Meeker Tamara M
Pediatric General, Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
J Perinat Neonatal Nurs. 2009 Apr-Jun;23(2):186-94. doi: 10.1097/JPN.0b013e3181a3b4e5.
Pentalogy of Cantrell is a rare syndrome characterized by 5 distinctive anomalies: epigastric omphalocele, sternal cleft, diaphragmatic defect, diaphragmatic pericardial defect, and intracardiac defect. There are limited case reports documented since its first description in 1958 due to either underdocumentation or underrecognition. One possibility for underrecognition is the idea that there can be incomplete expressions of the syndrome, and these cases may be reported as separate diagnoses. This article explores the case of an infant with the prenatal diagnosis of giant omphalocele who was found to have a complete expression of the pentalogy. Infants with diagnoses such as giant omphalocele and diaphragmatic hernia require complex medical and surgical care. Nursing is in the perfect position to provide these infants with safe, quality, and interdisciplinary care and to provide vital, comprehensive teaching to their parents and families. The need for and value of comprehensive follow-up of these infants cannot be expressed enough.
坎特雷尔五联症是一种罕见的综合征，其特征为5种独特的异常：上腹部脐膨出、胸骨裂、膈肌缺损、膈心包缺损和心脏内缺损。自1958年首次描述以来，由于记录不足或认识不足，记录在案的病例报告有限。认识不足的一种可能性是，该综合征可能存在不完全表现，这些病例可能被报告为单独的诊断。本文探讨了一例产前诊断为巨大脐膨出的婴儿，该婴儿被发现患有完整表现的坎特雷尔五联症。患有巨大脐膨出和膈疝等诊断的婴儿需要复杂的医疗和外科护理。护士处于为这些婴儿提供安全、优质和跨学科护理，并为其父母和家庭提供重要、全面教导的理想位置。对这些婴儿进行全面随访的必要性和价值再怎么强调都不为过。
