Displasia Torácica Asfixiante o Síndrome de Jeune

Abstract

Jeune Syndrome is a rare skeletal dystrophy, characterized by short ribs and alterations in limbs and pelvis. The respiratory distress is determined by the degree of thoracic stricture, and later, pancreatic or renal insufficiency may occur. A case of a 7-months-old infant who came to the Vicente Corral Moscoso Hospital for pneumonia without treatment response in the cantonal hospital was presented. When the patient arrives he presents a respiratory insufficiency and it calls the attention a cylindrical thorax, markedly narrow and elongated in addition of micromelia and polydactyly. A respiratory support and antibiotics were given during hospitalization having a favorable evolution.