Prenatal and Postnatal Management of a Patient with Pentalogy of Cantrell and Left Ventricular Aneurysm

Abstract

Introduction: Omphalocele is often associated with cardiac diverticulum in the pentalogy of Cantrell, however a prenatal diagnosis of omphalocele has not been described with a broad-based, thin-walled aneurysm of the ventricular wall in a surviving patient. Case Report: A case of omphalocele with Morgagni hernia and left ventricular aneurysm diagnosed at 21 weeks, 6 days’ gestational age is reported. Initial ultrasound examination demonstrated the cardiac defect and subsequent ultrasounds revealed the abdominal wall defect. Prenatal monitoring included serial ultrasound and echocardiography. A viable fetus was delivered via cesarean section at 37 weeks’ gestational age. The omphalocele and Morgagni hernia were repaired on the infant’s 4th day of life. The cardiac defect continues to be monitored with echocardiography at 15 months of age. Discussion: The case is discussed along with a short review of the literature of midline thoracoabdominal defects and left ventricular apical aneurysms.

Pentalogy of Cantrell: case report

“…Even at birth, the full extent of the syndrome may not be apparent, as the sternal defect may be minor and therefore without true ectopia cordis. 3 In our case ectopia cordis was present and sternum was absent. In 1972, Toyama reported a survival rate of 20%.…”

“…The abdominal defect can range from a wide rectus muscle diastasis to a large omphalocoele. 3 The most common intracardiac defects are atrial septal defect, ventricular septal defect, and tetralogy of Fallot. The syndrome has been diagnosed prenatally, but as the defects range from subtle to severe; the ability to make the ultrasound diagnosis varies.…”

Pentalogy of Cantrell with Encephalocele and Cystic Hygroma

“…Çoğu hastada sporadik olduğu düşünülen Cantrell pentalojisinin, orta hat defektlerine neden olan bazı genlerle de ortaya çıkabileceği düşünülmektedir. 8 Yapılan araştırmalarda, Xq25-q26.1 bölgesindeki TAS gen mutasyonunun, ön abdomino-torakal füz-yon defektine ve Cantrell sendromuyla ilişkili bulgulara yol açtığı gözlenmiştir. 7,6 Buna rağmen Cantrell pentalojisi birçok anomalilerle birlikte olabileceğinden; majör kromozom anomalileri, mekanik teratojenler, majör gen mutasyonları (trizomi 13,18 vb.)…”

Incomplete pentalogy of Cantrell: a

“…If these two components had been present, a more complex surgical strategy would have been necessary [4]. In conclusion, we were lucky in the regard that the cardiac anomaly was easy to repair.…”