| Background Intestinal dysganglionosis(IDs)is a kind of intestinal motility disorder diseases,which because of intestinal dysganglionosis lead to abnormal distribution of the enteric nervous system,it is a common cause of pediatric chronic constipation,which includes Hirschsprung's disease(HD)and Hirschsprung's disease-allied disorders(HAD).Hirschsprung's disease(HD)is a functional intestinal obstruction characterized by severe constipation due to the absence of myenteric and submucosal ganglionic cells of the distal alimentary tract.HD accounts for 20 to 25 % of neonatal intestinal obstruction,Since it was first described by H?rald Hirschsprung in 1888,it has become the best characterized disease of intestinal dysganglionosis.With increasing knowledge of the pathophysiology of intestinal dysganglionosis,it became apparent that there is a spectrum of enteric nervous system(ENS)malformations with clinical presentation resembling HD despite the presence of ganglion cells on the patients' intestinal specimens.Since Meier-Ruge published his classic article describing intestinal neuronal dysplasia in 1971,numerous articles appeared in the literature reporting severe types of congenital ENS disorders,these diseases are called Hirschsprung's disease-allied disorders(HAD).The definitive diagnosis of HD and HAD is the pathologic examination of the intestine,especially full-thickness rectal biopsy provides the most definitive answer of HAD.However,this usually has some medical risks,it not only needs general anesthesia,but also the suturing of the biopsy site with potential,and severe com plications maybe occur.To avoid these complications,some noninvasive or less invasive tests including barium enema(BE),anorectal manometry(ARM)were developed,but those tests usually led to different conclusions which cause the difficulty in clinical diagnosis.For these HD and HAD children,conservative treaments such asg glycerol enema,physiological saline enema,drugs can be adopted,once it not useful or clinical sympotoms recurred,operation is needed to resect the dysganglionic bowel segments.Once the entire colon,even the ileocecal junction,are affected,one-stage intestinal colostomy is needed.For those children who with severe clinical symptoms,such as IND-A,intestinal perforation,need emergency operation,because of the serious conditions.There is a great variety of HADs,clinical manifestations lack of specificity,the positive rate of auxiliary examination is low,early clinical diagnosis are more difficult.Objective In this paper,from January 2013 to January 2017,clinical data of 120 cases of the Intestinal dysganglionosis(IDs)of the first affiliated hospital of zhengzhou university were retrospectively analyzed,which includes 101 cases of Hirschsprung's disease(HD)and 19 cases of Hirschsprung's disease-allied disorders(HAD).Combined with related literature at home and abroad,analysis those cases,compare the postoperative complications and effects,summary of clinical experiences,and give help for the clinical treatment and diagnose of the Hirschsprung's disease-allied disorders(HAD).Methods In this paper,from January 2013 to January 2017,clinical data of 120 cases of the Intestinal dysganglionosis(IDs)of the first affiliated hospital of zhengzhou university were retrospectively analyzed,which includes 101 cases of Hirschsprung's disease(HD)and 19 cases of Hirschsprung's disease-allied disorders(HAD).this group of children can be divided into two categories: Hirschsprung's disease(HD);Hirschsprung's disease-allied disorders(HAD),and the 101 cases of Hirschsprung's disease(HD)children can be divided into three categories: the short HD((S-HD),the long HD(L-HD),the total colonic aganglionosis(TCA),compare the postoperative complications and effects.Among HD group,9 cases of Hirschsprung's disease were treated with one-stage colostomy,and the modified Soave radical resection of colon was performed in the first stage of the patients with Hirschsprung's disease(),and 92 patients underwent one-stage modified Soave radical operation,Including 31 cases of laparoscopic assisted radical resection of the large intestine and abdomen,radical resection of the large intestine and abdomen in the treatment of Hirschsprung's disease in 18 cases,and the radical resection of the radical resection of the large intestine in the perineum of 43 cases;Hirschsprung allied disease group 1 cases received intestinal fistula,two patients underwent T fistula,2 cases underwent laparoscopic sigmoid colon resection anastomosis,2 cases received intestinal fistula,two patients underwent radical resection of colon(megacolon modified Soave subtotal resection in 1 cases total colectomy,1 cases),14 underwent a period of improved Soave Hirschsprung's disease,including laparoscopic assisted abdominoperineal resection in 9 cases of colon(sigmoid colon resection in 2 cases,subtotal colectomy in 7 cases),abdominal perineal megacolon radical operation(4 cases of sigmoid descending colon resection in 1 cases,.Intestinal subtotal resection in 1 cases,total colectomy in 2 cases),perineal megacolon radical operation in 1 cases(sigmoid colon resection),the sigmoid colon resection in 4 cases,subtotal colectomy in 9 cases,total colectomy in 3 cases.The postoperative recovery of children were follow-up by outpatient service and telephone