ミュラー管遺残症候群の2例

Abstract

1歳男児, 10ヵ月男児。共に鼠径ヘルニアと停留精巣を伴ったミュラー管遺残症候群であった

Persistent Mullerian duct syndrome is characterized by the retention of mullerian derivatives in patients otherwise normally virilized. Clinically, the persistence of uterus and tubes leads either to cryptorchidism or inguinal hernia. The condition is usually discovered at surgery. We report two boys with this anomaly. In each case, hysterectomy and orchidopexy were carried out. The gonads were testes and the karyotype was 46, XY. Long-term follow up for these patients is necessary because of an increased risk of testicular tumors and infertility which will develop in future.