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Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale
Published on: August 25, 2014
Can prognostic indicators be identified in a fetus with an encephalocele?
C M Bannister1, S A Russell, S Rimmer
1Fetal Management Unit, St. Mary's Hospital, Manchester, UK.
Insights
Prenatal ultrasound can identify fetuses with encephaloceles that have a favorable outcome. Isolated encephaloceles with cystic lesions or nubbins indicate a better prognosis for affected newborns.
Area of Science:
- Neuroscience
- Prenatal Diagnosis
- Medical Genetics
Background:
- Encephaloceles are congenital brain malformations diagnosable in utero.
- These can be complicated (with chromosomal or other organ abnormalities) or isolated.
- Prognosis varies significantly, impacting parental decisions regarding pregnancy management.
Purpose of the Study:
- To determine if prenatal assessment can reliably predict the outcome of fetal encephaloceles.
- To identify factors associated with favorable versus poor prognoses in affected fetuses.
Main Methods:
- Retrospective review of medical records and ultrasound scans.
- Analysis of 31 fetuses with encephaloceles referred to a Fetal Management Unit (1991-1997).
- Classification of encephaloceles as complicated or isolated based on associated abnormalities.
Main Results:
- Eighteen cases were complicated encephaloceles, with high rates of termination, intrauterine, or neonatal death.
- Among 13 isolated encephaloceles, those with neural tissue in the sac had poor outcomes (termination, intrauterine death).
- Isolated encephaloceles with cystic lesions or nubbins showed favorable outcomes, with survivors having few or no abnormalities.
Conclusions:
- Prenatal ultrasound can identify fetuses with encephaloceles who have a favorable prognosis.
- The presence of neural tissue within the encephalocele sac is a key indicator of a poor outcome.
- This capability aids in informed decision-making for parents facing a fetal encephalocele diagnosis.
Abstract:
Encephaloceles, like other congenital malformations of the brain diagnosable in utero, can be either complicated (there being an associated chromosomal abnormality, abnormalities in the remainder of the central nervous system (CNS) and/or other organs), or isolated (no abnormalities in the chromosomes, the remainder of the CNS or other organs). Complicated cases invariably have a poor prognosis but amongst those with isolated lesions the outcome is variable with some affected children having poor mental and physical development but others who are only mildly or moderately disabled. To be able to make an informed decision about how to manage their pregnancy parents need to know what the prognosis is likely to be for their fetus with an encephalocele. To see if the necessary information could be reliably gathered by prenatal assessment of affected fetuses, a review was carried out of the medical records and ultrasound scans of 31 fetuses with encephaloceles referred to the Fetal Management Unit at St. Mary's Hospital in Manchester between January 1991 and December 1997. Eighteen of the cohort were classified as having a complicated encephalocele. Thirteen of the pregnancies were terminated; there were three intrauterine deaths, and one neonatal death. There is only one surviving child who is severely disabled. Thirteen fetuses were classified as having isolated encephaloceles, six had a mass of neural tissue in the encephalocele sac and were terminated, one died in utero and six had a cystic lesion or only a nubbin and have survived with few or no abnormalities. This study has shown that it is possible to identify fetuses with an encephalocele with a favourable outcome.
