Chung Ho Seok, Kim Sun-Ouck, Yu Ho Song, Kim Sung-Sun, Kwon Dong Deuk
Departments of Urology.
Pathology, Chonnam National University Medical School, Gwangju, Korea.
Medicine (Baltimore). 2018 Nov;97(48):e13305. doi: 10.1097/MD.0000000000013305.
Persistent Müllerian duct syndrome (PMDS) is rare form of male pseudohermaphroditism characterized by the presence of uterus and fallopian tubes with normal external genitalia and secondary sexual characteristics. Transverse testicular ectopia (TTE) is also a rare form of testicular ectopia that may be associated with PMDS.
We present a 2-year-old boy who presented with bilateral non-palpable testes with left inguinal mass.
TTE with PMDS.
On exploration, both testes were present in the left inguinal region. Uterus and fallopian tubes were located between the testes. A hysterectomy was perfomed with resection of the underdeveloped fallopian tubes. Bilateral orchiopexy was performed by placing both gonads into subdartos pouches in each scrotum with transseptal approach.
Both testes were palpable in both the scrotum at 1-year postoperative follow-up and we are planning a regular follow-up.
In case of TTE with PMDS, optimal surgical approach with orchiopexy and excision of Müllerian duct should be needed. A long-term postoperative follow-up is necessary for assessment of malignant transformation and infertility.
持续性苗勒管综合征（PMDS）是男性假两性畸形的一种罕见形式，其特征为具有正常的外生殖器和第二性征，但却存在子宫和输卵管。睾丸横过异位（TTE）也是一种罕见的睾丸异位形式，可能与PMDS有关。
我们报告一名2岁男孩，其双侧睾丸无法触及，左侧腹股沟有肿块。
TTE合并PMDS。
术中探查发现双侧睾丸位于左侧腹股沟区。子宫和输卵管位于双侧睾丸之间。行子宫切除术并切除发育不全的输卵管。采用经中隔入路将双侧性腺放入每个阴囊的肉膜囊内行双侧睾丸固定术。
术后1年随访时双侧睾丸均可在阴囊内触及，我们正计划进行定期随访。
对于合并PMDS的TTE病例，需要采用最佳的手术方法进行睾丸固定术并切除苗勒管。术后长期随访对于评估恶变和不育情况是必要的。
