Exfoliation syndrome in enucleated haemorrhagic and absolute glaucoma

Occurrence of exfoliation syndrome was studied in 172 eyes enucleated for haemorrhagic and absolute glaucoma in 1962-82, using histopathological techniques. Exfoliation was found in 33% of haemorrhagic glaucoma cases and in 22% of absolute glaucoma. In patients over 70, exfoliation was found in 53% of the haemorrhagic cases and in 46% of the absolute glaucoma cases. Capsular glaucoma may provoke haemorrhagic glaucoma and may lead to blindness and enucleation of eye with or without vascular catastrophe.     

Detection of extrascleral extension in uveal melanoma with histopathological correlation

Purpose: Uveal melanoma is the most common primary intraocular malignancy. Extrascleral extension (ESE) is rare, but associated with an increased rate of orbital recurrence and an overall poor prognosis. Clinical studies show low rates when compared with histological studies. Due to the prognostic importance of ESE, we sought to compare our clinical, intraoperative, and histological detection rates.

Design: A retrospective cross-sectional case series.

Methods: A list of eyes enucleated for uveal melanoma was compiled from the admissions records of the London Ocular Oncology Service during the 28-month period, i.e. January 2010–April 2012. The surgical and clinical notes of patients with histopathology proven ESE were reviewed to determine when it was first diagnosed or suspected. The subsequent management of these cases is discussed.

Results: A total of 16 out of 174 (9%) eyes had histologically proven ESE. Eight of 16 cases were detected preoperatively at clinical examination, including the use of ocular ultrasound, 3 of 16 were discovered intra-operatively, and 5 of 16 deemed microscopic ESE, were first detected on histological examination. Seven of 7 (100%) of cases with anterior ESE were detected clinically by slit lamp biomicroscopy, while only 1 out of 9 (11%) of cases with posterior ESE was detected preoperatively with ultrasound.

Conclusions: Slit lamp biomicroscopy is sensitive for detecting anterior ESE. Most posterior ESE is microscopic, but macroscopic posterior ESE may also be missed by B-scan ocular ultrasound. Orbital surgeons should be suspicious of clinically undetected posterior ESE, and consider adjuvant orbital radiotherapy in cases with macroscopic ESE.     

Histopathologic findings in eyes treated with a ruthenium plaque for uveal melanoma

Fifty-six globes that had to be enucleated following ruthenium plaque therapy were examined histopathologically. These eyes account for 10% of all uveal melanomas treated at the University Eye Clinic Essen up until 1985. All but one revealed at least some supposedly viable tumor cells. The most prominent findings within the tumors were tumor cell necrosis, vacuolization and balloon cell degeneration, vascular obstruction and fibrosis of the tumor stroma with accumulation of pigmented macrophages. Tumor necrosis was complete or nearly complete in five cases. Tumor regression correlated with cell type and pigmentary characteristics of the tumor, with epithelioid and heavily pigmented tumor cells being more radiosensitive. Tumor regression was inhomogeneous, possibly due to polyclonality, with tumor cells of varying radiosensitivitiy, or due to patchy areas of vascular obliteration. Among other ocular structures, extensive subretinal gliosis, chorioretinal atrophy and scarring of the sclera within the field of radiation were observed. Scleral necrosis was present in only five cases and was limited to areas in which the tumor had infiltrated the deep scleral layers. The findings described were considered to reflect radiation injury rather than spontaneous tumor regression when compared to 70 control eyes that had been enucleated without prior treatment for uveal melanoma.     

Exfoliation syndrome: prevalence and inheritance in a subisolate of the Finnish population

PURPOSE: To estimate the prevalence of pseudoexfoliation syndrome or exfoliation syndrome (ES) in a cross-sectional study and during a long-term follow-up, and to analyse how ES has been inherited in a large pedigree on an isolated population of K?kar island in southern Finland. METHODS: In a population-based study conducted between 1960 and 1962, a comprehensive ophthalmological examination was performed on 595 subjects (85% of the population). From then until 2002, 965 subjects were examined at least once. A pedigree was constructed for all ES-affected subjects according to genealogical studies. The genetic contribution to ES was investigated in this pedigree by segregation analysis and the heritability of the intraocular pressure (IOP) quantitative trait estimated using SOLAR and SAGE software. RESULTS: In the cross-sectional study, the prevalence of ES was 8.1% for 247 subjects over 50 years of age (males 7%, females 9%) and increased to 18.4% for 70 subjects over 70 years of age (males 13%, females 25%). In addition, two females less than 50 years of age were ES-affected. Between 1960 and 2002, 76 (14.3%) of 530 subjects over 50 years of age had ES [23 males (10%) and 53 females (18%)]. Exfoliation glaucoma (EG) was found more often in males (11 patients, 48%) than in females (13 patients, 25%) whereas primary open-angle glaucoma (POAG) was almost as frequent in males (seven patients, 3%) as in females (five patients, 2%). The relative risk (RR) of glaucoma (ES versus no ES) was 11.9 [95% confidence interval (CI) 6.2-22.9] for all the subjects - 14.6 for males (95% CI 6.3-34.0) and 11.8 for females (95% CI 4.4-31.6). Seventy-five pedigrees of 78 ES-affected patients were linked together into one large pedigree with 110 nuclear families. The segregation ratio of ES was 18% (8% for males, 24% for females) when both parents were unaffected, and 16% (9% for males, 27% for females) when at least one parent was affected. The heritability of IOP was estimated to be 30%. CONCLUSION: In this population-based family study, ES is consistent with an autosomal dominant trait with incomplete penetrance, where the penetrance is more reduced in males than in females. However, the presence of ES was a greater risk factor for developing glaucoma in males than in females.     

