ENT E-Update
February 2012

  In This Issue 

The Use of Propanolol in the Management of Head and Neck Hemangiomas
About Dr. Discolo

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March: Comprehensive Management of Sinonasal CSF Leaks

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Dear Colleague,   

 

In February's MUSC ENT E-Update, Christopher M. Discolo, M.D. discusses management of infantile hemangiomas with the use of propranolol, which has the potential to be the most effective treatment option to date.

 

Dr. Discolo specializes in Pediatric Otolaryngology, and is the Medical Director of the MUSC Craniofacial Anomalies and Cleft Palate Team. He is also co-directing the Charleston Sleep Surgery Symposium to be held February 24 - 25.

 

Please feel free to contact us with your feedback or questions about our

E-Update articles, your patients, or any ENT issues at ent update@musc.edu.

 

 

Paul R. Lambert, MD

Professor and Chair

The Use of Propranolol in the Management of Head and Neck Hemangiomas


Infantile hemangioma is the most common tumor of infancy. It is estimated that up to 10% of children develop hemangiomas. Babies born prematurely are at increased risk for developing hemangiomas and risk increases with decreasing birth weight. It is imperative that these lesions be differentiated from other vascular tumors (eg, hemangioendotheliomas) as well as from vascular malformations (eg, lymphatic malformation, venous malformation, etc) as treatment options and prognosis varies greatly amongst these conditions.

Most infantile hemangiomas are not present at birth and develop over the first few weeks of life. Occasionally, only a small telangiectatic patch is noticeable at birth which will eventually develop into a hemangioma. There is a predilection for the head and neck region with approximately 60% of all hemangiomas being located in this region of the body. Lesions can either be discrete or diffuse in nature. Children can have multiple discrete lesions in up to 20% of cases. Diffuse lesions are most commonly localized to the mandibular region ('beard distribution') and, in these patients, there is significantly increased risk of a subglottic hemangioma. Children may also have extracutaneous hemangiomas located in the brain, GI tract or liver and this possibility must be considered when multiple discrete or diffuse lesions are present.

 

Hemangiomas typically follow a predictable growth pattern. These tumors undergo a rapid phase of growth during the first four to six months of life however, in some children, this process can take upwards of one year to complete. During this time, endothelial cell proliferation is taking place and lesions can grow significantly. Following this, a period of involution occurs in which tumor cells are replaced by fibrofatty tissue. Clinically, hemangiomas often regress during this phase. It is estimated that 60% of hemangiomas can be observed and will have acceptable functional and cosmetic outcomes without the need for any treatment. However, hemangiomas located in or near vital structures such as the eye or airway can cause significant functional issues during the proliferative phase. Because of this, several treatments have been utilized over the years. Both medical and surgical options exist and these include pulsed-dye laser (PDL) therapy, corticosteroids (both systemic and intralesional delivery), interferon therapy, chemotherapy and surgical excision.

 

More recently, the use of propranolol to treat hemangiomas has received significant attention. Initial reports revealed children on propranolol for cardiac indications had regression of their hemangiomas, some of which were steroid resistant. Following this discovery, a significant amount of research and literature has been produced on this topic.

Case Report
A two month old girl was transferred to the Pediatric Intensive Care Unit at the Medical University of South Carolina for suspected subglottic hemangioma. She had a history of a diffuse cervico-facial hemangioma noted shortly after birth. She then developed progressive stridor and difficulty feeding over the course of a few weeks time. Outside airway examination was concerning for subglottic involvement. The patient was transferred on high-dose intravenous steroids without improvement in her symptoms. She was taken to the operating room shortly after arrival for an airway evaluation (See Figure 1A).
Figure 1a
Figure 1a
A soft compressible vascular mass was noted along the posterior subglottic region consistent with hemangioma. A 2.5 uncuffed endotracheal tube could be placed through this area however there was no air leak. She was started on propranolol at 2 mg/kg/day divided into three doses. Within 48 hours after initiation of propranolol therapy, she was noted to have noticeable improvement in her breathing. Steroids were weaned and discontinued. Her airway continued to improve as did feedings. She was discharged to home approximately 1 week after admission with no stridor and tolerating full feedings.
Figure 1b
Figure 1b
Her follow up airway evaluation 2 months later revealed dramatic improvement in her airway (See Figure 1B). She received no other medical treatments or airway interventions after starting her propranolol therapy. She remained stable on her propranolol with no adverse sequelae or recurrence of symptoms. Of note, her cervico-facial lesion also regressed significantly while on therapy.

