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Pulmonary dysfunction and reduced exercise capacity in patients with myelomeningocele
M S Sherman1, J M Kaplan, S Effgen
1Department of Medicine, MCP Hahnemann School of Medicine, Allegheny University of the Health Sciences, Philadelphia, Pennsylvania, USA.
Insights
Children with myelomeningocele often have reduced pulmonary function and exercise capacity, with muscle weakness masking underlying lung issues. Pulmonary function testing is recommended for early detection of these abnormalities.
Area of Science:
- Pediatric Pulmonology
- Neuromuscular Disorders
- Rehabilitation Medicine
Background:
- Myelomeningocele is a complex congenital condition affecting neurological and musculoskeletal systems.
- Pulmonary function and exercise capacity are critical indicators of health and quality of life in children.
Purpose of the Study:
- To evaluate pulmonary function and exercise capacity in children diagnosed with myelomeningocele.
- To identify the prevalence of restrictive lung disease and respiratory muscle weakness in this population.
Main Methods:
- Prospective evaluation of 12 children (10-17 years) with myelomeningocele and 12 matched controls.
- Measurements included spirometry, lung volumes, maximum respiratory pressures, and maximal oxygen uptake during arm ergometry.
Main Results:
- Children with myelomeningocele showed significantly lower lung volumes and forced vital capacity.
- 75% exhibited respiratory muscle weakness, and 58% had restrictive lung disease.
- Exercise capacity was reduced, evidenced by lower maximal oxygen consumption and anaerobic threshold.
Conclusions:
- Exercise capacity in myelomeningocele is often limited by skeletal muscle weakness, potentially masking pulmonary abnormalities.
- Pulmonary function deficits, including restrictive lung disease and respiratory muscle weakness, are common.
- Pulmonary function testing is advised for screening these subclinical abnormalities.
Objective:
To evaluate pulmonary function and exercise capacity in children with myelomeningocele.
Study Design:
Prospective evaluation in a randomly selected cohort of 12 subjects (10 to 17 years of age) with myelomeningocele and 12 control subjects matched for age, sex, and arm span.
Methods:
Spirometry, lung volumes, maximum respiratory pressures, maximum oxygen expenditure during arm ergometry, and anaerobic threshold were measured.
Results:
Mean total lung capacity and fractional lung volumes were significantly lower in case subjects than control subjects. Eleven subjects (92%) had a reduced forced vital capacity; seven (58%) had restrictive disease as evidenced by reductions in total lung capacity with normal or increased forced expiratory volume in 1 second/forced vital capacity ratio. Nine subjects (75%) had respiratory muscle weakness as evidenced by reduced maximum respiratory pressures or a low maximum voluntary ventilation. Exercise capacity was reduced as evidenced by a lower maximum oxygen consumption at peak exercise (13.8 +/- 4.8 vs 21.3 +/- 7.5 ml/min per kilogram of body weight; p < 0.02) and a lower anaerobic threshold (12.4 +/- 5.1 vs 17.3 +/- 4.2 ml/min per kilogram; p < 0.01) than the control group. Though the majority of subjects with myelomeningocele had a significant degree of restrictive disease, respiratory muscle weakness, or both, only one subject had pulmonary symptoms during exercise.
Conclusions:
Though most subjects with myelomeningocele had a significant degree of restrictive lung disease, respiratory muscle weakness, or both, exercise capacity was mostly limited by arm weakness. Skeletal muscle weakness may mask the symptoms of an underlying pulmonary abnormality, which may not be evident unless a pathologic cause of increased ventilation is present. Pulmonary function testing is suggested to screen for these abnormalities.