PERSISTENT MULLERIAN DUCT SYNDROME TYPE I: CAUSES, COMPLIKA TIONS, AND MODERN METODS OF DIAGNOSIS AND TREATMENT.
Abstract
Currently, the number of males suffering from Persistent Müllerian Duct Syndrome (PMDS) is increasing worldwide every year. This syndrome is one of the rare disorders of sexual development in phenotypically male individuals with a 46XY karyotype. It is characterized by the presence of remnants (derivatives) of the Müllerian ducts and, in some cases, remnants of the uterus, fallopian tubes, and the upper part of the vagina. The only clinical manifestation of this syndrome is cryptorchidism. This article discusses the causes of Persistent Müllerian Duct Syndrome, its symptoms, complications, and modern methods of diagnosis and treatment.
References
Knebelmann B., Boussin L., Guerrier D., et al. Anti Mullerian hormone Bruxelles: a nonsense mutation associated with persistent Müllerian duct syndrome. PNAS. 1991;88(9):3767 3771.
Barthold J.S., Gonzalez R. The epidemiology of congenital cryptorchidism, testicular ascent and orchiopexy. Journal of Urology. 2003;170:2396 2401.
Brandli D.W., Akbal C., Eugster E., et al. Persistent Müllerian duct syndrome with bilateral abdominal testis: surgical approach and review of literature. Journal of Pediatric Urology. 2005;1(6):423 427.
Naouar S., Maazoun K., Sahnoun L., et al. Transverse testicular ectopia: case report and review of the literature. Urology. 2008;71(6):1070 1073.
Wuerstle M., Lesser T., Hurwitz R., et al. Persistent Müllerian duct syndrome and transverse testicular ectopia: embryology, presentation and management. Journal of Pediatric Surgery. 2007;42(12):2116 2119.
Okulov A.B., Brovin D.N., Shirakov K.K., et al. Diagnosis and surgical correction of persistent Müllerian ducts in children. Pediatric Surgery. 2015.
Chernykh V.B., Kurilo L.F. Persistent Müllerian duct syndrome (literature review). Problems of Reproduction. 2011.
