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
Specialty: Nephrology, Pedatrics