Management of Dandy-Walker complex-associated infant hydrocephalus by combined endoscopic third ventriculostomy and

Benjamin C Warf1, Michael Dewan, John Mugamba

  • 1Department of Neurosurgery, Children's Hospital Boston and Harvard Medical School, Boston, Massachusetts 02115, USA. benjamin.warf@childrens.harvard.edu

Insights

Endoscopic third ventriculostomy with or without choroid plexus cauterization (ETV/CPC) offers a high success rate for treating hydrocephalus associated with Dandy-Walker complex (DWC). This endoscopic approach is a viable alternative to shunting, reducing shunt dependence in affected infants.

Area of Science:

  • Pediatric Neurosurgery
  • Congenital Neurological Disorders
  • Hydrocephalus Management

Background:

  • Dandy-Walker complex (DWC) encompasses a spectrum of congenital brain anomalies including Dandy-Walker malformation (DWM), Dandy-Walker variant (DWV), Blake pouch cyst, and mega cisterna magna (MCM).
  • Hydrocephalus is a common complication of DWC, historically managed with shunting procedures, often leading to long-term shunt dependence.
  • Endoscopic third ventriculostomy (ETV) has been infrequently reported for DWC-associated hydrocephalus.

Purpose of the Study:

  • To evaluate the efficacy of ETV, particularly combined ETV and choroid plexus cauterization (ETV/CPC), as a primary treatment for hydrocephalus in infants with DWC.
  • To report the largest series to date on ETV/CPC for DWC-associated hydrocephalus.
  • To compare ETV/CPC outcomes across different subtypes of DWC.

Main Methods:

  • Retrospective review of a clinical database from 2004-2010 at CURE Children's Hospital of Uganda.
  • Identification of 45 pediatric patients diagnosed with DWC (DWM, DWV, MCM) and hydrocephalus, treated with ETV/CPC.
  • Exclusion of three patients with alternative causes of hydrocephalus (post-infectious, post-hemorrhagic).

Main Results:

  • The study included 45 patients, with a median age of 5 months at treatment; 88% were under 12 months.
  • ETV/CPC achieved a 74% success rate (no further operations required) with a mean follow-up of 24.2 months.
  • Success rates were 74% for DWM, 73% for DWV, and 100% for MCM, with 95% maintaining an open aqueduct and no need for posterior fossa shunting.

Conclusions:

  • Endoscopic treatment, specifically ETV/CPC, should be strongly considered as the primary management for DWC-associated hydrocephalus.
  • This endoscopic approach offers a successful alternative to traditional shunting, potentially avoiding shunt dependence.
  • ETV/CPC demonstrates favorable outcomes across various DWC subtypes in young infants.
Abstract

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