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© 2005 by the American Institute of Ultrasound in Medicine
J Ultrasound Med 24:1697-1709 • 0278-4297

Case Series

Magnetic Resonance Imaging of the Kinked Fetal Brain Stem

A Sign of Severe Dysgenesis

Annemarie Stroustrup Smith, MD, Deborah Levine, MD, Patrick D. Barnes, MD and Richard L. Robertson, MD

Harvard Medical School and the Harvard–Massachusetts Institute of Technology Division of Health Sciences and Technology, Boston, Massachusetts USA (A.S.S.); Department of Radiology, Beth Israel Deaconess Medical Center, Boston, Massachusetts USA (D.L.); Department of Radiology, Lucile Packard Children’s Hospital, Palo Alto, California USA (P.D.B.); and Department of Radiology, Children’s Hospital, Boston, Massachusetts USA (R.L.R.).

Address correspondence to Deborah Levine, MD, Department of Radiology, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Boston, MA 02215 USA. E-mail: dlevine{at}bidmc.harvard.edu

Objective. Magnetic resonance imaging (MRI) allows visualization of the fetal brain stem in a manner not previously possible. A "kinked" brain stem is a sign of severe neurodysgenesis. The purpose of this series was to describe cases of a kinked brain stem detected on prenatal MRI and to discuss the possible genetic and syndromic etiologies. Methods. Seven cases of a kinked brain stem on fetal MRI (gestational age range, 18–34 weeks) were reviewed and correlated with other clinical, genetic, imaging, and autopsy findings. Results. In all cases, there was associated cerebellar hypogenesis. Additional findings were ventriculomegaly (4 cases), cerebral hypogenesis (3 cases), microcephaly (4 cases), schizencephaly (1 case), cephalocele (1 case), hypogenesis of the corpus callosum (1 case), and hydrocephalus (1 case). In 2 cases, prenatal sonography misidentified the kinked brain stem as the cerebellum. Conclusions. A kinked brain stem is an indicator of severe neurodysgenesis arising early in gestation. Magnetic resonance imaging provides the necessary resolution to detect this sign and delineate any associated anomalies in utero to assist with further genetic evaluation, management, and counseling.



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  		Figure 8. Human fetal brain stem at 7 weeks’ gestational age. Note the kink formed by the mesencephalic, pontine, and cervical flexures. (Image courtesy of Veronika Doljenkova [Rhode Island School of Design, Providence, RI] with reference to Sarnat1 and Sadler.2)

 

Key Words: brain stem • midbrain • prenatal diagnosis • ventriculomegaly

Abbreviations: CNS, central nervous system • MRI, magnetic resonance imaging • SSFSE, single-shot fast spin echo

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