Surgical outcomes of congenital arachnoid cysts and their association with neurodevelopmental disorders.

BACKGROUND

The aim of this study is to evaluate the surgical outcomes of arachnoid cyst decompression in patients less than 16 years of age with an associated neurodevelopmental disorder (NDD).

METHODS

A retrospective analysis of surgically treated congenital arachnoid cyst within South Wales between 2010-2019. Surgical outcomes are measured according to clinical (Clinical Outcome Score [COG]) and radiological (Neuroimaging Outcome Score [NOG]) outcomes and the complication profiles are described. The association between arachnoid cysts and NDD both in South Wales and the literature review cohort is also discussed.

RESULTS

The prevalence of congenital arachnoid cysts in South Wales is 0.02% and their association with neurodevelopmental disorders among patients with a congenital arachnoid cyst is high (estimated prevalence 19%). Eighteen (51%) in total were surgically treated in our unit; seven with an associated NDD. All surgical modalities showed improved clinical and radiological outcomes in those with an associated neurodevelopmental disorder, four (15%) patients who had endoscopic treatment, and two (7%) of those who underwent craniotomy had complications. No complications following cystoperitoneal shunt were seen. Four (21%) surgically treated patients were ultimately shunted following initial treatment by endoscopy or craniotomy.

CONCLUSIONS

In a cohort of patients diagnosed with a congenital arachnoid cyst in association with a neurodevelopmental disorder, all surgical treatments show improved clinical and radiological outcomes. Cystoperitoneal shunting appears to have a favourable complication profile.