Persistent Müllerian Duct Syndrome: A Rare Etiology of Cryptorchidism

Objective & Hypothesis:ABSTRACT Persistent Müllerian duct syndrome (PMDS) is a male sexual differentiation disorder which is very rare and usually diagnosed incidentally.

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Key Findings & Data:In childhood, PMDS is related with chryptorchidism and infertility and increased testicular cancer in adulthood, therefore rapid onset diagnosis and treatment is very important.

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Conclusion & Impact:Key Words: Müllerian Ducts, Tstis, Cryptorchidism

🔬Design:Research Article

📍Scope:Comprehensive Sample

⚙️Variables:Müllerian kanal · testis · kriptorşidizm

Abstract

ABSTRACT Persistent Müllerian duct syndrome (PMDS) is a male sexual differentiation disorder which is very rare and usually diagnosed incidentally. It is characterised by unregressed Müllerian duct remnants in males, due to mutations in Anti-Müllerian hormone and/or its receptor genes. In childhood, PMDS is related with chryptorchidism and infertility and increased testicular cancer in adulthood, therefore rapid onset diagnosis and treatment is very important. PMDS is usually recognised in patients which are phenotipically male character, during investigations or surgery for chryptorchidism. Computed tomography (CT) or magnetic resonance imaging (MRI) can be usefull for diagnosis, however, diagnostic laparoscopy may performed in suspicious cases. In our study, twenty-one years old male is presented, which is admitted to outpatient clinic with hematospermia and inguinal pain, and Müllerian duct remnants have seen above bladder during diagnostic laparoscopy. Key Words: Müllerian Ducts, Tstis, Cryptorchidism

Özet

ÖZ Persistan Müllerian kanal sendromu (PMKS) oldukça nadir görülen ve genellikle insidental olarak tanı konulan erkek cinsel gelişim bozukluğudur. Anti-Müllerian hormon (AMH) veya reseptöründeki bir mutasyon sonucu Müllerian kanal yapılarının regrese olmamasıyla karakterizedir. Çocukluk çağında kriptorşidizmin eşlik ettiği, erişkin dönemde ise buna bağlı olarak infertilite ve artmış testis kanseri ile ilişkili olan PMKS’un erken dönem tanı ve tedavisi oldukça önemlidir. Fenotip olarak erkek karakterde bireyde çoğunlukla kriptorşidizm için yapılan araştırmalar veya cerrahi tedavi sırasında fark edilir. Bilgisayarlı tomografi (BT) veya manyetik rezonans görüntüleme (MRG) tanıya yardımcı olabilir ancak şüpheli olgularda tanısal laparoskopiden faydalınabilir. Çalışmamızda hematospermi ve inguinal ağrı ile kliniğimize başvuran ve laparoskopi sırasında mesane süperiorunda Müllerian kanal yapılarının bulunmasıyla tanı konulan yirmi bir yaşında erkek hasta sunulmuştur. Anahtar Sözcükler: Müllerian Kanal, Testis, Kriptorşidizm

Keywords

Müllerian kanal testis kriptorşidizm Müllerian Ducts Tstis Cryptorchidism

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Cite This Article

BATMAZ, O., CAYLAN, A. E., UÇAR, M., KÖKSAL, İ. T. (2022). Persistent Müllerian Duct Syndrome: A Rare Etiology of Cryptorchidism. Akdeniz Tıp Dergisi, Cilt: 8 - Sayı: 2, 232–235. https://ajindex.com/articles/725643

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