非触知精巣に対する腹腔鏡検査でミュラー管遺残症と診断した1例

Abstract

Persistent mullerian duct syndrome describes a group of patients with a 46 XY karyotype and normal male external genitalia, but internal mullerian structures. A 7-month-old boy presented with a left inguinal hernia, a left undescended testis and a right impalpable testis. Inguinal herniorrhaphy was performed and laparoscopy was done for search of a right impalpable testis and internal genitalia simultaneously. Laparoscopic examination revealed the structure like a mullerian duct remnant along the left spermatic duct in rectovesical fossa and right intra-abdominal testis. The structure like a mullerian duct remnant was not removed to preserve the left spermatic duct. Left orchiopexy and right laparoscopic staged Fowler- Stephens orchiopexy were performed. Chromosomal analysis was 46 XY karyotype and we diagnosed this case as persistent mullerian duct syndrome.