The Journal of Bone and Joint Surgery, Vol 66, Issue 4 588-601, Copyright © 1984 by Journal of Bone and Joint Surgery, Inc
Occult intraspinal anomalies and congenital scoliosis
MJ McMaster
Of 251 patients with congenital scoliosis, occult congenital intraspinal
anomalies were diagnosed in forty-six (18.3 per cent). A diastematomyelia
was the commonest anomaly (forty-one patients). Other less common
anomalies, occurring alone or in association with a diastematomyelia, were:
neurenteric, epidermoid, and dermoid cysts; teratoma; lipofibroma; absence
of nerve roots; fibrous bands; and a tight filum terminale. Intraspinal
anomalies were associated with all types and sites of congenital scoliosis
by far the highest incidence (52 per cent) occurred in association with a
unilateral unsegmented bar with contralateral hemivertebrae in the lower
thoracic or thoracolumbar regions. Thirty of the patients with an
intraspinal anomaly had neural abnormalities, which usually affected only
one lower extremity, and in twenty-four patients a paralytic foot deformity
developed. Neural deterioration occurred in nine of these patients before
the age of five years and was halted by excision of the anomaly. An
additional twelve patients (4.8 per cent) of the 251 with congenital
scoliosis also had a unilateral neural deficit in the lower limb and a
paralytic foot deformity, similar to those found in the patients with an
intraspinal anomaly, but had no myelographic evidence of a structural
anomaly.
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