MRI在下丘脑错构瘤分型中的应用价值

目的探讨MRI在下丘脑错构瘤分型中的应用价值。方法收集我院6例下丘脑错构瘤的临床资料和MR影像资料,并结合文献复习。结果将下丘脑错构瘤分为Ⅰ~Ⅳ型,Ⅰ型和Ⅱ型错构瘤均位于下丘脑下方、脚间池内,第三脑室底部无明显受压变形,其中Ⅰ型错构瘤与下丘脑附着面小,Ⅱ型错构瘤与下丘脑附着面宽大,但第三脑室底部变形不明显(无变形或变形10%);Ⅲ型错构瘤骑跨于第三脑室底,第三脑室底部受累变形明显;Ⅳ型错构瘤完全坐落于第三脑室底,位于三脑室内。本组Ⅰ型1例,Ⅱ型1例,Ⅲ型4例,无Ⅳ型。Ⅱ型和Ⅲ型下丘脑错构瘤以痴笑样癫痫发病,经过手术和药物治疗,癫痫明显减少。Ⅰ型下丘脑错构瘤以性早熟相关症状发病,经手术治疗后性激素水平降至儿童期正常水平。结论 MRI扫描可以对下丘脑错构瘤进行准确的分型,并对治疗方案的选择以及手术入路的制定提供依据。

The application value of MRI in hypothalamic hamartoma classification

Objective To discuss the application value of MRI in the hypothalamus hamartoma classification .Methods We collected the clinical data and MR imaging data of hypothalamic hamartoma of 6 patients from our hospital ,and com‐bined the literature to review .Results The hypothalamus hamartoma was diagnosed and divided into four categories （type I~ IV） ,the type I and type II hamartoma were located in the following position of hypothalamus and interpeduncular cis‐tern and there was no obvious compressive deformation at the bottom of the third ventricle ,there into the attachment sur‐face between the type I hamartoma and hypothalamic was small ,the attachment surface between the type II hamartoma and hypothalamic was wide ,but there was no obvious deformation at the bottom of the third ventricle （no deformation or de‐formation 〈10% ） .Type III （straddling type） defined as the hamartoma located partially within the hypothalamus and the third ventricle ,the affected deformation of the third ventricle bottom was obvious ;Type IV hamartoma was completely lo‐cated on the bottom of the third ventricle and located in the third ventricle .In this group ,1 case was in type Ⅰ ,1 case was in type Ⅱ ,4 cases were type III ,no one was in type IV .The patients who were in type II and type Ⅲ hypothalamic hamartoma were the patients with high wheezy epilepsy .After surgery and drug treatment ,epilepsy was significantly re‐duced .Hypothalamic hamartoma symptoms of TYPI patients were related to precocious puberty onset ,after surgical treatment ,the sex hormone level dropped to normal level of the childhood .Conclusion MRI has accurate classification of hypothalamic hamartoma and can provide basis for the choice of treatment and the establishment of surgical approach .