Outcome of Cervical Encephaloceles in Children

Outcome after Surgery

Preoperative planning with MRI and improvements in surgical technique have enhanced outcomes in some cases of cervical encephalocele.  However, the morbidity and mortality rates associated with cervical encephaloceles containing a large amount of functional brain tissue, as well as those that occur in infants with Chiari III malformations, remain quite high. 

• Size and content dictate outcome: Prognosis and long-term outcome for patients with cervical encephalocele depend primarily on the size and contents of the encephalocele, including the amount and type of neural tissue found in the sac, as well as associated syndromes, the severity of the associated cerebral anomalies, and the ease of surgical correction.
• Cervical meningocele: When there are no associated cerebral anomalies present, a complete recovery can occur.
• Cervical encephalocele: The more neural tissue that is present in the sac and the more associated cerebral anomalies that are present, the higher the chance of perioperative complications, neurocognitive delay, and poor outcome. This is especially true when there is brainstem herniation into the cervical encephalocele sac, since such herniation often results in respiratory insufficiency, dysphagia with secondary aspiration, dysfunction of the cranial nerves, and spastic or decreased muscle tone.
• Hydrocephalus: The prognosis is also worse if the ventricles extend into the sac or if there is hydrocephalus.