Clinical and histopathological characteristics of uveal melanoma in Asian Indians. A study of 103 patients

PURPOSE: To study the clinical and histopathological characteristics of uveal melanomas in Asian Indians and compare them with other ethnic groups. MATERIALS AND METHODS: One hundred and three enucleated eyes with clinical diagnosis of uveal melanoma were retrospectively studied (1987-2001) and the clinical and pathological features analysed. RESULTS: Uveal melanomas constituted 0.02% of outpatients in a referral eye hospital in India over a 12-year period. The patients were predominantly males. Uveal melanomas tended to occur in the younger age. The mean age was 45.7 +/- 14.2 years. Melanoma involved the choroid in 90 (87.37%) patients, and both choroid and ciliary body in 13 (12.62%) patients. Mean basal diameter of the tumour was 13.43 +/- 5.32 mm. Mixed cell type was more common. CONCLUSION: Uveal melanomas are rare in the Asian Indians compared to those in the West. They occur in younger persons, have a greater mean basal diameter than that of Caucasians and are predominantly of the mixed cell type.     

Deep optic nerve head morphology and glaucoma progression in eyes with and without laminar dot sign: a longitudinal comparative study

PurposeTo investigate the association between the laminar dot sign (LDS) and the deep optic nerve head (ONH) structure in eyes with primary-open-angle glaucoma (POAG).MethodsEighty-four eyes of 84 patients with POAG were prospectively included. All of the patients underwent stereo optic disc photography (SDP), red-free retinal nerve fibre layer (RNFL) photography, SS-OCT, and standard automated perimetry. By evaluating the SDP, patients were classified into laminar dot sign (LDS) and non-LDS groups. The deep structure of the ONH including the anterior prelaminar depth (APLD) and prelaminar tissue thickness (PTT) were quantitated using SS-OCT. Progression was assessed by structural or functional deterioration during the average 4.3 ± 1.2 years of follow-up.ResultsThe LDS group had deeper APLD (405.47 ± 107.55 vs. 302.45 ± 149.51, P < 0.001) and thinner PTT (74.34 ± 24.46 vs. 137.29 ± 40.07, P = 0.001) relative to the non-LDS group. By multivariate analysis, thin PTT was significantly associated with the presence of LDS (odds ratio = 0.939, P < 0.001). Structural progression was detected in 45 eyes (84.9%) in the LDS group and 8 eyes (25.8%) in the non-LDS group. Functional progression was demonstrated in 29 eyes (34.5%) in the LDS group and 6 eyes (19.4%) in the non-LDS group. The eyes with LDS had a significantly higher risk of glaucoma progression (χ² = 5.00, degree of freedom = 1, P = 0.033).ConclusionsIn eyes with POAG, the presence of LDS was associated with thinner prelaminar tissue and faster disease progression.Subject terms: Optic nerve diseases, Outcomes research, Optic nerve diseases, Outcomes research, Optic nerve diseases     

Peripapillary atrophy in primary angle-closure glaucoma: a comparative study with primary open-angle glaucoma

PURPOSE: To investigate the characteristics of peripapillary atrophy in primary angle-closure glaucoma and to compare peripapillary atrophy in primary angle-closure glaucoma with that in primary open-angle glaucoma. METHODS: Twenty-one eyes (of 21 patients) with chronic primary angle-closure glaucoma that had no evidence of an acute attack or combined-mechanism glaucoma and 31 eyes (31 patients) with primary open-angle glaucoma were enrolled in this study. The cup-to-disk area ratio and the visual field mean deviation were matched in both groups. The prevalence of peripapillary atrophy and peripapillary atrophy-to-disk area ratio of eyes with primary angle-closure glaucoma and eyes with primary open-angle glaucoma were compared. The correlation between the peripapillary atrophy area and the cup-to-disk area ratio or the mean deviation was also evaluated. RESULTS: Eight (38%) of 21 eyes with primary angle-closure glaucoma and 21 (68%) of 31 eyes with primary open-angle glaucoma had peripapillary atrophy. The peripapillary atrophy-to-disk area ratio averaged 0.16 in primary angle-closure glaucoma and 0.41 in primary open-angle glaucoma. There were statistically significant differences regarding the prevalence of peripapillary atrophy (P = .048) and peripapillary atrophy-to-disk area ratio (P = .005) between the two groups. There was no significant correlation between the peripapillary atrophy area and the cup-to-disk area ratio or the mean deviation in primary angle-closure glaucoma. In contrast, the peripapillary atrophy-to-disk area ratio significantly correlated with the cup-to-disk area ratio (P = .003) and the mean deviation (P = .0001) in primary open-angle glaucoma. CONCLUSIONS: The peripapillary atrophy in primary angle-closure glaucoma has a different relationship to the structural and functional optic disk changes than that in primary open-angle glaucoma. Different mechanisms seem to be involved in the development of the optic disk damage in the two types of glaucoma.     

