The Most Common Comorbidities in Dandy-Walker Syndrome Patients: A Systematic Review of Case Reports

J Child Neurol. 2017 Sep;32(10):886-902. doi: 10.1177/0883073817712589. Epub 2017 Jun 21.


Objective: Dandy-Walker syndrome (DWS) is a rare neurologic multi-entity malformation. This review aimed at reporting its main nonneurologic comorbidities.

Methods: Following PRISMA guidelines, search in Medline was conducted (2000-2014, keyword: dandy-walker). Age, sex, country, DWS type, consanguinity or siblings with DWS, and recorded coexistent conditions (by ICD10 category) were extracted for 187 patients (46.5% male, 43% from Asia) from 168 case reports.

Results: Diagnosis was most often set in <1 year old (40.6%) or >12 years old (27.8%). One-third of cases had a chromosomal abnormality or syndrome (n = 8 PHACE), 27% had a cardiovascular condition (n = 7 Patent Ductus Arteriosus), 24% had a disease of eye and ear (n = 9 cataract); most common malignancy was nephroblastoma (n = 8, all Asian). Almost one-fifth had a mental illness diagnosis; only 6.4% had mild or severe intellectual disability.

Conclusion: The spread of comorbidities calls for early diagnosis and multidisciplinary research and practice, especially as many cases remain clinically asymptomatic for years.

Keywords: Comorbidity; growth and development; malformations of cortical development; nervous system malformations.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Comorbidity
  • Dandy-Walker Syndrome / complications*
  • Dandy-Walker Syndrome / epidemiology*
  • Humans