Hydranencephaly: cerebral spinal fluid instead of cerebral mantles

Italian Journal of Pediatrics

Raffaele Falsaperla²  Giuseppe Pero³  Lorenzo Pavone²  Enrico Parano⁴  Renata Rizzo⁵  Martino Ruggieri¹  Giovanna Vitaliti²  Andrea D Praticò²  Piero Pavone² 

[1] Department of Formative Processes, University of Catania, Catania, Italy;Unit of Pediatrics and Pediatric Emergency, University Hospital ¿Policlinico-Vittorio Emanuele¿, Catania, Italy;Neuroradiologic Unit, Department of Radiology, University of Catania, Catania, Italy;Institute of Neurological Sciences, National Research Council, Catania, Italy;Chair of Child Neuropsychiatry, University of Catania, Catania, Italy

关键词: Severe hydrocephalus;    Brain malformation;    Congenital anomaly;    Holoprosencephaly;    Hydranencephaly;   

Others  :  1146363
DOI  :  10.1186/s13052-014-0079-1

 received in 2014-04-08, accepted in 2014-09-17,  发布年份 2014

【 摘 要 】

The authors report a wide and updated revision of hydranencephaly, including a literature review, and present the case of a patient affected by this condition, still alive at 36 months.

Hydranencephaly is an isolated and with a severe prognosis abnormality, affecting the cerebral mantle. In this condition, the cerebral hemispheres are completely or almost completely absent and are replaced by a membranous sac filled with cerebrospinal fluid. Midbrain is usually not involved. Hydranencephaly is a relatively rare cerebral disorder. Differential diagnosis is mainly relevant when considering severe hydrocephalus, poroencephalic cyst and alobar holoprosencephaly. Ethical questions related to the correct criteria for the surgical treatment are also discussed.

【 授权许可】

   
2014 Pavone et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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