• Lelio Guida, Sandro Benichi, Marie Bourgeois, Giovanna Paternoster, Syril James, Timothée De Saint Denis, Dangouloff RosVolodiaVDepartment of Pediatric Radiology, APHP, Hôpital Necker Enfants Malades, Paris , France.Université Paris Cité, UMR 1163, Institut Imagine, Paris , France., Kevin Beccaria, and Thomas Blauwblomme.
• Department of Pediatric Neurosurgery, APHP, Hôpital Necker Enfants Malades, Paris , France.
• Neurosurgery. 2023 Sep 1; 93 (3): 576585576-585.

BackgroundHydrocephalus frequently occurs with midline posterior fossa cystic collections. The classification of this heterogeneous group of developmental anomalies, including Dandy-Walker malformation, persisting Blake's pouch, retrocerebellar arachnoid cysts, and mega cisterna magna, is subject of debate. The absence of diagnostic criteria is confusing regarding the ideal management of PFCC-related hydrocephalus.ObjectiveTo decipher the surgical strategy for the treatment of children with PFCC-related hydrocephalus through a retrospective analysis of the surgical outcome driven by their clinical and radiological presentation.MethodsThis study enrolled patients operated of symptomatic PFCC-related hydrocephalus. Clinical and MRI features were examined, as well as the surgical outcome. Unbiased subgroup classification of the patients was performed with multiple component analysis as a function of imaging characteristics and hierarchical clustering on principal component. Outcome was assessed with binomial logistic regression and Kaplan-Meier analysis.ResultsFifty-four patients were included between 2007 and 2021. Multiple component analysis suggested that cerebellar and vermian hypoplasia, vermian rotation, basal-tentorial angle, and fastigial angle were strongly correlated. Hierarchical clustering and the distribution of the patients in the bidimensional plot showed the clear segregation of 3 major clusters, which correlated with the radiological diagnosis ( P < .01). Binomial logistic regression and survival analysis showed that endoscopic third ventriculostomy was an effective treatment for patients with persisting Blake's pouch, while failing to control hydrocephalus in most of patients with Dandy-Walker malformation.ConclusionPreoperative MRI in patients with PFCC-related hydrocephalus is essential to better define the diagnosis. The choice of treatment strategy notably relies on correct radiological diagnosis.Copyright © Congress of Neurological Surgeons 2023. All rights reserved.

29 keywords...

hide…