Pierre Robin Syndrome ini Sister and Brother

Lee, B.Y.; Park, S.H.; Ko, K.H.; Chung, H.Y.; Lee, W.B.; Sohn, H.J.; Choi, H.Y.

Case Report

Journal of the Korean Pediatric Society 1969;12(6):329-334.
Published online June 30, 1969.

Pierre Robin Syndrome ini Sister and Brother

B.Y. Lee¹, S.H. Park¹, K.H. Ko¹, H.Y. Chung², W.B. Lee², H.J. Sohn³, H.Y. Choi³

¹Department of Pediatrics, Yonsei University College of Medicine
²Department of Pediatrics, Korea Hospital
³Department of Pathology, Yonsei University College of Medicine

男妹에서 본 Pierre Robin Syndrome

李炳爆^¹^, 朴商厚^¹^, 高克勳^¹^, 鄭昰泳^²^, 李源柄^²^, 孫照俊^³^, 崔弘恱^³

^¹延世大學校醫科大學小兒科學敎室
^²高麗病院産婦人科
^³延世大學校醫科大學病理學敎室

Abstract

Two cases of Pierre Robin syndrome, which appeared in sister and brother, are reported. The first case was a girl who showed characteristic pictures of Pierre Robin syndrome, characterized by micrognathia, glossoptosis and cleft palate at birth. There had been dyspnea with feeding difficulties since birth but improved by supportive treatment only. The second case was a boy, who was born as a subsequent baby from the same mother, and revealed also typical picture. He expired 20 hours later and autopsy was performed. Mandibular growth was markedly impaired resulting glossoptosis. Mild coarctation of aorta was found with Meckefs diverticulum. A brief review of literature was done.