产前诊断的先天性十二指肠梗阻胎儿出生后的临床特点及治疗

Clinical characteristics and treatments of prenatally diagnosed congenital duodenal obstruction in children

目的总结产前诊断的先天性十二指肠梗阻胎儿出生后的临床特点及治疗经验。方法回顾性分析2011年1月至2020年12月浙江大学医学院附属儿童医院收治的产前诊断为十二指肠梗阻且出生后接受手术治疗的105例患儿临床资料,其中男48例,女57例;首次诊断胎龄在15~39周,出生时胎龄为31^(+1)~42周;患儿出生时体重为(2746.9±608.6)g;早产38例;入院时患儿中位年龄为9 h,范围在1 h至21 d。出生后患儿呕吐40例,其中未开奶26例,开奶14例。伴发其他系统畸形80例,其中先天性心脏病69例,36例患儿合并2种及2种以上畸形。所有患儿均接受手术治疗,术中证实患儿病理类型,术后记录患儿开始进食时间、术后住院时间,观察患儿并发症情况。结果105例患儿手术均成功完成,其中开腹手术23例,腹腔镜手术80例(中转开腹2例),机器人辅助2例。术中发现病理类型包括环状胰腺78例,十二指肠闭锁15例,十二指肠狭窄10例,肠旋转不良13例,十二指肠前门静脉2例,空肠起始部闭锁1例;其中14例患儿同时存在2处或2处以上梗阻。术后开始进食时间(7.64±3.28)d,术后住院时间(16.66±9.12)d。术后出现并发症3例,其中1例吻合口漏及1例肠梗阻患儿,经保守治疗后好转;另1例十二指肠远端闭锁及空肠近端Ⅲ型闭锁术后吻合口狭窄,再次行肠切除肠吻合联合十二指肠成形术后好转。结论产前诊断的先天性十二指肠梗阻胎儿出生后就诊及治疗时间提前,出生后应禁食及胃肠减压,早期手术治疗后预后良好。环状胰腺为最常见的病理类型。通常伴发合并其他系统畸形,产前需重点筛查染色体及心脏。

Objective To summarize the postnatal clinical characteristics and treatments of fetuses with prenatally diagnosed congenital duodenal obstruction(CDO).Methods A total of 105 children with prenatally diagnosed CDO operated after birth were reviewed between January 2011 and December 2020.There were 48 boys and 57 girls with a gestational age of 15-39 weeks at an initial diagnosis,a gestational age of 31^(+1)-42 weeks at birth and an average birth weight of 2746.9(1350-4250)gram.Among them,38 were born prematurely.The median age of admission was 9 hours(1-21 days).Postnatal vomiting occurred with(n=14)or without(n=26)breastfeeding.There were 80 cases with other system malformations,including congenital heart disease(n=69)and 2 or more types of other malformations(n=36).All pathological types were confirmed intraoperatively.Feeding time,length of hospital stay and postoperative complications were recorded.The relevant clinical data were processed by SPSS 19.0.Results All surgical procedures were successfully performed,including laparotomy(n=23),laparoscopy(n=80)(converting into laparotomy,n=2)and robot-assisted surgery(n=2).Annular pancreas(n=78),duodenal atresia(n=15),duodenal stenosis(n=10),malrotation(n=13),preduodenal portal vein(n=2)and jejunal origin atresia(n=1)were diagnosed.Among them,14 patients had two or more obstructions.Postoperative feeding time were(7.64±3.28)days and postoperative hospitalization stay(16.66±9.12)days.Postoperative complications occurred(n=3),including anastomotic leakage(n=1)and intestinal obstruction(n=1).Both improved after conservative measures.Another case of anastomotic stenosis after distal duodenal atresia and proximal jejunal type III atresia improved after intestinal resection and anastomosis plus duodenoplasty.Conclusions Time of admission and treatment of prenatally diagnosed CDO were advanced.Fasting and gastrointestinal decompression should be performed after birth and the prognosis is excellent after early surgery.Annular pancreas is the most common pathological type.Usually accompanied by other system malformations,prenatal screening should focus on chromosomes and heart.