Pleural fibrosis mediates shrinking lungs syndrome in children

Abstract

The etiology of restrictive lung physiology in SLS is not well-defined, and has been hypothesized to be due to defects in lung recoil, phrenic nerve function and diaphragmatic strength. We present a case of SLS in an adolescent in whom imaging and electrophysiology studies demonstrate pleural fibrosis as the fundamental defect accounting for the restrictive lung physiology.