Functional Disorders of the Functional Disorders of the Ileal Pouch - - PDF document

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Functional Disorders of the Functional Disorders of the Ileal Pouch Ileal Pouch Bo Shen, MD Bo Shen, MD The Cleveland Clinic Foundation The Cleveland Clinic Foundation Cleveland, OH Cleveland, OH December 4, 2009 December 4, 2009 Imaging

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Functional Disorders of the Ileal Pouch Functional Disorders of the Ileal Pouch

Bo Shen, MD The Cleveland Clinic Foundation Cleveland, OH December 4, 2009 Bo Shen, MD The 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 Pouches Anatomy 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/cuff Outlet/cuff Inlet Inlet Efferent limb Efferent limb

Pelvic J Pouch Pelvic J Pouch

J, S, K Pouches vs. Ileostomy J, S, K Pouches vs. Ileostomy

Kock Pouch Kock Pouch Brooke Ileostomy Brooke Ileostomy

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Ileal Pouch Disorders and Associated Complications Ileal Pouch Disorders and Associated Complications

Functional Surgical/ Mechanical Inflammatory/ Infectious

  • Anastomotic leaks
  • Pelvic sepsis
  • Pouch sinuses
  • Pouch fistulae
  • Strictures
  • Afferent limb syn.
  • Efferent limb syn.
  • Infecundity
  • Sexual

dysfunction

  • Portal vein thrombi
  • Pouch prolapse
  • Foreign bodies
  • Anastomotic leaks
  • Pelvic sepsis
  • Pouch sinuses
  • Pouch fistulae
  • Strictures
  • Afferent limb syn.
  • Efferent limb syn.
  • Infecundity
  • Sexual

dysfunction

  • Portal vein thrombi
  • Pouch prolapse
  • Foreign bodies
  • Pouchitis
  • Cuffitis
  • Crohn’s dis.
  • Small

bowel bacterial

  • vergrowth
  • Inflammatory

polyps

  • Pouchitis
  • Cuffitis
  • Crohn’s dis.
  • Small

bowel bacterial

  • vergrowth
  • Inflammatory

polyps

  • Irritable

pouch syn.

  • Anismus
  • Pouch inertia
  • Pseudo-
  • bstruction
  • Levator ani syn.
  • “Pouchalgia”
  • Irritable

pouch syn.

  • Anismus
  • Pouch inertia
  • Pseudo-
  • bstruction
  • Levator ani syn.
  • “Pouchalgia”

Dysplastic/ Neoplastic

  • Pouch/ATZ

dysplasia

  • r cancer
  • Lymphoma
  • Squamous

cell cancer

  • Pouch/ATZ

dysplasia

  • r cancer
  • Lymphoma
  • Squamous

cell cancer Systemic/ Metabolic

  • Anemia
  • Bone loss
  • B12

deficiency

  • Celiac dis?
  • Anemia
  • Bone loss
  • B12

deficiency

  • Celiac dis?

Practice Model in Pouch at Cleveland Clinic Practice Model in Pouch at Cleveland Clinic

Pouch Database Pouch Database

Pouchitis Database Pouchitis Database N 3500 1000 Established 1983 2002 Maintenance RNs MD Population All CCF CCF, non-CCF

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Crohn's Disease 21% Active Pouchitis 21% Refractory Pouchitis 10% Cuffitis 13% Functional Disease 0% Surg Complication 6% IPS 18% Nl Pouch 11%

Evolution of Pouchitis Clinic

N = 558 N = 558 2009 2009 2007 2007

Pouchitis 50% Cuffitis 7% IPS 43%

2002 2002 N = 61 N = 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 = 931 IBD Pouches N = 931 IBD 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/HY PERSENSIVITY (irritable pouch syndrome) HYPOMOTILITY/HY PERSENSIVITY (irritable pouch syndrome) MIXED MIXED Small bowel bacterial growth Small bowel bacterial growth Anismus Poucholgia Poucholgia HYPERMOTILITY (GI/pouch inertia, Pseudo-

  • bstruction)

HYPERMOTILITY (GI/pouch inertia, Pseudo-

  • bstruction)

Levator ani syndrome Levator ani syndrome Phantom pain Phantom pain

Diagnostic Tools

§ Pouchoscopy § Sugar breath test for small bowel bacterial

  • vergrowth?

§ 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

  • vergrowth?

