Gastrointestinal Surgery ConferenceScott NguyenEnglewood HospitalMay 21, 2003

Patient S.C.Newborn maleFull-term, uncomplicated vaginal deliveryNormal birth weight: 3115 gApgars 91, 95Mother: 36 yo, G1P0, healthy

Patient S.C.Started breast feeding DOL 1DOL 2-3 noted to have increasing abdominal distentionNo meconium passed in first 24 hrs of life1 episode Non-bilious emesis

Patient S.C.

Patient S.C.Pediatric Surgical ConsultRectal ExamEmpty rectal ampullaTight anal sphincterLarge amount of stool and air upon withdrawal of finger

Patient S.C.

Patient S.C.Rectal mucosal biopsyNo ganglia identified

Patient S.C.

Patient S.C.Pt taken to OR for end colostomy and Hartmanns pouchDilated descending and sigmoid colonProminent colonic blood vesselsSite of colostomy, frozen section of colonic muscularis propria revealed ganglion cells

Patient S.C.

Patient S.C.Postoperative course uneventfulStool from colostomy POD 1Tolerated breast feedingDischarged POD 62nd stage pull through procedure planned in several weeks

Hirschsprungs DiseaseScott NguyenEnglewood HospitalMay 21, 2003

Hirschsprungs DiseaseNeurogenic form of intestinal obstruction Absence of ganglion cells in the myenteric and submucosal plexusFailure in relaxation of the internal anal sphincter and affected bowelUpstream bowel becomes dilated secondary to functional obstruction

History1691 Ruysch latin texts1886 Harald Hirschsprung autopsy 1901 Tittel histologic findings1949 Swenson pathophysiology and definitive operative treatment

Epidemiology

Prevalence: 1/5000 births3-5% of pts have Downs syndromeDefinite family history80% affected are boysTotal colonic aganglionosis, 35% girls>95% cases are full term babies

Pathogenesis

Pathogenesis

Failure of neural crest cells to migrate caudallyAganglionosis begins at anorectal line80% involve only rectosigmoid area10% extend proximal to splenic flexure10% involves the entire colon and part of small bowelRarely involves entire gastrointestinal tract

Pathogenesisgenetics

10th chromosome RET-protooncogeneEndothelin B gene

Presentation

Presentation

Severe abdominal distention 95% - failure to pass meconium in first 24 hours lifeBilious vomiting Older children - constipation, failure to thrive10-15% - severe diarrhea alternating w/ constipationenterocolitis of Hirschsprungs disease

DiagnosisAbdominal plain X-raysBarium EnemaRectal BiopsiesAnal manometry

Abdominal X-ray

Barium Enema

Barium EnemaLess sensitive for detecting short lesions, total colon aganglionosis, and disease of the newbornMany newborns do NOT show definitive transition zoneDelayed evacuation of contrast

Rectal biopsy

Submucosal suction biopsy Meissners submucosal plexusFull thickness rectal biopsyAuerbachs myenteric plexusAcetylcholinesterase staining increased staining of neurofibrils

Anorectal manometry

Absent rectoanal inhibitory reflexLack of internal anal sphincter relaxation in response to rectal stretch

Surgical Options

Swenson Procedure (1948)Duhamel Procedure (1960)Soave Procedure (1963)

Swenson Procedure

Sharp extrarectal dissection down to 2 cm above the anal canalAganglionic colonic segment resectedEnd-to-end anastamosis of normal proximal colon to anal canalCompletely removes defective aganglionic colon

Swenson Procedure

Duhamel Procedure Posterior portion of defective colon segment resectedSide to side anastamosis to left over portion of rectumConstipation a major problem d/t remaining aganglionic tissueSimpler operation, less dissection

Duhamel Procedure

Soave ProcedureCircumferential cut through muscular coat of colon at peritoneal reflectionMucosa separated from the muscular coat down to the anal canalProximal normal colon is pulled through retained muscular sleeveTelescoping anastamosis of normal colon to anal canal

Soave Procedure

Soave ProcedureAdvantage: rectal intramural dissection ensures no damage to pelvic neural structures Higher rate enterocolitis, diarrheaProblems w/ cuff abscesses, often requires repeated dilations

Overall Mortality

Swenson procedure: 1-5%Duhamel procedure: 6%Soave procedure: 4-5%

Operative complications

Leak at anastamosis: 5-7%Postop Enterocolitis: 19-27% Constipation Stricture FormationIncontinence

One vs Two Stage procedure

Historically, two stage procedure performed: preliminary colostomy, then completion pull through Delicate muscular sphincters of newborn may be injured1980s, 1 stage procedures became more popular

One vs Two Stage procedureEarly complications: No difference in incidence of anastomotic leak, pelvic infection, prolonged ileus, wound infection, wound dehiscence Late complications: No difference in incidence of anastomonic stricture, late obstruction, constipation, incontinence, urgencyPostoperative enterocolitis higher in 1 stage (42% vs 22%)

Laparoscopic techniques

Small studies of laparoscopic pull through proceduresExcised aganglionic tissues removed through anal canal, no abdominal incisionBetter results in terms of pain, return of bowel function, hospital staySimilar incidence of leaks, pelvic abscesses, enterocolitis, postop bowel function

Dilated small and large bowel loops, prominent transverse, descending, sigmoidNarrow rectum, dilated sigmoid colon, normal appearing remaining large bowel.Minimal residual contrast left in colon