[A case of female pseudohermaphroditism caused by maternal androluteoma].

Abstract

Female pseudohermaphroditism is a condition characterized by various degree of external genitalia virilization in a patient with female internal genitalia and karyotype (XX). External genitalia is masculinized congenitally when female fetus is exposed to excess androgenic environment. Fetal metabolic abnormalities, like congenital adrenal hyperplasia, are the most common causes of female pseudohermaphroditism, however there is a low incidence of gestational hyperandrogenism caused by maternal pathology. We report a case of female pseudohermaphroditism secondary to a maternal ovarian luteoma of pregnancy producing androgenic hormones. The newborn presented a severe degree of external genitalia virilization with high urogenital sinus (stage Prader V). Moreover we describe the main steps of diagnostic iter that are necessary both to exclude other causes of virilization and to study all anatomical aspects in view of the surgical correction. The operation consists in two phases of action: an early clitorisvulvoplasty according to Passerini-Glazel and a late vaginal pull-through with anterior saggital transanorectal approach (ASTRA).

• Authors: C Gallo, M Pancaldi, G Gargano, P L Ceccarelli, D Biondini, S Cattani, C Rota, V De Caris, S Bernasconi, L Iughetti, F Ferrari, G B Cavazzuti
• Journal: Acta bio-medica de L'Ateneo parmense : organo della Societa di medicina e scienze naturali di Parma (Acta Biomed Ateneo Parmense) Vol. 71 Suppl 1 Pg. 765-7 ( 2000) Italy
• Vernacular Title: Caso di pseudoermafroditismo femminile da androluteoma materno.
• PMID: 11424843 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Disorders of Sex Development (etiology)
• Female
• Humans
• Infant, Newborn
• Luteoma
• Ovarian Neoplasms
• Pregnancy
• Pregnancy Complications, Neoplastic