Urologic Manifestations of Tethered Cord Syndrome: clinical and urodynamic findings

1. Urologic Manifestations of Tethered Cord Syndrome: clinical and urodynamic findings Jonah Murdock, MD PhD Mid Atlantic Urology Associates July 2011

2. Goals • Understand the relationship between Ehlers-Danlos syndrome, tethered cord syndrome, and neurogenic bladder • Define the role for Urodynamics in diagnosing tethered cord

3. Adult Tethered Cord • 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

4. 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

5. Ehlers-Danlos: Muscle Dysfunction or Neurologic Dysfunction from Tethered Cord • Potential problems from Ehlers-Danlos: • 1) Bladder muscle laxity can cause voiding problems • 2) Functional impairment of bladder’s nervesby tethered cord can cause voiding problems

6. Ehlers-Danlos and Voiding Dysfunction: Conventional View • Ehlers-Danlos Syndrome • Abnormal connective tissue • Associated Genitourinary Abnormalities • Enlarged bladder and bladder diverticuli • Incomplete Bladder Emptying • Enlarged Ureter • Prolapse • Incontinece

7. Ehlers-Danlos: Treatment of Bladder Muscle dysfunction • 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

8. Ehlers-Danlos: Treatment of Prolapse & Incontinence • Symptoms • Involuntary leakage with coughing and sneezing • Sensation of bulge • Treatment: Surgery • Use caution due to risk of poor wound healing and recurrence

9. Ehlers-Danlos Syndrome and Tethered Cord • 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

10. Bladder Physiology, the basics • Bladder filling • Bladder relaxes & • urethral sphincter contracts (to keep urine in) • Bladder emptying • Bladder contracts after • Urethral sphincter relaxes (to let urine out)

11. Bladder Neurophysiology • Sacral nerves (S2,S3,S4) control urethral sphincter • Pelvic nerves (parasympathetic system) control bladder contraction • Hypogastric nerve (sympathetic system) control bladder relaxation

12. Abnormal Bladder Physiology: 2 Examples • Neurogenic Bladder=abnormal bladder function from sacral and pelvic nerve dysfunction • Detrusor Sphincter Dyssinergia • Sphincter contracts at voiding • Symptoms: hesitancy and straining at void • HypercontractileNeurogenic Bladder • Bladder contracts during filling • Symptoms: urgency, frequency, incontinence

13. Neurourologic Manifestations of Adult Tethered Cord • Scant literature • Small series • No consensus

14. Objective Measurement of Lower Urinary Tract Dysfunction • Urodynamics • 1) Measure bladder storage (cystometrogram) • bladder compliance, sensation, and capacity • 2) Measure voiding • Bladder pressure, urinary flow, coordination of bladder and external sphincter

15. Why perform urodynamics? • 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

16. What is 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

17. Urodynamics Technique • Place pressure sensing catheters in the bladder, the rectum and near the urethral sphincter

18. HypercontractileNeurogenic Bladder or Overactive Bladder

19. Detrusorsphinctordyssinergia

20. Large Capacity Hyposensory Bladder

21. Bladder hypotonia with poor urinary flow pattern

22. Literature Review: Urologic & Urodynamic Findings in Tethered Cord • 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

23. Giddens (1999) Urodynamic findings in adults with the tethered cord syndrome n=20 Symptoms: irritative voiding, incontinence, and retention Most Common Symptoms: urgency (67%) and urge incontinence (50%) Urodynamic findings: detrusorhyperreflexia (72%), Detrusor external sphincter dyssinergia (22%), decreased sensation (17%), decreased compliance (17%), hypocontractiledetrusor (11%) Postoperative improvement in only 29% (n=4 pts)

24. Hellstrom (1986) Urological Aspects of Tethered Cord Syndrome JU 135(2):317 • 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%

25. Kondo (1986) Bladder function secondary to tethered cord syndrome in adults: is it curable? JU 135(2):313 • n=15 • 93% with abnormal urodynamics • Detrusorareflexia 60% • Postop restoration of bladder function 67% • Poor postoperative function: • If bladder symptoms >3 years • Cutaneous stigma

26. Garces-Ambrossi (2009) Neurological outcome after surgical management of adult tethered cord J Neurosurg Spine 11(3):304 • 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)

27. Drs Murdock & Henderson Series of Tethered Cord Patients (2010-2011) • 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

28. 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

29. Tethered Cord Surgery Results(Dr. F Henderson) • 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)

30. Tethered cord surgery results n=13 (Dr. F Henderson)

31. Treatment • 1) Surgical detethering: sectioning the filumterminale • 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

32. Conclusions: tethered cord and urodynamics • 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

33. Conclusions • 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 cordand predicting a good response to surgery

34. Acknowledgements • Dr. Fraser Henderson • Mackenzie Mathis • Jenna Sherry • Dr. Myron Murdock