Amiloidosis vítrea como manifestación primaria de enfermedad: reporte de caso / Vitreous amyloidosis as primary manifestation of disease: case report

Mujer de 35 años con disminución progresiva de visión en ambos ojos sin otros síntomas asociados manifestó como único antecedente la muerte de su hermano por amiloidosis. La agudeza visual fue de cuenta dedos a 30 centímetros en el ojo derecho y 20/70 en el ojo izquierdo. En la oftalmoscopia del ojo derecho se observó un vítreo blanco, homogéneo que no permitió valorar detalles y en el ojo izquierdo un vítreo blanquecino con opacidades en forma de bandas en la región cortical. Se realizó vitrectomía en el ojo derecho y estudio con inmunotinciones que fueron positivos para material amiloide. Publicamos un caso de amiloidosis diagnosticada con tinciones específicas en muestra vítrea cuya manifestación inicial y única fue la presencia de opacidades vítreas. El procesamiento de estas muestras debe considerarse en pacientes con antecedentes familiares, opacidades vítreas tempranas, resultados negativos de biopsia sistémica o curso atípico de la enfermedad

A 35-year-old woman with progressive decrease of vision in both eyes and with no other associated symptoms, manifested as the only antecedent, the death of her brother due to amyloidosis. The visual acuity was counting fingers at 30 centimetres in the right eye and 20/70 in the left eye. In ophthalmoscopy of the right eye, a white, homogeneous vitreous, that was difficult to assess in detail, was observed, and in the left eye a whitish vitreous with band-like opacities in the cortical region. Vitrectomy was performed in the right eye, and a study with specific stains showed positive for amyloid material. A case is reported of amyloidosis diagnosed with specific stains in a vitreous sample for which the initial and only manifestation was the presence of vitreous opacities. The processing of these samples should be considered in patients with family history, early vitreous opacities, negative results of systemic biopsy, or atypical course of the disease

Vitreous amyloidosis as primary manifestation of disease: case report. / Amiloidosis vítrea como manifestación primaria de enfermedad: reporte de caso.

A 35-year-old woman with progressive decrease of vision in both eyes and with no other associated symptoms, manifested as the only antecedent, the death of her brother due to amyloidosis. The visual acuity was counting fingers at 30 centimetres in the right eye and 20/70 in the left eye. In ophthalmoscopy of the right eye, a white, homogeneous vitreous, that was difficult to assess in detail, was observed, and in the left eye a whitish vitreous with band-like opacities in the cortical region. Vitrectomy was performed in the right eye, and a study with specific stains showed positive for amyloid material. A case is reported of amyloidosis diagnosed with specific stains in a vitreous sample for which the initial and only manifestation was the presence of vitreous opacities. The processing of these samples should be considered in patients with family history, early vitreous opacities, negative results of systemic biopsy, or atypical course of the disease.

Estudio comparativo de los hallazgos clínicos en la enfermedad orbitaria asociada a IgG4 con afectación unilateral vs. bilateral / Comparative study of clinical features between unilateral and bilateral orbital IgG4-related disease

OBJETIVO: Análisis descriptivo y comparativo de los pacientes diagnosticados de enfermedad orbitaria asociada a IgG4. MATERIAL Y MÉTODOS: Se revisaron y analizaron todos los casos diagnosticados de inflamación orbitaria asociada a IgG4 por el servicio de Patología del Hospital Dr. Luis Sánchez Bulnes. RESULTADOS: Encontramos un total de 9 casos. El 66% fueron mujeres, con una edad media de 48 años y tiempo medio de evolución al diagnóstico de 2 años. El 56% de los casos presentaron afectación unilateral: el 100% eran mujeres con clínica de dolor al ingreso y una respuesta óptima al tratamiento corticoideo en el 100% de los casos que precisaron tratamiento médico (un caso mostró resolución espontánea). En los casos bilaterales (44%) solo el 25% fueron mujeres y ninguno refirió dolor como síntoma de presentación. Además, el 25% de estos pacientes precisó de la combinación con inmunosupresores para conseguir el control del cuadro. CONCLUSIONES: La presentación clínica de los pacientes con inflamación orbitaria asociada a IgG4 difiere según sea una afectación uni- o bilateral

