When Faced With Anesthetizing an Infant With PHACE Syndrome: Watch Out for an Airway-Occluding Subglottic Hemangioma!

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We write to bring your readers’ attention to an unexpected airway problem in a clinically stable infant with PHACE (posterior fossa anomalies, hemangiomas, arterial lesions, coarctation of the aorta, and eye anomalies) syndrome, who was anesthetized for a magnetic resonance imaging (MRI) of the brain and neck.
PHACE syndrome is a neurocutaneous disorder of unknown etiology and includes posterior fossa anomalies, hemangiomas, arterial lesions, coarctation of the aorta, and eye anomalies.1 This is sometimes referred to as PHACES syndrome with the “S” emphasizing the possibility of sternal clefting or supraumbilical raphe. It is important to urgently evaluate these patients for coarctation of the aorta or cerebral vascular anomalies, because they are at increased risk for stroke. Oropharyngeal hemangiomas and airway hemangiomas have also been reported in these patients.2
One recent encounter with an infant with suspected PHACE syndrome warrants a critical safety message to all anesthesiologists who may encounter such a patient undergoing workup for this syndrome. In this case, a full-term 5-kg infant with eye swelling and a facial hemangioma presented for a sedated echocardiogram and MRI of the brain and neck for workup of PHACE syndrome. Since she was breathing spontaneously on room air and in no respiratory distress, anesthesia was induced with mask inhalation of sevoflurane for intravenous line placement. Sedation for the MRI was maintained via a propofol infusion, while administering oxygen via nasal cannula. During the scan, the radiologist informed us of near-complete obstruction at the subglottic region, possibly due to a hemangioma. After an immediate consultation with an ear, nose, and throat surgeon, the patient was rapidly transferred to the operating room for a direct laryngoscopy/bronchoscopy, which revealed an occluding subglottic hemangioma in the airway (Figure). The ear, nose, and throat surgeon recommended the use of a size 2.0 endotracheal tube if the child should require intubation in the near future. The patient was subsequently medically managed with steroids and propranolol.
Encountering an unexpected nearly complete airway obstruction in an infant is every anesthesiologist’s nightmare. If there is an urgent need to intubate this type of infant to bypass the obstructing vascular lesion, the risks and complications would be even higher in a remote location, such as the MRI induction bay or inside the scanner. If our team had intubated this child with a 3.0 cuffed endotracheal tube, as would be appropriate for her age, we may have inadvertently caused trauma to the hemangioma, leading to bleeding or further airway obstruction. Emergent airway manipulation in these patients, who may not be adequately anesthetized, also carries the risk of stroke, due to potentially coexisting cerebral cavernous malformations.3
The radiologist’s vigilance in detecting this mass in the airway during the scan and his quick response in alerting the anesthesiologist allowed the smooth transfer of the patient from the MRI area to the OR and avoidance of any urgent airway manipulation. We hope our positive team experience will help all anesthesiologists to consider the possibility of subtle occluding hemangiomas lurking in the airway (subglottic or lower), even if the infant is clinically asymptomatic, when faced with anesthetizing any infant with PHACE syndrome.
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