Airway Hemangiomas Research Articles

Response of infantile airway and facial hemangiomas to propranolol in a patient with PHACE syndrome

Response of infantile airway and facial hemangiomas to propranolol in a patient with PHACE syndrome

Propranolol in the Treatment of Upper Airway Hemangiomas

Airway hemangiomas (AHs), which are common in infant airways, often cause significant upper airway obstruction. The various therapies used for AH have limitations and complications. Propranolol may have a potential role in its treatment, since it leads to regression or stabilization of cutaneous infantile hemangiomas. To date, only 4 previous case reports (7 patients) in which propranolol was used for AH have been published. Based on encouraging preliminary data on propranolol use for AH treatment, our goal was to further investigate propranolol as an effective initial treatment of upper AHs that cause significant obstruction symptoms. In this retrospective case series, we reviewed the medical records of 5 consecutive pediatric patients with AH (glottic and subglottic) treated with propranolol at a tertiary care children's hospital. All 5 patients were 2 months of age at the time of hemangioma diagnosis and had stridor and physical signs of severe upper airway obstruction. Hemangioma was diagnosed by flexible laryngoscopy or flexible bronchoscopy. All patients received propranolol 2 mg/kg/day and showed significant relief of obstruction symptoms within 24 hours of treatment initiation. All patients tolerated propranolol without significant cardiovascular complications. Outcomes from this case series, in conjunction with available case reports in the literature, suggest that propranolol is a safe initial treatment for symptomatic upper AH.

Pediatric Neck Masses

Pediatric Neck Masses

Response of infantile airway and facial hemangiomas to propranolol in a patient with PHACE syndrome

Abstract PHACE syndrome is a neurocutaneous syndrome defined by posterior fossa malformations (P), hemangiomas (H), arterial anomalies (A), cardiac defects and coarctation of the aorta (C), and eye abnormalities (E). Propranolol is currently first line therapy for treatment of head and neck hemangiomas that cause functional defects or extreme disfigurement. The safety and efficacy of this treatment have been well documented in current literature, but few authors have reported the use of propranolol in patients with PHACE syndrome. We report a case of a patient with PHACE Syndrome and associated infantile airway and facial hemangiomas who was successfully treated with propranolol and a review of the literature on this subject.

Airway hemangiomas in PHACE syndrome

To describe the clinical presentation and airway characteristics of infants with airway hemangiomas and concomitant PHACE syndrome and to determine the prevalence of airway hemangiomas in PHACE subjects at our institution. Case series. Retrospective review including clinical presentation, airway findings, treatment measures, and outcomes. A total of 23 subjects were diagnosed with definite PHACE at our institution between September 1, 2005 and September 1, 2011. Twelve (52%) of these subjects had documented airway hemangiomas, six of whom were diagnosed and treated at our institution. All six subjects underwent direct laryngoscopy and bronchoscopy by a pediatric otolaryngologist. Five (83%) subjects had subglottic hemangioma. Three subjects (50%) had additional hemangioma within the airway located on the epiglottis, vocal folds, posterior pharyngeal wall, and tracheal wall. Five subjects (83%) were treated with propranolol, five (83%) were treated with systemic steroids, and one subject received vincristine. One subject required laser ablation of subglottic hemangioma and tracheotomy. All subjects were airway symptom free at last follow-up (average, 35 months; range, 13-76 months). Airway hemangiomas can be a life-threatening complication of PHACE syndrome. At our institution, 52% of all PHACE subjects were diagnosed with airway hemangiomas. Early detection of airway involvement is paramount. Given the high rates of airway hemangiomas, we recommend performing direct laryngoscopy and bronchoscopy in all PHACE patients with respiratory symptoms. We recommend having a low threshold for airway evaluation in asymptomatic PHACE patients, especially those who will not be otherwise started on propranolol.

Anestesia en embarazada para traqueostomía urgente por hemangioma sangrante en lengua

We present the case of a 20 year old pregnant patient diagnosed with cavernous hemangioma in her tonge, lips and neck with active bleeding. An emergency tracheostomy under general anesthesia was scheduled. Anesthesia was planned for conscious intubation with a fibrobronchoscope suited for the difficult airway management ASA algorithms. The airway was managed with a number 5.5Fr endotracheal tube under 0.02-0.05μg/kg/min remifentanyl infusion. The patient was sedated and given local anesthesia in the upper airway to reduce pain and gag reflex during spontaneous breathing. Intubation was successful. Having a total blood loss of 200cc, the patient was taken to the postoperative care room hour after surgery. The airway was managed with a tracheostomy procedure using a number 7.5Fr tube and without damaging the hemangioma. She was then scheduled for a vessel embolectomy and surgical removal. In this case, conscious intubation with remifentanyl was safe and adequate for the approach of the patient with bleeding airway hemangioma.

