The Strategy Of Laparoscopic Diagnosis And Treatment For Hirschsprung’s Disease And Its Allied Disorders

Purpose：Congenital megacolon (Hirschsprung’s disease, HD), also known asaganglionosis, is a common gastrointestinal malformation which ischaracterized by the absence of ganglion cells in the distal intestine.Hirschsprung’s allied disorders (HAD) represents as the reduction ordegeneration of ganglion cells in the intestine.The diagnosis of HD and HADmainly depends on the clinical manifestation and classical triple check, whichincluding barium enema, anorectal manometry and the intraoperative tissuebiopsy. Operation is considered to be the most important treatment for thediseases. Three procedures, including Swenson’s, Duhamel’s and Soave’soperation, are widely used for HD and HAD. With the development ofminimally invasive surgery (MIS), the laparoscopically assisted Soave’sone-stage endorectal pull-through has become the most common operation.The application of natural orifice transluminal endoscopic surgery (NOTES)makes HD operation less invasive. In this study, we retrospectively analyzedclinical data of children who underwent laparoscopy for HD and HAD in TheSecond Hospital of Hebei Medical University for three years, postoperativeoutcomes, diagnostic accuracy of barium enema and anorectal manometrywere compared. Factors which contribute to the differences were discussedand the strategies of personalized laparoscopic choice for differentpathological types of HD and HAD were explored.Methods:From January2010to December2012,90patients with HD or HADwere diagnosed and encountered various laparoscopic-assisted colon resection.There were63males and27females, and their age ranged from1month to14years old. All childreb were diagnosed confirmed by barium enema and a 24-hour residual evaluation, anoretcal manometry and histopathology. Thestatistical data were analyzed as follows:①The differences of diagnosis rateamong barium enema combined with a24-hour delayed evaluation, anoretcalmanometry and laparoscopic intraoperative inspection and biopsy for HD andHAD;②To compare the outcomes between NOTES and conventionallaparoscopic surgery. Incidence of postoperative complications, pre-andpost-operative parameters of anorectal manometry and postoperative bowelfunction recovery were also compared;③To compair the anorectal manometryparameter and the postoperative bowel function recovery between Duhamel’sprocedure and Soave’s procedure for subtotal colectomy.Results:1The diagnosis rate of barium enema was68.9%(62/90)for HD andHAD and the rate raise to82.2%(74/90)if using barium enema combined witha24-hour residual evaluation. The differences between two groups weresignificant (P=0.037). The diagnosis rate of anoretcal manometry was83.3%(75/90)， which was close to X-ray examination (P=0.844). If bothabove-mentioned methods were simultaneously applied, their diagnosis rateraised to95.6%(86/90), P=0.013. Comparing with contrast enema andanorectal manometry respectively, there were significant superiorities(P=0.004vs0.008). Diagnosis rate of laparoscopic inspection and biopsy was98.9%(89/90), it was similar to the rate of preoperative combinedexaminations (P=0.16). In HAD group, the diagnosis rate of X-rayexamination was81.8%(9/11), but the rate of anorectal manometry was36.4%(4/11), there was a significant difference (P=0.027). The laparoscopicdiagnostic rate for HAD was90.9%(10/11), there was no significantdifference compairing with X-ray examination (P=0.531).2With similar extent colectomy,60children under5years old underwentlaparoscopic endorectal pull-through (Soave’s procedure) were compared. Ofthem,27cases encountered NOTES proctosigmoidectomies and9NOTESsubtotal colectomies; while in conventional laparoscopic group,17casesunderwent rectosigmoidectomies and7subtotal colectomies. There were no differences in age, sex, weight and length of colectomy. All procedures wereperformed without intraoperative complicastions. Operative time (OT),estimated blood loss (EBL) and blow resuscitation (BR) were compared andanalyzed. Comparing to CL proctosigmoidectomies (OT135.86±6.32min,EBL20.0±9.01ml and BR1.4±0.6d), NOTES proctosigmoidectomies (OT110.87±9.11min, EBL15.93±7.47ml and BR1.44±0.75d), took much lesstime(P=0.019); whereas, no obvious OT differences (P=0.845) was observedbetween NOTES subtotal colectomies (OT182.78±44.52min, EBL28.89±9.61ml and BR1.56±0.88d) and CL subtotal colectomies (OT200.00±38.73min, EBL45.71±32.07ml and BR1.29±0.49d). Both approaches sharedsimilar blood loss and blow resuscitation. During the follow-up period, nosignificant different in incidence of postoperative complications, score ofdefecation control and the anorectal manometry parameters were found.3The incidence of postoperative complications had no significantdifference between Duhamel procedure (12.5%,1/8) and Soave procedure(17.4%,4/23) for subtotal colectomy. Postoperatively， a comparison ofDuhamel and Soave shown that the defecation frequencies of both group in1st,2nd,3rd,6th and12th month were7.75±0.50vs11.00±2.02,5.50±0.58vs7.57±1.33,3.50±0.58vs4.76±0.70and1.50±0.58vs2.71±0.46, the scores ofdefecation control were3.50±2.08vs3.38±1.36,4.50±1.73vs4.52±0.90,5.50±0.58vs5.05±0.67and6.00±0.00vs5.76±0.44. The defecationexcellent rate in3rd and6th month after subtotal colectomy were75%(6/8)and100%(5/5) in the Duhamel group.In Soave, this postoperativeparameter in3rd,6th and12th month were56.5%(13/23),78.3%(18/23) and100%(20/20) resbectively. There were no significant differences in thepostoperative anal resting pressure between both groups (P=0.643). The lengthof postoperative anal high pressure zone in Duhanmel group was shorter thanthat in Soave group (P=0.046). The rectal resting pressure were significantlydecreased in Duhanmel group (P=0.045). No differences in the rate of RAIRrecovery between both groups in postoperative6th and12th month were found,P=0.651and0.122. Conclusions:1The diagnosis rate of barium enema combined with barum residualevaluation24hours after barium enema is higher than barium enema for HDand HAD and the diagnosis rate could be further improved if the examinationof Imaging combined with anorectal manometry; The examination of bariumenema combined with barum residual evaluation24hours after barium enemahave more diagnostic value than anorectal manometry for HAD.2Comparing with CL, NOTES for HD and HAD has analogousoperative outcomes, meanwhile, NOTES can further reduce the injuries of theabdominal wall and has better cosmetic results.3In Subtotal colectomy, Duhamel procedure have shown better outcomethan Soave in defecation frequency, scores of defecation control andparameters of anorectal manometry. Postoperative diarrhea is respitedobviously in Duhamel procedure.4We provide personalized treatment plan according to the equipmentsaviliable, operator’s experience, phathological types of HD and HAD and theage of the patients, important factors we take into consideration are as follows:①laparoscope assisted completely transanal endorectal pull-through wasrecommended for the children who have the common type of HD under theage of5years, for long-segment type HD children, transumbilical single portlaparoscopy hybrid transanal endorectal pull-through was recommended, thisprocedure is less invasive, scars in the abdomen are invisible, and cosmetic;②Single incision laparoscopic Duhamel operation can be carried out on thepreceding abdominal incision to the patients with long-segment type HD whounderwent colostomy, minimized trauma and better bowel function recoverycan be expected;③Conventional laparoscopic operation is more convenientto free colon and rectum for older children.In summary, treatment of HD and HAD should be based on the variationof individual children and experiences of operators, in order to reach the bestdiagnosis and treatment outcomes, individualized diagnosis and treatmentstrategies should be selected to cure the disease thoroughly with minimized trauma and pain.