and extending from the vertebral body. She underwent surgical decompression. Histopathologic examination confirmed hydatid disease. At 6-month follow-up, functional improvement had occurred with full ambulation. She continues on long-term albendazole therapy. Two other patients are described, one with primary spinal disease and the other with cerebral disease and secondary seeding to the spine. Spinal hydatid disease is a rarity, even more so in children. Although secondary disease, primarily affecting bone, carries a poorer long-term outlook, the first patient made a dramatic recovery and has raised therapeutic dilemmas as to the total duration of continuing albendazole therapy. The literature documents some 37 reports, mostly in adults. Considering the frequency of hydatid disease in South Africa, the incidence in our population could be under-recorded. Unless the clinician includes spinal hydatid disease, in endemic areas, as part of the differential list for paralysis and considers performing neuroimaging, this potentially treatable diagnosis will be missed. (J Child Neurol 2003;18:343—346).

Global hydrocephalus epidemiology and incidence: systematic review and meta-analysis

OBJECTIVEHydrocephalus is one of the most common brain disorders, yet a reliable assessment of the global burden of disease is lacking. The authors sought a reliable estimate of the prevalence and annual incidence of hydrocephalus worldwide.METHODSThe authors performed a systematic literature review and meta-analysis to estimate the incidence of congenital hydrocephalus by WHO region and World Bank income level using the MEDLINE/PubMed and Cochrane Database of Systematic Reviews databases. A global estimate of pediatric hydrocephalus was obtained by adding acquired forms of childhood hydrocephalus to the baseline congenital figures using neural tube defect (NTD) registry data and known proportions of posthemorrhagic and postinfectious cases. Adult forms of hydrocephalus were also examined qualitatively.RESULTSSeventy-eight articles were included from the systematic review, representative of all WHO regions and each income level. The pooled incidence of congenital hydrocephalus was highest in Africa and Latin America (145 and 316 per 100,000 births, respectively) and lowest in the United States/Canada (68 per 100,000 births) (p for interaction < 0.1). The incidence was higher in low- and middle-income countries (123 per 100,000 births; 95% CI 98-152 births) than in high-income countries (79 per 100,000 births; 95% CI 68-90 births) (p for interaction < 0.01). While likely representing an underestimate, this model predicts that each year, nearly 400,000 new cases of pediatric hydrocephalus will develop worldwide. The greatest burden of disease falls on the African, Latin American, and Southeast Asian regions, accounting for three-quarters of the total volume of new cases. The high crude birth rate, greater proportion of patients with postinfectious etiology, and higher incidence of NTDs all contribute to a case volume in low- and middle-income countries that outweighs that in high-income countries by more than 20-fold. Global estimates of adult and other forms of acquired hydrocephalus are lacking.CONCLUSIONSFor the first time in a global model, the annual incidence of pediatric hydrocephalus is estimated. Low- and middle-income countries incur the greatest burden of disease, particularly those within the African and Latin American regions. Reliable incidence and burden figures for adult forms of hydrocephalus are absent in the literature and warrant specific investigation. A global effort to address hydrocephalus in regions with the greatest demand is imperative to reduce disease incidence, morbidity, mortality, and disparities of access to treatment.

Intraoperative ultrasound-guidance in neurosurgery.

ince the first recorded use of the “ultrasonoscope” for localizing subcortical intracerebral neoplasms in postmorS tem specimens in 1950 (4), technological advances in ultrasound have seen a transformation from poor-quality, A-mode wave forms to superb-quality, real-time images in 2 (2D) and 3 (3D) dimensions. As a result, intraoperative ultrasonography (IOUS) has undergone remarkable development, particularly in the last 2 decades.