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RUBACE Score of Wong et al for Prune Belly Syndrome: E Score for Extra-Genitourinary Manifestations

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) neurologic

(2) cardiac

(3) gastrointestinal

(4) musculoskeletal

(5) respiratory

 

Parameter

Finding

Points

neurologic

normal

0

 

seizures; tethered cord; spina bifida; hearing loss; intellectual delay; autism; severe neurologic condition

3

cardiac

normal

0

 

congenital septal defect (PDA, ASD, VSD) with spontaneous resolution

1

 

small septal defects not spontaneously resolved; minimal left to right shunting; PDA requiring surgical management

2

 

severe congenital or cyanotic heart disease; tetralogy of Fallot; left-sided obstructive lesions; reverse shunt; CHF; severe cardiogenic condition

3

gastrointestinal

normal

0

 

mild to moderate constipation managed with diet and laxatives

1

 

MACE/cecostomy required; malrotation S/P Ladd's

2

 

surgical bowel diversion required; GI malignancy (hepatoblastoma); severe GI condition

3

musculoskeletal

normal

0

 

mild facies; abnormally shaped fingers or toes without loss of function; rib flaring

1

 

scoliosis; hip dysplasia; club or rocker-bottom foot; genu valgum; pectus excavatum or carinum

2

 

arthrogryposis; muscular dystrophy; severe musculoskeletal malformation

3

respiratory

normal

0

 

minimal respiratory support at birth; some difficulty with coughing; mild intermittent asthma; mild reactive airway disease

1

 

persistent and moderate asthma; frequent respiratory tract infections; >= 2 hospitalizations for pneumonia

2

 

ventilator-dependent > 1 week; tracheostomy; history of pneumothorax; severe asthma

3

 

where:

• MACE = Malone antegrade continence enema

 

total score =

= SUM(points for all 5 parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 15

• A score of 3 in the neurologic, cardiac, GI or musculoskeletal category corresponds to PBS-Plus or syndromic PBS.


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