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RUBACE Score of Wong et al for Prune Belly Syndrome: RUBAC Score

Description

Wong et al reported a phenotypic scoring system to evaluate a patient with prune belly syndrome (Eagle-Barrett syndrome). RUBAC is an acronym for the key components of genitourinary changes. The authors are from University of Texas Southwestern Medical Center and Children's Health Dallas.


Patient selection: prune belly syndrome

 

The RUBACE score is the sum of the RUBAC and E (extra-genitourinary) scores.

 

Parameters:

(1) renal (R)

(2) ureteral (U)

(3) bladder/outlet (B)

(4) abdominal wall (A)

(5) cryptorchidism (C) and sex

(6) age

 

Parameter

Finding

Points

renal

normal

0

 

G1 or G2; structural damage with GFR >= 60

1

 

G3a or G3b; GFR 30-59

2

 

G4; GFR 15-29

3

 

renal failure; GFR < 15; on dialysis

4

 

status post renal transplant

4

ureteral

normal

0

 

low grade or absent VUR; distal ureter 4-10 mm

1

 

persistent high grade VUR, status post lower ureteral tract reconstruction; distal ureter 1-2 cm

2

 

persistent high grade VUR despite surgery; distal ureter > 2 cm

3

 

current diverting ureterostomy or nephrostomy

3

bladder/outlet

normal

0

 

urotherapy required to empty bladder; bladder size is <= 2 times normal

1

 

urethral catheterization or pharm required to empty bladder; bladder size > 2 times normal

2

 

antibiotic prophylaxis for recurrent UTI

2

 

current surgical diversion, urethral atresia; megalourethra

3

abdominal wall

normal

0

 

mild to moderate

1

 

severe

2

cryptorchidism

female

1

 

normal male

0

 

unilateral or palpable undescended testis

1

 

bilateral nonpalpable testes

2

age

< 2 years

2

 

2 to 12.99

1

 

>= 13

0

 

High grade vesicoureteral reflux (VUR) corresponds to the International Reflux Grades III to V.

 

Mild to moderate abdominal wall changes: can do sit-up; abdominal musculature mostly intact; minimal laxity; mild or non-convincing prune appearance.

 

Severe abdominal wall changes: severe abdominal laxity with obvious thinning of abdominal wall, classic prune appearance, status post abdominoplasty; unable to do sit-ups.

 

total score =

= SUM(points for all 6 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 16


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