Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell
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 abdomi...
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JCDR Research and Publications Pvt. Ltd.
2024-09-01
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| Series: | International Journal of Anatomy Radiology and Surgery |
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| Online Access: | https://www.ijars.net/articles/PDF/3017/71423_CE[Ra1]_F(SHU)_QC(PS_SHU)_PF1(JY_SHU)_redo_PFA_NC(SHU)_PN(SHU).pdf |
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| author | Arumulla Mithilesh Hira Muhammed Yugandhar Samireddypalle Nanditha Jangamreddy |
| author_facet | Arumulla Mithilesh Hira Muhammed Yugandhar Samireddypalle Nanditha Jangamreddy |
| author_sort | Arumulla Mithilesh |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-096599f2ab8a4b45b3d96bb0d88cce2b |
| institution | OA Journals |
| issn | 2277-8543 2455-6874 |
| language | English |
| publishDate | 2024-09-01 |
| publisher | JCDR Research and Publications Pvt. Ltd. |
| record_format | Article |
| series | International Journal of Anatomy Radiology and Surgery |
| spelling | doaj-art-096599f2ab8a4b45b3d96bb0d88cce2b2025-08-20T02:10:53ZengJCDR Research and Publications Pvt. Ltd.International Journal of Anatomy Radiology and Surgery2277-85432455-68742024-09-011305111310.7860/IJARS/2024/71423.3017Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of CantrellArumulla Mithilesh0Hira Muhammed1Yugandhar Samireddypalle2Nanditha Jangamreddy3Assistant Professor, Department of Radiodiagnosis, AIIMS Mangalagiri, Andhra Pradesh, India.Junior Resident, Department of Radiodiagnosis, AIIMS Mangalagiri, Andhra Pradesh, India.Assistant Professor, Department of Radiodiagnosis, AIIMS Mangalagiri, Andhra Pradesh, India.Senior Resident, Department of Obstetrics and Gynaecology, AIIMS Mangalagiri, Andhra Pradesh, India.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.https://www.ijars.net/articles/PDF/3017/71423_CE[Ra1]_F(SHU)_QC(PS_SHU)_PF1(JY_SHU)_redo_PFA_NC(SHU)_PN(SHU).pdfantenatal ultrasonographyanterior abdominal wall defectscongenital anomaliesfoetus |
| spellingShingle | Arumulla Mithilesh Hira Muhammed Yugandhar Samireddypalle Nanditha Jangamreddy Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell International Journal of Anatomy Radiology and Surgery antenatal ultrasonography anterior abdominal wall defects congenital anomalies foetus |
| title | Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell |
| title_full | Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell |
| title_fullStr | Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell |
| title_full_unstemmed | Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell |
| title_short | Ectopia Cordis with Omphalocele in First Trimester Pregnancy: A Case Report of Incomplete Type of Pentalogy of Cantrell |
| title_sort | ectopia cordis with omphalocele in first trimester pregnancy a case report of incomplete type of pentalogy of cantrell |
| topic | antenatal ultrasonography anterior abdominal wall defects congenital anomalies foetus |
| url | https://www.ijars.net/articles/PDF/3017/71423_CE[Ra1]_F(SHU)_QC(PS_SHU)_PF1(JY_SHU)_redo_PFA_NC(SHU)_PN(SHU).pdf |
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