RESULTS
CONCLUSION
To review the clinical manifestations and operative management of a large contemporary pediatric cohort of patients with prune-belly syndrome (PBS).
PBS patients aged <21 years followed up in our pediatric urology clinic were identied by the
International Classication of Diseases, Ninth Revision code (756.71). Demographics, concomitant
diagnoses, surgical history, imaging studies, and renal or bladder function were evaluated.
Data were available for 46 pediatric patients (44 boys and 2 girls). Mean age was 7.6 4.7 years
(range, 0.9-20 years). Average length of clinical follow-up was 6.8 5 years. Forty-ve children
(97.8%) had hydroureteronephrosis, and 36 of them (78.3%) had vesicoureteral reux. Five
patients (10.9%) had signicant pulmonary insufciency, and 2 patients (4.3%) were oxygen
dependent. Eighteen children (39.1%) had other congenital malformations, including cardiac in
4 patients (8.7%) and musculoskeletal anomalies in 10 patients (21.7%). Orchidopexy was the
most common surgery, with all boys aged 3 years having undergone the procedure. Twenty-two
patients (47.8%) had a history of ureteral surgery, 22 (47.8%) had bladder surgery, 11 (23.9%)
had renal surgery, and 6 (13%) had urethral procedures. Nineteen patients (41.3%) underwent
abdominoplasty. Eighteen children (39.1%) had documented chronic kidney disease, and 8
children (17.4%) underwent renal transplantation. Average age at transplantation was 5.1
2.9 years. The mean nadir creatinine level for patients with end-stage renal disease was 1.4 mg/dL
compared with 0.4 mg/dL for those not requiring transplantation (P <.001).
Children with PBS have signicant comorbidities and require frequent operative intervention,
with disease heterogeneity necessitating an individualized management approach. Early end-stage
renal disease is prevalent, with approximately 15% of children requiring kidney
transplantation. UROLOGY -: -e-, 2014. 2014 Elsevier Inc.
Financial Disclosure: The authors declare that they have no relevant nancial interests.
Ethical approval approved under the Childrens Healthcare of Atlanta IRB 14-034.
From the Department of Pediatric Urology, Childrens Healthcare of Atlanta and
Emory University School of Medicine, Atlanta, GA
Address correspondence to: Andrew J. Kirsch, M.D., Department of Pediatric
Urology, Childrens Healthcare of Atlanta and Emory University School of Medicine,
5445 Meridian Mark Rd, Suite 420 Atlanta, GA 30342. E-mail: akirschmd@gmail.
com
Submitted: August 13, 2014, accepted (with revisions): September 23, 2014
METHODS
Institutional review board approval was obtained. We identied a total of 46 patients diagnosed with PBS ranging in age
from 0.9 to 20 years (mean, 7.6 4.7 years). Children diagnosed with PBS, currently followed up in our outpatient pediatric urology clinic were identied by the International
Classication of Diseases, Ninth Revision code (756.71). The
electronic medical records of all pediatric patients with clinically conrmed PBS were retrospectively reviewed. Patient
demographics, concomitant diagnoses, respiratory status, imaging studies, and renal or bladder function were evaluated. All
surgical interventions and current bladder management were
also assessed. Patients aged >21 years and those not seen in the
previous 36 months were excluded from study. Statistical
analysis was performed using SAS 9.3 (SAS Institute, Cary,
NC), with P <.05 representing statistical signicance.
RESULTS
Patient Demographics
Fifty-seven patients with clinically conrmed PBS were
identied in our outpatient clinic system; detailed clinical
data were available on 46 pediatric patients (44 boys and
2 girls) who met all inclusion criteria. Mean age was 7.6
4.7 years (range, 0.9-20 years). Average length of
clinical follow-up was 6.8 5 years. Nineteen patients
(41.3%) were Caucasian, 18 (39.1%) African American,
3 (6.5%) Hispanic, 1 (2.2%) Asian, and 5 (10.9%) were
of mixed ethnicity.
Urologic Diagnoses
Forty-ve patients (97.8%) had bilateral hydroureteronephrosis, and 36 (78.3%) had a history of vesicoureteral reux (VUR); 34 cases were bilateral, and 2
cases were unilateral. The majority of patients (78.3%)
had at least 1 documented UTI, and 15 patients (32.6%)
had a history of febrile UTI. Of the 42 patients
aged 3 years, 14 of them (33.3%) experienced incontinence beyond expected for age and/or developmental
stage.
