TY - JOUR
T1 - Prune belly syndrome
T2 - Clinicopathologic study of 29 cases
AU - Manivel, J. Carlos
AU - Pettinato, Guido
AU - Reinberg, Yuri
AU - Gonzalez, Ricardo
AU - Burke, Barbara
AU - Dehner, Louis P.
N1 - Funding Information:
Dr. Pettinato was a research fellow at the University of Minnesota Hospital through a grant from the Associazione Italiana per la Ricerca sul Cancro, Milan, Italy. Presented in part at the annual meeting of the International Academy of Pathology, Washington, D.C., March 1988. Lab Investig 1988;58:59A. Dr. Dehner’s present address is Washington University School of Medicine, Barnes Hospital, St. Louis, Missouri 631 10. Address reprint requests to J. Carlos Manivel, M.D., Department of Laboratory Medicine and Pathology, Division of Surgical Pathology, Box 76 UMHC, University of Minnesota Hospital, 420 Delaware Street S.E., Minneapolis, Minnesota 55455.
PY - 1989
Y1 - 1989
N2 - The clinical course and the pathologic features of 29 patients with the prune belly syndrome (PBS) are reviewed. There were 26 males and 3 females. In addition to the classical triad of deficient abdominal musculature, urinary tract abnormalities, and cryptorchidism, a broader spectrum of other defects was found including musculoskeletal (58% and gastrointestinal (31% abnormalities. Genital anomalies were present in all three female patients. Many of these defects may be inapparent at birth, but art the cause of morbidity and mortality later in life. Severe urinary tract maldevelopment and pulmonary hypoplasia as part of the oligohydramnios syndrome was the most common cause of perinatal deaths. In these patients, major portions of the renal parenchyma were dysplastic, but in survivors, renal dysplasia, when present, was minor by comparison, and affected less than 1/3 of the parenchyma. Although several questions remain unanswered, we believe that the PBS results from the effect of one or more teratogenic agents on the somatic mesoderm, producing inappropriate mesenchymal development and inadequate mesenchymal-epithelial interactions that lead to abnormal development and dilatation of some of its derivatives (abdominal muscles, meter, bladder, prostate, urethra, and gubernaculum). Although abnormalities in derivatives of the intermediate mesoderm (kidney) may also be produced by the injurious agent(s), they are more likely a result of urinary obstruction. Abnormalities in other organs and systems are the consequence of oligohydramnios..
AB - The clinical course and the pathologic features of 29 patients with the prune belly syndrome (PBS) are reviewed. There were 26 males and 3 females. In addition to the classical triad of deficient abdominal musculature, urinary tract abnormalities, and cryptorchidism, a broader spectrum of other defects was found including musculoskeletal (58% and gastrointestinal (31% abnormalities. Genital anomalies were present in all three female patients. Many of these defects may be inapparent at birth, but art the cause of morbidity and mortality later in life. Severe urinary tract maldevelopment and pulmonary hypoplasia as part of the oligohydramnios syndrome was the most common cause of perinatal deaths. In these patients, major portions of the renal parenchyma were dysplastic, but in survivors, renal dysplasia, when present, was minor by comparison, and affected less than 1/3 of the parenchyma. Although several questions remain unanswered, we believe that the PBS results from the effect of one or more teratogenic agents on the somatic mesoderm, producing inappropriate mesenchymal development and inadequate mesenchymal-epithelial interactions that lead to abnormal development and dilatation of some of its derivatives (abdominal muscles, meter, bladder, prostate, urethra, and gubernaculum). Although abnormalities in derivatives of the intermediate mesoderm (kidney) may also be produced by the injurious agent(s), they are more likely a result of urinary obstruction. Abnormalities in other organs and systems are the consequence of oligohydramnios..
KW - Prune belly syndrome
UR - http://www.scopus.com/inward/record.url?scp=0024781301&partnerID=8YFLogxK
U2 - 10.3109/15513818909022376
DO - 10.3109/15513818909022376
M3 - Article
C2 - 2602227
AN - SCOPUS:0024781301
SN - 1551-3815
VL - 9
SP - 691
EP - 711
JO - Fetal and Pediatric Pathology
JF - Fetal and Pediatric Pathology
IS - 6
ER -