Clinicalanalysis Of 31 Cases Of Total Colonic Aganglionosis

Objective: To investigate the clinical features,treatment options and prognosis of total colonic aganglionosis(TCA).Methods: Case data of 31 children with TCA in the General Surgical Ward 2 Department of our hospital between January 2010 and April 2017 were analyzed.Every patients have pathological examination.To count the clinical manifestations,imaging examinations,pathological examinations,surgical methods,and follow-up defecation conditions of 31 children with TCA admitted in the General Surgical Ward 2 Department of our hospital from January 2010 to April 2017.Results: In the 31 children,all of them have the main symptom of abdominal distention or vomiting and only 2 cases on cardiac ultrasound from total 23 cares were normal,25 cases of barium enema angiography: 7cases of barium angiography prompted TCA,5 cases prompted fetal colon.31 cases of rectal mucosa biopsy,intraoperative frozen or postoperative pathological examination all confirmed that no gangliocyte were found between submucosal and muscular walls of the lesioned intestine.Thirteen children received stage I and II staging surgery in our hospital,and thenwere discharged successfully.Among them,8 children showed good defecation and 5 children were lost in the 3-month-5-year follow-up.Two children underwent radical surgery(stage I)in our hospital and then were discharged successfully;in the 7-month-2-year follow-up,1 child showed good defecation(BFS score,18)and another was lost.One child was treated with ileostomy in another hospital and radical colectomy for Hirschsprung's disease in stage II in our hospital,presenting good defecation after surgery.One child was subjected to ileostomy in our hospital and radical colectomy for Hirschsprung's disease in stage ? in another hospital.Five children only received ileostomy in our hospital;among them 1 child's family members gave up treatment due to severe renal dysfunction after surgery.Nine children's family members gave up treatment after confirmation of TCA.Conclusion: The onset of TCA is early and serious.Combined with clinical manifestations,barium enema,rectal mucosa biopsy or intraoperative frozen biopsy,postoperative pathological examination,TCA can be diagnosed.Stage I radical surgery is suitable for children who have definite preoperative diagnosis or good general conditions,Staging surgery is suitable for most TCA children.In our cases,9 children gave up treatment after confirmation of TCA,1 child gave up treatment because of severe renal function impairment.With the improvement of treatment of TCA diagnosis,the effect will be greatly improved.