Ridley Ashley, Chaussy Yann, Mottet Nicolas, Auber Frédéric
Réanimation pédiatrique, Centre Hospitalier Universitaire de Besancon, Besancon, France.
chirurgie pédiatrique, Centre Hospitalier Regional Universitaire de Besançon, Besançon, France.
BMJ Case Rep. 2018 Aug 4;2018:bcr-2018-225176. doi: 10.1136/bcr-2018-225176.
We report on the case of two digestive malformations in dizygotic/dichorionic/diamniotic twins born at 31 weeks of gestation. The mother (gravida 1 para 0) was treated by hydroxychloroquine for systemic lupus erythematosus during pregnancy. Twin A presented an arch-like dilatation on antenatal ultrasounds, characteristic of segmental volvulus. After birth, twin B presented repeated vomiting on feeding, leading us to diagnose ileal atresia despite normal antenatal ultrasounds. Both twins underwent surgery and the postoperative period was uneventful. After 1 year of follow-up, the twins are in excellent health without digestive sequelae. Genetic testing for cystic fibrosis was negative. The placenta showed diffuse signs of hypoxia and ischaemia, indicating that the root cause was vascular. The pathophysiology of intestinal atresia is hypothesised to derive from a vascular incident during fetal development. We are therefore led to believe that an intrauterine vascular event is the most likely cause of the dual malformation.
我们报告了一例孕31周出生的双卵双绒毛膜双羊膜囊双胞胎出现两种消化器官畸形的病例。母亲(孕1产0)孕期因系统性红斑狼疮接受羟氯喹治疗。双胞胎A产前超声显示呈弓状扩张,为节段性肠扭转特征。出生后,双胞胎B喂奶时反复呕吐,尽管产前超声正常,我们仍诊断为回肠闭锁。两名双胞胎均接受了手术,术后恢复顺利。随访1年后,双胞胎健康状况良好,无消化后遗症。囊性纤维化基因检测为阴性。胎盘显示弥漫性缺氧和缺血迹象,表明根本原因是血管性的。肠闭锁的病理生理学据推测源于胎儿发育期间的血管事件。因此,我们认为子宫内血管事件最有可能是这种双重畸形的原因。