Urologic Manifestations of Tethered Cord Syndrome: clinical and urodynamic findings
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Urologic Manifestations of Tethered Cord Syndrome:
clinical and urodynamic findingsJonah Murdock, MD PhD
Mid Atlantic Urology AssociatesJuly 2011
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Understand the relationship between Ehlers-Danlos syndrome, tethered cord syndrome, and neurogenic bladder
Define the role for Urodynamics in diagnosing tethered cord
Goals
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Sequela of deformative stress injury to distal spinal cord
Nerves serving the pelvis (e.g. bladder) and lower extremities do not function due to deformative stress
with Ehlers-Danlos more common than previously thought
Urologic symptoms◦ Not specific & frequently not acknowledged◦ Can’t feel when bladder is full◦ Bladder always feels full◦ Severe straining and intermittent stream when voiding
Adult Tethered Cord
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Aufschnaiter (2008) Neurosurg Rev 31(4):371
Literature review (n=386) Average age of symptom onset 36.5 years Predominant symptom: pain and weakness
lower extemities Precipitating incident: trauma, excessive
physical training
Adult Tethered Cord
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Potential problems from Ehlers-Danlos: 1) Bladder muscle laxity can cause voiding
problems 2) Functional impairment of bladder’s
nerves by tethered cord can cause voiding problems
Ehlers-Danlos: Muscle Dysfunction or Neurologic Dysfunction from Tethered Cord
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Ehlers-Danlos Syndrome◦ Abnormal connective tissue
Associated Genitourinary Abnormalities◦ Enlarged bladder and bladder diverticuli◦ Incomplete Bladder Emptying◦ Enlarged Ureter◦ Prolapse◦ Incontinece
Ehlers-Danlos and Voiding Dysfunction: Conventional View
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E-D can result in bladder laxity, a large sacculated poorly emptying bladder◦ Prone to urinary tract infections
Treatment:◦ Timed voiding◦ Double voiding to empty bladder◦ Antibiotic prophylaxis◦ Surgery: bladder diverticulectomy
Ehlers-Danlos: Treatment of Bladder Muscle dysfunction
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• Symptoms Involuntary leakage with coughing and sneezing Sensation of bulge
• Treatment: Surgery• Use caution due to risk of poor wound healing and
recurrence
Ehlers-Danlos: Treatment of Prolapse & Incontinence
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How does a tethered cord cause abnormal voiding?
Pelvic and sacral nerves impaired function◦ Control bladder storage of urine◦ Control emptying of urine at void
Symptoms◦ Can’t feel when bladder is full◦ Bladder always feels full◦ Severe straining and intermittent stream when
voiding
Ehlers-Danlos Syndrome and Tethered Cord
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Bladder filling◦ Bladder relaxes & ◦ urethral sphincter contracts (to keep urine in)
Bladder emptying◦ Bladder contracts after◦ Urethral sphincter relaxes (to let urine out)
Bladder Physiology, the basics
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Sacral nerves (S2,S3,S4) control urethral sphincter
Pelvic nerves (parasympathetic system) control bladder contraction
Hypogastric nerve (sympathetic system) control bladder relaxation
Bladder Neurophysiology
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Neurogenic Bladder=abnormal bladder function from sacral and pelvic nerve dysfunction
Detrusor Sphincter Dyssinergia◦ Sphincter contracts at voiding◦ Symptoms: hesitancy and straining at void
Hypercontractile Neurogenic Bladder◦ Bladder contracts during filling◦ Symptoms: urgency, frequency, incontinence
Abnormal Bladder Physiology: 2 Examples
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Scant literature Small series No consensus
Neurourologic Manifestations of Adult Tethered Cord
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Urodynamics 1) Measure bladder storage
(cystometrogram)◦ bladder compliance, sensation, and capacity
2) Measure voiding◦ Bladder pressure, urinary flow, coordination of
bladder and external sphincter
Objective Measurement of Lower Urinary Tract Dysfunction
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Urodynamic abnormality reflects nerve dysfunction◦ peripheral (e.g.sacral nerve) dysfunction or ◦ central (e.g. cervical spine) nerve dysfuntion
Uncover occult bladder dysfunction Confirm clinical and radiologic diagnosis Identify response to treatment
Why perform urodynamics?
