All issues > Volume 52(12); 2009

Case Report

Korean J Pediatr. 2009;52(12):1388-1391. Published online December 15, 2009.

The first Korean case of poland-Möbius syndrome associated with dextrocardia

Jiyoung Jy Jung¹, Han Gyu HG Kim¹, Hae-Mi HM Ahn¹, Su Jin SJ Cho¹, Eun Ae EA Park¹

¹Department of Pediatrics, School of Medicine, Ewha Womans University, Seoul, Korea

Correspondence Eun Ae EA Park ,Email: pea8639@ewha.ac.kr

Abstract

Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Möbius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing 2.670 g was born at 38+3 weeks of gestation with left facial paralysis, left chest wall defect with dextrocardia, and symbrachydactyly between the second and third fingers. The combination of Poland–Möbius syndrome is rare, and only 2 cases associated with dextrocardia have been reported worldwide. Here, we report the first case of Poland-Möbius syndrome associated with dextrocardia in Korea.

Keywords :Poland-Möbius syndrome, Dextrocardia