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Prune Belly Syndrome Associated With Congenital Cystic Adenomatoid Malformation of the Lung

Prune Belly Syndrome Associated With Congenital Cystic Adenomatoid Malformation of the Lung Abstract The first case of prune belly syndrome was reported in 1839. Since then, more than 150 cases have been described1-6 and an extensive review of the literature was made in 1950 by Silverman and Huang.7 This syndrome affects mostly boys and associates abdominal wall muscle deficiency, undescended testes, and urinary tract malformations. Other anomalies occur. The prognosis is poor and many patients die at an early age of urinary tract infection, renal failure, or pulmonary complications. Management includes the use of an abdominal binder and medical or surgical care of urinary and gastrointestinal problems. To our knowledge, no case of this syndrome associated with cystic adenomatoid malformation of the lung has yet been published. This report describes this occurrence in one child. Report of a Case.—A 7-day-old male infant was admitted to the hospital with the complaint of mild respiratory difficulties. Birth weight was 3,030 gm; length, References 1. Bourne CW, Cerny JC: Congenital absence of abdominal muscles: Report of 6 cases . J Urol 98:252-259, 1967. 2. Burke EC, Shin MH, Kelalis PP: Prune belly syndrome, clinical findings and survival . Am J Dis Chid 117:668-671, 1969. 3. Lattimer JK: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: A report of 22 cases . J Urol 79:343-352, 1958. 4. McGovern JH, Marshall VF: Congenital deficiency of the abdominal musculature and obstructive uropathy . Surg Gynecol Obstet 108:289-305, 1959. 5. Nunn IN, Stephens, FD: The triad syndrome: a composite anomaly of the abdominal wall, urinary system, and testes . J Urol 86:782-794, 1961. 6. Rogers LW, Ostrow PT: The prune belly syndrome: Report of 20 cases and description of a lethal variant . J Pediatr 83:786-793, 1973.Crossref 7. Silverman FN, Huang N: Congenital absence of the abdominal muscles. Associated with malformation of the genitourinary and alimentary tracts: Report of cases and review of the literature . Am J Dis Child 80:91-124, 1950. 8. Avery ME, Fletcher BD: The Lung and Its Disorders in the Newborn Infant , ed 3. Philadelphia, WB Saunders Co, 1974, p 149. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Prune Belly Syndrome Associated With Congenital Cystic Adenomatoid Malformation of the Lung

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120280100022
Publisher site
See Article on Publisher Site

Abstract

Abstract The first case of prune belly syndrome was reported in 1839. Since then, more than 150 cases have been described1-6 and an extensive review of the literature was made in 1950 by Silverman and Huang.7 This syndrome affects mostly boys and associates abdominal wall muscle deficiency, undescended testes, and urinary tract malformations. Other anomalies occur. The prognosis is poor and many patients die at an early age of urinary tract infection, renal failure, or pulmonary complications. Management includes the use of an abdominal binder and medical or surgical care of urinary and gastrointestinal problems. To our knowledge, no case of this syndrome associated with cystic adenomatoid malformation of the lung has yet been published. This report describes this occurrence in one child. Report of a Case.—A 7-day-old male infant was admitted to the hospital with the complaint of mild respiratory difficulties. Birth weight was 3,030 gm; length, References 1. Bourne CW, Cerny JC: Congenital absence of abdominal muscles: Report of 6 cases . J Urol 98:252-259, 1967. 2. Burke EC, Shin MH, Kelalis PP: Prune belly syndrome, clinical findings and survival . Am J Dis Chid 117:668-671, 1969. 3. Lattimer JK: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: A report of 22 cases . J Urol 79:343-352, 1958. 4. McGovern JH, Marshall VF: Congenital deficiency of the abdominal musculature and obstructive uropathy . Surg Gynecol Obstet 108:289-305, 1959. 5. Nunn IN, Stephens, FD: The triad syndrome: a composite anomaly of the abdominal wall, urinary system, and testes . J Urol 86:782-794, 1961. 6. Rogers LW, Ostrow PT: The prune belly syndrome: Report of 20 cases and description of a lethal variant . J Pediatr 83:786-793, 1973.Crossref 7. Silverman FN, Huang N: Congenital absence of the abdominal muscles. Associated with malformation of the genitourinary and alimentary tracts: Report of cases and review of the literature . Am J Dis Child 80:91-124, 1950. 8. Avery ME, Fletcher BD: The Lung and Its Disorders in the Newborn Infant , ed 3. Philadelphia, WB Saunders Co, 1974, p 149.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Mar 1, 1978

References