Hipospadias ve İnmemiş Testis Birlikteliğinde Ek Anomali Sıklığının Değerlendirilmesi

Amaç: Hipospadias erkek çocuklarda 200-300 canlı doğumda bir görülen genitoüriner sistem (GÜS) anomalisidir.

Anahtar Kelimeler:

Additional anomalies, Hypospadias

Evaluation of Additional Anomalies in Concomitance of Hypospadias and Undescended Testes

Objective: Hypospadias is a common genitourinary system (GUS) anomaly in boys occurring in 1 of 200 to 300live births. Undescended testes is frequently detected among accompanying anomalies in cases with hypospadias.Especially in proximal hypospadias and bilateral cases, this association may indicate sexual differentiation disorders. Theaim of the study was to evaluate the togetherness of additional anomalies in hypospadiac children with undescendedtestes.Material and Methods: Between 2007 and 2016, data of 392 children who underwent surgery for hypospadias wereevaluated retrospectively. Urethral meatus was present at scrotal and penoscrotal in 65 cases (16.6%) and glanular,coronal, subcoronal and midpenile in 327 cases (83.4%). The cases were divided into two groups as those with bothtestes in the scrotum and those with undescended testes, and the anomalies were recorded.Results: The mean age of the children with proximal hypospadias was 21 months (6-240 months). Of the childrenwith proximal hypospadias, 26 (40%) had undescended testes and 39 (60%) had testes in the scrotum. Undescendedtestes were detected bilaterally in 17 patients (65.4%) and unilaterally in nine patients (34.6%) in the undescendedtestes group. In the undescended testes group, 24 patients (92.3%) had suspected external genitalia (bifid scrotum,penoscrotal transposition, togetherness of micropenis and penoscrotal hypospadias, scrotalized labium majus) onphysical examination. In the undescended testes group, three patients (11.5%) had additional urinary pathology andten patients (38.5%) had other system anomalies. Ten patients (25.5%) had suspicious external genitalia on physicalexamination in the proximal hypospadias group who had both testes in scrotum. Additional urinary pathology wasdetected in one patient (2.5%) while other system anomalies were detected in five (12.8%) patients.The mean age of children with distal hypospadias was 33 months (6-156 months). Nine of 327 children with distalhypospadias (2.8%) had undescended testes. Of these, five were bilateral (55.6%) and 4 had unilateral undescendedtestes (44.4%). None of the patients with distal hypospadias had any suspicious genitalia on physical examination. Inthe undescended testes group, one patient (11.1%) had additional urinary pathology and one patient had other systemanomaly. Seven patients (2.2%) who had both testes in scrotum had additional urinary pathology and 32 patients (9.8%)had other system anomalies.Conclusion: According to our results, further tests such as genitourinary ultrasonography, pelvic MRI if necessary,endocrine and genetic evaluation should be considered in cases where the proximal hypospadias is accompanied byan undescended testicle, and detailed anamnesis and physical examination covering all systems is necessary in allhypospadiac children in terms of additional anomalies.

Keywords:

Additional anomalies, Hypospadias,

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1. Weidner IS, Moller H, Jensen TK, Skakkebaek NE. Hypospadias in Washington State: Maternal risk factors and prevalence trends. Pediatrics 2005;115:495-9.
2. Scorer C, Farrington GH. Congenital deformities of the testis and epididymis. New York: Appleton-Century Crofts, 1971.
3. Diamond DA, Chan IHY, Holland AJA, Kurtz MP, Nelson C, Estrada CR Jr, et al. Advances in pediatric urology. Lancet 2017;390:1061- 71.
4. Cox MJ, Coplen DE, Austin PF. The incidence of disorders of sexual differentiation and chromosomal abnormalities of cryptorchidism and hypospadias stratified by meatal location. J Urol 2008;180:2649-52.
5. Rajfer J, Walsh PC. The incidence of intersexuality in patients with hypospadias and cryptorchidism. J Urol 1976;116:769-70.
6. Rohatgi M, Menon PS, Verma IC, Iyengar JK. The presence of intersexuality in patients with advanced hypospadias and undescended gonads. J Urol 1987;137:263-7.
7. McAleer IM, Kaplan GW. Is routine karyotyping necessary in the evaluation of hypospadias and crytporchidism? J Urol 2001;165:2029-31.
8. Kaefer M, Diamond D, Hendren WH, Vemulapalli S, Bauer SB, Peters CA, et al. The incidence of intersexuality in children with cryptorchidism and hypospadias: Stratification based on gonadal palpability and meatal position. J Urol 1999;162:1003-6.
9. Manzoni G, Bracka A, Palminteri E, Marocco G. Hypospadias surgery: When, what and by whom? BJU Int 2004;94:1188-95.
10. Friedman T, Shalom A, Hoshen G, Brodovsky S, Tieder M, Westreich M. Detection and incidence of anomalies associated with hypospadias. Pediatr Nephrol 2008;23:1809-16.
11. Corder JP, Al Ahbabi FJS, Al Dhaheri HS, Chedid F. Demographics and co-occurring conditions in a clinic-based cohort with Down syndrome in the United Arab Emirates. Am J Med Genet 2017;173A:2395-407.
12. Brock WA, Pena A. Urological implications of imperforated anus. AUA Update Series 1991;X:202-7.
13. Yucel S, Dravis C, Garcia N, Henkemeyer M, Baker LA. Hypospadias and anorectal malformations by Eph/ephrin signaling. J Pediatr Urol 2007;3:354-63.
14. Chaijan P Y, Dorreh F, Sharafkhah M, Amiri M, Ebrahimimonfared M, Rafeie M, et al. Congenital urogenital abnormalities in children with congenital hypothyroidism. Med J Islam Repub Iran 2017;31:7.