Aural Stenosis with External Auditory Canal Cholesteatoma in Klippel-Trenaunay Syndrome

Abstract Klippel-Trenaunay Syndrome (KTS) is a complex congenital disorder characterized by the triad of capillary malformation (port-wine stain), venous malformation, and limb overgrowth, with or without lymphatic malformation. A 21-year-old male presented to the outpatient department with complaints of left ear pain for one week and left ear discharge for one day. The patient had a history of oral cavity swelling that progressed over two years. He reported no history of headache, giddiness, reduced hearing, tinnitus, nasal discharge, nasal obstruction, or throat pain. He was not a known case of diabetes, hypertension, tuberculosis, or bronchial asthma. Klippel-Trenaunay Syndrome is a rare congenital disorder with diverse manifestations that may mimic other conditions such as Parkes-Weber Syndrome. This case underscores the importance of distinguishing between the two, emphasizing the absence of arteriovenous shunting in KTS. The presence of external auditory canal cholesteatoma in KTS is exceedingly rare and demonstrates the need for comprehensive diagnostic and therapeutic strategies.