# Dandy-Walker malformation associated with hydrocephalus in a 15-month-old child: A case report with literature review

**Authors:** Turyalai Hakimi, Hasibullah Baha Nijrabi, Mohammad Yusuf Yadgari, Khalid Mohammad Qasem, Mohammad Anwar Jawed

PMC · DOI: 10.1016/j.ijscr.2025.111229 · International Journal of Surgery Case Reports · 2025-03-27

## TL;DR

A 15-month-old child with Dandy-Walker malformation and hydrocephalus showed significant improvement after a surgical shunt procedure.

## Contribution

This case report adds to the literature by documenting a successful treatment outcome in a young patient with Dandy-Walker malformation.

## Key findings

- The patient showed significant improvement in head size and motor function after ventriculoperitoneal shunt placement.
- Complete recovery from convulsions was observed over a six-month follow-up period.
- Dandy-Walker malformation is associated with nonspecific clinical manifestations requiring multidisciplinary management.

## Abstract

Dandy-Walker malformation is a posterior cranial fossa anomaly, characterized by the absence or hypoplasia of the vermis and cystic dilatation of the fourth ventricle, resulting in elevation of the tentorium and torcula. This condition can present with a broad range of neurological and developmental symptoms, highlighting the importance of early recognition and intervention to improve patient outcomes.

This case describes a 15-month-old male presenting with head enlargement and motor dysfunction. A computerized tomography scan identified absence of the cerebellar vermis and ventriculomegaly. The patient underwent ventriculoperitoneal shunt placement, leading to significant improvement (head size, motor function) and complete recovery from convulsion over a six-month follow-up.

The clinical presentation primarily originates from cerebellar dysfunction, impacting balance, coordination, vision, motor skills, cognition, and behavior. This condition occurs sporadically and is frequently associated with hydrocephalus. Treatment is centered on managing symptoms and related comorbidities.

Dandy-Walker malformation is a congenital anomaly of the posterior fossa that causes a wide range of neurological and developmental challenges, primarily hydrocephalus. Effective management requires a multidisciplinary team, including pediatric surgeons and pediatricians, with timely consultation with a pediatric neurosurgeon and neurologist being essential.

•Dandy-Walker malformation, also known as Dandy-Walker syndrome, is a rare congenital neurological anomaly that affects cerebellar development.•Clinical manifestations are nonspecific and depend on various factors, including the severity of hydrocephalus, intracranial hypertension, and related comorbidities.•Treatment primarily aims to alleviate hydrocephalus and posterior fossa symptoms, often involving surgical interventions such as ventriculoperitoneal or cystoperitoneal shunting.

Dandy-Walker malformation, also known as Dandy-Walker syndrome, is a rare congenital neurological anomaly that affects cerebellar development.

Clinical manifestations are nonspecific and depend on various factors, including the severity of hydrocephalus, intracranial hypertension, and related comorbidities.

Treatment primarily aims to alleviate hydrocephalus and posterior fossa symptoms, often involving surgical interventions such as ventriculoperitoneal or cystoperitoneal shunting.

## Linked entities

- **Diseases:** Dandy-Walker malformation (MONDO:0009072), hydrocephalus (MONDO:0001150)

## Full-text entities

- **Diseases:** cerebellar dysfunction (MESH:D002526), motor dysfunction (MESH:D000068079), congenital anomaly of the posterior fossa (MESH:D015192), Dandy-Walker malformation (MESH:D003616), hydrocephalus (MESH:D006849), convulsion (MESH:D012640)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## References

11 references — full list in the complete paper: https://tomesphere.com/paper/PMC11999529/full.md

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Source: https://tomesphere.com/paper/PMC11999529