K W Altman1, A J Woodring, J E Pappano. 1. Department of Otorhinolaryngology-Head and Neck Surgery, Hospital of the University of Pennsylvania, Philadelphia 19103, USA.
Abstract
PURPOSE: Hereditary angioedema is a rare disorder of deficient or dysfunctional C1-esterase inhibitor and usually manifests as edema of the face, tongue, supraglottis, extremities, or gastrointestinal tract. We report the case of a 40-year-old man with known hereditary angioedema who presented with a sore throat and a sensation of evolving airway obstruction. After a thorough search of the medical literature, we believe this to be the first reported case of angioedema manifesting in the retropharyngeal space. The pathophysiological factors of angioedema are discussed, along with its variable presentation and management issues. METHOD: Laryngoscopic examination was suggestive of posterior pharyngeal fullness; therefore, a computed tomographic scan of the neck was obtained, which showed a non-contrast-enhancing retropharyngeal edema from the base of the skull to below the level of the glottis. The patient had a history of multiple episodes of angioedema requiring hospitalization and three prior tracheotomies. RESULTS: Familiarity with the patient's history directed his rapid treatment course (including intravenous stanozolol, Solu-Medrol, and diphenhydramine), which significantly reduced his edema and avoided the need for tracheotomy. CONCLUSION: Hereditary angioedema may present in atypical locations, and expeditious treatment in a patient with a known history may avert the sequelae of evolving airway obstruction.
PURPOSE:Hereditary angioedema is a rare disorder of deficient or dysfunctionalC1-esterase inhibitor and usually manifests as edema of the face, tongue, supraglottis, extremities, or gastrointestinal tract. We report the case of a 40-year-old man with known hereditary angioedema who presented with a sore throat and a sensation of evolving airway obstruction. After a thorough search of the medical literature, we believe this to be the first reported case of angioedema manifesting in the retropharyngeal space. The pathophysiological factors of angioedema are discussed, along with its variable presentation and management issues. METHOD: Laryngoscopic examination was suggestive of posterior pharyngeal fullness; therefore, a computed tomographic scan of the neck was obtained, which showed a non-contrast-enhancing retropharyngeal edema from the base of the skull to below the level of the glottis. The patient had a history of multiple episodes of angioedema requiring hospitalization and three prior tracheotomies. RESULTS: Familiarity with the patient's history directed his rapid treatment course (including intravenous stanozolol, Solu-Medrol, and diphenhydramine), which significantly reduced his edema and avoided the need for tracheotomy. CONCLUSION:Hereditary angioedema may present in atypical locations, and expeditious treatment in a patient with a known history may avert the sequelae of evolving airway obstruction.