for a long time.Combined with related literature at home and abroad,Sorting and comparing complications of various operation was analyzed by statistical analysis.Results: All the patients in the Hirschsprung's disease group were improved after the modified Soave radical operation,but In the near future,most of the patients had different degrees of defecation and the skin redness,erosion and so on,In particular,children undergoing subtotal colectomy and total colectomy.Based on the evaluation criteria of Holschneider stool function,the patients were followed up for 1 years after radical operation,In 101 children with HD: The number of stool was normal in 93 cases,and the defecation time was less than 5 minutes in each case and in the case of more than 15 minutes in all of the 1 cases;97 cases had no stool,and the other one was contaminated in 4 cases,of which,there were more than 3 cases in each of the 97 cases.The anal sensation was normal in all the patients in 1 cases,and in the other cases,there were 3 cases disappeared in time,and all of them disappeared completely;The stool on the ability to control 97 cases up to more than 1 minutes,3 cases,1 cases of not less than 1 minutes;stool on 87 cases and 9 cases of normal stool shapeless but non watery,5 slightly dry stool;Monthly Enema Glycerini,diapers,drug treatment is less than 3 of the 93 cases,greater than or equal to 3 times of the 8 cases,0 cases were more than 3 times a week.In HAD: 1 patients underwent sigmoid colon resection after January because of the recurrence of symptoms underwent subtotal colectomy,with good recovery,1 cases of type T fistula after a year and a half of the children for plugging off after the fistula,good recovery;9 patients underwent subtotal colectomy and 3 patients underwent total colectomy,the number of recent defecation increased,with varying degrees of perianal skin erosion,Through 1 years after the operation of children with fecal function assessment: The number of stool was normal in 17 cases,and the defecation time was less than 5 minutes in 15 cases and in the case of more than 15 minutes in all of the 1 cases;18 cases had no stool,and the other one was contaminated in 1 cases;the anal sensation was normal in all the patients in 18 cases,and in the other cases,there were 1 case disappeared in time;The stool on the ability to control 17 cases up to more than 1 minutes,2 cases less than 1 minutes;stool on 16 cases are normal and 3 cases of normal stool shapeless but non watery;Monthly Enema Glycerini,diapers,drug treatment is less than 17 cases,greater than or equal to 3 times of the 2 cases,0 cases were more than 3 times a week.For those HAD children,Partial resection of the colon may result in the recurrence of the disease and the improvement of abdominal distension and constipation after subtotal colectomy and total colectomy;T type ostomy is an effective choice for the treatment of neonates and young children.The data were analyzed by statistical methods: rank sum test was used to evaluate the defecation function score of the common HD group,long segment HD group and the whole colon type HD group,P>0.05,Therefore,the improvement of Soave surgery on the postoperative defecation function of all types of HD improved the same situation;the HD group and HAD group,the prevalence of sex,meconium discharge chi square test,In the prevalence of gender,P=0.17>0.05,HD group and HAD group had no statistical significance in the prevalence of gender,the two groups were mostly male patients(82.2%,68.4%),it can be seen that HD and HAD were more common in male children,In the case of meconium discharge,P=0.00<0.05,was statistically significant,HD group and HAD group were 92.1% and 36.8% respectively,so it can be concluded that HAD children with meconium discharge delay is less than HD;HD group and HAD group after the improvement of defecation function of the indicators of the chi square test,P were >0.05,no statistical significance,so the two groups after surgery,bowel function recovery of the same situation;The results of fast frozen HD group and HAD group of intraoperative and postoperative pathological specimen consistency of chi square test,P=0.01<0.05,have statistical significance,HD group and HAD group consistency rates were 93.1%,73.7%,and HAD group of intraoperative frozen pathological results accuracy lower than HD group;The HD group and HAD group,postoperative complications were chi square test,P=0.001<0.05,have statistical significance,two groups of postoperative complications were 5.9%,31.6%,and HAD group of postoperative complications is higher than that of HD group.Conclusion 1.Improved Soave megacolon radical has same effective on improve the postoperative defecation functions for three types HD,The postoperative defecation functions of HAD and HD were improved at the same level,the incidence of postoperative complications on HAD is higher than HD 2.HAD and HD are usually happen in boys,but HAD does not have so much delayed meconinium exclusion.3.the accuracy of the frozen section examination on HAD is lower than HD.4.Subtotalcolectomy and totalcolectomy are the better methods to improve the postoperative defecation functions of HAD,the T enterostomy can be a effective choice for these newborn and premature infants. |
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