Neurogenic bladder

Neurogenic Bladder in children and its management

1. Neurogenic Bladder and its Management Dr . Faheem Ul Hassan Dr. Gowhar Mufti

2. Introduction The normal function of the urinary bladder is to store and expel urine in a coordinated, controlled fashion. This coordinated activity is regulated by central and peripheral nervous systems.

3. Introduction Three mixed sensory and motor nerves innervate the lower urinary tract. hypogastric pelvic and pudendal nerves

4. Introduction The hypogastric nerve carries sympathetic autonomic innervation, The pelvic nerve carries the parasympathetic autonomic innervation, Pudendal nerve carries the somatic innervation to the lower urinary tract.

5. Introduction (storage phase)

6. Introduction (voiding) Intense bladder afferent Firing through pelvic nerve to PMC PSO to Detrusor and urethral smooth muscles Inhibits pudendal outflow to EUS Detrusor contraction and EUS relaxation Micturition

7. Introduction Neurogenic bladder is a term applied to a malfunctioning urinary bladder due to neurologic dysfunction.

8. Uninhibited bladder lesions above the pontine micturition center produce an uninhibited bladder Due to reduced cortical inhibition of PMC there is Reduced awareness of bladder fullness and a Low capacity bladder

9. DSD lesions between the pontine micturition center and sacral spinal cord producing an upper motor neuron bladder Upper motor neuron neurogenic bladder dysfunction is characterized by detrusor-sphincter dyssynergia (DSD),

10. DSD In DSD simultaneous detrusor and urinary sphincter contractions occur The spinal cord damage renders the bladder and sphincters spastic , especially if lesions are above the T10 level

11. DSD The bladder capacity is usually reduced due to the high detrusor tonus ( neurogenic detrusor overactivity , or detrusor hyperreflexia ) If detrusor pressure exceeds EUS pressure in the proximal urethra, then incontinence may occur.

12. Mixed type A neurogenic Bladder In the mixed type A neurogenic bladder detrusor nucleus damage renders the detrusor flaccid ( detrusor areflexia ), The pudendal nucleus is intact & spastic producing a hypertonic EUS .

13. Mixed type A neurogenic Bladder The bladder is large and has low pressure spastic external sphincter produces urinary retention. The detrusor pressure is low so upper urinary tract damage from vesicoureteral reflux does not occur , and incontinence is uncommon.

14. Mixed type B neurogenic Bladder Flaccid EUS due to the pudendal nucleus lesion Spastic bladder due to the disinhibited detrusor nucleus. bladder capacity is low but vesicular pressures are usually not elevated This leads to problems with incontinence , however.

15. LMN neurogenic Bladder the sacral MCs or related peripheral nerves are damaged sympathetic outflow to the lower urinary tract is intact detrusor tone is low ( detrusor areflexia ) and internal urinary sphincter innervation is intact.

16. Detrusor hyperactivity with impaired bladder contractility DHIC first described in nursing home residents there is frequent but weak involuntary detrusor contractions causing incontinence & incomplete bladder emptying There is slow bladder contraction velocity, and elevated urinary residual volume

17. Types Detrusor Sphincter Over activity Over activity Inactivity Over activity Over activity Inactivity Inactivity Inactivity

18. The evaluation begins with both day and nighttime voiding patterns. Voiding symptoms can be irritative or obstructive . History and Physical Examination

19. Irritative Dysuria Urgency Frequency Obstructive Retention Hesitancy Staccato voiding Straining Feeling of incomplete voiding History and Physical Examination

20. Whether the child has had a urinary tract infection (UTI) UTI with fever ( pyelonephritis ) UTI without fever ( cystitis ) Constipation is noted lower extremity weakness or change in sensation are recorded. History and Physical Examination

21. Physical examination should include evaluation of the abdomen, back, genitalia, and lower extremities . History and Physical Examination

22. Abdominal examination should include palpation for fecaloma and distended bladder History and Physical Examination

23. Examination of the back is essential and should include Cutaneous findings of a hair patch or A dimple over the lumbosacral spine Asymmetric gluteal crease Patients who possess any of these cutaneous findings should undergo a MRI of the lumbosacral spine . History and Physical Examination

24. Examination of the male genitalia should include palpation along the entire urethra. Induration may be a sign of urethral inflammation and stricture. Position of the testicles should be noted ( cryptorchidism is common in myelodysplasia .) History and Physical Examination

25. The anus should be examined for Tone and Position. Finally, any deficits in lower extremity reflexes or motor strength should be noted History and Physical Examination

