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首页> 外文期刊>Journal of Clinical and Diagnostic Research >A Rare Case Report of Inguinal Hernia with Persistent Mullerian Duct and Klinefelter Syndrome
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A Rare Case Report of Inguinal Hernia with Persistent Mullerian Duct and Klinefelter Syndrome

机译：腹股沟疝持续性苗勒氏管和克氏综合征的罕见病例报告

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Inguinal hernia in male is a common problem but having female reproductive organs in hernial sac is rare. It occur because of failure of mullerian duct to regress in a male fetus during embryonic development, result in a syndrome known as Persistent Mullerian Duct Syndrome (PMDS), which is a rare entity of male pseudohermaphroditism. We hereby present a case of 21-year-old male patient reported with complains of cryptorchidism and inguinal hernia. Generally diagnosis of PMDS was established during investigation like ultrasonography, MRI for localization of undescended testis and during surgical exploration for inguinal hernia or cryptorchidism. Our patient was operated by bilateral inguinal incision; hernial sac contained adult size uterus fallopian tube and upper 2/3rd of vagina. On karyotyping it was found that he was a case of klinefelter syndrome also. Association of PMDS with klinefelter syndrome is very rare.
机译：男性腹股沟疝是一个普遍的问题，但在疝囊中具有女性生殖器官的情况很少。发生这种现象的原因是，在胚胎发育过程中，胎儿的苗勒氏管无法退化，从而导致一种称为持久性苗勒氏管综合症的综合征（PMDS），这是雄性假两性皮炎的一种罕见现象。我们特此介绍一例21岁男性患者，据报道其患有隐睾症和腹股沟疝。通常，在进行诸如超声检查，MRI对未降睾丸的定位等调查以及针对腹股沟疝或隐睾的外科手术探查过程中，可以确定PMDS的诊断。本例采用双侧腹股沟切口手术。疝囊包含成年大小的子宫输卵管和阴道上部2/3。在进行核型分析时，发现他也是克林费尔特氏综合症的病例。 PMDS与克氏综合征的关联非常罕见。

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