Developmental Retrocerebellar Cysts: A New Classification for Neuroendoscopic Management and Systematic Review

Posterior fossa cystic malformations are diversely classified with considerable overlap without therapeutic relevance. These cysts posterior to the cerebellum, presenting in children younger than 5 years, are labeled developmental retrocerebellar cysts (DRCCs) under a new classification in relation to neuroendoscopy.

DRCC was categorized as type 0 for asymptomatic enlarged cistern magna and was not treated. Among symptomatic cases, cysts with a compressed fourth ventricle were labeled type 1, whereas cysts in continuity with the fourth ventricle were termed type 2. They were further categorized as subtype B if hydrocephalus was greater relative to the cyst, or otherwise as subtype A. The literature was reviewed according to PRISMA guidelines.

There were 13 children aged 3-48 months. Type 1A DRCC was noted in 5 patients, with onset before 6 months, 4 of whom (80%) had intracranial hypertension. All underwent suboccipital endoscopic deroofing and cisternostomy (SEDC), a new technique. Type 1B DRCC was seen in 2 patients, with onset at 8-9 months, who underwent endoscopic third ventriculostomy (ETV) + endoscopic ventriculocystostomy (EVC). Type 2A DRCC was observed in 4 patients, with onset at 5-47 months, who underwent SEDC. Type 2B DRCC was noted in 2 patients, with onset 6-8 months, who underwent ETV. With a mean follow-up of 32 months, all showed clinicoradiologic improvement. The application of our classification to other studies showed ETV/EVC to be successful in only 67% of type 1A DRCC and 72% of type 2A DRCC, compared with 100% efficacy of SEDC in our series.

This is probably the first ever endoscopic classification of pediatric posterior fossa cyts, elucidating pathophysiology, presentation, and treatment. Patients with type 1 DRCC present early because of extraneous compression. Among patients with type 2 DRCC, posterior fossa compliance determines the degree of hydrocephalus. The newly described SEDC seems more appropriate for types 1A and 2A DRCC. ETV is adequate in type 2B DRCC and effective with EVC in type 1B.