26. A urinalysis should be obtained The presence of red cells, white cells, glucose, and protein should be noted. A low specific gravity may reflect poor concentrating ability secondary to renal dysfunction . History and Physical Examination

27. Radiographic and Dynamic Assessment of Bladder Dysfunction

28. BLADDER ULTRASOUND The bladder ultrasound can provide many clues like Thickened bladder wall (UTI & BOO) Calculation of PVRU for assessing whether the patient can efficiently empty the bladder. Bladder Ultrasound

29. VOIDING CYSTOURETHROGRAPHY MCU provides information about Bladder neck, and urethral anatomy Trabeculations Diverticuli Vesicoureteral reflux is a common finding in both anatomic and functional causes of bladder dysfunction VCUG

30. The urodynamic evaluation provides indepth functional information regarding the bladder. It establishes bladder capacity, bladder filling pressures , patient’s perception of bladder filling , and the ability to empty efficiently and in proper coordination with EUS UDS

31. Neurologic modulation combined with the viscoelastic properties of the healthy detrusor muscle allow the bladder to maintain fairly constant pressure throughout the filling phase. As a result, intravesical pressures in the healthy bladder remain at or below 5 to 10 cm H2O pressure UDS

32. compliance can be adversely affected (mechanical obstruction or by U & LMN lesions) resulting in increased intravesical storage pressures. Storage pressures higher than 40 cm H2O , lead to renal injury. UDS

33. Neuropathic Causes of Bladder Dysfunction

34. Neuropathic bladder secondary to myelodysplasia Myelodysplasia , defined as abnormal development of the spinal canal and spinal cord It is the most common etiology of NB in children. Workup is done in the neonatal period , usually after the surgical closure of the defect. workup will include RBUS, urodynamic study & MCU

35. This baseline information can identify children at risk for urinary tract deterioration (poor compliant or overactive detrusor or outflow obstruction as a part of DSD.) In such cases proactive approach is necessary. Myelodysplasia

36. The neurological lesion produced by MMC can be variable . The vertebral level provides little or no clue to the exact neurologic level or lesion produced. Myelodysplasia

37. MMC influences lower urinary tract function in a variety of ways and cannot be predicted by spinal anomalies or the neurogenic function of the lower extremities. The neurologic lesion in MMC is a dynamic disease process in which changes take place throughout childhood . Myelodysplasia

38. Three categories of lower urinary tract dynamics may be detected: synergic (26%) dyssynergic with and without poor detrusor compliance (37% ) and complete denervation (36%). Myelodysplasia

39. Dyssynergy : When the external sphincter fails to decrease or actually increases in its activities during a detrusor contraction It is associated with a poorly compliant bladder with high pressures. This results in a bladder that empties only at high pressures. Myelodysplasia

40. Synergy is characterized by complete silencing of the sphincter during a detrusor contraction Voiding pressures are usually within normal range. Myelodysplasia

41. Complete denervation : is noted when no bioelectric potentials are detectable in the region of the EUS at any time during micturition cycle. Myelomeningocele

42. Within the first 3 years of life urinary tract deterioration is seen in 71% of newborn with DSD 17% of synergic children and 23% of completely denervated patients . Myelomeningocele

43. Outlet obstruction is a major contributor to the development of urinary tract deterioration. Leak point pressure and detrusor filling pressure both are significant markers to predict upper tract deterioration. Detrusor filling pressures should be maintained lower than 30 cm H2O . Myelomeningocele

44. clean intermittent catheterisation (CIC) is indicated when detrusor filling pressures exceed 30 cm H2O and voiding pressure exceeds 100 cm H2O , is necessary. Sequential urodynamic studies, on a yearly base during the first 5 years of life , provide a safe monitoring of these children. Myelomeningocele

45. Occult Spinal Dysraphisms Affects the formation of the spinal column but does not result in an open vertebral canal. In children younger than 3 months the vertebral bones have not ossified , thus a window exists for ultrasound to screen spinal cord lesions. Occult spinal dysraphism

46. 90% of children with various occult dysraphic status have cutaneous lesions . small dimple skin tag tuft of hair dermal vascular malformation, subcutaneous lipoma asymmetrically curving gluteal cleft. Occult spinal dysraphism

47. On careful inspection of the lower extremities, we can detect a high arched foot, alteration of configuration of toes, or pressure zones on the feet with atrophic lesions on the skin . Lower urinary tract function is abnormal in 40–90% of affected elder children. Occult spinal dysraphism

48. In most of the children lower extremity examination is normal Patients become symptomatic during pubertal Growth spurt During pubertal growth spurt differential growth of spinal cord and vertebral bodies produces compression of nerve roots Occult spinal dysraphism

