Congenital perineal lipoma presenting as ambiguous genitalia

Congenital perineal lipoma presenting as ambiguous genitalia

Autor Guerra-Junior, G. Google Scholar
Aun, A. M. E. Google Scholar
Miranda, M. L. Google Scholar
Beraldo, L. P. Google Scholar
Moraes, S. Guimaraes Google Scholar
Baptista, M. T. M. Google Scholar
Marques-de-Faria, A. P. Google Scholar
Maciel-Guerra, A. T. Google Scholar
Instituição Universidade Federal de São Paulo (UNIFESP)
Universidade Estadual de Campinas (UNICAMP)
PUC SP
Resumo Background: Congenital perineal lipoma is extremely rare and may lead to a misdiagnosis of ambiguous genitalia.Case Reports: We report on two girls referred to our service for ambiguous genitalia. Patient 1 (17 clays old) and patient 2 (2 months old) had Unremarkable gestational and perinatal histories. Both had normal female external genitalia and a 46,XX karyotype. Patient 1 had a polypoid, protruding 3.0 x 2.0 x 1.5-cm phallic-like mass arising at the inferior border of the left labium majora, and patient 2 had a similar mass of 1.5 x 1.5 x 1.0 cm at the same site and an imperforate anus. in both cases the mass was removed and found to be a lipoma.Discussion: To our knowledge, perineal lipoma has been reported only in eleven girls, nine of them with associated anorectal malformation. Migration and fusion of the labioscrotal folds and formation of the urorectal septum are sirnultaneous developmental events occurring in the same region, which may explain the association of perineal lipoma and anorectal malformations.
Palavra-chave ambiguous genitalia
perineal lipoma
neonates
sexual differentiation
anorectal malformation
Idioma Inglês
Data de publicação 2008-08-01
Publicado em European Journal of Pediatric Surgery. Stuttgart: Georg Thieme Verlag Kg, v. 18, n. 4, p. 269-271, 2008.
ISSN 0939-7248 (Sherpa/Romeo, fator de impacto)
Publicador Georg Thieme Verlag Kg
Extensão 269-271
Fonte http://dx.doi.org/10.1055/s-2008-1038366
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:000258868900014
Endereço permanente http://repositorio.unifesp.br/handle/11600/30831

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