Abstract
Treatment of congenital kyphosis with severe angular dysplastic spine in children with myelomeningocele (MMC) is one of the most difficult spinal procedures. Most of the surgeons support kyphectomy with long segmental spinal instrumentation and postoperative immobilization by thoracolumbosacral orthosis.
Several spinal deformities are seen frequently in patients who have MMC. The deformity may be congenital or paralytic. Congenital lumbar kyphosis is less common, but most difficult in patients with MMC, occurring in 10–20% of patients. Most curves are congenital and rigid, often more than 80° at birth, and rapidly progresses.
With progression of kyphotic deformity, patients experience recurrent skin breakdown over the apex of the kyphos; impaired sitting balance; the necessity of using their hands for support; collapsing spine and decreasing of lumbar height reduce the capacity of the abdominal cavity and resulting in reduced respiratory capacity and malnutrition. The poor posture and short abdomen make it difficult to manage the patients’ urological needs. A severe deformity raises difficulties in social and psychological development.
Non-operative treatment with spinal orthoses may provide only temporary correction of a kyphotic deformity, but does not prevent progression and skin breakdown.
The goal of surgical treatment is correction of spinal deformity by long segmental instrumentation and achievement of a solid spine fusion in order to allow a balanced sitting position and to prevent complications.
From 1983 to 2001, 6 patients with thoracic level myelomeningocele and severe kyphotic deformity were referred for surgical correction. There were 5 males and 1 female patients with average age at the time of surgery of 8.3 years (range 4.3–13 years). All patients suffered from severe kyphosis, range 90° to 130°, average – 108°. All of them underwent posterior ligation of spinal cord during resection of lordotic segment of the kyphos, and segmental spinal fixation of the deformity from the thoracic spine to the sacrum. In all cases following the resection of the vertebrae it was possible to correct the deformity.
All patients were available for follow-up with range of 6–216 months, average 85 months. All of them were satisfied with the surgical outcome and presented in their final clinical examination with balanced and comfortable sitting, without soft tissue complications. In all cases a significant correction of the deformity was achieved (15°–30°) and enabled comfortable and stable sitting. Two patients suffered post-operative complications, one from surgical wound infection which required surgical debridement followed by soft tissue covering, and the other suffered from distal migration of the rod which was shortened later on.
Discussion: Kyphotic deformity in a patient who has MMC is a challenge for the orthopaedic surgeon and requires major surgical intervention. Resection of the kyphos with posterior instrumentation and fusion may solve patient’s functional problems.
The abstracts were prepared by Orah Naor. Correspondence should be addressed to him at the Israel Orthopaedic Association, PO Box 7845, Haifa 31074, Israel.
