Abstract：
PMDS (persistent Müllerian duct syndrome) is a rare disorder of sex development characterised by the presence of Müllerian duct remnants in a phenotypically male individual with a 46XY karyotype. Radiological investigations play a crucial role in diagnosing and characterising this condition. Ultrasound and MRI are the modalities of choice. They help to non-invasively localise the gonads and Müllerian duct derivatives. Broadly, PMDS has two anatomical variants: male type and female type. The case report presented here does not fit into these classically described variants and can be called a variant of the female type. There is a risk of infertility and malignant transformation of undescended testis and Müllerian duct derivatives in cases of PMDS. Hence, management is focused on preventing these risks. Surgical intervention involves orchidopexy, removal of Müllerian duct derivatives and inguinal hernia repair.
摘要：
PMDS（持续性苗勒管综合征）是一种罕见的性发育障碍，其特征是在具有46XY核型的表型男性个体中存在苗勒管残留物。放射学检查在诊断和表征这种情况中起着至关重要的作用。超声和MRI是选择的模式。它们有助于非侵入性地定位性腺和穆勒管衍生物。广义上,PMDS具有两种解剖学变体:男性型和女性型。此处提供的病例报告不适合这些经典描述的变体，可以称为女性类型的变体。在PMDS的情况下，存在不孕症和未降睾丸和苗勒管衍生物恶性转化的风险。因此，管理层的重点是防范这些风险。手术干预涉及睾丸固定术，去除苗勒管衍生物和腹股沟疝修补术。