Osteopontin expression and serum levels in metastatic uveal melanoma: a pilot study

PURPOSE: This was a pilot study conducted to examine the expression of osteopontin in uveal melanoma and to determine whether serum osteopontin can be used in detecting metastatic uveal melanoma. METHODS: Osteopontin mRNA was measured in three uveal melanoma cell lines of various invasive potential by real-time PCR. Tissue sections of primary and metastatic uveal melanomas were stained for osteopontin. Serum osteopontin levels were measured by ELISA assays in 15 patients with metastatic uveal melanoma and in 37 patients who were disease-free for at least 10 years after treatment of the primary tumor. Paired serum samples drawn from eight patients before and after development of metastasis were analyzed. RESULTS: By real-time PCR, highly invasive primary and metastatic uveal melanoma cells expressed 6- and 250-fold excess osteopontin mRNA, respectively, compared with poorly invasive primary uveal melanoma cells. Tissue sections of primary uveal melanomas lacking looping vasculogenic mimicry patterns either did not stain for osteopontin or exhibited weak, diffuse staining. In primary melanomas containing looping vasculogenic mimicry patterns, strong osteopontin staining was detected in the tumor periphery where patterns were located. Diffuse strong expression of osteopontin was detected in eight samples of uveal melanomas metastatic to the liver. Serum osteopontin levels were significantly higher in patients with metastatic uveal melanoma than in patients who had been disease free for at least 10 years after treatment (P = 0.0001) or in age-matched control subjects. Serum osteopontin levels were significantly higher (P = 0.008) after metastasis than before the detection of metastasis in eight patients. When a cutoff of 10 ng/mL was used, the sensitivity and specificity of serum osteopontin in detecting metastatic melanoma was 87.5%, and the area under the receiver operator characteristic curve was 96%. CONCLUSIONS: Osteopontin is expressed diffusely in tissue sections of hepatic metastases from uveal melanoma, and increased serum osteopontin levels correlate with melanoma metastasis to the liver with high specificity and sensitivity.     

Senile ectropion and entropion: a comparative histopathological study.

A microscopic histopathological study was done on 500 full-eyelid-thickness surgical specimens: 25 with the diagnosis of senile ectropion and 25 with that of senile entropion. Five different staining techniques were used. There appears to be significantly more orbicularis and Riolan's muscle ischemia, atrophy, and collagen fragmentation with ectropion than with entropion. Entropion shows more septal and tarsal atrophy. In both conditions, the skin and conjunctiva show chronic inflammation and scarring as a constant feature. Statistical significance at the 1% level was present for all six characteristics studied. These histopathological changes, if not etiological, are at least concomitant features differentiating senile ectropion from entropion at the microscopic tissue level.     

Histomorphometry of the optic disc in highly myopic eyes with absolute secondary angle closure glaucoma

AIM—To evaluate histomorphometrically the optic nerve head in highly myopic eyes with absolute secondary angle closure glaucoma.
METHODS—Optic disc sections of 16 highly myopic eyes with an axial length of more than 26 mm and of 19 eyes with an axial length of less than 26 mm were histomorphometrically evaluated. All eyes had been enucleated due to painful absolute secondary angle closure glaucoma.
RESULTS—In the highly myopic eyes compared with the non-highly myopic eyes, mean optic disc diameter was significantly larger (mean 2.33 (SD 0.55) mm versus 1.77 (0.50) mm; p=0.01), and the optic cup was significantly shallower (optic cup depth 0.34 (0.29) mm versus 0.63 (0.23) mm; p=0.03). The peripapillary scleral ring was significantly broader (0.58 (0.65) mm versus 0.08 (0.06) mm; p=0.001), and the β zone (0.83 (0.74) mm versus 0.28 (0.25) mm; p=0.006) of the parapapillary chorioretinal atrophy was significantly larger in the highly myopic eyes.
CONCLUSIONS—The results of the present study agree with biomorphometric data of the optic nerve head in highly myopic eyes with glaucoma. In the highly myopic group, a markedly enlarged peripapillary scleral ring characterised by absence of Bruch's membrane and choriocapillaris contributes in addition to α and β zone to the parapapillary atrophy.

Keywords: optic disc; histomorphometry; myopia; angle closure glaucoma