A differential diagnosis between the true hermaphroditism (TH) and mixed gonadal dysgenesis (MGD) has important clinical implications for gender assignment and the decision for early gonadectomy; however, variable clinical and histological features frequently lead to the confusion of TH with MGD. A 17- month-old boy was presented with proximal hypospadias with chordee and right non-palpable testis in his scrotum. He also had right auricular anomaly including a separated tragus with skin tag. Left testis was well palpable in his left scrotum. Diagnostic right inguinal exploration showed M?llerian structures such as a gonad like an ovary and a fallopian tube with a uterus, which were removed. Repair of hypospadias and right auricular anomaly was also done. Following ultrasonography (USG) showed a normal looking testis in left scrotum. His chromosome was 45, XO/46, XY. We report a difficult case of mixed gonadal dysgenesis mimicking true hermaphroditism which combines ipsilateral congenital auricular anomaly.

There have been no definitive preoperative diagnostic imaging studies for impalpable testes. We observed the effectiveness of laparoscopy for detecting impalpable testes not identified with ultrasonography (USG) or careful physical examination under general anesthesia. We retrospectively reviewed 117 patients (118 testes) who were operated upon for undescended testes from January 1998 to December 2004. The testes of these patients were palpable in 97(82 %) and impalpable in 21 (18 %). We analyzed the preoperative diagnostic method, site of the testes, operative method and operative findings of the 21 impalpable testes. Preoperative USG and physical examination under general anesthesia were performed on 20 patients, and 12 patients' testes could be localized. Eight patients whose testes could not be localized with USG and physical examination underwent laparoscopy. Seven of the 8 patients had testes in inguinal canal and 4 of these were atrophied and underwent orchiectomy because of atrophy (2) and vanishing (2). Only 1 patient had bilateral intraabdominal testes and one of the testes was atrophied. Laparoscopy was a useful method for detecting impalpable testes, but the clinical application might be limited because the location of atrophic or vanishing testes was mainly inferior to internal inguinal ring.