Abstract
BACKGROUND: Severe spinal deformity (Cobb angle > 90°) often leads to cardiopulmonary dysfunction, posing significant surgical risks. We report a rare case of severe kyphoscoliosis with cardiopulmonary failure treated with non-invasive ventilation, anti-shock treatment, Halo-pelvic traction (HPT), and orthopedic surgery, emphasizing the importance of multidisciplinary cooperation. CASE PRESENTATION: A 13-year-old Boy with genetic suspicion of distal arthrogryposis (Type 5D) presented with Cobb angles of 94° (scoliosis) and 69° (kyphosis), respiratory failure (PO(2) 36.3 mmHg), and pre-shock status. A multidisciplinary team stabilized the patient using non-invasive ventilation, nutritional optimization and HPT. Post-traction correction rates reached 46.8% (coronal) and 53.6% (sagittal). Subsequent posterior spinal fusion (T1-L5) achieved 69% correction, resolving cardiopulmonary dysfunction and resulting in a highly satisfactory therapeutic outcome. CONCLUSIONS: This case illustrates a case with severe spinal deformity combined with extremely severe cardiopulmonary dysfunction and highlights the importance of multidisciplinary cooperation in high-risk pediatric patients.