Urologic Manifestations of Tethered Cord Syndrome:
clinical and urodynamic findingsJonah Murdock, MD PhD
Mid Atlantic Urology AssociatesJuly 2011
Understand the relationship between Ehlers-Danlos syndrome, tethered cord syndrome, and neurogenic bladder
Define the role for Urodynamics in diagnosing tethered cord
Goals
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
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
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
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
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
• 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
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
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
Sacral nerves (S2,S3,S4) control urethral sphincter
Pelvic nerves (parasympathetic system) control bladder contraction
Hypogastric nerve (sympathetic system) control bladder relaxation
Bladder Neurophysiology
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
Scant literature Small series No consensus
Neurourologic Manifestations of Adult Tethered Cord
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
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?
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?
Place pressure sensing catheters in the bladder, the rectum and near the urethral sphincter
Urodynamics Technique
Hypercontractile Neurogenic Bladder or Overactive Bladder
Detrusor sphinctor dyssinergia
Large Capacity Hyposensory Bladder
Bladder hypotonia with poor urinary flow pattern
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
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
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
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
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
• 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)
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)
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)
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)
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
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
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
Dr. Fraser Henderson Mackenzie Mathis Jenna Sherry Dr. Myron Murdock
Acknowledgements