Airway Infantile Hemangiomas Research Articles

Infant in extremis: respiratory failure secondary to lower airway infantile hemangioma

BackgroundInfantile hemangiomas (IHs) are vascular tumors that commonly affect infants and usually regress spontaneously or can be easily treated as an outpatient with topical beta-blockers. However, IHs that present in the airway may cause life-threatening symptoms due to airway obstruction or risk of bleeding. Here we present the first documented case of an infant with rapid deterioration and acute respiratory failure secondary to a lower airway hemangioma.Case presentationThis 3-month-old male initially presented in respiratory distress with symptoms consistent with a viral respiratory infection, however showed no clinical improvement with standard therapies. An urgent CT scan revealed a mass occluding the right mainstem bronchus. Upon transfer to a tertiary care facility, he developed acute respiratory failure requiring emergent intubation and single lung ventilation. The availability of multiple subspecialists allowed for stabilization of a critically ill child, expedited diagnosis, and ultimately initiation of life-saving treatment with beta blockers. After 17 total hospital days, he was extubated successfully and discharged home in good condition.ConclusionsWhile IH is a rare cause of infantile respiratory distress, we present multiple pearls for the general pediatrician for management of IHs of the airway.

Management of Upper Airway Infantile Hemangiomas: Experience of One Italian Multidisciplinary Center.

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.

Clinical Practice Guideline for the Management of Infantile Hemangiomas.

Infantile hemangiomas (IHs) occur in as many as 5% of infants, making them the most common benign tumor of infancy. Most IHs are small, innocuous, self-resolving, and require no treatment. However, because of their size or location, a significant minority of IHs are potentially problematic. These include IHs that may cause permanent scarring and disfigurement (eg, facial IHs), hepatic or airway IHs, and IHs with the potential for functional impairment (eg, periorbital IHs), ulceration (that may cause pain or scarring), and associated underlying abnormalities (eg, intracranial and aortic arch vascular abnormalities accompanying a large facial IH). This clinical practice guideline for the management of IHs emphasizes several key concepts. It defines those IHs that are potentially higher risk and should prompt concern, and emphasizes increased vigilance, consideration of active treatment and, when appropriate, specialty consultation. It discusses the specific growth characteristics of IHs, that is, that the most rapid and significant growth occurs between 1 and 3 months of age and that growth is completed by 5 months of age in most cases. Because many IHs leave behind permanent skin changes, there is a window of opportunity to treat higher-risk IHs and optimize outcomes. Early intervention and/or referral (ideally by 1 month of age) is recommended for infants who have potentially problematic IHs. When systemic treatment is indicated, propranolol is the drug of choice at a dose of 2 to 3 mg/kg per day. Treatment typically is continued for at least 6 months and often is maintained until 12 months of age (occasionally longer). Topical timolol may be used to treat select small, thin, superficial IHs. Surgery and/or laser treatment are most useful for the treatment of residual skin changes after involution and, less commonly, may be considered earlier to treat some IHs.

Analysis of the therapeutic evolution in the management of airway infantile hemangioma.

To analyze the evolution in the management of airway infantile hemangioma (AIH) and to report the results from 3 pediatric tertiary care institutions. A retrospective study of patients with diagnosis of AIH and treated in 3 pediatric tertiary care institutions from 1996 to 2014 was performed. Twenty-three patients with diagnosis of AIH were identified. Mean age at diagnosis was 6 mo (range, 1-27). Single therapy was indicated in 16 patients and 7 patients received combined therapy. Two therapeutic groups were identified: Group A included 14 patients who were treated with steroids, interferon, laser therapy and/or surgery; group B included 9 patients treated with oral propranolol. In group A, oral corticosteroids were used in 9 patients with a good response in 3 cases (no requiring other therapeutic option), the other patients required additional treatment options. Cushing syndrome was observed in 3 patients. One patient died of a fulminant sepsis. Open surgical excision and endoscopic therapy were performed in 11 patients (in 5 of them as a single treatment) with a response rate of 54.5%. Stridor persisted in 2 cases, and one patient died during the clinical course of bronchial aspiration. In group B, oral propranolol was used in 9 patients (in 8 of them as a single treatment) with a response rate of 100%, with an mean treatment duration of 7 mo (range, 5-10); complications were not observed. Our experience and the medical literature support the use of propranolol as a first line of treatment in AIH.

