Atypical form of Mayer-Rokitansky-Küster-Hauser syndrome: A case report.

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital disorder syndrome characterized by failure of the uterine and vaginal organs to develop normally. The prevalence of MRKH is estimated to occur in about 1 in 5000 of female live births. A 25-year-old female patient comes to a general obstetric and gynecological polyclinic with complaints of not being able to menstruate at all since birth. There is a history of vaginal discharge but it is neither viscous nor had an odor. On ultrasound examination, the structure of the uterus and ovaries was not found in a normal place. On follow up MRI examination showed agenesis of the uterus and proximal two-third of the vagina accompanied by abnormal positioning of both ovaries, thus supporting to atypical form of MRKHS. The patient is not given drug therapy, but she was planned to transplant the uterine organs. This case report suggests MRKH syndrome can be characterized by ectopic ovaries and a uterus that is not fully developed and can also be accompanied by agenesis of the vaginal organs. Pelvic ultrasound is the main modality chosen to be performed in patients with symptoms of primary amenorrhea. When pelvic organs cannot be visualized properly, it will be performed MRI examination. MRI examination is known to have sensitivity and specificity up to 100% in diagnosing MRKH syndrome. This case report describes a 25-year-old woman with primary amenorrhea with MRKH syndrome. MRI is a sensitive and specific examination to confirm the diagnosis.

Prepared for the Unexpected: Accessory Ovaries with Abernethy Malformation.

Supernumerary and accessory ovaries are collectively coined ectopic ovaries. These are rarely encountered by the benign gynecologist and are often discovered incidentally during evaluation for other gynecologic, gastrointestinal, or urologic pathologies. We report the presentation of a patient with multiple accessory ovaries in addition to a rare congenital anomaly of the splanchnic vasculature called an Abernethy malformation. Incidental identification of ectopic ovaries necessitates a search for additional malformations outside of the genitourinary tract that can have larger implications for long-term health.

A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation.

Altinay-Kirli E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.