Surgical Treatment of Scoliosis in Larsen Syndrome With Bilateral Hip Dislocation

Abstract

This is a report of a 12-year-old girl treated surgically for scoliosis associated with bilateral hip dislocation in Larsen syndrome. To describe a rare case of scoliosis associated with Larsen syndrome and bilateral hip dislocation that was treated surgically with follow-up for 15 years. There are few reports of the long-term follow-up of cases involving surgically treated scoliosis associated with bilaterally dislocated hips. The patient's spine showed a right thoracic curve (T5-T12) with a Cobb angle of 77 degrees and did not show pelvic obliquity on an anterior-posterior radiograph film. On the sagittal alignment of her spine, the thoracic spine showed an abnormal lordosis (T5-T12: 19 degrees), and the lumbar spine had a hyperlordosis (L1-S1: 57 degrees) with a large lumbosacral angle (72 degrees ). We performed a posterior spinal fusion between T4 and L2 using Cotrel-Dubousset Instrumentation, anticipating the restoration of normal lumbar and cervical lordosis, as well as thoracic kyphosis. The Cobb angle of thoracic scoliosis improved from 77 degrees to 28 degrees, and a thoracic kyphosis of 12 degrees (T5-T12) was obtained. Subsequently, on the sagittal plane, the lumbosacral angle (sacral anteflexion) decreased from 72 degrees to 52 degrees, comparable to that of patients with hip dislocation, and the lumbar lordotic angle increased from 57 degrees to 66 degrees. The restoration of thoracic kyphosis resulted in an increase of lumbar lordosis and decrease of sacral anteflexion. At the 15-year follow-up,although the thoracic scoliosis (T5-T12) had increased to 36 degrees, good coronal and sagittal balance had been maintained. The patient is asymptomatic in her spine and hip. A case of scoliosis associated with dislocated hips in a patient with Larsen syndrome was successfully treated with posterior correction surgery. Fusion surgery between T4 and L2 provided an ideal sagittal balance of the total spine, while preserving 4 lumbar mobile segments.