Orbital inflammatory disease.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

Enfermedad inflamatoria orbitaria idiopática / Orbital inflammatory disease

La enfermedad inflamatoria orbitaria idiopática (EIOI), comúnmente conocida como pseudotumor orbitario, es una enfermedad inflamatoria de etiología desconocida. Sus síntomas pueden ser muy variables tanto en intensidad, gravedad, formas de presentación o gravedad. Esta heterogeneidad ha condicionado que sea una entidad difícil de definir y clasificar. El pronóstico de la EIOI depende de su localización, presentación e histología. La EIOI suele responder favorablemente a los corticoides sistémicos, sin embargo, este hecho puede hacer que la entidad sea confundida con otras enfermedades que también tienen buena respuesta a corticoides, como la enfermedad relacionada con la IgG4 y las enfermedades linfoproliferativas. Esta controversia ha alzado una polémica entre autores que defienden la realización de biopsia previa al tratamiento en la mayoría de los casos, frente a otros que afirman que la biopsia debe indicarse en lesiones que no responden adecuadamente al tratamiento médico empírico. Si bien los corticoides se sitúan como los protagonistas de la EIOI, los efectos secundarios, las tasas de recidivas y la falta de respuesta de algunos subtipos han permitido el paso a agentes inmunosupresores e inmunomoduladores que ocupan un escalón fundamental en la terapia combinada o ahorradora de corticoides, junto con la radioterapia y la cirugía. El objetivo de esta revisión es actualizar la evidencia sobre el diagnóstico y tratamiento de la EIOI.

Orbital inflammatory disease (OID), commonly known as orbital pseudotumour, is an inflammatory disease of unknown cause. It has different forms of presentation and different degrees of severity. Its variable nature is the main cause for this disease to be misdiagnosed and misclassified. The prognosis of OID depends on the tissues affected and the histology. OID usually responds favourably to systemic steroid treatment. However, empiric steroids may mask other underlying diseases that respond well to this treatment as well, namely, IgG4-related disease or lymphoproliferative disorders. This fact has led to controversy among various authors as some recommend performing a biopsy in most of the cases, whereas others defend that this procedure should only be performed if the patient has not responded to empiric steroid treatment. Although steroids have been the mainstream treatment of OID, the side effects, relapse rates and lack of response in some cases have resulted in them being replaced by immunosuppressive and immunomodulator therapies that currently stand as a key steroid-sparing treatment option, in addition to radiotherapy and surgery. The aim of this review is to update the evidence on the diagnosis and treatment of OID.

Extracción de cuerpo extraño orbitario con ayuda de electroimán ocular externo: instrumentos del pasado tienen cabida en el presente / Extraction of an intraorbital foreign body with the help of an ocular external electromagnet: Instruments of the past still have a place in the present

Los cuerpos extraños intraorbitarios (CEIO) son objetos que se sitúan en la órbita, normalmente metálicos, que pueden llegar a provocar serios daños estructurales y funcionales en el ojo y en el contenido orbitario. Presentamos el caso de un paciente con un CEIO metálico de localización anterior que se extrajo con la ayuda de un electroimán ocular externo tipo Livingston-Mansfield. A pesar de tratarse de un instrumento que podría considerarse «del pasado», sigue teniendo cabida en nuestro medio en determinadas circunstancias

Intraorbital Foreign Bodies (IOFB) are objects, usually of metallic nature, located outside the orbit cavity, and can potentially cause serious damage to ocular and orbital structures. The case is presented of a patient with an anterior metallic IOFB that was extracted with the aid of a Livingston-Mansfield ocular external electromagnet. Despite being an instrument «of the past», we believe that the electromagnet can still be useful in certain circumstances

Extraction of an intraorbital foreign body with the help of an ocular external electromagnet: Instruments of the past still have a place in the present. / Extracción de cuerpo extraño orbitario con ayuda de electroimán ocular externo: instrumentos del pasado tienen cabida en el presente.

Intraorbital Foreign Bodies (IOFB) are objects, usually of metallic nature, located outside the orbit cavity, and can potentially cause serious damage to ocular and orbital structures. The case is presented of a patient with an anterior metallic IOFB that was extracted with the aid of a Livingston-Mansfield ocular external electromagnet. Despite being an instrument «of the past¼, we believe that the electromagnet can still be useful in certain circumstances.

