Two-step surgery for a unique case of idiopathic female pseudohermaphroditism Surgical treatment in an exceptional combined uro-genital malformation


COD: 06_2012_571-576 Categorie: ,

Nicolò Scuderi, Giovanni Alei, Michele Maruccia, Piero Letizia, Valentina Sorvillo, Francesco Ricottilli, Lavinia Alei, Maria Giuseppina Onesti

Ann. Ital. Chir., 2012 83: 571-575

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A 15 years-old patient was brought to our attention in June 2010 with a complex genito-urinary malformation: fusion of the labia majora and a partial union of labia minora, common urinary and vaginal meatus and clitoromegaly. The patient had a normal sexual feminine hormonal development. After diagnostic iter the patient underwent surgery twice. No signs of hormonal deficency was shown authorizing us to approach the disease as a congenital malformation due to an abnormality of intrauterine development. So surgical treatment was carried out, considering it as the gold standard for this type of malformations. It’s considerable to emphasize that the association between clitoromegaly and fusion of the lower third of the urethra with the vagina has not been previously reported in the literature.