Pierre Robin Syndrome

PULMONARY hypertension, cardiomegaly, and pulmonary edema have been reported in respiratory obstruction resulting from hypertrophied tonsils and adenoids, and from tracheomalacia in childhood.^1-5However, these cardiorespiratory changes have not been documented in the respiratory obstruction secondary to the micrognathia and the glossoptosis of the Pierre Robin syndrome.^6,7The present report describes a case of this syndrome with severe respiratory obstruction, hypercapnia, cardiomegaly, right ventricular hypertrophy, and pulmonary edema. Tracheostomy resulted in gradual regression of these manifestations.

Report of a Case

A white female infant was born at St. Francis Hospital on March 1, 1966, with an Apgar score of 10. Her birth weight was 2,760 gm (6 lb 2 ounces). She was noted to have the following classical features of the Pierre Robin syndrome: micrognathia, glossoptosis, and cleft palate accompanied by slight respiratory distress and cyanosis. She was kept in the prone position, and on March 2, 1966,