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 DWM/V and 1995-2005 for MCM (defined as a cisterna magna > or =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 DWM/V 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 DWM/V owing to the high rate of associated anomalies. The outcome appears better with MCM, especially if this is an isolated finding.
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 DWM/V and 1995-2005 for MCM (defined as a cisterna magna > or =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 DWM/V 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 DWM/V owing to the high rate of associated anomalies. The outcome appears better with MCM, especially if this is an isolated finding.
Authors: Deniz Vatansever; Vanessa Kyriakopoulou; Joanna M Allsop; Matthew Fox; Andrew Chew; Joseph V Hajnal; Mary A Rutherford Journal: Cerebellum Date: 2013-10 Impact factor: 3.847
Authors: Giuseppe Talamonti; Marco Picano; Alberto Debernardi; Moreno Bolzon; Mario Teruzzi; Giuseppe D'Aliberti Journal: Childs Nerv Syst Date: 2010-05-19 Impact factor: 1.475
Authors: Fernanda Silveira Bello de Barros; Luiz Cláudio de Silva Bussamra; Edward Araujo Júnior; Leonardo da Silva Valladão de Freitas; Luciano Marcondes Machado Nardozza; Antonio Fernandes Moron; José Mendes Aldrighi Journal: ISRN Obstet Gynecol Date: 2012-11-14
Authors: Lisa G Shaffer; Jill A Rosenfeld; Mindy P Dabell; Justine Coppinger; Anne M Bandholz; Jay W Ellison; J Britt Ravnan; Beth S Torchia; Blake C Ballif; Allan J Fisher Journal: Prenat Diagn Date: 2012-07-30 Impact factor: 3.050