All issues > Volume 25(4); 1982

Case Report

J Korean Pediatr Soc. 1982;25(4):419-422. Published online April 30, 1982.

A case of Pierre-Robin Syndrome.

Soon Ja Han, yeongok bang, Dong Hwan Lee, Sang Jhoo Lee

¹Department of Pediatrics, Soon Chun Hyang Medical College, Seoul, Korea.

Abstract

Micrognathia, cleft palate and glossoptosis, a combination of anomalies known as Pierre Robin syndrome, causes severe respiratory and alimentary difficulties in affected infants. With appropriate care and treatment, mental development can be made normal. But about 10~25% of children become retarded. We have experienced above case in a female infant, aged 6 months. She was acmitted on account of respiratory difficulty for days. The family history was not contributory. The child was born at full term, with a normal delivery, weighing 3.8kg. Cyanotic attack was not noticed since birth. Examination on admission showed small mandile, glissoptosis, cleft palate and low set ear. On auscultation of chest wall, fine moist rales were audible over right middle lung field. Marked supra and substernal retraction during respiration were noted also. She was treared under the impression of aspiration pueumonia. But frequent cyanotic attack was noticed during the course of hospitalization in spite of prone position. 9 th hospital day, tracheostomy was perfoermed. She was expired on the 10 th hospital day.

Keywords :Pierre Robin Syndrome;Respiratory Distress;Micrognathia