Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation

Abstract

Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.