背景:体壁异常包括广泛的畸形。肢体壁复合体(LBWC)代表了该组中最严重的表现,几乎所有病例都有危及生命的畸形,包括颅面,体壁缺陷,和肢体异常.关于其病因和折叠和胃泌素缺陷尚未达成共识。此外,受损的血管生成已被认为是一个致病过程。
方法:我们介绍一个男性死产的案例,15岁第一次怀孕的产品,健康的母亲由于胎膜早破,他在妊娠31周时分娩。他表现出多种畸形,包括广泛的体壁缺损,多器官疝和右下肢小脑。
结论:LBWC代表一种严重且总是致命的病理。没有描述的危险因素,然而,这个案例出现在一个十几岁的母亲身上,其他体壁异常的风险因素。它的诊断使我们能够区分需要产前或产后专门治疗的其他病理。
BACKGROUND: Body wall anomalies comprise a wide range of malformations. Limb-Body wall complex (LBWC) represents the most severe presentation of this group, with life threatening malformations in practically all the cases, including craniofacial, body wall defects, and limb anomalies. There is no consensus about its etiology and folding and
gastrulation defects have been involved. Also, impaired angiogenesis has been proposed as a causative process.
METHODS: We present the case of a masculine stillborn, product of the first pregnancy in a 15-year-old, apparently healthy mother. He was delivered at 31 weeks of gestation due to an early rupture of membranes. He presented with multiple malformations including a wide body wall defect with multiple organ herniation and meromelia of the lower right limb.
CONCLUSIONS: LBWC represents a severe and invariably fatal pathology. There are no described risk factors, nevertheless, this case presented in a teenage mother, a well-described risk factor for other body wall anomalies. Its diagnosis allows us to discriminate between other pathologies that require prenatal or postnatal specialized treatment.