Toxicidad por interferón alfa y neuropatía óptica bilateral reversible: una retirada a tiempo / Interferon-alpha toxicity and reversible bilateral optical neuropathy: A timely withdrawal of the drug

CASO CLÍNICO: Paciente que acude por pérdida crónica de visión bilateral indolora en el que destaca el uso de Interferón alfa (IFNα) y ribavirina para un trasplante hepático. Con un fondo de ojo normal, la sospecha de neuropatía óptica retrobulbar viene confirmada por la prolongación de la latencia de los potenciales evocados visuales. Sin antecedentes de riesgo y con un estudio sistémico normal, la mejoría clínica y de las pruebas electrofisiológicas tras retirar el fármaco señalan al IFNα como causa de la afectación del nervio óptico bilateral. DISCUSIÓN: El IFNα es un tratamiento usado en enfermedades virales y neoplásicas. Actualmente es formulado como Interferón alfa pegilado (IFNα-p) para reducir toxicidad y mejorar su tolerancia. Sus efectos secundarios más comunes son síndrome gripal, astenia y pérdida de peso. La afectación ocular es rara y la neuropatía óptica es una complicación infrecuente siendo más frecuente la retinopatía en el inicio del tratamiento. La hipótesis más aceptada como causa de la toxicidad es la existencia de complejos inmunes circulantes. Así pues es de vital importancia para el oftalmólogo conocer la toxicidad de este fármaco para saber retirar a tiempo el mismo, evitando una posible pérdida visual irreversible

CLINICAL CASE: A patient with chronic, painless, bilateral loss of vision, after significant intake of interferon (IFNα) and ribavirina due to liver transplant. Ocular fundus is normal. A suspected retrobulbar optic neuropathy is confirmed by a prolongation of the latency of the patient's visual evoked potential. There being no prior record of risk factors and with the patient's systemic analysis giving normal results, the clinical improvement and the electro-physiological tests conducted after the drug was withdrawn point to interferon as negatively affecting the bilateral optic nerve. Discussion: Interferon-α is used in the treatment of viral and neoplastic illnesses. Currently the drug is formulated as Interferon alfa pegilado (IFNα-p) in order to reduce toxicity and increase tolerance. The most common secondary effects are flu symptoms, asthenia and weigh loss. Affected ocular tissue is rare and optic neuropathy is also an infrequent complication: retinopathy at the beginning of treatment is, however, more frequent. The most widely accepted hypothesis as to the cause of toxicity is the presence of circulating immune complexes. It is, therefore, essential for ophthalmologists to be aware of the toxicity of this drug in order to be able to withdraw it in good time, thus preventing potentially irreversible sight loss

[Interferon-alpha toxicity and reversible bilateral optical neuropathy: a timely withdrawal of the drug]. / Toxicidad por interferón alfa y neuropatía óptica bilateral reversible: una retirada a tiempo.

Clinical case A patient with chronic, painless, bilateral loss of vision, after significant intake of interferon (IFNα) and ribavirina due to liver transplant. Ocular fundus is normal. A suspected retrobulbar optic neuropathy is confirmed by a prolongation of the latency of the patient's visual evoked potential. There being no prior record of risk factors and with the patient's systemic analysis giving normal results, the clinical improvement and the electro-physiological tests conducted after the drug was withdrawn point to interferon as negatively affecting the bilateral optic nerve. Discussion Interferon-α is used in the treatment of viral and neoplastic illnesses. Currently the drug is formulated as Interferon alfa pegilado (IFNα-p) in order to reduce toxicity and increase tolerance. The most common secondary effects are flu symptoms, asthenia and weigh loss. Affected ocular tissue is rare and optic neuropathy is also an infrequent complication: retinopathy at the beginning of treatment is, however, more frequent. The most widely accepted hypothesis as to the cause of toxicity is the presence of circulating immune complexes. It is, therefore, essential for ophthalmologists to be aware of the toxicity of this drug in order to be able to withdraw it in good time, thus preventing potentially irreversible sight loss.

Distrofia en huella dactilar: brotes de astigmatismo irregular y su demostración topográfica / Fingerprint microcystic dystrophy: Episodes of irregular astigmatism and their topographical representation

Caso clínico: Varón de 44 años con brotes indoloros de visión borrosa sin repercusión oftalmológica visible inicialmente. Tras uno de sus episodios, se visualiza en polo anterior imágenes en huella digital así como cambios corneales, paquimétricos y topográficos que originan cambios refractivos sintomáticos. Discusión: La distrofia en huella dactilar es una entidad diagnosticada por erosiones corneales recurrentes. Su base patogénica, una membrana basal epitelial alterada, favorece la separación del epitelio corneal de las capas subyacentes. En la medida que ésta sea total o parcial ocasionará erosiones corneales recurrentes o, con menor frecuencia, episodios de visión borrosa por edema y engrosamiento corneal(AU)

Case Study: A 44 year-old male patient suffering from painless bouts of blurred vision and with no visible ophthalmological repercussions initially. After one of these clinical episodes we managed to visualise fingerprint images in the anterior pole, as well as corneal, pachymetric and topographical changes, which in turn produce the symptomatic refractive changes. Discussion: Fingerprint keratopathy is a condition diagnosed through recurring corneal erosion. The pathogenic origin of the condition-an altered epithelial basal membrane- may encourage the separation of the corneal epithelium from its underlying layers. Depending on whether this separation is partial or total, this will lead to spontaneous corneal erosion or, less frequently, episodes of blurred vision caused by oedema and corneal swelling(AU)

[Fingerprint microcystic dystrophy: Episodes of irregular astigmatism and their topographical representation]. / Distrofia en huella dactilar: brotes de astigmatismo irregular y su demostración topográfica.

