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

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 clinical outcomes before and after these two different surgical procedures. This research seeks to inform surgical decision-making in treating DWS-related hydrocephalus.

Clinical data from 12 children with DWS-related hydrocephalus (1 case of Dandy-Walker malformation, 11 cases of Dandy-Walker variant) treated between January 2010 and June 2024 were retrospectively analyzed. Among these, eight patients underwent VPS, whereas four received CPS. The follow-up outcomes were classified into good, fair, and poor based on established criteria, including the Evans index, Vineland Adaptive Behavior Scales (VABS) scores, and the presence or absence of hydrocephalus or interstitial edema.

The follow-up duration ranged from 6 months to 13 years, yielding nine cases with good outcomes and three fair outcomes. Among the VPS group, two patients achieved normal postoperative ventricular size, while six exhibited mild to moderate ventricular dilation. Seven patients showed good cerebellar vermis development, and all of these patients achieved good outcomes. In contrast, the CPS cohort includes one case with mild ventricular dilatation and three cases with moderate ventricular dilatation. Three CPS cases showed cerebellar vermis dysplasia, with one case exhibiting persistent cerebellar vermis dysplasia postoperatively. One patient in the CPS group had good outcomes, while three had fair outcomes. The choice of shunt procedure was statistically significantly associated with clinical outcome (χ² = 8.00, P = 0.018), postoperative FOHR (t = 2.414, P = 0.036), and postoperative VABS scores (t = 3.2109, P = 0.0093); patients receiving VPS were superior to CPS in these associations. Additionally, cerebellar vermis development was also strongly correlated with outcome (χ² = 8.00, P = 0.018). Among cases with good outcomes, eight had well-developed cerebellar vermis, while one had underdevelopment. Among those with fair outcomes, one case lacked development, and two showed underdevelopment.

Children with DWS-related hydrocephalus who undergo hydrocephalus treatment via a single shunt procedure exhibit well-developed cerebral structures and may have better outcomes with VPS than with CPS.