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

Author:

Stambolliu Emelina12,Ioakeim-Ioannidou Myrsini13,Kontokostas Kimonas4,Dakoutrou Maria15,Kousoulis Antonis A.16

Affiliation:

1. Society of Junior Doctors, Athens, Greece

2. Department of Internal Medicine, General Hospital of Kalavryta, Kalavryta, Greece

3. Medical School, University of Athens, Athens, Greece

4. Department of Neurology, General Hospital “Pammakaristos,” Athens, Greece

5. First Department of Paediatrics, “Aghia Sophia” Children’s Hospital, University of Athens, Greece

6. Faculty of Epidemiology and Population Health, London School of Hygiene & Tropical Medicine, London, United Kingdom

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.

Publisher

SAGE Publications

Subject

Clinical Neurology,Pediatrics, Perinatology, and Child Health

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