Symptomatic Congenital Kyphoscoliosis Due to Concomitant Wedged Vertebra and Sotos Syndrome: A Case Report

Abstract

There are few reports of syndromic scoliosis accompanied by a congenital vertebral anomaly. We report a case of Sotos syndrome with a concomitant congenital wedged vertebra whose kyphoscoliosis progressed rapidly and presented with myelopathy during the growth-spurt period. A 12-year-old male suffering from Sotos syndrome with T10-wedged vertebra presented with paraparesis and urinary dysfunction. Magnetic resonance imaging showed that the spinal cord was deviated to the concave side and was compressed from the right pedicle and posterior wall of the vertebral body at T10 level. He underwent direct decompression surgery including posterior right T10 pediculectomy and corrective fixation from T5 to L1. After the surgery, his gait disturbance improved, and he no longer had urinary incontinence. His kyphoscoliosis also improved. Two congenital diseases, Sotos syndrome and congenital vertebral anomaly, such as in the present case, require careful follow-up or early surgical intervention, especially during the growth-spurt period, to avoid missing the good timing for surgery because of the possibility of rapid deterioration of kyphoscoliosis and subsequent spinal cord disorders.