小儿肠壁肌腺体异位错构瘤2例并文献复习

Pediatric intestinal wall muscular gland ectopic hamartoma:two cases and literature review

目的探讨肠壁肌腺体异位错构瘤的临床病理学特征、诊断及治疗方法。提高临床工作中的确诊率。方法回顾性分析本院收治的两例小儿肠壁肌腺体异位错构瘤的临床资料和病理学特点并复习相关文献。结果病例1,男,4岁,以“阵发性腹痛10天,加重1天”收入本院。急诊复查腹部彩超提示:右下腹低回声包块(考虑肠套叠可能)。急诊拟“肠套叠”收入本院。病例2,女,1岁1个月,以“呕吐6小时余”为主诉入院。行腹部彩超示:中腹部腹腔内见实质性低回声包块,肠套叠图像。拟“肠套叠”收入我院。两例患者均行手术切除,术后病理诊断为肠壁肌腺体异位错构瘤。结论肠壁肌腺体异位错构瘤临床非常少见,国内外相关文献报道也较少,该病通过临床症状及影像学相关检查很难确诊,临床确诊需依赖于病理学镜下观察。

Objective To investigate the clinicopathological features,diagnosis and treatment of ectopic hamartoma of muscle glands in the intestinal wall.So as to improve the rate of diagnosis in clinical work.Methods Retrospectively analyze the clinical data and pathological characteristics of 2 cases of pediatric intestinal wall muscular gland ectopic hamartoma in our hospital and review relevant literature.Results Case 1,male,4 years old,was admitted to our hospital due to“paroxysmal abdominal pain for 10 days and aggravation for 1 day”.Emergency examination of abdominal color ultrasound suggested hypoechoic mass in the right lower abdomen(intussusception was considered).The the patients was planned to be admitted to our hospital due to intussusception according to emergency diagnose.Case 2,female,1 year old and 1 month,was admitted to hospital due to"vomiting for over 6 hours"as the chief complaint.Abdominal color doppler ultrasound was performed and a substantial hypoechoic mass was found in the abdominal cavity of the middle abdomen,and also displayed an imaging of intussusception.And the female patients was planned to hospitalize in our hospital due to intussusception too.Both cases underwent surgical resection,and the postoperative pathological diagnosis was confirmed as ectopic hamartoma of muscle glands in the intestinal wall.Conclusions The clinical diagnosis of ectopic hamartoma of muscle glands in the intestinal wall is very rare,and there are few reports in related literatures at home and abroad.The disease is difficult to be diagnosed through clinical symptoms and imaging examination,and the clinical diagnosis depends on pathological observation.