Cingel Vladimir, Bohac Martin, Mestanova Veronika, Zabojnikova Lenka, Varga Ivan
Department of Pediatric Surgery, Faculty of Medicine, Children's Medical Hospital, Comenius University in Bratislava, Limbova Street 1, 833 40, Bratislava, Slovakia.
Surg Radiol Anat. 2013 Oct;35(8):639-46. doi: 10.1007/s00276-013-1083-7. Epub 2013 Feb 19.
Poland syndrome is a rare congenital anomaly described by Sir Alfred Poland over 170 years ago. Combination of unilateral aplasia of the sternocostal head of musculus pectoralis major, and an ipsilateral hypoplastic hand with simple syndactyly and short fingers is typical for this condition. It occurs more frequent among males, and is usually situated on the right hemithorax in the unilateral form. The pathogenesis of Poland syndrome is not clear. Most of the authors assume that the etiologic insult is vascular in nature. During the sixth week of gestation, not only the pectoral mass splits (future muscles of the thorax) and intervening tissue between the finger rays of hands starts to disappear but also the vascular differentiation from six aortic arches begins. In our paper we report two cases of children with Poland syndrome, who underwent surgical procedure in the Department of Pediatric Surgery, Comenius University in Bratislava, Slovakia. Our case reports are focused on pre-operatively and also post-operatively imaging (RTG, CT, and 3D CT imaging) of the affected thorax and arm, as well as the operative reconstruction technique of abnormal ribs. We also discussed the possible embryonic backgrounds of this anomaly as well as the importance of plastic surgery resulting in patients' normal life.
波兰综合征是170多年前由阿尔弗雷德·波兰爵士描述的一种罕见先天性异常。典型表现为单侧胸大肌胸肋头发育不全，以及同侧手部发育不全并伴有单纯并指和手指短小。该病在男性中更为常见，单侧形式通常位于右半胸。波兰综合征的发病机制尚不清楚。大多数作者认为病因是血管性的。在妊娠第六周，不仅胸肌团块分裂（未来的胸部肌肉），手部指骨间的中间组织开始消失，而且来自六个主动脉弓的血管分化也开始了。在我们的论文中，我们报告了两例波兰综合征患儿，他们在斯洛伐克布拉迪斯拉发夸美纽斯大学小儿外科接受了手术。我们的病例报告重点关注了患侧胸部和手臂的术前及术后影像学检查（X线、CT和3D CT成像），以及异常肋骨的手术重建技术。我们还讨论了这种异常可能的胚胎学背景以及整形手术对患者正常生活的重要性。
