When Rare Anomalies Converge: Anesthetic Management of Pediatric Frontal Encephalocele With Chiari I Malformation and Syringomyelia

Maya Damor; Jishna Vaghela; Janki Panchal; Parth Vaghela

doi:10.31636/prmd.v9i1.6

Authors

Maya Damor Department of Anaesthesia, GCS Medical College, Hospital & Research Centre, Ahmedabad, Gujarat, India
Jishna Vaghela Department of Orthodontics & Dentofacial Orthopaedics, College of Dental Sciences, Amargarh, Bhavnagar, Gujarat, India
Janki Panchal Department of Anaesthesia, GCS Medical College, Hospital & Research Centre, Ahmedabad, Gujarat, India
Parth Vaghela Department of Cardiothoracic and Vascular Surgery, GCS Medical College, Hospital & Research Centre, Ahmedabad, Gujarat, India https://orcid.org/0000-0003-3688-6096 (unauthenticated)

DOI:

https://doi.org/10.31636/prmd.v9i1.6

Keywords:

frontal encephalocele, Chiari I malformation, syringomyelia, pediatric anaesthesia, rare anomaly

Abstract

Background. Encephaloceles associated with craniovertebral junction anomalies such as Chiari malformation and syringomyelia are exceedingly rare and pose unique anesthetic challenges due to risks of diffi cult airway, raised intracranial pressure (ICP), and potential neurological compromise during airway manipulation and positioning.

Case Presentation. We report the anesthetic management of a 13-year-old girl with a large congenital frontal encephalocele (45 × 45 × 52 mm), Chiari I malformation, extensive syringomyelia, basilar invagination, occipitalized C1, and C2–C3 fusion. Airway management was carefully planned with prolonged preoxygenation, intravenous induction, and direct laryngoscopy using manual in-line stabilization. Anesthesia was maintained with sevoflurane and atracurium, ensuring stable hemodynamics and strict neck neutrality. The 10-hour surgical repair was uneventful, and the patient was extubated postoperatively without neurological deterioration.

Conclusion. Th is case highlights the importance of individualized anesthetic strategies focusing on airway safety, ICP control, and neuroprotection in pediatric patients with encephaloceles and complex craniovertebral anomalies.

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