Dandy-Walker syndrome with hydrocephalus undergoing VPS or CPS?——A single-centre retrospective study

Abstract Objective: Dandy-Walker syndrome (DWS) is often associated with hydrocephalus, yet the choice between lateral ventriculoperitoneal shunt (VPS) or posterior cranial fossa cystoperitoneal shunt (CPS) is still controversial. This retrospective study aims to compare the clinical features , cranial imaging changes and prognosis before and after different these two surgical procedures.This research seeks to inform surgical decision-making in treating DWS-related hydrocephalus. Methods: Clinical data from 13 children with DWS-related hydrocephalus (1 case of Dandy-Walker malformation, 12 cases of Dandy-Walker variant) treated between January 2010 and June 2024 were retrospectively analyzed. Eight patients underwent VPS, and 5 received CPS. Follow-up outcomes were graded as good, fair, and ineffective. A good prognosis was defined by an EVANS index < 0.3, or ≥ 0.3 but without hydrocephalus or interstitial edema, and a Vineland Adaptive Behavior Scales (VABS) score ≥ 75. Fair prognosis indicated stable hydrocephalus (EVANS ≥ 0.3) with no cerebral or interstitial edema and VABS scores of 60–75. Ineffective prognosis reflected unrelieved hydrocephalus and VABS < 60. Results: Follow-up ranged from 3 months to 13 years, yielding 10 good and 3 fair outcomes. Of 8 VPS cases, 6 achieved normal postoperative ventricular size, while 2 had mild to moderate dilation. Seven showed good cerebellar vermis development, with all achieving a good prognosis. Among 5 CPS cases, 2 had mild ventricular dilatation, 3 moderate ventricular dilatation, and 3 showed cerebellar vermis dysplasia. One case retained a cerebellar vermis defect postoperatively. Good prognosis was observed in 2 CPS cases and fair prognosis in 3. Shunt type correlated significantly with prognosis (X² = 6.24, P = 0.035), postoperative ventricular size (X² = 6.96, P = 0.021), and postoperative cerebellar earthworm development (X² = 5.92, P = 0.0319). In good prognosis cases, 8 had well-developed cerebellar earthworms, while 2 cases of lower earthwormsshowed underdevelopment. Among fair outcomes, 1 case lacked cerebellar earthworms development, and 2 showed underdevelopment, indicating a significant correlation between prognosis and cerebellar earthworms' development (X² = 6.24, P =0.035). Conclusion: Children with DWS-related hydrocephalus who undergo hydrocephalus correction via a single shunt exhibit a well-developed cerebral structure and may be better off with VP than with CP.