1. 排尿障礙治療中心 版權所有 Dysfunctional Voiding in Children Hann-Chorng Kuo Department of Urology Buddhist Tzu Chi General Hospital

2. 排尿障礙治療中心 版權所有 Development of Urethral Sphincter Specific striated sphincter muscle closely applied to the smooth muscle at membranous urethra and mid-urethra A ring shape sphincter in early adolescence, which account for initial high voiding pressure in infancy and early vesicoureteral reflux An omega shape shincter in adolescence after development of urogenital septum

3. 排尿障礙治療中心 版權所有 Congenital Abnormalities Myelodysplasia Lipomeningocele Sacral agenesis Tethered cord Cerebral palsy Bladder extrophy Posterior urethral valve Anorectal malformations

4. 排尿障礙治療中心 版權所有 Myelomeningocele The most common form of NVD in children Early detection and folic acid treatment markedly decrease spinal defects Upper and lower motor bladder dysfunction and pelvic floor dysfunction may occur in thoracic or sacral lesions Early prophylactic treatment of DESD by CIC, anticholinergics are beneficial

5. 排尿障礙治療中心 版權所有 Myelomeningocele, detrusor areflexia and incontinence

6. 排尿障礙治療中心 版權所有 Meningomyelocele & Bilateral VUR & Recurrent UTI

7. 排尿障礙治療中心 版權所有 Lipomeningocele Difficult to identify by physical examination, MRI is the best diagnostic method Intradural lipoma results in disease and presentation The most common urodynamic findings are consistent with an upper motor neuron lesion DESD is less common Detrusor hyperreflexia and areflexia can be found in this group of lesion

8. 排尿障礙治療中心 版權所有 Sacral agenesis Often discovered at older children with incontinence Loss of the lower vetebral bodies by X- ray or MRI Patients have stable neurological lesion Patients may have no signs of denervation, hyperreflexia, areflexia, intact sphincter, sphincter dyssynergia

9. 排尿障礙治療中心 版權所有 Tethered cord syndrome Most commonly seen in patients after surgery for myelomeningocele Isolated tethered cord is less common Severe bladder dysfunction and refractory incontinence may occur Surgical division of the filum may improve symptoms

10. 排尿障礙治療中心 版權所有 Cerebral Palsy Develops most commonly in premature infant Infection and anoxia result in a non- progressive brain lesion and muscular disability Continence is often delayed to develop but intact Uninhibited detrusor contractions without DESD is the most commonly urodynamic finding Pseudodyssynergia may occur

11. 排尿障礙治療中心 版權所有 Cerebral palsy with frequency dysuria due to DI

12. 排尿障礙治療中心 版權所有 Bladder extrophy Characterized by extrophic bladder, abdominal wall defect, epispadias, pelvic diastasis, VU reflux, inguinal hernia Staged reconstruction by abdominal wall closure, epispadias repair, bladder neck reconstruction and correction of VUR Improved pelvic floor reconstruction after osteotomy has better continence rate Bladder augmentation may be indicated

13. 排尿障礙治療中心 版權所有 Posterior Urethral Valve The most common cause of BOO in newborn Present with incontinence and recurrent UTI Severe PUV may be detected antenatally, mild form is found in older children Bilateral hydroureter and hydronephrosis may develop in severe form of valve disease Transurethral ablation of valve resumes normal bladder but bladder function depends Anticholinergics, CIC and augmentation by ureter may be indicated

14. 排尿障礙治療中心 版權所有 Anorectal Malformations Rare congenital lesions of cloaca Associated with congenital GU abnormalities in 20% with low and 60% high lesions,VUR, NVD, renal agenesis, renal dysplasia, cryptorchidism Urethrorectal fistula may develop at at high, intermediate or low level Neurogenic voiding dysfunction in 50% Tethered cord is the main vertebral abnormality, which account for NVD

