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目的:回顾分析含有肝脏组织的巨型脐膨出(GO)的临床特征，探讨最佳治疗方式，改善预后，提高GO患儿救治率。方法:收集2009年1月至2019年5月在武汉儿童医院新生儿外科住院的GO患儿，治疗方法包括：1.使用类似Silo袋的方法，分期手术；2.一期直接或联合补片修补；3.保守治疗2～8周后延期一期手术。收集的数据包括患儿孕周、性别、出生体质量、脐膨出直径、膨出物内容、合并畸形、治疗方法、呼吸机使用时间及临床结局。根据脐膨出的内容物，本组患儿分为肝脏膨出组和肝脏合并其他组织组，总结并比较不同组别临床基本资料及临床结局。结果:16例患儿中，男9例，女7例。就诊时日龄1～2 d[(1.25±0.45) d]，体质量(2.48±0.37) kg，孕周(36.23±1.17)周，脐膨出大小为5～15 cm，直径为(9.88±3.30) cm。肝脏膨出组7例(43.75%)，其中一期修补6例，保守治疗延期手术1例。肝脏合并其他组织组9例，膨出物除肝脏外，还包括小肠、结肠、脾脏等组织，一期修补、保守治疗延期手术、使用Silo袋者各3例。肝脏膨出组及肝脏合并其他组织组中使用呼吸机的例数分别为3例(42.8%)及8例(88.89%)，与肝脏膨出组比较，肝脏合并其他组织组使用呼吸机的n OR值为5.143(95%n CI：0.727～36.368)，且应用呼吸机的时间更长[(106.25±69.36) h比(30.67±19.0) h，n P＝0.021]。肝脏膨出组无死亡病例，肝脏并其他组织组死亡3例。术后患儿随访3个月～4年，5例出现腹壁疝：肝脏膨出组1例(14.3%)；肝脏合并其他组织组4例，去除死亡的3例后，其占比66.7%(4/6例)。n 结论:含肝脏膨出的脐膨出患儿，需强调个体化治疗。孤立的肝脏膨出者预后好于肝脏并其他脏器膨出者。孤立性肝脏膨出，建议一期手术；并其他脏器膨出的肝脏膨出，在囊膜完整的情况下，建议保守治疗后延期手术。肝膨出时需要考虑合并副肝的可能性。“,”Objective:To investigate the proper choice and improve the survival rate in neonates suffering from giant omphalocele (GO)with liver protrusion by analyzing the clinical characteristics, different treatments and outcomes.Methods:Neonates with GO admitted in Department of Neonatal Surgery in Wuhan Children′s Hospital were retrospected from January 2009 to May 2019.The treatment methods include: (1) repaired with similar silo-bag by staged operation; (2) one-stage repair with patch or not; (3) delayed repair by conservative treatment for some time (from 2 to 8 weeks) firstly.Data included gestational age, gender, birth weight, average diameter of omphalocele, contents of omphalocele, associated malformation, treatment method, duration of ventilator use and clinical outcome.These neonates were divided into 2 groups according to the contents of protrusion: group with liver protrusion and group with liver and other organs protrusion.Clinical data were summarized and compared between different groups.Results:Sixteen cases were collected, including 9 males and 7 females.The average age was (1.25±0.45) d (1-2 days), the average birth weight was (2.48±0.37) kg, and pregnant week was (36.23±1.17) weeks.The average diameter of the omphalocele was (9.88±3.30) cm, ranging from 5 cm to 15 cm.Seven cases were belonged to the group with liver protrusion, cases undergone one-stage repair, delayed repair were 6 cases and 1 case, respectively.There were 9 cases in group with liver and other organs protrusion, and the protruded organs included liver, intestine, colon or spleen.Among them, cases needed one-stage repair, delayed repair and silo-bag repair were 3 cases, 3 cases, 3 cases, respectively.Cases needed respiratory machine were 3 cases (42.8%) and 8 cases (88.89%) in group with liver protrusion and group with liver and other organs protrusion, respectively.Neonates had a longer time of needing respiratory machine in group with liver protrusion, comparing with the neonates in group with liver and other organs protrusion[(30.67±19.0) h n vs.(106.25±69.36) h, n P=0.021], and the risk rate to use respiratory machine was 5.143(95% n CI: 0.727-36.368). There were no dead cases in group with liver protrusion.Three cases were dead in group with liver and other organs protrusion.All the patients were followed from 3 months to 4 years, and hernia in abdominal wall was found in 5 cases, including 1 case in group with liver protrusion and 4 cases in group with liver and other organs protrusion.If the dead cases had been excluded, the rate of hernia was 66.7% (4/6 cases) in group with liver and other organs protrusion.n Conclusions:Individual treatment should be emphasized in neonates suffering from GO with liver protrusion.Neonates with single liver protrusion have a better prognosis than those with liver and other organs protrusion.One-stage repair is recommended in the former, and the delayed repair is highly recommended in the latter.The accessory liver should be considered when GO neonates presenting liver protrusion.