Akutes Glaukom mit einseitigem akutem Visusverlust

We report the case of a 31-year-old patient who presented with unilateral acute loss of vision, severe headache, and symptoms of an acute angle-closure glaucoma. Surprisingly, a prepapillary uveal melanoma was the underlying reason; that is, a masquerade syndrome was seen. Enucleation and histopathological investigation of that eye were performed within 3 days after first presentation. Acute angle-closure glaucoma as the initial presentation of uveal melanoma is rare. Nevertheless, clinicians should be aware that patients with refractory unilateral angle-closure glaucoma and asymmetrically opaque media may harbor an occult uveal melanoma.     

Uveal melanoma presenting as acute angle-closure glaucoma: report of two cases

PURPOSE: To present two cases of uveal melanoma presenting as acute angle-closure glaucoma. DESIGN: Observational case reports.Assessment and management of two patients with uveal melanoma presenting with acute angle-closure glaucoma. RESULTS: One patient was initially treated with antiglaucoma medications, and a laser peripheral iridotomy was performed. The second patient had opaque media preventing visualization of the posterior segment, but the presence of an occult mass was suspected as the cause of angle closure. In both cases, B-scan ultrasonography revealed the presence of a tumor causing angle-closure glaucoma. The eyes were enucleated, and the diagnosis of uveal malignant melanoma was confirmed by histopathology. CONCLUSION: Although acute angle-closure as the initial presentation of uveal melanoma is rare, the presence of a uveal melanoma should be suspected in eyes unresponsive to medical therapy, especially in unilateral cases in which the view of the posterior segment is obscured.     

Acute angle closure glaucoma secondary to a choroidal melanoma.

PURPOSE: Report a case of acute angle closure glaucoma secondary to a choroidal melanoma. METHODS: Case report of a 75-year-old woman who presented with acute angle closure glaucoma with bilateral narrow angles. After medical management with drops and peripheral laser iridotomies in both eyes, the pressure was controlled and the angles were noted to be open. This allowed safe dilation of the pupils to perform funduscopic examination. A large choroidal melanoma was noted in the eye with the acute angle closure attack. RESULTS: Dilated fundus exam after peripheral laser iridotomies revealed a choroidal melanoma as the cause for the acute angle closure glaucoma. CONCLUSION: It is important to do a thorough eye examination to rule out secondary causes of angle closure, such as a potentially life-threatening tumor, when a patient presents with acute angle closure glaucoma.     

Secondary glaucoma as initial manifestation of uveal melanoma

PurposeSecondary glaucoma can be induced by a variety of local ocular problems. Intraocular tumors may initially present as secondary glaucoma.Methods8 consecutive patients with secondary glaucoma were found to have uveal melanoma. Thorough examination included detailed history, fundus examination with scleral depression, B scan ultrasonography, and CT/MRI scanning techniques.ResultsA single case presented with spontaneous hyphema, two patients presented with secondary glaucoma, extraocular melanoma and metastases, a single case was found to have angle block by an iridociliary ring melanoma and 4 cases presented with neovascular glaucoma. Enucleation was necessary in all 8 cases.ConclusionsGeneral ophthalmologists should be aware of these rare initial manifestations of intraocular tumors as secondary glaucoma. Enucleation would be recommended in most cases of intraocular malignancy manifesting as secondary glaucoma. One should be extremely cautious in doing a penetrating surgery in such cases.     

