Persistent Mullerian Duct Syndrome (PMDS) is a rare disorder of defective sexual development in males. It is characterized by aberrant synthesis or inadequate action of Mullerian inhibiting factor resulting in derivatives of Mullerian duct, i.e. uterus, fallopian tube and upper vagina, to persist in a phenotypic male with 46XY karyotype. Here, we report a 19-year-old male with bilateral undescended testes. Further evaluation revealed that the patient had both his testes placed intra-abdominally along with a rudimentary uterus.

To evaluate fertility potential after orchidopexy for bilateral undescended testis and compare two surgical fixation techniques for effect on fertility. Men older than 22 years who had either tunica albuginea orchidopexy (TAO) or \"no-touch\" technique (NTO) in childhood for bilateral undescended testis (BUDT) were selected. Participants filled out a questionnaire followed by physical examination, had testicular ultrasound, blood sample and semen analysis. Statistical testing was performed using general linear modelling. Sixty-seven out of 166 individuals responded. Forty-nine completed the questionnaire, and nine (18.3%) reported having fathered children. Thirty-six showed up for further examination, 26 had TAO and 10 NTO. Impaired hormonal spermatogenesis regulation (34.6% vs. 20%), higher subfertility rate (46% vs. 20%) and lower means of motile spermatozoa (58.1 × 106 spz vs. 177.9 × 106 spz) were observed in the TAO versus the NTO group; none of these were statistically significant. Four (15.4%) of the TAO and two (20%) of the NTO group have azoospermia. Although the operation technique did not have a significant impact on fertility, unfavourable outcomes were more common after surgery involving the tunica albuginea of the testis. Larger sample sizes are needed to ascertain whether the trends favouring the NTO technique are of any significance.

OBJECTIVE: We determined relative semen quality in youths diagnosed with cryptorchidism or varicocele as a surrogate for ultimate paternity potential. We hypothesized that youths with varicocele would be at lower risk for subfertility based on semen analysis than their counterparts with surgically corrected cryptorchidism.
METHODS: We retrospectively reviewed the records of patients with a history of cryptorchidism or varicocele. Patients were placed in 1 of 3 groups based on diagnosis, including group 1-untreated varicocele, group 2-treated bilateral cryptorchidism and group 3-treated unilateral cryptorchidism. Age and semen parameters (density, volume, count, motility and total motile count) were compared for each group.
RESULTS: A total of 193 subjects were studied. Median age was 18.3 (IQR 18.1-19.3), 18.6 (IQR 18.3-21.0) and 18.5 years (IQR 18.2-19.6) in the 76 group 1, 21 group 2 and 96 group 3 patients, respectively. Total motile count in groups 1, 2 and 3 was 14.6 (IQR 4.7-29.3), 4.0 (IQR 0-38.0) and 34.1 million sperm (IQR 7.6-90.8), respectively. No significant difference existed between the groups in age, volume (p = 0.106) or motility (p = 0.197). However, density (p = 0.0001), count (p = 0.0001) and total motile count (p = 0.0002) achieved significance. For each of these parameters a significant difference was noted for group 1 vs 3 and group 2 vs 3 but not for group 1 vs 2.
CONCLUSIONS: The semen quality of youths with varicocele more closely resembles that of youths with bilateral cryptorchidism than those with unilateral cryptorchidism. This is concerning and should challenge current treatment paradigms for adolescents with varicocele.