Central precocious puberty associated with pituitary duplication and midline defects.
Central precocious puberty (CPP) is due to premature activation of the hypothalamic-pituitary-gonadal axis. It may be idiopathic or result from congenital or acquired CNS lesions. We describe a 7.4 year-old Brazilian girl with CPP who also presented hypertelorism, limitation of lateral neck rotation and synkinesis of the hands. Sellar and cervical column MRIs revealed pituitary duplication and rudimentary intervertebral disks. We present the clinical and imaging observations of this case, and a thorough literature review of this rare developmental abnormality.
Division of Neuroendocrinology, Department of Medicine, Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil. teresavieira-endo@pesquisa.epm.br
Teresa C
TC
Vieira
Renata N
RN
Chinen
Maria R F
MR
Ribeiro
Roberto Gomes
RG
Nogueira
Julio
J
Abucham
D002648:Child; D005260:Female; D006222:Hamartoma / Q000150:complications / Q000175:diagnosis; D006801:Humans; D007027:Hypothalamic Diseases / Q000150:complications / Q000175:diagnosis; D007030:Hypothalamo-Hypophyseal System / Q000378:metabolism; D007403:Intervertebral Disc / Q000002:abnormalities*; D008279:Magnetic Resonance Imaging; D010902:Pituitary Gland / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000378:metabolism; D010913:Pituitary-Adrenal System / Q000378:metabolism; D011629:Puberty, Precocious / Q000175:diagnosis* / Q000209:etiology*; D011859:Radiography