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Timothy W. Vogel, Biji Bahuleyan, Shenandoah Robinson and Alan R. Cohen

Object

Hydrocephalus remains a major public health problem. Conventional treatment has relied on extracranial shunting of CSF to another systemic site, but this approach is associated with a high rate of complications. Endoscopic third ventriculostomy (ETV) is a novel treatment for select forms of hydrocephalus that can eliminate the need for implantation of a lifelong ventricular shunt system. However, the indications for ETV are contested and its long-term effectiveness is not well established.

Methods

The authors selected 100 consecutive patients who underwent ETV for hydrocephalus beginning in 1994. Patients were enrolled and treated at a single institution by a single surgeon. The primary outcome was success of ETV, with success defined as no need for subsequent surgery for hydrocephalus.

Results

Ninety-five patients satisfied the inclusion criteria. The mean follow-up period was 5.1 years (median 4.7 years) with follow-up data available for as long as 17 years. Patients commonly presented with headache (85%), ataxia (34%), emesis (29%), and changes in vision (27%). The success rate for ETV was 75%. Twenty-one patients (22%) in the series had malfunctioning shunts preoperatively and 13 (62%) were successfully treated with ETV. Preoperative inferior bowing of the third ventricle floor on MRI was significantly associated with ETV success (p < 0.05).

Conclusions

Endoscopic third ventriculostomy is an effective and durable treatment for select patients with hydrocephalus. When successful, the procedure eliminates the lifelong complications associated with implanted ventricular shunts.

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John Weaver, Sunil Manjila, Biji Bahuleyan, Barbara A. Bangert and Alan R. Cohen

Object

Rhombencephalosynapsis (RS) is a rare congenital posterior fossa malformation characterized by dorsal fusion of the cerebellar hemispheres, hypogenesis or agenesis of the vermis, and fusion of the dentate nuclei and superior cerebellar peduncles. The objective of this institutional study is to review the clinical conditions associated with RS and analyze the varied biological profile of this unique condition.

Methods

The study data were collected retrospectively from the medical records of patients at Rainbow Babies and Children's Hospital. After required institutional review board approval, the authors obtained information regarding the cases of RS reviewed by the Departments of Radiology, Genetics, and/or Pediatric Neurology. Medical charts were systematically reviewed, and 9 patients were analyzed in detail.

Results

The authors describe 6 cases of RS and 3 cases of partial RS. This case series demonstrates an association between RS and symptomatic hydrocephalus (7 of 9 patients) and RS and Chiari malformation (5 of 9 patients). Patients with symptomatic hydrocephalus underwent endoscopic third ventriculostomy or ventriculoperitoneal shunt insertion. One of the patients with an associated Chiari malformation underwent foramen magnum decompression.

Conclusions

The authors present a large case series of RS. Patients with RS often had hydrocephalus and/or a Chiari Type I or II hindbrain malformation. Neuroimaging findings of RS are presented along with hypotheses to explain the embryopathology of this unusual condition.

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Clarence S. Greene Jr.

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Biji Bahuleyan, Girish Menon, Easwer Hariharan V, Mridul Sharma and Suresh Nair

Symptomatic subdural hygroma due to foramen magnum decompression for Chiari malformation Type I is extremely rare. The authors present their experience with 2 patients harboring such lesions and discuss treatment issues. They conclude that the possibility of subdural hygromas should be considered in all patients presenting with increased intracranial tension following foramen magnum decompression for Chiari malformation Type I. Immediate neuroimaging and appropriate surgical intervention provides a good outcome.

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Biji Bahuleyan, Sunil Manjila, Shenandoah Robinson and Alan R. Cohen

Object

Surgery for medically intractable epilepsy secondary to unihemispheric pathology has evolved from more aggressive hemispherectomy to less aggressive variations of hemispherotomy. The authors propose a novel minimally invasive endoscopic hemispherotomy that should give results comparable to conventional open craniotomy and microsurgery.

Methods

Endoscopic transventricular hemispherotomy was performed in 5 silicon-injected cadaveric heads in the authors' minimally invasive neurosurgery laboratory. The lateral ventricle was accessed endoscopically through a frontal and occipital bur hole. White matter disconnections were performed to unroof the temporal horn and to disconnect the frontobasal region, corpus callosum, and fornix.

Results

Using an endoscopic transventricular approach, all white matter disconnections were successfully performed in all 5 cadavers.

Conclusions

The authors have demonstrated the feasibility of endoscopic transventricular hemispherotomy in a cadaveric model. The technique is simple and could be useful in a subgroup of patients with parenchymal volume loss and ventriculomegaly.

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Oral Presentations

2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010