1. CKD & OBSTRUCTIVE
UROPATHY
Jan23-24 2012@PNAN, Ile-Ife
Felicia Eke, MD
University of Port Harcourt
Nigeria

2. Outline
 1. Definition CKD & Obstr Uropathy
 Mechanism Obstr.Urop®CKD
 Case presentations
 Quiz
 Management

3. Definition-Obstructive Uropathy
 Obstruction anywhere
down the Urinary tract

4. Stages of CKD—KDOQI, 2002
Stag
e
Description GFR
ml/min/1.73m2
1 Kidney
damage* with
nl or GFR
³90
2 Kidney
damage* with
mild GFR
60-89
3 Moderate
GFR
30-59
**
**
**
**
4 Severe GFR 15-29
5 Kidney failure 15 or dialysis
*Kidney damage for 3 months, as defined by pathologic abnormalities or
markers of kidney damage, including abnormalities in blood or urine or
diagnostic imaging studies.

5.  A: MECHANICAL OBSTRUCTION
 B: NON-MECHANICAL- FUNCTIONAL

6. Mech:Developmental
Anomalies
 Posterior Urethral Valves
 Pelvi-ureteric Junction
Obstruction
 Ureteric Stricture
 Ureterocoele
 Tumours-
Rhabdomyosarcoma
 Ureteric Bladder
stones- Cystinuria
 Meatal stenosis
 Distal urethral
stenosis
 Cystic
malformations,
 Duplex
 Dysplastic Kidneys

7. Mech: Acquired Anomalies
 Tumours
 Bladder ureteric stones- Melamine
stones from Infant formula

8. Functional: Damaged
kidneys®CKD
 Vesico-Ureteric Reflux
 Spina Bifida
 Myelomeningocoele
 Neurogenic bladder eg
from Prune belly etc

9. PUV: Case 1-PO
 One of v. few success
stories of PUV
 Noted at birth to have
poor urine stream
 Big abdomen-bladder
 Both kidneys enlarged

12. Mechanism by which
Obstr®CKD

13. Posterior Urethral valves(PUV)
Entire urinary tract develops in environment
of high intraluminal pressure from
Obstruction ®Cell apoptosis, inflammation
Permanent defects - parenchymal dysplasia
of kidneys, ureters, bladder despite adequate
decompression of the urinary tract pre or post
natally

14. PATHOPHYSIOLOGY (cont)
 In some, renal tubules mainly affected® poor
urine concentrating ability, ® diuresis ®
ureteral bladder dysfunction
 Affected kidneys may function well initially,
but have a reduced renal reserve
 Renal deterioration also frm chronic
pyelonephritis from Vesicoureteric (V-U)
reflux, urinary stasis

15. PUV Clinical Presentation
 Extremely variable
 Prenatal US D:Oligohydramnios
hyroureters, hydronephrosis
 Respiratory distress-pulmonary hypoplasia
 Potter’s facies, ascites, and abdominal
swelling
 Birth: Poor, dribbling urine stream
 So check urine stream of all males b/4
discharge from hospital

16. PUV Clinical Presentation
 Toddlers - voiding dysfunction or UTI
 School-aged boys usually come to the
clinician's attention because of urinary
incontinence
 All enuretics should be screened at least
with abdominal palpation urine MCS

17. Note Hypertrophied bladder neck and dilated
posterior urethra proximal to valve narrowing.

18. Anteroposterior view of the abdomen during a void cystourethrographic study.
This image demonstrates a dilated bladder with trabeculation, diverticula, and
massive reflux.

19. Lateral view of a voiding cystourethrographic study during voiding after catheter removal.
The dilated posterior urethra is highly suggestive of a posterior urethral valve, which is
seen as the nonopacified line that separates the dilated posterior urethra from the normal-caliber
distal urethra.

