Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants.

[hydrocephalus with stenosis of the aqueduct of sylvius]

The authors have previously reported on the overall improved efficacy of endoscopic third ventriculostomy (ETV ) combined with choroid plexus cauterization (CPC ) for infants younger than 1 year of age . In the present study they specifically examined the long -term efficacy of ETV with or without CPC in 35 infants with congenital aqueduct stenosis treated at CURE Children 's Hospital of Uganda during the years 2001 -2006 .Infants with congenital aqueductal stenosis were treated during 2 distinct treatment epochs : all underwent ETV alone , and subsequently all underwent ETV-CPC . Prospectively collected data in the clinical database were reviewed for all infants with an age < 1 year who had been treated for hydrocephalus due to congenital aqueductal stenosis . Study exclusion criteria included : 1 ) a history or findings on imaging or at the time of ventriculoscopy that suggested a possible infectious cause of the hydrocephalus , including scarred choroid plexus ; 2 ) an open aqueduct or an aqueduct obstructed by a membrane or cyst rather than by stenosis ; 3 ) severe malformations of the cerebral hemispheres including hydranencephaly , significant segments of undeveloped brain , or schizencephaly ; 4 ) myelomeningocele , encephalocele , Dandy -Walker complex , or tumor ; or 5 ) previous shunt insertion . The time to treatment failure was analyzed using the Kaplan-Meier method to construct survival curves . Log-rank (Mantel-Cox ) and Gehan-Breslow-Wilcoxon tests were used to determine whether differences between the 2 treatment groups were significant .Thirty -five patients met the study criteria . Endoscopic third ventriculostomy alone was performed in 12 patients (mean age 4 .7 months ), and combined ETV-CPC was performed in 23 patients (mean age 3 .5 months ). For patients without treatment failure , the mean and median follow-ups were , respectively , 51 .6 and 48 .0 months in the ETV group and 31 .2 and 26 .4 months in the ETV-CPC group . Treatment was successful in 48 .6 % of the patients who underwent ETV alone , as accurately predicted by the Endoscopic Third Ventriculostomy Success Score (ETVSS ), and in 81 .9 % of the patients who underwent ETV-CPC (p = 0 .0119 , log-rank test ; p = 0 .0041 , Gehan-Breslow-Wilcoxon test ; HR 6 .42 [95 % CI 1 .51 -27 .36 ]).Combined ETV-CPC is significantly superior to ETV alone for infants younger than 1 year of age with congenital aqueductal stenosis . The fact that the outcome for ETV alone was accurately predicted by the ETVSS suggests that these results are applicable in developed countries .

Diseases
Validation

Diseases presenting "myelomeningocele" symptom

22q11.2 deletion syndrome

congenital diaphragmatic hernia

hirschsprung disease

hydrocephalus with stenosis of the aqueduct of sylvius

This symptom has already been validated