1/39. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.- - - - - - - - - - ranking = 1keywords = anophthalmos (Clic here for more details about this article) |
2/39. Sclerotomy in uveal effusion syndrome.PURPOSE: To report visual and anatomic outcomes after modified scleral surgery in patients with uveal effusion syndrome with retinal and choroidal detachment. methods: In five eyes of four patients with retinal and choroidal detachment and uveal effusion syndrome due to nanophthalmos, we performed pars plana full-thickness unsutured sclerotomies without sclerectomy. RESULTS: In all cases, uveal effusion with choroidal and retinal detachment resolved within 3 weeks, and all patients showed improved vision. The functional and anatomic results remained stable for over 2 years. CONCLUSIONS: Uveal effusion syndrome that is refractory to medical treatment (high-dose systemic corticosteroids) can be managed effectively by pars plana full-thickness unsutured sclerotomy without sclerectomy.- - - - - - - - - - ranking = 0.125keywords = anophthalmos (Clic here for more details about this article) |
3/39. Reconstruction of the lower lid in congenital microphthalmos and anophthalmos.In congenital microphthalmos and anophthalmos, the socket and lids are often underdeveloped. Progressive dilation of the socket often does not increase the horizontal lid aperture or permit the use of a larger prosthesis. The authors present two cases in which a modified Mustarde cheek flap, lined with a tarsal-conjunctival graft, was used to reconstruct and lengthen the lower lid. This procedure results in a larger horizontal lid aperture and permits a larger prosthesis to be contained in the socket.- - - - - - - - - - ranking = 0.625keywords = anophthalmos (Clic here for more details about this article) |
4/39. Cataract surgery with primary posterior chamber intraocular lens implantation in nanophthalmos.A 56-year-old Asian woman with nanophthalmos and cataract in the right eye underwent extracapsular cataract extraction with primary posterior chamber intraocular lens implantation of 31.0 D. Intraoperatively, there was severe vitreous upthrust; however, there was no vitreous loss and the lens could be placed in the posterior chamber. The patient's postoperative course was uneventful except for a moderate degree of coagulum over the lens. Her best-corrected visual acuity was 20/80. The patient required YAG capsulotomy one year later. Her intraocular pressure was under control without any medication. With appropriate precautionary measures, primary posterior chamber intraocular lens implantation can be performed in nanophthalmic eyes with a favorable visual outcome.- - - - - - - - - - ranking = 0.625keywords = anophthalmos (Clic here for more details about this article) |
5/39. Nanophthalmos with uveal effusion. A new clinical entity.Five patients exhibited the association of nanophthalmos and uveal effusion, apparently on a familial basis. glaucoma, occurring in the fourth to sixth decades of life, required surgical intervention that was followed by the development of secondary retinal and choroidal detachment. Recognition of this syndrome is important because: (1) surgical procedures for glaucoma should be avoided, if possible, in order to prevent the development of uveal effusion; (2) retinal detachment surgical procedures are ineffective in uveal effusion and should be avoided, and (3) choroidal elevation occurring in the uveal effusion phase may be erroneously diagnosed as an intraocular tumor and unnecessary enucleation may follow.- - - - - - - - - - ranking = 0.625keywords = anophthalmos (Clic here for more details about this article) |
6/39. genetic heterogeneity of syndromic X-linked recessive microphthalmia-anophthalmia: is Lenz microphthalmia a single disorder?Nonsyndromic congenital microphthalmia or anophthalmia is a heterogeneous malformation with autosomal dominant, autosomal recessive, and X-linked modes of inheritance. Lenz microphthalmia syndrome comprises microphthalmia with mental retardation, malformed ears, skeletal anomalies, and is inherited in an X-linked recessive pattern. Prior studies have shown linkage of both isolated (or nonsyndromic) anophthalmos (ANOP1, [MIM 301590]) and Lenz syndrome [MIM 309800] to Xq27-q28. Nonsyndromic colobomatous microphthalmia [MIM 300345] has been linked to Xp11.4-Xq11.1. We describe a five-generation African-American family with microphthalmia or anophthalmia, mental retardation, and urogenital anomalies, in an X-linked recessive inheritance pattern, consistent with Lenz syndrome. Initial linkage analysis with microsatellite markers excluded the region in Xq27-q28 previously reported as a candidate region for ANOP1 [MIM 301590]. An X-chromosome scan revealed linkage to a 10-cM region between markers DXS228 and DXS992 in Xp11.4-p21.2. Multipoint analysis gave a maximum lod score of 2.46 at marker DXS993. These data show that X-linked recessive syndromic microphthalmia exhibits genetic heterogeneity. In addition, it suggests that Lenz microphthalmia syndrome, previously thought to be a single disorder, may represent an amalgam of two distinct disorders.- - - - - - - - - - ranking = 0.125keywords = anophthalmos (Clic here for more details about this article) |
