A case report of proboscis lateralis: emphasis on the reconstruction of the lacrimal drainage system.

Abstract

Background: Proboscis lateralis (PL) is a rare congenital malformation of the craniofacial structure. On the basis of 34 reported cases, Boo-Chai developed the first classification system in 1985 based on commonly associated anomalies of the eyes, palate, and lips. Sinonasal deformity is the most prevalent systemic abnormality associated with PL, accounting for 87.9%, and concomitant ocular anomalies account for 44-70%.

Case description: We report a case of PL in a 20-month-old female patient with a mass in the left medial canthal area, and ipsilateral symptomatic epiphora. The removal of the proboscis at 4 months without the reconstruction of the nasolacrimal duct resulted in secondary sequelae that lasted 16 months. A second operation by a multidisciplinary team released the pressure on the lacrimal sac and reconstructed the lacrimal system. External dacryocystorhinostomy (DCR) is performed through the original external incision aided by nasal endoscopic examination. The bony passage between the nasal cavity and the lacrimal sac was reconstructed, and nasal endoscopy revealed a wide opening in the nasal cavity of at least 6 mm. Follow ups ensured a patent nasal airway, without complications.

Conclusions: It is instructive to learn from this case that treatment plans for PL should consider associated ocular anomalies and lacrimal drainage reconstruction, following a comprehensive and multidisciplinary approach.