Patterns of evolution
- Skin-covered cystic sac: Neonates present with a skin-covered cystic sac protruding from the back of the neck resulting from herniation of neural tissue through a fusion defect in the posterior cervical spine.
- Small encephalocele frequently asymptomatic: Infants with small encephaloceles with little or no herniating brain tissue may present with adequate breathing and no symptoms.
- Large encephalocele with extensive deficits: When there is herniation of the brainstem into the cervical encephalocele sac, affected neonates can present with respiratory insufficiency and dysphagia with secondary aspiration.
- Seizures: Seizures can also occur.
Time for evolution
- Symptom evolution: Depends primarily on the size and contents of the encephalocele as well as associated syndromes, cerebral anomalies, and ease of surgical correction.
Intervention at Presentation
- Admit to ICU: The infant should be admitted to the neonatal intensive care unit, kept NPO, started on IVFs D10 at a rate of 100 ml/kg/day, and kept either prone or on the side to reduce mechanical trauma to the encephalocele.
- Hypothermia: Heat loss should be prevented through judicious use of heating.
- Hypoglycemia: Hypoglycemia should be avoided through monitoring of serum glucose and treating as indicated.
- Protect the encephalocele: The encephalocele should be examined for skin breakdown and CSF leak.
- Associated cerebral anomalies: Patients with extensive neural tissue in the sac and associated cerebral anomalies must be evaluated for respiratory insufficiency and dysphagia with secondary aspiration and managed with mechanical ventilatory support and/or a feeding tube as indicated.
Preparation for definitive intervention, nonemergent
- Operation usually not emergent: The majority of cervical encephaloceles are covered with skin. Therefore, there is no emergent need to take the child to surgery within 24–48 hours of birth.
- Routine preparation: The preparation of the neonate with a cervical encephalocele for surgery is usually not difficult. Most have a high hematocrit as well as adequate intravascular volume, making fluid resuscitation unnecessary.
Preparation for definitive intervention, emergent
- CSF leak: If the sac is ruptured and there is concomitant CSF leakage, repair should occur shortly after birth to reduce the risk of infection and intracranial hypotension. The infant may be started on intravenous antibiotics (e.g., ampicillin and gentamicin, with dose based on weight) at the discretion of the neurosurgeon.
- Skin necrosis and ulceration: If the encephalocele sac is large, care must be taken to prevent torsion of the sac and concomitant arterial or venous compromise (25). The skin must be monitored for necrosis and/or ulceration, which, if found, necessitates earlier repair to prevent meningitis/sepsis.
- Intraoperative hemorrhage risk: Given the risk of bleeding at the time of surgery, two large-bore intravenous lines should be inserted and an arterial line should be placed to monitor blood pressure and to follow blood chemistries during the procedure. At least two units of packed red blood cells should be available at the beginning of the operation.
- Activity: The infant must be on the side or prone, with no pressure on encephalocele sac until cleared by a neurosurgery consult.
- Vital signs: Temperature, heart rate, respiratory rate, and blood pressure should be monitored every hour for 24 hours and then every 4 hours. Head circumference should be determined every 24 hours. Length should be obtained on admission and once a week thereafter. Weight should be obtained on admission and then each day.
- Call orders: The neurosurgeon should be called if CSF drainage from the encephalocele is observed.
- Lines/IV care: No scalp IVs
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