Untreated patient of Pentalogy of Cantrell surviving into the sixth decade: A unique case report

The Pentalogy of Cantrell: A Case Report

“…Prenatal imaging studies are crucial for prenatal counseling and adequate postnatal therapeutic planning [25]. The diagnosis of PC may be established by fetal ultrasonography during the first trimester of the pregnancy as early as 10 weeks of pregnancy [26,27]. Attempts should be made to exclude this syndrome in patients with omphalocele, diaphragmatic hernia and pericardial defect accompanied by pericardial effusion detected during prenatal assessment [26].…”

“…Attempts should be made to exclude this syndrome in patients with omphalocele, diaphragmatic hernia and pericardial defect accompanied by pericardial effusion detected during prenatal assessment [26]. Amniocentesis might be performed because of its association with genetic disorder [27].…”

“…Fetal echocardiography and MRI will provide more detail assessments as well as more comprehensive examination of the cardiac structure, which is important for pediatrician and surgeon to provide care and treatment for the infant following delivery [26,28]. Nowadays, three-dimensional sonography has been widely used and preferred by physicians for these patients [27]. While other study stated that 2D sonography with Doppler imaging is the choice for sonography assessment of abdominal wall defects during the first trimester.…”

“…However, Nichal et al reported a unique case which untreated patient of PC survived into sixth decade with omphalocele and bifid lower sternum and herniation of cardiac apex. He was managing the swelling with an abdominal wall harness for support [27].…”