Functional Disorders of the Ileal Pouch

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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


Diagnostic AlgorithmDiagnostic Algorithm

DyscheziaIncompleteEvacuation


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.


SBBOPouch Strictures

GI Inertia

SBBOPouch Strictures

GI Inertia

NeuromaAdhesionsPoucholgiaPhantom

NeuromaAdhesionsPoucholgiaPhantom

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Predominant SymptomsPredominant Symptoms



Management AlgorithmManagement Algorithm





PainPain

Irritable Pouch Syn.Irritable Pouch Syn. 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

Functional Disorders of the Ileal Pouch

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