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Functional Disorders of the Ileal Pouch
Functional Disorders of the Ileal Pouch
Bo Shen, MDThe Cleveland Clinic Foundation
Cleveland, OH
December 4, 2009
Bo Shen, MDThe Cleveland Clinic Foundation
Cleveland, OH
December 4, 2009
Imaging Quiz
§ IUD§ Bladder stones§ Jewelry smuggler§ Atherosclerotic plaques§ ?
§ IUD§ Bladder stones§ Jewelry smuggler§ Atherosclerotic plaques§ ?
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Anatomy of Pelvic PouchesAnatomy of Pelvic Pouches“J” “S” “W”
Tip of “J”Tip of “J”
Afferent limb (neo-TI)Afferent limb (neo-TI)
Efferent limb Efferent limb
Outlet/cuffOutlet/cuff
InletInlet
Efferent limb
Efferent limb
Pelvic J PouchPelvic J Pouch
J, S, K Pouches vs. IleostomyJ, S, K Pouches vs. Ileostomy
Kock PouchKock PouchBrooke IleostomyBrooke Ileostomy
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Ileal Pouch Disorders and Associated ComplicationsIleal Pouch Disorders and Associated Complications
FunctionalSurgical/Mechanical
Inflammatory/Infectious
- Anastomotic leaks- Pelvic sepsis- Pouch sinuses- Pouch fistulae- Strictures- Afferent limb syn.- Efferent limb syn.- Infecundity- Sexualdysfunction
- Portal vein thrombi- Pouch prolapse- Foreign bodies
- Anastomotic leaks- Pelvic sepsis- Pouch sinuses- Pouch fistulae- Strictures- Afferent limb syn.- Efferent limb syn.- Infecundity- Sexualdysfunction
- Portal vein thrombi- Pouch prolapse- Foreign bodies
- Pouchitis- Cuffitis- Crohn’s dis.- Smallbowel bacterialovergrowth
-Inflammatorypolyps
- Pouchitis- Cuffitis- Crohn’s dis.- Smallbowel bacterialovergrowth
-Inflammatorypolyps
- Irritablepouch syn.
- Anismus- Pouch inertia- Pseudo-obstruction
- Levator ani syn.- “Pouchalgia”
- Irritablepouch syn.
- Anismus- Pouch inertia- Pseudo-obstruction
- Levator ani syn.- “Pouchalgia”
Dysplastic/Neoplastic
- Pouch/ATZdysplasiaor cancer
- Lymphoma- Squamous cell cancer
- Pouch/ATZdysplasiaor cancer
- Lymphoma- Squamous cell cancer
Systemic/Metabolic
- Anemia- Bone loss- B12deficiency
- Celiac dis?
- Anemia- Bone loss- B12deficiency
- Celiac dis?
Practice Model in Pouch at Cleveland ClinicPractice Model in Pouch at Cleveland Clinic
Pouch Database
Pouch Database Pouchitis
DatabasePouchitis Database
N 3500 1000
Established 1983 2002
Maintenance RNs MD
Population All CCF CCF, non-CCF
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Crohn's Disease21%
Active Pouchitis21%
Refrac tory Pouchitis10%
Cuffitis13%
Funct ional Disease0%
Surg Complication6%
IPS18% Nl Pouch
11%
Evolution of Pouchitis Clinic
N = 558N = 558
2009200920072007
Pouchitis50%
Cuffitis7%
IPS43%
20022002
N = 61N = 61
IPS 18%
Active pouchitis 20%
Refractory pouchitis
13%
Crohn's disease 20%
Cuffitis 10%
Nl pouch 10%
Surg complication
9%
Anismus 0.2%
N = 931IBD Pouches
N = 931IBD Pouches
Disease Classification
§ Irritable pouch syndrome§ GI-pouch inertia/pseudo-obstruction§ Anismus§ Pouch prolapse§ Pouch-associated pain syndrome§ “Pouchalgia fugax”§ Levator ani syndrome§ Phantom pain
§ Irritable pouch syndrome§ GI-pouch inertia/pseudo-obstruction§ Anismus§ Pouch prolapse§ Pouch-associated pain syndrome§ “Pouchalgia fugax”§ Levator ani syndrome§ Phantom pain
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Functional Pouch Disorders:Spectrum of Pathophysiological Processes
HYPOMOTILITY/HYPERSENSIVITY(irritable pouch
syndrome)
HYPOMOTILITY/HYPERSENSIVITY(irritable pouch
syndrome)
MIXEDMIXED
Small bowel bacterial growth
Small bowel bacterial growth
AnismusPoucholgiaPoucholgia
HYPERMOTILITY(GI/pouch inertia,
Pseudo-obstruction)
HYPERMOTILITY(GI/pouch inertia,
Pseudo-obstruction)
Levator ani syndrome
Levator ani syndrome
Phantom pain
Phantom pain
Diagnostic Tools
§ Pouchoscopy§ Sugar breath test for small bowel bacterial
overgrowth?§ Ano-pouch manometry§ KUB, small bowel series or CT enterography§ Gastrograffin enemas§ Barium defecography§ MRI defecography§ Barostat?
