Neonatology, KK Women's and Children's Hospital, Singapore.
Neonatology, Salmanya Medical Complex, Kingdom of Bahrain, Bahrain.
BMJ Case Rep. 2021 Jan 28;14(1):e238392. doi: 10.1136/bcr-2020-238392.
A preterm female infant was admitted at birth with respiratory distress. On examination, she had an asymmetric right chest wall and ipsilateral small hand. Air entry was reduced over the right chest. A clinical diagnosis of Poland's syndrome was made based on the hypoplasia of the right pectoral muscles, absent nipple, deformed ribs and symbrachydactyly of the ipsilateral hand. Chest X-ray suggested and ultrasound confirmed eventration of the right hemidiaphragm. 'Subclavian artery supply disruption sequence' (SASDS) theory by Bavnick and Weaver remains the most accepted pathogenic mechanism in Poland's syndrome. This case reinforces SASDS theory associated with the genesis of Poland's syndrome that relates to the pathogenicity of vascular disruption of subclavian artery, characteristics of which are unilateral pectoral defects, symbrachydactyly and eventration of the diaphragm. At 2 months, she underwent diaphragm plication. She is under review by our multidisciplinary surgical team for reconstruction of the chest deformity.
一名早产女性婴儿出生时因呼吸窘迫入院。检查时，她的右侧胸壁不对称，同侧小手也较小。右侧胸部的空气进入减少。根据右侧胸肌发育不良、乳头缺失、肋骨变形和同侧手的并指畸形，临床诊断为波兰氏综合征。胸部 X 线片提示右侧膈肌膨出，超声检查也证实了这一点。Bavnick 和 Weaver 的“锁骨下动脉供应中断序列”（SASDS）理论仍然是波兰氏综合征最被接受的发病机制。这个病例加强了与波兰氏综合征发生相关的 SASDS 理论，该理论与锁骨下动脉血管中断的致病性有关，其特征是单侧胸肌缺陷、并指畸形和膈肌膨出。她在 2 个月大时接受了膈肌折叠术。她正在接受我们多学科手术团队的审查，以进行胸部畸形的重建。
