Resumen

BORREGO LOPEZ, Julio Aurelio; VARONA SANCHEZ, Joel Alejandro; ARECES DELGADO, Georgina  y  FORMOSO MARTIN, Luis Ernesto. Morris Syndrome. Rev Cubana Obstet Ginecol [online]. 2012, vol.38, n.3, pp. 415-423. ISSN 0138-600X.

Introduction:the syndrome of complete androgen resistance, testicular feminization or Morris syndrome may occur in one in 20 000 to 64 000 of male newborns. Objective: to present a case with male genotype and female phenotype given by breast development, female external genitalia with hypoplasia of the labia and very short vagina ending in a blind pouch Methods: a 19 year- old female patient is assisted in consultation due primary amenorrhea and unsatisfactory sexual contact (impossibility of penetration). Results: at examination, we found a rudimentary vagina of about two inches and underdeveloped external genitalia. The exams confirmed increased LH and testosterone levels and decreased estradiol. It is also found a 46 XY karyotype, so Morris syndrome is suspected. Combined surgery is planned for the vagina and abdomen. Total videolaparoscopy right adnexectomy and vaginal reconstruction by technique of Williams are performed. We had satisfactory postoperative and subsequent follow-up consultation where functional vagina and emotional stability of the patient were checked. Conclusions: the pathological study verifies the existence of ovary and testis in the surgical specimen confirming the diagnosis of Morris syndrome or complete androgen resistance.

Palabras clave : primary amenorrhea; Morris syndrome; rudimentary vagina; androgen resistance.

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