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Measure pressure in the bladder◦ While it fills◦ During voiding
Measure pressure of the urinary sphincter◦ During bladder filling◦ During voiding
Measure the urinary stream◦ Force and pattern of the urinary stream at void
What is Urodynamics?
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Place pressure sensing catheters in the bladder, the rectum and near the urethral sphincter
Urodynamics Technique
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Hypercontractile Neurogenic Bladder or Overactive Bladder
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Detrusor sphinctor dyssinergia
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Large Capacity Hyposensory Bladder
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Bladder hypotonia with poor urinary flow pattern
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Scant Literature Husman (1995) Occult spinal dysraphism
(the tethered cord) and the urologist.◦ There is no typical urologic dysfunction◦ Treatment is based on urodynamic evaluation
Literature Review: Urologic & Urodynamic Findings in Tethered Cord
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n=20Symptoms: irritative voiding, incontinence, and retention
Most Common Symptoms: urgency (67%) and urge incontinence (50%)
Urodynamic findings: detrusor hyperreflexia (72%), Detrusor external sphincter dyssinergia (22%), decreased sensation (17%), decreased compliance (17%), hypocontractile detrusor (11%)
Postoperative improvement in only 29% (n=4 pts)
Giddens (1999) Urodynamic findings in adults with the tethered cord syndrome
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n=18 Urodynamic findings: “flaccid bladder”50%,
“uninhibited bladder” 28%, “mixed bladder dysfunction” 11%, Normal 11%
Postoperative improvement: ◦ Uninhibited bladder resolved in all◦ Flaccid bladder resolved or improved in 45%
Hellstrom (1986) Urological Aspects of Tethered Cord Syndrome JU 135(2):317
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n=15 93% with abnormal urodynamics
◦ Detrusor areflexia 60% Postop restoration of bladder function 67% Poor postoperative function:
◦ If bladder symptoms >3 years◦ Cutaneous stigma
Kondo (1986) Bladder function secondary to tethered cord syndrome in adults: is it curable? JU 135(2):313
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n=29 48% had urinary symptoms
◦ 47% had postsurgical improvement of urinary symptoms
◦ Mean time to urinary symptom improvement 4.3 months (vs 1 month for pain improvement)
Garces-Ambrossi (2009) Neurological outcome after surgical management of adult tethered cord J Neurosurg Spine 11(3):304
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• n=43• Ehlers-Danlos• 85% with radiologic evidence of tethered
cord• Standard symptom questionnaire
• All with tethered cord symptoms• 60% have urologic symptoms
• Urodynamics performed on all preoperatively
Drs Murdock & Henderson Series of Tethered Cord Patients (2010-2011)
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of Patients with Ehlers-Danlos & tethered cord symptoms:
75% have abnormal urodynamics◦ 37% hyposensory bladder with retention◦ 34% hypertonic bladder◦ 28%% detrusor sphincter dyssinergia
25% have normal urodynamics
Drs Murdock & Henderson Series of Tethered Cord Patients (2010-2011)
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Abnormal urodynamics (n=14):◦ 86% Symptom improvement after surgery◦ 14% No symptom improvement after surgery
Normal urodynamics (n=3):◦ 100% symptom improvement◦ (not all patients with tethered cord have abnormal
urodynamics)
Tethered Cord Surgery Results(Dr. F Henderson)
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Neurologi-cal Change
Functional Change
Quality of Life
Pain Change
0
2
4
6
8
10
12
ImprovedWorsened No change
Tethered cord surgery results n=13 (Dr. F Henderson)
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1) Surgical detethering: sectioning the filum terminale
2) If residual symptoms after correction of tethered cord:
Detrusor Sphincter Dyssinergia◦ Medical management: Alpha blocker
Large capacity bladder◦ Timed voiding
Hypercontractile bladder◦ Medical management, Anti-muscarinic
Treatment
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Abnormal urodynamics is present even in the absence of lower urinary tract symptoms in most patients with tethered cord
Most common urodynamic findings are: hypertonic bladder, bladder hypotonia with urinary retention, and detrusor sphincter dysinergia
Conclusions: tethered cord and urodynamics
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Tethered cord is common among properly screened patients with Ehlers Danlos syndrome
Urodynamics is a good predictor alongside clinical symptoms and radiography in diagnosing tethered cord and predicting a good response to surgery
Conclusions
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Dr. Fraser Henderson Mackenzie Mathis Jenna Sherry Dr. Myron Murdock
Acknowledgements