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"Urologic Manifestations of Tethered Cord Syndrome: clinical and urodynamic findings" presentation by Jonah Murdock, MD, PhD at EDNF's 2011 Learning Conference. The relationship between EDS, tethered cord syndrome, and neurogenic bladder.
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EDNF 2011 Conference 8/5/11
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Jonah Murdock, MD PhD Mid Atlantic Urology Associates
July 2011
} Understand the relationship between Ehlers-Danlos syndrome, tethered cord syndrome, and neurogenic bladder
} Define the role for Urodynamics in diagnosing tethered cord
EDNF 2011 Conference 8/5/11
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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
} 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
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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 Syndrome ◦ Abnormal connective tissue
} Associated Genitourinary Abnormalities ◦ Enlarged bladder and bladder diverticuli ◦ Incomplete Bladder Emptying ◦ Enlarged Ureter ◦ Prolapse ◦ Incontinece
EDNF 2011 Conference 8/5/11
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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
• Symptoms � Involuntary leakage with coughing and sneezing � Sensation of bulge
• Treatment: Surgery • Use caution due to risk of poor wound healing and
recurrence
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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
} Bladder filling ◦ Bladder relaxes & ◦ urethral sphincter contracts (to keep urine in)
} Bladder emptying ◦ Bladder contracts after ◦ Urethral sphincter relaxes (to let urine out)
EDNF 2011 Conference 8/5/11
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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
} 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
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} Scant literature } Small series } No consensus
} Urodynamics } 1) Measure bladder storage (cystometrogram) ◦ bladder compliance, sensation, and capacity
} 2) Measure voiding ◦ Bladder pressure, urinary flow, coordination of
bladder and external sphincter
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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
} 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
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} Place pressure sensing catheters in the bladder, the rectum and near the urethral sphincter
Hypercontractile Neurogenic Bladder or Overactive Bladder
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Detrusor sphinctor dyssinergia
Large Capacity Hyposensory Bladder
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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
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n=20 Symptoms: 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)
} 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%
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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
} 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)
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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
} 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
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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)
0
2
4
6
8
10
12
Neurological Change
Functional Change
Quality of Life
Pain Change
Improved
Worsened
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
} 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
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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
} Dr. Fraser Henderson } Mackenzie Mathis } Jenna Sherry } Dr. Myron Murdock
