Epigastric heteropagus twins with omphalocele: a case report and literature review
Source: PubMed Central Open Access, NCBI / U.S. National Library of Medicine
Introduction: Epigastric heteropagus (EH) is a rare congenital anomaly, and its management is further complicated by the presence of an omphalocele. We present a case of EH attached to a host with a large omphalocele, along with a review of previously reported cases. We believe it will contribute to increased insight and enable meaningful comparisons on the topic. Case presentation: A 15-hour-old, 3.4-kg male neonate was born at 38 weeks of gestation to a 26-year-old mother. At presentation, he was stable, with a partially formed parasitic twin attached to the epigastrium and an anterior abdominal wall defect measuring approximately 5 cm in diameter. Laboratory investigations were unremarkable. The infant underwent surgery on the fifth postnatal day. Intraoperatively, no viscera were identified within the parasite except for a 30-cm blind-ending intestinal segment communicating with the omphalocele sac. Two large feeding vessels, arising from the liver and the internal mammary artery, were identified. The autosite’s abdominal viscera appeared normal. The omphalocele sac was completely excised, and tension-free primary fascial closure was performed for both the omphalocele and the parasite excision site. The infant tolerated the procedure well and remained stable throughout. Clinical discussion: Conjoined twins are broadly categorized into symmetrical and asymmetrical forms, with the latter referred to as parasitic or heteropagus twins. Heteropagus twinning is characterized by
Abstract
Introduction: Epigastric heteropagus (EH) is a rare congenital anomaly, and its management is further complicated by the presence of an omphalocele. We present a case of EH attached to a host with a large omphalocele, along with a review of previously reported cases. We believe it will contribute to increased insight and enable meaningful comparisons on the topic. Case presentation: A 15-hour-old, 3.4-kg male neonate was born at 38 weeks of gestation to a 26-year-old mother. At presentation, he was stable, with a partially formed parasitic twin attached to the epigastrium and an anterior abdominal wall defect measuring approximately 5 cm in diameter. Laboratory investigations were unremarkable. The infant underwent surgery on the fifth postnatal day. Intraoperatively, no viscera were identified within the parasite except for a 30-cm blind-ending intestinal segment communicating with the omphalocele sac. Two large feeding vessels, arising from the liver and the internal mammary artery, were identified. The autosite’s abdominal viscera appeared normal. The omphalocele sac was completely excised, and tension-free primary fascial closure was performed for both the omphalocele and the parasite excision site. The infant tolerated the procedure well and remained stable throughout. Clinical discussion: Conjoined twins are broadly categorized into symmetrical and asymmetrical forms, with the latter referred to as parasitic or heteropagus twins. Heteropagus twinning is characterized by an incompletely developed parasitic fetus attached to a relatively normal autosite, upon which it is entirely dependent. Visceral sharing between the autosite and parasite is uncommon, and, as a result, surgical separation is typically straightforward, except in the rare instances where complex anatomical fusion is present. Conclusion: Epigastric heteropagus is a rare parasitic twinning condition with minimal organ sharing, which can usually be safely excised with a good outcome.