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,
Jeresaty RM, Huszar RJ, Basu S. Pierre Robin Syndrome: Cause of Respiratory Obstruction, Cor Pulmonale, and Pulmonary Edema. Am J Dis Child. 1969;117(6):710–716. doi:10.1001/archpedi.1969.02100030712018
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