Retrospective Analysis of Associated Anomalies in 636 Patients with Operatively Treated Congenital Scoliosis

Author:

Wu Nan123ORCID,Liu Lian1234,Zhang Yuanqiang1235,Wang Lianlei1235,Wang Shengru123,Zhao Sen1236,Li Guozhuang1236ORCID,Yang Yang123ORCID,Lin Guanfeng123,Shen Jianxiong123,Wu Zhihong123,Qiu Guixing123,Zhang Terry Jianguo123

Affiliation:

1. Department of Orthopedic Surgery, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, People’s Republic of China

2. Beijing Key Laboratory for Genetic Research of Skeletal Deformity, Beijing, People’s Republic of China

3. Key Laboratory of Big Data for Spinal Deformities, Chinese Academy of Medical Sciences, Beijing, People’s Republic of China

4. Department of Emergency Surgery, Qilu Hospital of Shandong University, Jinan, Shandong Province, People’s Republic of China

5. Department of Orthopedic Surgery, Qilu Hospital of Shandong University, Jinan, Shandong Province, People’s Republic of China

6. Graduate School of Peking Union Medical College, Beijing, People’s Republic of China

Abstract

Update This article was updated on December 22, 2023, because of a previous error. Specifically, there was an error in one of the grant numbers mentioned in the article. In the Source of Funding paragraph, the text that had previously read “2022-PUMCH-D-007” now reads “2022-PUMCH-D-004.” Background: Congenital scoliosis is frequently associated with anomalies in multiple organ systems. However, the prevalence and distribution of associated anomalies remain unclear, and there is a large amount of variation in data among different studies. Methods: Six hundred and thirty-six Chinese patients who had undergone scoliosis correction surgery at Peking Union Medical College Hospital from January 2012 to July 2019 were recruited, as a part of the Deciphering disorders Involving Scoliosis and COmorbidities (DISCO) study. The medical data for each subject were collected and analyzed. Results: The mean age (and standard deviation) at the time of presentation for scoliosis was 6.4 ± 6.3 years, and the mean Cobb angle of the major curve was 60.8° ± 26.5°. Intraspinal abnormalities were found in 186 (30.3%) of 614 patients, with diastematomyelia being the most common anomaly (59.1%; 110 of 186). The prevalence of intraspinal abnormalities was remarkably higher in patients with failure of segmentation and mixed deformities than in patients with failure of formation (p < 0.001). Patients with intraspinal anomalies showed more severe deformities, including larger Cobb angles of the major curve (p < 0.001). We also demonstrated that cardiac anomalies were associated with remarkably worse pulmonary function, i.e., lower forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF). Additionally, we identified associations among different concomitant malformations. We found that patients with musculoskeletal anomalies of types other than intraspinal and maxillofacial were 9.2 times more likely to have additional maxillofacial anomalies. Conclusions: In our cohort, comorbidities associated with congenital scoliosis occurred at a rate of 55%. To our knowledge, our study is the first to show that patients with congenital scoliosis and cardiac anomalies have reduced pulmonary function, as demonstrated by lower FEV1, FVC, and PEF. Moreover, the potential associations among concomitant anomalies revealed the importance of a comprehensive preoperative evaluation scheme. Level of Evidence: Diagnostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Orthopedics and Sports Medicine,General Medicine,Surgery

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