坎特雷尔五联症。1例死产病例报告

Maribel Palencia Palacios, Blanca Viviana Fajardo Idrobo, López Mosquera López Mosquera, Jaime Antonio Álvarez Soler
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摘要

简介:Cantrell五联症是一种罕见的先天性疾病,以中线出生异常为特征。其胚胎起源与腹壁异常有关,腹壁异常阻止胸腔闭合。其病因尚不清楚,但它与侧板中胚层向中线迁移失败有关。病例描述:妊娠25.2周的死产,诊断为坎特雷尔五联症。母亲为14岁青少年,无相关疾病史。在妊娠19周进行了产科超声检查,发现提示Cantrell五联症。母亲被告知胎儿的潜在风险和未来的并发症,但她选择继续怀孕。在怀孕25.2周时,母亲因下腹疼痛并伴有中度阴道出血和胎儿不活动而去了急诊室。一旦经超声确认胎儿死亡,就引产,导致无脑畸形、胸发育不全、胃裂裂和肝外翻的死产。结论:充分的产前护理,包括严格的超声随访,对发现胎儿未来的并发症至关重要;就可能与生命不相容的畸形提供建议,例如坎特雷尔五联症;确定最好的治疗方法。
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Pentalogy of Cantrell. A stillbirth case report
Introduction: Pentalogy of Cantrell is a rare congenital disorder characterized by midline birth anomalies. Its embryological origins are related to anomalies of the abdominal wall that prevent the closure of the thorax. Its etiology is not yet clear, but it has been associated with a failure of migration of the lateral plate mesoderm to the midline. Case description: A stillbirth at 25.2 weeks of gestation diagnosed with pentalogy of Cantrell. The mother was a 14-year-old teenager with no relevant history of disease. An obstetric ultrasound was performed at 19 weeks of gestation and revealed findings suggestive of pentalogy of Cantrell. The mother was informed of the potential risks and future complications for the fetus, yet she chose to continue with the pregnancy. At 25.2 weeks of gestation, the mother went to the emergency department due to pain in the hypogastrium accompanied by moderate vaginal bleeding and absence of fetal activity. Once fetal death was confirmed by ultrasound, labor was induced, resulting in stillbirth with anencephaly, thoracic hypoplasia, gastroschisis, and eventration of the liver. Conclusions: Adequate antenatal care, including strict ultrasound follow-up, is essential to detect future complications in the fetus; to provide advice on possible malformations incompatible with life, such as pentalogy of Cantrell; and to determine the best therapeutic approach.
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