SPINAL CORD TERMINATING AT T3: CHALLENGES OF MANAGEMENT

Abstract

We describe a case of a three year old girl with Caudal Regression Syndrome (CRS) at the ninth thoracic vertebral level with termination of the spinal cord at the unusually high level of the third thoracic vertebra. We describe this rare condition and discuss the challenging management in an extremely rare case where there is termination of the spinal cord at a high thoracic level.

CRS is a severe congenital neural and skeletal deficiency that is characterized by absence of the entire sacrum and of variable amounts of the lumbar and occasionally thoracic spine with associated neural elements. This is accompanied by a number of congenital visceral abnormalities.

Controversy belies the optimal orthopaedic management of the spinal anomaly and the associated lower extremity deformities in this condition. Affected children have multiple musculoskeletal abnormalities, including foot deformities, knee and hip flexion contractures, dis-located hips, spino-pelvic instability, and scoliosis.

We believe the care of these complex patients should be highly individualized.

Patients with types I and II lumbosacral agenesis have an excellent chance of becoming community ambulators and early interventions should be taken to correct the associated orthopaedic deformities. Treatment of types III and IV lumbosacral agenesis is controversial. In these severe forms of agenesis periodic examinations of the spine for scoliosis should be performed and the patient must be monitored for spinopelvic instability as indicated by a worsening posture. The management of these and other orthopaedic deformities is controversial but we do advocate the surgical correction of fixed deformities of the lower extremities which interfere with sitting or with the wearing of braces or shoes thereby avoiding amputation and maintaining body image.