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Bilateral Lacrimal Mucoceles in a Setting of Congenital Arhinia Mohammad Javed Ali, M.D., F.R.C.S. Dacryology Service, L.V. Prasad Eye Institute, Hyderabad, India (E-mail: [email protected])
FIG. 1. Congenital arhinia with its lacrimal and ocular associations.
C
ongenital 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.
Accepted for publication July 3, 2014 The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mohammad Javed Ali, M.D., F.R.C.S., Dacryology Service, L.V. Prasad Eye Institute, Road No 2, Banjara Hills, Hyderabad, Andhra Pradesh 500028, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000284
Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
e167
Bilateral Lacrimal Mucoceles in a Setting of Congenital Arhinia Mohammad Javed Ali, M.D., F.R.C.S. Dacryology Service, L.V. Prasad Eye Institute, Hyderabad, India (E-mail: [email protected])
FIG. 1. Congenital arhinia with its lacrimal and ocular associations.
C
ongenital 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.
Accepted for publication July 3, 2014 The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mohammad Javed Ali, M.D., F.R.C.S., Dacryology Service, L.V. Prasad Eye Institute, Road No 2, Banjara Hills, Hyderabad, Andhra Pradesh 500028, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000284
Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
e167
