Gómez Veiga F A, Chantada Abal V, Casas Muiño R, Serrano Barrientos J, González Martín M
Arch Esp Urol. 1989 Jun;42(5):455-8.
We report on a 14-year-old boy who was admitted to the emergency department for acute retention of urine. At age 1 year, he had been submitted to surgery for left cryptorchidism and left-sided herniation, associated with hypospadias of the glans penis. Radiologic and endoscopic work up revealed a large retrovesical cavity with septation in its upper portion communicating with the prostatic urethra. These structures were removed and pathologic examination disclosed remnants of Müllerian ducts. This uncommon disease entity whose etiopathogenesis is ascribed to a deficient activity of the Müllerian inhibiting factor (MIF) classically presents in phenotypically male subjects with unilateral cryptorchidism, contralateral herniation, and persistent Müllerian remnants. The literature is reviewed, highlighting the diagnostic and therapeutic aspects of this syndrome.
我们报告一名14岁男孩，因急性尿潴留入院急诊。1岁时，他因左侧隐睾症、左侧疝气伴阴茎头型尿道下裂接受了手术。影像学和内镜检查发现一个大的膀胱后腔，其上部有分隔，与前列腺尿道相通。切除了这些结构，病理检查发现有苗勒管残余。这种罕见的疾病实体，其发病机制归因于苗勒抑制因子（MIF）活性不足，典型地表现为表型为男性的单侧隐睾症、对侧疝气和持续性苗勒管残余。本文回顾了相关文献，重点介绍了该综合征的诊断和治疗方面。
