The Ram Sign: Detecting Previously Undiagnosed Congenital Laryngeal Clefts in Adults

Abstract

Objectives We present the clinical characteristics of a case series of adult patients with type III laryngeal clefts according to the classification of Benjamin and Inglis, in an attempt to make practitioners aware of the “ram sign,” a clinical finding associated with laryngeal clefts in adults. Laryngeal clefts are uncommon defects that are nearly universally identified during infancy as a result of persistent aspiration and pneumonia. Undiagnosed laryngeal clefts in adults are extremely rare. Methods Three type III laryngeal clefts were identified in adults (29, 48, and 60 years of age) from one clinic over an 18-month period. The existing literature features only one type III cleft, to our knowledge. The 60-year-old patient represents the oldest person in the English-language literature to have a newly diagnosed laryngeal cleft. All three cases presented with various degrees of aspiration over an extended period. Results The computed tomographic imaging and endoscopic findings from these three patients were reviewed. The videolaryngoscopic images demonstrated that the “ram sign” — an endoscopic finding associated with redundant soft tissue overlying the arytenoid cartilages that prolapses into the cleft, creating the appearance of ram's horns — was a consistent and striking feature in all three patients. Conclusions Although rare, laryngeal clefts may represent an underdiagnosed entity in the adult population. With increased awareness, they may be identified more frequently as a treatable cause of aspiration and recurrent pneumonia.