Congenital Tracheal Stenosis in a Patient with Cleft Lip

Abstract

Congenital tracheal stenosis (CTS) is a rare condition characterised by different patterns of tracheal narrowing. The pathological hallmark is the presence of complete tracheal rings, with or without associated anomalies. We present a case of asymptomatic CTS in a patient with unilateral cleft lip. Tracheal stenosis was discovered incidentally immediately prior to surgery for correction of the cleft lip, when several attempts at intubation proved unsuccessful. A CT scan of the neck and proximal chest demonstrated a localised region of airway narrowing of the distal trachea, in addition to a right sided cleft lip without cleft palate. Management of CTS depends on several factors, with surgery being the definitive form of treatment. We review the pathology of CTS, its relationship with craniofacial defects, the role of radiology and management of this disease entity.