RESUMO
INTRODUCTION: Hypothalamic hamartomas are very rare (1:200,000) and range in size from 17.9 to 18 mm. When their dimensions exceed 30-40 mm, they are classified as giant hypothalamic hamartomas. METHODS: We present a 14-month-old boy with central precocious puberty and gelastic seizures in whom a magnetic resonance imaging scan revealed a giant hypothalamic hamartoma measuring 50 × 50 × 40 mm. RESULTS: In the 11 cases described so far, we found that in comparison to the average-size lesion, giant hypothalamic hamartomas had a lower frequency of precocious puberty, but a similar frequency of seizures. The mean age at diagnosis was younger, and males were more affected than females. Magnetic resonance imaging results were similar with the exception of mass effect. CONCLUSIONS: Giant hypothalamic hamartomas had a higher tendency to adhere to surrounding structures. Their invasiveness and cystic degeneration were frequent findings among the 11 studies. Surgical removal was ineffective in controlling refractory epilepsy and caused postoperative morbidity in all patients.
Assuntos
Epilepsia/cirurgia , Hamartoma/patologia , Doenças Hipotalâmicas/patologia , Puberdade Precoce/cirurgia , Epilepsia/etiologia , Hamartoma/complicações , Hamartoma/diagnóstico por imagem , Hamartoma/cirurgia , Humanos , Doenças Hipotalâmicas/complicações , Doenças Hipotalâmicas/diagnóstico por imagem , Doenças Hipotalâmicas/cirurgia , Lactente , Masculino , Tamanho do Órgão , Puberdade Precoce/etiologia , Radiografia , Resultado do TratamentoAssuntos
Adenoma/diagnóstico por imagem , Hamartoma/diagnóstico por imagem , Hemorragia/diagnóstico por imagem , Doenças Hipotalâmicas/diagnóstico por imagem , Neoplasias Hipofisárias/diagnóstico por imagem , Adenoma/complicações , Adulto , Feminino , Galactorreia/etiologia , Hamartoma/complicações , Hemorragia/complicações , Humanos , Doenças Hipotalâmicas/complicações , Neoplasias Hipofisárias/complicações , Tomografia Computadorizada por Raios X , Adulto JovemAssuntos
Hamartoma/patologia , Doenças Hipotalâmicas/patologia , Síndrome de Pallister-Hall/patologia , Determinação da Idade pelo Esqueleto , Pré-Escolar , Diagnóstico Diferencial , Pé/diagnóstico por imagem , Hamartoma/diagnóstico , Hamartoma/diagnóstico por imagem , Mãos/inervação , Humanos , Doenças Hipotalâmicas/diagnóstico , Doenças Hipotalâmicas/diagnóstico por imagem , Extremidade Inferior/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Síndrome de Pallister-Hall/diagnóstico , Síndrome de Pallister-Hall/diagnóstico por imagem , PrognósticoAssuntos
Humanos , Feminino , Adulto , Adulto Jovem , Neoplasias Hipofisárias/diagnóstico por imagem , Adenoma/diagnóstico por imagem , Hamartoma/diagnóstico por imagem , Hemorragia/diagnóstico por imagem , Doenças Hipotalâmicas/diagnóstico por imagem , Neoplasias Hipofisárias/complicações , Tomografia Computadorizada por Raios X , Adenoma/complicações , Galactorreia/etiologia , Hamartoma/complicações , Hemorragia/complicações , Doenças Hipotalâmicas/complicaçõesRESUMO
OBJECTIVE: The diagnosis of subclinical central hypothyroidism in hypothalamic-pituitary patients cannot be established by serum markers of thyroid hormone action. Myocardial function by echocardiography has been shown to reflect thyroid hormone action in primary thyroid dysfunction. We evaluated the performance of echocardiography in diagnosing subclinical central hypothyroidism. DESIGN: Cross-sectional and before and after. METHODS: Echocardiography and serum thyroid hormones were assessed in overt primary (n=20) and central (n=10) hypothyroidism, subclinical primary hypothyroidism (n=10), hypothalamic-pituitary disease with normal free thyroxine (FT(4); n=25), and controls (n=28). Receiver operating characteristic (ROC) curves were generated using overt hypothyroidism patients and selected cut-off values were applied to detect both primary and central subclinical hypothyroidism. After levothyroxine (l-T(4)) intervention, patients were echocardiographically reevaluated at predefined targets: normal thyrotropin (TSH) in primary hypothyroidism, normal FT(4) in overt central hypothyroidism, and higher than pretreatment FT(4) in echo-defined subclinical central hypothyroidism. RESULTS: Parameters with highest areas under the ROC curves (area under the curve (AUC) ≥0.94) were as follows: isovolumic contraction time (ICT), ICT/ejection time (ET), and myocardial performance index. Highest diagnostic accuracy (93%) was obtained when at least one parameter was increased (positive and negative predictive values: 93%). Hypothyroidism was echocardiographically diagnosed in eight of ten patients with subclinical primary hypothyroidism and in 14 of 25 patients (56%) with hypothalamic-pituitary disease and normal serum FT(4). Echocardiographic abnormalities improved significantly after l-T(4) and correlated (0.05