Coexistence of Posterior Staphyloma, Retinitis Pigmentosa and Moderate Myopia.

The authors report a 22-year patient presented with night-blindness and progressive visual loss. Ophthalmologic examination included slit lamp biomicroscopy, best-corrected distance visual acuity, cycloplegic refraction, A-mode and B-mode ultrasonography, and full-field flash electroretinogram (ERG). Cycloplegic refractions were -6.00 (-1.25 x 175) dpt in the right eye and -7.00 (-1.50 x 165) dpt in the left eye. Slit lamp examination showed normal anterior segment. Fundoscopic examination revealed bone-spicule pigmentations, waxy optic disc, arteriolar narrowing and posterior staphyloma, with 5 optic disc in diameter in the right eye and 4 optic disc diameter in the left. Ocular ultrasonography confirmed staphyloma. Full-field ERG showed evidence of a generalized retinal dysfunction involving both rod and cone responses, supported the diagnosis of retinitis pigmentosa.

CLASSIFICATION OF SCLEROCHOROIDAL CALCIFICATION BASED ON ENHANCED DEPTH IMAGING OPTICAL COHERENCE TOMOGRAPHY "MOUNTAIN-LIKE" FEATURES.

PURPOSE: To describe distinct enhanced depth optical coherence tomography patterns of sclerochoroidal calcification and their correlation to clinical features. METHODS: Retrospective chart review of 67 eyes of 46 patients with spectral domain optical coherence tomography imaging. RESULTS: The mean patient age at diagnosis was 68 years. There were 20 (43%) men and 26 (57%) women of white (n = 45, 98%) or Hispanic (n = 1, 2%) heritage. The most prominent sclerochoroidal calcification lesions were located in the superotemporal quadrant (n = 57, 85%) between the temporal arcades and the equator (n = 58, 87%). On enhanced depth optical coherence tomography, the sclerochoroidal calcification was located within the sclera in all cases and the inner surface topography assumed specific "mountain-like" patterns, including flat (Type 1) (n = 9) at median thickness of 1.2 mm, rolling (Type 2) (n = 28) at 1.4 mm thickness, rocky-rolling (Type 3) (n = 21) at 2.1 mm thickness, and table mountain (Type 4) (n = 9) at a thickness of 1.9 mm. The retinal layers were undisturbed in flat lesions, and outer retinal abnormalities were found in all other types. A comparison of the 4 types revealed that Type 3 lesions were thickest (P < 0.001), showing abnormalities in the retinal pigment epithelium, ellipsoid region, and external limiting membrane most commonly (P < 0.05) and demonstrating the most dramatic thinning of the overlying choroid (P < 0.01) and retina (P < 0.05). Type 4 lesions showed greatest basal diameter (P < 0.01) and least outer retinal abnormalities (P < 0.05) or choroid thinning (P < 0.05). CONCLUSION: In this report, enhanced depth optical coherence tomography has demonstrated that sclerochoroidal calcification is a scleral-based disease and can be classified based on four "mountain-like" topographic patterns, associated with variable effects on the choroid and retina.

Prosthetic guidelines for ocular rehabilitation in patients with phthisis bulbi: a treatment-based classification system.

Prosthetic rehabilitation of phthisis bulbi defects is the only treatment option for cosmetic rehabilitation of patients with such defects. Currently, there is no treatment-based classification for prosthetic rehabilitation of patients with phthisis bulbi. Phthisical ocular defects and/or prosthetic rehabilitation were evaluated in an attempt to establish prosthetic guidelines that could be organized into a classification system. Fifty patients who received rehabilitation for phthisis bulbi were reviewed. Phthisis bulbi defects were divided into 4 classes. All the patients had rehabilitation, depending upon the class to which they were assigned. The aim of this treatment-oriented classification system was to organize and define the complex nature of the restorative decision-making process for patients with phthisis bulbi.

Grading of posterior staphyloma with spectral-domain optical coherence tomography and correlation with macular disease.

OBJECTIVE: To develop a novel grading system for posterior staphyloma imaged by spectral-domain optical coherence tomography (SD-OCT) and to correlate the incidence of macular disease (vitreomacular traction, epiretinal membrane, macular schisis, lamellar macular hole, and full-thickness macular hole) with each grade. DESIGN: Retrospective chart review. PARTICIPANTS: A total of 150 eyes from 89 patients with posterior staphylomas were examined at Jules Stein Eye Institute, University of California Los Angeles. METHODS: Retrospective analysis of the Cirrus SD-OCT (Carl Zeiss Meditec, Dublin, Calif.) database at a large institution (Jules Stein Eye Institute) was performed and eyes with a posterior staphyloma involving the macula were evaluated for the presence of macular pathology. The radius of each circle was measured and graded, and the incidence of macular pathology was correlated with each staphyloma grade. Statistical analysis was done using Fisher exact and Kruskal-Wallis tests. RESULTS: The overall incidence rate of macular disease was 50.6% (76/150 eyes). The incidence rate of macular schisis was 17.3% (26/150) and was significantly greater with steeper (grade 3 [25.0%] and grade 4 [30.3%] staphyloma) versus shallower grade (grade 1 [5.6%]) staphyloma (p = 0.016). CONCLUSIONS: Imaging of staphyloma via SD-OCT is a valuable tool for determining the severity of posterior staphyloma and for determining risk stratification for various macular diseases.

Clinical characteristics of posterior staphyloma in eyes with pathologic myopia.

PURPOSE: To determine the morphologic features (grade and type) of posterior staphylomas and to analyze the relationship between the morphologic features and the incidence of myopic macular lesions. DESIGN: Observational case series. METHODS: Two hundred and nine eyes of 108 consecutive patients with high myopia were studied. The grade of staphylomas was determined from B-scan ultrasonographic images across the optic disk. The type of staphyloma was determined by binocular funduscopy and was classified according to the criteria of Curtin. The participants were divided into two groups: younger than 50 years and 50 years and older. The long-term morphologic progression of staphylomas was analyzed in nine patients who were followed up for more than 20 years. RESULTS: Ninety percent of 209 eyes had a staphyloma. The prevalence of staphylomas and more advanced grades of staphylomas (> grade 2) were significantly higher in the older than in the younger patients. The higher grades of staphylomas were associated with more severe myopic retinal degeneration. Type II staphyloma was the most prominent overall; however, in older subjects, the incidence of type II was decreased significantly, and that of type IX was increased significantly. The eyes with type IX staphyloma tended to have more severe myopic retinal degeneration than eyes with type II staphylomas. The long-term follow-up study demonstrated a progression from type II to type IX with increasing age. CONCLUSIONS: These results suggest that the morphologic features of staphylomas worsens as the patient ages. The progression from type II to type IX probably increases the mechanical tension on the macular area of highly myopic eyes, which then leads to myopic fundus lesions.