§ Pouchoscopy§ Sugar breath test for small bowel bacterial
overgrowth?§ Ano-pouch manometry§ KUB, small bowel series or CT enterography§ Gastrograffin enemas§ Barium defecography§ MRI defecography§ Barostat?
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Ano-pouch Manometry
§ Pouch volume§ Reflex§ Balloon expulsion§ Synergistic contraction§ Sphincter tune
§ Pouch volume§ Reflex§ Balloon expulsion§ Synergistic contraction§ Sphincter tune
Upper balloonUpper balloon
Lower balloonLower balloon
Upper balloonUpper balloon
Lower balloonLower balloon
SqueezeSqueezeStrainStrain
Pouch-pelvic Dyssynergia-Anismus
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Barostat Barostat
Anterior Pouchocele-Gastrograffin EnemaAnterior Pouchocele-Gastrograffin Enema
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Barium DefecographyBarium Defecography
Pouch Prolapse-MRI Defecography
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Distal Pouch Prolapse
Predominant SymptomsPredominant Symptoms
DiarrheaUrgencyCramp
DiarrheaUrgencyCramp
Diagnostic AlgorithmDiagnostic Algorithm
DyscheziaIncompleteEvacuation
DyscheziaIncompleteEvacuation
NauseaVomitingBloating
NauseaVomitingBloating
KUB/SBSCT enterography
Gastrograffin enema
KUB/SBSCT enterography
Gastrograffin enema
Gastrograffin enemaMRI/Ba defecography
Manometry
Gastrograffin enemaMRI/Ba defecography
Manometry
Pouch endoscopyLabs
Pouch endoscopyLabs
PainPain
Endoscopy probingDifferential nerve block
Endoscopy probingDifferential nerve block
PouchitisCuffitis
Crohn’s dzInfectious
Irritable Pouch Syn.
PouchitisCuffitis
Crohn’s dzInfectious
Irritable Pouch Syn.
Afferent Limb Syn.Efferent Limb Syn.Irritable Pouch Syn.
AnismusPouch Inertia
Afferent Limb Syn.Efferent Limb Syn.Irritable Pouch Syn.
AnismusPouch Inertia
SBBOPouch Strictures
GI Inertia
SBBOPouch Strictures
GI Inertia
NeuromaAdhesionsPoucholgiaPhantom
NeuromaAdhesionsPoucholgiaPhantom
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Predominant SymptomsPredominant Symptoms
DiarrheaUrgencyCramp
DiarrheaUrgencyCramp
Management AlgorithmManagement Algorithm
DyscheziaIncompleteEvacuation
DyscheziaIncompleteEvacuation
NauseaVomitingBloating
NauseaVomitingBloating
PainPain
Irritable Pouch Syn.Irritable Pouch Syn. AnismusPouch Inertia
AnismusPouch Inertia
GI InertiaGI Inertia PoucholgiaPhantomPoucholgiaPhantom
Medical TherapyMedical Therapy
BiofeedbackSurgery
BiofeedbackSurgery
Miralax/LactuloseSurgery
Miralax/LactuloseSurgery
Albuterol InhalerNeurotin/Lyrica
B & O suppositoryNerve abalation
Albuterol InhalerNeurotin/Lyrica
B & O suppositoryNerve abalation
Irritable Pouch Syndrome
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Afferent-limb Rectal CuffPouch
Pouch Mucosal Biopsy
Irritable Pouch Syndrome
Differential Diagnosis- Those with Normal Pouch Endoscopy/Biopsy
§ Celiac disease§ Small bowel bacterial overgrowth§ Fructose intolerance§ Infectious diarrhea (Giardia, crypto)§ Clostridium-associated pouchitis
