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