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Managing an open nasofrontal encephalocele after birth
Nicolas Apostolou1, Daniel Gräfe2, Matthias Knüpfer3
1Department of Pediatric Neurosurgery, University Hospital Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany. Nicolas.Apostolou@medizin.uni-leipzig.de.
Insights
This study reports a rare case of open nasofrontal encephalocele in a neonate. Early surgical repair on the first day of life did not impact the infant's neurological outcome.
Area of Science:
- Neuroscience
- Pediatric Surgery
- Medical Genetics
Background:
- Encephaloceles are uncommon neural tube defects, with occipital encephalocele being most frequent.
- Open frontal encephaloceles are rare, often diagnosed prenatally, leading to termination.
- These cases present significant surgical and anesthetic challenges due to infant comorbidities.
Observation:
- A rare case of an open nasofrontal encephalocele is presented.
- The neonate had microcephaly, abnormal intracranial anatomy, and low birth weight.
- Incomplete cutaneous coverage posed an immediate risk of meningitis.
Findings:
- The neonate underwent surgical repair within the first day of life.
- The surgical intervention did not adversely affect the neurological outcome.
- This case highlights successful management of a rare congenital anomaly.
Implications:
- Early surgical intervention is crucial for neonates with open encephaloceles to prevent life-threatening complications like meningitis.
- Management requires a multidisciplinary approach involving neurosurgeons and anesthesiologists.
- Further research can explore long-term outcomes and optimal surgical timing for such rare conditions.
Abstract:
Encephaloceles are relatively uncommon in western countries. Most of the reported cases involve occipital encephaloceles. Open frontal encephaloceles comprise a rare entity. Most of them will be detected during early prenatal diagnostic, whereas the majority of the pregnancies will be terminated after the consent of the parents. Open frontal encephaloceles pose a great challenge to neurosurgeons as well as anesthesiologists, as these infants present with a microcephaly, non-physiological intracranial anatomy, and low birth weight, thus making the infant prone to excessive blood loss, hypothermia, and death. Neonates born with an incomplete cutaneous coverage are exposed to an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. We represent a rare case of an open nasofrontal encephalocele managed surgically in the first day of life. Surgery did not influence the neurological outcome of the patient.
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