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Open Access Review

Hemihydranencephaly: living with half brain dysfunction

Piero Pavone1*, Francesco Nigro2, Raffaele Falsaperla1, Filippo Greco3, Martino Ruggieri4, Renata Rizzo5, Andrea D Praticò3 and Lorenzo Pavone1

Author Affiliations

1 Unit of Pediatrics and Pediatric Emergency, Azienda Ospedaliera Universitaria Vittorio-Emanuele-Policlinico, Via Plebiscito 767, 95123, Catania, Italy

2 Unit of Neonatology, Hospital Garibaldi, Catania, Italy

3 Department of Pediatrics, University of Catania, Catania, Italy

4 Department of Formative Processes, University of Catania, Catania, Italy

5 Chair of Child Neurospychiatry, University of Catania, Catania, Italy

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Italian Journal of Pediatrics 2013, 39:3  doi:10.1186/1824-7288-39-3

Published: 16 January 2013

Abstract

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.

Keywords:
Unilateral hydranencephaly; Congenital anomaly; Brain malformation; Carotid artery anomaly