Abstract
Background
Choroidal abnormality manifesting as a bright patchy lesion under infrared monochromatic light has previously been described in neurofibromatosis type I patients in whom the choroid appears normal under conventional ophthalmoscopic examination or on the fluorescein angiogram. We investigated the correlation between patient age and the number of choroidal abnormalities, as well as the anatomic distribution of choroidal abnormalities in the fundus.
Methods
We examined the fundus of 28 eyes in 14 patients with neurofibromatosis type I. Patients ranged in age from 2 to 38 years and were examined between April 2001 and April 2002 by confocal scanning laser ophthalmoscopy with infrared monochromatic light (780 nm wavelength). We divided the fundus into five regions (one within the retinal vascular arcade and those supero-temporal, infero-temporal, supero-nasal, and infero-nasal to it), and lesions on the border between regions were assigned to the region containing the greater part of the lesion. We studied the total number of choroidal abnormalities and the correlation between the total number and age.
Results
A positive correlation was found between the total number of choroidal abnormalities and age (Spearman rank correlation coefficient, r=0.6209, P=0.0178). There was a significantly greater number of choroidal abnormalities in the arcade region than in the other four regions (ANOVA, P<0.001).
Conclusions
Choroidal abnormalities tend to increase with age and are most often observed within the vascular arcade.
Similar content being viewed by others
References
Aoki S, Barkovich AJ, Nishimura K, Kjos BO, Machida T, Cogen P, Edwards M, Norman D (1989) Neurofibromatosis type 1 and 2: cranial MR findings. Radiology 172:527–534
Boettner EA, Wolter JR (1962) Transmission of the ocular media. Invest Ophthalmol Vis Sci 1:776–783
Bolande RP (1974) Neurocristopathies: a unifying concept of disease arising in neural crest maldevelopment. Hum Pathol 5:409–429
Duffner PK, Cohen ME, Seidel FG, Shucard DW (1989) The significance of MRI abnormalities in children with neurofibromatosis. Neurology 39:373–378
Elsner AE, Burns SA, Weiter JJ, Delori FC (1996) Infrared imaging of sub-retinal structures in the human ocular fundus. Vis Res 36:191–205
Green WR (1986) The choroid. In Spencer WH (ed) Ophthalmic pathology: an atlas and text book, 3rd edn. Saunders, Philadelphia
Griffiths PD, Blaser S, Mukonoweshuro W, Armstrong D, Milo-Manson G, Cheung S (1999) Neurofibromatosis bright objects in children with neurofibromatosis type 1: a proliferative potential? Pediatrics 104:e49
Hogan MJ, Alvarado JA, Weddell JE (1971) Histology of the human eye: an atlas and textbook. Saunders, Philadelphia
Huson SM, Harper PS, Compston DA (1988) Von Recklinghausen neurofibromatosis. A clinical and population study in south-east Wales. Brain 111:1355–1381
Hunson S, Jones D, Beck L (1987) Ophthalmic manifestations of neurofibromatosis. Br J Ophthalmol 71:235–238
Itoh T, Magnaldi S, White RM, Denckla MB, Hofman K, Naidu S, Bryan RN (1994) Neurofibromatosis type 1: the evolution of deep gray and white matter MR abnormalities. Am J Neuroradiol 15:1513–1519
Kissel P, Andre JM, Jacquire A (1981) The neurocristopathies. Masson, New York, pp 223–232
Klein RM, Glassman L (1985) Neurofibromatosis of the choroid. Am J Ophthalmol 99:367–368
Lee WR (2002) Ophthalmic Histopathology, 2nd edn. Springer-Verlag, London, pp 260–261
Nichols JC, Amato JE, Chung SM (2003) Lisch nodule asymmetry in a patient with neurofibromatosis type 1. J Pediatr Ophthalmol Strabismus 40:243–244
Nichols JC, Amato JE, Chung SM (2003) Characteristics of Lisch nodules in patients with neurofibromatosis type 1. J Pediatr Ophthalmol Strabismus 40:293–296
Rajadhyaksha M, Grossman M, Esterowitz D, Webb RH, Anderson RR (1995) In vivo confocal scanning laser microscopy of human skin: melanin provides strong contrast. J Invest Dermatol 104:946–952
Rescaldani C, Nicolini P, Fatigati G, Bottini FG (1998) Clinical application of digital indocyanine green angiography in choroidal neurofibromatosis. Ophthalmologica 212:99–104
Stumpf DA, Alksne JE, Annegers JF (1988) Neurofibromatosis: conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 45:575–578
Salzmann M (1912) The anatomy and histology of the human eyeball in the normal state (translated by EVL Brown). University of Chicago Press, Chicago
Warwar RE, Bullock JD, Shields JA, Eagle RC Jr (1998) Coexistence of 3 tumors of neural crest origin: neurofibroma, meningioma and uveal malignant melanoma. Arch Ophthalmol 116:1241–1243
Yasunari T, Shiraki K, Hattori H, Miki T (2000) Frequency of choroidal abnormalities in neurofibromatosis type 1. Lancet 356:988–992
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Nakakura, S., Shiraki, K., Yasunari, T. et al. Quantification and anatomic distribution of choroidal abnormalities in patients with type I neurofibromatosis. Graefe's Arch Clin Exp Ophthalmo 243, 980–984 (2005). https://doi.org/10.1007/s00417-005-1184-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00417-005-1184-z