Rare Diseases Symptoms Automatic Extraction

Is there an indication for ETV in young infants in aetiologies other than isolated aqueduct stenosis?

[hydrocephalus with stenosis of the aqueduct of sylvius]

The objective was to determine whether endoscopic third ventriculostomy (ETV) has a role in the management of obstructive hydrocephalus in aetiologies other than idiopathic aqueduct stenosis (AS) in infants.In addition to reviewing the literature, we performed a retrospective analysis of our endoscopy database, which was established in 1998, and analysed the outcome results of all cases of ETV in those under 1 year of age which were performed between 1998 and 2003. We included cases of idiopathic AS in the outcome analysis as a benchmark of successful outcome. Successful outcome was assessed by resolution of the presenting clinical features and shunt freedom.Aetiologies that were identified included, in addition to idiopathic AS, suprasellar arachnoid cysts, AS in association with post-haemorrhagic hydrocephalus (PHH), post-infectious hydrocephalus (PIH), tumour-related hydrocephalus and a heterogenous group including Dandy-Walker malformation and other developmental anomalies. Suprasellar arachnoid cysts had a 100% outcome success. Idiopathic AS had a 50% outcome success and the successful outcome of PHH cases was poor at 18%. A statistical analysis revealed no effect of age at the time of ETV on the outcome in the aetiological groups.The definitive initial neurosurgical management of suprasellar arachnoid cysts causing significant hydrocephalus is ETV, whereas that for PHH and PIH is probably that of a ventriculo-peritoneal shunt placement. We speculate that there may be a sub-group of AS cases, termed pure or idiopathic AS, which have a higher rate of successful outcome that may be durable from a younger age. The role of repeat ETV is controversial.