Retinosis pigmentaria sine pigmento. Inicio con edema macular / Retinitis pigmentosa sine pigmenti. Debut with macular oedema

CASO CLÍNICO: Mujer de 25 años que presenta metamorfopsia y disminución de agudeza visual en ojo izquierdo de un año de evolución. A la exploración se aprecia un edema macular quístico (EMQ) y atenuación vascular bilateral. Se describen las pruebas diagnósticas, así como el diagnóstico diferencial y la respuesta al tratamiento con inhibidores de anhidrasa carbónica. DISCUSIÓN: La retinosis pigmentaria sine pigmento es un subtipo de retinosis pigmentaria atípica, caracterizada por la ausencia de depósitos pigmentarios. La hemeralopía es más leve y la afectación campimétrica y electrorretinográfica es menor. El EMQ constituye una causa importante de pérdida de visión central, y responde al tratamiento con inhibidores de anhidrasa carbónica

CASE REPORT: A 25-year-old woman, with metamorphopsia in her left eye of one year onset. The examination revealed a bilateral cystoid macular oedema (CME) and vascular attenuation. We describe the diagnostic tests, as well as differential diagnosis and treatment response with carbonic anhydrase inhibitors. DISCUSSION: The retinitis pigmentosa sine pigment is a subtype of atypical retinitis pigmentosa characterised by the absence of pigment deposits. The night blindness is milder, and perimetric and electroretinographic impairment is lower. CME is an important cause of central vision loss, and responds to anhydrase carbonic inhibitors

Retinitis pigmentosa sine pigmenti. Debut with macular oedema.

CASE REPORT: A 25-year-old woman, with metamorphopsia in her left eye of one year onset. The examination revealed a bilateral cystoid macular oedema (CME) and vascular attenuation. We describe the diagnostic tests, as well as differential diagnosis and treatment response with carbonic anhydrase inhibitors. DISCUSSION: The retinitis pigmentosa sine pigment is a subtype of atypical retinitis pigmentosa characterised by the absence of pigment deposits. The night blindness is milder, and perimetric and electroretinographic impairment is lower. CME is an important cause of central vision loss, and responds to anhydrase carbonic inhibitors.

Bilateral choroidal metastases as presentation of dissemination of cutaneous malignant melanoma.

Case Report. A 47-year-old man presented with blurred vision in the right eye. Ophthalmoscopic examination showed several placoid, pigmented lesions in the posterior pole and midperiphery of the retina of both eyes. Results. Patient referred a cutaneous malignant melanoma on the back skin removed 6 years ago. A systemic workup revealed multiple metastases in liver and spleen. After an exhaustive study we concluded that it was a dissemination of a cutaneous malignant melanoma with bilateral choroidal metastases, liver and spleen metastases. The patient obtained clinical ocular improvement after palliative chemotherapy, although he died in the following months. Pathological examination of the lesions confirmed the diagnosis of choroidal metastases from a malignant cutaneous melanoma. Conclusions. Monitoring patients who have had cutaneous malignant melanoma is very important, since melanoma metastases may occur even many years after the diagnosis of the primary tumor. Choroidal metastases from cutaneous melanoma are uncommon but we should be aware because their appearance worsens prognosis.

Agudeza visual a largo plazo en pacientes con degeneración macular asociada a la edad tratados con ranibizumab y persistencia de fluido subretiniano / Long-term visual acuity in patients with age-related macular degeneration and persistence of subretinal fluid after treatment with ranibizumab

Objetivo: Analizar la agudeza visual (AV) a largo plazo en pacientes con DMAE tratados con ranibizumab con persistencia de líquido subretiniano después del tratamiento de inducción y/o en los controles sucesivos. Método: Hemos revisado las historias clínicas, tomografías de coherencia óptica (OCT) y angiografías fluoresceínicas de los 216 pacientes tratados con ranibizumab entre enero de 2008 y abril del 2010, seleccionando aquellos que han presentado fluido subretiniano de forma persistente o recurrente a lo largo del seguimiento mínimo de un año. Resultados: Hemos incluido 36 ojos de 34 pacientes; 19 ojos (52,7%) presentaban persistencia y 17 (47,2%) recurrencia de fluido subretiniano a lo largo del seguimiento (media 29,06±9,28 meses).nLa media de inyecciones fue de 7,89 ± 3,2. El espesor macular central (EMC) inicial fue de 330 ± 84μm, a los 3 meses de 265,2 ± 62microm y de 294,5 ± 37μm al final del seguimiento. La AV media inicial fue de 0,3±0,2, a los 3 meses 0,43±0,2 (p<0,05) y al final del seguimiento de 0,41±0,22 (p<0,05). La aparición de hemorragias en las recurrencias se asoció con peor visión final en comparación con los que no las presentaron (p=0,004). Al final del seguimiento18 ojos (50%) continúan en tratamiento con ranibizumab, 16 ojos (44%) se mantienen en observación y 2 pacientes han fallecido. No existen diferencias entre AV y EMC entre ambos grupos. Conclusión: La persistencia o recurrencia de fluido macular subretiniano en pacientes tratados con ranibizumab no disminuye significativamente la ganancia visual obtenida después del tratamiento de inducción, a pesar de la interrupción del mismo durante el seguimiento. La aparición de hemorragias en las recurrencias se asoció con peor AV final(AU)

