A Theme from the Bottom:
Modern Surgical Management of
Pediatric Colorectal Disorders
Jason C. Fisher, MD
Assistant Professor of Surgery
NYU School of Medicine
Joseph M. Sanzari Children’s Hospital
Hackensack University Medical Center
From the Top to the “Bottom”
• Defining pediatric colorectal disorders
• The pediatrician’s role in workup
• Disease highlights
• Post-operative & long-term considerations
Anorectal Malformations
Hirschsprung’s Disease
Inflammatory Bowel Disease
Idiopathic Constipation
From the Top to the “Bottom”
• Defining pediatric colorectal disorders
• The pediatrician’s role in workup
• Disease highlights
• Post-operative & long-term considerations
Pediatrician’s Workup:
Anorectal Malformations
• Are there other anomalies?
• Is this baby safe to operate on?
• How quickly do we need the OR?
Pediatrician’s Workup:
Anorectal Malformations
• R enal Ultrasound
• E chocardiogram
• P ass an NGT into stomach
• A bdominal X-ray
• S acral X-ray
• S pinal Ultrasound
Pediatrician’s Workup:
Anorectal Malformations
• Can you see meconium?
• How distended is the baby?
• Who are your surgeons?
Pediatrician’s Workup:
Anorectal Malformations
vs
The Surgeon & Pediatrician As Team:
Anorectal Malformations
Scenario A
Scenario B
Scenario C
Pediatrician
Observation
• No other anomaly
• I can see poop !!!
• Abdomen is soft
• No other anomaly
• I see no poop
• Abdomen is soft
• Major anomaly
• I see no poop, or…
• Distended abdomen
Surgeon
Observation
• Perineal fistula
• Vestibular fistula
• No perforation risk
• Bulbar / Prostatic
• Bladder-neck fistula
• Cloaca (1-hole)
• No perforation risk
• Bulbar / Prostatic
• Bladder-neck fistula
• Cloaca (1-hole)
• Risk of perforation
Team
Management
Pathway
• Establish IV access
• Establish IV access
• Begin dilations, or…
• NGT decompression
• Invertogram
• Colostomy + MF
• Primary Anoplasty
• Establish IV access
• NGT decompression
• Treat comorbidities
• Urgent Colostomy
Pediatrician’s Workup:
Hirschsprung’s Disease
• Did this baby poop in first 48 hrs?
• Reliant on rectal stimulation?
• Episodes of fever / irritability?
Pediatrician’s Workup:
Hirschsprung’s Disease
• Avoid Rectal Stimulation
• Abdominal X-ray
• Contrast Enema
• Colonic Irrigations
• Oral Flagyl
Pediatrician’s Workup:
Hirschsprung’s Disease
• Is this baby sick ?
• Enterocolitis can be lethal
• A rectal exam can save a life !!
The Surgeon & Pediatrician As Team:
Hirschsprung’s Disease
Scenario A
Scenario B
Scenario C
Pediatrician
Observation
• Healthy baby
• Delayed meconium
• Soft but distended
• Sick baby
• Explosive & stinky
• Distended, unhappy
• Older child
• Chronic constipation
• Soft but distended
Surgeon
Observation
• Normal anatomy
• Distal obstruction
• No enterocolitis
• Normal anatomy
• Distal obstruction
• Enterocolitis
• Normal anatomy
• No enterocolitis
• No rectal stim
• Contrast enema
• Suction Rectal Bx
• Abx, IVF, rectal exam
• Flagyl + Irrigations
• Contrast enema
• Emergent colostomy
• or Suction Rectal Bx
• Thorough re-eval
• Contrast enema
• Rectal EUA
• Full-thickness Bx
Team
Management
Pathway
Pediatrician’s Workup:
Inflammatory Bowel Disease
• Have we determined UC vs Crohns?
• Patient compliant with GI visits ?
• Sx progressing despite meds ?
