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Urogynecology in EDS Joan L. Blomquist, MD Greater Baltimore Medical Center August 2018

Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

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Page 1: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Urogynecology in EDS

Joan L. Blomquist, MDGreater Baltimore Medical Center

August 2018

Page 2: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

“One in three like me”

Page 3: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Voiding IssuesFrequency/UrgencyUrinary Incontinence

“neurogenic bladder”

Page 4: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 5: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 6: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Neurologic supply of the bladder

Page 7: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Evaluation of Bladder Issues

• History• Urinary Diary• Physical examination• Post void residual test– Ultrasound or catheter

• Urodynamics

Page 8: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 9: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Voiding Issues• Symptoms

– Urinary hesitancy– Slow stream– Urinary frequency– Bladder infections– Kidney problems

• Causes– Inadequate detrusor

contraction– Inadequate urethral

relaxation– High tone pelvic floor– Pelvic organ prolapse

Page 10: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

• Inadequate urethral sphincter relaxation– Pelvic floor physical

therapy– Medications

• Cardura• tamsolosin

– Self catheterization

Page 11: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

• High tone pelvic floor– Pelvic floor physical

therapy– Relaxation techniques– Muscle relaxants

• Vaginal suppositories

Page 12: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 13: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Urinary Frequency and Urgency

• Voiding dysfunction• Overactive bladder• Interstitial cystitis

Page 14: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Overactive Bladder

Page 15: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

OAB – treatment options

Behavioral modifications-weight loss-avoidance of bladder irritants-fluid management

Page 16: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

OAB - treatment

• Pelvic floor muscle exercises

• Physical therapy

Page 17: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

OAB - treatment

• Medicationsanticholinergics

oxybutynintolterodinedarifenicinesolifenicinetrospium

Beta agonistmirabegron

Page 18: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Percutaneous Tibial Nerve Stimulation

Page 19: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Sacral Nerve Stimulation

Page 20: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Botox

Page 21: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Interstitial cystitis/Painful bladder syndrome

• AUA (2011)– unpleasant sensation (pain, pressure, discomfort)

perceived to be related to the urinary bladder, associated with lower urinary tract symptoms of more than six weeks duration, in the absence of infection or other identifiable causes.

• ICS (2002)– the complaint of suprapubic pain related to bladder

filling, accompanied by other symptoms such as increased daytime and night-time frequency, in the absence of proven urinary infection or other obvious pathology

Page 22: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Pathophysiology

• No recognized comprehensive pathophysiologic explanation

• Theories include• Autoimmune• Systemic disease• Neurogenic inflammation• Allergic/Mast cell activation• Defective GAG layer (‘leaky epithelium’)

Page 23: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

PathophysiologyMultifactorial Model

Initiating event

Change in urothelial function

‘Leaky’ Urothelium

Sensitization of peripheral nerves

Release of substance P and inflammatory mediators

Mast cell activation

Vascular congestion and protein extravesation

Page 24: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Diagnosis

Typical appearance of glomerulations after bladder distention in a patient with nonulcerative IC

Typical appearance of Hunner’s ulcer in an IC patient before bladder distention

Walsh: Campbell's Urology, 8th ed., Copyright © 2002 Elsevier

Page 25: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

First Line Treatments

• General relaxation/stress management• Pain management– Calcium Glycerophosphate, phenazopyridine, aloe

vera• Pt education• Behavioral modification– Adequate water, dietary modifications

Page 26: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Second Line Treatments

• Appropriate manual physical therapy techniques

• Oral: amitriptyline, cimetidine, hydroxyzine, Pentosan polysulfate

• Intravesical: DMSO, heparin, lidocaine• Pain managment

Page 27: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Physical Therapy for IC/PBS

Page 28: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Third Line therapies

• Cystoscopy under anesthesia with hydrodistention

• Neuromodulation• Intravesical botox

Page 29: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Types of Urinary Incontinence

Stress IncontinenceUrge IncontinenceMixed IncontinenceFunctional IncontinenceOverflow IncontinenceFistula

Page 30: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Stress Incontinence

• Leakage of urine during physical movement or activity– Coughing, Laughing, Sneezing, Running,

Exercising, Lifting, Straining

• Increases in abdominal pressure are transmitted to the bladder

Page 31: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 32: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Stress Incontinence

Page 33: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Treatment for Stress Incontinence

• Pelvic Floor Exercises• Pessary/Impressa• Surgery• Urethral injections

Page 34: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Pelvic Floor Exercises

Page 35: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Pelvic Floor Muscle Therapy

• Kegel, 1948– Successful therapy of 64

patients with SUI

Page 36: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Electrical Stimulation

Page 37: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Pessary

Page 38: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Impressa

Page 39: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

Transvaginal Sling

Page 40: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)
Page 41: Urogynecology in EDS - The Ehlers Danlos Society · 2018. 9. 28. · Interstitial cystitis/Painful bladder syndrome • AUA (2011) – unpleasant sensation (pain, pressure, discomfort)

• Unique aspects – Involvement of pelvic

floor muscles– Tethered cord– Mast cell activation– POTS– Multiple medications

• Recommendations– Knowledgeable provider– Conservative treatments– Pelvic physical therapist