Dandy-Walker Malformation

Not Verified
This information is AI generated and has not yet been reviewed by a specialist physician. AI can make mistakes

Disease overview

Congenital neurological anomaly with agenesis or hypoplasia of the cerebellar vermis and enlargement of the fourth ventricle, often leading to hydrocephalus

Common symptoms

Hydrocephalusmacrocephaly with bulging occiputintracranial hypertensionsevere headacheblurred visionvomitingseizurenystagmusataxia.

WHEN TO SUSPECT

  • Recommendation 1

    Cranial ultrasound or brain CT or MRI. Prenatal diagnosis of Dandy-Walker malformation is sometimes made by ultrasound or fetal MRI.

HOW TO TEST

  • Recommendation 1

    Cranial ultrasound or brain CT or MRI. Prenatal diagnosis of Dandy-Walker malformation is sometimes made by ultrasound or fetal MRI.

TREATMENT

  • Recommendation 1

    supportive

  • Recommendation 2

    control intracranial pressure, usually through surgery (ventriculoperitoneal (VP) or cystoperitoneal (CP) shunts) or endoscopy (endoscopic third ventriculostomy (ETV) [more]

PRIMARY CARE

  • Recommendation 1

    Continuous support based on patient symptoms. Vaginal delivery is not contraindicated but people with a pregnancy affected by Dandy-Walker malformation should receive more frequent ultrasounds and close obstetrics follow-up

  • Recommendation 2

    cesarean section may be necessary due to macrocephaly [more]. Offer families genetic counseling. Children are likely to have developmental delay and other birth defects requiring longitudinal monitoring [more]

FURTHER SUPPORT

  • Recommendation 1

    Often seen for treatment in Neurosurgery, and followed by Pediatric Neurology and Medical Genetics. Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health.

CLINICAL TRIALS

Sources

No data available

Interested in learning more about Dandy-Walker Malformation?

Follow to receive regular updates to the latest research development on this disease via email newsletter.