ckd & obstructive uropathy jan23-24 2012@pnan, ile-ife felicia eke, md university of port...
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CKD & OBSTRUCTIVE UROPATHY
Jan23-24 2012@PNAN, Ile-Ife
Felicia Eke, MD
University of Port HarcourtNigeria
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Outline 1. Definition CKD & Obstr Uropathy Mechanism Obstr.UropCKD Case presentations Quiz Management
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Definition-Obstructive Uropathy
Obstruction anywhere down the Urinary tract
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Stages of CKD—KDOQI, 2002 Stage Description GFR
ml/min/1.73m2
1 Kidney damage* with nl or GFR
90
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.
**
**
****
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A: MECHANICAL OBSTRUCTION B: NON-MECHANICAL- FUNCTIONAL
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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
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Mech: Acquired Anomalies Tumours Bladder & ureteric stones-
Melamine stones from Infant formula
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Functional: Damaged kidneysCKD Vesico-Ureteric
Reflux Spina Bifida Myelomeningocoele Neurogenic bladder
eg from Prune belly etc
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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
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Mechanism by which ObstrCKD
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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
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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
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PUV Clinical Presentation 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
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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
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Note Hypertrophied bladder neck and dilated posterior urethra proximal to valve narrowing.
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Anteroposterior view of the abdomen during a void cystourethrographic study. This image demonstrates a dilated bladder with trabeculation, diverticula, and massive reflux.
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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.
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Ectopic Ureter
•Continuous urinarydribbling vs intermittent
•Dry when supine with vaginal pooling
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Non-Mechanical Obstruction Non-Obstructed ‘Obstructed’
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22
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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
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DysplasticKidneys
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3
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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
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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
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5
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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
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Rhabdomyosarcoma-bladder masses
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15 yr Survey of 45 CRF,PHPrimary renal dis. No.(%) Male Female
Glomerulopathies 23 (53.3) 9 14
Obstructive uropathy
13 (28.9) 13 0
Vascular,PN,Malign,Malaria Nephropathy
9 (17.8) 5 4
Anochie I, Eke F.Ped Nephrol 2003;18:692-5
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Yearly Incidence ESRF
UPTH,Nigeria
2.1
2.5
313
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
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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
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Mechanical Obstruction only one Aspect
B: Medical Immediate catheterizaton-size 3 Foleysfluid & 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
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
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Bladder sphincterdysfunction
Recurrent UTIs
V U RBowel
dysfunction
Bowel dysfunction is often part of the constellation; (Dysfunctional elimination syndrome)
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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
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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
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Renal osteodystrophy
Treatment hydroxylated vitamin D therapy - 1 hydroxycholecalciferol - 1-
25dihydroxycholecalciferol by daily oral administration
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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
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Despite all these, MOST PROGRESS to ESRF
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Obstetrician
PaedNephrologist PaedUrologist
Parents PaedNurses
Neonatologist
U/S
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Paed Nephrologist
±UrologistVascular/TransplantSurgeon
Dieticians
Social WorkersAdult Nephrologist Churches
TeachersPsychiatrists PlayTherapists
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End Stage Renal Disease in PUV
100% 90% 45% 1919 1990 2011
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Survival in PUV
19191990 2011
25%60% 90%
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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
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QUIZ Can a bladder
rhbdomyosarcoma cause this anomaly?
What is the likely cause?
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QUIZ True or False All children with Posterior Urethral
valves have Chronic Kidney Disease
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MEMBERSHIP AFPNA
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Learn more about afpna & its members by visiting the website www.afpna.com PNAN integral part of AFPNA & her
Mtgs are advertised there
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OfficersSecretary-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
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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
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IPNA Fellows Dr Franca Ikimalo Port Harcourt, Nigeria CT, SA 1.9 03 – 29.2.04DrAmos Odit Uganda CT, SA 1.3.04 –31.8.04
Dr.Godson Achugwo Owerri,Nigeria CT, SA 05-10.05DrFrancis Lalya Cotonou,Benin CT, SA 14.10.05–30.4.06
Again 12.08-12.09Dr Hemed Twahir Mombasa,Kenya CT, SA 1.4.06 –5.3.07
& again in 2009Dr.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 9.08-2.09Dr Chris Esezobor Lagos, NigeriaDr David Galgallo Kenya
Dr Elena Naicker Durbar, SA Joburg,SA 5-12.09Dr Odetunde Enugu, Nigeria CT, SA 3.11Dr NosaIdurionye Benin, Nigeria CT, SA 6.11-
Total 14
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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
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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)
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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
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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.
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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
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Management of neuropathic bladderAims: Preserve renal function Improve continenceMethods: Conservative/ medical management Surgical
Failure of above methods to prevent upper tract dilation/ongoing renal damage
Unacceptable level of incontinence using conservative means
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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.
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Urodynamics
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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.
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URODYNAMICSURODYNAMICS
vesical
abdominal
detrusor-= ves - abd
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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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PATIENT
PD Nursing Team
Ward Nursing Team
Administration of fluid deliveries
Social Worker Counsello
r
Dietitian
Medical Team
The Multidisciplinary Team
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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.
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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.
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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.
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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.
.
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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.
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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.
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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
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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 ablation.
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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 stomal stenosis. These are rare.
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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.
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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.
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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.