Lancon J A, Haines D E, Raila F A, Parent A D, Vedanarayanan V V
Department of Neurosurgery, The University of Mississippi Medical Center, School of Medicine, Jackson 39216-4505, USA.
J Neurosurg. 1996 Dec;85(6):1127-34. doi: 10.3171/jns.1996.85.6.1127.
Expanding cysts of the septum pellucidum, although rare, may be a cause of significant neurological dysfunction. Most become symptomatic as a result of obstruction of the interventricular foramina and produce headaches, papilledema, emesis, and loss of consciousness. Behavioral, autonomic, and sensorimotor symptoms occur when an expanding cyst impinges on the structures of the hypothalamoseptal triangle or impairs the deep cerebral venous drainage. Neuroophthalmological symptoms may develop as a consequence of hydrocephalus or direct compression of visual structures. The authors describe the case of a young boy with an expanding septum pellucidum cyst who presented with a sudden, severe headache and loss of consciousness. In addition, he had a history of hyperactivity and progressively declining school performance. All symptoms resolved following decompression of the cyst. Seventeen cases from the literature are reviewed. The pathophysiological mechanisms underlying the development of symptoms secondary to expanding septum pellucidum cysts are outlined, and the related clinical neuroanatomy is described. A model is proposed for the natural history of expanding septum pellucidum cysts that provides a rational basis for understanding their clinical behavior and response to intervention. In most cases, fenestration or shunting will relieve the obstructive hydrocephalus and mass effect caused by the cyst and will produce rapid symptomatic improvement.
透明隔囊肿扩大虽然罕见,但可能是严重神经功能障碍的一个原因。大多数囊肿因室间孔阻塞而出现症状,导致头痛、视乳头水肿、呕吐和意识丧失。当扩大的囊肿压迫下丘脑 - 透明隔三角结构或损害大脑深部静脉引流时,会出现行为、自主神经和感觉运动症状。神经眼科症状可能是脑积水或视觉结构直接受压的结果。作者描述了一名患有透明隔囊肿扩大的小男孩的病例,他突然出现严重头痛和意识丧失。此外,他有多动病史,学业成绩逐渐下降。囊肿减压后所有症状均得到缓解。回顾了文献中的17例病例。概述了透明隔囊肿扩大继发症状发展的病理生理机制,并描述了相关的临床神经解剖学。提出了一个透明隔囊肿扩大自然史的模型,为理解其临床行为和对干预的反应提供了合理依据。在大多数情况下,开窗术或分流术将缓解囊肿引起的梗阻性脑积水和占位效应,并将迅速改善症状。