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Updated: Jul 21, 2026
Generation and Long-term Maintenance of Nerve-free Hydra
Published on: July 7, 2017
Prolonged survival with hydranencephaly: report of two patients and literature review
1Department of Pediatrics (Neurology), University of Medicine and Dentistry of New Jersey School of and Osteopathic Medicine, Stratford, New Jersey, USA.
Insights
Infants with hydranencephaly can survive longer than expected, challenging previous assumptions about prognosis. This study highlights cases of prolonged survival, offering new insights into the condition.
Area of Science:
- Neurology
- Pediatrics
- Developmental Biology
Background:
- Hydranencephaly, a severe congenital brain malformation, is typically associated with a poor prognosis and limited life expectancy.
- Previous reports suggest prolonged survival in hydranencephaly patients may be misdiagnosed cases of other neurological conditions.
Observation:
- Two infants with prenatally acquired hydranencephaly demonstrated prolonged survival, living for 66 and 24 months.
- A review of published literature identified clinical and laboratory features associated with survival exceeding 6 months.
Findings:
- Prolonged survival, up to 19 years, is possible in hydranencephaly patients.
- Survival can occur even in the absence of rostral brain regions, with isoelectric electroencephalograms (EEGs), and absent evoked potentials.
Implications:
- These findings challenge the presumed reduced life expectancy for infants with hydranencephaly.
- The data necessitates a re-evaluation of prognostic assumptions and clinical management strategies.
- Ethical considerations regarding anencephaly and organ transplantation are discussed in light of these survival data.
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.
