Abstract

Pentalogy of Cantrell (PC) is a malformation characterized by defects in the ventral abdominal wall, lower sternum, diaphragmatic pericardium, anterior diaphragm associated with omphalocele, thoraco-abdominal ectopia cordis, diaphragmatic hernia, and intracardiac abnormalities. PC is stratified as complete or incomplete and we present both the complete and incomplete forms.

Highlights

  • Pentalogy of Cantrell (PC) is a malformation characterized by defects in the ventral abdominal wall, lower sternum, diaphragmatic pericardium and anterior diaphragm associated with omphalocele, thoraco-abdominal ectopia cordis, diaphragmatic hernia, and intracardiac abnormalities, all of which were present below in Case 1 [1]

  • A 39-week gestation infant was born to a 20-year-old primigravida whose pregnancy was complicated by pre-gestational diabetes mellitus and late prenatal care starting at 31 weeks, when the fetus was diagnosed with PC

  • Prenatal ultrasound showed a large omphalocele with herniation of liver, anterior diaphragmatic hernia, absence of lower sternum, ectopia cordis, and a ventricular septal defect (VSD)

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Summary

Introduction

Pentalogy of Cantrell (PC) is a malformation characterized by defects in the ventral abdominal wall, lower sternum, diaphragmatic pericardium and anterior diaphragm associated with omphalocele, thoraco-abdominal ectopia cordis, diaphragmatic hernia, and intracardiac abnormalities, all of which were present below in Case 1 (clinical and autopsy findings) [1]. We describe both the complete and incomplete forms of PC

Case 1
Case 2

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