title: Prolonged survival with hydranencephaly: report of two patients and literature review.
abstract: Infants with hydranencephaly are presumed to have a reduced life expectancy, with a survival of several weeks to months. Rarely, patients with prolonged survival have been reported, but these infants may have had other neurologic conditions that mimicked hydranencephaly, such as massive hydrocephalus or holoprosencephaly. We report two infants with prenatally acquired hydranencephaly who survived for 66 and 24 months. We reviewed published reports to ascertain the clinical and laboratory features associated with survival of more than 6 months. This review demonstrates that prolonged survival up to 19 years can occur with hydranencephaly, even without rostral brain regions, with isoelectric electroencephalograms, and with absent-evoked potentials. Finally, the ethical aspects of these findings, as they relate to anencephaly and organ transplantation, are discussed.
authors.affiliation: Department of Pediatrics (Neurology), University of Medicine and Dentistry of New Jersey School of and Osteopathic Medicine, Stratford, New Jersey, USA.
authors.forename: G N
authors.initials: GN
authors.lastname: McAbee
authors.forename: A
authors.initials: A
authors.lastname: Chan
authors.forename: E L
authors.initials: EL
authors.lastname: Erde
mesh_terms: D001921:Brain / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001926:Brain Death / Q000331:legislation & jurisprudence* / Q000503:physiopathology; D002675:Child, Preschool; D004569:Electroencephalography; D004992:Ethics, Medical; D006801:Humans; D006832:Hydranencephaly / Q000000981:diagnostic imaging / Q000401:mortality*; D008017:Life Expectancy; D008297:Male; D011379:Prognosis; D020858:Tissue and Organ Harvesting / Q000331:legislation & jurisprudence; D014057:Tomography, X-Ray Computed; D014481:United States; D017287:Ventriculoperitoneal Shunt