Costa Jorge Victor da Silva, Melonio Carlos Eduardo Coimbra, Vieira Ciro Bezerra, Oliveira Caio Marcio Barros de, Leal Plínio da Cunha, Servín Elizabeth Teixeira Noguera, Gomes Lyvia Maria Rodrigues de Sousa, Moura Ed Carlos Rey
Hospital São Domingos, São Luís, MA, Brasil.
Universidade Federal do Maranhão (UFMA), São Luís, MA, Brasil.
Braz J Anesthesiol. 2019 May-Jun;69(3):322-325. doi: 10.1016/j.bjan.2018.09.002. Epub 2018 Nov 20.
Pentalogy of Cantrell is a congenital anomaly associated with defects in the abdominal wall, sternum, diaphragm, and diaphragmatic pericardium formation, in addition to the development of cardiac abnormalities. It is a rare disease with an estimated incidence of one case for every 65,000 births, being more common in males (60% of cases). It has a reserved prognosis with mortality around 63%, and a maximum of 9 months survival after surgery. There are few case reports addressing the pentalogy of Cantrell, which is justified by the rarity of this pathology. In this report our objective was to describe a surgical case of a female patient and make some anesthetic considerations about this rare congenital malformation.
坎特雷尔五联症是一种先天性异常，除了心脏异常的发生外，还与腹壁、胸骨、膈肌和膈心包膜形成缺陷有关。它是一种罕见疾病，估计发病率为每65000例出生中有1例，在男性中更常见（占病例的60%）。其预后不佳，死亡率约为63%，术后最长存活9个月。关于坎特雷尔五联症的病例报告很少，这是由这种病理情况的罕见性所决定的。在本报告中，我们的目的是描述一名女性患者的手术病例，并对这种罕见的先天性畸形进行一些麻醉方面的考虑。
