The naso-ocular cleft(AACPR classification) is an extremely rare congenital anomaly and may be accompanied by facial disfigures and deformity of digits. Historically, 5 cases of coloboma exteding into facial cleft were recorded by Warren B. Davis(1935). Since then, sporadic cases have been reported in the literature but true nature of this condition was not fully recognized and very little information is available to establish any statistical conclusion.
Recently Tessier(1973) proposed a rather detailed classification. The cleft are numbered from 0 to 14 and extended along constant lines or axis through the eyebrows or eyelids, the maxilla, the nostril and the lips. According to Tessier classification, this patient is campatible with No. 3 cleft. This cleft has been categorized as a naso-ocular cleft(AACPR), a Group-A para-axial cleft(Karfik, 1966), a nasomaxillary cleft(Gunter), an oblique facial cleft(Sakurai et al, 1966).
No. 3 cleft can be the result of lack of fusion, insufficient mesodermal penetration, or the failure of the naso-optic groove to invaginate and form the tubular nasolacrimal system. The mildest expression is represented by a coloboma of the nasal ala. The vertical distance between the ala base and the medial canthus is shortened. The medial canthal tendon is hypoplastic and it’s insertion is inferiorly displaced.
Using the reported cases that cleft occures in nearly equal frequency between sexes. The side of involvment is also approximately equal with a third occuring on the right, a third occuring on the left, and a third bilaterally. We observed a patient of 6 years old boy, who has a coloboma on right ala nasi, palpable groove on right nasolacrimal groove and shortened distance between ala nasi and right medial canthus. The coloboma was corrected by composite graft from ipsilateral auricle and now preparing for 2nd operation.
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