Total urogenital mobilization by CAH: A step-by-step illustration of the technique

• Autor(a) principal: Macedo, Antonio [UNIFESP]
• Data de Publicação: 2015
• Outros Autores: Cruz, Marcela Leal da [UNIFESP], Liguori, Riberto [UNIFESP], Trivelato, Rodrigo [UNIFESP], Ottoni, Sergio Leite [UNIFESP], Gomes Oliveira, Diego Estevam [UNIFESP], Souza, Georgia Rubiane [UNIFESP], Ortiz, Valdemar [UNIFESP]
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UNIFESP
• Texto Completo: http://dx.doi.org/10.1016/j.jpurol.2014.08.014; http://repositorio.unifesp.br/handle/11600/38649
• topic: Congenital adrenal hyperplasia; Pediatric; Genitalia

IntroductionCongenital adrenal hyperplasia (CAH) resulting from deficient 21-hydroxylase activity is an autosomal recessive disorder with an incidence of 1: 5000-25,000 in Caucasian populations. Despite various techniques to treat CAH, total urogenital mobilization (TUM) has gained popularity. This technique has low morbidity, and can be performed by the perineal route with the patient in the dorsal lithotomy position without the need to separate the urethra from the vagina. We aim to demonstrate in this video the TUM technique step by step.MethodsA 9-month-old child, born with ambiguous genitalia (Prader 3) with a 46XX karyotype and CAH diagnosis presented to our facility. Endoscopy showed a 2-cm common channel.Results and discussionThe patient was treated as described in the video and she had an uneventful clinical outcome with complete healing. TUM is an excellent alternative for treating CAH.