Impact of neurological level and spinal curvature on pulmonary function in adults with spina bifida

Issue title: Spina Bifida Care Among a Global Community

Guest editors: Jonathan Castillo

Article type: Research Article

Authors: Crytzer, Theresa M.^{a; b; c; *} | Cheng, Yu-Ting^b | Bryner, Mary Jo^d | Wilson III, Robert^d | Sciurba, Frank C.^{d; 1} | Dicianno, Brad E.^{a; b; c; e; f; 1}

Affiliations: [a] Human Engineering Research Laboratories, Veterans Affairs Pittsburgh Healthcare System, Pittsburgh, PA, USA | [b] Department of Rehabilitation Science and Technology, School of Health and Rehabilitation Sciences, University of Pittsburgh, Pittsburgh, PA, USA | [c] Center for Assistive Technology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA | [d] Division of Pulmonary, Allergy, and Critical Care Medicine, Emphysema COPD Research Center, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, USA | [e] Department of Physical Medicine and Rehabilitation, School of Medicine, University of Pittsburgh, Pittsburgh, PA, USA | [f] Adult Spina Bifida Clinic, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

Correspondence: [*] Corresponding author: Theresa M. Crytzer, Department of Rehabilitation Science and Technology, School of Health and Rehabilitation Sciences, University of Pittsburgh, Bakery Square, Suite 400, 6425 Penn Avenue, Pittsburgh, PA 15206, USA. Tel.: +1 412 822 3694; E-mail: [email protected].

Note: [1] These authors contributed equally to the senior leadership on this manuscript, Brad Dicianno, MD with respect to disease content and Frank Sciurba, MD with respect to pulmonary physiology.

Abstract: PURPOSE: To describe pulmonary function and determine the impact of neurological level, scoliosis, and obesity on pulmonary function in people with spina bifida (SB). METHODS: Participants with SB (N= 29) (15 females; age, 30 ± 12 years) completed spirometry and body plethysmographic lung volume testing. Univariate and multivariate regression analyses were used to describe the factors associated with pulmonary function in people with SB. RESULTS: Distribution of category of impairment in pulmonary function was: 55% (n= 16) restricted, 6.9% (n= 2) spirometric restricted, 1 combined obstructed and restricted, and 35.5% (n= 10) normal. In univariate analyses, neurological level was negatively associated with pulmonary function parameters, i.e., forced vital capacity (FVC) (p= 0.005), forced expiratory volume in 1 second (FEV1) (p= 0.008), total lung capacity (TLC) (p= 0.001), and degree of scoliosis were inversely associated with FVC (p= 0.005), FEV1 (p= 0.003), and TLC (p= 0.004). In multivariate models, level of lesion and degree of scoliosis independently contributed to the degree of lung function impairment. Restrictive pulmonary function was observed in 9/10 (90%) of those with thoracic neurological levels and was associated with decreased inspiratory capacity (IC) and expiratory reserve volume (ERV). Lumbar level lesions were associated with either normal lung function or an isolated reduction in FVC due to reduction in only ERV and preserved TLC representing spirometric restriction. CONCLUSIONS: High prevalence of restrictive pulmonary physiology is present in people with SB, with more rostral neurological levels and greater degree of scoliosis associated with a higher degree of pulmonary function impairment.

Keywords: Spina bifida, myelomeningocele, pulmonary function, scoliosis, neurological level, restrictive lung disease, total lung capacity, wheelchair, spirometry, lung volume, spirometric restriction, mobility impairment

DOI: 10.3233/PRM-179451

Journal: Journal of Pediatric Rehabilitation Medicine, vol. 11, no. 4, pp. 243-254, 2018