15. 排尿障礙治療中心 版權所有 Dysfunctional Voiding A group of neurologically intact children presents with incontinence, dysuria, large residual urine, recurrent UTI, unilateral or bilateral hydronephrosis Urodynamically classified into small capacity hypertonic bladder, detrusor hyperreflexia, lazy bladder syndrome,non-neurogenic neurogenic bladder Treatment bases on interaction of bladder and external sphincter

16. 排尿障礙治療中心 版權所有 Patient evaluation – history Antenatal GU abnormalities – hydronephrosis, enlarged bladder, open spinal cord defect Past surgical history – detethering procedure, VP shunt, urinary diversion Occurrence of UTI and antibiotics Bowel habit, fecal incontinence, and stool softeners Catheterization schedule, urine amount Medication and adverse effects

17. 排尿障礙治療中心 版權所有 Physical examination Neurological examination – gait, discrimination of extremities, motor strength, DTR (S1,2), BCR (S2-4) Sacral dimple, hair patch, lipoma Enlarged bladder Vincent curtsey Anal tone, volitional contraction of pelvic floor muscles

18. 排尿障礙治療中心 版權所有 Urodynamic study Estimated bladder capacity: (age+2)x30 ml Infusion rate: 10% of capacity Catheter: <6Fr intraurethral dual channel catheter, suprapubic catheter is preferable for pressure flow study Abdominal pressure by rectal catheter Pelvic floor EMG – surface or needle Measuring bladder compliance, detrusor pressure, and EMG activities coordination

19. 排尿障礙治療中心 版權所有 Detrusor external sphincter dyssynergia (DESD) Type 1: Onset of EMG activity with initiation of voiding Type 2: intermittent inappropriate external sphincter contraction during voiding,which causes a reflex inhibition of detrusor contraction Type 3: Persistent increased EMG activity during filling and voiding phases, which causes large residual urine and incontinence Pseudodyssynergia: presence of urodynamic DESD in neurologically intact patient

20. 排尿障礙治療中心 版權所有 Leak-point pressures Detrusor leak-point pressure (DLPP): The detrusor pressure causing urinary leakage per urethrum in the absence of detrusor contractions A DLPP of more than 40 cm water has a risk of upper tract deterioration Valsalvar LPP (VLPP): Assessing urethral resistance by abdominal straining, a VLPP <60 cm water indicates intrinsic sphincter deficiency

21. 排尿障礙治療中心 版權所有 Indications for urodynamic study in children Spinal dysraphisms Spinal cord injury Cerebral palsy with voiding dysfunction Sacral agenesis Imperforated anus Diurnal enuresis Suspicious voiding dysfunction and UTI Dysfunctional voiding

22. 排尿障礙治療中心 版權所有 Urodynamic studies in children with dysfunctional voiding Uroflowmetry with surface EMG Cystometry with abdominal pressure and EMG Pressure flow study recording Pves,Pabd, Pdet, EMG activity, and uroflowmetry Videourodynamic study by suprapubic catheter or intra-urethral catheter

23. 排尿障礙治療中心 版權所有 Uroflowmetry – flat flow pattern with non-relaxing ES

24. 排尿障礙治療中心 版權所有 Uroflowmetry – Staccato pattern and poor relaxing ES

25. 排尿障礙治療中心 版權所有 Videourodynamics via cystostomy pressure flow study

26. 排尿障礙治療中心 版權所有 Dysfunctional Voiding Associated with the followings Diurnal enuresis Urinary urgency Urinary frequency Constipation Urinary tract infection Vesicoureteral reflux

27. 排尿障礙治療中心 版權所有 Pathogenesis of dysfunctional voiding Increased voiding pressure during voiding with contraction of the urethral sphincter Dysfunctional bowel evacuation and constipation Treatment directed at urodynamic abnormalities reduce the incidence of breakthrough UTI and increase resolution of vesicoureteral reflux

