Xu Beiyu, Li Chao, Qi Longtao, Zhao Yao, Wu Wence, Yang Chengxian, Zhu Ranlv, Yu Zhengrong, Li Chunde
Department of Orthopedic/Spine Surgery, Peking University First Hospital, Beijing, 100034, China.
Ital J Pediatr. 2025 Mar 24;51(1):92. doi: 10.1186/s13052-025-01925-9.
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.
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 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.
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.
严重脊柱畸形（Cobb角＞90°）常导致心肺功能障碍，带来重大手术风险。我们报告一例罕见的严重脊柱后凸畸形合并心肺功能衰竭病例，采用无创通气、抗休克治疗、头盆环牵引（HPT）及矫形手术进行治疗，强调多学科合作的重要性。
一名13岁男孩，疑似遗传性远端关节挛缩症（5D型），Cobb角为94°（脊柱侧凸）和69°（脊柱后凸），出现呼吸衰竭（动脉血氧分压36.3 mmHg）及休克前期状态。一个多学科团队通过无创通气、营养优化及HPT使患者病情稳定。牵引后矫正率达到46.8%（冠状面）和53.6%（矢状面）。随后的后路脊柱融合术（T1-L5）实现了69%的矫正，解决了心肺功能障碍问题，取得了非常满意的治疗效果。
本病例展示了一例严重脊柱畸形合并极其严重心肺功能障碍的病例，并强调了多学科合作在高危儿科患者中的重要性。
