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羊膜帶畸形:雙胞胎中之一病例報告

Amniotic Band Disruption Complex: Report of One Case in Twins

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摘要


羊膜帶畸形(Amniotic band disruption complex)是一種以骨骼及肌肉異常為主的疾病,主要特徵是四肢、顱部、腹部的畸形。本病例為雙胞胎中之一男嬰,自然生產,懷孕34週,出生體重2350公克,於身體檢查時發現有左側兔唇及左側完全性顎裂,左翼塌陷,左手食指及中指第三指節節有環狀壓痕,無名指第三指節末端缺損有環狀壓痕,無名指只有一節且末端有環狀壓痕及壞死;左脚五隻趾頭的第三指皆因缺損而變的短小;右脚缺小趾,其他四趾第三指節因缺損而變得短小,經診斷為少見的雙胞胎羊膜帶畸形。雙胞胎另一位亦為男嬰,但外觀則完全正常,在此提出報告。

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並列摘要


Amniotic band disruption complex is a congenital disorder with the principal features vary from simple digital band constrictions to major craniofacial and visceral defects. Early amniotic rupture results in multiply affected infants who are frequently aborted or stillborn, whereas later rupture results primarily in limb involvement. Here we report a case of amniotic band disruption complex, a male infant (twin A) weighing 2350 gm with Apgar scores of 9 and 10 at one and five minutes. The second, third and fourth fingers on the left hand appeared to have ring constictions, the distal part of the fourth finger had been amputated distally, There were amputations and ring constrictions of the distal parts of the second, third and fifth digits on the right hand, the fourth finger had only proximal third. All of the toes on the left foot had been amputated distally. The fifth toe on the right foot were absent, and the others had been partly amputated. Left cleft lip and palate were present with sunken left nasal flare. This baby underwent the cranial ultrasound examination, which demostrated on structural anomalies, and both of the twins had the karyotype of 46XY. No other abnormalities was noted in the infant. The 890 gm placenta was dilivered spontaneously. Two umbilical cords were present, each with normal vessels. The examination of the placenta revealed it to be dichorionic and diamniotic, and numerous fibrous strings extending form the fetal surface.

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