ckd & obstructive uropathy jan23-24 2012@pnan, ile-ife felicia eke, md university of port...

CKD & OBSTRUCTIVE UROPATHY

Jan23-24 2012@PNAN, Ile-Ife

Felicia Eke, MD

University of Port HarcourtNigeria

Outline 1. Definition CKD & Obstr Uropathy Mechanism Obstr.UropCKD Case presentations Quiz Management

Definition-Obstructive Uropathy

Obstruction anywhere down the Urinary tract

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.

**

**

****

A: MECHANICAL OBSTRUCTION B: NON-MECHANICAL- FUNCTIONAL

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

Mech: Acquired Anomalies Tumours Bladder & ureteric stones-

Melamine stones from Infant formula

Functional: Damaged kidneysCKD Vesico-Ureteric

Reflux Spina Bifida Myelomeningocoele Neurogenic bladder

eg from Prune belly etc

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

Mechanism by which ObstrCKD

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

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

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

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

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 normal-caliber distal urethra.

Ectopic Ureter

•Continuous urinarydribbling vs intermittent

•Dry when supine with vaginal pooling

Non-Mechanical Obstruction Non-Obstructed ‘Obstructed’

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

DysplasticKidneys

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

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

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

Rhabdomyosarcoma-bladder masses

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

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

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

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

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

Bladder sphincterdysfunction

Recurrent UTIs

V U RBowel

dysfunction

Bowel dysfunction is often part of the constellation; (Dysfunctional elimination syndrome)

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

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

Renal osteodystrophy

Treatment hydroxylated vitamin D therapy - 1 hydroxycholecalciferol - 1-

25dihydroxycholecalciferol by daily oral administration

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

Despite all these, MOST PROGRESS to ESRF

Obstetrician

PaedNephrologist PaedUrologist

Parents PaedNurses

Neonatologist

U/S

Paed Nephrologist

±UrologistVascular/TransplantSurgeon

Dieticians

Social WorkersAdult Nephrologist Churches

TeachersPsychiatrists PlayTherapists

End Stage Renal Disease in PUV

100% 90% 45% 1919 1990 2011

Survival in PUV

19191990 2011

25%60% 90%

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

QUIZ Can a bladder

rhbdomyosarcoma cause this anomaly?

What is the likely cause?

QUIZ 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

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

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

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

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

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)

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

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.

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

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

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.

Urodynamics

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.

URODYNAMICSURODYNAMICS

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

PATIENT

PD Nursing Team

Ward Nursing Team

Administration of fluid deliveries

Social Worker Counsello

r

Dietitian

Medical Team

The Multidisciplinary Team

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.

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.

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.

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.

.

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.

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.

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

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.

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.

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.

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.

ckd & obstructive uropathy jan23-24 2012@pnan, ile-ife felicia eke, md university of port...

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