危重型巨大脐膨出的临床特征与治疗策略

Clinical characteristics and treatment of critical giant omphalocele

目的：探讨危重型巨大脐膨出的临床特点和治疗策略。方法收集7例危重型巨大脐膨出患儿的临床资料，分析其临床特点、治疗方法及转归，并报道1例经重组人表皮生长因子（rhEGF）同时缓慢施压还纳内容物的保守方法治愈的危重型巨大脐膨出典型患儿的治疗经过。结果7例患儿缺损直径6～8 cm，突出器官包括肝、胆囊、胃、肠，合并畸形5例，合并感染6例。早期关腹6例，延迟关腹1例，关腹时间1～100 d，机械通气时间5～110 d。择期手术5例，急诊手术1例，保守治疗1例。术后死亡4例，存活需二期手术1例。保守治疗成功1例。该例患儿的胎龄35＋2周，出生体质量2.6 kg，缺损直径8 cm，采取经囊膜悬吊并缓慢加压还纳膨出物，每日勺刮皮肤与囊膜交界处皮缘，同时涂抹 rhEGF 促进皮肤生长，每日消毒、保湿囊膜，15周后囊膜逐渐被爬升的皮肤及肉芽组织替代，呼吸机使用时间仅18 d。结论危重型巨大脐膨出病情危重，多合并畸形，易发生感染，手术治疗效果欠佳，可以尝试 rhEGF 促进皮肤生长联合缓慢施压还纳治疗，可能有一定效果。

Objective To investigate the clinical characteristics and treatment of critical giant omphalocele.Methods Clinical data of 7 neonates diagnosed with critical giant omphalocele were retrospec-tively reviewed to analyze the clinical characteristics,treatment and clinical outcomes.One case with critical giant omphalocele was healed by administration of recombinant human epidermal growth factor （rhEGF）com-bined with conservative reduction of omphalocele contents.Results Seven neonates presented with abdominal defects with a diameter of 6-8 cm.The affected organs included liver,gallbladder,stomach and intestine.Five children were complicated with deformity and six with infection.Six cases had early abdominal closure and one had delayed abdominal closure.The time of abdominal closure ranged from 1 to 100 d and the time of mechani-cal ventilation was between 5 and 110 d.Five children were scheduled to undergo surgery,one received emer-gency surgery and one subjected to conservative therapy.Four patients died after surgery.One child who sur-vived required two-stage operation.One case was successfully healed by conservative therapy.This child was born at 35 ＋2 weeks with birth weight of 2.6 kg.The diameter of abdominal defect was 8 cm.The patient re-ceived trans-membrane suspension combined with reduction of omphalocele contents,and the junction skin be-tween the skin and cyst membrane was scraped daily and administered with rhEGF to accelerate skin growth.Disinfection and cyst membrane moisture were performed daily.At 15 weeks,cyst membrane was gradually re-placed by the skin and granulation tissues.The time of respirator use was only 18 d.Conclusions Critical gi-ant omphalocele is a severe disease,constantly complicated with deformity and infection.Surgical approach yields poor clinical efficacy.Combined therapy of use of rhEGF and reduction of omphalocele contents might be efficacious in treating critical giant omphalocele.