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~1%
Siblings 25%, 50% in 1st 2 yrs, parent to child 66%, twins 66% suggest AD + var penetrance
PAX-2 knockout mouse metanephric arrest and megaureter. Expressed on cells destined for ureter, family of
genes responsible for body segmentation and cell specification.
Pathophysiology
Extension of inner long. Muscle forms sup. Trigone. Adventitial layer (Waldeyers sheath) continuous with deep
trigone. This arrangement maintains one way flap valve at UVJ. Pressure compresses lumen.
Variables are tunnel length (normal is 5:1), VUR likley below 3:1
muscular backing, eg attenuation 2o Hutch divertic, everting ureterocoele.
intact trigonal muscle tone - interruption l/t lateral + sup migration of UO
adequate storage reservoir.
Hyperreflexic bladder or dyssynergia, Rx with anticholinergics lead to > resolution of VUR over
time. Need to address dysfunctional voiding
Non-compliant ureter 2o inflammation, ureteral paralysis d/t bact endotoxins may contribute
Ie need to address these primary pathologies
Classification:
% resolution in 3 yrs
IRS Grade 1 ureter only 87
Grade 2 to pelvis, no dilatation 63
Grade 3 mild dilatation of pelvis 53
Grade 4 mod calyceal dilatation and blunting 33
Grade 5 severe c. dilatation and ureteral tortuosity
Nuclear grading has 3 grades
Resolves with time axial growth with lengthening of sm tunnel, also developmental improvement in bladder
dynamics.
Morphology on cystoscopy is not an accurate predictor of grade, or chance of resolution
Natural Hx of children presenting at av age 3 with UTI, 1/3 will show a reduction of grade each year. Cessation
also more likely if unilateral, black vs white
Evaluation:
Clinical:
Voiding Hx strong assoc b/n dysfunctional voiding, recurrent UTIs and resolution of VUR
Soiling
Mass, tenderness
Back occult dysraphism
BP
Imaging:
1. US
2. MCU in all <10yrs b/c scars still occur up till this age
3. Nuclear cystogram interval and post surgical monitoring, 200x less gonadal irradiation. Poor anatomic detail
and diff b/n grades of reflux
4. DMSA diff renal function and scar distribution
Sequelae of Reflux:
Scarring:
M>F (prob reflects overall higher grade reflux in boys)
Prop. to no of UTIs, and age (<4) of 1st UTI)
Intrarenal reflux in poles b/c compound papillae in which papillary ducts open at right angles to calyx. Resistance
to IRR is lower in younger kids. At 1/12 occurs at pressures as low as 2mmHg. At 1 yr require 20mmHg. Also
compounded by immature bladder with frequent contractions.
Infection needed for devp of scars, vs dysplasia which may be congenitally assoc with high grade reflux (Mackie
Stephens theory).
Impairment of RF initially tubular dysfunction, if progressive get drop in GFR and CRF.
HT:
20% with established scars. Bilat > unilat.
Not without scars. Renin dependent mechanism
Management:
Based on principles that VUR is a mechanical problem that can resolve with time, and that sterile low pressure
reflux is not harmful to kidney.
Medical:
Controversy is Grade III IV. IRS in 1981 randomized to medical vs Sx. Published 1992 both = effective in
preventing new scarring 19/155 med vs 20/151 surgically Rx. Birmingham Reflux study group made same
conclusion.
Based on Smellies work (metaanalysis of 1720 pts with reflux), continuous low dose Abs better than courses
Bactrim 2mg/kg/day, nitrofurantoin 2mg/kg/day
Sting procedure
using teflon paste <0.2ml under UO. Success 80% with 1 application. Second injection brings rate to 89%.
Concerns about Teflon migration.
Injectable collagen used, but need greater volume (5ml / ureter), shows volume loss with time ie need more
frequent reinjection. 63% success with 1, 79% with 2 injections. Uncertainty about persistence of correction
Search continues for better substance. Atala et al looking at autologous tissue chondrocytes, smooth muscle.