Encephalocele (Cerebral Hernia)

Protrusion of brain tissue or meninges through a congenital opening in the skull.

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Skull Base and Pituitary Surgery

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1Overview

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What is this Condition?

Symptoms

Soft, congenital mass-like swelling in the occipital, vertex, or forehead region
Frontonasal type with increased intercanthal distance, facial shape abnormality
Basilar type with unilateral nasal obstruction or clear fluid drainage from nose
Head circumference enlargement due to cerebrospinal fluid accumulation (hydrocephalus), anterior fontanelle tension
Feeding difficulty, restlessness, vomiting
Developmental delay or seizures in some infants

Diagnosis Methods

Diagnosis typically begins with ultrasound during pregnancy; MRI can be performed prenatally for detailed assessment. After birth, the visible sac is evaluated by physical examination. Contrast-enhanced brain MRI is the gold standard for definitive evaluation; CT can be used to clearly demonstrate the bony defect, and special MRI studies can be performed to assess vascular structures.

Treatment Methods

Treatment is surgical. The goal is to appropriately assess the protruding tissue, close the defect in the skull, repair the meninges, and correct the appearance. The timing of surgery is planned according to the infant's condition, location, and size of the sac. If cerebrospinal fluid accumulation (hydrocephalus) is present, an additional shunt procedure may be required.

Surgical repair: closure of the defect and repair of meninges
Cosmetic repair to correct appearance in frontonasal type
Shunt surgery for associated fluid accumulation (hydrocephalus)
Postoperative pediatric neurology and developmental follow-up

References

  1. Greenberg MS. Handbook of Neurosurgery. 10th ed. Thieme; 2023.
  2. Winn HR, ed. Youmans and Winn Neurological Surgery. 6th ed. Elsevier; 2011.
  3. Di Rocco C, Pang D, Rutka JT, eds. Textbook of Pediatric Neurosurgery. Springer; 2020.

This content is for informational purposes and based on academic sources; consult your physician for diagnosis and treatment.

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Encephalocele (Cerebral Hernia) (ICD-10: Q01) | Dr. Özgür AKŞAN