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Lookup NU author(s): Professor Steve RobsonORCiD
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Introduction Limited data exist on the outcome of Dandy-Walker malformation (DWM), Dandy-Walker variant (DWV) and mega-cisterna magna (MCM). We report the first population-based study of posterior fossa anomalies from the northern region of England. Methods Cases were identified from the Northern Congenital Abnormality Survey (NorCAS) and regional Fetal Medicine Unit databases for the period 1986-2004 for DWMN and 1995-2005 for MCM (defined as a cisterna magna >= 10 mm). Outcome data was obtained from pediatric records and/or general practitioner/health visitor questionnaires for all survivors. Results A prenatal diagnosis of a posterior fossa abnormality was made in 91 cases, with a further 12 cases of DWM/V diagnosed postnatally, giving incidences of DWMN and MCM of 1/11574 and 1/8268 births respectively. In five cases where DWM/V was suspected prenatally, the diagnosis was not confirmed. Of the 47 with DWM/V, 41 (87%) had additional anomalies. There were three survivors, all with neurodevelopmental disability. Of the 39 cases of MCM, 24 (62%) had additional anomalies. There were 30 survivors; one child died at 3 months and the outcome was normal in 25 children including 12/13 (92%) with isolated MCM. Summary Posterior fossa anomalies are relatively common. The outcome is very poor in DWMN owing to the high rate of associated anomalies. The outcome appears better with MCM, especially if this is an isolated finding. Copyright (c) 2006 John Wiley & Sons, Ltd.
Author(s): Long A, Moran P, Robson S
Publication type: Article
Publication status: Published
Journal: Prenatal Diagnosis
ISSN (print): 0197-3851
ISSN (electronic): 1097-0223
Publisher: John Wiley & Sons Ltd.
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