1. Gastrointestinal Surgery Conference Scott Nguyen Englewood Hospital May 21, 2003

2. Patient S.C. Newborn male Full-term, uncomplicated vaginal delivery Normal birth weight: 3115 g Apgars 9 1, 9 5 Mother: 36 yo, G 1 P 0, healthy

3. Patient S.C. Started breast feeding DOL 1 DOL 2-3 noted to have increasing abdominal distention No meconium passed in first 24 hrs of life 1 episode Non-bilious emesis

4. Patient S.C.

5. Pediatric Surgical Consult Rectal Exam –Empty rectal ampulla –Tight anal sphincter –Large amount of stool and air upon withdrawal of finger

6. Patient S.C.

7. Rectal mucosal biopsy –No ganglia identified

8. Patient S.C.

9. Pt taken to OR for end colostomy and Hartmann’s pouch Dilated descending and sigmoid colon Prominent colonic blood vessels Site of colostomy, frozen section of colonic muscularis propria revealed ganglion cells

10. Patient S.C.

11. Postoperative course uneventful Stool from colostomy POD 1 Tolerated breast feeding Discharged POD 6 2 nd stage pull through procedure planned in several weeks

12. Hirschsprung’s Disease Scott Nguyen Englewood Hospital May 21, 2003

13. Hirschsprung’s Disease Neurogenic form of intestinal obstruction Absence of ganglion cells in the myenteric and submucosal plexus Failure in relaxation of the internal anal sphincter and affected bowel Upstream bowel becomes dilated secondary to functional obstruction

14. History 1691 Ruysch latin texts 1886 Harald Hirschsprung – autopsy 1901 Tittel – histologic findings 1949 Swenson – pathophysiology and definitive operative treatment

15. Epidemiology Prevalence: 1/5000 births 3-5% of pts have Down’s syndrome Definite family history 80% affected are boys Total colonic aganglionosis, 35% girls >95% cases are full term babies

16. Pathogenesis

17. Failure of neural crest cells to migrate caudally Aganglionosis begins at anorectal line 80% involve only rectosigmoid area 10% extend proximal to splenic flexure 10% involves the entire colon and part of small bowel Rarely involves entire gastrointestinal tract

18. Pathogenesis—genetics 10th chromosome RET-protooncogene Endothelin B gene

19. Presentation

20. Severe abdominal distention 95% - failure to pass meconium in first 24 hours life Bilious vomiting Older children - constipation, failure to thrive 10-15% - severe diarrhea alternating w/ constipation—enterocolitis of Hirschsprung’s disease

21. Diagnosis Abdominal plain X-rays Barium Enema Rectal Biopsies Anal manometry

22. Abdominal X-ray

23. Barium Enema

24. Less sensitive for detecting short lesions, total colon aganglionosis, and disease of the newborn Many newborns do NOT show definitive transition zone Delayed evacuation of contrast

25. Rectal biopsy Submucosal suction biopsy –Meissner’s submucosal plexus Full thickness rectal biopsy –Auerbach’s myenteric plexus Acetylcholinesterase staining –increased staining of neurofibrils

26. Anorectal manometry Absent rectoanal inhibitory reflex Lack of internal anal sphincter relaxation in response to rectal stretch

27. Surgical Options Swenson Procedure (1948) Duhamel Procedure (1960) Soave Procedure (1963)

28. Swenson Procedure Sharp extrarectal dissection down to 2 cm above the anal canal Aganglionic colonic segment resected End-to-end anastamosis of normal proximal colon to anal canal Completely removes defective aganglionic colon

29. Swenson Procedure

30. Duhamel Procedure Posterior portion of defective colon segment resected Side to side anastamosis to left over portion of rectum Constipation a major problem d/t remaining aganglionic tissue Simpler operation, less dissection

31. Duhamel Procedure

32. Soave Procedure Circumferential cut through muscular coat of colon at peritoneal reflection Mucosa separated from the muscular coat down to the anal canal Proximal normal colon is pulled through retained muscular sleeve Telescoping anastamosis of normal colon to anal canal

33. Soave Procedure

34. Advantage: rectal intramural dissection ensures no damage to pelvic neural structures Higher rate enterocolitis, diarrhea Problems w/ cuff abscesses, often requires repeated dilations

35. Overall Mortality Swenson procedure: 1-5% Duhamel procedure: 6% Soave procedure: 4-5%

36. Operative complications Leak at anastamosis: 5-7% Postop Enterocolitis: 19-27% Constipation Stricture Formation Incontinence

37. One vs Two Stage procedure Historically, two stage procedure performed: preliminary colostomy, then completion pull through Delicate muscular sphincters of newborn may be injured 1980s, 1 stage procedures became more popular

38. One vs Two Stage procedure –Early 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, urgency –Postoperative enterocolitis higher in 1 stage (42% vs 22%)

39. Laparoscopic techniques Small studies of laparoscopic pull through procedures Excised aganglionic tissues removed through anal canal, no abdominal incision Better results in terms of pain, return of bowel function, hospital stay Similar incidence of leaks, pelvic abscesses, enterocolitis, postop bowel function