Abstract

Airway infantile hemangiomas (IHs) can represent a life-threatening condition since the first months of life. They may be isolated or associated to cutaneous IHs, and/or part of PHACES syndrome. Diagnosis, staging, and indication to treatment are not standardized yet despite the presence in the literature of previous case series and reviews. The diagnosis might be misleading, especially in the absence of cutaneous lesions. Airway endoscopy is the gold standard both for diagnosis and follow-up since it allows evaluation of precise localization and entity of obstruction and/or stricture. Proliferation of IH in the infant airways manifests frequently with stridor and treatment is required as soon as possible to prevent further complications. The first line of therapy is oral propranolol, but duration of treatment is not yet well-defined. All considered, we report the experience of our multidisciplinary center from 2009 to date, on 36 patients affected by airway IHs, and successfully treated with oral propranolol. Thus, the authors propose their experience for the management of airway IHs, specifically early diagnosis, when to perform endoscopy, how to interpret its findings, and when to stop the treatment.

Highlights

  • Infantile hemangiomas (IHs) are the most common benign vascular tumors in children and are a neoplastic proliferation of endothelial cells

  • We present a case series of 36 patients with airway involvement successfully treated with oral propranolol and discuss our multidisciplinary management

  • Airway IHs cause functional damage due to upper airway obstruction leading to respiratory insufficiency and lifethreatening complications in the absence of treatment

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Summary

INTRODUCTION

Infantile hemangiomas (IHs) are the most common benign vascular tumors in children and are a neoplastic proliferation of endothelial cells. Airway IHs (AIHs) constitute a rare occurrence with an unknown incidence They may be associated with cutaneous IH with a particular distribution in 50% of cases, called “beard” area or S3 area, according to Haggstrom and Frieden’s classification [2]. Endoscopy is usually performed using a 4 mmdiameter rigid fiber-optic or 2 mm, based on patient age or size of the stenotic tract. This procedure grants the identification of the AIH, its location, extent, and degree of obstruction. In our series all patients with recurrence of symptoms after the end of the therapy had endoscopic evidence of persistence of the IH, with subglottic obstruction ≥30% (range 30–50%). In 10 patients we found extra-laryngeal airway localizations of AIHs: pharynx in seven cases and trachea in three patients

DISCUSSION
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DATA AVAILABILITY STATEMENT
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