Nadir Bir Olgunun Yönetimi: Kalıcı Mülleryen Kanal Sendromu ile İlişkili Transvers Testiküler Ektopi

Transverse testicular ectopia (TTE) is a rare congenital anomaly in boys, which is characterized by the migrate of both testicles towards the same hemiscrotum or inguinal region. TTE cases are accompanied by the characterized persistent mullerian duct syndrome, which is phenotypically male, with the presence of mullerian duct structures. Persistent Müllerian duct syndrome is caused either by the genetic defect of the anti-mullerian hormone or its receptor. In this case, we reported a TTE associated with persis-tent mullerian duct syndrome in a 12-year-old boy who presented with swelling in the right groin and non-palpable left testis. The patient was treated with high ligation of the hernia sac, excision of mullerian structures and transseptal orchiopexy. In the surgical approach of the cases, we think that removing the mullerian structures as much as possible and making the testicles palpable without damaging the testicles and other adjacent structures are important.

Management of a Rare Case: Transverse Testicular Ectopia Associated With Persistent Mullerian Duct Syndrome

Transvers testiküler ektopi (TTE), her iki testisin de aynı hemiskrotuma veya inguinal bölgeye migrasyonu ile karakterize erkek çocuklarda nadir görülen bir konjenital anomalidir. TTE olgularına, müllerian kanal yapılarının varlığı ile birlikte fenotipik olarak erkek olan, karakterize kalıcı müllerian kanal sendromu eşlik eder. Persistan Müllerian kanal sendromu, anti-müllerian hormonun veya reseptörünün genetik kusurundan kaynaklanır. Bu olguda, sağ kasıkta şişlik ve palpe edilemeyen sol testis ile başvuran 12 yaşında bir erkek çocukta persistent müllerian duktus sendromu ile ilişkili bir transvers testis ektopisi bildirdik. Hasta fıtık kesesinin yüksek ligasyonu, müllerian yapıların eksizyonu ve transseptal orşiopeksi ile tedavi edildi. Olguların cerrahi yaklaşımında, testislere ve diğer komşu yapılara zarar vermeden, müllerian yapıların mümkün olduğunca çıkarılması ve testislerin palpe edilebilmesi gerektiğini düşünüyoruz.

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