Giant occipital encephalocele: a case report, surgical and anesthetic challenge and review of literature

Abstract

Background An encephalocele is a congenital neural tube defect characterized by herniation of cranial contents through a defect in the cranium and is caused by failure of the closure of the cranial part of the developing neural tube. An encephalocele is termed as “giant encephalocele” when the size of encephalocele is larger than the size of the head. They depend on size of the sac, percentage of neural tissue content, hydrocephalus, infection, and other associated pathologies for a favorable neurological outcome. Case presentation We report a case of a four-month-old boy with a giant occipital encephalocele measuring 21 × 15 × 19 cm in size, which was a surgical and anesthetic challenge for us. Intubation was achieved in lateral position. Part of occipital and cerebellar parenchyma was present in the sac and bony defect was approximately 2.5 cm in occipital bone in midline. We performed surgical excision and repair with a good overall outcome. Conclusion Perioperative management of a giant occipital encephalocele is a challenge for both anesthesiologists and neurosurgeons. Managing such a case demands a search for other congenital abnormalities, expertise in handling airway, and proper intraoperative care. Careful planning and perioperative management are essential for a successful outcome.

Challenging, giant occipital encephalocele in a pediatric saipanese male

“…An MRI was obtained showed a giant encephalocele sac containing a large amount of dysplastic brain tissue and ventricular components with a robust vascular supply as demonstrated on MR venogram (Figures 2 and 3). As noted by Zhahid and Khizar, 8 the meningeal membrane that surrounds the giant encephalocele can be covered by a normal membrane, one that is unusually thin, or alternatively a dysplastic (abnormal) membrane, as was the case with our patient. The “large amount of dysplastic brain tissue” evident on MRI is therefore a reference to the large amount of abnormal tissue surrounding the brain that will eventually require dissection and removal.…”

“…Numerous factors affect the outcome of surgical intervention in patients with encephaloceles, with the occiput being the most common location for this class of cranial dysraphisms. Among these factors include location, the size of the sac, the amount of brain matter herniated into the contents of the sac, the presence versus absence of the brainstem, occipital lobe, and dural sinuses within the sac, and whether or not hydrocephalus is present 8 . Even in cases where the surgeon is well aware of these factors and fully equipped to address them, complications such as intraoperative blood loss and perioperative hypothermia are in some cases inevitable and the potential for their occurrence lends an added layer of complexity to these cases.…”

Chiari III malformation in a neonate

“…The management additionally includes correcting electrolyte imbalances (due to CSF loss) and sudden hypothermia (due to autonomic dysfunction). Postoperative complications, including central nervous system (CNS) infections, respiratory distress, aspiration pneumonia, irreversible impairment of the vagus nerve and hypothermia, need to be promptly addressed 10 12. Fortunately, in our patient, there were no such complications.…”

“…Twenty-one of these cases were either lost to follow-up or their outcome was not mentioned in the literature. Of the rest, 11 cases had a good outcome with minimal or no neurological deficits, 12 cases showed mental retardation with or without global developmental delay, 3 developed infantile spasms or seizures, 2 required continued ventilatory support, 1 developed CNS infection, 1 was stillborn, 1 developed abnormal movements and 7 patients passed away, either due to immediate postoperative complications or later in life due to pneumonia 1 5 12 13 19 20. Given this data, about 18% of the total cases had good outcomes that we know of.…”

Double encephalocele with an excellent outcome postoperatively: A case report from Iraq

“…“An encephalocele is a congenital neural tube defect characterized by herniation of cranial contents through a defect in the cranium and is caused by failure of the closure of the cranial part of the developing neural tube” [ 1 ]. Based on the site of the defect, it is divided into a variety of types, such as occipital, sub-occipital, parietal and basal [ 2 ].…”