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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:1034-1036, June-July 2004
© 2004 American Society of Neuroradiology

Case Report
PEDIATRICS

Prenatal MR Findings of the Middle Interhemispheric Variant of Holoprosencephaly

Steven B. Pulitzer^d, Erin M. Simon^a, Timothy M. Crombleholme^b and Jeffrey A. Golden^c

^a Department of Radiology, Children’s Hospital of Philadelphia, Philadelphia, PA
^b Department of Pediatric General and Thoracic Surgery and Center for Fetal Diagnosis and Treatment, Children’s Hospital of Philadelphia, Philadelphia, PA
^c Department of Pathology, Children’s Hospital of Philadelphia, Philadelphia, PA
^d Department of Radiology, Mount Sinai School of Medicine, New York, NY

Address correspondence to Erin M. Simon, MD, Department of Radiology, Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4399

Summary: We report a case of the middle interhemispheric variant of holoprosencephaly (MIH) with noncleavage of the posterior portion of the frontal lobes and the parietal regions in a fetus at 22 weeks’ gestation. To our knowledge, this is the first case of the rare MIH variant to be diagnosed in utero by use of ultrafast MR imaging and one of the few such reports to document gross and microscopic pathologic findings. Neuroimaging results correlated with those of gross and microscopic pathologic specimens obtained from the stillborn child. We conclude that ultrafast MR imaging can accurately distinguish holoprosencephaly subtypes in utero, which may affect counseling of parents.