Prenatal diagnosis and management challenges of Pentalogy of Cantrell at term in a resource-constrained setting: A rare case report.

INTRODUCTION AND

IMPORTANCE

Pentalogy of Cantrell (POC) is a rare and often fatal congenital malformation characterized by a midline developmental defect involving five anatomical structures: the lower sternum, anterior diaphragm, diaphragmatic pericardium, abdominal wall, and heart. This condition presents significant diagnostic and therapeutic challenges, particularly in resource-limited settings where advanced imaging and surgical interventions are limited. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with POC.

CASE PRESENTATION: Here we present a case of a 32-year-old female (Gravidity 2 Parity 1 Living 1) presented to our specialty hospital in Northern Tanzania at 31 weeks and 3 days of gestational age for regular antenatal visit clinic with a complain of mild lower abdominal pain for 1 week. Obstetric ultrasound revealed a single fetus with an estimated gestational age of 31 weeks and 3 days and a 3.3 cm anterior chest wall defect with partial cardiac herniation, along with herniation of the liver, bowel, kidney, and urinary bladder with thoracolumbar kyphotic deformity was also seen. The radiological findings were consistent with limb body wall complex syndrome and POC. A multidisciplinary board team of obstetrician, pediatricians, surgeons and radiologist discussed the patient and planned for medical termination of the pregnancy. The patient was discharged home after receiving a psychological care. Psychological support was provided, and the patient remained emotionally stable during the follow-up.

CLINICAL DISCUSSION: This case highlights the antenatal diagnostic and postnatal management challenges of POC in resource-constrained settings.

CONCLUSION

Patients diagnosed with this condition should receive thorough antenatal counseling regarding the associated risks of morbidity and mortality. Currently, there are no established guidelines or studies defining the optimal mode of delivery for these cases. In case of severe malformations and or confirmed chromosomal abnormalities, and where legally permissible, pregnancy termination may be considered.