49. Lipomeningocele , intradural lipoma , and other anomalies of filum terminale may be present. Urodynamic evaluation reveals abnormalities in one third These congenital anomalies produce different neurologic findings. Occult spinal dysraphism

50. Lipomas cause UMN lesio n alone or in combination with LMN lesion . The reason to consult a specialist may be difficulty in toilet training, urinary incontinence after an initial period of successful dryness recurrent UTI, and fecal soiling . Occult spinal dysraphism

51. In such lesions RBUS, MRI studies are needed and a full urodynamic evaluation with EMG should be done. The therapeutic measures should be discussed with a neurosurgeon . MCU is warranted when the urodynamics parameters suggest risk to the upper urinary tract Occult spinal dysraphism

52. Sacral Agenesis Defined as the partial or complete absence of lowermost vetebral bodies There may be absence of just two or three vetebral bodies or Absence of scaral and several lumbar bones These children appear normal with no lower extremity abnormality Usually failed toilet training brings the child to consult his paediatrician . Sacral agenesis

53. Patient has flat bottom , and even absent vertebrae in palpating the coccyx. The pathognomic sign is absence of the upper end of the gluteal cleft , with flattened buttocks. The diagnosis can be confirmed by a simple lateral plain x-ray of the sacral area. spinal ultrasound in infants A spinal MR reveals a sharp cut off to the cord at about T-12, with nerve roots streaming from it . Sacral agenesis

54. Urodynamic Study may show either overactive detrusor with sphincter dyssynergy (UMN) acontractile detrusor with complete denervation in the urethral sphincter (LMN) UM lesions are characterized by an overactive detrusor, exaggerated sacral reflex and DSD The VCUG shows thick trabeculated bladder with closed bladder neck. Sacral agenesis

55. Lower motor lesions show an acontractile detrusor with open bladder neck VCUG shows small thin walled bladder . Managemen t will depend mainly on the urodynamic findings and the morphological aspect on the VCUG. Sacral agenesis

56. Anorectal Malformations (ARM) Spinal cord anomalies ( tethered cord, filum terminale anomalies, and a lipoma ) may be found in up to 50% of cases of ARM, specially in high type ARM. Neurogenic bladder dysfunction is a frequent finding Bladder dysfunction can be either due to the congenital spinal anomalies or secondary to the surgical trauma .

57. Most common finding on urodynamic studies is an upper motor lesion with DSD C urrently urodynamic studies are reserved to children who have symptom s or Who have abnormalities on the simplified urodynamic studies ( flowmetry , EMG, postvoid residual ). Anorectal Malformations (ARM)

58. Cerebral Palsy Cerebral palsy is a non progressive injury of the brain occurring in the perinatal period. Most children with cerebral palsy develop total urinary control. Incontinence is a feature in some Overactive detrusor with DSD is the most common finding. Cerebral palsy

59. Most common injuries are of the cervical and high thoracic regions. The high spinal lesions will produce an UMN lesion with overactive detrusor and DSD Urodynamic studies are done after stabilisation of the spinal trauma , usually 2 months after the injury and to be repeated 6–9 months after. Traumatic Injuries of the Spine

60. sacral spinal injuries cause acontractile detrusor with inactive external sphincter . There is low risk for the urinary tract deterioration but need specific measures for incontinence. Traumatic Injuries of the Spine

61. Management of Neurogenic Bladder in children

62. Management of a child with a neurogenic bladder the main objective to preserve a normal upper urinary tract. The second main objective is to improve the social life and the quality of life of these children, giving them urinary and fecal continence

63. These goals need a reservoir (the bladder ) with adequate capacity and low storage pressure , able to empty itself with a normal micturation pressure. Management of NB

64. The management will have to be tailored according to urodynamic results and motor and intellectual capacities of the patient. Management of NB

65. to achieve a complete emptying of the bladder to maintain or restore an adequate bladder capacity and compliance , and to reinforce the sphincter outlet resistance when needed . Goals of treatment

66. Clean Intermittent Catheterization (CIC) CIC was introduced by Lapides in 1971 Remains the most important tool in the management NB. As fewer than 10% of children with congenital NB will develop satisfactory bladder control, Parents are reminded at periodic follow-ups to expect this intervention by the age of toilet training

67. Clean Intermittent Catheterization (CIC) Some authors prefer early institution of CIC because by the age of 3 years, CIC will be required in all for achieving continence, starting CIC in toddlers is difficult . CIC may be realized with classical Nelaton catheter kept in an antiseptic solution.

