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Evaluation of Additional Anomalies in Concomitance of Hypospadias and Undescended Testes

Yıl 2019, Cilt: 13 Sayı: 4, 222 - 226, 30.07.2019
https://doi.org/10.12956/tjpd.2018.361

Öz

Objective: Hypospadias is a common genitourinary system (GUS) anomaly in boys occurring in 1 of 200 to 300

live 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. The

aim of the study was to evaluate the togetherness of additional anomalies in hypospadiac children with undescended

testes.

Material and Methods: Between 2007 and 2016, data of 392 children who underwent surgery for hypospadias were

evaluated 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 both

testes 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 children

with proximal hypospadias, 26 (40%) had undescended testes and 39 (60%) had testes in the scrotum. Undescended

testes were detected bilaterally in 17 patients (65.4%) and unilaterally in nine patients (34.6%) in the undescended

testes 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) on

physical examination. In the undescended testes group, three patients (11.5%) had additional urinary pathology and

ten patients (38.5%) had other system anomalies. Ten patients (25.5%) had suspicious external genitalia on physical

examination in the proximal hypospadias group who had both testes in scrotum. Additional urinary pathology was

detected 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 distal

hypospadias (2.8%) had undescended testes. Of these, five were bilateral (55.6%) and 4 had unilateral undescended

testes (44.4%). None of the patients with distal hypospadias had any suspicious genitalia on physical examination. In

the undescended testes group, one patient (11.1%) had additional urinary pathology and one patient had other system

anomaly. 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 by

an undescended testicle, and detailed anamnesis and physical examination covering all systems is necessary in all

hypospadiac children in terms of additional anomalies.

Kaynakça

  • 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.

Hipospadias ve İnmemiş Testis Birlikteliğinde Ek Anomali Sıklığının Değerlendirilmesi

Yıl 2019, Cilt: 13 Sayı: 4, 222 - 226, 30.07.2019
https://doi.org/10.12956/tjpd.2018.361

Öz

Amaç: Hipospadias erkek çocuklarda 200-300 canlı doğumda bir görülen genitoüriner sistem (GÜS) anomalisidir.
Hipospadias olgularına eşlik eden anomaliler arasında inmemiş testis saptanması sıktır. Özellikle proksimal hipospadias
ve bilateral inmemiş testis birlikteliği cinsel farklılaşma bozukluklarına işaret edebilir. Bu çalışmada inmemiş testisin eşlik
ettiği hipospadiaslı çocuklarda ek anomalilerin değerlendirilmesi amaçlandı.
Gereç ve Yöntemler: 2007-2016 yılları arasında hipospadias nedeniyle ameliyat olan 392 çocuğun verileri geriye dönük olarak tarandı.
Bunlardan 65 (%16.6) olguda meatus skrotal ve penoskrotal, 327’sinde (%83.4) glanüler, koronal, subkoronal ve midpenil yerleşimliydi.
Olgular her iki testisi yerinde olan ve olmayanlar olarak ayrıldı ve ek anomaliler kaydedildi.
Bulgular: Proksimal hipospadiaslı çocukların ortanca yaşı 21 ay (6-240 ay)’dı. Proksimal hipospadiaslı çocuklardan 26’sının (%40) inmemiş
testisi mevcutken, 39 (%60) olguda testisler skrotumda ele geldi. İnmemiş testisi olan gruptaki hastaların 17’sinde bilateral (%65.4),
dokuzunda tek taraflı (%34.6) inmemiş testis saptandı. İnmemiş testisi olan grupta 24 hastada (%92.3) fizik muayenede kuşkulu dış
genitalya (bifid skrotum, penoskrotal transpoziyon, mikropenis ve penoskrotal hipospadias beraberliği, skrotalize labium majus) mevcuttu.
İnmemiş testisi olan grupta üç hastada (%11.5) ek üriner anomali, on hastada (%38.5) ise diğer sistem anomalilerinin eşlik ettiği saptandı.
Her iki testisin ele geldiği proksimal hipospadias hasta grubunda 10 hastada ( %25.5) kuşkulu dış genitalya mevcuttu. Her iki testisi
skrotumda ele gelen 39 hastanın birinde (%2.5) ek üriner patoloji, beşinde (%12.8) ise diğer sistem anomalilerine rastlandı.
Distal hipospadiaslı çocukların ortanca yaşı 33 ay (6-156 ay)’dı. Distal hipospadiaslı 327 çocuğun 9’unda (%2.8) inmemiş testis mevcuttu.
Bunlardan beşinde bilateral (%55.6), 4’ünde tek taraflı (%44.4) inmemiş testis izlendi. Distal hipospadiası olan hastaların hiç birinde (%0)
fizik muayenede kuşkulu genitalya saptanmadı. İnmemiş testisi olan grupta bir hastada (%11.1) ek üriner patoloji, bir hastada (%11.1) ise
diğer sistem anomalilerinin eşlik ettiği saptandı. Her iki testisi skrotumda ele gelen yedi hastada (%2.2) ek üriner patoloji, 32 hastada (%9.8)
ise diğer sistem anomalilerine rastlandı.
Sonuç: Sonuçlarımıza dayanarak inmemiş testisin eşlik ettiği proksimal hipospadiaslı olgularda ürogenital sisteme yönelik ultrasonografi,
gerektiğinde pelvik MRG, endokrinolojik analiz, genetik inceleme gibi ileri tetkik gerekliliğinin akılda tutulması gerektiği, tüm hipospadiaslı
çocuklarda ek anomaliler açısından tüm sistemleri içeren ayrıntılı anamnez ve fizik muayene ile tetkik edilmesi gerektiği kanısındayız

Kaynakça

  • 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.
Toplam 14 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular İç Hastalıkları
Bölüm ORIGINAL ARTICLES
Yazarlar

Günay Ekberli

Yayımlanma Tarihi 30 Temmuz 2019
Gönderilme Tarihi 25 Ocak 2018
Yayımlandığı Sayı Yıl 2019 Cilt: 13 Sayı: 4

Kaynak Göster

Vancouver Ekberli G. Evaluation of Additional Anomalies in Concomitance of Hypospadias and Undescended Testes. Türkiye Çocuk Hast Derg. 2019;13(4):222-6.

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