Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell

Autor: Arumulla Mithilesh, Hira Muhammed, Yugandhar Samireddypalle, Nanditha Jangamreddy
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: International Journal of Anatomy Radiology and Surgery, Vol 13, Iss 05, Pp 11-13 (2024)
Druh dokumentu: article
ISSN: 2277-8543
2455-6874
DOI: 10.7860/IJARS/2024/71423.3017
Popis: Ectopia Cordis (EC) is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. It results from the failure of migration of the lateral mesoderm into the midline. The four main ectopic positions are cervical, thoracic, thoracoabdominal and abdominal. EC can manifest as an isolated deformity or as part of a broader group of ventral body wall defects affecting the abdomen, thorax, or both. Pentalogy of Cantrell is a well known association that comprises EC, omphalocele (typically supraumbilical), congenital diaphragmatic hernia, sternal cleft and congenital heart disease. A 27-year-old primigravida came for a routine antenatal ultrasound at 13 weeks of gestation. There was no family history of congenital anomalies, genetic abnormalities, or exposure to teratogenic agents. The ultrasound showed a single foetus corresponding to 13 weeks of gestation with an anterior thoracic defect and an extrathoracic heart, along with partial herniation of the liver near the midline in the epigastric region. These findings were confirmed by foetal Magnetic Resonance Imaging (MRI). An unfavourable prognosis for the foetus was explained to the parents, and medical termination of pregnancy was performed. Therefore, prenatal ultrasonographic diagnosis of EC should be followed by a thorough search for associated defects, as the prognosis may vary.
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