Diagnosis of Pentalogy of Cantrell in the Fetus Using Magnetic Resonance Imaging and Ultrasound

Abstract

We report two cases of pentalogy of Cantrell diagnosed in utero using a combination of fetal echocardiography and magnetic resonance imaging. The cardiac component consisted of tetralogy of Fallot in the first fetus and ventricular septal defect in the second fetus. Whereas fetal echocardiography allowed accurate delineation of the cardiac anatomy, prenatal magnetic resonance imaging allowed clearer delineation of the extent of the thoracic and abdominal wall defects. Fetal magnetic resonance imaging in conjunction with prenatal echocardiography allows optimal assessment of the fetus with ectopia cordis, which has significant implications from the standpoint of preoperative planning and providing prognostic information. This report represents the first description of applying magnetic resonance imaging in combination with echocardiography toward a better understanding of this clinical entity in the fetus.

Diagnosis of Pentalogy of Cantrell Using 2- and 3-Dimensional Sonography

“…However, in patients who have fetuses with smaller or transient defects and in those who decide to continue the pregnancy, MRI and fetal echocardiography can provide important prognostic information as well as more detailed measurements and structural cardiac information for the pediatric and surgical teams who may be caring for the fetus after delivery. 4 In our case, the fetus had major intracardiac structural abnormalities noted on autopsy. However, the sonographic diagnosis of these anomalies was considerably impaired by fetal motion.…”

“…It has been suggested that magnetic resonance imaging (MRI) and prenatal fetal echocardiography provide optimal assessment of fetuses with this syndrome. 4 However, in cases with large defects similar to the one we have described, we believe that a combination of 2D and 3D sonography is sufficient. A large defect was clearly seen on sonography in this case, and given the poor prognosis in cases with such a large defect, we do not think that MRI or fetal echocardiography would have added any additional information in counseling the patient.…”

Case Report of Pentalogy of Cantrell

“…If pericardial effusion can be seen, associated anterior diaphragmatic hernia and diaphragmatic pericardial defects may be suspected and specific and detailed search for the features of the pentalogy of Cantrell should be done. 7 Use of prenatal magnetic resonance imaging (MRI) may enhance the visualization of the fetal anomalies. 8 Although most cases of the ectopia cordis appear as isolated, sporadic defects, other associated anomalies, including chromosomal abnormalities (trisomy 18) are reported in literature.…”

Antenatal Diagnosis of Omphalocele

“…The earliest that an omphalocele can be detected is at 12 weeks of menstrual age. Magnetic resonance imaging (MRI) facilitates perinatal management and parental counseling [6]. An omphalocele is diagnosed on USG when a fetal anterior midline mass consisting of abdominal contents that have herniated through a midline central defect at the base of umbilical insertion is demonstrated.…”