§ Celiac disease§ Small bowel bacterial overgrowth§ Fructose intolerance§ Infectious diarrhea (Giardia, crypto)§ Clostridium-associated pouchitis
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N Variable OR (95% CI) P-value
Pouchitis 61
Colectomy for dysplasia 3.89 (1.69, 8.98) 0.001
NSAID use 3.24 (1.71, 6.13) 0.0003
Never smoked 5.09 (1.01, 25.69) 0.049
Ex-smoker 0.44 (0.11, 1.80) 0.25
Arthralgia 1.01 (0.53, 1.92) 0.97
Non-use of anti-anxiety agents 5.19 (1.45, 18.59) 0.01
Crohn’s Pouch 39
Duration of IPAA 1.20 (1.12, 1.30) < 0.0001
NSAID use 0.47 (0.21, 1.06) 0.068
Current smoking 4.77 (1.39, 16.25) 0.01
Ex-smoker 0.67 (0.16, 2.80) 0.58
Cuffitis 41 Young Age 1.16 (1.01, 1.33) 0.04
Arthralgia 4.13 (1.91, 8.94) 0.0003
Irritable pouch syndrome
50 Anti-depressant use 4.17 (1.95, 8.92) 0.0002
Anti-anxiety agent use 3.21 (1.34, 7.47) 0.007
Logistic Regression Model for Risk Factors
Shen B, et sl. CGH 2006
Comparison of Cleveland Global QOL Scores
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1
Cle
vela
nd
Glo
bal
QO
L (S
cale
0-1
.0)
NormalPouchN = 14
IrritableBowel
SyndromeN = 10
IrritablePouch
SyndromeN = 13
PouchitisN = 11
CuffitisN = 7
P < 0.05
Shen B, et al. DCR 2006
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Comparison of IBS-QOL Scores
0
20
40
60
80
100
120
140
160
IBS
-QO
L (S
cale
34
-17
0)
NormalPouchN = 14
IrritableBowel
SyndromeN = 10
IrritablePouch
SyndromeN = 13
PouchitisN = 11
CuffitisN = 7
P < 0.05
Shen B, et al. DCR 2006
0
50
100
150
200
ml
Pouch Tone Pouch Tone
30 minPostprandial
30 minPostprandial
10 minPreprandial
10 minPreprandial
30 min preprandial
30 min preprandial
Normal Pouch N = 11Normal Pouch N = 11
Irritable Pouch SyndromeN = 8Irritable Pouch SyndromeN = 8
10 minPostprandial
10 minPostprandial
P > 0.05P > 0.05
Shen B, Sanmagel C, Parsi M, et al DDW 2004
14
-50
0
50
100
150
200
250
300
350
400
450
4mmHg 8mmHg 12mmHg 16mmHg 20mmHg 24mmHg 28mmHg
ml
Normal Pouch Irritable Pouch Syndrome
Pouch Compliance Pouch Compliance
P > 0.05
Shen B, Sanmagel C, Parsi M, et al DDW 2004
Visceral Hypersensitivity-Visual Analogue ScaleVisceral Hypersensitivity-Visual Analogue Scale
Pressure 8 Pressure 16 Pressure 24 Pressure 36
Gas
IPS 2.0 ± 0.5 4.5 ± 0.7 5.7 ± 0.6 7.4 ± 0.5
Nl Pouch 0.3 ± 0.2 0.8 ± 0.3 1.6 ± 0.6 2.3 ± 0.7
Pouchitis 1.0 ± 0.4 1.9 ± 0.4 3.4 ± 0.5 4.4 ± 0.6
Pain
IPS 1.9 ±0.6 3.8 ± 0.8 4.9 ± 0.8 6.2 ± 0.8
Nl Pouch 0.1 ± 0.04 0.6 ± 0.2 1.3 ± 0.6 1.8 ± 0.6
Pouchitis 0.7 ± 0.3 1.3 ± 0.5 2.2 ± 0.6 3.7 ± 0.7
Urge
IPS 1.9 ± 0.5 5.3 ± 0.7 5.9 ± 0.7 7.6 ± 0.5
Nl pouch 0.1 ± 0.1 0.9 ± 0.3 1.7 ± 0.6 2.9 ± 0.9
Pouchitis 0.9 ± 0.5 2.3 ± 0.7 3.3 ± 0.8 4.9 ± 0.9
Sample size: IPS=18, nl pouch = 12, pouchitis=11Sample size: IPS=18, nl pouch = 12, pouchitis=11
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Normal Pouch Irritable Pouch Syndrome
Serotonin 20X
Shen B, et al. AJG 2008
Enterochromaffin Cell Hyperplasia in IPSEnterochromaffin Cell Hyperplasia in IPSVariable IPS
N = 35Normal Pouch
N = 25 P
PMN infiltration score 0.40 ± 0.50 0.52 ± 0.51 0.36
