Sanchís Solera L, Beltrá Picó R, Castro Sánchez M, Serrano González A, Sánchez López J M, Hernández Navarro J, Arteaga García R, Santana Ramírez R, Maldonado Artero J, Uroz Tristán J
Servicio de Cirugía Pediátrica, Hospital Materno-Infantil, Las Palmas de Gran Canaria.
Cir Pediatr. 1992 Apr;5(2):101-4.
Since MAYOR and CANTRELL described in 1953 and in 1958 the ectopia cordis associated to other four anomalies, few cases have been reported, and only a small part of them have survived. We report the case of a neonate who presented with the features of the pentalogy. On the 3rd day we found at operation the heart laying over the left hepatic lobe, no pericardium could be identified and diaphragm was absent at its anterior edge. We made an "isolating sac" with duramadre fixed to the anterior thoracic wall, to both sides and to the posterior diaphragmatic defect. The initial management of the ventral defect is described. At the age of two years we initiated the definitive treatment aiming to: 1. To protect the heart. 2. To correct the huge abdominal eventration following the closure of the omphalocele. We described in details the technic employed to cover the precordial defect, to reconstruct the diaphragm, and finally as we treated in three consecutives stages the huge abdominal eventration.
自1953年和1958年MAYOR和CANTRELL描述了与其他四种异常相关的心脏异位以来，报道的病例很少，其中只有一小部分存活下来。我们报告了一例具有五联症特征的新生儿病例。在第3天手术时，我们发现心脏位于左肝叶上方，未发现心包，且膈肌前缘缺失。我们用硬脑膜制作了一个“隔离囊”，固定在前胸壁、两侧和后膈肌缺损处。描述了腹侧缺损的初始处理方法。在患儿两岁时，我们开始进行确定性治疗，目标是：1. 保护心脏。2. 纠正脐膨出闭合后巨大的腹壁疝。我们详细描述了用于覆盖心前区缺损、重建膈肌以及最终分三个连续阶段治疗巨大腹壁疝所采用的技术。
