The Journal of Bone and Joint Surgery, Vol 75, Issue 9 1308-1317, Copyright © 1993 by Journal of Bone and Joint Surgery, Inc
Evaluation and treatment of diastematomyelia
A Miller, JT Guille and JR Bowen
Alfred I. duPont Institute, Wilmington, Delaware 19899.
We reviewed the results for forty-three patients who had a
diastematomyelia. All of the patients had been skeletally immature when the
diagnosis was made, the mean age being six years (range, birth to thirteen
years), and were skeletally mature by the time that they were evaluated by
us. When they were first seen at our institution, twenty-four patients (56
per cent) had a cutaneous lesion, such as hairy patch, dimple, hemangioma,
subcutaneous mass, or teratoma at or near the level of the
diastematomyelia; thirty-four patients (79 per cent) had congenital
scoliosis; and forty-two patients (98 per cent) had at least one associated
musculoskeletal anomaly, such as spinal dysraphism, asymmetry of the lower
extremities, club foot, or a cavus foot. In twenty-seven patients (63 per
cent), the diastematomyelia was located in the lumbar spine. Thirty-six
patients had eighty-four neurological manifestations. Resection of the spur
was performed in thirty-three patients at a mean age of seven years (range,
three months to seventeen years). Twenty-two patients who had a resection
had no change in neurological condition, nine patients had improvement, and
one patient had one symptom improve and another symptom worsen after the
operation. We believe that resection of the spur should be performed in
patients who have progressive neurological manifestations. Patients who do
not have progressive neurological manifestations should be observed; if
progression is noted, a resection should then be performed.
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