十二指肠重复畸形的临床特点及诊治  

Clinical characteristics ,diagnosis and treatment of duodenal duplication

作　　者：王凯[1] 彭春辉[1] 庞文博[1] 王增萌[1] 吴东阳[1] 陈亚军[1] Wang Kai;Peng Chunhui;Pang Wenbo;Wang Zengmeng;Wu Dongyang;Chen Yajun(Department of General Surgery,Beijing Children’s Hospital,Capital Medical University,National Center for Children’s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心首都医科大学附属北京儿童医院普外科,北京100045

出　　处：《中华小儿外科杂志》2019年第11期1025-1029,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的 研究十二指肠重复畸形的临床特点、诊治及预后情况.方法 回顾性分析2007年5月1日至2018年5月1日就诊于北京儿童医院,行手术治疗,术后病理诊断为十二指肠重复畸形,资料完整且可获得随访信息的患儿4例.总结归纳其临床特点、诊治及预后情况.4例患儿中,男女各2例,发病年龄1个月至9岁.临床表现为呕吐(4例),腹痛(2例),发现腹部肿物(2例),孕期B型超声提示腹部肿物(1例).4例患儿B型超声检查均发现右上腹囊性肿物,且囊壁与肠壁结构相似,其中1例囊壁存在明显蠕动.CT及上消化道造影可发现囊肿压迫周围组织.4例患儿均为囊肿型十二指肠重复畸形.其中1例患儿外院误诊为假性胰腺囊肿行囊肿外引流术,本次手术探查时腹腔粘连严重,经术中B型超声定位明确十二指肠重复畸形位置后,完整剥除重复畸形内壁黏膜组织;另外3例患儿行十二指肠重复畸形完整切除术.结果 4例患儿术后病理均回报囊壁内衬肠道黏膜,可及肠壁肌层,其中2例患儿存在异位胰腺组织.术后随访期间,患儿一般情况良好,均无胰腺炎发作,无腹痛、呕吐症状,无腹胀、停止排气排便等肠梗阻症状.结论 十二指肠重复畸形罕见,临床表现与其大小、部位、是否存在异位黏膜组织、是否与肠腔及胰胆管相通有关,明确诊断靠术后病理.重复畸形完整切除或行黏膜剥除者,预后良好;但是若患儿一般情况弱,存在腹腔严重粘连等情况时,仅行开窗内引流术也需完整剥除重复畸形的黏膜组织,确保无异位黏膜组织残留,防止癌变发生.Objective To explore the clinical characteristics , diagnosis and treatment of duodenal duplication .Methods From May 1 , 2007 to May 1 , 2018 , 4 surgical children were diagnosed pathologically as duodenal duplication .With complete follow-up data , their managements and prognosis were analyzed .There were 2 boys and 2 girls with an age range from 1 month to 9 years .The clinical symptoms included vomiting (n=4) ,abdominal pain (n=2) ,abdominal mass (n=2) and ultrasonic finding of abdominal mass (n= 1) .Ultrasound revealed cystic mass in right upper quadrant (n=4) .Cystic wall had the characteristics of intestinal wall and even peristalsis was noticed (n= 1 ) .Computed tomography (CT ) and upper gastroenterography indicated tissue compressing around cyst .All of them had cystic duodenal duplication .One case was misdiagnosed as pancreatic pseudocyst and underwent external drainage .And subsequent severe abdominal adhesion required cystic mucous stripping .The other 3 patients underwent total cyst removal .Results Pathological examination revealed intestinal mucosa (n=4) and ectopic pancreas (n= 2) .During follow-ups ,4 children recovered well and there was no occurrence of vomiting ,abdominal pain or pancreatitis . Conclusions Duodenal duplication is rare and its clinical manifestations are correlated with size , location ,ectopic mucosa and communication with intestinal cavity or pancreatic bile tract .Diagnosis is mainly based upon pathology .Total removal of duplication or mucous stripping has an excellent prognosis .Endoscopic marsupialization should be coupled with mucous stripping for malignant transformation .

关 键 词：十二指肠 畸形 诊断 治疗 

分 类 号：R73[医药卫生—肿瘤]