Multicenter Evaluation of the Effectiveness of Systemic Propranolol in the Treatment of Airway Hemangiomas.

To evaluate the effectiveness of systemic propranolol in airway infantile hemangioma (AIH) treatment. Case series with chart review. Patients with AIH treated with propranolol between 2009 and 2012 with at least a 1-year follow-up. Presenting age, treating institution, syndrome presence, presenting AIH stage, endoscopy status, propranolol initiation location/dose/duration, time to stridor resolution, adjunctive medical and surgical therapy, and treatment-associated complications. Twenty-seven patients met inclusion criteria. Average age of diagnosis was 2.3 months (range, 1-5 months). The AIHs were stage 1 in 7 of 27 (26%), stage 2 in 12 of 27 (44%), and stage 3 in 8 of 27 (30%). Propranolol initiation was inpatient in 25 of 27 (93%) and outpatient in 2 of 27 (7%). Propranolol dose was maintained at 2 mg/kg/d in all patients for a minimum of 7 months (range, 7-34 months; median, 15 months). Stridor was eliminated within 24 hours or less of propranolol initiation in 23 of 27 (85%). At diagnosis, staging and propranolol initiation in 11 of 27 (41%) were managed with propranolol alone; the remaining 16 of 27 (59%) also had a steroid injection. The use of adjuvant therapy at the time of propranolol initiation and the size of the AIH were not statistically correlated. Twelve patients had additional treatments after the initiation of propranolol due to recurrence of respiratory symptoms, 1 of 27 (4%) of whom was considered a nonresponder. No complications related to propranolol use were noted. This multisite study of AIH treatment with propranolol demonstrates similar effectiveness to surgical treatment modalities. Propranolol therapy for AIH had no complications, had potentially lower resource utilization, and should be considered a first-line AIH treatment.

Management and Outcome of Airway Infantile Hemangiomas

Objectives: (1) Analyze the data of our series of cases of infantile hemangiomas (IH). (2) Describe the clinical characteristics and contemporary treatment modalities of the disease to general and pediatric otorhinolaryngologists. Methods: The medical records of children diagnosed with airway hemangiomas at Hacettepe University Hospital, Department of Otolaryngology between 2005 and 2013 were reviewed retrospectively. The study included 17 patients with IH of the airway. Four patients (23.5%) were male and 13 (76.5%) were female. All of the patients received medical treatment. Six patients (35%) did not respond to medical treatment and surgical intervention was needed. Five in the surgical group required tracheotomy. Results: All patients with tracheotomies were decanulated and stridor in all of them either disappeared or significantly improved. Endoscopic examinations of all of the patients revealed an open and safe lumen. After beginning propranalol as the medical agent, only one patient needed surgical intervention. None of the patients had any major complications. Conclusions: Medical treatment with propranalol of subglottic hemangioma is the first line choice. If medical treatment options fail, surgical intervention should be considered. Overall purpose should be the patient’s airway safety.

Hemangioma subglótico tratado con propranolol: Una nueva alternativa terapéutica. Caso clínico

Infantile Subglottic Hemangiomas (ISH) are rare and potentially fatal. Many treatments have been described for this condition. Propranolol is a new therapeutic alternative, with successful results in skin and airway infantile hemangiomas. Case Study: The case of an infant with ISH and 90% blockage of his airway is presented. The patient underwent a tracheostomy procedure and then treated with oral propranolol, showing a good response after a month of treatment, presenting 10% obstruction and consequently, ending the tracheostomy early. Conclusion: The discovery of the effect of propranolol in the ISH has revolutionized the management of this condition, displacing steroids and surgery as first-line treatments. This study discusses the current ISH management.

