Síndromes paraneoplásicos en oftalmología / Paraneoplastic syndromes in ophthalmology

Los síndromes paraneoplásicos consisten en la afectación de órganos y tejidos alejados de un tumor primario, y que no son consecuencia directa de la invasión tumoral ni de sus metástasis. Se sabe que en su fisiopatología desempeñan un papel importante la autoinmunidad y la síntesis de autoanticuerpos debido a un proceso de mimetismo molecular. Los síndromes paraneoplásicos de afectación oftalmológica son una enfermedad poco frecuente, pero es importante reconocerlos clínicamente debido a que en algunas ocasiones los síntomas derivados preceden al diagnóstico de la neoplasia de base. El tumor más frecuentemente relacionado con esta enfermedad es el carcinoma microcítico pulmonar, pero también existe relación con otras etiologías tumorales como el timoma, los tumores ginecológicos o el neuroblastoma en niños. Los síndromes paraneoplásicos de afectación oftalmológica pueden dividirse entre los que afectan a la vía visual aferente, como la retinopatía asociada al cáncer, la retinopatía asociada al melanoma o la neuropatía óptica paraneoplásica; o a la vía visual eferente, como las pupilas tónicas bilaterales, la miastenia gravis, el síndrome de Lambert-Eaton o la degeneración cerebelosa paraneoplásica. Cada vez se conocen más autoanticuerpos relacionados y su positividad es de ayuda en la práctica clínica, pero la negatividad no excluye el diagnóstico. Aunque su evolución y pronóstico va ligado al de la enfermedad causal, en algunos casos el tratamiento específico, habitualmente mediante terapia inmunosupresora puede ayudar a mejorar la calidad de vida de estos pacientes (AU)

Paraneoplastic syndromes consist on systemic manifestations associated with certain cancers which are not a direct consequence of tumor invasion or its metastases. It is known that autoimmunity and autoantibody synthesis play an important role in its pathophysiology due to a process of molecular mimicry. Paraneoplastic syndromes in ophthalmology are rare, but it is important to recognize them clinically because in some cases symptoms preceded the diagnosis of an underlying neoplasia. Most frequently involved cancer is small cell lung carcinoma, but there is also a relationship with other tumor etiologies such as thymoma, gynecological tumors or neuroblastoma in children. Paraneoplastic syndromes with ocular involvement can be divided into those that affect the afferent visual pathway, such as cancer-associated retinopathy, melanoma-associated retinopathy, or paraneoplastic optic neuropathy; and the ones that affect the efferent visual pathway, such as bilateral tonic pupils, Myasthenia Gravis, Lambert-Eaton syndrome or paraneoplastic cerebellar degeneration. The presence of autoantibodies is helpful in clinical practice but negativity does not exclude this diagnosis. Although evolution and prognosis is linked to primary disease, in some cases specific treatment, usually immunosuppressive therapy, can help improving patients quality of life (AU)

Paraneoplastic syndromes in ophthalmology.

Paraneoplastic syndromes consist on systemic manifestations associated with certain cancers which are not a direct consequence of tumor invasion or its metastases. It is known that autoimmunity and autoantibody synthesis play an important role in its pathophysiology due to a process of molecular mimicry. Paraneoplastic syndromes in ophthalmology are rare, but it is important to recognize them clinically because in some cases symptoms preceded the diagnosis of an underlying neoplasia. Most frequently involved cancer is small cell lung carcinoma, but there is also a relationship with other tumor etiologies such as thymoma, gynecological tumors or neuroblastoma in children. Paraneoplastic syndromes with ocular involvement can be divided into those that affect the afferent visual pathway, such as cancer-associated retinopathy, melanoma-associated retinopathy, or paraneoplastic optic neuropathy; and the ones that affect the efferent visual pathway, such as bilateral tonic pupils, Myasthenia Gravis, Lambert-Eaton syndrome or paraneoplastic cerebellar degeneration. The presence of autoantibodies is helpful in clinical practice but negativity does not exclude this diagnosis. Although evolution and prognosis is linked to primary disease, in some cases specific treatment, usually immunosuppressive therapy, can help improving patients quality of life.

