Thoracic Dystrophy

Patrick Berg, M.D., Raquel González, MD, MHCM, Heather Nolan, M.D., Paul D Danielson, MD, Nicole Chandler, M.D., Mark V Mazziotti, MD, Meghna V Misra, MD, Fizan Abdullah, MD, PhD, David Sigalet, MD, PhD, Jamie C Harris, MD

Introduction

Asphyxiating thoracic dystrophy, or Jeune syndrome, was first described in 1954 as a case report describing a newborn with a narrow, rigid chest and multiple cartilage anomalies that led to respiratory insufficiency [1]. Other noted associations include limb defects and mild dwarfism. Ultimately, short, wide ribs and abnormal costochondral junctions result in a narrow bell-shaped chest.

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Last updated: November 14, 2021