Hemihydranencephaly: living with half brain dysfunction

• Main Authors: Pavone Piero, Nigro Francesco, Falsaperla Raffaele, Greco Filippo, Ruggieri Martino, Rizzo Renata, Praticò Andrea D, Pavone Lorenzo
• Format: Article
• Language: English
• Published: BMC 2013-01-01
• Series: Italian Journal of Pediatrics
• Subjects: Unilateral hydranencephaly; Congenital anomaly; Brain malformation; Carotid artery anomaly
• Online Access: http://www.ijponline.net/content/39/1/3
• ISSN: 1720-8424; 1824-7288

<p>Abstract</p> <p>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.</p> <p>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.</p> <p>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.</p>