PHACES syndrome in association with airway hemangioma: First report from Saudi Arabia and literature review

“PHACES” is a neurocutaneous syndrome that refers to the following associations: Posterior fossa malformations, Hemangiomas, Arterial malformations, Coarctation of the aorta/Cardiac defects, Eye abnormalities, and Sternal defects. Herein, we report the association of PHACES syndrome with airway hemangioma, a serious association that should not be overlooked. The findings of such an association presented here are the first to be reported from Saudi Arabia.

Propranolol for the treatment of subglottic hemangiomas

Introduction Infantile subglottic hemangiomas are rare causes of airway obstruction. They begin to proliferate at 1–2 months of age and can cause biphasic stridor with or without respiratory distress. Diagnosis requires direct visualization by direct laryngoscopy and bronchoscopy. Various therapeutic options have been utilized for treatment, including tracheotomy, open surgical excision, laser ablation, intralesional steroid injection, systemic steroids, and now oral propranolol. Methods We present a retrospective chart review of infantile subglottic hemangiomas over a 5-year span (January 2005–2010) at a tertiary care pediatric hospital. IRB approval was obtained, and charts were reviewed to find patients with subglottic hemangiomas, including patient characteristics, presentation, workup, medical and surgical management, and outcomes. A case presentation demonstrates diagnostic, management, and treatment strategies and dilemmas encountered. Results Nine patients were found to have infantile subglottic hemangiomas. Six of nine patients were treated with laser excision, with five of the six having localized subglottic hemangiomas. In 2009, three of four patients were initiated on propranolol as first-line treatment; the fourth had comorbidities which precluded this. Of the three, two showed improvement, while a third, who also had bearded hemangioma, required tracheotomy. Discussion Infantile subglottic hemangiomas are rare but essential in the differential diagnosis of biphasic stridor. Although propranolol has been effective in treating cutaneous and airway hemangiomas, our experience suggests that this is not consistent for subglottic hemangiomas. In an area where airway compromise can be lethal, we must extend caution and monitor these patients closely as they may require adjuvant therapy.

KTP laser ablation of extensive tracheal hemangiomas

We are reporting the case of a female child presenting for the management of symptomatic extensive tracheal hemangiomas who was successfully treated with multiple ablative procedures using KTP laser. This is the first description in the English literature of the use of KTP laser as primary treatment modality for the endoscopic ablation of tracheal hemangiomas in a child. We review treatment recommendations for symptomatic airway hemangiomas in the pediatric population, highlighting the advantages of KTP laser for tracheal lesions.

Clinical Spectrum and Risk of PHACE Syndrome in Cutaneous and Airway Hemangiomas

To describe the clinical presentation and risk of PHACE syndrome in infants with large facial hemangiomas and concomitant airway hemangiomas. The study involved a case series of infants with cutaneous hemangiomas and airway hemangiomas extracted from a prospective multicenter cohort study. Data regarding clinical features, diagnosis, treatment, and clinical course were obtained from medical charts and physician intake forms. All patients were evaluated for PHACE syndrome using a standardized protocol. Six academic pediatric dermatology clinics. The study included 17 patients younger than 1 year who were diagnosed as having large (>22 cm(2)) facial hemangiomas and airway hemangiomas. Thirteen patients (76%) had hemangiomas in the bilateral mandibular distribution. Other observed facial patterns included limited involvement of the lip and chin, unilateral reticular frontotemporal and preauricular hemangiomas, and large unilateral hemifacial hemangiomas. Fourteen patients (82%) had symptomatic airway involvement. All symptomatic patients had subglottic airway hemangiomas. The airway hemangioma was circumferential in 10 patients (58%) and more focal in distribution in 7 patients (42%). All patients were treated with oral prednisolone. Eleven patients required additional multimodal therapy. Eight patients (47%) met the criteria for PHACE syndrome. Airway hemangiomas represent a potentially fatal complication of infantile hemangiomas. Our data highlight cutaneous presentations in patients with subglottic hemangiomas and large (>22 cm(2)) cutaneous hemangiomas. PHACE syndrome was detected in 8 such patients (47%) in our series.

