The Role of Propranolol in the Treatment of Subglottic Hemangioma: Case Report and Review of the Literature

Case Report

Austin J Otolaryngol. 2015;2(2): 1029.

The Role of Propranolol in the Treatment of Subglottic Hemangioma: Case Report and Review of the Literature

Elaine Odiase, Gopi Shah and Eric Berg*

Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center, USA

*Corresponding author: Eric Berg, Department of Otolaryngology-Head and Neck Surgery, University of Texas Southwestern Medical Center and Children’s Health of Dallas, 2350 Stemmons Freeway, ENT F6.218, Dallas, TX 75207, USA

Received: November 03, 2015; Accepted: February 16, 2015; Published: February 18, 2015

Case Report

A full-term 36 day old female was brought to the emergency room by her mother who reported 6 days of progressive stridor. Patient had been previously seen by the primary care physician who also noted the noisy breathing and elected close follow-up. In the interim the patient’s mother noted that patient was choking more, having decreased oral intake, and decreased wet diapers. Mom also noted some purple discoloration to the lips when she observed her child having difficulty breathing. Mom denied other symptoms of upper respiratory illness or sweating during feeds, but did mention that the child’s noisy breathing was most notable when crying or agitated and improved when placed prone or over mother’s shoulder.

During Otolaryngology consultation, fiberoptic laryngoscopy was concerning for laryngomalacia. The subglottis was not clearly visualized. The patient was then admitted to the hospital for continued observation where symptoms progressed with desaturations, increased work of breathing with retractions, nasal flaring, and a “barking” cough despite IV steroids and multiple doses of racemic epinephrine. She was then transferred to the intensive care unit and operative laryngoscopy and bronchoscopy were performed for complete airway assessment. The subglottis was found to be 90% obstructed by a soft, vascular appearing submucosal lesion consistent with a hemangioma. With a single isolated lesion, further diagnostic imaging studies were not indicated and corticosteroids were injected intralesionally and propranolol therapy was initiated. Propranolol was titrated to a weight-based dosage of 2 mg/kg/day divided three times daily and continued to approximately 18 months of age. It was well tolerated without significant adverse events.

Questions

Discussion

Hemangiomas are congenital vascular neoplasms generally diagnosed in infancy [1]. They typically present as cutaneous lesions, but can be found in the upper airway, the liver, and most areas of the body. Typically, they present a few weeks after birth and grow quickly during their proliferative phase for anywhere from 6-12 months. This is subsequently followed by spontaneous involution of the lesion around 12-18 months of age. Complete resolution is generally seen by age 3-7 years.

Although benign, hemangiomas occurring in the larynx can be far from safe. Subglottic hemangiomas can lead to signs of respiratory distress and even complete airway obstruction or even death [2]. The diagnosis is made and/or confirmed by microlaryngoscopy and bronchoscopy with the purple vascular lesion noted in the airway along the lateral or posterior aspects of the airway [3]. Diagnosis is generally based on clinical presentation and appearance alone. Imaging studies may be considered for lesions which have an unusual appearance or atypical clinical history. In cases of multiple cutaneous lesions, liver ultrasound may be considered to rule out undetected internal lesions.

These vascular lesions are seen in for 4 to 5 percent of the pediatric population. [1,4] Although hemangiomas account for the most common head and neck tumors found in children, subglottic hemangiomas are rare, accounting for only 1.5% of congenital laryngeal lesions [4,2].

50% of patients with subglottic hemangiomas also have cutaneous hemangiomas, more often in a “beard” distribution across the preauricular, perioral, and anterior cervical skin [2]. When large, plaque-like lesions are present in the head and neck, PHACES association (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe) should be considered. If cerebrovascular malformations are found to be present, propranolol may be contraindicated due to the potential risk of cerebrovascular accident. Subglottic hemangiomas are seen in females twice more frequently than in their male counterparts. In addition, premature neonates and babies with low birth weights are more likely to suffer from these tumors [4].

Citation: Odiase E, Shah G and Berg E. The Role of Propranolol in the Treatment of Subglottic Hemangioma: Case Report and Review of the Literature. Austin J Otolaryngol. 2015;2(2): 1029. ISSN :2473-0645