Indications
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The presence of an encephalocele is an indication for surgical repair. Encephaloceles are typically diagnosed at birth, although many are now identified in utero by ultrasound.
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Indications for the immediate repair of an encephalocele include open exposure to any meninges or brain, a ruptured encephalocele sac, and leakage of cerebrospinal fluid. Expeditious repair of the encephalocele in these circumstances minimizes the risk of a central nervous system infection.
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If the skin overlying the encephalocele is intact, surgical repair can be done on an elective basis.
Contraindications
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There are few contraindications for repair of a congenital encephalocele because lethal meningitis eventually occurs if the encephalocele is left untreated. Surgical treatment is recommended unless the amount of herniated brain tissue exceeds the remaining intracranial brain tissue.
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Severe encephaloceles with other coexisting systemic or central nervous system abnormalities may be considered incompatible with long-term survival. Corrective or palliative surgery in these patients may have limited benefit.
Planning and positioning
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Of children with encephaloceles, 20% may have other congenital neurologic deformities that can affect the success of surgical treatment and long-term prognosis. Many of these children also have abnormalities of other organ systems that can affect treatment decisions. A complete multisystemic assessment of the patient is critical before surgical planning.
Figure 48-1:
Preoperative work-up should include magnetic resonance imaging (MRI) and magnetic resonance venography to determine the exact nature of the encephalocele and its contents. The extent of neural tissue herniation is the most important prognostic indicator and should be carefully assessed. The location of relevant vascular structures should be studied, especially venous sinus drainage.
Figure 48-2:
Positioning and surgical treatment of encephaloceles vary depending on the location of the lesion. In North America, approximately 80% of encephaloceles are found in the occipital region. These patients should be positioned prone on a horseshoe headrest after induction of anesthesia.
Procedure
