An Infant with Large Frontonasal Encephalocele: A Case-based Airway Perspective to Neuroanesthesia

Dear Editor, Encephalocele is a congenital anomaly caused by a neural tube defect, resulting in herniation of intracranial contents through abnormal skull openings.[1,2] Frontonasal encephalocele includes nasofrontal, naso-ethmoidal, and naso-orbital defects.[3] Frontonasal encephaloceles are challenging to anesthesiologists as they might obstruct the nasal cavity resulting in difficult mask ventilation and difficult intubation.[4] This letter highlights the airway management and anesthetic considerations of 12-month-old infant with frontonasal encephalocele. A 12-month-old infant, weighing 7.5 kg, was planned for right ventriculoperitoneal (VP) shunt and excision of frontonasal encephalocele with craniofacial reconstruction. On examination, the infant had a bony defect in the frontal bone at frontonasal region (15.2 mm × 20 mm) with herniation of bilateral frontal lobes, part of the left parietal and temporal lobes along with the meninges [Figure 1A]. On preoperative evaluation, the infant was active with normal cry but delayed milestones. Two-dimensional echocardiography showed normal left ventricular systolic function with a 60% ejection fraction. Hemogram showed anemia (hemoglobin: 10.3 g/dL) with thrombocytosis (platelet count: 857,000/mcL). The surgery was planned under general anesthesia. Informed written consent was taken from the infant’s mother.Figure 1: A: Depicts a large frontonasal encephalocele in an infant. B: Depicts frontonasal encephalocele moved to right before bag-mask ventilation. C: Successful bag-mask ventilation in an infant with frontonasal encephalocele. D: Successful endotracheal intubation with tube secured on left sideOn the day of surgery, the difficult airway cart was kept ready. All the American Standard of Anesthesiologists monitors were attached. As this swelling was movable, we moved the swelling gently to the right side during bag-mask ventilation, as shown in Figure 1B and C. The infant was premedicated with fentanyl (2 mcg/kg) and induced with propofol (2 mg/kg) and atracurium (0.5 mg/kg). In view of difficult intubation, we performed video laryngoscopy guided intubation and fixed endotracheal tube (3 mm cuffed) on the left side [Figure 1D]. Being a major surgical procedure with expected blood loss of more than the maximum allowable blood loss (~37 mL) (frontonasal encephalocele excision followed by craniofacial reconstruction), right-sided arterial line and left-sided internal jugular vein (IJV) were secured (on right side VP shunt was planned). Anesthesia was maintained with sevoflurane (0.2–2% w/v) and oxygen in the air (1:1). Intraoperative fluid management was done with ringer lactate at 4 mL/kg/hr. Blood transfusion was done (10 mL/kg). Normothermia was maintained throughout the surgery. The surgical procedure lasted for 9 h. The infant was extubated at the end of surgery and was shifted to the pediatric intensive care unit for monitoring. In this case, the main anesthesia-related concerns were the infant age group, difficult airway, and blood loss. Patients with frontonasal encephaloceles not only have difficult intubation but also difficult bag-mask ventilation because in some cases, it might completely obstruct the nasal cavity.[5] So, we should always be ready with plan B in case bag-mask ventilation is difficult. Our plan B was to insert a supraglottic airway device for ventilation (after giving propofol) followed by intubation (after muscle relaxation). But in this case, the swelling was soft and movable, so gently moving the swelling toward the right side provided us space for keeping the mask covering the nose and mouth. As craniofacial reconstruction was planned in this case, so blood loss was our second concern. The left-sided IJV was secured as peripheral venous access was also difficult in this infant. The learning objective, in this case, is to be ready with a difficult airway cart and plan B for difficult bag-mask ventilation and difficult intubation. Ethics approval As it is a letter to the editor, ethics approval was not deemed necessary. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.