Splenogonadal fusion-limb deformity syndrome: a rare but important cause of undescended testis.

BACKGROUND

Splenogonadal fusion is a rare congenital anomaly which is characterized by fusion formation between the spleen and gonad.

METHODS

We report a case of a 14-month boy with spleongonadal fusion-limb deformity syndrome focusing on the importance of awareness of this syndrome.

RESULTS

The patient was admitted to our clinic because of a left undescended testis, and preoperative diagnosis was not made. During the operation, "spleen-like" tissue attached to the gonad induced splenogonadal fusion, which was confirmed by laparoscopy. The patient also had a short right femur, hip dysplasia and a syndromic face.

CONCLUSION

Splenogonadal fusion anomaly should be considered in the evaluation of undescended testis, especially in patients with facial and limb deformities.