Propranolol is a β-adrenergic antagonist and can be used in both adults and children. There are several theories as to the mechanism of action of propranolol in the setting of hemangioma treatment however, none have been definitively proven. Interestingly, propranolol has shown efficacy not just during the proliferative phase but also during the period of involution and can be considered for treatment almost at any time during the natural history of the hemangioma. Unlike the other drugs used to treat hemangiomas, propranolol has a favorable side effect profile and is considered safe for long term usage.

 

Currently most centers use propranolol dosed at 1-3 mg/kg/day divided into three times daily dosing. Generally a brief period of dose escalation is used to ensure that children will tolerate the medication prior to using the full recommended dose. Because propranolol has the potential for cardiovascular effects, a thorough history and physical exam is warranted prior to initiation of therapy. Obtaining an electrocardiogram (ECG) to rule out a cardiac conduction abnormality is indicated prior to starting therapy. If there is any history of cardiovascular or pulmonary disease, most centers would recommend a formal consultation with Pediatric Cardiology for medical clearance to undergo therapy. Consideration can also be given to overnight observation in the hospital at the initiation of therapy although much of the current literature describes purely outpatient therapy with only a brief period of in-office observation being used. Duration of therapy is somewhat arbitrary but most suggest continuing therapy at least until the lesion would be expected to be within the involution phase. Fortunately, most evidence currently suggests that propranolol can be restarted with similar efficacy if lesions start to grow once therapy is discontinued. It is recommended to taper the medication when therapy is finished so as to avoid any rebound effects. Side effects are generally well tolerated and do not result in discontinuation of therapy in the majority of cases. One of the most concerning of these is hypoglycemia, especially in young infants. This side effect seems to be related to prolonged periods of fasting or other feeding issues. Other side effects reported with this medication in children include fatigue, gastrointestinal upset, sleeping problems and bronchospasm.

 

As with any new treatment, the literature is replete with case series describing a single center's experience using propranolol and there is a general lack of randomized controlled trials at this time. A recent meta-analysis by Peridis, et al. sought to evaluate the role of propranolol for airway hemangiomas. When compared to traditional treatments such as carbon dioxide laser excision or steroids, propranolol was shown to be a superior intervention. Although relatively new, propranolol has the potential to be the most effective treatment to date for the management of hemangiomas. It is gaining recognition as the 'go to' first line therapy and should be added to the treatment armamentarium of all those who care for children afflicted with this condition.
 
 
Christopher M. Discolo, M.D.
Assistant Professor, Otolaryngology - Head & Neck Surgery
About Dr. Discolo...
Christopher M. Discolo, MD   
Christopher M. Discolo, MD

Assistant Professor

Medical Director, Craniofacial  Anomalies

and Cleft Palate Team

 

  MD: State University of New York Health

Science Center at Brooklyn

Residency: Cleveland Clinic

Fellowship: University of Minnesota

Pediatric ENT Associates

Special interest: Pediatric otolaryngology, cleft lip and palate repair, mandible distraction,

head and neck masses


Read more about Dr. Discolo

Medical University of South Carolina Department of Otolaryngology - Head & Neck Surgery

135 Rutledge Avenue, MSC 550, Charleston, SC 29425-5500 | Phone: 843.792.8299 | Website: ENT.musc.edu | � 2012