METHODS: Cochrane, MEDLINE and EMBASE were searched between 1 January 1992 and 31 December 2022 using terms and variations: omphalocele, exomphalos, giant, closure and outcome. Papers were selected using Preferred Reporting Items for Systematic review and Meta-Analyses 2020 criteria. Data collected included demographics, timing and technique of surgical repair, morbidity and mortality.
RESULTS: We identified 342 papers; 34 met inclusion criteria with a total 356 neonates. Initial non-operative management was described in 26 papers (14 dressings, eight silo, four serial sac-ligation). Operative techniques by paper were as follows: Early closure: nine primary suture closure without patch, two primary closure with patch and four mixed methods. Delayed closure: five simple, four-component separation technique, four tissue expanders, one Botox/pneumoperitoneum and two with patch. Median number of procedures was two (1-6) in the early group versus three (1-4) in the delayed. The most favourable was early primary closure with biological patch. The most unfavourable was delayed closure with patch. Cumulative reported mortality remained high, mostly due to non-surgical causes.
CONCLUSIONS: Definitions of giant omphalocele in the literature were heterogeneous with a variety of management approaches described.
方法:Cochrane,在1992年1月1日至2022年12月31日之间使用术语和变体搜索了MEDLINE和EMBASE:exomphalos,巨人,关闭和结果。论文是使用2020年系统评价和荟萃分析标准的首选报告项目选择的。收集的数据包括人口统计,手术修复的时机和技术,发病率和死亡率。
结果:我们确定了342篇论文;34篇符合纳入标准,共有356例新生儿。26篇论文描述了初始非手术治疗(14篇敷料,八个筒仓,四个连续囊结扎)。论文的手术技术如下:早期闭合:无贴片的九种主要缝合闭合,两种主要的补片封闭和四种混合方法。延迟关闭:五个简单,四组分分离技术,四个组织扩张器,一个肉毒杆菌/气腹和两个带贴片。手术的中位数在早期组为2(1-6),在延迟组为3(1-4)。最有利的是早期用生物贴片进行初级闭合。最不利的是用补丁延迟关闭。累计报告死亡率仍然很高,主要是由于非手术原因。
结论:文献中巨大脐膨出的定义与所描述的各种管理方法不同。