儿童中枢性性早熟的临床与影像学表现分析

Central Precocious Puberty in Children:the Radiologic Findings and Clinical Analysis

目的分析引起儿童中枢性性早熟的常见病因及影像学表现,从而提高其诊断水平。方法回顾性分析7年内经临床、实验室检查、影像及病理检查确诊的33例中枢性性早熟患儿的临床及影像学资料。结果 33例患儿均行影像学检查,17例(女9例,男8例)发现器质性病变,包括下丘脑错构瘤6例,空蝶鞍3例,生殖细胞瘤3例,恶性畸胎瘤1例,胼胝体脂肪瘤1例,胶质瘤2例(其中1例为NF-Ⅰ型),鞍上池蛛网膜囊肿1例;2例合并脑积水。16例(女15例,男1例)未发现器质性病变,诊断为特发性性早熟。结论儿童中枢性性早熟包括特发性和器质性性早熟,女孩大多数为特发性性早熟,而男孩多为器质性性早熟。因此,对于<4岁的女孩及所有男孩,性早熟发展迅速,有神经系统的症状和体征,实验室检查提示病理性性早熟者均应行头颅影像学检查,脑部MRI是排除器质性性早熟的基本检查方法。

Objective Determining the lesions that cause central precocious puberty and their imaging findings so as to improve the diagnosis of the disease.Methods Review the clinical and radiological findings of 33 patients diagnosed with central precocious puberty clinically according to the imaging,laboratory and pathological examinations in a period of 7 years.Results Out of 33 patients,imaging findings were positive in 17 patients（9 Female cases and 8male cases）.The organic lesions of CPP included hypothalamic hamartoma in 6 cases,empty sella in 3 cases,germinomas in 3cases,malignant teratomas in 1 case,lipoma of the corpus callosum in 1case,glioma 2cases（one with NF-1）,and arachnoid cyst in the suprasellar region in 1 case.Two patients had findings accompanied with hydrocephalous.16 cases（9 Female cases and 8male cases） were negative and idiopathic precocious puberty was considered.Conclusion Central precocious puberty may be idiopathic or organic according to the etiology.CPP in girls was usually idiopathic,whereas the vast majority of cases in boys were due to an intracranial lesion.Thus,CNS imaging was suggested for girls less than 4 years old and all boys,rapid rate of progression or inappropriate sequence of development of secondary sexual characteristics,neurological signs and symptoms,and laboratory results suggestive organic CPP.CNS MRI was the first choice for excluding secondary lesions.