Lega Bradley, Mullin Jeffrey, Wyllie Elaine, Bingaman William
Epilepsy Center, Neurological Institute, Cleveland Clinic, 9500 Euclid Ave, Cleveland, OH, 44195, USA.
Childs Nerv Syst. 2014 Nov;30(11):1831-7. doi: 10.1007/s00381-014-2483-7. Epub 2014 Oct 9.
The term "hemispheric malformation of cortical development" (MCD) has come into the medical lexicon in the past 20 years as improvements and availability of advanced imaging techniques have permitted more precise diagnosis of a variety of brain developmental disorders that affect large regions of brain. These conditions are united by their propensity to elicit seizures that are difficult to control with medication in the children who suffer them.
The goal of surgical intervention is always to achieve seizure freedom and thereby give the affected child the best possible hope for neurological development. Even when seizure freedom cannot be achieved, a reduction in seizure burden is necessary to permit the survival of the child in many cases of MCD.
A presurgical evaluation of a patient presenting with severe epilepsy and a possible hemispheric malformation can be divided into three stages. The first includes an evaluation of available imaging, clinical, and genetic data to accurately diagnose the child and help determine if surgical intervention is an option. The next includes an evaluation of EEG and neurological data, although this has limited utility in many clinical circumstances. Finally, a clinical team must decide upon an appropriate surgical strategy among a variety of options.
In this review, we will examine the set of diagnoses and associated imaging characteristics that describe the set of conditions for which surgical intervention is a possibility. We include a discussion of available surgical options, describing our own experience with surgery for MCD and the associated postoperative considerations including rates of seizure freedom, considerations for reoperation, and hydrocephalus.
在过去20年里，“大脑皮质发育半球畸形”（MCD）一词进入了医学词汇表，这是因为先进成像技术的改进和普及使得对影响大脑大片区域的各种脑发育障碍能够进行更精确的诊断。这些病症的共同特点是，患病儿童容易引发药物难以控制的癫痫发作。
手术干预的目标始终是实现无癫痫发作，从而为受影响的儿童提供神经发育的最大希望。即使无法实现无癫痫发作，在许多MCD病例中，减轻癫痫负担对于儿童的生存也是必要的。
对患有严重癫痫且可能存在半球畸形的患者进行术前评估可分为三个阶段。第一阶段包括评估现有的影像学、临床和基因数据，以准确诊断患儿并帮助确定手术干预是否可行。第二阶段包括评估脑电图和神经学数据，不过在许多临床情况下其作用有限。最后，临床团队必须在多种选择中决定合适的手术策略。
在本综述中，我们将研究一系列诊断方法及相关影像学特征，这些特征描述了可能进行手术干预的病症。我们将讨论现有的手术选择，介绍我们自己对MCD手术的经验以及术后相关注意事项，包括无癫痫发作率、再次手术的考虑因素和脑积水问题。
