Charlesworth P, Ervine E, McCullagh M
The Royal Alexandra Hospital for Sick Children, Brighton, UK.
Pediatr Surg Int. 2009 Jan;25(1):77-81. doi: 10.1007/s00383-008-2292-8. Epub 2008 Nov 12.
In exomphalos major (EM), closure of the defect in the abdominal wall presents a challenge. The aim of this study is to evaluate a single centre experience of EM.
A 15-year retrospective case-note review; data presented as median (range).
Fourteen infants (7 female) were born with EM: birth weight 2.9 (1.2-3.8) kg, gestational age 38 (31-39) weeks. One infant died in utero and one within the first hour of life. Severe pulmonary hypoplasia was present in 7/13 (54%), and there was a mortality of 6/13 (46%) live births. Infants were treated non-operatively primarily. Two infants underwent early surgery: one infant, born with a ruptured sac, had a surgical silo constructed on day 1 and closure on day 8, while a second infant had partial closure (skin only) on day 11. Ten infants had application of silver sulphadiazine to the sac 2-3 times per week. Enteral feeds were established soon after birth. They were discharged from hospital to allow granulation. Ventral hernia closure was performed on a subsequent admission.
Exomphalos major can be successfully treated non-operatively, allowing immediate enteral feeding and early discharge while granulation takes place. In this series, exomphalos major has an incidence of 1 in 26,000, mortality is 46% and severe pulmonary hypoplasia is present in 54% of infants.
在巨大脐膨出（EM）中，腹壁缺损的闭合是一项挑战。本研究的目的是评估单中心处理巨大脐膨出的经验。
一项为期15年的回顾性病例记录研究；数据以中位数（范围）呈现。
14例婴儿（7例女性）患有巨大脐膨出：出生体重2.9（1.2 - 3.8）kg，胎龄38（31 - 39）周。1例婴儿在子宫内死亡，1例在出生后1小时内死亡。严重肺发育不全在7/13（54%）的婴儿中存在，活产婴儿的死亡率为6/13（46%）。婴儿主要接受非手术治疗。2例婴儿接受了早期手术：1例出生时囊膜破裂的婴儿在第1天构建了手术袋，并于第8天闭合，而另1例婴儿在第11天进行了部分闭合（仅皮肤）。10例婴儿每周2 - 3次在囊膜上应用磺胺嘧啶银。出生后不久即开始肠内喂养。他们出院以促进肉芽组织形成。随后入院时进行腹疝修补术。
巨大脐膨出可以通过非手术成功治疗，在肉芽组织形成期间允许立即进行肠内喂养并早期出院。在本系列研究中，巨大脐膨出的发病率为26000分之一，死亡率为46%，54%的婴儿存在严重肺发育不全。
