Encephalocele and myelomeningocele are congenital defects in the cranium and spine with herniation of contents into an extracranial and extraspinal sac, respectively. The occurrence of encephalocele and myelomeningocele in the same patient has rarely been described in the literature. The anesthetic management of such cases is associated with multiple challenges, which include difficulty in securing the airway, prone positioning, blood loss, electrolyte imbalance, hypothermia, cardiorespiratory disturbances, and perioperative care. The main aims are, to prevent hemodynamic fluctuations and excessive pressure on the sac to avoid premature rupture and manage a possible difficult airway due to the head and neck mass. We report such a rare case to highlight and share our experiences faced during perioperative management of a giant vascular occipital encephalocele with impending rupture and thoracic myelomeningocele requiring surgical excision and repair. Previous similar case reports were also reviewed, and potential perioperative complications were discussed.
Keywords: difficult airway; encephalocele; encephalocele with myelomeningocele; multiple neural tube defect; myelomeningocele; neonates; paediatric anesthesia; paediatric neurosurgery.
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