Das maligne Melanom des Ziliarkörpers Ein diagnostisches Problem

Background. Ciliary body melanoma is often diagnosed too late and therefore has a poor prognosis. The circular infiltration of the iridocorneal angle by malignant cells can lead to secondary glaucoma, often misdiagnosed as pigmentary glaucoma. Material and methods. In a 40-year-old patient the diagnosis “atypical pigmentary glaucoma” was made due to elevated intraocular pressure and pigmentation of the iridocorneal angle; no changes were present in the iris. In a second case pronounced pigmentation of the chamber angle and secondary glaucoma led to the clinical diagnosis of a ring melanoma and resulted in enucleation of the globe. Results. In the first case, increasing pigmentation of the chamber angle over 4 weeks led to the presumed diagnosis of a ring melanoma. Histopathological evaluation confirmed the diagnosis of a ring melanoma of the ciliary body. In the second case histology revealed retinal detachment with intraocular bleeding without intraocular melanoma. The pigmentation in the lower part of the chamber angle was due to hemosiderosis. Conclusion. Diagnosis of ciliary body melanomas may be difficult in some cases due to its similarity to other eye diseases. The differential diagnosis of malignant melanoma of the ciliary body should be considered in cases of unilateral pigmentary glaucoma.     

Proton beam therapy for iris melanoma: a review of 15 cases

AIM: To report results of proton beam therapy for iris melanoma. METHODS: A retrospective case series of 15 patients with nonresectable iris melanomas treated with proton beam therapy between August 1998 and August 2004. The main outcome measures were (1) local tumour control, (2) complications, and (3) eye retention. RESULTS: Of the 15 cases, 11 patients showed documented growth (including two cases of local recurrence following iridocyclectomy) while a further three cases were biopsy-proven melanoma. One patient presented with a newly acquired vascular nodule of the iris associated with angle seeding and glaucoma. Tumour control at mean follow-up of 34 months was 93% (14 of 15 eyes). Common complications included glaucoma in 53% (five patients had glaucoma prior to irradiation), dry eye (27%) and cataract in three patients (20%). Eye retention was possible in 80% (12 cases). CONCLUSION: Proton beam therapy is an effective treatment for cases of nonresectable iris melanoma. The major complications are cataract and glaucoma.     

Intracranial extension of choroidal melanoma via the optic nerve.

A 62-year-old woman was treated with cyclocryotherapy for neovascular glaucoma of uncertain origin. Fifteen months later she was diagnosed as having a choroidal melanoma, which extended extrasclerally to fill the orbit and invaded the optic nerve intracranially to the chiasm. She was treated by orbital exenteration and neurosurgical resection of the intracranial portion of the optic nerve. The patient is alive and clinically free of metastasis five years after the onset of neovascular glaucoma and almost four years after surgical resection. The significance of this highly unusual growth pattern of uveal melanoma is discussed.     

弥漫型脉络膜黑色素瘤的临床病理特点

目的 观察弥漫型脉络膜黑色素瘤临床病理特点。方法 回顾分析119例病理学检查确诊的脉络膜黑色素瘤患者中11例弥漫型脉络膜黑色素瘤患者的临床病理资料。患者中10例因视力丧失或眼痛就诊,1例外地医院诊断Coats病,继发性青光眼和眼球萎缩要求眼球摘除。临床诊断脉络膜肿物或黑色素瘤8例,绝对期青光眼2例,Coats病、眼球萎缩1例。眼球摘除9例,眼球摘除和眶内肿物切除2例。应用Ki-67免疫组织化学染色,检测细胞增生情况。结果 11例弥漫型脉络膜黑色素瘤均为基底广泛的扁平状肿物。肿瘤基底直径12～20 mm,厚度 2～4 mm。混合细胞型9例,上皮样细胞型1例,坏死型1例。瘤细胞侵犯巩膜7例、侵犯眶内3例,继发性青光眼7例。Ki-67阳性细胞7%～13%,平均阳性细胞9%,多分布于肿瘤基底部,上皮样瘤细胞的表达高于梭形瘤细胞。结论 弥漫型脉络膜黑色素瘤具有特殊生长方式,早期临床诊断困难,有些病例容易诊断为其它脉络膜肿物或青光眼。由于肿瘤基底广泛,容易侵犯眶内和发生转移,预后较差。     

Exfoliation syndrome in Nordic countries: a comparative histopathological study of Danish and Finnish eyes with absolute glaucoma and uveal melanoma