28. 排尿障礙治療中心 版權所有 Typical spinning top voiding cystourethrography

29. 排尿障礙治療中心 版權所有 Development of dysfunctional voiding Long-standing pelvic floor dysfunction results in paradoxical sphincter contraction Pelvic laxity Inappropriate stimulation of guarding reflex results in inhibition of detrusor contraction

30. 排尿障礙治療中心 版權所有 Detrusor instability without dyssynergic external sphincter

31. 排尿障礙治療中心 版權所有 Dysfunctional voiding and Urinary tract infection Elevated postvoid residual urine Host resistance – ability of bladder to wash out pathogens Well hydration, void with strong stream, and complete voiding are important in prevention of UTI Treatment aims at relaxation of the pelvic floor rather than the bladder

32. 排尿障礙治療中心 版權所有 Recurrent UTI in siblings with Dysfunctional voiding

33. 排尿障礙治療中心 版權所有 Non-neurogenic neurogenic bladder – Hinman syndrome The severest form of dysfunctional voiding Symptom complex including nocturnal enuresis, diurnal enuresis, constipation, encopresis, UTI, and upper tract dilatation Uninhibited detrusor contractions and dyssynergic external sphincter

34. 排尿障礙治療中心 版權所有 Treatment of non-neurogenic neurogenic bladder Voiding retraining Biofeedback Anticholinergic therapy Hypnosis Psychotherapy Management of constipation Antibiotics Clean intermittent catheterization

35. 排尿障礙治療中心 版權所有 Dysfunctional voiding and Vesicoureteral reflux Play a major role in etiology of congenital VUR Important in development of VUR in older child without congenital VUR Responsible for reflux exacerbation and renal scarring Therapy to VUR should aim at correction of dysfunctional voiding

36. 排尿障礙治療中心 版權所有 Bilateral VUR in a girl with Dysfunctional voiding

37. 排尿障礙治療中心 版權所有 Right VUR and DI without dysfunctional voiding

38. 排尿障礙治療中心 版權所有 Resolution of VUR after Anticholinergic therapy

39. 排尿障礙治療中心 版權所有 Urodynamic studies in infants High voiding pressures (160cm water) with low bladder capacity in infant with gross dilating reflux Voiding pressure in infant without reflux is 80 cm water By age 2 years, voiding pressure diminished (70 cm water) and capacity increased, but unstable detrusor remain

40. 排尿障礙治療中心 版權所有 High voiding pressures in infancy Transient functional bladder outlet obstruction Boys with high grade reflux have dilated posterior urethra Higher voiding pressure is seen in children with grades IV and V reflux Normalization of voiding pressures explains high rate of reflux resolution in childhood

41. 排尿障礙治療中心 版權所有 Urodynamic studies in older children Up to 60% of children with reflux have urodynamic abnormality Detrusor overactivity and sphincter dyscoordination Primary sphincter overactivity is more associated with high grade reflux and renal scarring Bladder instability improves over time

42. 排尿障礙治療中心 版權所有 Pitfalls in urodynamic study in infants and children Poor cooperation of patient Appropriate size of intra-urethral catheter – 3 Fr, 5 Fr, 7 Fr? Frequent increased abdominal pressure Different infusion rate and compliance in different age Differential diagnosis of volitional voiding and detrusor overactivity

43. 排尿障礙治療中心 版權所有 Urodynamics and Clinical course of VUR Treatment of detrusor overactivity with anticholinergics improves resolution or improvement in VUR than stable bladders A higher surgery rate in stable bladder with VUR Controversy remains in correlation of urodynamic abnormalities with grades of VUR and anticholinergic treatment with resolution rate of VUR

44. 排尿障礙治療中心 版權所有 Resolution of VUR and improved DI after anticholinergic and CIC in myelomeningocele

45. 排尿障礙治療中心 版權所有 Dysfunctional elimination syndromes (DES) Children are both infrequent voiders and constipated Associated with an increased risk of urinary tract infection With or without reflux Incontinent day and night with fecal soiling Observed to engage in holding maneuver to avoid urination and defecation

