神经节细胞减少症引起新生儿急性肠梗阻的诊治

目的 探讨神经节细胞减少症引起新生儿急性肠梗阻的诊断、治疗方法.方法 选取我院在2012年3月～2013年3月收治的24例神经节细胞减少症引起的急性肠梗阻新生儿作为研究对象,对其诊断、治疗方法,术前检查及临床表现进行回顾性分析.结果 16例（66.7％）钡剂灌肠后可见扩张段、狭窄段,与先天性巨结肠相比,无明显移行段,扩张段为肠腔稍扩张,狭窄段较短,结肠壁僵硬.有20例（83.3％）患儿的24小时钡剂排空较差,17例（70.8％）患儿的AChE呈阳性.10例可见U型或W型RIAR,肠蠕动频率及节律性降低.行巨结肠根治术后,未发生吻合口瘘,未出现大便失禁及复发者.2例行关瘘术的患儿,未出现腹胀、排便困难症状.无1例死亡.结论 神经节细胞减少症可引起新生儿急性肠梗阻,临床诊断需采用病理检查手段,临床治疗需采用肠造瘘或巨结肠根治术.

Diagnosis and treatment of ganglion cells of neonatal thrombocytopenia induced acute intestinal obstruction

Objective： To investigate the cause of ganglion cells to reduce disease diagnosis , treatment of neonatal acute intestinal obstruction. Methods ： 24 cases of acute intestinal obstruction in the newborn ganglion cells induced thromboeyto- penia in our hospital from March 2012 to March 2013 were selected as the study objects, and the prognosis, treatment method, preoperative examination and clinical manifestations were retropeetively analyzed. Results： After the expansion section visible barium enema 16 eases （ 66.7% ） , a narrow segment , compared with Hirschsprung~ disease , no signifi- cant transitional segment , expanding segment of the intestine is slightly expanded , the shorter the stricture , colon wall stiffness. There are 20 cases （ 83.3% ） children 24 hours barium emptying poor , AChE 17 patients （ 70.8% ） children were positive. 10 cases seen U - shaped or W - shaped RIAR, decreased bowel frequency and rhythm . Line Hirschsprung postoperative anastomotic leakage did not occur , fecal incontinence and recurrence was observed. 2 children routinely shut fistula , abdominal distension , bowel symptoms do not appear difficult . No one died. Conclusion ： The ganglion cells of neonatal thrombocytopenia can cause acute intestinal obstruction , clinical pathology diagnosis requires the use of tools, clinical treatment requires the use of intestinal fistula or megacolon radical mastectomy.