OBJECTIVE: Descriptive and comparative study of patients with orbital IgG4-related disease. MATERIAL AND METHODS: A review and analysis of the cases diagnosed with inflammatory orbital lesion related to IgG4 by the Ophthalmic Pathology Service in the Dr. Luis Sánchez Bulnes Hospital. RESULTS: A total of 9 cases were found, in which 66% were women, and with a mean age of 48 years and time to diagnosis of 2 years. Unilateral involvement was observed in 56% of cases. All the females experienced pain, and there was an optimal response to corticosteroid treatment in 100% of patients who required medical treatment (one case showed spontaneous resolution). In bilateral cases (44%), only 25% were female, and none had pain as a presenting symptom. Furthermore, 25% of these patients required a combination with immunosuppressants to control inflammation. CONCLUSIONS: Clinical presentation of patients with unilateral orbital IgG4-related disease differs from those with bilateral involvement

[Comparative study of clinical features between unilateral and bilateral orbital IgG4-related disease]. / Estudio comparativo de los hallazgos clínicos en la enfermedad orbitaria asociada a IgG4 con afectación unilateral vs. bilateral.

OBJECTIVE: Descriptive and comparative study of patients with orbital IgG4-related disease. MATERIAL AND METHODS: A review and analysis of the cases diagnosed with inflammatory orbital lesion related to IgG4 by the Ophthalmic Pathology Service in the Dr. Luis Sánchez Bulnes Hospital. RESULTS: A total of 9 cases were found, in which 66% were women, and with a mean age of 48 years and time to diagnosis of 2 years. Unilateral involvement was observed in 56% of cases. All the females experienced pain, and there was an optimal response to corticosteroid treatment in 100% of patients who required medical treatment (one case showed spontaneous resolution). In bilateral cases (44%), only 25% were female, and none had pain as a presenting symptom. Furthermore, 25% of these patients required a combination with immunosuppressants to control inflammation. CONCLUSIONS: Clinical presentation of patients with unilateral orbital IgG4-related disease differs from those with bilateral involvement.

Lesión linfoproliferativa del saco lagrimal: caso clínico / Lacrimal sac lymphoproliferative lesion: Case report

CASO CLÍNICO: Se presenta el caso de una mujer de 51 años con tumoración firme a nivel del canto interno del ojo derecho de 5 años de evolución. La biopsia excisional mediante dacriocistectomía estableció el diagnóstico de lesión linfoproliferativa de bajo grado (hiperplasia linfoide reactiva). DISCUSIÓN: Los tumores del saco lagrimal son muy raros, con un pico de incidencia en la quinta década de la vida. La clínica en fases iniciales es parecida a la obstrucción lagrimal por otras causas, de ahí que hasta un 40% de estos tumores no se sospechen y sean diagnosticados durante la realización de una dacriocistorrinostomía

CASE REPORT: The case is presented of a 51 year-old woman with a firm mass at the medial canthus of the right eye of five years onset. A low-grade lymphoproliferative lesion (reactive lymphoid hyperplasia) was diagnosed from an excisional biopsy. DISCUSSION: Lacrimal sac tumors are rare, with a peak incidence in the fifth decade of life. The initial clinical features are epiphora and medial canthus swelling. As it mimics nasolacrimal duct obstruction, up to 40% of these tumors are misdiagnosed until undergoing surgery

Lacrimal sac lymphoproliferative lesion: case report.

CASE REPORT: The case is presented of a 51 year-old woman with a firm mass at the medial canthus of the right eye of five years onset. A low-grade lymphoproliferative lesion (reactive lymphoid hyperplasia) was diagnosed from an excisional biopsy DISCUSSION: Lacrimal sac tumors are rare, with a peak incidence in the fifth decade of life. The initial clinical features are epiphora and medial canthus swelling. As it mimics nasolacrimal duct obstruction, up to 40% of these tumors are misdiagnosed until undergoing surgery.

