Iranian Journal of Neonatology

Iranian Journal of Neonatology

Pentalogy of Cantrell: A case report of probable pentalogy of Cantrell in a full-term neonate

Document Type : Case Report

Authors
Mina Khosravifar 1
Homa Babaei 2
Nona Rahbari 1
1 Student Research Committee, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran
2 Neonatal Ward, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran
10.22038/ijn.2020.50902.1895
Abstract
Background: Pentalogy of Cantrell (PC) is an extremely rare congenital anomaly which was first described in 1985. The incidence of the PC has been reported to vary from 5.5-7.9 cases per million live births. This anomaly involves diaphragmatic pericardial defects, lower sternal defect, intracardiac anomalies, ventral abdominal wall abnormality, and anterior diaphragmatic defect. Considering the number of presented anomalies, PC is classified into three groups of certain, probable, or incomplete pentalogy. Although the etiology of PC is unknown, it has been assumed that abnormalities in intra embryonic mesodermal differentiation formation and migration at around 14-18 days after conception are responsible for the anomalies observed in PC. The prenatal diagnosis of the PC can be made via prenatal two-dimensional or three-dimensional ultrasound.
Case report: This case report investigated an Iranian 2-hour-old full-term neonate who was referred to Imam Reza Hospital, Kermanshah, Iran, due to respiratory distress and abdominal wall defect. The patient was finally diagnosed with a probable PC, although his prenatal investigations were considered normal.
Conclusion: Although ultrasonography is a widely available tool for prenatal diagnosis of PC, as in our case, the absence of ectopic heart and omphalocele makes the prenatal diagnosis of PC via ultrasonography less possible.
 
Keyword
Keywords
Abdominal wall defects
Diaphragmatic defects
Pentalogy of cantrell
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Iranian Journal of Neonatology
Volume 12, Issue 1
Winter 2021
Pages 96-99