Literature DB >> 26642076

Long-term Follow-up of Pulmonary Function and Scoliosis in Patients With Duchenne's Muscular Dystrophy and Spinal Muscular Atrophy.

Kenon Chua1, Chin Yik Tan, Zhaojin Chen, Hee Kit Wong, Eng Hin Lee, Stacy K H Tay, Hian Tat Ong, Daniel Y T Goh, James H P Hui.   

Abstract

BACKGROUND: Spine surgery for neuromuscular scoliosis in patients with Duchenne's Muscular Dystrophy (DMD) and Spinal Muscular Atrophy (SMA) remained controversial. This study aimed to review the long-term results of spine surgery and its effect on pulmonary function in these patients.
METHODS: A retrospective review was conducted for the above patients who had undergone surgery from 1990 to 2006 in a tertiary hospital. Their yearly lung function tests, clinical records, and x-ray films before and after surgery were reviewed. All patients had at least 2 lung function tests performed before surgery and at least 3 lung function tests performed after surgery. Records of perioperative pulmonary infections that resulted in hospital admissions were also retrieved from the hospital computer system.
RESULTS: Forty patients were reviewed: 29 with DMD, 11 with SMA. The mean follow-up period was 11.6 years. For patients with DMD, the mean correction of Cobb's angle from surgery was 34.1 degrees. The rate of decline of the predicted forced vital capacity preoperatively was 7.80% per year, and was reduced to 4.26% per year postoperatively (P<0.001). For patients with SMA, the mean correction of Cobb's angle from surgery was 44.1 degrees. The rate of decline of the predicted forced vital capacity preoperatively was 5.31% per year, and was reduced to 1.77% per year postoperatively (P<0.001). For both DMD and SMA patients, the difference between the rate of preoperative and postoperative pulmonary infections that resulted in hospital admission were, however, not significant (P=0.433 and 0.452, respectively).
CONCLUSIONS: Scoliosis surgery in patients with DMD and SMA results in a long-term decreased rate of decline in pulmonary function over a follow-up period of more than 10 years. The level of the apical vertebrae of the scoliosis did not demonstrate a significant trend on the pulmonary function. The frequency of chest infections did not improve by scoliosis surgery. LEVEL OF SIGNIFICANCE: Level III—Retrospective study.

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Year:  2016        PMID: 26642076     DOI: 10.1097/BPO.0000000000000396

Source DB:  PubMed          Journal:  J Pediatr Orthop        ISSN: 0271-6798            Impact factor:   2.324


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7.  Short-term effect and effect on rate of lung function decline after surgery for neuromuscular or syndromic scoliosis.

Authors:  Esther S Veldhoen; Anneloes de Vries; Tom P C Schlosser; Moyo C Kruyt; Ruben P A van Eijk; Joyce M Tersmette; Erik H Hulzebos; Ludo W van der Pol; Roelie M Wösten-van Asperen; Cornelis K van der Ent
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  8 in total

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