|Year : 1995 | Volume
| Issue : 1 | Page : 33
Spontaneous vitreous base avulsion in a patient with neurofibromatosis
Uday R Desai
From Henry Ford Hospital, Detroit, Michigan, USA
Uday R Desai
Henry Ford Hospital, 2799 W. Grand Blvd. K-10, Detroit, Michigan 48202
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Desai UR. Spontaneous vitreous base avulsion in a patient with neurofibromatosis. Indian J Ophthalmol 1995;43:33
Neurofibromatosis is a congenital hereditary disorder resulting from displasia of neuroectodermal and mesodermal tissues. The eye and adenexa manifest an unusual assortment of features and scarcely any structure or tissue within or around the eye, except the lens seems immune. However, vitreous base avulsion without any history of trauma is not reported in patients with neurofibromatosis to the best of our knowledge.
| Case report|| |
A 49-year-old white female presented to the retina clinic with a two-month history of floaters in her right eye. Ocular history was non-contributory. There was no history of ocular trauma or surgery. Medical history was positive for neurofibromatosis. She was on no medications. Family history did not reveal any members with retinal abnormalities. She was anisometropic with her right eye refraction being -7.75 + 2.00 X 90 and her left eye refraction being -5.00 + 2.00 X 90. Her visual acuity was 6/9 in the right eye and 6/6 in the left. The left eye revealed Lisch nodules on the iris but was otherwise within normal limits. The right eye similarly exhibited Lisch nodules. It also showed the presence of a vitreous base avulsion on the temporal side [Figure - 1]. Deep choroidal lesions which seemed to be consistent with cobblestone degeneration were also present temporally. There was no evidence of angle recession, iridodialysis, phacodonesis, or choroidal ruptures. The retina did not show any retinal tear. On follow-up examination, the margin of the vitreous base avulsion broke into two segments but otherwise the patient was relatively stable.
| Discussion|| |
Vitreous base avulsion is an entity which is commonly seen in association with blunt ocular trauma. The rapid outward extension of the retina, choroid and sclera is not matched by the relatively inelastic vitreous. Because of this, the vitreous base separates from the underlying retina and pars plana. This is commonly associated with retinal dialysis in the same quadrants. While thought to be pathognomonic for previous blunt trauma, non-traumatic vitreous base avulsion can be seen in young patients with inferotemporal dialysis.
Our patient presented with a vitreous base avulsion with no history of trauma. Her ocular examination also did not reveal any associated findings which may be linked to blunt ocular trauma. Her medical history was significant for neurofibromatosis. Although vitreous base avulsion has not been previously described in patients with neurofibromatosis, there may be an association. To our knowledge, patients with neurofibromatosis have abnormalities in neural cells, neuroglial cells and fibroblasts.
Fibroblastic cells are identified in the cortex of the vitreous base. These fibroblasts are thought to play a role in the formation of collagen fibrils which insert through the basal lamina of the pars plana and peripheral retina. It is the presence of these collagen fibrils which allow the vitreous base to adhere to the underlying tissues. It is possible that suboptimal fibroblastic function in our patient may have resulted in faulty collagen production. This might allow separation of the vitreous base in the absence of blunt ocular trauma. While this is only speculative, it would be interesting to see if others have noted vitreous base abnormalities in patients with neurofibromatosis.
| References|| |
Weidenthal DT, Schepens CL. Peripheral fundus changes associated with ocular contusion. Am J Ophthalmol 62:465, 1966.
Cotran RS, Kumar V, Robbins SL. Genetic disorders in Robbin's pathologic basis of disease. 4th ed. Philadelphia, WB Saunders Co., 1989, pp. 139.
Sebag J. Vitreous biochemistry, morphology and clinical examination. In: Duane's Clinical Ophthalmology, Chapter 38, pp. 1-21. Philadelphia, J.B. Lippincott Company, 1992.
[Figure - 1]