‘Steeple sign’ in PHACE syndrome

Abstract

‘Steeple sign’ demonstrates subglottic narrowing on anteroposterior radiographs of neck soft-tissues, indicating critical narrowing in the proximal trachea (2). In this case, this has been caused by a reoccurrence of a subglottic haemangioma.
Airway haemangiomas are a serious complication of PHACE syndrome (1, 3). They are associated with an increased morbidity and mortality and as such, are important to identify and treat appropriately.
In conclusion Steeple sign is a radiological finding on AP radiograph, which denotes subglottic narrowing.