儿童结肠系膜淋巴管畸形的临床特点分析

Clinical features of colonic mesenteric lymphatic malformation in children

目的分析儿童结肠系膜淋巴管畸形的临床特点及手术方式选择。方法回顾性分析2010年1月至2021年12月首都医科大学附属北京儿童医院手术治疗的44例结肠系膜淋巴管畸形患儿的临床资料,分析其囊肿特点及手术方式选择的影响因素。通过门诊或电话随访预后,包括有无残留、复发或粘连性肠梗阻等并发症。通过多因素二分类logistic回归分析探究选择开腹或腹腔镜手术的影响因素。结果男24例,女20例,平均就诊年龄为2.9岁。腹痛、腹胀和发热为主要临床症状。囊肿主要位于乙状结肠系膜(31.8%,14/44)和横结肠系膜(27.3%,12/44);临床分型主要为Ⅰ型(34.1%,15/44)和Ⅱ型(45.5%,20/44);病理类型包括大囊型(86.4%,38/44)和混合型(13.6%,6/44);囊肿平均长径为8.5 cm,囊液性质清亮占56.8%(25/44),浑浊占34.1%(15/44)。术前主要并发症为囊内出血(43.2%,19/44)和囊肿感染(25.0%,11/44)。44例患儿行开腹手术(含腹腔镜中转开腹)31例(70.5%),腹腔镜手术13例(29.5%);其中单纯囊肿剥除23例(52.3%),完整囊肿切除并肠切除17例(38.6%),囊肿部分切除并开窗引流4例(9.1%)。Ⅰ型结肠系膜淋巴管畸形行开腹手术(13/15比10/20,P=0.034)及完整囊肿切除并肠切除(13/15比2/20,P<0.001)的比例明显高于Ⅱ型。多因素分析表明Ⅰ型结肠系膜淋巴管畸形(OR=7.0,95%CI 1.1~45.5,P=0.034)和术前囊内出血(OR=8.3,95%CI 1.3~52.6,P=0.026)是选择开腹或腹腔镜中转开腹的主要原因。43例(97.7%)患儿获得随访,平均随访时间为4.3年,9例(20.5%,9/44)出现术后并发症(残留或复发5例,粘连性肠梗阻2例,腹壁切口疝1例,污粪1例)。结论结肠系膜淋巴管畸形好发部位与系膜范围相关。该病术前并发症发生率高,术前囊内出血是除临床分型外决定手术方式选择的最主要因素。

Objective To explore the clinical features and surgical approaches of colonic mesenteric lymphatic malformation in children.Methods From January 2010 to December 2021,the relevant clinical data were retrospectively reviewed for 44 operated children of colonic mesenteric lymphatic malformation.Cyst characteristics and influencing factors of surgical approaches were recorded.Follow-ups of postoperative complications(residual cyst,recurrence&adhesive ileus)were conducted at outpatient clinics or through telephone interviews.Multivariate binary Logistic regression analysis was employed for examining the influencing factors of selecting laparotomy or laparoscopy.Results There were 24 boys and 20 girls with an average age of 2.9 years.The major symptoms were abdominal pain,abdominal distention and fever.Cysts of colonic mesenteric lymphatic malformation were located at sigmoid mesocolon(31.8%)and transverse mesocolon(27.3%).Type Ⅰ(34.1%)and type Ⅱ(45.5%)dominated.The pathological types were macrocystic(86.4%)and mixed(13.6%).Average long diameter of cyst was 8.5 cm.Fluid was serous(56.8%)and turbid(34.1%).Hemorrhage(43.2%)and infection(25.0%)of cyst were the predominant preoperative complications.The procedures included laparotomy(n=31,70.5%),including laparoscopy converting into laparotomy,and laparoscopy(n=13,29.5%).Simple cyst excision(n=23,52.3%),complete cyst excision with segmental bowel resection(n=17,38.6%)and partial cyst excision&drainage(n=4,9.1%)were performed.The percentages of laparotomy(13/15 vs 10/20,P=0.034)and complete cyst excision with segmental bowel resection(13/15 vs 2/20,P<0.001)were significantly higher performed in type Ⅰ than in type Ⅱ patients.Multivariate analysis revealed that type Ⅰ classification(OR=7.0,95%CI 1.1-45.5,P=0.034)and preoperative hemorrhage(OR=8.3,95%CI 1.3-52.6,P=0.026)were two major factors for selecting laparotomy.Forty-three patients(97.7%)were followed up with a mean period of 4.3 years.Among 9 complicated cases(20.5%),there were residual cyst or recurrence(n=5),adhesive ileus(n=2),incisional hernia(n=1)and soiling(n=1).Conclusions The incidence of colonic mesenteric lymphatic malformation is correlated with area of mesocolon.Preoperative complications are common in children with colonic mesenteric lymphatic malformation.Preoperative hemorrhage and specific types may determine the choice of surgical approaches.