小肠镜在儿童波伊茨-耶格综合征中的应用价值及治疗时机

Application value and treatment opportunity of enteroscopy in children with Peutz-Jeghers syndrome

目的探讨单气囊小肠镜在儿童波伊茨-耶格综合征(PJS)中的应用价值及治疗时机。方法回顾性分析湖南省儿童医院2011年1月至2021年9月确诊33例PJS患儿的年龄、性别、家族史、临床症状、小肠镜检查及治疗情况、息肉切除数目、术中及术后并发症、外科手术情况、复发及随访等临床资料,并应用χ2检验进行统计学分析。结果共纳入33例PJS患儿,均接受至少1次单气囊小肠镜检查,其中男21例,女12例;年龄(9.00±3.13)岁。患儿主要表现为黑斑(33例)及消化道多发息肉(31例)。黑斑分布:口唇33例,同时伴随手指末端3例,脚趾末端2例,指趾末端6例。手术过程中共切除息肉391粒,以空肠息肉为主(37.08%,145/391粒)。11例PJS患儿发生肠套叠,其中小肠套叠占90.91%(10/11例)。接受外科手术治疗10例(30.30%,10/33例),因急性难治性肠套叠接受外科手术占72.73%(8/11例)。术中出现肠穿孔1例,出血2例,予止血夹封闭及外科手术治疗均完全恢复。年龄>8岁患儿空回肠息肉和巨大息肉发生率均高于≤8岁患儿[92.55%(149/161粒)比7.45%(12/161粒),96.20%(76/79粒)比3.80%(3/79粒)],差异均有统计学意义(χ2=9.854、8.711,均P<0.05);不同年龄段内肠套叠的发生比例比较,差异无统计学意义(P>0.05)。33例PJS患儿中,57.58%(19/33例)术后1~3年复发,随访至今无癌变病例。结论PJS患儿小肠息肉常见,单气囊小肠镜在PJS患儿中的应用效果可靠,安全性高;8岁以上患儿易发生空回肠息肉及巨大息肉,建议对8岁以上PJS患儿至少完成1次小肠镜检查。

Objective To investigate the application value and treatment opportunity of single balloon enteroscopy in children with Peutz-Jeghers syndrome(PJS).Methods A retrospective analysis was conducted on 33 children diagnosed with PJS in Hunan Children′s Hospital from January 2011 to September 2021.The patient′s age,gender,family history,clinical symptoms,enteroscopy examination and treatment,number of polyps,intraoperative and pos-toperative complications,surgical treatment,recurrence of surgery,and follow-up data were analyzed,and the chi-square test was used for statistical analysis.Results A total of 33 PJS children aged(9.00±3.13)years,including 21 males and 12 females,were included.All of them received at least once single balloon enteroscopy test.The main manifestations of the children were black spots(33 cases)and multiple polyps in the digestive tract(31 cases).In all the 33 cases,black spots were distributed on lips.Some black spots were also found at the end of fingers(3 cases),at the end of foot toes(2 cases),and at the end of finger toes(6 cases).During the operation,391 polyps were removed,most of which were jejunum polyps(37.08%,145/391 polyps).Eleven children with PJS has intussusception,of which intestinal intussusception accounted for 90.91%(10/11 cases).Ten cases(30.30%,10/33 cases)received surgical treatment,and 72.73%(8/11 cases)underwent surgery for acute refractory intussusception.One case had intestinal perforation and 2 cases were bleeding during the operation,and the 3 cases recovered completely after hemostatic clip sealing and surgical treatment.The incidence of hollow ileum polyps and giant polyps in children aged>8 years was higher than that in children aged≤8 years[92.55%(149/161 polyps)vs.7.45%(12/161 polyps),96.20%(76/79 polyps)vs.3.80%(3/79 polyps)].The differences were statistically significant(χ2=9.854,8.711,all P<0.05).There was no significant difference in the incidence of intussusception among different age groups(P>0.05).Among the 33 children with PJS,57.58%(19/33 cases)had recurrence 1-3 years after operation,and no cases of cancer have been followed up so far.Conclusions Intestinal polyps are common in children with PJS,and the application of single-balloon enteroscopy in children with PJS is reliable and safe.Children over 8 years old are more vulnerable to empty ileum polyps and giant polyps.Therefore,it is advised that children aged above 8 years with PJS should undergo at least once enteroscopy.