Neuropatía óptica compresiva secundaria a reacción alérgica a hialuronidasa / Compressive optic neuropathy secondary to an allergic reaction to hyaluronidase

Una mujer de 58 años presentó quemosis intensa y oftalmoparesia en el ojo izquierdo 8 h después de cirugía de catarata no complicada bajo anestesia subtenoniana. Tras tratamiento corticoideo y antihistamínico, se observó recuperación de la motilidad extrínseca pero se apreciaron un edema de papila no hemorrágico y un defecto concéntrico de campo visual. El caso evolucionó a atrofia papilar con agudeza visual central preservada pero con una contracción significativa del campo visual. El estudio etiológico reveló una alergia a la hialuronidasa, usada como adyuvante a la anestesia. Esta complicación debe ser diagnosticada y tratada precozmente, puesto que el edema de los tejidos orbitarios puede dañar el nervio óptico

A 58 year-old woman presented with severe chemosis and ophthalmoparesis on her left eye 8 hours after uncomplicated cataract surgery under sub-tenon anaesthesia. Recovery of extrinsic motility was observed after corticosteroid and antihistamine treatment, but a non-haemorrhagic papillary oedema and a concentric defect of visual field were found. It progressed to papillary atrophy with preserved central vision, but with a significant visual field constriction. The aetiological study revealed an allergy to hyaluronidase that was used as adjuvant to the anaesthesia. This complication needs to be promptly diagnosed and treated, as the swelling of the orbital tissues can cause damage to the optic nerve

Compressive optic neuropathy secondary to an allergic reaction to hyaluronidase. / Neuropatía óptica compresiva secundaria a reacción alérgica a hialuronidasa.

A 58 year-old woman presented with severe chemosis and ophthalmoparesis on her left eye 8hours after uncomplicated cataract surgery under sub-tenon anaesthesia. Recovery of extrinsic motility was observed after corticosteroid and antihistamine treatment, but a non-haemorrhagic papillary oedema and a concentric defect of visual field were found. It progressed to papillary atrophy with preserved central vision, but with a significant visual field constriction. The aetiological study revealed an allergy to hyaluronidase that was used as adjuvant to the anaesthesia. This complication needs to be promptly diagnosed and treated, as the swelling of the orbital tissues can cause damage to the optic nerve.

[Low-fluence photodynamic therapy in chronic central serous chorioretinopathy]. / Tratamiento de coriorretinopatía serosa central crónica mediante terapia fotodinámica de baja fluencia.

OBJECTIVE: To evaluate safety and efficacy of low-fluence photodynamic therapy (LFPDT) with verteporfin in patients affected with chronic central serous chorioretinopathy (CCSC), in terms of visual acuity (VA) and macular morphology measured with optical coherence tomography (OCT). METHODS: A retrospective, non-randomized and interventionist analysis was performed on 16 eyes in 15 patients with CCSC treated with LFPDT. Best corrected visual acuity (BCVA) with ETDRS optotypes and central foveal thickness (CFT) in OCT were evaluated as outcome measures. RESULTS: The mean follow-up was 10.8 months. The mean BCVA improved from 58.12 to 68.68 ETDRS letters, and CFT decreased from 280.5 to 172.18 microns, with subretinal fluid resolution in 14 eyes (87.5%), two of them after a second LFTPD. No complications related to treatment were recorded. CONCLUSIONS: LFPDT with verteporfin can be useful in CCSC to stabilise or improve BCVA, reabsorb subretinal fluid and reduce CFT. Randomised studies with a longer follow-up are required to assure the role of this treatment and to optimise parameters for higher efficacy and safety in CCSC patients.

