malformations, including exposure to infections and toxins during pregnancy. Perturbation of these events may result in a wide range of congenital eye malformations. To the best of our knowledge, the association of NF-1 and anophthalmia/microphthalmia is rare. Galofaro and Scoppa 6 repor- ted that microphthalmia was observed in 1 patient with NF-1 caused by a rapidly invasive orbital tumor. Jacquemin et al 7 reported 2 patients with abnormal development of the lesser wing of the sphenoid bone and microphthalmos but without the cutaneous stigmata of NF-1. However, anophthalmia in patients with NF-1 has not been previously reported. In this report, a young woman with NF-1 presents with congenital unilateral anophthalmia without or- bital tumor, according to clinical course and brain CT and MRI. Although the precise etiology of anophthalmia in a patient with NF-1 remains speculative, several explanations are possible. First, the generalized phenotype suggests mutations resulting in NF-1 occurred very early in the patient. 12 As is known, anophthalmia usually occurs at the fourth gestational week, so it is supposed that the develop- mental timing of NF-1 and anophthalmia might overlap. Further- more, NF-1 is one of most vulnerable results of mutation in the human genome 1 ; therefore, we postulate that a new mutation is re- sponsible for the patient’s phenotype of anophthalmia and NF-1. In the future, novel NF-1 mutations in the patient might be detected to add to the phenotype-genotype data on these intriguing conditions. Sec- ond, an NF-1 patient with microphthalmia was found to have mutations in 3 genes that affect eye development, that is, NF-1, OTX2, and PAX6. 13 Consequently, polygenic inheritance early in development is a likely possibility for the patient. Lastly, faulty induction of the neu- roectoderm in the optic primordial, caused by maldevelopment of the sphenoid mesoderm, was regarded as a precipitating factor of micro- phthalmos. Alternatively, a neural crest cell migration anomaly or teratogenic insult may be responsible for the concomitant mal- development of the eye and sphenoid. As a result, anophthalmia in this patient might be induced by local anomalous tissue interactions. However, it is also possible that anophthalmia in this patient is a coincidental finding. Regarding treatment, it would be necessary to remove multi- ple small cutaneous neurofibromas by surgery. In addition, disease management in this patient includes referral to specialists for the treatment of complications involving the eye and orbit. Surgery to repair the skeletal malformation and to fit an ocular prosthesis is warranted. However, the patient refused all aggressive surgery. A careful annual follow-up is obviously required for this patient. In conclusion, we first report an extremely rare case with con- comitant NF-1 and anophthalmia. As is known, NF-1 presents with extremely variable clinical manifestations because of genetic, de- velopmental, and pathophysiologic aspects. This case demonstrates the need to recognize that, on rare occasions, anophthalmia may be an unusual manifestation of NF-1. REFERENCES 1. Lazaro C, Ravella A, Gaona A, et al. Neurofibromatosis type 1 due to germ-line mosaicism in a clinically normal father. N Engl J Med 1994;331:1403Y1407 2. Boyd KP, Korf BR, Theos A. Neurofibromatosis type 1. J Am Acad Dermatol 2009;61:1Y14 3. Shen MH, Harper PS, Upadhyaya M. Molecular genetics of neurofibromatosis type 1 (NF1). J Med Genet 1996;33:2Y17 4. Debella K, Szudek J, Friedman JM. Use of the National Institutes of Health criteria for diagnosis of neurofibromatosis 1 in children. Pediatrics 2000;105:608Y614 5. Beauchamp GR. Neurofibromatosis type 1 in children. Trans Am Ophthalmol Soc 1995;93:445Y472 6. Galofaro A, Scoppa F. Childhood microphthalmic neurofibromatosis. Ital J Neurol Sci 1989;10:523Y525 7. Jacquemin C, Mullaney P, Bosley TM. Abnormal development of the lesser wing of the sphenoid with microphthalmos and microcephaly. Neuroradiology 2001;43:178Y182 8. Hoover-Fong JE, Cai J, Cargile CB, et al. Facial dysgenesis: a novel facial syndrome with chromosome 7 deletion p15.1Y21.1. Am J Med Genet A 2003;117:47Y56 9. Srsen S. Congenital anophthalmos in two siblings. Acta Univ Carol Med Monogr 1973;56:136Y139 10. Bermejo E, Martinez-Frias ML. Congenital eye malformations: clinical-epidemiological analysis of 1,124,654 consecutive births in Spain. Am J Med Genet 1998;75:497Y504 11. Verma AS, Fitzpatrick DR. Anophthalmia and microphthalmia. Orphanet J Rare Dis 2007;2:47 12. Ruggieri M, Pavone P, Polizzi A, et al. Ophthalmological manifestations in segmental neurofibromatosis type 1. Br J Ophthalmol 2004;88:1429Y1433 13. Henderson RA, Williamson K, Cumming S, et al. Inherited PAX6, NF1 and OTX2 mutations in a child with microphthalmia and aniridia. Eur J Hum Genet 2007;15:898Y901 Frontal Sinus Agenesis Using Computed Tomography Seyed Amir Danesh-Sani, DDS,* Reza Bavandi, MD,Þ Maryam Esmaili, BSþ Abstract: In adults, paranasal sinus agenesis is an uncommon anomaly that appears mainly in the frontal sinus. The aim of this study was to investigate the prevalence of agenesis of the frontal sinus using computed tomography in a population of Iranian individuals. Computed tomographic scans in the axial and coronal planes of the FIGURE 2. A, A CT scan of the brain shows left anophthalmia and severe osseous orbital dysplasia. B, An MRI image reveals left anophthalmia and a large protruding frontotemporal encephalocele. C, An enhanced MRI image shows that neither orbital neurofibromas nor intracranial tumors were displaced in the brain. From the *Department of Oral and Maxillofacial Surgery, Dental Research Center, Mashhad Dental Faculty, †Department of Radiology, and ‡De- partment of Oral and Maxillofacial Surgery, Dental Research Center, Mashhad Dental Faculty, Mashhad University of Medical Science, Mashhad, Iran. Received June 7, 2011. Accepted for publication July 16, 2011. Address correspondence and reprint requests to Seyed Amir Danesh-Sani, DDS, Department of Oral and Maxillofacial Surgery, Dental Research Center, Mashhad Dental Faculty, Vakilabad Blvd, 65176-59114, Mashhad, Iran; E-mail: amirds_dds@yahoo.com; daneshh@mums.ac.ir The authors report no conflicts of interest. Copyright * 2011 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0b013e318231e26c Brief Clinical Studies The Journal of Craniofacial Surgery & Volume 22, Number 6, November 2011 e48 * 2011 Mutaz B. Habal, MD Copyright © 2011 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.