Giant accessory female phallic urethral diverticulum.

Anomalies of the urogenital sinus have been described in association with male pseudohermaphroditism, bladder and uterine duplication, and a spectrum of external genital anomalies, including a female phallic urethra. The evaluation and optimal surgical management of these anomalies can be complex. We describe a case of a female patient with an accessory phallic urethra and a urogenital sinus anomaly. The presence of stenosis of the accessory urethral meatus led to the formation of a giant diverticulum with unusual features. The complete repair of the anomaly incorporated creation of labia minora from portions of the diverticulum.

Evaluation and management of genital anomalies in two patients with Klinefelter syndrome and review of literature.

OBJECTIVES: To present the evaluation and management of cryptorchidism and varicocele in patients with Klinefelter syndrome and review the literature. Recent advances in reproductive medicine have resulted in fertility in patients with Klinefelter syndrome. However, cryptorchidism and varicocele can affect the fertility potential on their own. METHODS: We present 2 cases and review the literature with an emphasis on the fertility potential. RESULTS: Germ cells were present in the testicular biopsy of an infant with cryptorchidism but not in the adolescent with a varicocele. The latter biopsy showed tubular sclerosis. CONCLUSIONS: The fertility potential of patients with Klinefelter syndrome and cryptorchidism may be preserved or enhanced by an early orchiopexy. However, the potential benefit of ligation of internal spermatic veins in patients with Klinefelter syndrome and a varicocele warrants further study. Cryopreservation should be considered early in adolescence or adulthood as germ cells become depleted at an accelerated rate after puberty.