Pediatrician’s Workup:
Inflammatory Bowel Disease
• Any peri-anal disease (CD)
• Intermittent obstructive Sx (CD)
• Hospital for pain/abscess (CD)
• Worsening bloody stools (UC)
• Poor nutrition/weight loss (Both)
• Medication side effects (Both)
Pediatrician’s Workup:
Inflammatory Bowel Disease
• Detailed timeline of meds and interventions
• Slow decompensation vs severe
acute-on-chronic presentation
• Parent / Patient perceptions of
their disease status
• Who is your surgeon?
Pediatrician’s Workup:
Inflammatory Bowel Disease
vs
The Surgeon & Pediatrician As Team:
Inflammatory Bowel Disease
Scenario A
Scenario B
Scenario C
Pediatrician
Observation
• Progressive decline
• Failure to thrive
• Family frustration
• GI care optimized
• Acute on chronic
• Dz controlled
• Non-toxic patient
• Dx uncertain
• Acute on chronic
• Any Dz status
• Toxic patient
Surgeon
Observation
• Recurrent abscesses
• Progressive wt loss
• Refractory colitis
• Peri-anal disease
• First hospitalization
• Stricture or abscess
• Pathology unclear
• Toxic megacolon
• Perforation
• Severe obstruction
• Major hemorrhage
Team
Management
Pathway
• Peds GI opinion
• Nutrition evaluation
• CT/MR enterography
• Role of laparoscopy
• Peds GI opinion
• Verify optimal meds
• Rectal EUA
• Laparoscopy + Endo
• Critical care resusc
• Stabilization
• Emergent exploration
• Guided by next step
Pediatrician’s Workup:
Idiopathic Constipation
• Truly idiopathic process ?
• Hirschsprung’s excluded ?
• Underlying genetic disease ?
Pediatrician’s Workup:
Idiopathic Constipation
• Abdominal X-ray
• Contrast enema
• Colonoscopy
• Peds GI recommendations
• Dietary modifications
• Informal bowel regimen
Pediatrician’s Workup:
Idiopathic Constipation
• Anatomic vs Functional?
• Role of underlying disease
• Patient / family perceptions
and compliance
The Surgeon & Pediatrician As Team:
Idiopathic Constipation
Scenario A
Scenario B
Scenario C
Pediatrician
Observation
• Chronic constipation
• Chronic constipation
• Chronic constipation
• Neonatal Hx unclear
• Genetic Dz
• Hx Hirschprung/ARM
• No underlying dz
• Noncompliant pt
Surgeon
Observation
• No anatomic defect
• No prior operations
Team
Management
Pathway
• Peds GI consultation
• Contrast enema
• Contrast enema
• Rectal EUA +/- Bx
• Colonoscopy
• Surgical revision prn
• Rectal EUA + Biopsy
• Review OR records
• Correct initial surgery
• Complications of
constipation
• Megarectum
• Peds GI consultation
• Contrast enema
• Colonoscopy
• Address complications
• Formal bowel mgmnt
From the Top to the “Bottom”
• Defining pediatric colorectal disorders
• The pediatrician’s role in workup
• Disease highlights
• Post-operative & long-term considerations
Surgical Highlights:
“Low” Anorectal Malformations
Traditional Operative Strategies
• Dilation as definitive therapy
• Primary “cut-back” anoplasty
Pros
Cons
Quick
Safe
Technically Easy
Anus not in sphincter
Require additional surgery
Long term stooling issues
Surgical Highlights:
“Low” Anorectal Malformations
Surgical Highlights:
Anorectal Malformations
Surgical Highlights:
“Low” Anorectal Malformations
Modern Operative Strategies
• Primary Definitive Anoplasty in Newborn
Pros
Cons
Anus placed in sphincter
Single operation
Technically challenging
Slightly ↑ infection risk
Surgical Highlights:
“High” Anorectal Malformations
Traditional Operative Strategies
• Newborn colostomy creation
• Delayed PSARP
Pros
Cons
Safe
Deep, blind dissection
May require laparotomy
Surgical Highlights:
“High” Anorectal Malformations
Modern Operative Strategies
• Laparoscopic-assisted PSARP
Pros
Cons
Excellent visualization
Small perineal incision
Technically challenging
Limited to high ARM’s
Surgical Highlights:
Hirschsprung’s Disease
Traditional Operative Strategies
• Staged approach  leveling colostomy
• Laparotomy with endorectal pull-through
Pros
Cons
Quick initial operation
Technically easy
2 or 3 surgeries needed
Laparotomy
Surgical Highlights:
Hirschsprung’s Disease
Modern Operative Strategies
• Single operation, transanal approach
• Laparoscopic-assisted mobilization as needed
Pros
Cons
Single operation
No abdominal incisions
Requires expert pediatric
radiology & pathology
Medical Highlights:
Inflammatory Bowel Disease
• Serology Testing
• Step-Up vs Step-Down
• Nutrition as Therapy
Medical Highlights:
IBD – Serology Testing
• Why do we pursue a blood-test for IBD ?
