Congenital idiopathic hydrocephalus of infancy: the results of treatment by endoscopic third ventriculostomy with or without choroid plexus cauterization and suggestions for how it works.

BACKGROUND: The traditional bulk flow model of communicating hydrocephalus does not support treatment by endoscopic third ventriculostomy (ETV), but successful treatment is reported in adults. This study is the first to report long-term success of ETV +/- choroid plexus cauterization (CPC) for infants with congenital idiopathic hydrocephalus (CIH). The results are interpreted in the context of current models for hydrocephalus.
METHODS: The CURE Children's Hospital of Uganda prospective database was reviewed for the years 2001-2006 to identify children <24 months of age meeting the criteria for CIH who underwent ETV with or without bilateral CPC. Kaplan-Meier method was used to assess treatment success survival. Gehan-Breslow-Wilcoxin and logrank tests were used to determine significance of survival differences. Fisher's exact test was used to determine the significance of differences between groups.
RESULTS: Sixty-four infants (mean/median age, 6.1/5.0 months) met the inclusion criteria. Sixteen consecutive patients were treated by ETV alone, and the subsequent 48 by ETV/CPC (mean/median follow-up 34.4/36.0 months). ETV was successful in 20 % and ETV/CPC in 72.4 % at 4 years (p < 0.0002, logrank test; p = 0.0006, Gehan-Breslow-Wilcoxin; hazard ratio 6.9, 95 % CI 2.5-19.3).
CONCLUSIONS: ETV/CPC was significantly more successful than ETV alone in treating CIH. The primary effect of ETV, as a pulsation absorber, and of CPC, as a pulsation reducer, may be to abate the net force of intraventricular pulsations that produce ventricular expansion. ETV alone may be less successful for infants because of greater brain compliance. ETV/CPC should be considered an effective primary treatment option.