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胎儿心包内 Morgagni 膈疝伴大量心包积液的诊断和处理。

Diagnosis and management of fetal intrapericardial Morgagni diaphragmatic hernia with massive pericardial effussion.

机构信息

Unidad De Gestión Clinica De Genética, Reproducción Y Medicina Fetal, Hospitales Universitarios Virgen Del Rocío, 41013 Sevilla, Spain.

出版信息

J Pediatr Surg. 2010 Feb;45(2):424-6. doi: 10.1016/j.jpedsurg.2009.11.009.

Abstract

Herniation of the liver into the fluid-filled pericardial sac resulting in a thoracic mass is a particularly rare form of Morgagni hernia (congenital diaphragmatic hernia of Morgagni). We report an early antenatal diagnosis of congenital diaphragmatic hernia of Morgagni with pericardium effussion at 21 weeks' gestation. Two pericardiocentesis were performed at 21 and 22 4/7 weeks' gestation because of recurrence of pericardial effussion. Regular ultrasound assessments showed progressive herniation of the liver to practically fill the right hemithorax. An ex utero intrapartum treatment procedure was performed at 37 weeks' gestation to rescue maximum intrathoracic space for ventilation of the remaining functional lung tissue and to establish an airway for postnatal support. After birth, the patient successfully underwent early correction of the hernia. Postoperative course was uneventful, and the newborn girl was discharged 18 days later without complications and is currently doing well.

摘要

肝脏疝入充满液体的心包囊,导致胸部肿块,是 Morgagni 疝(先天性膈疝 Morgagni)的一种特别罕见的形式。我们报告了一例 21 孕周时经产前诊断的 Morgagni 先天性膈疝合并心包积液。由于心包积液复发,在 21 周和 22 周 4/7 时进行了两次心包穿刺术。定期超声评估显示肝脏逐渐疝入,几乎填满了右胸腔。在 37 孕周时进行了子宫外产时治疗程序,以最大限度地为剩余功能肺组织的通气保留胸腔内空间,并建立气道以备产后支持。出生后,患儿成功地进行了早期疝修补术。术后过程顺利,新生儿女孩在没有并发症的情况下于 18 天后出院,目前情况良好。

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