20. Ectopic Ureter
•Continuous
urinarydribbling
vs intermittent
•Dry when
supine with
vaginal pooling

21. Non-Mechanical Obstruction
 Non-Obstructed ‘Obstructed’

22. 22

23. Prune Belly Syndrome`
 Triad of
 1. Weak abdominal muscles
 2 Undescended testes (cryptorchidism)
 3. Urinary tract problems- atonic
ureters® End Stage Renal Failure
 Formerly didn’t survive; Now do with
EARLY SURGERY to correct abd.
muscles

24. DysplasticKidneys

25. 3

26. Bladder Extrophy
 Bladder is turned inside out“; Lr portion
fails to form correctly
 Urethra genitalia not formed completely
(epispadias)
 Anus vagina appear anteriorly displaced
 Pelvic bones widely separated –diastasis
 Rx: Series of Surgical Operations

27. 8

28. Myelomeningocoele
 Myelomeningocele - backbone and spinal
canal not close before birth. Associated :
 1. Hydrocephalus in 90%
 2. Big bladder- atonic bladder
 3. Anus: Patulous: lack bowel control
 4.Legs Partial or complete paralysis of
legs loss of sensation
28

29. 5

30. Myelomeningocoele Rx
 Parents fully aware BEFORE BIRTH
 Surgery to repair defect at an early age
 3.V-P shunt for hydrocephalus
 4.Antibiotics 4 meningitis or UTI
 5.Wheel chair
 6. Bladder catheterization, Rx CRF- DT
30

31. Rhabdomyosarcoma-bladder
masses

32. 15 yr Survey of 45 CRF,PH
Primary renal dis. No.(%) Male Female
Glomerulopathies 23 (53.3) 9 14
Obstructive
uropathy
13 (28.9) 13 0
Vascular,PN,Mali
gn,Malaria
Nephropathy
9 (17.8) 5 4
Anochie I, Eke F.Ped Nephrol 2003;18:692-5

33. Yearly Incidence ESRF
UPTH,Nigeria
2.1
2.5
3
13
1985-91
1986-91
1991-2000
2007-2010
Red X, Sth Africa
EkeF.NigMedPract1992;35:37 EkeF,EkeN:PedNephrol1994;8:383-386
AnochieI,EkeF:PedNephrol2003:18:692-695
Dysplasia
PUV
PCKD
REFLUX
GN
FSGS
SLE
Other

34. Approach to preventing
Deterioration in CKD
 A: Surgical
 In Utero endoscopic valve ablation or vesico-amniotic
shunting
 Endoscopic resection of valves 2-3 days of
birth
 Operation should be done by the most
experienced. Valvotomy in UCH-OPD.
Catheter few hrs post Op
 Severe valves may need vesicostomy or
ureterostomy; Bladder augmentation later

35. Mechanical Obstruction only
one Aspect
 B: Medical
 Immediate catheterizaton-size 3 Foleys
fluid electrolyte Rx
 Long term monitoring of renal bladder
function
 Dysplastic kidneys, Ureters Bladder
 These not often respond to the mechanical
relief of Obstruction

36. Bladder Renal Care Vital
 Intermittent catheterization by patient or
parents often an integral part of Rx
 Yearly U/S- continued or recurrent stasis
 Counselling re avoidance of UTI stasis
 Yearly UE Cr, if renal function normaltests
for Renal Osteodystrophy, bone mineral
density, DMSA scan etc
 Pts have lost their Txpt. Kidney from poor
bladder management
 Avoid constipation

37. Bladder sphincter
dysfunction
Recurrent UTIs
V U R
Bowel
dysfunction
Bowel dysfunction is often part of the constellation;
Dysfunctional elimination syndrome)

38. Aims of CRF Management
 feel normal - well
 be normal - like your friends
- intellectual development
- school / other activities
 maintain normal growth
 preserving normal family functioning
 slow progression to ESRF
 prepare for ESRF treatment

39. CRF Management Points
 nutrition
 fluid and electrolyte
balance
 acid base status
 renal osteodystrophy
 infection
 anaemia
 growth
 hypertension
 preservation of renal
function
 education and
preparation
 social /psychological
support

40. Renal osteodystrophy
Treatment
 hydroxylated vitamin D therapy
- 1a hydroxycholecalciferol
- 1-25dihydroxycholecalciferol
 by daily oral administration

41. Counselling
 Surgery NOT A CURE
 Long term Follow Up
 Clearly told Paediatrician or Nephrol for
Life
 Urological Follow Up may also be for Life
 Counsel against ‘It is not my portion’
 Multidisciplinary Counselling best

42. Despite all these, MOST
PROGRESS to ESRF

43. Obstetrician
U/S
PaedNephrologist PaedUrologist
Neonatologist
Parents PaedNurses

44. Paed Nephrologist
Vascular/Transplant ±Urologist
Surgeon
Dieticians
Teachers
Psychiatrists PlayTherapists
Social WorkersAdult Nephrologist Churches

