Nanophthalmos and Uveal Effusion

Richard G. Gieser, M.D. Jared S. Nielsen, M.D. Department of Ophthalmology Loyola University Chicago

Nanophthalmos
Small organized eye, with essential normal structure Bilateral inherited condition, usually autosomal recessive,

Associated with the mucopolysaccharidoses, fetal alcohol syndrome, myotonic dystrophy, and achondroplasia
Visual acuity is typically good in youth

Aging nanophthalmic sclera becomes less elastic increasing resistance to vortex venous outflow leading to choroidal congestion, thickening, and even choroidal effusion
Angle-closure glaucoma regularly occurs because of the large lens in the small eye

What is nanophthalmos?

What is nanophthalmos?

Nanophthalmos is a description of extremely hyperopic eyes, +13.00D to +19.00D, of either sex.

What are the characteristics of the eyes?

What are the characteristics of the eyes?

The eyes have small corneas, 10mm horizontally and between 7-10mm vertically. The AP length is about 17mm. The sclera is thick - 2.5mm.

Source: Arq. Bras. Oftalmol. Sept./Oct. 2004;5: 815-818

When does uveal effusion develop?

When does uveal effusion develop?

The first indication of serious ocular difficulty is glaucoma which is usually acute and occurs between the fourth to sixth decades. Uveal effusion usually occurs after a glaucoma procedure. Differential diagnosis includes retinal detachment or tumor. There is no anterior ocular inflammation which is characteristic of Haradas syndrome

What is the treatment?

What is the treatment?

A procedure that could be tried in cases with uveal effusion is the creation of Gass windows. In this procedure a partial thickness flap is made in each quadrant with a full thickness punch of the sclera if the effusion occurs.

Uveal Effusion Syndrome

Choroidal and ciliary body congestion from abnormal trans-scleral outflow. Vision fluctuates with the course of the disease Ciliochoroidal detachment may develop. RPE alterations and exudative retinal detachment may also accompany this disease.

What disease states cause exudative detachment of the choroid and ciliary body?

What disease states cause exudative detachment of the choroid and ciliary body? Exudative detachment of the choroid and ciliar body may occur after postoperative hypotony, scleral buckling procedures, and scleritis.

What individuals are most likely to get the idiopathic uveal effusion syndrome?

What individuals are most likely to get the idiopathic uveal effusion syndrome?

The uveal effusion syndrome usually occurs in healthy middle-aged males and has occurred in children as young as 13. Usually refraction is within 1.5D of emmetropia.

What are the clinical characteristics of this syndrome and what are the findings?

What are the clinical characteristics of this syndrome and what are the findings? The patients typically note a gradual loss of he superior field. The findings include dilated episcleral vessels, blood in Schlemms canal, normal inraocular pressure, afew vitreous cells, a non-rhegmatogenous retinal detachment with shifting fluid. The fluorescein angiographic findings are dramatic in characteristic. There is some delay in perfusion of thee choroid. There is depigmentation of the epithelium and leopard spot clumping of the pigment in the region of the pigment epithelium. There is background choroidal fluorescence in most cases.

What is thought to be the etiology?

What is thought to be the etiology?

It was thought by Dr. Gass that the abnormal sclera does not allow the normal egress of fluid.

What is the treatment?

What is the treatment?

Treatment has been attempted with steroids, antimetabolites, and scleral buckling. This does not work. Dr. Gass suggested making partial thickness scleral flaps in each quadrant near the equator which are 5xmm and then a full thickness opening with a scleral punch. The openings are left unclosed.

References
Brockhurst, RJ. Nanophthalmos with uveal effusion. A new clinical entity. Arch Ophthalmol. 1975;93:1989-1999 Brockhurst, RJ. Vortex vein decompression for nanophthalmic uveal effusion. Arch Ophthalmol. 1980;98:1987-1990. Singh OS, Simmons RJ, Brockhurst RJ, Trempe CL. Nanophthalmos: a perspective on identification and therapy. Ophthalmology. 1982 Sep;89(9):1006-12. Yanoff: Ophthalmology, 2nd ed, 2004 Mosby, St.ouis. 518-9