Case Series
Volume 9 Issue 8 - 2020
Prune Belly Syndrome, the Unusual Presentation of the Rare Pathology
Wasim Khasawneh* and Saif Aldin Rawabdeh
Department of Pediatrics and Neonatology, Jordan University of Science and Technology, Irbid, Jordan
*Corresponding Author: Wasim Khasawneh, Department of Pediatrics and Neonatology, Jordan University of Science and Technology, Irbid, Jordan.
Received: May 10, 2020; Published: July 22, 2020




Abstract

Prune-belly syndrome (PBS) is a rare congenital disorder that mainly affects males and is manifested by a triad of weak or deficient abdominal wall muscles, urinary tract malformation, and cryptorchidism. It has an estimated incidence of 2 - 3 per 100,000 live births. Although it has been reported to almost always affect males, there have been a few reported cases of females affected with PBS in the literature [1].

We hereby report three cases of PBS delivered at King Abdullah University Hospital (KAUH), a tertiary academic hospital in North of Jordan. Each of these cases has something peculiar that makes this series worth reporting. We also conducted a thorough literature review about PBS and included a quick review about presentation, management and suggestions.

Keywords: Prune Belly Syndrome; Neonates; In Utero; Nephrostomy

References

  1. Y Reinberg., et al. “Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital systems”. The Journal of Pediatrics 3 (1991): 395-398.
  2. E Straub and J Spranger. “Etiology and pathogenesis of the prune belly syndrome”. Kidney International 1981 20.6 (1981): 695-699.
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  6. D Shah., et al. “VACTERL association with Prune-Belly syndrome”. Indian Academy of Pediatrics8 (2004): 845-847.
  7. MR Brinker., et al. “The orthopaedic manifestations of prune-belly (Eagle-Barrett) syndrome”. Journal of Bone and Joint Surgery American2 (1995): 251-257.
  8. D Goswami., et al. “The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association”. JCDR - Journal of Clinical and Diagnostic Research 8 (2013): 1727-1729.
  9. MR Perez-Brayfield., et al. “In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia”. Urology6 (2001): 1178.
  10. Z Seifi., et al. “Candiduria in children and susceptibility patterns of recovered Candida species to antifungal drugs in Ahvaz”. Journal of Nephropathology 2 (2013): 122-128.
  11. RV Patel., et al. “Prune belly sequence in a non-identical twin”. BMJ Case Report (2014).
  12. MM Bogart., et al. “Prune-Belly Syndrome in Two Children and Review of the Literature”. Pediatric Dermatology 4 (2006): 342-345.
  13. RI Lopes., et al. “27 years of experience with the comprehensive surgical treatment of prune belly syndrome”. Journal of Pediatric Urology 5 (2015): 276.e1-276.e7.
Citation: Wasim Khasawneh and Saif Aldin Rawabdeh. “Prune Belly Syndrome, the Unusual Presentation of the Rare Pathology”. EC Paediatrics 9.8 (2020): 149-152.

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