Аннотация:Congenital arhinia is an extremely rare disorder with less than 35 cases reported since 1931. It is characterized by absence of external nasal structures (Fig. 1A) secondary to maldevelopment of paired nasal placodes during embryogenesis. Ophthalmic associations known include microphthalmia (Fig. 1B), anophthalmia, optic atrophy and uveal colobama. This patient has gross bilateral lacrimal sac mucoceles secondary to maldevelopment of the nasolacrimal duct (Fig. 1A & 1B). The visual acuity recorded was 20/400 OU. Additional features noted include hypertelorism, low set ears and dental abnormalities. The patient is an obligate mouth breather and is scheduled for a multistaged nasal reconstruction. Since the canalicular system was found to be patent on probing, a dacryocystorhinostomy would be performed following the nasal reconstruction.FIG. 1: Congenital arhinia with its lacrimal and ocular associations.