An Infantile Bronchial Hemangioma Unresponsive to Propranolol Therapy

Current options for treating airway hemangiomas include systemic and intralesional corticosteroids, interferon therapy, chemotherapy, use of lasers, open surgical excision, tracheotomy, sleeve resection, and selective artery embolization. The significant rates of complications described for these methods 1,2 make the discovery of a novel therapy an attractive prospect. Several case reports and case series 3-9 have recently appeared in the literature describing the use of propranolol as a highly effective option for the treatment of these airway lesions. Therefore, we reviewed the literature for information on the management of airway hemangiomas by performing a comprehensive PubMed search, focusing particularly on the use of propranolol in the treatment of airway hemangiomas, defined as occurring in the subglottis, trachea, and/or bronchi. We also describe a a 3-month-old boy with a large bronchial hemangioma that did not respond to propranolol therapy.

Propranolol for the treatment of airway hemangiomas: A case series and treatment algorithm

Objectives (1) To present six patients with symptomatic airway hemangiomas treated with oral propranolol. (2) To review the diagnostic and treatment options for airway hemangiomas and propose a new management protocol. Study design Retrospective review. Setting Tertiary care children's hospital. Subjects and methods Pediatric patients diagnosed with obstructive airway hemangiomas treated with oral propranolol. Patients were followed for symptomatic improvement and relief of airway obstruction on imaging or laryngoscopy. Results Seven patients presenting with airway obstruction were treated with propranolol. One patient had a focal hemangioma confined to the subglottis. Four patients had airway hemangiomas that extended beyond the confines of the larynx and trachea. A sixth patient had a bulky supraglottic hemangioma. A seventh patient with an extensive maxillofacial lesion failed propranolol therapy and was found to have a pyogenic granuloma on final pathology after excision. Six patients had failed standard medical therapy and/or surgical interventions and were treated successfully with oral propranolol with improvements in airway symptoms and oral intake, requiring no further surgical intervention. Treatment was initiated as early as 1.5 months of age, and as late as 22 months. No adverse side effects of propranolol were noted. Conclusions Oral propranolol was successfully used to treat airway hemangiomas, resulting in rapid airway stabilization, obviating the need for operative intervention, and reducing the duration of systemic corticosteroid therapy while causing no obvious adverse effects. These outstanding results enable the possibility of use of a standardized diagnostic and treatment algorithm for airway hemangiomas that incorporates systemic propranolol.

Propranolol in the Management of Airway Infantile Hemangiomas

To report our experience with propranolol in managing airway infantile hemangiomas. Case series of 3 consecutive patients who had extensive, symptomatic airway infantile hemangiomas treated with propranolol. Tertiary academic medical center. Three infants with facial cutaneous hemangiomas who developed stridor that progressed to respiratory distress, which according to laryngoscopic examination results was confirmed to be caused by extensive subglottic hemangiomas. These patients underwent follow-up during their course of therapy, ranging from 3 weeks to 15 months. Patient 1 failed to respond to systemic corticosteroids, laser ablation, and intravenous vincristine for her airway hemangioma and had to undergo tracheotomy. She was given propranolol after her tracheotomy and had a significant reduction in her subglottic airway obstruction. Patient 2 developed progressive stridor secondary to airway hemangioma at age 6 1/2 months following tapering of systemic corticosteroids prescribed for her periorbital hemangioma. Systemic corticosteroids were restarted with the addition of propranolol. The stridor improved within 24 hours, and she was able to be weaned off corticosteroids. Patient 3 was also treated with initial combined therapy of systemic corticosteroids and propranolol. He had a significant reduction in stridor within 24 hours and was weaned off corticosteroids. Our 3 patients had severe respiratory symptoms related to their airway infantile hemangiomas. In the first patient, propranolol was used when other treatments were ineffective or associated with intolerable adverse effects. In the second and third patients, propranolol was part of a dual regimen that resulted in rapid resolution of airway symptoms and allowed for quicker weaning of corticosteroids.

Extensive Airway Hemangiomas in Two Patients Without Beard Hemangiomas

Airway hemangiomas are most often seen in association with cutaneous hemangiomas involving the "beard area." We report two unusual cases of extensive airway hemangiomas developing in patients with facial hemangiomas predominantly involving the upper face, emphasizing the need to consider the possibility of airway hemangiomas even in the absence of "beard area" hemangiomas.

Case report: Treatment failure using propanolol for treatment of focal subglottic hemangioma

Subglottic hemangioma is a rare, potentially life threatening tumor of infancy which poses serious treatment challenges. A number of medical and surgical therapies over the years have met with variable success, and are associated with numerous potential morbidities. A potential windfall in the management of infantile hemangiomas has arisen with the recent identification of propanolol as a highly efficacious and relatively safe new treatment modality. At least five reports in the literature have described the rapid, successful treatment of airway hemangiomas with oral propanolol. We describe the first reported treatment failure with propanolol for subglottic hemangioma in an infant who initially responded dramatically to the medication.

