Lasfar Wafae, van den Bosch Ronald T A, Pot Dirk Jan, Klijn Aart J, de Wildt Michel J A M, Gratama Jan Willem C
Gelre Ziekenhuizen, Afd Radiologie, Apeldoorn, The Netherlands.
Ned Tijdschr Geneeskd. 2010;154:A155.
Two newborn boys aged 2 and 3 months with unilateral inguinal hernia and a contralateral impalpable, non-scrotal testis, and a third boy aged 2.5 years with an impalpable non-scrotal testis were found to have transverse testicular ectopia. This is an uncommon abnormality in which both gonads migrate toward the same hemiscrotum. We illustrate that unilateral cryptorchidism and a contralateral inguinal hernia may indicate the presence of a rare type of male pseudohermaphroditism: persistent müllerian duct syndrome (PMDS). This syndrome is characterized by the presence of a uterus and fallopian tubes associated with abdominal testes and frequently inguinal hernia in a phenotypically and genotypically normal male. This syndrome is often discovered during repair of inguinal hernia or non-descended testes (cryptorchidism). Pre-operative ultrasonography in children with impalpable non-scrotal testis and a contralateral inguinal hernia (patent processus vaginalis) may enable an early diagnosis of transverse testicular ectopia and proper surgical planning. Surgical orchidopexy was carried out and in the first two patients resection of the müllerian duct remnant (utriculus masculinus).
两名年龄分别为2个月和3个月的男婴患有单侧腹股沟疝,对侧睾丸无法触及且不在阴囊内,还有一名2.5岁的男婴睾丸无法触及且不在阴囊内,他们均被发现患有睾丸横过异位。这是一种罕见的异常情况,即两个性腺都向同一侧阴囊迁移。我们证明,单侧隐睾和对侧腹股沟疝可能提示存在一种罕见类型的男性假两性畸形:持续性苗勒管综合征(PMDS)。该综合征的特征是在表型和基因型正常的男性中,存在与腹腔内睾丸相关的子宫和输卵管,且常伴有腹股沟疝。这种综合征常在腹股沟疝修补术或隐睾手术中被发现。对于睾丸无法触及且不在阴囊内同时伴有对侧腹股沟疝(鞘状突未闭)的儿童,术前超声检查可能有助于早期诊断睾丸横过异位并进行恰当的手术规划。已实施手术睾丸固定术,前两名患者还切除了苗勒管残余(男性子宫)。