Two patients are reviewed, each having an unusual clinical syndrome of progressive congenital cervical scoliosis and unilateral arm paralysis. This appears to be a unilateral developmental defect in both mesenchymal and neural tissue. There was a unilateral spina bifida without myelomeningocele, meningocele, or lipoma. The cervical spine deformity was progressive at a young age, one patient having been fused at age 2 years, the other at age 5 years. At surgery, the dura was intact but unprotected on the concavity of the curve. Bridging bone grafts were utilized to span the defects. The arm paralysis was extensive in both patients, but improvement in function was accomplished by appropriate reconstructive procedures.
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