Baeza-Herrera C, Cortés-García R, Villalobos-Castillejos A, Velázquez-Pino H

Language: Spanish
References: 7
Page: 61-63
PDF size: 191.16 Kb.

ABSTRACT

“Prune-belly” syndrome and Bochdalek hernia is unusual congenital association of malformations of the newborn that lead to incomplete formation of the diaphragm and the genitourinary tract systems. Currently, incomplete mesodermal development is considered as the cause for these complex non genetic syndromes; however the etiology is still unknown. We present an unusual case sharing characteristics of “prune-belly” syndrome and Bochdalek hernia. We hypothesize that they are malformations due to a failure to create a normal mesodermal interaction.

REFERENCES

1. Eagle JF, Barrett GS. Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome report of 9 cases. Pediatrics 1950;6:721-36.

2. Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology 2010;76:44-8.

3. Giuliani S, Vendryes C, Malhotra A, Shaut D, Anselmo DM. Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant. J Pediatr Surg 2010;45:E39-E42.

4. Straub E, Spranger J. Etiology and pathogenesis of the prune belly syndrome. Kidney Int 1981;20:695-9.

5. Levin TL, Soghier L, Blitman NM, Vega-Rich C, Nafday S. Megacystis-microcolon-intestinal hypoperistalsis and prune belly: overlapping syndromes. Pediatr Radiol 2004;34:995-8.

6. Bohn D. Congenital diaphragmatic hernia. Am J Respiratory Care Med 2002;166:911-15.

7. Ching-Ping C. Syndromes and disorders associated with omphalocele (III): Single disorders, neural tube defects, diaphragmatic defects and others. Taiwan J Obstet Gynecol 2007;46:111-20.