Prune-belly syndrome

Main Article Content

Yenny Yenny
Kusuma P. A
Damanik M. P.

Abstract

Prune-belly syndrome, also known as Eagle-Barret
syndrome, is a congenital anomaly comprising
three clinical findings: deficient abdominal
musculature, urinary tract anomalies, and
bilateral cryptorchidism. Other clinical findings involving
respiratory, skeletal, digestion and cardiovascular system
may also accompany the syndrome. The incidence is
approximately 1 : 30,000 to 40,000 live births and 95%
of cases occur in boys. Pulmonary hypoplasia and kidney
failure are important prognostic factors that contribute
to 60% of mortality rate. Treatment includes surgical
correction of the abdominal wall and urinary tract,
orchidopexy and other supportive managements.l-4 We
report 4 cases on typical Prune-belly syndrome, together
with other clinical variants.

Article Details

How to Cite
1.
Yenny Y, P. A K, M. P. D. Prune-belly syndrome. PI [Internet]. 31Oct.2009 [cited 27Nov.2020];49(5):304-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/588
Section
Case Report
Author Biographies

Yenny Yenny

Department of Child Health, Medical School, Gadjah Mada University, Dr. Sardjito Hospital, Yogyakarta, Indonesia.

Kusuma P. A

Department of Child Health, Medical School, Gadjah Mada University, Dr. Sardjito Hospital, Yogyakarta, Indonesia.

Damanik M. P.

Department of Child Health, Medical School, Gadjah Mada University, Dr. Sardjito Hospital, Yogyakarta, Indonesia.
Received 2016-09-11
Accepted 2016-09-11
Published 2009-10-31

References

1. Druschel CM. A descriptive study of Prune-belly syndrome
in New York state. Arch Pediatr Ado! Med. 1983-
1989; 149:70-6.
2. Crankson S, Ahmed S. The Prune-belly syndrome. Aust N
Z J Surg. 1992;62:916-21.
3. Fallat ME, Skoog SJ, Belman AB, Eng G, Randolph JG.
The Prune-belly syndrome: a comprehensive approach to
management. JUral. 1989; 142:802-5.
4. Kabakus N, Serhatlioglu S, Akfirat M, Kazez A, Aydinoglu H,
Ozerzan I, et al. Prune Belly Syndrome associated with extra
abdominal abnormalities in a 7 year old boy. Turk J Pediatr.
2000;42: 158-61.
5. Sutherland RS, Mevorach RA, Kogan BA. The Prune-belly
syndrome: current insights. Pediatr Nephrol. 1995;9:770-8.
6. Gonzalez R, Reinberg Y, Burke B. Early bladder outlet
obstruction in fetal lambs induces renal dysplasia and the
Prune belly syndrome. J Pediatr Surg. 1990;25:342-5.
7. Reinberg Y, Shapiro E, Manivel JC, Manley C, Pettinato
G, Gonzales R. Prune-belly syndrome in females: a triad
of abdominal musculature deficiency and anomalies of the
urinary and genital systems. J Pediatr. 1991; 118:395-8.
8. Brincher MR, Palutsis RS, Sarwak JF. The orthopaedic
manifestations of Prune-belly syndrome. J Bone Joint surg.
1995;77:251-7.
9. Loder RT, Guiboux JP, Bloom DA, Bensinger RN. Musculoskeletal
aspects of Prune-belly syndrome. Am J Dis Child.
1992; 146:1224-9.
10. Crompton CH, Maclusky IB, Geary DF. Respiratory
function in the Prune-belly syndrome. Arch Dis Child.
1993;68:505-6.
11. Noh PH, Cooper CS, Winkler AC, Zderic SA, Snyder
HM, Canning DA. Prognostic factors for long term renal
function in boys with the Prune-belly syndrome. J Ural.
1999;162: 1399-401.
12. Woodhouse CRJ, Neild GH. A child with recurrent
infections and undescended testes. Nephrol Dial Transplant.
1995;10:711-3.
13. Ehrlich RM, Lesavoy MA, Fine RN. Total abdominal wall
reconstruction. JUral. 1986;136:282-5.
14. Furness PD, Cheng EY, Franco I, Firlit CF. The Prune-belly
syndrome: a new and simplified technique of abdominal wall
reconstruction. JUral. 1998;160: 1195-7.
15. Crompton CH, Balfe JW, Khoury A. Peritoneal dialysis in the
Prune-belly syndrome. Perit Dial Int. 1994; 14:17-21.
16. Reinberg Y, Manivel JC, Pettinato G, Gonzales R. Development of renal failure in children with the Prune-belly
syndrome. J Urol. 1991;145:1017-9.