Congenital scrotal disorders are unusual, including penoscrotal transposition, bifid scrotum, ectopic scrotum and accessory scrotum. The latter 2 entities are extremely rare. Because accessory scrota usually arise in the absence of associated anomalies, an etiology has been enigmatic. We present 2 cases of accessory labioscrotal folds, including a female patient. An exhaustive literature review allowed comparison with all reported cases (23) and showed a frequent association with perineal lipoma (83%). On that basis, we were able to classify accessory labioscrotal folds into 2 types and recommend a different course of management for each. Accessory labioscrotal folds usually develop when intervening mesenchymal tissue disrupts the continuity of the caudally developing labioscrotal swelling.
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