Pentalogy of Cantrell with Left Ventricular Diverticulum: A Case Report and Review of Literature

Abstract

Pentalogy of Cantrell is a rare congenital anomaly involving deficiency of the following structures: anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and associated congenital intracardiac abnormality. We describe a 3-month-old child with this syndrome having left ventricular diverticulum along with omphalocele who presented to us with a pulsating mass in the epigastrium. The defect was evaluated and defined by computed tomography scan. A team of pediatric, cardiac, and plastic surgeons successfully repaired the defects. This case report discusses the review of literature along with management options and concludes that there must be an emphasis on early repair of left ventricular diverticulum to prevent complications. Antenatal ultrasound can also detect the anomaly, and early postnatal diagnosis of the syndrome, followed by immediate surgical repair, can prevent lethal complications.

Cantrell syndrome with complex cardiac malformations: a case report

“…This recommendation has been adopted by most surgeons who report Cantrell syndrome [7,8]. Onestage definitive repair has been attempted with success [9,10] but has also resulted in rapid death [11]. Unsuccessful 1-stage repair is likely underreported.…”

Prenatal Diagnosis of Cantrell’s Pentalogy Associated with Agenesis of Left Limb in a Twin Pregnancy

“…Sternal malformations that can be seen in PC include bifid sternum, absent xiphoid process, short sternum, and defective formation of lower third [9]. Differential diagnosis of midline defects includes PC, LBWC, body stalk anomaly, and amniotic band syndrome.…”

Class I pentalogy of Cantrell: a rare case report

“…The most common abdominal wall defect is omphalocele (62%) [7]. Epigastric or umbilical hernia, and diastasis recti might also be seen [8]. Ninety-one percent of patients have ventral diaphragmatic hernia.…”

“…Mendaluk et al [10] have reported discharging a patient on the 35 th day after operating the omphalocele and thoracoabdominal ectopia cordis on the 2 nd day of life. As patients with intracardiac diverticulum are at risk of complications such as rupture, tachyarrhythmia and thrombosis, early excision is recommended [8]. Our patient was operated on the 8 th day of life; diverticulum resection, diaphragm, and omphalocele sac reparations were done during the same operation by two different teams.…”