Hirschsprungs disease
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Hirschsprung’s Disease
R.K.BagdiSenior Consultant
Apollo Children;s Hospital
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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 Hartmann’s 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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Hirschsprung’s Disease
R.K.Bagdi
Apollo Children’s Hospital
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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
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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 Down’s syndrome
• Definite family history
• 80% affected are boys
• Total colonic aganglionosis, 35% girls
• >95% 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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Pathogenesis—genetics
• 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/
constipation—enterocolitis of Hirschsprung’s 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
– Meissner’s submucosal plexus
• Full thickness rectal biopsy
– Auerbach’s 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