Objective: To analyse the long-term visual acuity (VA) in patients with age-related macular degeneration (ARMD) treated with ranibizumab, and who had persistent subretinal fluid after the induction therapy and/or in the successive controls. Materials and methods: We reviewed the medical records, optical coherence tomography (OCT) and fluorescein angiograms of 216 patients treated with ranibizumab between January 2008 and April 2010, selecting those who had persistent subretinal fluid or recurrent fluid for at least one year of follow-up. Results: A total of 36 eyes from 34 patients were included, with 19 eyes (52.7%) having persistent, and 17 (47.2%) recurrent subretinal fluid throughout the follow- up (mean 29.06±9.28 months). The average number of injections was 7.89±3.2. The central macular thickness (CMT) at the start of follow-up was 330±84μm, at 3 months 265.2±62micrem, and 294.5±37μm at the end of the follow-up. The initial mean VA was 0.3±0.2, at 3 months 0.43±0.2 (P<.05) and at the final review, 0.41±0.22 (P<.05). Haemorrhages in recurrences were associated with a worse final VA (P=.004). At the end of follow-up, 18 eyes (50%) continued with ranibizumab treatment, 16 eyes (44%) were kept under observation, and 2 patients died. There were no differences between VA and CMT between the groups. Conclusions: The persistence or recurrence of macular subretinal fluid in patients treated with ranibizumab does not significantly reduce the visual gain obtained after induction therapy, despite discontinuation of treatment during follow-up. Haemorrhages in the recurrences were associated with a worse final VA(AU)

[Long-term visual acuity in patients with age-related macular degeneration and persistence of subretinal fluid after treatment with ranibizumab]. / Agudeza visual a largo plazo en pacientes con degeneración macular asociada a la edad tratados con ranibizumab y persistencia de fluido subretiniano.

OBJECTIVE: To analyse the long-term visual acuity (VA) in patients with age-related macular degeneration (ARMD) treated with ranibizumab, and who had persistent subretinal fluid after the induction therapy and/or in the successive controls. MATERIALS AND METHODS: We reviewed the medical records, optical coherence tomography (OCT) and fluorescein angiograms of 216 patients treated with ranibizumab between January 2008 and April 2010, selecting those who had persistent subretinal fluid or recurrent fluid for at least one year of follow-up. RESULTS: A total of 36 eyes from 34 patients were included, with 19 eyes (52.7%) having persistent, and 17 (47.2%) recurrent subretinal fluid throughout the follow- up (mean 29.06±9.28 months). The average number of injections was 7.89±3.2. The central macular thickness (CMT) at the start of follow-up was 330±84µm, at 3 months 265.2±62µm, and 294.5±37µm at the end of the follow-up. The initial mean VA was 0.3±0.2, at 3 months 0.43±0.2 (P<.05) and at the final review, 0.41±0.22 (P<.05). Haemorrhages in recurrences were associated with a worse final VA (P=.004). At the end of follow-up, 18 eyes (50%) continued with ranibizumab treatment, 16 eyes (44%) were kept under observation, and 2 patients died. There were no differences between VA and CMT between the groups. CONCLUSIONS: The persistence or recurrence of macular subretinal fluid in patients treated with ranibizumab does not significantly reduce the visual gain obtained after induction therapy, despite discontinuation of treatment during follow-up. Haemorrhages in the recurrences were associated with a worse final VA.

Histopathological and immunohistochemical characteristics of two canine lipid-rich mammary carcinomas.

Clinicopathological and immunohistochemical findings of two uncommon canine lipid-rich mammary carcinomas are described. The predominant histological feature in both tumours was the presence of at least 80% of cells with intracytoplasmic vacuoles which stained positively with Sudan IV but not with alcian-blue periodic acid-schiff method. In both tumours, small groups of non-vacuolated cells were identified among the vacuolated cells. However, histological and immunohistochemical differences were also found between these tumours. Thus, one of them was composed of tumour cells with a large and single vacuole, which were arranged in lobular pattern, while the other neoplasm showed an intraductal growth of tumour cells with a fine vacuolated cytoplasm. Immunohistochemically, in the first tumour most vacuolated cells were positive for CK (cytokeratin)8-7, indicating a secretory epithelial immunophenotype while CK5 and CK8-7-expressing non-vacuolated cells were associated with luminal duct immunophenotype. However, in the second tumour the expression of CK14 in most of vacuolated cells and alpha-smooth muscle actin (alpha-SMA) in non-vacuolated cells, alone or in combination with CK5 suggested a myoepithelial immunophenotype for both cell types. These results suggest heterogeneity of the cell type and growth pattern for this type of canine tumour as has been described in women but not in dogs.