Subglottic hemangioma: Understanding the association with facial segmental hemangioma in a beard distribution

https://doi.org/10.1016/j.ijporl.2018.07.019Get rights and content

Abstract

Objective

A subglottic hemangioma (SGH) is a benign tumor of infancy that can cause severe obstruction of the airway. Infantile hemangiomas, in general, are the most common head and neck tumor in children, affecting 4–5% of the pediatric population. This retrospective cohort study characterizes subglottic infantile hemangiomas at a single vascular anomaly center over a 5-year period (2013–2017) during the era of propranolol treatment.

Methods

Queried the Vascular Anomaly Database at Children's Hospital of Pittsburgh for all infantile hemangioma(s) and then identified case of subglottic hemangiomas. Characterized key features of presentation, natural history and management for subglottic hemangiomas. A secondary differentiation focused on differences between subglottic hemangiomas associated with Beard Distribution (BD) vs not (NBD).

Results

Analysis of 761 cases of infantile hemangiomas demonstrated only 13 patients with subglottic hemangiomas (1.7%). Of those 13 patients, only 4 patients (30%) had BD while 2 patients (15%) had other cutaneous hemangiomas and 7 patients (55%) had no cutaneous hemangiomas. Secondarily, a total of 31 case of beard distribution cutaneous hemangiomas with 11 patients having oropharyngeal involvement (35%) but only 4 patients with subglottic hemangiomas (13%). Interestingly, 2 of the 4 BD patients had treatment failure on propranolol and required second line treatment with steroids or surgical excision while only 1 of 9 NBD patients failed propranolol treatment. As well the same 2 BD patients which failed propranolol also had PHACES syndrome.

Conclusion

Subglottic hemangiomas are a rare presentation of infantile hemangiomas but with significant morbidity. While the classic teaching that a segmental beard distribution hemangioma raises concern for a subglottic hemangioma, this cohort indicates subglottic hemangiomas occur in a NBD presentation (1.3%), and demonstrated only an approximate 10% incidence rate with a beard distribution. But more importantly, this study raises the question that beard distribution in setting of PHACES syndrome may herald a more recalcitrant and complicated natural history for a subglottic hemangioma. This is of significant concern as risk for CVA in setting of PHACES is highest with use of steroid treatment. None of our patients had high risk extra or intra cranial vascular arterial anomalies and no CVA were noted.

Introduction

The infantile hemangioma (IH) is a proliferative vascular tumor consisting of endothelial cells which can occur anywhere in the body. IH is the most common head and neck tumor in children with an incidence of 4–5% in the general population [1] but involvement in the subglottic region is both rare and life threatening. The classic teaching is that the IH is not present at birth but then develops in the first months of life. The IH first enters a proliferation phase and rapidly grows for 3–5 months. This is followed by a prolonged involution phase with spontaneous regression over the course of years [2]. Because of the small caliber of the infant airway, during the proliferative phase the rapidly growing IH of the subglottic region can lead to a life-threatening airway obstruction. A study by Perkins et al. evaluating US trends in management of subglottic IH demonstrated increased morbidity (30% re-hospitalization) and mortality (2%) in this subpopulation [3]. Therefore, early diagnosis and efficacious treatment modalities are crucial to the successful management of children with subglottic IH.

The standard teaching is that a segmental IH in a beard distribution heralds airway involvement. One study demonstrated that airway involvement in the oral cavity, oral pharynx, hypopharynx, glottis, or sub glottis was seen in 29% of the children with beard distribution hemangioma [4]. In contrast, another classic presentation of subglottic IH is after a persistent croup-like illness without any cutaneous involvement [5]. The literature has not given a complete picture of presentation and natural history of the subglottic IH. The narrative has become more complicated with the recent advent of propranolol and a shift in treatment modalities away from oral steroids and surgical resection as demonstrated by a recent multi-center trial [6].

The purpose of this study is to characterize the presentation, natural history and management of subglottic IH at a single vascular anomaly center over a 5-year period in the age of propranolol management. The aim is to determine indicators of early diagnosis and markers of disease prognosis.

Section snippets

Method

This study was IRB approved by the Institutional Review Board at Children's Hospital of Pittsburgh of UPMC. Retrospective review was preformed to identify all patients presenting to the Vascular Anomaly Center at Children's Hospital of Pittsburgh between 2013 and 2017 with an infantile hemangioma of the subglottic region. Data was collected on patient demographics, cutaneous involvement, characteristics of subglottic obstruction and response to treatment. A secondary analysis reviewed all

Results

From 2013 to 2017 there were 761 individual patients seen at the Vascular Anomaly Center at Children's Hospital of Pittsburgh. Of those 761 patients, only 13 patients diagnosed with a subglottic hemangioma. Demonstrating an incidence of 1.7% of patients presenting to a Vascular Anomaly Center. As well, only 31 patients had facial segmental hemangiomas in zone 3 (beard distribution) with 11 patients having associated oropharyngeal involvement but (35%) but only 4 of those patients having

Discussion

This retrospective cohort studies aimed to evaluate the natural history and treatment response rates of subglottic hemangiomas in the era of propranolol treatment. The study demonstrated that children with segmental infantile hemangiomas in the beard distribution of the face can have associated oropharyngeal at similar rates as previous studies described. This study aimed to differentiate oropharyngeal involvement from subglottic lesions. In this study, subglottic lesions occurred with an

References (7)

There are more references available in the full text version of this article.

Cited by (0)

View full text