Urogenital Sinus Developmental Anomaly with Phallus and Accessory Phallic Urethra Presented as Disorder of Sex Differentiation in Female

Figure 1: Enlarged phallus with accessory phallic urethra in female (46XX) Figure 2: Absent labia minora and normal vaginal and anal openings Figure 3 – Schematic diagram showing phallus with accessory phallic urethra A newborn presented on second day of life with enlarged phallus with urethral opening in it (Fig. 1), im-palpable external gonads, normal vaginal opening, and normal anus (Fig. 2). Mother had no signs of viri-lisation and no history of androgenic drug intake in antenatal period. MRI showed normal uterus, bilateral ovaries and normal other abdominal organs. A phallic urethra was complete and 3F catheter can be introduced into the phallic meatus with difficulty, which entered the bladder about 0.5 cm. anterior to the perineal urethra (Fig. 3) as seen on cystoscopy. Genitogram showed normal vagina. Karyotyping was 46 XX. Blood glucose level, serum sodium, serum potassium, and 17 hydroxyprogesteron levels were normal.