Tratamiento de coriorretinopatía serosa central crónica mediante terapia fotodinámica de baja fluencia / Low-fluence photodynamic therapy in chronic central serous chorioretinopathy

Objetivo: Evaluar la seguridad y la eficacia en términos de agudeza visual (AV) y morfología macular mediante tomografía de coherencia óptica (OCT) de la aplicación de terapia fotodinámica de baja fluencia (LFPDT) con verteporfina en pacientes afectos de coriorretinopatía serosa central crónica (CCSC). Método: Análisis retrospectivo e intervencionista de casos consecutivos, no aleatorizados. Se siguieron un total de 16 ojos de 15 pacientes afectos de CCSC tratados con LFPDT. Se evaluaron mejor agudeza visual corregida (MAVC) mediante escala de optotipos ETDRS y grosor foveal central (CFT) en OCT como indicadores de resultados. Resultados: El seguimiento medio fue de 10,8 meses. La MAVC media mejoró de 58,125 a68,68 letras ETDRS, y el CFT se redujo de 280,5 a 172,18 micras, con desaparición del fluido subretiniano en 14 de los casos (87,5%), en 2 de ellos tras una segunda aplicación de LFPDT.No se registraron complicaciones asociadas al tratamiento. Conclusiones: LFPDT con verteporfina puede ser útil en CCSC para estabilizar o mejorar MAVCy reabsorber el fluido subretiniano y reducir el CFT. Se requieren estudios aleatorizados con seguimiento más prolongado para confirmar el papel de este tratamiento y optimizar losparámetros que permitan mayor eficacia y seguridad en su aplicación en pacientes afectos de CCSC(AU)

Objective: To evaluate safety and efficacy of low-fluence photodynamic therapy (LFPDT) withverteporfin in patients affected with chronic central serous chorioretinopathy (CCSC), interms of visual acuity (VA) and macular morphology measured with optical coherencetomography (OCT). Methods: A retrospective, nonrandomized and interventionist analysis was performed on16 eyes in 15 patients with CCSC treated with LFPDT. Best corrected visual acuity (BCVA) with ETDRS optotypes and central foveal thickness (CFT) in OCT were evaluated as outcomemeasures. Results: The mean follow-up was 10.8 months. The mean BCVA improved from 58.12 to 68.68 ETDRS letters, and CFT decreased from 280.5 to 172.18 microns, with subretinal fluidresolution in 14 eyes (87.5%), two of them after a second LFTPD. No complications related to treatment were recorded. Conclusions: LFPDT with verteporfin can be useful in CCSC to stabilise or improve BCVA, reabsorb subretinal fluid and reduce CFT. Randomised studies with a longer follow-up are required to assure the role of this treatment and to optimise parameters for higher efficacy and safety in CCSC patients(AU)

[Bullous retinal detachment in chronic central serous chorioretinopathy treated with photodynamic therapy]. / Desprendimiento de retina bulloso en coriorretinopatía serosa central crónica tratado con terapia fotodinámica.

CASE REPORT: We describe a case of serous retinal detachment as an atypical presentation of bilateral chronic central serous chorioretinopathy, DISCUSSION: We present its differential diagnosis and therapeutical management with low-fluence photodynamic therapy, achieving satisfactory anatomical and functional results.

Desprendimiento de retina bulloso en coriorretinopatía serosa central crónica tratado con terapia fotodinámica / Bullous retinal detachment in chronic central serous chorioretinopathy treated with photodynamic therapy

Caso clínico: Se presenta un caso de desprendimiento de retina seroso como forma de presentaciónatípica en un paciente afecto de coriorretinopatía serosa central crónica bilateralDiscusión: Presentamos su diagnóstico diferencial y su manejo terapéutico mediante terapiafotodinámica de baja fluencia obteniendo resultados anatómicos y funcionales satisfactorios(AU)

Case report: We describe a case of serous retinal detachment as an atypical presentation ofbilateral chronic central serous chorioretinopathy,Discussion: We present its diferential diagnosis and therapeutical management withlow-fluence photodynamic therapy, achieving satisfactory anatomical and functionalresults(AU)