Surgical treatment of segmental spinal dysgenesis: a report of 19 cases
- PDF / 1,124,330 Bytes
- 9 Pages / 595.276 x 790.866 pts Page_size
- 45 Downloads / 166 Views
CASE SERIES
Surgical treatment of segmental spinal dysgenesis: a report of 19 cases Rodrigo G. Remondino1 · Carlos A. Tello1 · Ernesto S. Bersusky1 · Alejandra Francheri Wilson1 · Mariano A. Noel1 · Eduardo Galaretto1 · Lucas Piantoni1 Received: 6 June 2020 / Accepted: 8 September 2020 © Scoliosis Research Society 2020
Abstract Study design Retrospective review. Objective To describe clinical presentation, surgical management, long-term results, and complications in patients with segmental spinal dysgenesis (SSD). In addition, we sought to emphasize early surgery for this complex congenital abnormality. Summary SSD is a rare congenital malformation characterized by focal stenosis, spinal subluxation, kyphosis, and absence of the nerve roots. Neurologic function ranges from normal to complete paraplegia. Progression of the deformity and neurologic deterioration is the rule. Methods An independent spinal surgeon reviewed the complete records of 19 patients with SSD, between 1998 and 2015 at a single institution. Mean follow-up was 10 years and 6 months (2–14 years). Results We evaluated 11 males and 8 females, with a mean age of 2 years and 9 months (5 months–15 years). The dysgenetic segment involved an average of 2.9 vertebrae (1–5); the upper thoracic region was most commonly involved in ten cases. Fifteen patients had severe spinal stenosis. 14 patients presented neurological deficits and 15 patients had associated organ and musculoskeletal anomalies.Twenty-seven surgeries were performed, a mean of 1.76 procedures (1–5) to obtain solid fusion. Neurologic function improved in four, deteriorated in three, and remained unchanged in 12 patients Seven complications were recorded. Conclusion We strongly recommend decompression and fusion as soon as possible to preserve or prevent neurologic deterioration. Although challenging, it was possible to achieve a solid instrumented fusion in all cases; however, a high rate of patients may deteriorate or not recover neurological status after surgery. Level of evidence Level IV evidence Keywords Segmental spinal dysgenesis · Congenital subluxation · Deformity progression · Neurological impairment · Early surgery
Introduction Segmental spinal dysgenesis (SSD) is a rare congenital anomaly, usually located in the thoracolumbar or lumbar spine, and focally characterized by kyphosis or kyphoscoliosis, vertebral subluxation, spinal instability associated with a stenotic canal, narrowing of the thecal sac, and absence of nerve roots within the involved segments [1, 10]. Relative * Rodrigo G. Remondino [email protected] 1
Spine Unit, Juan P. Garrahan. Pediatric Hospital, 1881Combate de los Pozos. First floor, office 7484, Buenos Aires, Argentina
indemnity of the vertebrae and spinal-cord segments cephalad and caudal to the injury is typical. SSD is usually discovered on prenatal ultrasonography. In 1988, Scott et al. [1] published the first series of three patients with dysgenesis of the lumbar and thoracolumbar spine characterized by focal abnormalities ass
Data Loading...
