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Morgagni疝相关的畸形。

Associated malformations in Morgagni hernia.

作者信息

Cigdem Murat Kemal, Onen Abdurrahman, Okur Hanifi, Otcu Selcuk

机构信息

Department of Pediatric Surgery, Dicle University Medical Faculty, 21280 Diyarbakir, Turkey.

出版信息

Pediatr Surg Int. 2007 Nov;23(11):1101-3. doi: 10.1007/s00383-007-2011-x. Epub 2007 Sep 8.

Abstract

Morgagni hernia (MH) is a congenital herniation of the abdominal organs through the subcostosternal portion of the diaphragm. It is a rare type of diaphragmatic hernia that has been associated with other congenital anomalies. The purpose of this clinical review was to determine the incidence of associated anomalies in patients with MH at a major pediatric referral center. The medical records of all patients with the diagnosis of MH were reviewed retrospectively between 1983 and 2006. The age, sex, side of herniation, presenting symptoms and signs, associated anomalies, radiologic features, intraoperative findings, postoperative course, complications, and mortality were recorded. There were 11 males and 5 females. The average age of the patients was 34.5 months (range 2 months-14 years). Twelve patients (75%) had at least one associated congenital malformation and eight (66.6%) of them had multiple anomalies. Six (37.5%) patients had a chromosomal anomaly, Down's syndrome in five and Turner syndrome in one. MH has a high incidence of associated congenital malformations.

摘要

莫尔加尼疝(MH)是腹腔脏器经膈肌肋弓下部分的先天性疝出。它是一种罕见的膈疝类型,常与其他先天性畸形相关。本临床综述的目的是确定在一家大型儿科转诊中心,MH患者中相关畸形的发生率。回顾性分析了1983年至2006年间所有诊断为MH的患者的病历。记录了患者的年龄、性别、疝出部位、临床表现和体征、相关畸形、放射学特征、术中发现、术后病程、并发症及死亡率。其中男性11例,女性5例。患者的平均年龄为34.5个月(范围2个月至14岁)。12例患者(75%)至少有1种相关先天性畸形,其中8例(66.6%)有多种畸形。6例(37.5%)患者有染色体异常,5例为唐氏综合征,1例为特纳综合征。MH合并先天性畸形的发生率很高。

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