45. End Stage Renal Disease in
PUV

 100% 90% 45%
 1919 1990 2011

46. Survival in PUV
25% 1919
60% 90%
1990 2011

47. QUIZ
 On presentation 8days
after birth, this patient’s
serum creatinine is 500
Umol
 The management should
be
 1. Urgent peritoneal
dialysis
 2.Urgent haemodialysis
 3. Something else

48. QUIZ
 Can a bladder
rhbdomyosarcoma cause
this anomaly?
 What is the likely cause?

49. QUIZ
 True or False
 All children with Posterior Urethral
valves have Chronic Kidney Disease

50. MEMBERSHIP AFPNA

51. Learn more about afpna its
members by visiting the
website www.afpna.com
 PNAN integral part of AFPNA her
Mtgs are advertised there

52. Officers
Secretary-General Felicia Eke, Nigeria
Secretary-Treasurer Bashir Admani, Kenya
Member IPNA Councillor Udai Kala, South Africa
Member IPNA Councillor Safaa Medani, Sudan (North africa)
Member Priya Gajjar, South Africa
Member Francis Lalya, Benin
Congress President Elect Sampson Antwi, Ghana
Ex-Officio Mignon Mc Culloch

53. Dates of Annual Meetings
 3yrly Mtg –
 5th Congress Ghana Feb 20-23, 2013
 4th Nairobi March19-21, 2010
 3rd Cape Town, March 2006
 2nd Port Harcourt, Nigeria 2002
 1st Cairo, Egypt 2000

54. IPNA Fellows
Dr Franca Ikimalo Port Harcourt, Nigeria CT, SA 1.9 03 – 29.2.04
DrAmos Odit Uganda CT, SA 1.3.04 –31.8.04
Dr.Godson Achugwo Owerri,Nigeria CT, SA 05-10.05
DrFrancis Lalya Cotonou,Benin CT, SA 14.10.05–30.4.06
Again 12.08-12.09
Dr Hemed Twahir Mombasa,Kenya CT, SA 1.4.06 –5.3.07
again in 2009
Dr.Bashir Admani Nairobi,Kenya CT, SA 8.06-8.08
Dr.Isaac Ocheke Jos, Nigeria CT,SA 7.3.07 – 2.08
Dr Sampson Antwi Kumasi,Ghana CT, SA 4/09-4.10
Dr Seyi Oniyangi Abuja,Nigeria Joburg,SA 9.08-2.09
Dr Chris Esezobor Lagos, Nigeria
Dr David Galgallo Kenya
Dr Elena Naicker Durbar, SA Joburg,SA 5-12.09
Dr Odetunde Enugu, Nigeria CT, SA 3.11
Dr NosaIdurionye Benin, Nigeria CT, SA 6.11-
Total 14

55. IPNA Discount Membership
Fee for Regional Societies
 IPNA at its recent Council Meeting in Sao
Paulo, Brazil (October 25-26th 2011) offers
discounted membership fee to members of
its affiliated Regional Societies, namely the
ASPN, ESPN, African Pediatric Nephrology
Association (AFPNA), AsPNA,
JSPN,ANZPNA ALANEPE

56. IPNA Discount Membership
Fee for Regional Societies
 The current membership fee is US$175/yr
 The discounted membership fee for Regional
Society members is US$50/yr® privileges
 1. IPNA newsletter
 2. On-line access to Ped Nephrol Journal
 3. On-line access to Ped Nephrol textbook
 4. IPNA Congress member discount
 5. Print version of Pediatric Nephrology at an
additional rate of US$65 annually (total
US$115 annually)

57. IPNA Discount Membership
Fee for Regional Societies
 Individual paid-up members of the Regional
Soc are eligible 4 discounted membership.
Combined fee administered either by IPNA
or the Regional Soc accordingly. thus:
 ASPN: US$300+US$50 (USA)
 US$150+US$50 (Canada)
 ESPN: US$70 (Euro$50) + US$50
 JSPN: US$135 (Y10,000) + US$50
 AsPNA: US$10 + US$50
 ANZPNA: US$105 (Aust$100) + US$50

58. IPNA Discount Membership
Fee for Regional Societies
 AFPNA: US$25 + US$50
 For fees paid through IPNA, the currency will
be in USD. IPNA will then transfer the Reg
Soc dues accordingly at the end of each
financial year. The fee for wire transfer will be
charged to each Reg Soc accordingly. The
IPNA secretariat will only deal with the
payment of full fees for Reg Soc. Discounted
fees for Reg Soc should be dealt with by the
respective Reg Soc.

