Cervicomedullary junction decompression in a case of Marshall-Smith syndrome. Case report.
The case is reported of a 2-year-old boy born with Marshall-Smith syndrome who had difficulty in swallowing and who exhibited spasticity and quadriparesis due to compression of the medulla and cervical spine. This is the first child with this rare condition reported to have brain-stem compression from bone abnormalities at the craniovertebral junction and who has required surgery.
Pappas
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CT
Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Arizona.
Rekate
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HL
D000015:Abnormalities, Multiple; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000601:surgery; D001929:Brain Edema / Q000150:complications / Q000601:surgery; D002675:Child, Preschool; D005147:Facial Bones / Q000002:abnormalities; D006130:Growth Disorders; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008526:Medulla Oblongata / Q000000981:diagnostic imaging; D013117:Spinal Cord Compression / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery; D013577:Syndrome; D014057:Tomography, X-Ray Computed