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