Severe restrictive lung disease and vertebral surgery in a pediatric population

The aim of this study is to describe the outcome of surgical treatment for pediatric patients with forced vital capacity (FVC) <40% and severe vertebral deformity. Few studies have examined surgical treatment in these patients, who are considered to be at a high risk because of their pulmonary disease, and in whom preoperative tracheostomy is sometimes recommended. Inclusion criteria include FVC <40%, age <19 years and diagnosis of scoliosis. The retrospective study of 24 patients with severe restrictive lung disease, who underwent spinal surgery. Variables studied were age and gender, pre- and postoperative spirometry (FVC, FEV1, FEV1/FVC), preoperative, postoperative and late use of non-invasive ventilation (BiPAP) or mechanical ventilation, associated multidisciplinary treatment, type and location of the curve, pre- and postoperative curve values, type of vertebral fusion, intra- and postoperative complications, duration of intensive care unit (ICU) stay and length of postoperative hospitalization. Mean age was 13 years (9–19) of which 13 were males and 11 females. Mean follow-up was 32 months (24–45). The etiology was neuromuscular in 17 patients and other etiologies in 7 patients. Mean preoperative FVC was 26% (13–39%). Eight patients had preoperative home BiPAP, 15 preoperative in-hospital BiPAP, and 2 preoperative mechanical ventilation. Nine patients had preoperative nutritional support. Preoperative curve value of the deformity was 88° (40°–129°). Nineteen patients with posterior fusion alone and 5 with anterior and posterior fusion were found. Mean duration of ICU stay was 5 days (1–21). Total postoperative hospital stay was 17 days (7–33). Ventilatory support in the immediate postoperative includes 16 patients requiring BiPAP and 2 volumetric ventilation. None of the patients required a tracheostomy. The intraoperative complications include one death due to acute heart failure; immediate postoperative, four respiratory failures (2 required ICU readmission) and one respiratory infection; and other minor complications occurred in six patients. Overall, 58% of patients had complications. Percentage of angle correction was 56%. After a follow-up of 30 months, FVC was 29% (13–50%). In conclusion, corrective scoliosis surgery in pediatric patients with severe restrictive lung disease is well tolerated, but the management of this population requires extensive experience with the vertebral surgery involved, and a multidisciplinary approach that includes pulmonologists, nutritionists and anesthesiologists. Currently, there is no indication for routine preoperative tracheostomy.