Thoracic kyphoscoliosis resembling neurofibromatosis: a case report focusing on subfascial instrumentation.

Abstract

The management of early-onset progressive scoliosis is controversial. To describe the unusual surgical management of a young female with an early-onset progressive, short, angular kyphoscoliosis resembling neurofibromatosis. A case report reviewing the treatment of an unusual occurrence of kyphoscoliosis. After compliant Milwaukee brace wear had failed to stop deformity progression, surgical management including segmental anterior and posterior T5-T10 arthrodesis, and posterior T3-L4 subfascial rod instrumentation was performed at age 4 years and 6 months. After 10 rod lengthenings, segmental anterior T10 to L3 arthrodesis and posterior T3-L4 instrumentation and arthrodesis were done at age 11 years and 5 months. From preoperative to 3-year postoperative definitive surgery, her T3 to L4 instrumented spine length increased by 9.5 cm; 4 cm after the initial surgery, 3 cm between the initial surgery and the definitive surgery and 2.5 cm after the definitive surgery. From preoperative to latest follow-up, her thoracic scoliosis was reduced from 89 to 31 degrees, her thoracolumbar compensatory scoliosis from 59 to 37 degrees and her kyphosis from 70 to 17 degrees. The combination of early definitive anterior and posterior major curve arthrodesis with a subfascial rod lengthening program to control the remainder of the thoracolumbar spine was a satisfactory solution for this unusual case of early-onset, progressive, short, angular kyphoscoliosis.