Transcript CKD&ObstrUropathyPNAN,IfeJan2012

CKD & OBSTRUCTIVE UROPATHY
Jan23-24 2012@PNAN, Ile-Ife
Felicia Eke, MD
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University of Port Harcourt
Nigeria
Outline
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1. Definition CKD & Obstr Uropathy
Mechanism Obstr.UropCKD
Case presentations
Quiz
Management
Definition-Obstructive Uropathy
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Obstruction anywhere
down the Urinary tract
Stages of CKD—KDOQI, 2002
Stage
Description
GFR
ml/min/1.73m2
1
90
4
Kidney
damage* with
nl or GFR
Kidney
damage* with
mild GFR
Moderate
GFR
Severe GFR
5
Kidney failure
2
3
**
60-89
**
30-59**
**
15-29
<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.
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A: MECHANICAL OBSTRUCTION
B: NON-MECHANICAL- FUNCTIONAL
Mech:Developmental
Anomalies
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Posterior Urethral Valves
Pelvi-ureteric Junction
Obstruction
Ureteric Stricture
Ureterocoele
TumoursRhabdomyosarcoma
Ureteric & Bladder
stones- Cystinuria
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Meatal stenosis
Distal urethral
stenosis
Cystic
malformations,
Duplex
Dysplastic Kidneys
Mech: Acquired Anomalies
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Tumours
Bladder & ureteric stones- Melamine
stones from Infant formula
Functional: Damaged
kidneysCKD
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Vesico-Ureteric Reflux
Spina Bifida
Myelomeningocoele
Neurogenic bladder eg
from Prune belly etc
PUV: Case 1-PO
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One of v. few success
stories of PUV
Noted at birth to have
poor urine stream
Big abdomen-bladder
Both kidneys enlarged
Mechanism by which
ObstrCKD
Posterior Urethral valves(PUV)
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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
PATHOPHYSIOLOGY (cont)
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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
PUV Clinical Presentation
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Extremely variable
Prenatal US :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
PUV Clinical Presentation
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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
Note Hypertrophied bladder neck and dilated
posterior urethra proximal to valve narrowing.
Anteroposterior view of the abdomen during a void cystourethrographic study.
This image demonstrates a dilated bladder with trabeculation, diverticula, and
massive reflux.
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 normalcaliber distal urethra.
Ectopic Ureter
•Continuous
urinarydribbling
vs intermittent
•Dry when
supine with
vaginal pooling
Non-Mechanical Obstruction
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Non-Obstructed ‘Obstructed’
22
Prune Belly Syndrome`
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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
DysplasticKidneys
3
Bladder Extrophy
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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
8
Myelomeningocoele
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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
5
Myelomeningocoele Rx
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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
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Rhabdomyosarcoma-bladder
masses
15 yr Survey of 45 CRF,PH
Primary renal dis.
No.(%)
Glomerulopathies
23 (53.3) 9
14
Obstructive
uropathy
13 (28.9) 13
0
Vascular,PN,Malig
n,Malaria
Nephropathy
9 (17.8)
4
Anochie I, Eke F.Ped Nephrol 2003;18:692-5
Male Female
5
Yearly Incidence ESRF
UPTH,Nigeria
Red X, Sth Africa
Dysplasia
2.1
2.5
13
3
PUV
1985-91
PCKD
1986-91
REFLUX
1991-2000
GN
2007-2010
FSGS
SLE
Other
EkeF.NigMedPract1992;35:37 EkeF,EkeN:PedNephrol1994;8:383-386
AnochieI,EkeF:PedNephrol2003:18:692-695
Approach to preventing
Deterioration in CKD
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A: Surgical
In Utero endoscopic valve ablation or vesicoamniotic 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
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
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Bladder & Renal Care Vital
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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 U&E & 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
Recurrent UTIs
VUR
Bladder sphincter
dysfunction
Bowel
dysfunction
Bowel dysfunction is often part
(Dysfunctional elimination synd
Aims of CRF Management
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feel normal
be normal
- well
- like your friends
- intellectual development
- school / other activities
maintain normal growth
preserving normal family functioning
slow progression to ESRF
prepare for ESRF treatment
CRF Management Points
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nutrition
fluid and electrolyte
balance
acid base status
renal osteodystrophy
infection
anaemia
growth
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hypertension
preservation of renal
function
education and
preparation
social /psychological
support
Renal osteodystrophy
Treatment
 hydroxylated vitamin D therapy
- 1 hydroxycholecalciferol
- 1-25dihydroxycholecalciferol
 by daily oral administration
Counselling
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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
Despite all these, MOST
PROGRESS to ESRF
U/S
PaedNephrologist
Parents
Obstetrician
PaedUrologist
Neonatologist
PaedNurses
Paed Nephrologist
±Urologist
Vascular/Transplant
Surgeon
Dieticians
Teachers
Psychiatrists
Social Workers Adult Nephrologist
PlayTherapists
Churches
End Stage Renal Disease in PUV
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100%
1919
90%
1990
45%
2011
Survival in PUV
25%
1919
60%
1990
90%
2011
QUIZ
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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
QUIZ
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Can a bladder
rhbdomyosarcoma cause
this anomaly?
