Hypothalamic Hamartoma in An Unusual Case with Delayed Puberty
Hypothalamic hamartoma (HH) is a rare intracranial lesion that usually presents with classic triad of central precocious puberty, gelastic epilepsy, and developmental delay. Herein, a 14-year old boy is presented in whom the diagnosis of HH was made by magnetic resonance imaging. While he did not have any complain of precocious puberty, he surprisingly suffered from delay in puberty. The definite diagnosis of HH can only be made by appropriate imaging, in a case with atypical feature of delay in puberty and in the absence of gelastic epilepsy. To our best knowledge, this is the first case of HH who is presented with delay in puberty as of first manifestation.
Maixner W. Hypothalamic hamartomas--clinical, neuropathological and surgical aspects. Childs Nerv Syst 2006;22(8):867-73.
Arita K, Kurisu K, Kiura Y, Iida K, Otsubo H. Hypothalamic hamartoma. Neurol Med Chir (Tokyo) 2005;45(5):221-31.
Nguyen D, Singh S, Zaatreh M, Novotny E, Levy S, Testa F, Spencer SS. Hypothalamic hamartomas: seven cases and review of the literature. Epilepsy Behav 2003;4(3):246-58.
Luo S, Li C, Ma Z, Zhang Y, Jia G, Cheng Y. Microsurgical treatment for hypothalamic hamartoma in children with precocious puberty. Surg Neurol 2002;57(5):356-62.
Rousso IH, Kourti MR, Papandreou DR, Tragiannidis AR, Athanasiadou F. Central precocious puberty due to hypothalamic hamartoma in a 7-month-old infant girl. Eur J Paediatr 2007;167(5):538-85.
Arita K, Ikawa F, Kurisu K, Sumida M, Harada K, Uozumi T, Monden S, Yoshida J, Nishi Y. The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. J Neurosurg 1999;91(2):212-20.
Winter WE, Rosenbloom AL, Maclaren NK, Mickle JP. Solitary central maxillary incisor associated with precocious puberty and hypothalamic hamartoma. J Pediatr 1982;101(6):965-67.
Pinyerd B, Zipf WB. Puberty-timing is everything. J Pediatr Nurs 2005;20(2):75-82.
Rogol AD. Androgens and puberty. Mol Cell Endocrinol 2002;198(1-2):25-9.
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