Paediatrica Indonesiana, Vol. 59, No. 1, January 2019
Baby girl with pentalogy of Cantrell: a case report on an extremely rare condition
Widiastuti1, Suyono1, Johannes Berchmans Prasodjo1, Sri Lilijanti Widjaja2
In 1958, Cantrell et al. described an extremely rare syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart malformation.1 The incidence of pentalogy of Cantrell (POC) is one in 65,000 live births. 1,2,3 Only one case was reported in Dr. Moewardi Public Hospital between 1999 and 2016. The exact etiology of this condition is unknown, but developmental failure of mesoderm at 4 weeks of gestational age may contribute to the condition. 4 The prognosis depends on the degree of heart failure and the malformations that occur. Heart failure, arrhythmia, cardiac rupture, cardiac tamponade, endocarditis, and peripheral emboli are described as the main complications and causes of death. 5,6 The aim of this report was to add to reference data about complete POC and the prognostic outcome. [Paediatr Indones. 2019;59:51-4; doi : http://dx.doi. org/10.14238/pi59.1.2019.51-4]
Keywords: pentalogy of Cantrell; ectopia cordis; omphalocele
From the Department of Radiology1and Department of Child Health2, Universitas Sebelas Maret Medical School/Dr. Moewardi Public Hospital, Surakarta, Central Java, Indonesia. Corresponding author: Widiastuti. Department of Radiology, Universitas Sebelas Maret Medical School/Dr. Moewardi Public Hospital. Telp. 0271648667, Email: email@example.com.