Van Lanschot J J, Hazebroek F W, Drop S L, Ten Kate F W
Tijdschr Kindergeneeskd. 1985 Dec;53(6):227-32.
Persistent Müllerian duct syndrome (also known as uterine inguinal hernia) is described and two cases are reported. In this rare type of male pseudohermaphroditism there is no disturbance of either primary or secondary external masculinization. Consequently, this anomaly is always diagnosed incidentally, generally at operation for cryptorchidism or inguinal hernia. In addition to bilateral normal testes, these patients possess a uterus with two uterine tubes. The persistence of the internal female genitalia is caused by the insufficiency of the MIF hormone (Müllerian Inhibiting Factor). Early orchiopexy is recommended. Removal of the female internal genitalia is seldom required.
本文描述了持续性苗勒管综合征(又称子宫腹股沟疝)并报告了两例病例。在这种罕见的男性假两性畸形类型中,原发性或继发性外生殖器男性化均无异常。因此,这种异常通常是在偶然情况下被诊断出来的,一般是在因隐睾症或腹股沟疝进行手术时发现。除了双侧正常睾丸外,这些患者还拥有子宫及双侧输卵管。女性内生殖器的持续存在是由苗勒管抑制因子(MIF)激素不足所致。建议尽早进行睾丸固定术。很少需要切除女性内生殖器。