Paraplegia resulting from thoracolumbar stenosis in a seven-month-old achondroplastic dwarf.
In young achondroplastic children, neurological manifestations have been found to include macrocephaly, hydrocephalus or ventriculomegaly and cervicomedullary compression. Occasionally in the second decade, lumbar radiculopathy or paraparesis resulting from severe thoracolumbar kyphosis develops. In this paper, we report the unique case of an achondroplastic dwarf who developed paraplegia due to thoracolumbar spinal cord compression at the age of 7 months. Compromise of the spinal canal was found to be due not to bony stenosis, but to a second layer of fibrous tissue, histologically identical to the dura. Treatment consisting of decompressive laminectomy and resection of the constricting tissue allowed the child to recover completely. Clinical, radiographic and pathological findings are discussed.
Hahn
Y S
YS
Pediatric Neurosurgery, Loyola University of Chicago, Ill.
Engelhard
H H
HH
Naidich
T
T
McLone
D G
DG
D000130:Achondroplasia / Q000150:complications*; D004392:Dwarfism / Q000150:complications*; D005260:Female; D006801:Humans; D010264:Paraplegia / Q000209:etiology* / Q000601:surgery; D013117:Spinal Cord Compression / Q000150:complications* / Q000601:surgery