Observaciones clínicas sobre el uso de propranolol en hemangiomas de vía aérea infantil

Treatment of subglottic hemangiomas in children has traditionally been characterized as a challenging situation with multiple therapeutic options without consensus as to which one is the best and with risks of severe side effects. Recent reports on the experience of propranolol use in the treatment of paediatric airway hemangiomas suggest favourable reasons for this use due to the rapid improvement and its lack of severe side effects. In this paper we report the experience with 6 children having symptomatic airway hemangiomas treated with propranolol. All children improved their respiratory symptoms dramatically and did not need additional interventions.

Initial experience using propranolol as the sole treatment for infantile airway hemangiomas

The objective of this study is to describe the initial use of propranolol as the sole treatment for focal infantile airway hemangiomas, and to report on available literature describing the use of propranolol for airway lesions. This retrospective case series was carried out at a tertiary pediatric medical center. We obtained the following results: two children demonstrated significant response to oral propranolol therapy and avoided not only invasive surgical procedures, but also long-term administration of oral corticosteroids. This is the first report of treating infantile airway hemangiomas with only propranolol without additional surgical intervention or corticosteroid use. Review of literature reveals initial case series with similar, successful results using propranolol as an adjuvant treatment along with other medications and surgical interventions. We conclude that the initial use of propranolol as the sole treatment for infantile airway hemangioma is promising. Literature review reveals that propranolol as the sole treatment for most head and neck hemangiomas shows significant promise based on early case reports. Further studies are needed to determine the long-term effectiveness, dosing strategies, and side effect profile of propranolol treatment for hemangiomas.

Segmental hemangiomas of the upper airway

To characterize the anatomic distribution of segmental hemangiomas of the larynx and to describe indications for treatment modalities. Retrospective chart review. We performed a retrospective chart review of patients with cutaneous hemangiomas at a tertiary care center over a 4-year period. Only patients with upper airway hemangiomas were studied. We reviewed the anatomic distribution of hemangiomas within the upper airway and the treatment course of each patient. Of 1,226 patients with cutaneous hemangiomas, 108 (9%) were segmental in distribution. There were 56 patients (52%) who had a V3 distribution pattern, and 16 patients (29%) with upper airway involvement. All of these patients had associated V3 or mandibular segmental hemangiomas. As with the cutaneous manifestation, the distribution of hemangioma within the upper airway was segmental. This included the following anatomic sites: oral cavity, oropharynx, hypopharynx, intrinsic structures of the larynx, and subglottis. Many of these patients had diffuse mucosal involvement, including tracheal involvement. A total of 13 out of 16 patients underwent medical intervention, and seven also required surgical intervention. Medical management included systemic (12 patients) and intralesional (two patients) steroids. One patient received chemotherapy prior to referral. Surgical treatment included tracheostomy (four patients prior to referral) and laser ablation of subglottic involvement (total of four patients). A high percentage of patients with V3 cutaneous hemangiomas (29%) will manifest with upper airway involvement, the distribution of which is segmental. Treatment should take this diffuse pattern of involvement into consideration.

Proposal for staging airway hemangiomas

Objective To describe a method of airway infantile hemangioma staging using standardized assessment of airway narrowing, and hemangioma location and volume, as determined with endoscopy and CT angiography. Study design Case series with chart review. Setting Tertiary pediatric hospital, 2003-2008. Subjects and methods Subjects included airway hemangioma patients evaluated at a tertiary pediatric hospital. Data collected were age at first symptoms, diagnostic evaluation, percent airway compromise, and estimated hemangioma volume. Data were analyzed with descriptive and Fisher exact statistics. Results Twelve patients were identified and seven had complete data sets. Mean age at first symptoms was 1.9 months (SD 1.09 months, range 0.5-4 months). Evaluation consisted of nasopharyngoscopy, microlaryngoscopy, CT angiography, and/or MRI. Mean laryngeal airway narrowing was estimated at 63.75 percent (SD 19.0%, range 40%-90%). Total hemangioma volume was less in patients with isolated (focal) endolaryngeal hemangiomas compared with airway hemangiomas associated with extralaryngeal (segmental) hemangiomas. Airway hemangioma stages were stage one (5 of 12; 41.6%), stage two (6 of 12; 50.0%), and stage three (1 of 12; 8.3%). Conclusion This method of airway hemangioma staging may be applicable to treatment planning and used to measure treatment outcomes.

Propranolol for the treatment of airway hemangiomas

Propranolol for the treatment of airway hemangiomas