Fotofobia y dolor neuropático en el síndrome de Sudeck / Photophobia and neuropathic pain in Sudecks síndrome

Presentamos a un paciente de 14 años diagnosticado de síndrome de Sudeck secundario a traumatismo no complicado del pie, derivado a nuestro servicio por disminución de la agudeza visual junto con fotofobia y dolor ocular intenso no correlacionado con los hallazgos exploratorios. El síndrome de Sudeck es una enfermedad inflamatoria neuropática idiopática caracterizada por un dolor que es desproporcionado al evento lesivo que lo origina y que puede generalizarse. Recientemente se ha descrito una nueva enfermedad, el dolor ocular neuropático, caracterizado por un dolor exagerado donde los signos clínicos no se correlacionan con el dolor. En nuestro caso al generalizarse el dolor, generó fotofobia y dolor neuropático ocular muy intenso, que creemos que puede ser la causa de la disminución visual que presenta nuestro paciente. Proponemos que el síndrome de Sudeck entre a formar parte del diagnóstico diferencial del dolor ocular neuropático

The case is presented of a 14 year-old patient diagnosed with Sudeck's syndrome secondary to uneventful foot trauma. The patient complained of decreased visual acuity along with photophobia and intense ocular pain not correlated with the exploratory findings. Sudeck's syndrome is an idiopathic neuropathic inflammatory disease characterised by disproportionate pain, unrelated to a previous traumatic event, which can evolve to severe and generalised pain. A new explanation has recently described this as "neuropathic eye pain" for those patients with severe eye pain that do not correlate with clinical signs. In the case presented here, the pain became widespread and led to photophobia and very intense ocular neuropathic pain. It is believed that this was the cause of the visual decrease presented by this patient. It is proposed that the Sudeck syndrome should become part of the differential diagnosis of neuropathic eye pain

Photophobia and neuropathic pain in Sudecks syndrome. / Fotofobia y dolor neuropático en el síndrome de Sudeck.

The case is presented of a 14 year-old patient diagnosed with Sudeck's syndrome secondary to uneventful foot trauma. The patient complained of decreased visual acuity along with photophobia and intense ocular pain not correlated with the exploratory findings. Sudeck's syndrome is an idiopathic neuropathic inflammatory disease characterised by disproportionate pain, unrelated to a previous traumatic event, which can evolve to severe and generalised pain. A new explanation has recently described this as "neuropathic eye pain" for those patients with severe eye pain that do not correlate with clinical signs. In the case presented here, the pain became widespread and led to photophobia and very intense ocular neuropathic pain. It is believed that this was the cause of the visual decrease presented by this patient. It is proposed that the Sudeck syndrome should become part of the differential diagnosis of neuropathic eye pain.

Enfermedad de Best multifocal: la importancia de los test genéticos / Multifocal Best's disease: The importance of genetic tests

Objetivos y métodos: Nuestro objetivo es describir una variante multifocal en la presentación de la enfermedad de Best. Las lesiones en esta enfermedad pueden variar en forma y tamaño, algunas llegan a medir un diámetro de disco y presentan un contorno irregular. Describimos el caso de un varón de 21 años que fue remitido por pérdida visual progresiva. Confirmamos su baja agudeza visual y realizamos un examen completo, donde pudimos apreciar flecks maculares con pigmento amarillento en su periferia. Sospechamos de una enfermedad de Best multifocal y realizamos los test genéticos pertinentes. La enfermedad de Best multifocal es parecida a la forma clásica, aunque suele cursar con electrooculograma normal y sin antecedentes familiares de la misma. Conclusión: La enfermedad de Best multifocal debe ser sospechada en el caso de lesiones múltiples vitelorruptivas cercanas al polo posterior. Las pruebas genéticas constituyen la clave diagnóstica (AU)

Objectives and methods: Our objective is to describe a multifocal vitelliform presentation of Best's disease. The lesions in this disease may vary in size and shape, some may be a disc diameter in size, and often have some irregularity in their contour. The case is described of a 21-year-old male patient referred to our ophthalmology department due to a progressive loss of vision. His poor visual acuity was confirmed, and a complete examination was performed, in which macular flecks were observed, with yellow pigment arranged in oval distribution near their periphery. Due to the suspicion of Best's multifocal disease, genetic tests were performed. Multifocal vitelliform disease with the same features as those in Best's disease occurs most frequently in patients with a normal electro-oculogram (EOG), and a normal family history. Conclusion: Best's multifocal disease must be suspected in case of multiple vitelliruptive lesions close to the posterior pole. Genetic testing is essential for its diagnosis (AU)

Multifocal Best's disease: The importance of genetic tests. / Enfermedad de Best multifocal: la importancia de los test genéticos.

OBJECTIVES AND METHODS: Our objective is to describe a multifocal vitelliform presentation of Best's disease. The lesions in this disease may vary in size and shape, some may be a disc diameter in size, and often have some irregularity in their contour. The case is described of a 21-year-old male patient referred to our ophthalmology department due to a progressive loss of vision. His poor visual acuity was confirmed, and a complete examination was performed, in which macular flecks were observed, with yellow pigment arranged in oval distribution near their periphery. Due to the suspicion of Best's multifocal disease, genetic tests were performed. Multifocal vitelliform disease with the same features as those in Best's disease occurs most frequently in patients with a normal electro-oculogram (EOG), and a normal family history. CONCLUSION: Best's multifocal disease must be suspected in case of multiple vitelliruptive lesions close to the posterior pole. Genetic testing is essential for its diagnosis.