Ulcer score 0 35 (100%) 25 (100%) 1.00
<25% 0 (0%) 0 (0%)
25-50% 0 (0%) 0 (0%)
>50% 0 (0%) 0 (0%)
Intraepithelial lymphocytes 22.3 ± 12.1 20.8 ± 11.4 0.52
CD3 233.5 ± 104.3 219.5 ± 82.5 0.59
CD25 3 (0 – 6.2) 3 (1 – 6) 0.62
Serotonin 54.8 ± 24.9 36.7 ± 17.5 0.005
Tryptase 363.2 ± 144.6 386.5 ± 132.8 0.6
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Association between EC cells and mucosal immune cells with Symptoms
Shen B. AJG 2009Shen B. AJG 2009
Proposed Pathophysiology of IPS
Central Nervous SystemCentral Nervous System
Neuroenteric SystemNeuroenteric System
End Organ (pouch)End Organ (pouch)
Visceral hypersensitivityVisceral hypersensitivity
Abnormal serotonin pathwayAbnormal serotonin pathwayProximal small bowel bacterial overgrowthProximal small bowel bacterial overgrowth
Psychosocial factorsPsychosocial factors
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Treatment of Irritable Pouch Syndrome
§ Low-carb diet§ Antispasmodics§ Amitriptyline 25-75mg QHS§ Opium tincture/Paragoric§ Belladonna-Opioid suppository
Pouch Failure Is Uncommon!
§ Low-carb diet§ Antispasmodics§ Amitriptyline 25-75mg QHS§ Opium tincture/Paragoric§ Belladonna-Opioid suppository
Pouch Failure Is Uncommon!
Irritable Pouch Syndrome and Pouch Failure – A Nomogram Model
Irritable Pouch Syndrome and Pouch Failure – A Nomogram Model
Shen B. DDW 2010Shen B. DDW 2010
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Classification of Prolapse
§ Mucosal vs. full-thickness§ Proximal vs. distal pouch§ Intermittent vs. persistent
§ Mucosal vs. full-thickness§ Proximal vs. distal pouch§ Intermittent vs. persistent
Pouch ProlapsePouch Prolapse
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Prolapse in K Pouch
Pouch Prolapse
Age GenderIndicationfor pouch
Pouch type
Time to Prolapse
yrsTreatment
Pouch loss
34 M UC J, DS 6 Biofeedback No
32 F FAP + Ca J, M 1 Pouchpexy No
15 F UC J, DS 5 Pouchpexy No
48 M UC + Ca S, M 1 Mucosal excision No
38 F Inertia J, DS 2 Pouchpexy Yes
21 F UC J, DS 4 Pouchpexy No
40 M UC S, M 1.5 Biofeedback No
22 M UC Redo J, M 1 Pouchpexy Yes
34 M UC J, M 6 Ripstein Yes
41 M UC Redo J, M 1 Local procedure No
23 F UC J, DS 2 Pouchpexy mesh No
Joyce M, et al. ACRSR 2007
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“Pouchalgia fugax”
GI/Pouch Inertia
Lactulose?Miralax?
Ileostomy
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Pouch-Pelvic Dysynergia
Summary
§ Functional complications are common in patients with ileal pouches
§ Functional disorders can be associated structural abnormalities
§ Combined endoscopic, manometric, imaging, and histologic evaluations are often needed
§ Treatment is still empiric
§ Functional complications are common in patients with ileal pouches
§ Functional disorders can be associated structural abnormalities
§ Combined endoscopic, manometric, imaging, and histologic evaluations are often needed
§ Treatment is still empiric
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