Airway Hemangiomas in PHACE Syndrome: A Multicenter Experience.

OBJECTIVE

To describe the prevalence and clinical characteristics of airway findings in a multi-institutional cohort of PHACE patients.

STUDY DESIGN

Multicenter retrospective case series.

SETTING

Multidisciplinary vascular anomalies clinics at 2 institutions.

METHODS

Data were collected from the electronic medical record, including clinical presentation, airway findings, treatment, and outcomes.

RESULTS

Of 55 PHACE patients, 22 (40%) had airway hemangiomas. Patients with airway involvement were more commonly female ( = .034, odds ratio [OR] 23, 95% confidence interval [CI] 1.3-410) and of Caucasian ethnicity ( = .020, OR 5.3, 95% CI 1.3-21). Anatomically, patients with bilateral S3 involvement had higher rates of airway disease ( = .0012, OR 15, 95% CI 2.9-77). Most patients with airway hemangiomas had stridor (68%). Of the patients managed in the propranolol era (2008 or later, n = 35), 14 had airway involvement. All 14 were treated with propranolol, whereas 13 (62%) of 21 nonairway patients were treated with propranolol. The average treatment duration was longer in the airway patients (22.1 vs 16.7 months). All patients who underwent tracheostomy (n = 4) did so before 2008.

CONCLUSION

Risk factors for airway involvement in PHACE include female gender, Caucasian ethnicity, and stridor. Since the widespread use of propranolol, fewer patients have required surgical management of their airway disease. Given the high prevalence of airway involvement even in patients without stridor, assessment of the airway is a crucial component of a comprehensive PHACE workup.