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三绒毛膜三羊膜囊妊娠减胎术后先天性皮肤发育不全的罕见表现。

A rare presentation of aplasia cutis congenita after feto-reduction in a trichorionic-triamniotic pregnancy.

作者信息

Vettori D J, Jairath P

机构信息

Pinnacle Health Medical Group, Maternal Fetal Medicine, Harrisburg, PA, USA.

Department of Pediatrics (Neonatal Division), Pinnacle Health/Pediatrix Medical Group, Harrisburg, PA, USA.

出版信息

J Neonatal Perinatal Med. 2015;8(3):275-81. doi: 10.3233/NPM-15915003.

Abstract

Aplasia cutis congenita (ACC) is rare skin disorder of newborns that has been linked to both assisted reproductive technology (ART) and feto-reduction procedures. ACC is characterized by well-demarcated lesions that are devoid of all skin layers. Group-V ACC presents with a distinctive and symmetrical distribution pattern. It is thought to result from an insult to the fetus after concomitant twin demise and is almost exclusively reported in monochorionic gestations.A 41-year-old female with an in vitro fertilization (IVF) assisted tri-chorionic gestation subsequently underwent selective feto-reduction of Fetus C. The patient delivered two pre-term neonates secondary to pre-eclampsia. The initial exam of Twin B showed extensive, well-demarcated, symmetrical areas devoid of any skin over the anterior and lateral trunk, extending up the lateral thoracic walls. Chest and abdominal viscera were visible through a thin fibrous membrane. The skin defects were managed conservatively with twice-daily dressings of Aquaphor, and Vaseline gauze. The areas of aplasia slowly contracted, though residual scarring was noted. After four weeks in the NICU, most of the areas were healed.ACC in multi-fetal pregnancies is a rare, but well-described complication. This is, to our knowledge, the first reported case in a tri-chorionic IVF gestation after feto-reduction. With increased incidence of ART-associated pregnancies and the use of feto-reduction for higher order gestations, this may become more common. Neonates often require specialized intensive care. Conservative management usually will suffice, although surgical grafting may be required. Physicians should be aware of this condition and counsel their feto-reduction patients of the risk.

摘要

先天性皮肤发育不全(ACC)是一种罕见的新生儿皮肤疾病,与辅助生殖技术(ART)和减胎术均有关联。ACC的特征是界限清晰的皮损,所有皮肤层均缺失。V型ACC呈现出独特的对称分布模式。它被认为是由于双胎之一死亡后对胎儿造成损伤所致,几乎仅见于单绒毛膜妊娠。一名41岁接受体外受精(IVF)辅助的三绒毛膜妊娠女性随后对胎儿C进行了选择性减胎。该患者因先兆子痫分娩了两名早产儿。对双胞胎B的初始检查显示,其前侧和外侧躯干有广泛、界限清晰、对称的无皮肤区域,向上延伸至侧胸壁。透过一层薄纤维膜可见胸部和腹部脏器。皮肤缺损采用每日两次涂抹水合软膏和凡士林纱布进行保守处理。发育不全区域逐渐缩小,不过仍有残留瘢痕。在新生儿重症监护病房(NICU)住了四周后,大部分区域愈合。多胎妊娠中的ACC是一种罕见但已有充分描述的并发症。据我们所知,这是首例三绒毛膜IVF妊娠减胎后报告的病例。随着ART相关妊娠发生率的增加以及高阶妊娠减胎术的应用,这种情况可能会变得更加常见。新生儿通常需要专门的重症监护。保守治疗通常就足够了,不过可能需要手术植皮。医生应了解这种情况,并告知减胎患者相关风险。

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