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© 2005 by the American Institute of Ultrasound in Medicine
J Ultrasound Med 24:841-848 • 0278-4297

Case Series

Prenatal Diagnosis of Herniated Dandy-Walker Cysts

Wesley Lee, MD, Ivana M. Vettraino, MD, Christine H. Comstock, MD, Nirish Lal, MD, Chris Kazmierczak, MD, Anil Shetty, PhD, Gil Raff, MD, Karol Zakalik, MD and Roberto Romero, MD

Division of Fetal Imaging, Department of Obstetrics and Gynecology (W.L., I.M.V., C.H.C.), Department of Diagnostic Radiology (N.L., C.K., A.S.), Division of Cardiology, Department of Internal Medicine (G.R.), and Division of Neurosurgery and Department of Pediatrics (K.Z.), William Beaumont Hospital, Royal Oak, Michigan USA; and Perinatology Research Branch, National Institute of Child Health and Human Development, National Institutes of Health, Department of Health and Human Services, Bethesda, Maryland USA (W.L., R.R.).

Address correspondence to Wesley Lee, MD, Division of Fetal Imaging, Department of Obstetrics and Gynecology, William Beaumont Hospital, 3601 W Thirteen Mile Rd, Royal Oak, MI 48073-6769 USA. E-mail: wlee{at}beaumont.edu

Objective. The purpose of this series is to describe the prenatal diagnosis and pregnancy outcome of fetuses affected with Dandy-Walker malformation in which a posterior cyst herniated through a bony defect of the occipital skull, foramen magnum, or both. Methods. Two- and 3-dimensional sonography were used to examine 2 fetuses with poorly delineated cerebellar structures and a large posterior cystic neck mass. Fetal magnetic resonance imaging (MRI) was added to this evaluation as a complementary diagnostic modality. Results. Three-dimensional sonography helped characterize the precise site of cyst herniation through the occipital skull or foramen magnum. Fetal MRI confirmed the sonographic findings. Neonatal MRI studies identified heterotopic gray matter as evidence of a neuronal migration disorder in both fetuses. The second fetus also had agenesis of the corpus callosum. Retrospective review of the fetal MRI (25.9 weeks’ menstrual age) and 3-dimensional sonographic (18.7 weeks’ menstrual age) studies confirmed ventricular wall nodularity involving the occipital horns of the second fetus. Conclusions. The antenatal detection of a large posterior cystic neck mass and a poorly defined or nonvisualized cerebellar vermis suggest Dandy-Walker malformation with a herniated cyst. Three-dimensional sonography and fetal MRI are important adjunctive methods that can be used to evaluate the herniation site and a possible neuronal migrational disorder.

Key Words: Dandy-Walker malformation • fetal magnetic resonance imaging • herniated cyst • heterotopia • 3-dimensional sonography

Abbreviations: DWM, Dandy-Walker malformation • MA, menstrual age • MRI, magnetic resonance imaging

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Copyright © 2005 by the American Institute of Ultrasound in Medicine.