68. It allows a complete emptying of the bladder lowers the risk of UTI protects the upper urinary tract against high bladder pressure Is a valuable tool to keep child dry Clean Intermittent Catheterization (CIC)

69. CIC is a clean but not sterile procedure. Starting CIC will frequently result in chronic colonization of the bladder by bacteria. These bacteria are generally of low clinical significance Antibiotic use should be reserved only for a symptomatic episode of UTI & pseudomonas and proteus . Clean Intermittent Catheterization (CIC)

70. CIC should be started as soon as possible to avoid UTI and deterioration of the bladder. CIC may be realized through the urethra or through a continent cystostomy (the Mitrofanoff principle). Clean Intermittent Catheterization (CIC)

71. In cases where despite CIC urinary tract deterioration occurs, the overnight catheter drainage of the bladder may be tried. Overnight drainage may increase bladder compliance and capacity Clean Intermittent Catheterization (CIC)

72. Pharmacologic Treatment of NB Aim of the pharmacotherapy is Decrease detrusor over activity Increase bladder capacity Increase bladder outlet resistance ( in incontinence) Dercrease bladder outlet resistance (in DSD)

73. Oxybutinin remains the gold standard of treatment of bladder overactivity . It is as an anticholinergic agent acts on M3 muscarinic receptors. It decreases intravesical pressures and uninhibited contractions and indirectly increases bladder capacity . Oxybutynin

74. It also has an anti-spasmodic, local anaesthetic and Calcium channel blocking It converts overactive detrusor into an inactive reservoir Oxybutynin

75. It is administered orally in the dose of 0.2-0.4 mg/kg/day in 2-3 divided doses. side effects of altered thermoregulation and constipation. These side effects may be reduced by direct instillation of the oxybutinin in the bladder. Oxybutynin

76. Other bladder relaxant drugs include propiverine (0.8mg/kg/day), trospium , and tolterodine . The current experience with these compounds is still limited Other bladder Relaxants

77. Madersbacher et al * concluded that long-term efficacy and tolerability of propiverine in children is promising with clinically relevant improvements in key urodynamic outcomes. * Madersbacher H, Mürtz G, Alloussi S, Domurath B,Henne T, Körner I et al. Propiverine vs oxybutynin for treating neurogenic detrusor overactivity in children and adolescents: results of a multicentre observational cohort study. BJU Int 2009;103:776-81. Propiverine

78. Another study evaluated long-term efficacy and safety of tolterodine in 30 children with neurogenic detrusor overactivity . They concluded that tolterodine was effective and well tolerated in children with neurogenic detrusor overactivity *Reddy PP, Borgstein NG, Nijman RJ, Ellsworth PI. Longterm efficacy and safety of tolterodine in children with neurogenic detrusor overactivity . J Pediatr Urol 2008;4:428-33. Tolterodine

79. α - Blocker For children with DSD, one may try alpha-blocker therapy such as tamsulosin , prazosin or doxazosin . These have achieved success, are low risk to try, They can be followed for effectiveness by symptomatic improvement , as well as with a noninvasive uroflow study with postvoid residue

80. Antibiotics The problem of daily antibiotics is the risk of developing antibiotic-resistant organisms Low dose, low efficacy antibiotics such as cotrimoxazole and nitrofurantoin should be used in an alternative fashion for prophylaxis and High-efficacy full dose drugs are used for an active infection.

81. Botulinum toxin A BOTOX results in decreased muscle activity by blocking the release of acetylcholine from the neuron This effectively weakens the muscle for a period of three to four months It increases bladder capacity , decreased pressures, decreased incidence of reflux and improves continence .

82. Botulinum toxin A is injected in the detrusor muscle under cystoscopic guidance 5 IU/kg body weight with a maximum dose of 300 IU. The sites of injection are spread through the bladder except the trigone not to induce reflux. The effect lasts between 6 to 9 months . This minimally invasive procedure, may be repeated. Botulinum toxin A

83. It is useful to consider strategies of scheduled evacuation. This includes healthy Toilet trainaing dietary fiber intake and a daily stool softener , coupled with a method of daily evacuation to achieve effective bowel management. Bowel management Mineral Oil 2-4 ml/kg/day Milk of Magnesia 1-3 ml/kg/day Polyethylene glycol 0.5-1.5 g/kg/day Sorbitol or lactulose 1-2 ml/kg/day

84. A number of surgical options are available should a combination of pharmacotherapy and CIC prove inadequate in halting the progression of urinary tract damage. It is imperative that one must have a mechanism of satisfactory bladder emptying. Bladder Augmentation

