Surgical resection therapy of a rare presentation of persistent Mullerian duct syndrome: a case review.

Persistent Mullerian Duct Syndrome (PMDS) is an extremely rare disease with less than 300 cases recorded in medical literature. Our patient was a 37 year old male who presented at the medical office with hematospermia as his sole complaint. He had previously undergone left orchidopexy and presented with hypotrophic left testicle and right testicle agenesis. PMDS differential was considered with the clear observation of a uterus-like structure during pelvic ultrasonography. The organs were later studied in magnetic resonance imaging and confirmed by post-surgery anatomopathological examination. Patient was discharged 24 h after surgery and developed azoospermia post-surgery.

Operative correction of an extremely rare condition called Persistent Mullerian Duct Syndrome (PMDS). What is PMDS? PMDS is a disease which has less than 300 cases in medical literature. It is a congenital condition characterized by the development of female genital organs such as the uterus and ovaries, in an otherwise normal male individual. The fetal development of these structures begins when the male fetus develops his genitalia, during the period when he must produce a hormone (anti-mullerian hormone), which suppresses female genitalia growth. Since this fetal stage is the turning point for genital development, lack of this hormone commonly results in the presence of functional female genital organs in an adult male, which characterizes the syndrome. Multiple reports also associate the syndrome with ectopic testis (cryptorchidism) or gonadal absence and dysfunctional sexual cell production. What was the aim of the report? The aim is to present a rare presentation of an already extremely rare disease in order to enrich the literature with another case of PMDS and the outcome of surgical correction. How was the patient treated? After discovering female organs in the male pelvis during an ultrasound scan, an elective surgery was performed to evaluate the removal of the uterus, fallopian tubes, ovary and vaginal canal through video laparoscopy. Why is this case important? The overall medical knowledge about PMDS is rather limited due to the reduced number of cases and the relatively wide variety of presentations. This article is useful to present a rather rare presentation, in which cryptorchidism and testicular agenesis were concomitant with hematospermia. Other than that, the diagnosis was done late in the patient's life, having lived over three decades with female genitals in his pelvis without any malignant (cancerous) mutations. The case report can also provide a record for the outcome of azoospermia, which is the absence of motile (and hence viable) sperm in the semen, following a non-complicated post-surgical recovery, which suggests unknown mechanisms may be involved in gonadal development after birth, and a different endocrine balance in patients with the syndrome.

[Accuracy of oncotic cytology for HPV infection diagnosis on the cervix uteri of HIV-infected women]. / Acuidade da citologia oncótica para o diagnóstico da infecção pelo HPV no colo uterino de mulheres portadoras do HIV.

PURPOSE: to verify the accuracy of uterine cervix cytology for HPV diagnosis, as compared to polymerase chain reaction (PCR) in samples of women with HIV. METHODS: 158 patients who had undergone a first collection of material from the uterine cervix with Ayre's spatula for PCR were included in the study. Then, another collection with Ayre's spatula and brush for oncotic cytology was performed. Only 109 slides were reviewed, as 49 of them had already been destructed for have being filed for over two years. RESULTS: the prevalence of HPV was 11% in the cytological exam and 69.7% in the PCR. Age varied from 20 to 61 years old, median 35 years. The HIV contagious route was heterosexual in 91.8% of the cases, and 79.1% of the patients had had from one to five sexual partners along their lives. The most frequent complaint was pelvic mass (5.1%), and 75.3% of the women had looked for the service for a routine medical appointment. The categorical variable comparison was done through contingency tables, using the chi2 test with Yates's correction to compare the ratios. The Fisher's test was used when one of the expected rates was lower than five. In the comparison of diagnostic tests, sensitivity, specificity and similarity ratios have been calculated. Among the 76 patients with HPV, detected by PCR, only 12 had the diagnosis confirmed by cytology (sensitivity=15.8%), which on the other hand did not present any false-positive results (specificity=100%). Concerning the HPV presence, the cytological prediction for positive results was 100% and 33.3% for negative, when both results were compared. Among the 12 patients with HPV positive cytology, four (33.3%) presented cervical intraepithelial neoplasia (OR=56; positive similarity ratio=positive infinity; negative similarity ratio=0.83). CONCLUSIONS: As the cytology specificity is quite high, it is possible to rely on the positive result, which means that a positive result will surely indicate the presence of HPV. The low sensitivity of cytology does not qualify it as a survey exam for HPV detection in this female group.