Hypothalamic hamartomas are aberrant masses, composed of abnormally distributed neurons and glia. Along endocrine and cognitive symptoms, they may cause epileptic seizures, including the specific gelastic and dacrystic seizures. Surgery is the treatment of drug-resistant hamartoma epilepsy, with associated positive results on endocrine, psychiatric, and cognitive symptoms. Recently, alternatives to open microsurgical treatment have been proposed. We review these techniques and compare their efficacy and safety. Open resection or disconnection of the hamartoma, either through pterional, transcallosal, or transventricular approach, leads to good epileptological control, but its high complication rate, up to 30%, limits its indications. The purely cisternal peduncular forms remain the only indication of open, pterional approach, while other strategies have been developed to overcome the neurological, endocrine, behavioral, or cognitive complications. Laser and radiofrequency thermocoagulation-based disconnection through robot-guided stereo-endoscopy has been proposed as an alternative to open microsurgical resection and stereotactic destruction. The goal is to allow safe and complete disconnection of a possibly complex attachment zone, through a single intraparenchymal trajectory which allows multiple laser or radiofrequency probe trajectory inside the ventricle. The efficacy was high, with 78% of favorable outcome, and the overall complication rate was 8%. It was especially effective in patients with isolated gelastic seizures and pure intraventricular hamartomas. Stereotactic radiosurgery has proved as efficacious and safer than open microsurgery, with around 60% of seizure control and a very low complication rate. Multiple stereotactic thermocoagulation showed very interesting results with 71% of seizure freedom and 2% of permanent complications. Stereotactic laser interstitial thermotherapy (LiTT) seems as effective as open microsurgery (from 76 to 81% of seizure freedom) but causes up to 20% of permanent complications. This technique has however been highly improved by targeting only the epileptogenic onset zone in the hamartoma, as shown on preoperative functional MRI, leading to an improvement of epilepsy control by 45% (92% of seizure freedom) with no postoperative morbidity. All these results suggest that the impact of the surgical procedure does not depend on purely technical matters (laser vs radiofrequency thermocoagulation or stereotactic vs robot-guided stereo-endoscopy) but relies on the understanding of the epileptic network, including inside the hamartoma, the aim being to plan an effective disconnection or lesion of the epileptogenic part while sparing the adjacent functional structures.

中文翻译：

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下丘脑错构瘤的外科治疗。

下丘脑错构瘤是异常肿块，由异常分布的神经元和神经胶质组成。沿着内分泌和认知症状，它们可能引起癫痫发作，包括特定的神经胶质和乳突性癫痫发作。手术是对耐药性错构瘤癫痫的治疗，在内分泌，精神病和认知症状方面均具有相关的积极结果。最近，已经提出了开放式显微外科治疗的替代方案。我们回顾了这些技术，并比较了它们的功效和安全性。开放式切除或错开错构瘤，无论是通过翼状，肉，经call门或脑室入路均可达到良好的癫痫控制，但其高并发症率（高达30％）限制了其适应症。单纯的脑池根状蒂形式仍然是开放，翼状ter入方法的唯一标志，同时开发了其他策略来克服神经，内分泌，行为或认知方面的并发症。已经提出通过机器人引导的立体内窥镜进行基于激光和射频热凝的分离术，作为开放式显微外科手术切除和立体定向破坏的替代方法。目的是通过单个实质内轨迹允许安全和完全断开可能复杂的附着区，该轨迹允许心室内的多个激光或射频探头轨迹。疗效高，有78％的良好结果，总并发症发生率为8％。它对孤立的全突性癫痫发作和单纯脑室内错构瘤的患者特别有效。立体定向放射外科手术已被证明比开放式显微外科手术更有效，更安全，癫痫发作控制率约为60％，并发症发生率很低。多次立体定向热凝显示出非常有趣的结果，癫痫发作自由度为71％，永久并发症为2％。立体定向激光间质热疗（LiTT）似乎与开放式显微外科手术一样有效（癫痫发作自由度从76％到81％），但可引起高达20％的永久性并发症。但是，如术前功能MRI所示，仅针对错构瘤中的致癫痫发作区，已对该技术进行了高度改进，从而使癫痫控制提高了45％（癫痫发作自由度为92％），且无术后并发症。