Pericardio-Amniotic Shunting for Incomplete Pentalogy of Cantrell

Abstract

A 27-year-old woman, gravida 2, para 0, presented with an incomplete Pentalogy of Cantrell with an omphalocele, diaphragmatic hernia, and a pericardial defect at 32 weeks' gestation. A large pericardial effusion compressed the lungs and had led to a reduced lung growth with an observed-to-expected total lung volume of 28% as measured by MRI. The effusion disappeared completely after the insertion of a pericardio-amniotic shunt at 33 weeks. After birth, the newborn showed no signs of pulmonary hypoplasia and underwent a surgical correction of the defect. Protracted wound healing and a difficult withdrawal from opioids complicated the neonatal period. The child was discharged on postnatal day 105 in good condition. This case demonstrates that in case of Pentalogy of Cantrell with large pericardial effusion, the perinatal outcome might be improved by pericardio-amniotic shunting.

Pentalogy of Cantrell

“…The in utero pericardio-amniotic shunting described by Engels et al for a patient with a large pericardial effusion was the first procedure of its kind for a patient with this disease. The therapeutic draining of the effusion allowed for a suspected improvement in lung growth and development prior to delivery (43). However, aggressive prenatal interventions must be balanced with associated risks as well as informed post-natal expectations for prognosis.…”

Omphalocele and Cardiac Abnormalities—The Importance of the Association

“…In the presence of the association of several congenital anomalies, the mother will be directed to a tertiary center specialized in maternal–fetal medicine, both for counseling, follow-up, pre-, and postnatal treatment. From the point of view of prenatal treatment, few interventions can be performed safely, although Engels reported a case of PC in which he performed a pericardio-amniotic shunt for large pericardial effusion, with good results [ 73 ]. The survival rate in patients with omphalocele and cardiac anomalies detected antenatally is 23% compared to an 80% rate in live birth cases detected postnatally [ 14 ].…”