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双侧先天性 Morgagni-Larrey 疝。

Bilateral congenital Morgagni-Larrey's hernia.

机构信息

Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia.

出版信息

World J Pediatr. 2010 Feb;6(1):76-80. doi: 10.1007/s12519-010-0011-8. Epub 2010 Feb 9.

Abstract

BACKGROUND

Congenital Morgagni-Larrey's hernia (CMLH) is rare and known to be associated with a high incidence of bilaterality and associated anomalies. This study aimed to review our patients with bilateral CMLH and evaluate their presentation, associated anomalies, diagnostic difficulties and therapy.

METHODS

From January 1989 to December 2007, we treated 8 children with bilateral CMLH at our hospital. Their medical records were retrospectively reviewed for age, sex, symptoms, associated anomalies, diagnosis, operative findings, treatment and outcome.

RESULTS

Among the 8 children, 4 were male and 4 female, with a mean age of 22.74 months (range: 0.93-108 months). Six of them sustained repeated attacks of pneumonia. In 5 children, bilaterality was diagnosed at surgery. Associated anomalies were observed in all children, including congenital heart disease in 5, Down's syndrome in 4, malrotation in 3, inguinal hernia in 2, umbilical hernia in 1, and pyloric stenosis in 1. All of these children were operated on transabdominally. All of them did well postoperatively and on follow-up ranging from 1.5 years to 5 years (mean: 3 years), one had recurrence of the hernia as well as an incisional hernia.

CONCLUSIONS

The diagnosis of bilateral CMLH may be difficult preoperatively, especially if one of the hernial sacs is empty. CT scan is valuable to diagnose bilateral hernias. To repair these hernias, we advocate a transabdominal approach, which allows easy reduction and inspection of contents, access and repair of bilateral hernias, and correction of associated malrotation if present.

摘要

背景

先天性 Morgagni-Larrey 疝(CMLH)罕见,与双侧疝和相关畸形的高发生率相关。本研究旨在回顾我们双侧 CMLH 患者,并评估其表现、相关畸形、诊断困难和治疗。

方法

1989 年 1 月至 2007 年 12 月,我院治疗 8 例双侧 CMLH 患儿。回顾性分析其年龄、性别、症状、相关畸形、诊断、手术发现、治疗和结果。

结果

8 例患儿中,男 4 例,女 4 例,平均年龄 22.74 个月(0.93-108 个月)。6 例反复发生肺炎。5 例患儿术中诊断为双侧性。所有患儿均有相关畸形,包括先天性心脏病 5 例、唐氏综合征 4 例、旋转不良 3 例、腹股沟疝 2 例、脐疝 1 例、幽门狭窄 1 例。所有患儿均经腹手术治疗。术后及随访 1.5-5 年(平均 3 年),所有患儿均恢复良好,1 例出现疝复发及切口疝。

结论

双侧 CMLH 术前诊断可能较困难,尤其是当一个疝囊为空时。CT 扫描对诊断双侧疝有价值。为修复这些疝,我们主张采用经腹入路,可方便地还纳和检查内容物,处理双侧疝,并纠正相关的旋转不良。

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