Hirschsprungs Disease: Options for Surgical CorrectionGeorge W. Holcomb, III, M.D., MBAChildrens Mercy HospitalKansas City, Missouri

Hirschsprungs DiseasePrinciplesBarium enema for suspected diseaseAnorectal biopsy for confirmation of diagnosisSelection of operation based on extent of disease need biopsy of ganglionated colon

Hirschsprungs Disease

Colostomy at diagnosisPullthrough 6 - 12 months laterDuhamelSoaveSwensonHistorical Approach

Hirschsprungs DiseaseCurrent Surgical OptionsOne-stageSwensonDuhamelSoaveLaparoscopic-assistedTransanalTwo-stageSwensonDuhamelSoave(Laparoscopic-assisted)(Transanal)

Hirschsprungs DiseaseOne stage operation for rectosigmoid disease is current preferred approachLaparoscopic-assisted or transanal approach best suited for typical rectosigmoid diseaseUnclear (in my mind) whether one or two stage procedure is best for disease proximal to splenic flexure

Hirschsprungs DiseaseInitial trans-abdominal biopsy with frozen section is important in determining what surgical option is best for each patientBiopsy can be performed through umbilicus easily

Hirschsprungs DiseaseExtent of DiseaseOptimal Surgical OptionRectosigmoidLaparoscopic-assisted ortransanalDescending colonLaparoscopic-assistedTransverse colonTwo-stage approachRt colon; ileumTwo-stage approach Ileal pullthrough(? Laparoscopic)

Hirschsprungs DiseasePersonal ApproachLaparoscopic directed biopsy thru umbilicusFor rectosigmoid disease, laparoscopic assisted pullthroughTension free relocation of ganglionic colon to anusAlready in abdomen for laparoscopic directed biopsy

Patient PositioningBaby across O.R. tableCircumferential, full body prepUrinary catheter

Port PositioningPort placement5 mm umbilical5 mm RUQ 5 mm RLQ3 mm (optional) LUQ

TechniqueLigation of mesenteric vessels

TechniqueMobilization of rectum

TechniqueMobilization of rectum

TechniqueMobilization of left colon

Laparoscopic PullthroughLaparoscopic mobilization of recto-sigmoid vasculatureExtracorporeal endorectral dissection

Transanal Dissection

Transanal Dissection

Transanal Dissection

TechniqueCompleted pullthrough

Laparoscopic PullthroughPrimary Procedure - No Colostomy

Transanal ApproachBest suited for low rectosigmoid diseaseConcern is tension on vasculature as it is brought to anusAppropriate technique if transabdominal biopsy is performed to define extent of diseaseIf biopsy done, why not mobilize laparoscopically?If no biopsy, must be ready to do transverse colon or ileal pullthrough in newborn period

Hirschsprungs DiseasePrimary PullthroughObviates need for colostomy careSecond operation and hospitalization not requiredNormal bowel function established earlyReduced cost of care

Childrens Mercy HospitalResults2000 - 200115 Pts Mean # Pts Age (Wk) Wt(kg)7 366.41 (1 - 138)(2.7 - 11.5)

8 277.24 (12 - 54)(5.3 - 8.1)Primary Lap Pullthrough

Colostomy + Open Pullthrough

Childrens Mercy HospitalResultsTotal Postop TotalHospitalization(D)Cost ($)

3.7$38,489 (3-7)(21,040 - 71,034)

3.4 + 3.5$70,858(2-19) (2-4)(20,087 - 165,642)Primary Lap PullthroughColostomy + Open Pullthrough

Hirschsprungs DiseasePrimary PullthroughOpen+ transanal1990sAllows biopsyGood resultsLaparoscopy + transanal Allows biopsy Mobilization of rectum, sigmoid, left colon Good early resultsTransanal approach alone No ABD explorationPt. selection very important Good early results

Postoperative CareBiggest problem is enterocolitis

Postoperative ManagementAnorectal dilationsBegin 3rd week, Hegar 8 BIDAdvance to Hegar 14 QD, then QOD, then Q3D, up to Q weekFlagyl/anorectal irrigations if develop enterocolitisPersonal Experience (50 cases/7 yrs)All well except 2 with colostomy (one closed)

SummaryFunctional results following pullthrough are probably similar b/w approachesPatient selection important for primary pullthrough regardless of approachLaparoscopic one stage approach affords good results, has less total hospitalization and is less costly than two stage approach

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