Congenital Thoracic Scoliosis With Unilateral Unsegmented Bar, Convex Hemivertebrae, and Fused Concave Ribs With Severe Progression After Posterior Fusion at Age 2

Abstract

Case report with 40-year follow-up after definitive surgery. To show that extensive spine fusion in a young child can control the curve and does not necessarily lead to early death. Recent efforts to avoid early spine fusion by using either progressive lengthening of spinal or rib cage implants are based on the theory that early spine fusion is deleterious and results in early death due to pulmonary compromise. Unfortunately, there is little to no documentation to support this theory. This is a single case report of a child who at the age of 3 months had a 32° congenital thoracic congenital scoliosis with a unilateral unsegmented bar, concave fused ribs, and convex hemivertebrae. By age 2 years, the curve had progressed to 64°, so a posterior fusion was done from T5 to T12. By age 8 years, the curve had dramatically increased, and she was referred to the author. She underwent a double-wedge osteotomy, both anteriorly and posteriorly, plus anterior fusion T4-L3 and posterior fusion from T1 to L3. A halo cast was used for correction. At a 40-year follow-up after her definitive surgery at the age of 8 years, she is still alive and functioning well, although her vital capacity is poor. Extensive thoracic spine fusion at an early age did not result in early death, but the patient is far from ideal.