7/39. The management of glaucoma in nanophthalmos.patients with nanophthalmos are prone to develop a chronic painless type of glaucoma in middle age, probably due to the natural increase in the size of the lens which is already relatively too large for the small eye. Although the underlying mechanism is obscure, a slowly progressive "creeping" chronic angle-closure is postulated, but gonioscopic evaluation is difficult due to the shallow anterior chamber, with grade I and slit angles. Response to medical treatment is poor and miotics may even make the condition worse by producing relative pupillary block and by relaxing the lens zonule. Ordinary glaucoma surgery is to be avoided in nanophthalmos because of the fear of postoperative ciliary-block malignant glaucoma. Periopheral iridectomy performed in five eyes at an advanced stage of the chronic angle-closure did not facilitate glaucoma control in three eyes, and in two eyes in which the operation was combined with posterior sclerotomy, the eyes became blind from vitreous hemorrhage. Lenx extraction in five eyes controlled the glaucoma but was followed by choroidal effusion and nonrhegmatogenous retinal detachements in two eyes and blindness in another eye when combined with a posterior sclerotomy. No firm therapeutic recommendations can be made on the basis of the author's experience in the six reported cases. Conventional medical therapy seems ineffectual even when begun early in the glaucoma. Conventional glaucoma surgery must be performed early, before permanent damage to the outflow mechanism occurs but removal of the lens must be anticipated. The surgeon must also remain aware of the high incidence of serious posterior-segment complications which inexplicably follow glaucoma or lens surgery in nanophthalmos, as described by Brockhurst.- - - - - - - - - - ranking = 0.875keywords = anophthalmos (Clic here for more details about this article) |
8/39. Familial nanophthalmos.Four members of one sibship had microphthalmos with fundi having an irregular "rippled* appearance with alternating light and dark red coloration. fluorescein angiography revealed patchy choriocapillaris perfusion with unusually rapid diffusion into the retina. Genealogic and geographic data strongly suggest autosomal-recessive inheritance. Only one patient developed glaucoma. The unique combination of abnormalities in this family suggests that a new recessive mutation may be responsible.- - - - - - - - - - ranking = 0.5keywords = anophthalmos (Clic here for more details about this article) |
9/39. A case of angle-closure glaucoma, cataract, nanophthalmos and spherophakia in oculo-dento-digital syndrome.BACKGROUND: We report a new case of oculo-dento-digital syndrome. methods: Case report. RESULTS: We saw a 34-year-old women with oculo-dento-digital syndrome. visual acuity was no perception of light (RE) and 1/35 (LE). Biomicroscopy revealed a flat anterior chamber and an advanced cataract. The intraocular pressure (IOP) was 60 mmHg in both eyes. Ultrasonographic biomicroscopy demonstrated a closed chamber angle in both eyes. Measurements of the axial length and of the diameter of the lens were 18.7 mm/6.0 mm for the RE and 18.7 mm/5.8 mm for the LE respectively. In the time following we conducted a cyclodestructive procedure in the RE and a cataract extraction with implantation of an intraocular lens in the LE. This led to a considerable reduction of the IOP in the RE and combined with local therapy to IOP regulation in the LE. CONCLUSION: In this patient nanophthalmos, cataract and spherophakia led to angle-closure glaucoma in both eyes. We recommend early monitoring of IOP, axial length and lens diameter. This case demonstrates that an early cataract extraction might beneficially influence the natural course of the disease.- - - - - - - - - - ranking = 0.625keywords = anophthalmos (Clic here for more details about this article) |
10/39. Angle closure glaucoma in nanophthalmos and pigmentary retinal dystrophy: a rare syndrome.The simultaneous occurrence of nanophthalmos, angle closure glaucoma and pigmentary retinal dystrophy documented in the present case represents a rare syndrome.- - - - - - - - - - ranking = 0.625keywords = anophthalmos (Clic here for more details about this article) |
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