Abstract
Objective
Subglottic hemangiomas may present a diagnostic challenge in the stridorous infant without a cutaneous lesion.
Methods
Consecutive case series with chart review of isolated subglottic hemangiomas without cutaneous lesions.
Results
Two patients with isolated subglottic hemangiomas without cutaneous lesions were identified. Both patients presented with stridor and were initially treated with systemic corticosteroids, racemic epinephrine, and supportive medical therapy for a presumed infectious etiology. After failure to clinically improve over an expected period of time, endoscopy was performed. Propranolol was initiated immediately after the diagnosis was made. Clinical response was noted in the first 24 hours after administration. Both children were able to be safely titrated up to the therapeutic dose (2 mg/kg/day). No complications or re-admissions were encountered.
Conclusions
Clinical suspicion for a subglottic hemangioma in an infant with stridor may be high in cases where there is a synchronous cutaneous lesion. In infants without cutaneous involvement, diagnosis may be delayed and more challenging, given the possibility of an infectious etiology. These infants without a synchronous cutaneous lesion tend to present at an older age than infants with cutaneous lesions and may be managed successfully with propranolol.
1
Introduction
Management of subglottic hemangiomas has evolved significantly over the past several decades. Propranolol has fundamentally altered the landscape of treatment for this clinical problem. Propranolol was first introduced as an effective treatment for severe, cutaneous infantile hemangiomas in 2008. One year later, Denoyelle et al. were the first to report successful treatment of subglottic hemangiomas in two patients with severe disease, one of which was associated with a posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft and supraumbilical raphe syndrome (PHACES) child and the other with widespread cutaneous involvement . Following this, several other single case reports also supported this finding .
Rosbe et al. described a small case series of three consecutive patients with synchronous, cutaneous infantile hemangiomas that developed stridor and were found to have subglottic involvement. All three also presented with cutaneous facial lesions, raising the clinical suspicion . Isolated hemangiomas without a synchronous cutaneous lesion may be clinically challenging to diagnose, as the presentation of these infants will mimic other infectious or congenital subglottic pathology. Two patients are described to highlight the importance of considering this pathology in infants under one year of age with stridor without cutaneous hemangiomas and the effectiveness of propranolol in management.
2
Materials and methods
A description is provided of two patients with subglottic hemangiomas without cutaneous lesions that presented to a tertiary-care children’s hospital. Institutional review board (IRB) approval was sought and was deemed exempt from review.
Immediately after the diagnosis of a subglottic hemangioma was made, institutional propranolol titration protocol was initiated during the patients’ inpatient admissions and started at 0.5 mg/kg/day divided three times per day on the first day. The medication was administered during feedings. Blood pressure and heart rate monitoring was performed one hour after dosing was given. Propranolol was then titrated up to 1 mg/kg/day divided three times per day the following day, and finally up to the therapeutic dose of 2 mg/kg/day divided three times per day on the third day. The patients and families were discharged home after detailed instructions on administration and education regarding side effects to be aware of with propranolol.
2
Materials and methods
A description is provided of two patients with subglottic hemangiomas without cutaneous lesions that presented to a tertiary-care children’s hospital. Institutional review board (IRB) approval was sought and was deemed exempt from review.
Immediately after the diagnosis of a subglottic hemangioma was made, institutional propranolol titration protocol was initiated during the patients’ inpatient admissions and started at 0.5 mg/kg/day divided three times per day on the first day. The medication was administered during feedings. Blood pressure and heart rate monitoring was performed one hour after dosing was given. Propranolol was then titrated up to 1 mg/kg/day divided three times per day the following day, and finally up to the therapeutic dose of 2 mg/kg/day divided three times per day on the third day. The patients and families were discharged home after detailed instructions on administration and education regarding side effects to be aware of with propranolol.
3
Results
Two patients diagnosed with isolated subglottic hemangiomas without cutaneous lesions were identified. Both of the patients were male, and the age at presentation was 5 and 7 months. They presented with stridor without any history of prematurity or prior intubation. Concomitant increased secretions and respiratory symptoms were also present.
Both were initially treated with systemic corticosteroids, racemic epinephrine, and supportive medical therapy for a presumed infectious etiology. They underwent respiratory panel testing in which the results were unremarkable. After failure to significantly clinically improve over 10 days in both cases, endoscopy was performed, and the diagnosis of subglottic hemangioma was made ( Fig. 1 and 2 ). Propranolol was initiated immediately after the diagnosis was made. Clinical response was noted almost immediately after initiation of therapy, with symptoms of stridor and feeding difficulties improved within the first 24 hours of administration. Both children were able to be safely titrated up to the therapeutic dose (2 mg/kg/day) and discharged home shortly after starting on propranolol, 4 and 2 days, respectively, with tapering courses of oral corticosteroids. Neither infant required intubation at any time during their hospitalization. The average length of hospital stay was 14 and 12 days, respectively. The patients were on propranolol therapy for 7 months and 6 months in total duration, respectively and no difficulties with propranolol and no readmissions to the hospital for complications related to propranolol administration were experienced. After three and two months being symptom free from stridor after discontinuation of propranolol therapy, respectively, both patient’s experienced acute croup-like symptoms and were managed successfully with oral corticosteroid tapers. Neither patient was restarted on propranolol.