Comorbidities
Twenty-one children (45.7%) had other congenital
malformations, including cardiac anomalies in 4 (8.7%)
and musculoskeletal anomalies in 10 (21.7%). Eleven
patients (23.9%) had associated gastrointestinal diagnoses; an additional 13 children (28.3%) experienced
constipation. Five patients (10.9%) had signicant pulmonary insufciency, and 2 of them (4.3%) were oxygen
dependent. Three children (6.5%) had documented
psychiatric disorders including oppositional deant disorder, anxiety, and adjustment disorder. Urologic diagnoses and comorbidities are detailed in Table 1.
Urologic Surgeries
All children required surgical intervention (Table 2).
The most prevalent surgery was open or laparoscopic
orchidopexy, with 40 of 41 male patients having undergone bilateral orchidopexy by at least 3 years of age. A
2
44 (95.7)
2 (4.3)
7.6
6.3
36
36
15
45
14
(78.3)
(78.3)
(32.6)
(97.8)
(33.3)
21
4
10
5
5
11
5
2
25
(45.7)
(8.7)
(21.7)
(10.9)
(10.9)
(23.9)
(10.9)
(4.3)
(54.3)
6
5
1
22
5
16
4
1
1
1
1
41
11
8
8
1
22
19
7
3
(13.0)
(10.9)
(2.2)
(47.8)
(10.9)
(34.8)
(8.7)
(2.2)
(2.2)
(2.2)
(2.2)
(93.2)
(23.9)
(17.4)
(17.4)
(2.2)
(47.8)
(41.3)
(15.2)
(6.5)
(-), 2014
COMMENT
Optimal management of children with PBS remains
challenging, and treatment is further confounded by the
UROLOGY
(-), 2014
Table 3. Indications and ndings of urodynamic testing obtained in children with prune-belly syndrome
Indication
Capacity
Compliance
mL/cm H2O
PVR,
mL (%)
Preoperative planning
2.9
27
522 (50)
Prolapsing vesicostomy,
preoperative planning
0.5
44
110 (100)
Preoperative planning
5.5
110
1850 (100)
Preop planning,
vesicostomy
0.8
26
139 (100)
2.7
28
Preoperative planning,
hx of VUR
Preoperative planning
5.2
204
710 (91)
7
8
Preoperative planning
Preoperative planning
2
3.2
39
90
440 (92)
200 (42)
1.1
20
50 (14)
10 Preoperative planning
2.7
111
11
12
13
14
1.3
1.5
1.9
2.5
32
41
84
26
2.4 1.4
63 50
Preoperative planning
Recurrent febrile UTIs
Preoperative planning
Stomal leakage
Mean
435
200
342
150
(100)
(73)
(100)
(22)
63.1 38.9
Additional Findings
Management
Bilateral VUR
Appendicovesicostomy
and reimplant
Unable to ll due to Vesicostomy takedown and
appendicovesicostomy
prolapsing
vesicostomy,
did not void
per urethra
Appendicovesicostomy
and reduction cystoplasty
Left VUR, did not
Vesicostomy takedown,
void per urethra
left reimplant, and
appendicovesicostomy
No VUR
Abdominoplasty without
concomitant procedures
Abdominoplasty without
concomitant procedures
Appendicovesicostomy
Abdominoplasty without
concomitant procedures
VUR into transplant Increase frequency of CIC
kidney
per appendicovesicostomy
Bilateral VUR
Abdominoplasty and
bilateral reimplant
Appendicovesicostomy
Bilateral VUR
CIC per urethra
Appendicovesicostomy
Increase frequency of CIC
per appendicovesicostomy
CIC, clean intermittent catheterization; PVR, postvoid residual; other abbreviations as in Table 1.
Capacity multiplied by normal estimated bladder capacity for age using the formula (age 2) 30 mL. Postvoid residual recorded as
volume as well as percentage of individual bladder capacity.
(-), 2014
CONCLUSION
Children with PBS require multiple procedures to correct
associated urologic anomalies and address comorbidities
UROLOGY
(-), 2014