What is the likely cause?
QUIZ
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True or False
All children with Posterior Urethral
valves have Chronic Kidney Disease
MEMBERSHIP AFPNA
Learn more about afpna & its
members by visiting the
website www.afpna.com

PNAN integral part of AFPNA & her Mtgs
are advertised there
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
Dates of Annual Meetings
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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
IPNA Fellows
Dr Franca Ikimalo
DrAmos Odit
Port Harcourt, Nigeria
Uganda
CT, SA
CT, SA
1.9 03 – 29.2.04
1.3.04 –31.8.04
Dr.Godson Achugwo
DrFrancis Lalya
Owerri,Nigeria
Cotonou,Benin
CT, SA
CT, SA
05-10.05
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
Dr Chris Esezobor
Dr David Galgallo
Abuja,Nigeria
Lagos, Nigeria
Kenya
Joburg,SA
9.08-2.09
Dr Elena Naicker
Dr Odetunde
Dr NosaIdurionye
Durbar, SA
Enugu, Nigeria
Benin, Nigeria
Joburg,SA
CT, SA
CT, SA
5-12.09
Total 14
3.11
6.11-
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Combined fee administered either by IPNA or
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The discounted membership fee will be
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Management of neuropathic
bladder
Aims:
 Preserve renal function
 Improve continence
Methods:
 Conservative/ medical management
 Surgical
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Failure of above methods to prevent upper
tract dilation/ongoing renal damage
Unacceptable level of incontinence using
conservative means
Cornerstone of management
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Clean intermittent catheterisation
(CIC)
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Anticholinergics (counteract detrusor
overactivity)
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4 X daily
Oxybutynin
Tolterodine
Early intervention. Prevents
progressive detrusor damage with loss
of compliance and later requirement
for cystoplasty.
Urodynamics
Indications for urodynamics
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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.
URODYNAMICS
vesical
abdominal
detrusor-= ves - abd
Urodynamic Features associated
with renal deterioration
Detrusor sphincter dyssynergia
 High filling/voiding pressures
 Decreased bladder compliance
 Leak point pressure>40 cm H2O
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The Multidisciplinary
Team
PD Nursing Team
Ward Nursing
Team
Administratio
n of fluid
deliveries
Medical Team
PATIENT
Dietitian
Social
Worker
Counsellor
COMPLICATIONS
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In the newborn
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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
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Due to renal dysplasia
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Elevated detrusor pressure and back flow of urine.
COMPLICATIONS (cont)
Vesicoureteral reflux
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Commonly associated with PUV and is present in as many as one
third of patients
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Vesicoureteral reflux in most children is believed to be due to an
abnormal insertion of the ureter into the bladder.
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When associated with PUV, reflux is generally secondary to
elevated intravesical pressures.
Urinary tract infections
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Recurrent UTIs are common in patients with PUV.
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Elevated intravesical pressures predispose patients to infection,
possibly by altering urothelial blood flow.
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Additionally, patients with PUV may have elevated post void
residual urine volumes, leading to stasis of urine.
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Dilated upper urinary tracts, with or without vesicoureteral reflux,
further elevate UTI risk.
COMPLICATIONS (cont)
Bladder dysfunction
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Thought to be caused by alterations in collagen deposition and the
development of detrusor smooth muscle cells.
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In mild cases, incontinence may be present;
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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)
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Progression of ESRD is accelerated at the time of puberty due to the
increased metabolic workload placed on the kidneys.
PROGNOSIS
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.
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.
PROGNOSIS
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Additionally, medical and surgical management can achieve urinary
continence in nearly all patients
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An interesting group of patients are those with vesicoureteral reflux
dysplasia (VURD) syndrome.
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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.
CONCLUSION
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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.
INVESTIGATIONS (cont)
Nuclear Renography
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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
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
TREATMENT (cont)
Cutaneous ureterostomies:
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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 ureteroureterostomy), and
-Ring ureterostomy techniques
Potential complications of cutaneous
ureterostomies include ureteral
TREATMENT (cont)
Secondary bladder surgery
Augmentation cystoplasty
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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.
TREATMENT (cont)
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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:
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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.
TREATMENT (cont)
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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:
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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.