PEDIATRICS Vol. 73 No. 4 April 1984, pp. 470-475
This Article
Right arrow Full Text (PDF)
Right arrow Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Moerman, P.
Right arrow Articles by Lauweryns, J. M
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Moerman, P.
Right arrow Articles by Lauweryns, J. M
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Pathogenesis of the Prune-Belly Syndrome: A Functional Urethral Obstruction Caused by Prostatic Hypoplasia

Philippe Moerman MD1, Jean-Pierre Fryns MD1, Paul Goddeeris MD1, and Joseph M Lauweryns MD, PhD1

1 From the Departments of Pathology I and Human Biology, Division of Human Genetics, Katholieke Universiteit Leuven, Belgium

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy—ie, the degeneration of already formed muscle—and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prunebelly syndrome.

Key Words: prune belly • urethral obstruction • prostatic hypoplasia

Submitted on March 21, 1983
Accepted on June 10, 1983


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:


Home page
 Endocr. Rev.Home page
J. M. Hutson, S. Hasthorpe, and C. F. Heyns
Anatomical and Functional Aspects of Testicular Descent and Cryptorchidism
Endocr. Rev., April 1, 1997; 18(2): 259 - 280.
[Abstract] [Full Text]

Home page
 Arch Pediatr Adolesc MedHome page
R. T. Loder, J.-P. Guiboux, D. A. Bloom, and R. N. Hensinger
Musculoskeletal Aspects of Prune-Belly Syndrome: Description and Pathogenesis
Arch Pediatr Adolesc Med, October 1, 1992; 146(10): 1224 - 1229.
[Abstract] [PDF]