Virilizing diseases and tumours of childhood. Pediatric surgical aspects.
In the treatment of AGS, the pediatric surgeon is faced with the task of correcting the form and appearance of the virilized female genitalia as early as possible. For severe forms, a pull-through operation of the vagina and closure of the urethral fistula is necessary. At the same time, the hypertrophied clitoris should be corrected by a reduction plasty or backward displacement of the crura. The prospects for cure after treatment of adrenal cortical or ovarian tumours are usually poor. The tumours are malignant in over 85% of cases and metastasize early. The secondary sexual characteristics produced by the tumours regress only partially. When bony development is advanced, these patients inevitably remain small after growth ceases. Cortisone substitution under the guidance of the pediatric endocrinologist is essential at all surgical procedures.
D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000601:surgery; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D002648:Child; D002987:Clitoris / Q000601:surgery; D005260:Female; D005836:Genitalia, Female / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000601:surgery*; D010051:Ovarian Neoplasms / Q000150:complications / Q000601:surgery; D014770:Virilism / Q000209:etiology / Q000601:surgery*