79th PostGraduate Assembly in Anesthesiology 2025

Background

Encephalocele

An encephalocele is a neural tube defect involving a skull defect and spread of intracranial structures extracranially.
Arises from a failure of separation between surface ectoderm from neuroectoderm.
Occipital encephaloceles (OE) are the most common, usually over the midline.
Incidence: 0.8-5.6/10,000 live births, more common in females than males
Associated with hydrocephalus in 60% of OE cases.

Complications of Encephalocele

Complications relate to size, location, and composition of the herniation.
Injury to the skin can cause rupture of the encephalocele and a large volume loss of CSF leading to rapid hemodynamic instability.
Patient cannot be placed supine on a flat surface for induction of anesthesia.

Case Presentation

A three-day old male born via C-section at 37 4/7 weeks with an occipital encephalocele.

Past Medical History

Airway Positioning and Management

Goal was to prevent trauma to the fragile encephalocele and to optimize positioning for successful first attempt at intubation.
Patient was intubated supine with the head placed in a horseshoe headrest.
Encephalocele was stabilized throughout induction by the neurosurgery team.
Patient was preoxygenated, pretreated with glycopyrrolate to limit secretions, and induced with incremental dosing of ketamine to maintain spontaneous respirations.
Video laryngoscopy used – Miller 0 with size 3 ETT.
Paralyzed with rocuronium and maintained with sevoflurane.
Patient was carefully proned in the horseshoe headrest for surgery.

Discussion

Hospital course: Patient extubated to face mask, transported to NICU. VP shunt placed 2 weeks later.

Medically complicated course but discharged from NICU after 1 month.
At 6 months, babbling, responds to sounds, recognizes familiar faces, sits unassisted, transfers objects

Alternative approaches considered: lateral positioning, maintain spontaneous respirations, fiberoptic

Concerns: longer duration, distorted view of airway structures while lateral, secretions may obscure view
Difficulty of surgical airway in neonates (short neck and relatively thick anterior neck tissues)

The Horseshoe Hold: Navigating the Neonatal Airway Around an Occipital Encephalocele