摘要
目的了解B型肠神经元发育异常症的诊断与手术治疗效果。方法对 4 5例该病患儿进行回顾性分析及远期随访。术前所有患儿均行钡灌肠X线检查 ,2 3例行下消化道动力学检测 ,17例行直肠粘膜活检加S10 0蛋白免疫组织化学染色。所有患儿均行病变肠段切除、结肠直肠吻合术。行多处全层活检手术标本。结果本组中 16例为单纯性B型肠神经元发育异常症 ,2 8例合并先天性巨结肠 ,1例合并肠神经节细胞减少。 16例单纯性行钡灌肠X线检查时仅有 4例可见确切的狭窄段 ,9例单纯性中有 6例未出现直肠肛管抑制反射 ,17例活检中只有 7例可获得提示性诊断。全层活检可准确诊断该病。术后 3例发生小肠结肠炎患儿经保守治疗痊愈 ,1例发生闸门症候群而再次手术 ,其他患儿均能自解、自控大便。结论全层活检是诊断该病的可靠方法。病变肠段切除、结肠直肠吻合术治疗本病可获得满意的疗效。
Objective To evaluate the diagnosis and treatment of intestinal neuronal dysplasia type B (IND) in childhood. MethodsForty-five patients underwent preoperative barium enema examination, 23 patients underwent electromanometry, and mucosal biopsy and immunohistochemical staining for S100 protein were performed in 17 cases. All 45 patients underwent resection of the invalid segment with coloproctostomy. Whole layer was sampled on several sites of the resected segment and examined by two independent pathologists. All patients were followed up from 3 months to 9 years (mean 4.6 years).ResultsTwenty eight patients were complicated with Hirschsprung′s disease, one patient with hypogangliosis, and isolated IND was diagnosed in the other 16 children. The narrowed distal segment with proximal dilatation was merely noted in 4 children with isolated IND. Internal sphincter relaxations were missing in 6 children with isolated IND. The indicative diagnosis might be merely gained in 7 patients by the mucosa biopsy. The correct diagnosis can be established by whole layer biopsy of the resected segment. Three children with enterocolitis after operation were cured by conservative treatment. One patient suffering from postoperative sluice syndrome underwent second resection. Postoperative continence was achived in all patients. ConclusionThe correct diagnosis of IND can be obtained by biopsy of whole layer, and resection of invalid bowel segment with coloproctostomy is the choice of therapy.
出处
《中华普通外科杂志》
CSCD
北大核心
2003年第6期350-352,共3页
Chinese Journal of General Surgery
关键词
B型肠神经元发育异常症
诊断
治疗
外科手术
Digestive system abnormalities
Diagnosis
Surgical procedure, operative
