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Sirenomelia


"Sirenomelia is a rare congenital anomaly characterized by abnormal development of the caudal region of the body with variable degree of fusion of lower extremities. It derives its name by its resemblance to the Mermaid ( Siren ) of Greek mythology. About 300 cases have been reported in the world so far out of which 9 have been reported in India. Sirenomelia is also known as Sirenomelia sequence, Symmelia, Symposia, Uromelia and Monopodia. The exact etiopathogenesis is uncertain. The proposed theories are Vascular steal or Vitelline artery steal theory by Stevenson which is the most accepted. Others include teratogenic effects of lead, cadmium and vit.A, primitive streak defect, axial mesodermal dysplasia sequence.Maternal diabetes mellitus and genetic predisposition is also implicated. Vitelline artery steal theory states that an aberrant vessel(derived from the vitelline artery) arises from high abdominal aorta in the affected fetus and functions as the vessel returning blood through the umbilical cord to placenta. This vessels steals blood from structures distal to its origin."
Read the full case report here (Full Text Available Free)
Sirenomelia Reviewed by Sumer Sethi on Friday, October 06, 2006 Rating: 5

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