46. 排尿障礙治療中心 版權所有 DES – A learned habit A learned habit acquired during toilet training Most often occur in girls Recurrent cystitis due to short urethra and bladder colonization Congenital VUR or secondary VUR due to these aberrant toilet training habits

47. 排尿障礙治療中心 版權所有 Breakthrough UTI and Dysfunctional voiding Girls with history of voiding dysfunction have higher rates of breakthrough UTI (4 times more common in DES) Unsuccessful surgical outcome was seen in children with DES Adequate hydration, timed voiding, stool softeners, laxatives, as well as anticholinergics may be helpful

48. 排尿障礙治療中心 版權所有 Voiding dysfunction without UTI Children with mono-symptomatic enuresis have a very low urodynamic abnormality VUR has been found in child with frequency urgency and urinary incontinence without history of UTI 15% of children had positive urodynamic findings and 16% had renal scarring

49. 排尿障礙治療中心 版權所有 Diurnal incontinence due to pelvic floor hypertonicity & DI

50. 排尿障礙治療中心 版權所有 Urge incontinence in a girl with dysfunctional voiding & DI

51. 排尿障礙治療中心 版權所有 Treatment of Dysfunctional voiding in Children Adequate hydration and timed voiding Stool softeners and laxatives Anticholinergics – Ditropan, tolterodine Biofeedback – pelvic floor relaxation, computerized game Intermittent catheterization Antibiotics for recurrent UTI

52. 排尿障礙治療中心 版權所有 Improved bladder compliance and DI after ditropan therapy in myelomeningocele

53. 排尿障礙治療中心 版權所有 Adequate hydration Provide adequate urine production and wash out effect of bladder Prevent constipation and reduce colonization of pathogen in perineum Reduce detrusor instability through dilution of urine and decrease urine permeability into urothelium Time voiding is required

54. 排尿障礙治療中心 版權所有 Medication for dysfunctional voiding Oxybutynin – effective in reducing detrusor overactivity, side effects of mucosal dryness & constipation Ditropan XL – elimination of peak drug effect and reduce adverse effects Tolterodine – M3 anticholinergic Alpha-adrenergic blocker to reduce urethral resistance Phenylpropanolamine, pseudoephedrine – in ISD with incontinence ready for CIC

55. 排尿障礙治療中心 版權所有 Pelvic floor rehabilitation Identification of pelvic floor muscles Regular pelvic floor muscle exercises provide adequate relaxation of pelvic floor including urethral sphincter A synergistic voiding pattern can be achieved after rehabilitation Combined with fluid and anticholinergic therapy

56. 排尿障礙治療中心 版權所有 Biofeedback for pelvic floor muscle relaxation Correcting paradoxical contractions of pelvic floor and urinary sphincter muscles with voiding Success relies on motivation of children Uroflow- surface EMG integrated biofeedback Cystometry biofeedback to inhibit detrusor overactivity in patients with DI Visual or audio biofeedback may be more successful than verbal biofeedback

57. 排尿障礙治療中心 版權所有 Biofeedback Pelvic floor muscle retraining

58. 排尿障礙治療中心 版權所有 Electrical stimulation to inhibit detrusor overactivity

59. 排尿障礙治療中心 版權所有 CMG biofeedback to inhibit Detrusor overactivty

60. 排尿障礙治療中心 版權所有 Urethral injection of Botulinum A toxin in dysfunctional voiding New technique in reducing urethral resistance by paralyzing striated urethral sphincter Intra-detrusor injection to reduce detrusor overactivity and increase bladder capacity Restoration of normal voiding pattern Repeat injection may be necessary

61. 排尿障礙治療中心 版權所有 Botulinum A toxin (Botox)

62. 排尿障礙治療中心 版權所有 Reduction of MUCP after Botulinum A toxin injection

63. 排尿障礙治療中心 版權所有 Reduction of voiding pressure after Botulinum A toxin