Current Concepts - Congenital Scoliosis

Congenital scoliosis is one of the 'difficult to treat' scenarios which a spine surgeon has to face. Multiple factors including the age of child at presentation, no definite pattern of deformity and associated anomalies hinder the execution of the ideal treatment plan. All patients of congenital scoliosis need to be investigated in detail. X rays and MRI of spine is usually ordered first. Screening investigations to rule out VACTERL (Visceral, Anorectal, Cardiac, Tracheo-esophageal fistula, Renal and Lung) abnormalities are required. They are cardiac echocardiography and ultrasonography of abdomen and pelvis. CT scan is required to understand the complex deformity and is helpful in surgical planning.

A comprehensive medical literature review was done to understand the current surgical and non surgical treatment options available. An attempt was made to specifically study limitations and advantages of each procedure.

The treatment of congenital scoliosis differs with respect to the age of presentation. In adults with curves more than 50 degrees or spinal imbalance the preferred treatment is osteotomy and correction. In children the goals are different and treatment strategy has to be varied according to the age of patient. A single or two level hemivertebra can easily be treated with hemivertebra excision and short segment fusion. However, more than 3 levels or multiple fused ribs and chest wall abnormalities require a guided growth procedure to prevent thoracic insufficiency syndrome. The goal of management in childhood is to allow guided spine growth till the child reaches 10 - 12 years of age, when a definitive fusion can be done. The current research needs to be directed more at the prevention and understanding the etiology of the disease. Till that time, diagnosing the disease early and treating it before the sequels set in, is of paramount importance.

The primary aim of treatment of congenital scoliosis is to allow the expansion of chest and abdominal cavity, while keeping the deformity under control. Various methods can be categorized into definitive (hemivertebrectomy) or preventive (guided growth). Casting, Growth rods, Convex Epiphysiodesis are all guided growth measures. The guided growth procedure either 'corrects the deformity' or will have to be converted to a final fusion surgery once the child completes the spinal growth which is preferably done around 10 - 12 years of age. Future directions should aim at genetic counselling and early detection.