PURPOSE: To compare the frequency of exfoliation syndrome (EXS) in Denmark and Finland in eyes enucleated for absolute glaucoma and uveal melanoma and to correlate these results to reported clinical prevalence rates. METHODS: The material consisted of 304 and 39 eyes consecutively removed because of absolute glaucoma and of 240 and 149 eyes enucleated because of uveal melanoma in Denmark and Finland, respectively. Histological sections of all eyes were stained with haematoxylin-eosin and periodic acid-Schiff and examined under a light microscope for EXS. Diagnosis of EXS was made by the consensus of the investigators. RESULTS: In absolute glaucoma eyes, EXS was observed in 12 out of 304 eyes (3.9%, 95% CI 2.1-6.8) in Denmark, and in 16 out of 39 eyes (41%, 95% CI 26-58) in Finland (p < 0.0001). In the melanoma eyes, EXS was noted in two out of 240 eyes (0.8%, 95% CI 0.1-3.0) in Denmark and in nine out of 149 eyes (6.0%, 95% CI 2.8-11) (p < 0.0037) in Finland. CONCLUSIONS: The frequency of EXS was significantly higher in eyes removed for absolute glaucoma and malignant melanoma in Finland than in Denmark. This supports the clinical impression that EXS is rare in Denmark compared to other Nordic countries.     

Ring melanoma of the iris

Clinicopathologic case report of a boy aged 17 with a ring melanoma of the iris with endothelial overgrowth and glaucoma, with reference to the first case of the iridocorneal endothelial syndrome reported from the same laboratory by Rochat & Mulder in 1924. When a melanoma spreads around the globe within the ciliary body, or around the angle of the anterior chamber, or diffusely over the surface of the iris, it is called a ring melanoma (5).     

Iris melanoma in a ten-year-old boy with Familial Atypical Mole-Melanoma (FAM-M) syndrome

A ten-year-old boy was referred with an enlarging amelanotic iris tumor and secondary glaucoma in the left eye. After excisional biopsy it proved histopathologically to be an iris melanoma. The patient also had multiple nevi on his trunk, scalp, and buttocks suggestive of familial atypical mole melanoma (FAM-M) syndrome and confirmed histopathologically. To the authors' knowledge, this is the first report of iris melanoma in association with FAM-M syndrome. The young age at tumor onset is suggestive of a predisposing condition such as FAM-M syndrome. The association of uveal melanoma with FAM-M syndrome is discussed.     

脉络膜黑色素瘤临床病理分析

本文总结了我院１９８５年至１９９１年经病理证实的４０例脉络膜黑色素瘤。分析其临床、超声波、眼底荧光血管造影的特点及病理改变。并对荧光血管造影的病理机制进行初步探讨。眼底荧光血管造影中于肿瘤内的低荧由肿瘤细胞及其内色素、坏死、血窦等所致。而高荧主要由肿瘤内血管和其上脱落的变性的色素上皮细胞所致。     

