Introduction: Hemivertebrae is the congenital abnormal formation of one or multiple vertebrae. This malformation occurs due to a disruption in the normal growth of the bony spine during early fetal development, due to a lack of one of the centers of chondrification. Individuals with hemivertebrae are often seen to have focal wedging which is a common cause of congenital scoliosis. Diastematomyelia is the spinal cord’s rare sagittal division into two parts. The two hemicords can be contained either within a single dural sac or individual sacs separated by a cartilaginous or bony septum. This condition is typically seen in the lower thoracic and upper lumbar sections of the spine. The exact cause of diastematomyelia remains unclear but may be associated with spina bifida.
Methods: This article uses a comprehensive review of the most up-to-date literature on managing multiple intraspinal anomalies (congenital scoliosis, diastematomyelia, and hemivertebrae). Additionally, expert neurosurgeons gave recommendations on management when compared to the management found in the literary review.
Results: The outlook for patients with congenital scoliosis, diastematomyelia, and hemivertebrae who do not undergo surgery is not clear. When a neurological deficit is present with congenital scoliosis, it is recommended to perform resection of diastematomyelia before proceeding with spinal correction. However, in some patients with pre-existing neurological abnormalities, unresected diastematomyelia caused progressive neurological deterioration. Therefore, prophylactic excision of hemivertebrae depends on the type (semi-segmented, incarcerated, nonincarcerated). The size of the curve and its progression should dictate the treatment of congenital scoliosis in the presence of diastematomyelia.
Conclusion : Congenital scoliosis associated with anomalies are rarely seen together. The few studies we found suggest the majority of patients are managed conservatively, even with neurological deficits. Surgery is indicated when follow-up examinations present with progressive scoliosis and/or a worsening neurological exam. When managing a patient with scoliosis, it is imperative to consult specialists in neurosurgery and orthopaedic surgery to closely follow and assess patients for changes in Cobb angles, rule out tension on the spinal cord, and monitor for compression fractures
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