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두피 피부 무형성증을 동반한 양막대증후군 1례

A Case of Amniotic Band Syndrome Associated with Aplasia Cutis of the Scalp

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초록

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

Abstract

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

참고문헌 (14)

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  2. Cho JH, "A case of amniotic syndrome as a cause of anencephaly", Korean J Obstet Gynecol, 41, pp.1993~1997 (1998)
  3. Park YK, "A case of congenital craniofacial anomaly due to amniotic band syndrome", Korean J Obstet Gynecol, 41, pp.3081~3084 (1998)
  4. 전규정, "두개안면 기형과 하지절단을 동반한 양막대증후군 1예", Korean Journal of Obstetrics and Gynecology, 45(4), pp.27~27 (2002), 대한산부인과학회
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  7. 박소양, "사지 기형을 동반한 양막대증후군 1예", Korean Journal of Obstetrics and Gynecology, 45(2), pp.25~25 (2002), 대한산부인과학회
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  9. Lubinsky M, "Familial amniotic bands", Am J Med Genet, 14, pp.81~87 (1983)
  10. Torpin R, "Amniochorionic mesoblastic fibrous strings and amnionic bands: associated constricting fetal malformations or fetal death", Am J Obstet Gynecol, 91, pp.65~75 (1965)
  11. Bronstein M, "Do amniotic bands amputate fetal organs?", Ultrasound Obstet Gynecol, 10, pp.309~311 (1997)
  12. Van Allen MI, "Limb body wall complex: I. Pathogenesis", Am J Med Genet, 28, pp.529~548 (1987)
  13. Baker CJ, "Congenital ring constrictions and intrauterine amputations", Am J Dis Child, 121, pp.393~400 (1971)
  14. Nagore E, "Radius hypoplasia, radial palsy, and aplasia cutis due to amniotic band syndrome", Pediatr Dermatol, 16, pp.217~219 (1999)

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