Diffuse iris melanoma: a report of 25 cases

BACKGROUND: Diffuse iris melanoma is a rare variant of iris melanoma that has a flat growth pattern and often presents as unilateral hyperchromic heterochromia and glaucoma. There have been no large clinical reports of diffuse iris melanoma. DESIGN: Single-center retrospective case series. PARTICIPANTS: Twenty-five consecutive patients with diffuse iris melanoma. METHODS: A review of the clinical features, management, histopathologic analysis, and prognosis was performed. MAIN OUTCOME MEASURES: Clinical features, histopathologic features, management, and prognosis. RESULTS: At the time of diagnosis, the mean patient age was 49 years. The initial complaint was unilateral darker eye color in 13 cases (52%) and blurred vision in 6 (24%). Six patients (24%) were symptom free. On presentation elsewhere, the initial diagnosis was iris melanoma or nevus in 11 cases (44%) and glaucoma in 14 (56%), 8 (32%) of whom had prior laser or surgical treatment for glaucoma. The 25 patients were observed for a mean of 30 months before the diagnosis of melanoma was suspected and referral to our center for evaluation. The mean intraocular pressure at referral was 36 mmHg, and the mean number of clock hours involved by tumor was 11. Heterochromia iridis was present in all 25 cases (100%), corectopia in 23 (92%), and ectropion iridis in 21 (84%). Associated findings included unilateral cataract in 8 (32%), a prominent episcleral (sentinel) vessel in 7 (28%), and anterior chamber inflammation in 5 (20%), and synechiae (anterior or posterior) in none. The tumor was managed by enucleation in 22 cases (88%) and by plaque brachytherapy in 3 (12%). Five cases (20%) were classified as spindle melanoma, 17 (68%) as mixed cell type, and 3 (12%) as epithelioid cell type. Therefore, histopathologic examination showed that 80% of cases contained epithelioid cells. All 22 enucleated cases were found to have tumor invasion in the trabecular meshwork. Tumor invasion of Schlemm's canal was found in 18 (82%), minor pars plicata in 12 (55%), and episclera in 7 cases (28%). Of seven cases with episcleral invasion, four underwent surgical treatment for glaucoma. Excluding one recent case, the patients were observed for a mean of 78 months. Liver metastasis developed in 3 cases (13%). CONCLUSIONS: Diffuse iris melanoma is a serious ocular condition that causes unilateral hyperchromic heterochromia and secondary glaucoma, often leading to a delay in diagnosis. Local invasion of adjacent ocular structures is common, and distant metastasis occurs in 13% of cases at mean follow-up of 78 months.     

Ciliary body melanoma with limited nodular extrascleral extension and diffuse iris-angle infiltration treated by whole anterior segment plaque radiotherapy

Background Primary uveal malignant melanoma of the ciliary body associated with nodular extrascleral extension, diffuse iris-angle infiltration, and secondary glaucoma is usually treated by prompt enucleation. We report a patient with ciliary body melanoma associated with nodular extrascleral extension and diffuse infiltration of the iris and angle treated conservatively because the fellow eye was blind. Methods The clinical features and surgical management of a melanoma of the ciliary body with extrascleral extension and diffuse infiltration of the iris and angle are presented. The tumor was treated with focal I-125 plaque radiotherapy followed by supplemental whole anterior segment I-125 plaque radiotherapy. Results The patient has been followed for over 2.5 years since the initial plaque radiotherapy and over 1.5 years since the supplemental whole anterior segment radiotherapy. His visual acuity is correctable to 20/40 OD and there is no evidence of metastatic disease. His glaucoma is well controlled following trabeculectomy and tube shunt procedure. Conclusion Whole anterior segment plaque radiotherapy for ciliary body melanoma with diffuse iris-angle infiltration provided palliative local tumor control without significant local complications through available follow-up. Supported in part by: a grant from Research to Prevent Blindness, New York, NY to James J. Augsburger, MD; Chairman, University of Cincinnati, Department of Ophthalmology     

Glaucomas Secondary to Intraocular Melanomas

Intraocular melanomas are not common entities but often lead to secondary glaucoma. Intraocular melanomas should be considered in the differential diagnosis of patients with unilateral glaucoma. Occasionally, the workup of unilateral glaucoma leads to a diagnosis of an intraocular melanoma. Intraocular melanomas represent the majority of intraocular tumors. The prevalence of intraocular melanomas as well as associated secondary glaucomas will be reviewed. The workup needed for unilateral glaucoma will be covered in detail. Treatment options for secondary glaucomas include topical and oral medications as well as argon laser trabeculoplasty and transscleral cyclophotocoagulation.     