– Differentiate IBD from other functional bowel disease
– Make Dx in cases of severe / indeterminate colitis
– Predict disease phenotype or complication risk
Medical Highlights:
IBD – Serology Testing
• Have we succeeded ???
… sort-of
– Prometheus® IBD Serology 7
• 5 ELISAs (ASCA IgA, ASCA IgG, ANCA, CBir-1, OmpC)
• 2 pANCA Immunofluorescence Tests
Diagnosis
Predictive
values
85% Pre-test 15% Pre-test
Probability
Probability
IBD
PPV
NPV
98%
60%
68%
98%
CD
PPV
NPV
99%
50%
81%
98%
UC
PPV
NPV
99%
64%
82%
99%
Medical Highlights:
IBD – Serology Testing
• The current state of serology testing:
– Certain profiles are specific to UC versus CD
– Unable to diagnose UC or CD in indeterminate colitis
– Certain patterns correlate with Crohn’s phenotypes
– Certain patterns correlate with surgical complications
– Few are using serology to prospectively guide therapy
– May not be covered by insurance
Medical Highlights:
IBD – Step Up vs. Step Down
• Current therapeutic pyramid for Crohn’s Disease
Severe
Surgery
anti-TNFα
Moderate
MTX
AZA / 6-MP
Systemic steroids
Mild
Budesonide
Antibiotics
5-ASA
Medical Highlights:
IBD – Step Up vs. Step Down
• Early use of biological therapies in Crohn’s
– Better targeting of inflammatory response mediators
– Can treat active disease and maintain remission
– Highly effective for management of fistulae
– May alter long-term course (disease modification)
Medical Highlights:
IBD – Step Up vs. Step Down
• Remicaide ™ (Infliximab): Anti-TNFα
10 wks
Before Remicaide
After Remicaide
Rutgeerts P, et al Gastro Endoscopy 2006
Medical Highlights:
IBD – Step Up vs. Step Down
Anti TNF
Anti TNF
AZA/MTX
AZA/MTX
Combination
Steroids
Steroids
5-ASA/SPS
Step-Up
Therapy
Top-Down
Therapy
Lichtenstein GR et al. Inflamm Bowel Dis. 2004
Medical Highlights:
IBD – Step Up vs. Step Down
• Multiple trials testing efficacy & safety
– PRECiSE 1/2, CHARM, ACCENT 1/2, SONIC, TREAT
Favors Step Down
Against Step Down
Less clinical relapse
Less steroid use
Overtreatment in about 30%
Better mucosal healing
Long-term safety concerns
Fewer strictures
Benefit reduced if maintenance?
Fewer hospitalizations
Fewer surgeries
Medical Highlights:
IBD – Nutrition as Therapy
• Drivers of malnutrition leading to growth failure
–
–
–
–
–
Inadequate caloric intake
Increased energy expenditure
Enteric losses / malabsorption
Steroid therapy
Inflammation / disease activity
Medical Highlights:
IBD – Nutrition as Therapy
• Providing nutritional therapy via NG feeding
–
–
–
–
–
Increased caloric intake
Anti-inflammatory properties
Immune-modulating effects of fatty-acids
Modification of intestinal flora
May be as effective as steroids
in inducing remission
Heuschkel RB, et al. J Pediatr Gastroenterol Nutr 2000
Medical Highlights:
IBD – Nutrition as Therapy
• Why isn’t nutritional therapy used more often?