59. IPNA Discount Membership
Fee for Regional Societies
 For fees paid through the Reg Soc the
Regional Societies can charge in the
appropriate currency, but the corresponding
total sum should be paid to IPNA in USD. The
Regional Society must send the appropriate
membership information to IPNA in order to
effect the membership privileges.
 The discounted membership fee will be
implemented from January 1st 2012

64. Management of neuropathic
bladder
Aims:
 Preserve renal function
 Improve continence
Methods:
 Conservative/ medical management
 Surgical
 Failure of above methods to prevent upper
tract dilation/ongoing renal damage
 Unacceptable level of incontinence using
conservative means

65. Cornerstone of management
 Clean intermittent catheterisation
(CIC)
 4 X daily
 Anticholinergics (counteract detrusor
overactivity)
 Oxybutynin
 Tolterodine
 Early intervention. Prevents
progressive detrusor damage with
loss of compliance and later
requirement for cystoplasty.

66. Urodynamics

67. Indications for urodynamics
 Spinal cord pathology/features of neuropathic
bladder
 Anorectal malformations
 Infravesical obstruction (PUVs)
 Unresolving VUR with renal scarring
 Voiding dysfunction/incontinence failing to
respond conventional measures
 NB** Should only be undertaken
with a clinical implication in mind.

69. UURROODDYYNNAAMMIICCSS
vesical
abdominal
detrusor-= ves - abd

70. Urodynamic Features associated
with renal deterioration
 Detrusor sphincter dyssynergia
 High filling/voiding pressures
 Decreased bladder compliance
 Leak point pressure40 cm
H2O

71. PD Nursing Team
PATIENT
Ward Nursing
Team
Administratio
n of fluid
deliveries
Medical
Team
Social
Worker Counsellor
Dietitian
The Multidisciplinary
Team

72. COMPLICATIONS
 In the newborn
 Pulmonary hypoplasia secondary to intrauterine renal
dysfunction and Oligohydramnios is the primary cause of patients
death.
 Other complications of PUV are generally secondary to chronic
bladder changes, leading to elevated detrusor pressures. This, in
turn, leads to progressive renal damage, infection, and
incontinence.
 Renal insufficiency
 Due to renal dysplasia
 Elevated detrusor pressure and back flow of urine.

73. COMPLICATIONS (cont)
Vesicoureteral reflux
 Commonly associated with PUV and is present in as many as one
third of patients
 Vesicoureteral reflux in most children is believed to be due to an
abnormal insertion of the ureter into the bladder.
 When associated with PUV, reflux is generally secondary to
elevated intravesical pressures.
Urinary tract infections
 Recurrent UTIs are common in patients with PUV.
 Elevated intravesical pressures predispose patients to infection,
possibly by altering urothelial blood flow.
 Additionally, patients with PUV may have elevated post void
residual urine volumes, leading to stasis of urine.
 Dilated upper urinary tracts, with or without vesicoureteral reflux,
further elevate UTI risk.

74. COMPLICATIONS (cont)
Bladder dysfunction
 Thought to be caused by alterations in collagen deposition and the
development of detrusor smooth muscle cells.
 In mild cases, incontinence may be present;
 In severe cases, ongoing deterioration of renal function occurs.
 Bladder dysfunction often improves over time after definitive
treatment of the obstruction.
End Stage Renal Disease( ESRD)
 Progression of ESRD is accelerated at the time of puberty due to
the increased metabolic workload placed on the kidneys.

75. PROGNOSIS
 Over the last 30 years, the prognosis of children with PUV has steadily
improved.
 In the past, most children were found to have PUV only after presenting with
urosepsis or progressive renal insufficiency. Older series demonstrated mortality
rates approaching 50% by late adolescence.
 Today, most individuals with PUV are discovered when prenatal ultrasonography
reveals hydronephrosis.
 Historically, of patients with adequate pulmonary function, approximately 25%
died of renal insufficiency in the first year of life, 25% died later in childhood, and
50% survived to adulthood with varying degrees of renal function.
 Prompt resolution of bladder obstruction, aggressive treatment of bladder
dysfunction, and improved surgical techniques have lowered the neonatal
mortality rate to less than 3%.
 Approximately one third of patients progress to renal insufficiency in their
lifetimes. Improved dialysis and transplantation techniques have significantly
improved not only the mortality rate for these children but also their quality of life.
.

