PUV Flashcards
incidence
1/4000 live born boys
PUV progression to renal failure
1/4 to 1/5 30-50% patients 20% by age 16 ESRF 25-30% by age 20years CKD 50% by over 20 years half will have day and night incontinence by 5 yo
no. get UTI with PUV
50% will get UTIs
three causes of renal failure in PUV
First hit – nephrogenic zone has back pressure, has peripheral cortical cyst formation, renal dysplasia
Kidneys not normal in first place
Second factor, UTI – very high risk 50%, circumcision reduces risk by 90%
Third hit from bladder dysfunction, progressively obvious after first few years of life
PLUTO trial
Trying to manage antenatally
High number of children being terminated
Vesicoamniotic shunts not shown to be viable and sensible option
AN diagnosis is not functional test just observational
high rate of termination
insertion of catheter
high risk patient
when do cystoscopy
insert catheter straight away 6F feeding tube post obstructive diuresis start antibiotics get mcug when creatinine stable 2.5 kg to 3kg 9Fr cystoscope cold blade
long term outcomes of PUV
renal failure 45% dead or ESRF by 40s CKD Incontinence - compared to normal boys only 20% will be dry overnight aged 5, and less than half dry at ten year malignancy Augmentation with transplant infertility
after catehter monitor post obstructive diuresis
UO for last 4 hours then add 10%
replace UO then add 30mls/kg/day
look at weight of baby
may require oral sodium bicarb
initial management of PUV
catheter diuresis acidosis once creatinine and diuresis resolve do MCUG then cystoscopy
cystoscopy of PUV
where incise valves
circumcision
repeat cystoscopy?
Cystoscopy using retrograde flow
Flaps of PUV can flatten against posterior urethra and can miss diagnosis
Important to recognise secondary associations
As pass through sphincter see very in flat horizontal position rather than vertical position
PU is very dilated
Bladder neck very high and hypertrophic
Markedly trabeculated bladder
Sacculations very large
Turn off flow, perform give antegrade flow, as withdraw scope from bladder, can see flaps of PUV inflate like sail on sailing boat
Then incise valves at 5, 7 and 12 oclock
Circumcision at same sitting reduces UTI by 90%
Repeat cystoscopy at 6 weeks to 3 months postop
long term management PUV
UTI renal dysplasia bladder dysfunction see 6 monhtly till 2, then yearly till 5 then 1-2 yearly until 16 worsening HN VUDS in any boys with incontinence routine VUDS at 5 years
high pressure bladder on VUDS on follow up PUV
may need vesicostomy until age 2 if high pressure bladder
older boys can have mitrofanoff
when do augmentation
if bladder capacity less than 50% predicted
pressures above 25cm water at end fill
what if bladder large but not empyting
many not tolerate CISC
mitrofanoff may be good option
risk of malignancy with bladder augmentation
high as 15%
higher in combination with transplantation and immunosuppression
