Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliera Universitaria Vittorio-Emanuele-Policlinico, Via Plebiscito 767, 95123, Catania, Italy.
Ital J Pediatr. 2013 Jan 16;39:3. doi: 10.1186/1824-7288-39-3.
Hemi-hydranencephaly is a very rare condition characterized by complete or almost near-complete unilateral absence of the cortical cortex, which is filled by a sac of cerebrospinal fluid. Prenatal vascular disruption with occlusion of the carotid artery territories ipsilateral to the damaged brain is the presumed pathogenesis.We have selected nine cases that fit the clinical and pathologic characteristics of hemi-hydranencephaly, demonstrating that destruction of one hemisphere may be not always associated with severe neurologic impairment and may allow an almost normal life. This disorder is an example of a possible prenatal re-organization in which the right and left cerebral hemispheres present functional potentiality to make up the damaged brain.The cases reported in the literature are discussed, including a patient previously reported and followed-up for 10 years. A review of the cases is performed with an evaluation of the most important aspect of this rare and mysterious disorder.
半脑无脑畸形是一种非常罕见的疾病，其特征是大脑皮质的单侧完全或几乎近乎完全缺失，由脑脊液囊填充。产前血管破裂，损伤侧大脑的颈动脉区域闭塞，被认为是发病机制。我们选择了 9 例符合半脑无脑畸形的临床和病理特征的病例，证明一侧半球的破坏并不总是与严重的神经损伤相关，并且可能允许几乎正常的生活。这种疾病是产前重新组织的一个例子，其中左右大脑半球具有功能潜力来弥补受损的大脑。本文讨论了文献中报道的病例，包括一名曾报道并随访 10 年的患者。对这些病例进行了回顾，并对这种罕见而神秘疾病的最重要方面进行了评估。