76. PROGNOSIS
 Additionally, medical and surgical management can achieve urinary
continence in nearly all patients
 An interesting group of patients are those with vesicoureteral reflux
dysplasia (VURD) syndrome.
 In these patients, one kidney is hydronephrotic, nonfunctioning, and
has high-grade vesicoureteral reflux. The high-grade reflux is thought
to act as a pop-off valve, leading to reduced overall bladder pressures
and preservation of contralateral renal function.
 In the past, these patients were thought to have a better outcome due
to preserved renal function in one kidney at the sacrifice of the other.
 More recent data suggests that, although short-term serum creatinine
levels may be favorable, these patients may suffer long-term adverse
renal function with hypertension, proteinuria, and renal failure In the
long.

77. CONCLUSION
 Posterior urethral valve though not a
common congenital anomaly can be fatal
 Early detection, prompt management and
routine follow up of patients is needed for
a good life expectancy.

78. INVESTIGATIONS (cont)
Nuclear Renography
 Detects upper-tract consequences of obstruction
 Absent or dysplastic kidney ® photopenic area in
renal fossa
 Altered renal function ® Delayed visualization of
renal unit with slow rise to peak activity

79. TREATMENT (cont)
Vesicostomy
 When urethral size precludes safe valve
ablation, a communicating channel between
the bladder and lower abdominal wall (ie,
vesicostomy) can be created to provide bladder
drainage
 Generally, an 18-20F stoma is created
approximately midway between the pubis and
umbilicus in the midline
 Take care to bring the dome of the bladder to
the skin and to limit the stomal size to prevent
prolapse of bladder urothelium through the
vesicostomy
 Its use has decreased because most patients
can be safely drained and can undergo valve

80. TREATMENT (cont)
Cutaneous ureterostomies:
 Bilateral cutaneous ureterostomies can also be
placed to provide for urinary drainage
 Techniques for cutaneous ureterostomy
include:
-End stomal ureterostomy,
-loop ureterostomy,
-Y-ureterostomy (in which the ureter is
divided and one end is brought to the skin and
the other is reanastomosed in a uretero-ureterostomy),
and
-Ring ureterostomy techniques
 Potential complications of cutaneous
ureterostomies include ureteral
devascularization, inadequate drainage, and

81. TREATMENT (cont)
Secondary bladder surgery
Augmentation cystoplasty
 Indications for bladder augmentation include :
-Inadequately low bladder storage volumes
-High bladder pressures despite anticholinergic
medication and clean intermittent catheterization.
 The ileum is most commonly used; however, large bowel,
stomach, and ureter are also used, depending on clinical
conditions and surgeon preference.
 Augmentation should only be offered to patients willing to
commit to lifelong intermittent catheterization.

82. TREATMENT (cont)
 Potential complications include
-Bladder rupture (approximately 10% of patients);
-Electrolyte disturbances, which may be worsened by the
placement of intestinal mucosa in contact with urine.
-Several cases of malignant degeneration in augmented
bladder have been reported.
 Despite these risks, augmentation can significantly improve
patient lifestyle in those who have intractable incontinence
due to poor compliance and bladder over activity.
Continent Appendicovesicostomy :
 Also called the Mitrofanoff technique,
 Involves placement of a non refluxing tubular conduit for
catheterization between the bladder and skin to provide an alternative
channel for catheterization.

83. TREATMENT (cont)
 Potential complications include
-Bladder rupture (approximately 10% of patients);
-Electrolyte disturbances, which may be worsened by the
placement of intestinal mucosa in contact with urine.
-Several cases of malignant degeneration in augmented
bladder have been reported.
 Despite these risks, augmentation can significantly improve
patient lifestyle in those who have intractable incontinence
due to poor compliance and bladder over activity.
Continent Appendicovesicostomy :
 Also called the Mitrofanoff technique,
 Involves placement of a non refluxing tubular conduit for
catheterization between the bladder and skin to provide an alternative
channel for catheterization.