Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2018.09.011
Brazilian Journal of Anesthesiology
Clinical Information

Anesthesia for surgical repair of the pentalogy of Cantrell: case report

Anestesia em cirurgia para correção de pentalogia de Cantrell: relato de caso

Jorge Victor da Silva Costa; Carlos Eduardo Coimbra Melonio; Ciro Bezerra Vieira; Caio Marcio Barros de Oliveira; Plínio da Cunha Leal; Elizabeth Teixeira Noguera Servín; Lyvia Maria Rodrigues de Sousa Gomes; Ed Carlos Rey Moura

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Abstract

Abstract 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.

Keywords

Pentalogy of Cantrell, Congenital malformation, Rare disease

Resumo

Resumo A pentalogia de Cantrell é uma anomalia congênita associada a defeitos na formação da parede abdominal, do esterno, diafragma e pericárdio diafragmático, além do desenvolvimento de anomalias cardíacas. É uma doença rara, com incidência estimada em um caso para cada 65.000 nascimentos, mais comum no sexo masculino (60% dos casos). Apresenta prognóstico reservado com mortalidade em torno de 63% e sobrevida após procedimento cirúrgico de no máximo nove meses. São escassos os relatos de casos referentes à pentalogia de Cantrell, o que se justifica pela raridade dessa patologia. Com este relato, os autores objetivam descrever um caso cirúrgico, em paciente do sexo feminino, e tecer algumas considerações anestésicas sobre essa malformação congênita rara.

Palavras-chave

Pentalogia de Cantrell, Malformação congênita, Doença rara

References

Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107:602-14.

Divkovic D, Kvolik S, Sipl M. A successful early gore-tex reconstruction of an abdominal wall defect in a neonate with Cantrell pentalogy: a case report and literature review. Clin Case Rep. 2015;3:19-23.

Saito T, Suzuki A, Takahata O. Anesthetic management of a patient with Cantrell's pentalogy diagnosed prenatally. Can J Anaesth. 2004;51:946-7.

Wheeler DS, St Louis JD. Pentalogy of Cantrell associated with hypoplastic left heart syndrome. Pediatr Cardiol. 2007;28:311-3.

Rubens FJ, Sosa CEF, Díaz GL. Cardiac malformations in patients with pentalogy of Cantrell and ectopia cordis. Rev Esp Cardiol. 2011;64:615-8.

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Braz J Anesthesiol

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