Tumores del saco lagrimal que se presentan como obstrucción de la vía lagrimal. Estudio retrospectivo en la población mexicana durante 2007-2012 / Lacrimal sac tumors presenting as lacrimal obstruction. Retrospective study in mexican patients 2007-2012

OBJETIVO: Conocer las características demográficas y clínicas de los tumores primarios del saco lagrimal, que debutan clínicamente como obstrucción de la vía lagrimal. MÉTODOS: Estudio retrospectivo y descriptivo de los pacientes operados por obstrucción baja de la vía lagrimal en el Hospital Dr. Luis Sánchez Bulnes, Asociación para evitar la Ceguera en México I AP, entre los años 2007-2012. RESULTADOS: Los tumores primarios del saco lagrimal representaron el 2,5% de todas nuestras obstrucciones lagrimales, siendo más frecuentes en mujeres (8:1). El 89% fueron de estirpe no epitelial, siendo las más frecuentes las lesiones de naturaleza linfoide. Los tumores benignos se presentaron a edades más tempranas (50 años) que los malignos (70 años). El 33% de nuestros casos resultaron hallazgos inesperados durante el procedimiento quirúrgico (100% lesiones benignas).El 55% se correspondían a tumores malignos (1,4% de las obstrucciones), todos ellos lesiones linfoproliferativas. La clínica más frecuente fue tumoración en canto interno asociada o no a epifora. El tiempo de evolución varió en función del grado de agresividad del linfoma (3 meses-10 años). CONCLUSIONES: Los tumores del saco lagrimal son raros pero debemos tenerlos en cuenta ante la clínica de obstrucción de la vía lagrimal para un tratamiento adecuado

OBJECTIVE: To determine the demographic and clinical data of primary tumors of the lacrimal sac presenting as lacrimal obstruction. METHODS: Retrospective and descriptive study was conducted between the years 2007 to 2012 on all patients undergoing surgery for low lacrimal obstruction at Dr. Luis Sanchez Bulnes Hospital, an Association for the prevention of blindness in Mexico IAP. RESULTS: Primary tumors of the lacrimal sac represented 2.5% of all lacrimal obstructions, being more common in women than in men (8:1). The large majority (89%) of the cases were non-epithelial, with lymphoid lesions being the most frequent. Benign tumors were presented at a younger age (50 years old) than malignant (70 years old). One-third (33%) of cases were unexpected findings during surgery (100% benign). Just over half (55%) were malignant tumors (1.4% of obstructions), all of them lymphoproliferative lesions. The most frequent clinical tumor was in the inner edge, either with or without epiphora. The progression time varied according to the degree of aggressiveness of the lymphoma (3 months-10 years). CONCLUSIONS: Lacrimal sac tumors are rare, but they must be taken into account in patients with an unusual clinical presentation of lacrimal obstruction

[Lacrimal sac tumors presenting as lacrimal obstruction. Retrospective study in Mexican patients 2007-2012]. / Tumores del saco lagrimal que se presentan como obstrucción de la vía lagrimal. Estudio retrospectivo en la población mexicana durante 2007-2012.

OBJECTIVE: To determine the demographic and clinical data of primary tumors of the lacrimal sac presenting as lacrimal obstruction. METHODS: Retrospective and descriptive study was conducted between the years 2007 to 2012 on all patients undergoing surgery for low lacrimal obstruction at Dr. Luis Sanchez Bulnes Hospital, an Association for the prevention of blindness in Mexico IAP. RESULTS: Primary tumors of the lacrimal sac represented 2.5% of all lacrimal obstructions, being more common in women than in men (8:1). The large majority (89%) of the cases were non-epithelial, with lymphoid lesions being the most frequent. Benign tumors were presented at a younger age (50 years old) than malignant (70 years old). One-third (33%) of cases were unexpected findings during surgery (100% benign). Just over half (55%) were malignant tumors (1.4% of obstructions), all of them lymphoproliferative lesions. The most frequent clinical tumor was in the inner edge, either with or without epiphora. The progression time varied according to the degree of aggressiveness of the lymphoma (3 months-10 years). CONCLUSIONS: Lacrimal sac tumors are rare, but they must be taken into account in patients with an unusual clinical presentation of lacrimal obstruction.