Resumen

COLMENARES, Belkis; COLMENARES, Jesús; VILELA, Ottoniel  y  ALONSO, José Francisco. Síndrome de Mayer-Rokitansky-Kuster-Hauser: a propósito de un caso. Rev Obstet Ginecol Venez [online]. 2007, vol.67, n.2, pp.131-134. ISSN 0048-7732.

The case of a 26 years old patient that consulted for primary amenorrhea is presented. Physical examination: phonotypical female, neck with wide base, normal external genitalia, internal genitalia with absence of vagina. Complementary exams: abdomino-pelvic ultrasound, TAC of abdomen and pelvis, elimination urography: absence of uterus, left kidney and its excretory system; unique rotated and ectopic (pelvic) right kidney, with ecstasy of the excretory system. Radiology: fusion of C5 - C6 vertebral bodies with reduction of AP diameters, cervical ribs, elevation of scapula, spina bifida in S1 and right sublujation of coccigeal segments. Normal function of the hypothalamus-hypofisis-ovary axis. Karyotype: 46 XX. Laparoscopy confirm the diagnostic of Mayer-Rokitansky-Kuster-Hauser syndrome. A vulvovaginoplasty was performed using a skin graft.

Palabras clave : Syndrome of Mayer; Rokitansky; Kuster; Hauser. Absence of vagina and uterus.