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Evaluation and treatment of diastematomyelia
A Miller1, J T Guille, J R Bowen
1Alfred I. duPont Institute, Wilmington, Delaware 19899.
Insights
Diastematomyelia, a spinal condition, often presents with associated anomalies. Surgical spur resection is recommended for patients with progressive neurological symptoms, while others may benefit from observation.
Area of Science:
- Pediatric Neurosurgery
- Spinal Surgery
- Developmental Pediatrics
Background:
- Diastematomyelia is a congenital vertebral anomaly.
- It often occurs in skeletally immature patients and is associated with various cutaneous and musculoskeletal abnormalities.
Purpose of the Study:
- To review the clinical presentation, associated anomalies, and outcomes of surgical intervention for diastematomyelia.
- To establish guidelines for the management of diastematomyelia based on neurological progression.
Main Methods:
- Retrospective review of 43 skeletally immature patients diagnosed with diastematomyelia.
- Analysis of associated cutaneous lesions, congenital scoliosis, musculoskeletal anomalies, and neurological manifestations.
- Evaluation of surgical outcomes following spur resection.
Main Results:
- The majority of patients presented with cutaneous lesions (56%), congenital scoliosis (79%), and musculoskeletal anomalies (98%).
- Lumbar spine involvement was most common (63%).
- Of 33 patients who underwent spur resection, 22 showed no neurological change, 9 improved, and 1 had mixed results.
Conclusions:
- Surgical resection of the diastematomyelia spur is indicated for patients with progressive neurological deficits.
- Observation is recommended for asymptomatic patients, with surgical intervention considered if neurological progression occurs.
Abstract:
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.