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

Abstract:

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.

journal_name

J Child Neurol

authors

Stambolliu E,Ioakeim-Ioannidou M,Kontokostas K,Dakoutrou M,Kousoulis AA

doi

10.1177/0883073817712589

subject

Has Abstract

pub_date

2017-09-01 00:00:00

pages

886-902

issue

10

eissn

0883-0738

issn

1708-8283

journal_volume

32

pub_type

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