How Airway Venous Malformations Differ From Airway Infantile Hemangiomas

To compare airway infantile hemangiomas (IHs) and venous malformations (VMs) clinically, radiographically, endoscopically, and histologically. Retrospective cohort study. Tertiary care pediatric hospital. The study included patients seen in the Vascular Anomaly Clinic, Seattle Children's Hospital, Seattle, Washington, between 2001 and 2008. All patients with airway vascular anomalies were identified by searching the Vascular Anomaly Quality Improvement Database and hospital discharge data. The data, which were analyzed with descriptive statistics and the Fisher exact test, included presenting age, sex, presenting signs, lesion site, and radiographic, endoscopic, and histologic findings.. Seventeen patients with airway lesions were identified, 6 with VMs and 11 with IHs. Patients with VMs presented at a mean (SD) age of 11.3 (13.7) months (age range, 3-39 months), while those with IHs presented at 3 (1.8) months of age (age range, 1-6 months) (P=.03). The patients with IHs were predominantly female (9 of 11 [81%]), while no sex difference was noted among the patients with VMs (3 of 6 [50%]). All patients with IHs presented with stridor and cutaneous lesions, whereas patients with VMs more often presented with hemoptysis or dysphagia (P=.001). Computed tomographic angiograms demonstrated enhancing endolaryngeal lesions in all IHs, while VMs enhanced poorly. Endoscopically, IHs were transglottic, while VMs were postcricoid or epiglottic (P<.001). Histologically, immunostained lesions showed submucosal lobules of capillaries lined by GLUT-1 (glucose transporter isoform 1)-positive endothelium in IHs, whereas VMs consisted of loosely organized venous channels that lacked GLUT-1 staining. Patients with airway IHs and VMs differ in presenting age and signs, sex, airway lesion location, enhancement on computed tomographic angiograms, and histologic appearance.

Management of airway hemangiomas

Airway infantile hemangiomas can cause life-threatening airway compromise from the first year of life. Diagnosis, treatment protocols and outcome measures are not standardized for this condition, making systematic assessment of treatments and outcomes difficult. This article summarizes the treatment options in use and provides an overview of their benefits and drawbacks. It also emphasizes the need for further investigation in this field and discusses the standardization that is required for such research to proceed in a useful manner. The article is divided into discussions of airway infantile hemangioma in general, medical therapy and surgical therapy. It concludes with predictions about the near future of airway infantile hemangioma research and therapy.

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.

Emerging concepts in airway infantile hemangioma assessment and management

Emerging concepts in airway infantile hemangioma assessment and management

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.

Emerging concepts in airway infantile hemangioma assessment and management

Objective The purpose of this study was to evaluate changes in airway infantile hemangioma treatment. Study Design Retrospective. Subjects Airway hemangioma patients, tertiary pediatric hospital. Methods Data collected included age at diagnosis, evaluation methods, hospitalizations, airway size, and interventions. Patients were divided into group A (1981-1993) and group B (1994-2005) and were analyzed using descriptive statistics, the Fisher exact test, and the Student t test. Results Thirty-two subjects were identified. Nasopharyngoscopy was used more in group B (11/16 [69%]) than group A (4/16 [25%], P = 0.032). CT angiography (3/16 [19%]) and laryngeal distractors (11/16 [69%]) were only used in group B; these techniques showed airway hemangiomas to be “transglottic,” not just “subglottic.” Intralesional steroids alone (3/16 [19%]) and primary hemangioma excision (2/16 [13%]) were new treatments used in group B. Frequent direct laryngoscopies (>six) correlated with tracheotomy (5/32 [16%], P = 0.015). Presenting age (<2 months) impacted treatment more than airway hemangioma size. Conclusions New methods of airway infantile hemangioma assessment changed our concept of airway hemangiomas and their primary treatment.