Cliinicalanalysis Of Total Colonic Aganglionosis In Children

Objective: To explore clinicalfeature,diagnosis and treatment of Total colonic aganglionosis in children.Methods: The clinical data were retrospectively analyzed for 41 TCA children admitted from January 2010 to June 2018.Result:(1)There are 41 cases in this group,including 31 males and 10 females,with a ratio of 3: 1;There were 38 full-term infants(92.7%)and3 premature infants(7.3%).There were 39 cases(95.1%)with normal birth weight and 2 cases(4.9%)with low birth weight.(2)The main manifestations included abdominal distention(90.2%),delay defecation(82.9%),emesis(65.9%)and constipation(63.4%),and there is no specificity between this group of TCA with other types of Hirschsprung’s disease(P>0.05).(3)33 children accepted barium enema before operation,7(21.2%)of them were suggested TCA,but 32 children could see delayed barium emptying and markedly inflatable small intestine in the background of barium enema.the positive rate of anorectal manometry was 71.4%;the positive rate of rectal mucosal biopsy was100%.(4)There were 38 children accepted the operation,25 of them were cured by operation and 21 of them were followed.The changes of intestinal morphology in TCA: 7(18.4%)children could only see the terminal ileum stenosis;5(13,2%)children only saw the total colonic stenosis;24(63.2%)children could see both terminal ileum and total colonic stenosis;2(5.2%)children couldn’t see any stenosis of ileum and colon.The postoperative pathological results all support the diagnosis of TCA.(5)Complications after radical operation were mainly enterocolitis(47.6%)and malnutrition(47.6%),and no anastomotic leakage occurred;14 children were accepted the quality of life score,10(71.4%)of them have a brilliant quality of life and 4(28.6%)of them have a good quality of life.Conclusion: The clinical diagnosis of Total colonic aganglionosis is difficult,barium enema can indicate the disease to a certain extent,diagnosis should combined with surgical exploration and pathological examination,through operation and postoperative standardized treatment,the long-term prognosis is good.