– Bias, cost, and noncompliance
– Fear of parental acceptance
• Can be utilized in all stages of disease
–
–
–
–
Provide 80 – 100% of nutrition via NGT overnight
Use of elemental formula (Optimental, Peptamin AF)
Option of taking NGT out during day
Regular diet as tolerated during day
Surgical Highlights:
Laparoscopy in Ulcerative Colitis
• Emergent operations  3 stage approach
– Toxic megacolon / fulminant colitis
– Concurrent high-dose steroids
– Acute on chronic colonic hemorrhage
Surgical Highlights:
Laparoscopy in Ulcerative Colitis
• Elective operations  2 or 1 stage approach
– Decide whether protective ileostomy is needed
– Completely laparoscopic approach
– Largest incision is the stoma site
From the Top to the “Bottom”
• Defining pediatric colorectal disorders
• The pediatrician’s role in workup
• Disease highlights
• Post-operative & long-term considerations
Postoperative Considerations:
Anorectal Malformations
• Maintaining the anoplasty
• Managing constipation
• Addressing any urologic issues
Postoperative Considerations:
Anorectal Malformations
• Confirm that parents are doing BID dilations
Age
Hegar Dilator
1 – 4 months
#12
4 – 8 months
#13
8 – 12 months
#14
1 – 3 years
#15
3 – 12 years
#16
> 12 years
#17
Once a given size
passes easy and
with no pain
• Q-Day x 1 month
• QOD x 1 month
• BIW x 1 month
• Q-Week x 1 month
•Q-month x 3 months
Postoperative Considerations:
Anorectal Malformations
• All ARM pt’s have some degree of constipation
– Must be pro-actively managed in all patients
– Distinguish between mild versus complicated cases
– Ask about episodes of soiling or incontinence
– Low threshold for early referral back to surgeon
Postoperative Considerations:
Anorectal Malformations
• Frequency of urologic anomalies = 50–60%
• Urologic injuries can occur
Reflux
Ectopic kidney
Hydronephrosis
Megacystis
Renal agenesis
Neurogenic bladder
Megaureter
Urethral stenosis
Renal dysplasia
UV obstruction
Ectopic ureter
• May require vesicostomy or CIC regimens
Postoperative Considerations:
Hirschsprung’s Disease
• Be wary of enterocolitis… always!
• Know how to perform a colonic irrigation
• Identify a child “not doing well”
Postoperative Considerations:
Hirschprung’s Disease
• Remain at life-long risk for enterocolitis
– Even after successful resection, entercolitis can occur
– Triggered by fecal stasis, compounded by infection
– Low threshold for rectal exam, irrigation, and flagyl
Postoperative Considerations:
Hirschprung’s Disease
• What is a colonic irrigation? …it’s not an enema
Enema
• Technique used for constipation
• Large volume of irritant solution
• Colon contracts
• Colon expunges the contents
Irrigation
• Technique used to decompress stasis
• Insert catheter & drain all liquid & gas
• Inject 10-15 ml saline at a time
• Manually drain contents with syringe
• Repeat this maneuver many times
Postoperative Considerations:
Hirschprung’s Disease
• The post-pull through patient not doing well…
–
–
–
–
Child continues to have soiling episodes
Recurrent episodes of enterocolitis
Progressive abdominal distention
Generalized failure to thrive
Comprehensive Diagnostic
Algorithm
• Re-evaluate pathology
• Verify surgical anatomy
• Assess bowel control & motility
Postoperative Considerations:
Inflammatory Bowel Disease
• J-pouch sequelae  loose stools & pouchitis
• Lifelong rectal surveillance begins
in the pediatrician’s office
• Localize recurrent disease flares
Postoperative Considerations:
Inflammatory Bowel Disease
• Frequent stools after J-pouch reconstruction (UC)
– Can exceed 6 – 8 loose bowel movements per day
– Time and intestinal adaptation provide some relief
