Thoracic Dystrophy

Mark V Mazziotti, MD, Meghna V Misra, MD, Fizan Abdullah, MD, PhD, David Sigalet, MD, PhD, Jamie C Harris, MD
Thoracic Dystrophy is a topic covered in the Pediatric Surgery NaT.

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Introduction

Asphyxiating thoracic dystrophy, or Jeune syndrome, was first described in 1954 [1]. The case report was of a newborn with a narrow, rigid chest and multiple cartilage anomalies that led to respiratory insufficiency. Patients also have limb defects and mild dwarfism. The short, wide ribs and abnormal costochondral junctions result in a narrow bell shaped chest.

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Introduction

Asphyxiating thoracic dystrophy, or Jeune syndrome, was first described in 1954 [1]. The case report was of a newborn with a narrow, rigid chest and multiple cartilage anomalies that led to respiratory insufficiency. Patients also have limb defects and mild dwarfism. The short, wide ribs and abnormal costochondral junctions result in a narrow bell shaped chest.

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Last updated: January 16, 2017