§ 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 balloon Upper balloon Lower balloon Lower balloon Upper balloon Upper balloon Lower balloon Lower balloon

Squeeze Squeeze Strain Strain

Pouch-pelvic Dyssynergia-Anismus

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Barostat Barostat

Anterior Pouchocele- Gastrograffin Enema Anterior Pouchocele- Gastrograffin Enema

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Barium Defecography Barium Defecography

Pouch Prolapse

  • MRI Defecography
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Distal Pouch Prolapse

Predominant Symptoms Predominant Symptoms

Diarrhea Urgency Cramp Diarrhea Urgency Cramp

Diagnostic Algorithm Diagnostic Algorithm

Dyschezia Incomplete Evacuation Dyschezia Incomplete Evacuation Nausea Vomiting Bloating Nausea Vomiting Bloating KUB/SBS CT enterography Gastrograffin enema KUB/SBS CT enterography Gastrograffin enema Gastrograffin enema MRI/Ba defecography Manometry Gastrograffin enema MRI/Ba defecography Manometry Pouch endoscopy Labs Pouch endoscopy Labs Pain Pain Endoscopy probing Differential nerve block Endoscopy probing Differential nerve block Pouchitis Cuffitis Crohn’s dz Infectious Irritable Pouch Syn. Pouchitis Cuffitis Crohn’s dz Infectious Irritable Pouch Syn. Afferent Limb Syn. Efferent Limb Syn. Irritable Pouch Syn. Anismus

Pouch Inertia

Afferent Limb Syn. Efferent Limb Syn. Irritable Pouch Syn. Anismus

Pouch Inertia

SBBO Pouch Strictures GI Inertia SBBO Pouch Strictures GI Inertia Neuroma Adhesions

Poucholgia Phantom

Neuroma Adhesions

Poucholgia Phantom

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Predominant Symptoms Predominant Symptoms

Diarrhea Urgency Cramp Diarrhea Urgency Cramp

Management Algorithm Management Algorithm

Dyschezia Incomplete Evacuation Dyschezia Incomplete Evacuation Nausea Vomiting Bloating Nausea Vomiting Bloating Pain Pain Irritable Pouch Syn. Irritable Pouch Syn. Anismus

Pouch Inertia

Anismus

Pouch Inertia

GI Inertia GI Inertia

Poucholgia Phantom Poucholgia Phantom

Medical Therapy Medical Therapy Biofeedback Surgery Biofeedback Surgery Miralax/Lactulose Surgery Miralax/Lactulose Surgery Albuterol Inhaler Neurotin/Lyrica B & O suppository Nerve abalation Albuterol Inhaler Neurotin/Lyrica B & O suppository Nerve abalation

Irritable Pouch Syndrome

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Afferent-limb Rectal Cuff Pouch 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

0.1 0.2 0.3 0.4 0.5 0.6 0.7 0.8 0.9 1 Cleveland Global QOL (Scale 0-1.0) Normal Pouch N = 14 Irritable Bowel Syndrome N = 10 Irritable Pouch Syndrome N = 13 Pouchitis N = 11 Cuffitis N = 7 P< 0.05

Shen B, et al. DCR 2006

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Comparison of IBS-QOL Scores

20 40 60 80 100 120 140 160 IBS-QOL (Scale 34-170) Normal Pouch N = 14 Irritable Bowel Syndrome N = 10 Irritable Pouch Syndrome N = 13 Pouchitis N = 11 Cuffitis N = 7 P< 0.05

Shen B, et al. DCR 2006

50 100 150 200

ml

Pouch Tone Pouch Tone

30 min Postprandial 30 min Postprandial 10 min Preprandial 10 min Preprandial 30 min preprandial 30 min preprandial Normal Pouch N = 11 Normal Pouch N = 11 Irritable Pouch Syndrome N = 8 Irritable Pouch Syndrome N = 8 10 min Postprandial 10 min Postprandial

P > 0.05 P > 0.05

Shen B, Sanmagel C, Parsi M, et al DDW 2004

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  • 50

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 Scale Visceral 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= 11 Sample 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 IPS Enterochromaffin Cell Hyperplasia in IPS

Variable IPS N = 35 Normal Pouch N = 25 P PMN infiltration score 0.40 ± 0.50 0.52 ± 0.51 0.36 Ulcer score 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 2009 Shen B. AJG 2009

Proposed Pathophysiology of IPS

Central Nervous System Central Nervous System Neuroenteric System Neuroenteric System End Organ (pouch) End Organ (pouch) Visceral hypersensitivity Visceral hypersensitivity Abnormal serotonin pathway Abnormal serotonin pathway Proximal small bowel bacterial overgrowth Proximal small bowel bacterial overgrowth Psychosocial factors Psychosocial 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 2010 Shen 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 Prolapse Pouch Prolapse

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Prolapse in K Pouch Pouch Prolapse

Age Gender Indication for pouch Pouch type Time to Prolapse yrs Treatment 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