– Important that patients understand this preop
– Primarily managed with diet and motility agents
• Pouchitis  50-60%, inflammation & infection
– Associated with metaplasia of pouch mucosa
– Presents with pain, tenesmus, increased stooling
– Treated with Flagyl  refer for surgical f/u with EUA
Postoperative Considerations:
Inflammatory Bowel Disease
Cumulative
Probability
of Cancer (%)
Years after Diagnosis
Postoperative Considerations:
Inflammatory Bowel Disease
• Pinpointing recurrent Crohn’s Disease flares
– We have limited ability to predict Crohn’s recurrences
– 1 year postop  93% have endoscopic recurrence
37% have symptoms
– 3 years postop  100% have endoscopic recurrence
86% have symptoms
– Histologic change seen on normal-appearing mucosa
Histologic ≠ Endoscopic ≠ Clinical
Olaison, et al, Gut, 1992
Rutgeerts, et al. Gastroenterology 1990;99:956
Postoperative Considerations:
Inflammatory Bowel Disease
• Pinpointing recurrent Crohn’s Disease flares
– Fluroscopic contrast studies  laborious, inaccurate
– CTAP  radiation, poor small bowel resolution
– CT / MR Enterography  an emerging new standard
Cross-Sectional Enterography
Uniform low-density oral contrast + IV
• Ultra-thin slice acquisition (CT)
• Protocols to assess lumen & mucosa
• Diminished or no radiation dosing
•
Postoperative Considerations:
Inflammatory Bowel Disease
• Confirms whether SB inflammation is present
• Distinguishes inflammation, fistula, abscess and stricture
Postoperative Considerations:
Final Thoughts…
Incredibly challenging for any single
provider to offer the best possible
treatment options to complex
pediatric colorectal patients
Complex Colorectal Patients
Fragmented & Decentralized Care
• Postoperative care deserves the same level of
attention, focus and expertise as surgery itself
• Families are mobile  regardless of where surgery
was done, we should be able to help these children
• Quality of life and family perceptions are often
overlooked in the treatment plans of providers
• Referrals to many doctors creates disorganized care
• Negative stigma which can create barriers to care
Complex Colorectal Patients
A Multidisciplinary Approach
Hirschprung’s Patient “Not Doing Well”
Urologic Problems in Anorectal Malformations
Refractory Constipation in Anorectal Malformation
Pouchitis / Excessive Stools after J-Pouch Surgery
Complicated / Recurrent Crohn’s Disease
Surveillance and Surgery for Familial Polyposis
Refractory Idiopathic Constipation or Soiling
Hirschprung’s Pt Not Doing Well
A Multidisciplinary Approach
Enterocolitis
Distention
Failure to thrive
Soiling
Are anal canal & sphincters intact?
Potential for bowel control?
Yes
Medical
Treatment
No
Enemas
Anatomic
problem
• Stricture
Pathologic
problem
• Aganglionic
pull through
• Duhamel pouch
Hypermotile Hypomotile
Loperamide
Constipating diet
Pectin
Laxatives
Loperamide,
constipating diet &
pectin if indicated
• Soave cuff
• Dilated retained
distal segment
• Kink or twist of
pull through
• Transition zone
pull through
Idiopathic Constipation
A Multidisciplinary Approach
Surgery
Radiology
Pathology
Peds GI
SW / NP
Nutrition
Review
previous
operations
Contrast
Enema
Evaluate
previous
biopsies
Motility
evaluation
Assess
Family
resources
Assess
current
status
Integrity of
anal canal &
sphincters
Daily AXR
during
Enema trial
Interpret
additional
specimens
Endoscopic
evaluation
Coordinate
home
treatments
Determine
need for
supplement
Biopsy or
colonic
resection
Assess for
other
mucosal dz
Readily
available
contact
Oversee
constipating
diets
Antegrade
continent
enemas
Formulate
treatment
enemas
Daily
treatment
adjustments
Titrate
Pectin /
Lomotil