Pseudo Prune Belly Syndrome with Preserved Renal Function and Bilateral Testicular Descent
Keywords:
Prune Belly syndrome, cryptorchidism, hydronephrosis.Abstract
Prune Belly syndrome is characterized by a classic triad: presence of dilation of the upper urinary tract, absence of abdominal wall muscles and bilateral cryptorchidism. The causes of this disease which is more frequent in men are unknown. Patients with a unilateral or bilateral descent of the testicles, partial or complete abdominal wall deficiency and newborn girls with lax abdominal wall are classified as with pseudo Prune Belly syndrome or incomplete Prune Belly syndrome. This article presents the case of a male patient who is currently two years old. At the patient's birth, a diagnosis of pseudo Prune Belly syndrome was made, a disease whose reported cases are very rare. This is the third diagnosis of Prune Belly syndrome at "William Soler" University Pediatric Hospital, in Havana.
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References
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https://www.nature.com/articles/jp200888
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http://www.njcponline.com/article.asp?issn=1119-3077;year=2014;volume=17;issue=4;spage=425;epage=430;aulast=ekwunife
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23. Lopes RI, Tavares A, Srougi M, Dénes FT. 27 years of experience with the comprehensive surgical treatment of prune belly síndrome. J Ped Urol. 2015;11(5):276.01-276.07. DOI: http://dx.doi.org/10.1016/j.jpurol.2015.05.018
https://doi.org/10.1016/j.ejmg.2017.11.008
2. Frohlich F. Der Mangel der Muskein, Insbesondere der Seitenbauchmuskein. Wurzburg. CA Surn;1839.
3. Parker R. Absence of abdominal muscles in an infant. Lancet. 1895;1:1252.
4. Wijesinghe US, Muthucumaru M, Beasley SW. Further evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female. J Pedia Surg. 2016;51(8):1390-3. DOI: http://dx.doi.org/10.1016/j.jpedsurg.2016.05.017
5. Aloni MN, Mujinga V, Tady BM, Nkidiaka ED. A First Description of Prune Belly Syndrome in Central Africa. Pedia Neo. 2015;56(5):355-6. DOI: http://dx.doi.org/10.1016/j.pedneo.2015.01.006
6. Fette A. Associated rare anomalies in prune belly syndrome: a case report. J Ped Surg Case Reports. 2015;3(2):65-71. DOI: http://dx.doi.org/10.1016/j.epsc.2014.12.007
7. Grover H, Sethi S, Garg J, Ahluwalia AP. Pseudo prune belly syndrome: diagnosis by imaging- a case report and brief review. Pol J Radiol. 2017;82:252-7. Disponible en:
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5436412/
8. Arlen AM, Kirsch SS, Seidel NE, Garcia Roig M, Smith EA, Kirsch AJ. Health-related quality of life in children with prune-belly syndrome and their care givers. Urol. 2016 [acceso 11/05/2020];87:224-7. DOI: http://dx.doi.org/10.1016/j.urology.2015.09.028
9. Grimsby GM, Harrison SM, Granberg CF, Bernstein IH, Baker LA. Impact and frequency of extragenitourinary manifestations of prune belly síndrome. J Ped Urol Case Report 2015;11(5):281-6. DOI: http://dx.doi.org/10.1016/j.jpurol.2015.06.005
10. De Bernardo G, Giordano M, De Brasi D. Pseudo Prune Belly syndrome: a case report with unilateral abdominal defect. Radio Case Reports 2019;14(8):941-5. DOI: https://doi.org/10.1016/j.radcr.2019.05.019
11. Roderick JM. Crisler HM, Granberg NM. Detailed evaluation of the upper urinary tract in patients with Prune Belly Syndrome using magnetic resonance urography. J Ped Urol. 2016;12(2):124-5. DOI: http://dx.doi.org/10.1016/j.jpurol.2015.11.013
12. Weber S, Thiele H, Mir S. Muscarinic acetylcholine receptor M3 mutation causes urinary bladder disease and a prune belly like syndrome. Am J Hum Genet. 2011 [acceso 18/05/2020];89(5):668-74. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3213389/
13. Granberg CF, Harrison SM, Dajusta D. Genetic basis of prune belly syndrome: screening for HNF1β gene. J Urol. 2012 [acceso 18/05/2020];187(1):272-8. Disponible en https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3399512/
14. Hassett S, Smith GHH, Holland AJA. Prune belly syndrome. Pedia Surg Int. 2012 [acceso 10/05/2020];28(3):219-28. Disponible en; https://www.researchgate.net/publication/51924507
15. Zugor V, Schott GE, Labanaris AP. The prune belly syndrome: urological aspects and long term outcomes of a rare disease. Pediatr Rep. 2012 [acceso 10/05/2020];4(2):e20. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3395978/
16. Herman TE, Siegel MJ. Prune belly syndrome. J Perinatol. 2009:29:69-71. Disponible en:
https://www.nature.com/articles/jp200888
17. Ekwunife O, Ugwu J, Modekwe V. Prune belly syndrome: early management outcome of nine consecutive cases. Niger J Clin Pract. 2014 [acceso 10/05/2020];17(4):425-30. Disponible en:
http://www.njcponline.com/article.asp?issn=1119-3077;year=2014;volume=17;issue=4;spage=425;epage=430;aulast=ekwunife
18. Manivel JC, Pettinato G, Reinbert Y. Prune-belly syndrome: clinicopathologic study of 29 cases. Pediatr Pathol. 1989;9(6):691-711. DOI: https://www.tandfonline.com/doi/abs/10.3109/15513818909022376
19. Durán Álvarez S. Seudo síndrome prune belly. Rev Cub Pedia 2020 [acceso 05/06/2020];92(1):e990. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75312020000100011&lng=es&nrm=iso&tlng=es
20. Ceballos López JE, Gómez Castellanos JC. Síndrome de abdomen en ciruela pasa: reporte de un caso y revisión de la literatura. Rev Mex Urol. 2015 [acceso 18/05/2020];75(4):219-22. DOI: http://dx.doi.org/10.1016/j.uromx.2015.04.005
21. Routh JC, Huang L, Retik AB. Contemporary epidemiology and characterization of newborn males with prune-belly syndrome. Urol 2010 [acceso 11/05/2020];76(1):44-8. Disponible en: https://pubmed.ncbi.nlm.nih.gov/20381841/
22. Holcomb III G, Murphy J, Shawn P. Holcomb and Ashcraft’s Pediatric Surgery. 7ma edición. Kansas City. Elsevier Inc. 2020 [CD-ROM];T1:942-9.
23. Lopes RI, Tavares A, Srougi M, Dénes FT. 27 years of experience with the comprehensive surgical treatment of prune belly síndrome. J Ped Urol. 2015;11(5):276.01-276.07. DOI: http://dx.doi.org/10.1016/j.jpurol.2015.05.018
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Published
2021-01-22
How to Cite
1.
Mendoza Sánchez LJ, Mendoza RC, Mendoza KM, Rodríguez MG. Pseudo Prune Belly Syndrome with Preserved Renal Function and Bilateral Testicular Descent. Rev. Cubana Urol. [Internet]. 2021 Jan. 22 [cited 2024 Nov. 24];10(2). Available from: https://revurologia.sld.cu/index.php/rcu/article/view/640
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Uropediatría
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