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Prevention of Pectus Excavatum for Children with Spinal Muscular Atrophy Type 1

Bach, John R. MD, FAAPMR, FCCP; Bianchi, Carlo MD

American Journal of Physical Medicine & Rehabilitation: October 2003 - Volume 82 - Issue 10 - p 815-819
doi: 10.1097/01.PHM.0000083669.22483.04
Case Reports: Pulmonary
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Bach JR, Bianchi C: Prevention of pectus excavatum for children with spinal muscular atrophy type 1. Am J Phys Med Rehabil 2003;82:815–819.

To demonstrate the elimination of pectus excavatum and promotion of more normal lung growth and chest wall development by the use of high-span positive inspiratory pressure plus positive end-expiratory pressure (PIP+PEEP), patients with spinal muscular atrophy type 1 with paradoxical breathing were placed on high-span PIP+PEEP when sleeping from the point of diagnosis of spinal muscular atrophy. Although the appearance of pectus excavatum is ubiquitous in untreated infants with spinal muscular atrophy type 1, after institution of high-span PIP+PEEP, pectus resolves and lungs and chest walls grow more normally. High-span PIP+PEEP is indicated for all infants diagnosed with spinal muscular atrophy who demonstrate paradoxical breathing for the purpose of promoting more normal lung and chest development.

From the Departments of Physical Medicine and Rehabilitation and Neurosciences, UMDNJ–New Jersey Medical School, Newark, New Jersey (JRB); and the Department of Physical Medicine and Rehabilitation of the Instituto Sacra Famiglia, Cesano Boscone, Italy (CB).

All correspondence and requests for reprints should be addressed to John R. Bach, MD, Department of Physical Medicine and Rehabilitation, University Hospital B-403, UMDNJ-New Jersey Medical School, 150 Bergen Street, Newark, NJ 07103.

© 2003 Lippincott Williams & Wilkins, Inc.