Özet:

Persistent Mullerian Channel Syndrome (PMKS) is a picture characterized by the presence of Mullerian residues in the male phenotype (the top 1/3 of the uterine, uterus and vagina). Mullerian inhibitor factor (MIF) is a male psodohermafroditism that occurs as a result of a lack of release from the fetal serum cells. This syndrome may rarely be accompanied by transversal testicular ectopy (detection of two testicles in the same scrotal comparison; TTE). TTE is a rare pathology and the number of phenomena is around 120. Patients are usually in normal male appearance, accompanied by one- or two-sided non-sided testicles. This article presents the PMKS and TTE association seen in a three-month male patient. A three-month-old boy was brought to our clinic with a right-handed swelling. In physical examination; except for men, the penis and scrotum were well developed, the right testis was in normal location, the right inguinal channel was massed, and the left testis was nonpalpabl. During the exploration, both gonads and mullerian channels were detected in the right fist. The right cord structures were separated from the muillerian structures and placed in the left testis scrotum. In the chromosome analysis, 46 XY caryotypes were. We believe that due to the risk of tumor that may develop in the PMKS floor, the muillerian waste should be removed in such a way that the testicular and vasal deference are not damaged. We believe that the long-term observations of the facts left by the residual muellery tissue are important in terms of early recognition of malignant conversion. Keywords: Transversal testicular ectopy, Mullerian channel syndrome, inguinal herni.   Abstract Persistent Mullerian duct syndrome (PMDS) is characterized by the presence of Mullerian duct structures such as fallopian tubes, uterus and 1/3 upper part of vagina in the phenotypic male and is considered to be caused by the failure of synthesis or action of Mullerian inhibitor substance. It is a rare form of male pseudohermaphroditism. This syndrome is rarely associated with transverse testicular ectopia (both testicles migrate toward the same hemiscrotum). PMDS and transvers testicular ectopi is extremely rare; there are about 120 cases in the literature. Patients are normally virilised, although uni- or bilateral cryptorchidism may be present. In this paper we report a PMDS associated with transverse testicular ectopia in a size. A 3-month-old-boy was admitted with right inguinal swelling. In this paper we report a PMDS associated with transverse testicular ectopia in a 3-mounth-old-boy. Physical examination demonstrated a normal phallus and scrotum but left testis was not palpable. At operation, both gonads and muillerian remnants with structures that appeared to be a well-developed uterus and fallopian tubes were found in right hernia sac. The Müllerian remnants was excised, bilateral orchidopexy was performed. Chromosomal analysis revealed a 46, XY, caryotype. Bilateral orchidopexy and resection of the remnant tissue is of utmost is the operative procedure of choice. These muillerian remnants are at an increased risk of malignant transformation, so remnants should be monitored for tumor formation in such cases. Keywords: Transverse testicular ectopia, muillerian duct syndrome, inguinal hernia.

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