Melanoma of the iris and pregnancy

Iris melanoma is a malignant melanocytic tumor, making up 1.2%-6.6% of uveal malignant melanomas. The growth of choroidal melanoma during pregnancy is described and a hormonal influence is suggested. We present a case of iris melanoma occurring during pregnancy. A 32-year-old woman, in the 28(th) week of her fifth pregnancy, was referred to the ophthalmologist with a 2-month history of blurred vision and pain in her left eye. The visual symptoms became progressively worse with decreasing vision. Her visual acuity was 20/70 in her left eye, with a temporal, pigmented, prominent iris mass extending into the trabecular meshwork and the corneal endothelium. Elsewhere other pigmented localizations were found in the iris and in the trabecular meshwork. There was secondary glaucoma with intraocular pressure of 36mmHg and C/D=0.9. The right eye was normal. A general physical examination found no pigmented lesions. Chest radiography and hepatic ultrasonography revealed no metastasis. The diagnosis of iris malignant melanoma was made from the diffuse involvement extending into the trabecular meshwork and the advanced secondary glaucoma; an enucleation was performed and histopathological examination confirmed the diagnosis of iris melanoma with involvement of the trabecular meshwork. The patient had no further problems (follow-up of 11 months). From this case report, we discuss the differential diagnosis of iris melanoma and the hormonal influence on its growth.     

Glaucomas secondary to intraocular melanomas

Intraocular melanomas are not common entities but often lead to secondary glaucoma. Intraocular melanomas should be considered in the differential diagnosis of patients with unilateral glaucoma. Occasionally, the workup of unilateral glaucoma leads to a diagnosis of an intraocular melanoma. Intraocular melanomas represent the majority of intraocular tumors. The prevalence of intraocular melanomas as well as associated secondary glaucomas will be reviewed. The workup needed for unilateral glaucoma will be covered in detail. Treatment options for secondary glaucomas include topical and oral medications as well as argon laser trabeculoplasty and transscleral cyclophotocoagulation.     

Choroidal melanoma in ocular melanosis

CASES: We present four cases of ocular melanosis. Choroidal melanoma was detected in all of them. Three eyes had decreased visual acuity and were enucleated because of their large, active tumours. In the fourth case the melanoma was detected in a routine examination and we were able to apply a preserving treatment with I125 brachytherapy. DISCUSSION: Melanosis oculi is often underestimated as a risk factor for uveal melanoma and glaucoma. Ophthalmic surveillance, every 6 or 12 months is important, in patients with ocular melanocytosis for early detection of high risk diseases.     

Borderline spindle cell nevus of the ciliary body as a second primary melanoma--a clinicopathologic case

We present the case of a 22-year-old woman who underwent surgical excision of a malignant melanoma of the skin three years previously and whom we saw for a pigmented tumor of the iridocorneal angle of her right eye associated with end-stage secondary glaucoma. After the clinical diagnosis of ring melanoma, she underwent enucleation of the eye. The pathologic examination showed a lesion predominantly appearing as a nevus with areas of borderline melanoma formation. The main features of this case were the young age of the patient, the predominant nevoid appearance of this diffuse ciliary body tumor with ring configuration, and the fact that it is a second primary melanoma in such a young patient.     

Decreased choroidal thickness in eyes with secondary angle closure glaucoma. An aetiological factor for deep retinal changes in glaucoma?

A decreased count of retinal photoreceptors all over the fundus and a loss of retinal pigment epithelium cells mainly in the parapapillary region have been reported to be associated with glaucoma. This study addressed the question whether this cell loss in the deep retinal layers may be connected with a change of the choroidal thickness in glaucomatous eyes. Histological sections of 12 eyes with secondary angle closure glaucoma due to a malignant melanoma of the ciliary body and 20 eyes with a malignant choroidal melanoma and normal intraocular pressure were histomorphometrically evaluated. Before enucleation the intraocular pressure was significantly higher in the glaucoma group compared with the control group. Thickness of the choroid was measured at 12 locations from the posterior pole to the fundus periphery. The choroid was significantly thinner in the glaucoma group than in the control group. The decreased choroidal thickness was mainly due to a diminished choroidal vessel diameter. The differences were more marked at the optic disc border than in the fundus periphery. The decreased choroidal thickness in the glaucomatous eyes suggests a reduced choroidal perfusion. It fits with the reported lack of autoregulation of the choroidal blood circulation. Considering the diminished choroidal thickness especially in the parapapillary region, it may be one among other factors explaining the changes of the deep retinal layers in eyes with glaucoma. It indicates that thinning of the choroid, besides the chorioretinal atrophy in the parapapillary region, should be added to the panoply of histological changes in glaucoma.