Lateral Proboscis (Elephant Tusk) with Orofacial Clefts: A Report of a Rare Case

Abstract

Lateral proboscis is a rare congenital anomaly. Lateral proboscis is a rare craniofacial malformation characterized by a rudimentary tubular, nose-like structure occurring in association with a wide spectrum of other anomalies. We presented a seven-month-old girl's lateral proboscis, cleft lip, and palate. Proboscis was excised by an elliptical incision, and the cleft was repaired at the same surgery.

A Case Report on Lateral Proboscis: A Rare Congenital Anomaly

“…As described by Ajike et al (2018), a cleft of the lip may occur concurrently. However, there is no direct explanation on the cleft and the proboscis occurring concurrently because, at this embryonic stage, the formation of various organs of the face occurs simultaneously.…”

“…Clinically, the lateral proboscis is described as a tubular protuberance about 2 to 3 cm long and 1 cm wide. It can happen either unilaterally or bilaterally (Ajike et al, 2018). It is usually attached to the medial canthal area, but other areas of attachment have been documented, including the lateral canthus, the chin, and the root of the nose (Martin et al, 2013).…”

“…There is a spectrum of clinical presentation, from the milder form of olfactory tract agenesis to the more severe malformation of cyclopia and cebocephaly (Schinzel et al, 1984). It can develop as an isolated abnormality or in association with other anomalies such as heminasal aplasia/hypoplasia, mental retardation, callosal agenesis, encephalocele, microphthalmia, holoprosencephaly, and clefts (Ajike et al, 2018). The most common associated condition is a hypoplasia of the ipsilateral nose, and the second most common is coloboma of the iris and cleft of the eyelids (Kirkpatrick, 1970).…”

“…Lateral proboscis is an uncommon congenital disorder identified by the presence of a primitive, tube-like structure that emerges from the medial orbital roof (Ajike et al, 2018). It refers to a primitive nasal structure or appendage that is positioned away from the face's vertical midline (Chauhan and Guruprasad, 2010).…”

“…The incomplete merger of the lateral nasal process with the maxillary process results in the formation of a lateral proboscis (Abou-Elhamd, 2004). The presence of heminasal aplasia is directly attributed to the abnormality in one of these nasal placodes and the corresponding processes (Martin et al, 2013), but some are caused by the presence of supernumerary nasal placodes (Ajike et al, 2018).…”