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June 1969

Pierre Robin Syndrome: Cause of Respiratory Obstruction, Cor Pulmonale, and Pulmonary Edema

Author Affiliations

Hartford, Conn
From the Section of Cardiopulmonary Medicine, St. Francis Hospital, Hartford, Conn.

Am J Dis Child. 1969;117(6):710-716. doi:10.1001/archpedi.1969.02100030712018

PULMONARY hypertension, cardiomegaly, and pulmonary edema have been reported in respiratory obstruction resulting from hypertrophied tonsils and adenoids, and from tracheomalacia in childhood.1-5 However, these cardiorespiratory changes have not been documented in the respiratory obstruction secondary to the micrognathia and the glossoptosis of the Pierre Robin syndrome.6,7 The 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,

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