Brain Stem and Cervical Cord Dysraphic Lesions in Iniencephaly |
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Authors: | C. C. Scherrer F. Hammer A. Schinzel J. Briner |
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Affiliation: | a Institute of Clinical Pathology, University of Zurich, Zurich, Switzerlandb Clinics and Policlinics of Obstetrics, University of Zurich, Zurich, Switzerlandc Institute of Medical Genetics, University of Zurich, Zurich, Switzerland |
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Abstract: | Iniencephaly is a rare, lethal, axial dysraphic malformation complex diagnosed on the basis of three cardinal features: deficiency of the occipital bone, cervicothoracic spinal retroflexion, and rachischisis. The majority of the patients also have various associated viscerae malformations. An iniencephalic female fetus delivered at 355/7 weeks of gestation revealed severe anomalies of the central nervous system and the spine: the cerebellar vermis was hypoplastic, the medulla oblongata was flattened and broadened, and the cervical canal was widely patent dorsally. The thoracolumbar spinal cord had a duplicated central canal and lacked a dorsal fissure, representing a minor degree of diastematomyelia. The cervicothoracic spine showed severe bony anomalies including aplasia and fusion of vertebral bodies. |
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Keywords: | central nervous system malformations dysraphic lesions iniencephaly |
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