Androgen Insensitivity Syndrome (AIS) is a disorder wherein a patient presents with a female phenotype but is actually genetically male with an XY karyotype. Typically, AIS is diagnosed at the beginning of second decade, when a phenotypically female patient complains of amenorrhea. It is extremely rare to make a first diagnosis of AIS after the fifth decade of life. This case report presents a 62-year old female who consulted because of primary amenorrhea and intraabdominal mass. Patient was diagnosed with Complete Androgen Insensitivity Syndrome based on physical exam findings, imaging studies, endocrine tests and karyotyping. She underwent exploratory laparotomy, adhesiolysis and bilateral orchiectomy. This report will discuss diagnosis and appropriate management of patients with Complete Androgen Insensitivity.
Androgen-Insensitivity Syndrome

Mullerian anomalies arise from the failure in the development of Mullerian ducts and their associated structures during organogenesis which confers adverse impact in fertility and reproductive health. Presented is a rare case of a 15 year old nulligravid, who presented with a chief complaint of severe cyclic hypogastric pain associated with primary amenorrhea. Complete clinical history, physical examination and sonographic findings pointed to a diagnosis of cervical hypoplasia associated with functioning uterine corpus and an absent vagina. Patient underwent total abdominal hysterectomy with left salpingectomy and bilateral oophorocystectomy, for hematometra, bilateral endometriotic cysts, and hematosalpinx. This case report discusses the management of cervicovaginal agenesis through a multidisciplinary approach by a team composed of an obstetrician-gynecologist, reproductive endocrinologist, pediatrician, and pediatric surgeon for proper evaluation, diagnosis, and management of this case.
Hematometra