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Successful sperm retrieval in prune belly syndrome |
Joshua A. Halpern,Arighno Das,Robert E. Brannigan()
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Department of Urology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA |
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Abstract Dear Editor, Eagle-Barrett or prune belly syndrome (PBS) is a rare congenital condition with an estimated incidence of 3.8 cases per 100 000 live births in the United States [1]. The syndrome is characterized by cryptorchidism, upper and lower urinary tract dilation, and either absence or deficiency of the abdominal wall musculature. While much of the literature has focused upon urinary tract malformation and cryptorchidism, fertility is a crucial aspect of urologic evaluation and care in these patients. Initial reports suggested that men with PBS were necessarily infertile, though subsequent studies and the advent of assisted reproductive technology have challenged the conventional wisdom, suggesting that spermatogenesis and fertility may be preserved in select men with PBS (Table 1) [2]. Here we report a case of successful sperm retrieval in a man with PBS and a review of the pertinent literature.
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Received: 14 March 2019
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Corresponding Authors:
Robert E. Brannigan
E-mail: r-brannigan@northwestern.edu
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Author | Year | Patients, n | Cohort description and outcomes | Uehling et al. [10] | 1984 | 7 | Absent spermatogenesis on testicular histology in patients with PBS | Woodhouse and Snyder [7] | 1985 | 9 | Elevated gonadotropins and impaired semen parameters in adult and adolescent patients with PBS | Burbige et al. [11] | 1987 | 50 | Long-term results of children treated for PBS | Kolettis et al. [6] | 1999 | 2 | Sperm retrieval in three men with PBS; ICSI in one couple resulting in twin live births | Patil et al. [12] | 2004 | 41 | Success of bilateral orchidopexy and maintenance of sexual function among boys with PBS | Fleming et al. [5] | 2013 | 1 | Live male birth following ICSI with use of ejaculated sperm from a man with PBS | Dénes et al. [2] | 2017 | 15 | Gonadotropins and impaired semen parameters among patients with history of surgical reconstruction for PBS |
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Case series reporting fertility or sexual function in men with PBS.
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[1] |
Routh JC, Huang L, Retik AB, Nelson CP. Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology 2010; 76:44-8.
doi: 10.1016/j.urology.2010.01.062
pmid: 20381841
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[2] |
Dénes FT, Tavares A, Cocuzza M, Tiseu B, Machado MG, Giron AM, et al. Gonadal function and reproductive system anatomy in post puberal prune belly sndrome patients. J Urol 2017;139:e863. https://doi.org/10.1016/j.juro.2017.02.2019.
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[3] |
Lopes RI, Tavares A, Srougi M, Dénes FT. 27 years of experience with the comprehensive surgical treatment of prune belly syndrome. J Pediatr Urol 2015;11:276.e1-7. https://doi.org/10.1016/j.jpurol.2015.05.018.
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[4] |
Orvis BR, Bottles K, Kogan BA. Testicular histology in fetuses with the prune belly syndrome and posterior urethral valves. J Urol 1988; 139:335-7.
doi: 10.1016/s0022-5347(17)42403-7
pmid: 3339735
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[5] |
Fleming SD, Varughese E, Hua VK, Robertson A, Dalzell F, Boothroyd CV. Normal live births after intracytoplasmic sperm injection in a man with the rare condition of Eagle-Barrett syndrome (prune-belly syndrome). Fertil Steril 2013; 100:1532-5.
doi: 10.1016/j.fertnstert.2013.07.1994
pmid: 23993927
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[6] |
Kolettis PN, Ross JH, Kay R, Thomas AJ. Sperm retrieval and intracytoplasmic sperm injection in patients with prune-belly syndrome. Fertil Steril 1999; 72:948-9.
pmid: 10561007
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[7] |
Woodhouse CR, Snyder HM. Testicular and sexual function in adults with prune belly syndrome. J Urol 1985; 133:607-9.
pmid: 3920404
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[8] |
Ramasamy R, Haviland M, Woodard JR, Barone JG. Patterns of inheritance in familial prune belly syndrome. Urology 2005; 65:1227.
pmid: 15922435
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[9] |
Boghossian NS, Sicko RJ, Giannakou A, Dimopoulos A, Caggana M, Tsai MY, et al. Rare copy number variants identi- fied in prune belly syndrome. Eur J Med Genet 2018; 61:145-51.
doi: 10.1016/j.ejmg.2017.11.008
pmid: 29174092
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[10] |
Uehling DT, Zadina SP, Gilbert E. Testicular histology in triad syndrome. Urology 1984; 23:364-6.
pmid: 6143434
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[11] |
Burbige KA, Amodio J, Berdon WE, Hensle TW, Blanc W, Lattimer JK. Prune belly syndrome: 35 years of experience. J Urol 1987; 137:86-90.
pmid: 2879047
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[12] |
Patil KK, Duffy PG, Woodhouse CRJ, Ransley PG. Long-term outcome of Fowler-Stephens orchiopexy in boys with prunebelly syndrome. J Urol 2004; 171:1666-9.
doi: 10.1097/01.ju.0000118139.28229.f5
pmid: 15017263
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