The diagnosis of mullerian duct or utricular cyst should be considered in a child with urinary difficulties and a palpable midline, anterior rectal mass. Endoscopic cannulation of the cyst has been found to be the most useful diagnostic test. Infection should be treated with the appropriate antibiotics before definitive treatment. Surgical excision offers the best result. The transperitoneal and posterior parasacral approaches have been described but we favour the transvesical, transtrigonal approach which we find highly satisfactory. The risk of malignancy at a later age is an added indication for surgery.
An eighteen month old phenotypically and genotypically normal male child was admitted with a left inguinal hernia and a right undescended testis. At operation, he was found to have a uterus, bilateral fallopian tubes, and a vagina in the left hernial sac. Bilateral orchidopexies and excision of the persistent Mullerian duct structures were carried out. This rare case of persistent Mullerian duct syndrome is due to a defect in Mullerian regression, which is in turn controlled by the Mullerian inhibiting substance (MIS). Orchidopexy with excision of the persistent Mullerian duct structures is usually not possible without damage to the vas deferens which is closely adherent to the wall of the uterus. The alternative of leaving the persistent Mullerian duct structures alone and performing a staged or primary orchidopexy has been suggested.
Obstructed Hemi Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rarely encountered müllerian duct anomaly. Delayed diagnosis is common due to normal onset of puberty and menstruation. We report a case of a woman in her early 20s with a background history of multiple emergency department visits, ward admissions and surgeries for chronic abdominal pain. She was reviewed at 1 month postlaparotomy for recurrent pelvic abscess and was finally diagnosed to have an OHVIRA syndrome, 11 years after her first clinical presentation. Excision of the vaginal septum completely resolved her symptoms. We are reporting this case to highlight the clinical implications resulting from the delayed diagnosis, to look into factors contributing to the delay and to highlight the importance of having a high index of suspicion to diagnose this unique condition.