Prune-belly syndrome
Keywords:
Prune-belly syndrome
Abstract
Prune-belly syndrome, also known as Eagle-Barretsyndrome, 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.
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.
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.
Published
2009-10-31
How to Cite
1.
Yenny Y, P. A K, M. P. D. Prune-belly syndrome. PI [Internet]. 31Oct.2009 [cited 24Nov.2024];49(5):304-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/588
Section
Case Report
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Received 2016-09-11
Accepted 2016-09-11
Published 2009-10-31
Accepted 2016-09-11
Published 2009-10-31
