Dandy-Walker syndrome associated with congenital heart defects: report of three cases

Area of Science:

• Pediatric Neurology
• Developmental Biology
• Neurosurgery

Background:

• Dandy-Walker syndrome is a congenital brain malformation.
• It is often associated with other developmental anomalies, including congenital heart defects.
• Understanding the etiology and optimal management is crucial for affected children.

Observation:

• Reported are three cases of Dandy-Walker syndrome co-occurring with congenital heart defects.
• Management strategies for these complex pediatric cases were documented.
• The study discusses various etiological theories for Dandy-Walker syndrome.

Findings:

• The theory of primary developmental atresia of the foramina of Magendie and Luschka remains a plausible explanation for Dandy-Walker syndrome.
• For extracranial cerebrospinal fluid (CSF) drainage in Dandy-Walker syndrome, a ventriculo-peritoneal shunt is preferred if cardiac anomalies are suspected.
• The proximal catheter placement is recommended in the posterior fossa cyst (dilated fourth ventricle), not the lateral ventricle.

Implications:

• This study provides guidance on surgical management for Dandy-Walker syndrome with cardiac anomalies.
• It emphasizes the importance of considering extracranial CSF drainage techniques in complex pediatric neurosurgical cases.
• The findings suggest a preferred approach for shunt placement to improve outcomes in affected infants.