85. The goal of bladder augmentation is to protect the upper urinary tract when the less invasive procedures are ineffective, and to induce dryness in children where the incontinence is related to low compliance and capacity. . Bladder Augmentation

86. Bladder Augmentation Bladder augmentation should only be realized in a patient when CIC is impossible by the urethra, sensitive or technically impossible Bladder augmentation is usually kept low on the list of surgical options due to long-term morbidity of these procedures Bladder Augmentation

87. Bladder Augmentation The augmentation induces many complications Infections stone formation metabolic complications perforation and cancer. Bladder Augmentation

88. Bladder Augmentation Enterocystoplasty and clam cystoplasty are good alternatives to autoaugmentation when the surgeon is faced with a small, scarred bladder. It is often associated with the construction of a catheterizable channel ( Mitrofanoff principle) Bladder Augmentation

89. Bladder Augmentation The choice of surgery leans towards continent urinary diversion and bladder augmentation in the event of intractable incontinence , diminishing renal functions and hypertension following long-term renal damage. Bladder Augmentation

90. Bladder Augmentation Neuromodulation therapy aims to treat the abnormal innervation of the bladder It tries to "re-train" the nerve-muscle interaction to attain more normal bladder function. Neuromodulation

91. Bladder Augmentation The available treatments include transurethral electrical bladder stimulation implantation of a sacral neuromodulation pacemaker device ; and operative procedures that reconfigure sacral nerve root anatomy Neuromodulation

92. Bladder Augmentation Sacral neuromodulation by a reversible implantable device ( Medtronic,USA ) , is thought to improve bladder function either by consistent stimulation of the efferent fibers of the sacral nerve roots or by providing rhythmic contractions of the pelvic floor Neuromodulation

93. Bladder Augmentation Another method of counteracting a small, hyperactive bladder is the intradural transection of the S2-S4 nerve roots Guys et al studied the results of sacral nerve stimulation (SNS) therapy in children with neurogenic bladder dysfunction. They did not find significantly better results in the SNS group Neuromodulation

94. Bladder Augmentation In case of Incompetent Bladder Outlet , bladder neck procedures are the way to achieve continence. Agents like Durasphere (zirconium oxide beads) & Deflux ( hyaluronic acid polymer) have been employed for this purpose The main disadvantage with this approach is the difficulty in CIC which follows . Bladder Neck Bulking Agents

95. Bladder Augmentation Neel et al have introduced the concept of TEM in children with noncompliant NB to address continence and treatment of VUR. Ten children with NB were treated with cystoscopic injection of botulinum-A toxin in the detrusor and subureteric injection of Deflux . There was significant increase in BC and decrease in max. detrusor pressure . VUR was successfully treated in 15 of 16 ureters Total endoscopic management (TEM)

96. Outlet Resistance In other patients the outlet resistance may be obtained by the open cervicoplasty sling suspension of the bladder neck artificial urinary sphincter bladder neck closure with mitrofanoff .

97. Bladder neck sling is considered by many as the procedure of choice in females and it has also been advocated in male patients by some . The sling is made of autologous material in most cases (rectus fascia). Castellan et al. report good continence with a mean follow-up of 4 years in 58 patients.

98. Artificial Urinary Sphincter (AUS ) is considered by many as the procedure of choice in boys when a reinforcement of outlet resistance is needed to gain adequate continence. Continence rates between 80% and 90 % are reported among children. AUS may be associated with erosion, sepsis or dysfunction.

99. Children with AUS undergo many procedures due to device technical problems . In one French multi-institutional study 63/107 children had to be re-operated on and 42 of them more than once. Most do not recommend implantation of an AUS device before puberty

100. , Bladder neck closure was initially proposed by Mitrofanoff in the management of children with a neuropathic bladder. it exposes the bladder and upper urinary tract to a high risk of deterioration in case of bad compliance to CIC.

101. Procedures Increasing the Outlet Resistance Any of these procedures imply the risk of bladder and upper urinary tract deterioration with renal insufficiency . A close follow-up is mandatory to detect any change in the urodynamic profile and any dilation of the upper urinary tract.

102. The management of a child with a neurogenic bladder needs a multidisciplinary team Treatment and has to be tailored to each child with respect to his familial environment , and his orthopedic and or mental limitations . Conclusion

103. CIC is the most important part of the treatment and should be started as early as possible after birth and repair of the spinal defect. It will not always be sufficient to gain continence but it is mandatory to protect the upper urinary tract which is the main goal of the treatment . Conclusion

104. Thank you