Gastrointestinal Surgery Conference

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Gastrointestinal Surgery Conference

• Scott Nguyen
• Englewood Hospital
• May 21, 2003

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Patient S.C.

• Newborn male
• Full-term, uncomplicated vaginal delivery
• Normal birth weight 3115 g
• Apgars 91, 95
• Mother 36 yo, G1P0, healthy

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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

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Patient S.C.
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Patient S.C.

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

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Patient S.C.
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Patient S.C.

• Rectal mucosal biopsy
• No ganglia identified

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Patient S.C.
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Patient S.C.

• Pt taken to OR for end colostomy and Hartmanns
  pouch
• Dilated descending and sigmoid colon
• Prominent colonic blood vessels
• Site of colostomy, frozen section of colonic
  muscularis propria revealed ganglion cells

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Patient S.C.
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Patient S.C.

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

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Hirschsprungs Disease

• Scott Nguyen
• Englewood Hospital
• May 21, 2003

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Hirschsprungs 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

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History

• 1691 Ruysch latin texts
• 1886 Harald Hirschsprung autopsy
• 1901 Tittel histologic findings
• 1949 Swenson pathophysiology and definitive
  operative treatment

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Epidemiology

• Prevalence 1/5000 births
• 3-5 of pts have Downs syndrome
• Definite family history
• 80 affected are boys
• Total colonic aganglionosis, 35 girls
• gt95 cases are full term babies

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Pathogenesis
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Pathogenesis

• 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

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Pathogenesisgenetics

• 10th chromosome
• RET-protooncogene
• Endothelin B gene

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Presentation
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Presentation

• 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/
  constipationenterocolitis of Hirschsprungs
  disease

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Diagnosis

• Abdominal plain X-rays
• Barium Enema
• Rectal Biopsies
• Anal manometry

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Abdominal X-ray
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Barium Enema
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Barium Enema

• 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

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Rectal biopsy

• Submucosal suction biopsy
• Meissners submucosal plexus
• Full thickness rectal biopsy
• Auerbachs myenteric plexus
• Acetylcholinesterase staining
• increased staining of neurofibrils

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Anorectal manometry

• Absent rectoanal inhibitory reflex
• Lack of internal anal sphincter relaxation in
  response to rectal stretch

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Surgical Options

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

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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

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Swenson Procedure
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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

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Duhamel Procedure
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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

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Soave Procedure
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Soave Procedure

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

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Overall Mortality

• Swenson procedure 1-5
• Duhamel procedure 6
• Soave procedure 4-5

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Operative complications

• Leak at anastamosis 5-7
• Postop Enterocolitis 19-27
• Constipation
• Stricture Formation
• Incontinence

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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

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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)

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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