CASE STUDY: A 44 year-old male patient suffering from painless bouts of blurred vision and with no visible ophthalmological repercussions initially. After one of these clinical episodes we managed to visualise fingerprint images in the anterior pole, as well as corneal, pachymetric and topographical changes, which in turn produce the symptomatic refractive changes. DISCUSSION: Fingerprint keratopathy is a condition diagnosed through recurring corneal erosion. The pathogenic origin of the condition-an altered epithelial basal membrane- may encourage the separation of the corneal epithelium from its underlying layers. Depending on whether this separation is partial or total, this will lead to spontaneous corneal erosion or, less frequently, episodes of blurred vision caused by oedema and corneal swelling.

[Iridoschisis associated to congenital syphilis: serological confirmation at the 80's]. / Iridosquisis asociada a sífilis congénita: confirmación serológica a los 80.

CLINICAL CASE: Female in her eighties is admitted suffering from unilateral ocular pain. On examination we observed, as well as corneal abrasion, a bilateral iridoschisis with a frayed iris, unfolded between its stromal layers. DISCUSSION: This rare case is related either to senile degenerative change or to angle-closure glaucoma. However, it is also associated with congenital syphilis with or without the presence of interstitial keratitis. Infant conjunctivitis and a "salt and pepper" appearance of the fundus oculi complete the diagnosis. It is confirmed that the patient had suffered from congenital late syphilis, cured by the age of 80: this confirmation is reached by treponemal (RPR-) and non-treponemal (TPHA+) serological tests.

Iridosquisis asociada a sífilis congénita: confirmación serológica a los 80 / Iridoschisis associated to congenital syphilis: serological confirmation at the 80's

Caso clínico: Mujer octogenaria que acude pordolor ocular unilateral. En la exploración observamos,además de una abrasión corneal, una iridosquisisbilateral con un iris desflecado, desdobladoentre sus capas estromales.Discusión: Esta rara entidad está relacionada conlos cambios degenerativos seniles o al glaucoma deángulo cerrado. Sin embargo, también es asociado ala sífilis congénita con o sin la coexistencia de queratitisintersticial. Las conjuntivitis en la infancia yel fondo de ojo en «sal y pimienta» complementanel diagnóstico de sospecha. Confirmándose, a los80 años una sífilis congénita tardía curada, por lostest serológicos treponémicos (RPR-) y no treponémicos(TPHA+)(AU)

Clinical case: Female in her eighties is admittedsuffering from unilateral ocular pain. On examinationwe observed, as well as corneal abrasion, abilateral iridoschisis with a frayed iris, unfoldedbetween its stromal layers.Discussion: This rare case is related either to seniledegenerative change or to angle-closure glaucoma.However, it is also associated with congenital syphiliswith or without the presence of interstitial keratitis.Infant conjunctivitis and a «salt and pepper» appearanceof the fundus oculi complete the diagnosis.It is confirmed that the patient had suffered fromcongenital late syphilis, cured by the age of 80: thisconfirmation is reached by treponemal (RPR-) andnon-treponemal (TPHA+) serological tests(AU)

[Congenital retinal macrovessels: a discovery by chance]. / Macrovasos congénitos retinianos: un hallazgo casual.

CLINICAL CASE: Congenital retinal macrovessels, where an aberrant retinal vessel, usually a venule, is present in the posterior pole and may cross the avascular foveal region, are seen rarely. DISCUSSION: Most of these cases are unilateral and stable with excellent visual prognosis and are detected on routine examination. Foveal cysts, hemorrhages and displaced foveola may also be seen. These entities must be distinguished from racemose angiomas, capillary hemangiomas of the retina and even when associated with neurological symptoms, should be considered as part of the Wyburn-Masson syndrome.

Macrovasos congénitos retinianos: un hallazgo casual / Congenital retinal macrovessels: a discovery by chance

Caso clínico: Los macrovasos congénitos retinianos son raros, son ramas aberrantes, vénulas en su mayoría, localizadas en el polo posterior que de forma típica cruzan la zona avascular foveal. Discusión: En la mayoría de los casos son lesiones unilaterales y estables, sin repercusión visual por lo que su diagnóstico es casual. En ocasiones se asocian a localizaciones atípicas de la fóvea, quistes foveales e incluso hemorragias. Por lo general debe realizarse un diagnóstico diferencial con otras entidades vasculares retinianas como el hemangioma capilar, angioma racemoso e incluso si se acompaña de síntomas neurológicos descartar el síndrome de Wyburn-Masson

Clinical case: Congenital retinal macrovessels, where an aberrant retinal vessel, usually a venule, is present in the posterior pole and may cross the avascular foveal region, are seen rarely. Discussion: Most of these cases are unilateral and stable with excellent visual prognosis and are detected on routine examination. Foveal cysts, hemorrhages and displaced foveola may also be seen. These entities must be distinguished from racemose angiomas, capillary hemangiomas of the retina and even when associated with neurological symptoms, should be considered as part of the Wyburn-Masson syndrome (Arch Soc Esp Oftalmol 2008; 83: 273-276)

[Outcomes of macular hole surgery using three different surgical techniques]. / Resultados de la cirugía del agujero macular con diferentes técnicas de tratamiento.

PURPOSE: To compare the effectiveness, efficiency and complications of three different surgical techniques employed for the treatment of macular holes between 1998 -2004 in our health care centre. METHODS: A retrospective, comparative, and non-randomized study of 131 eyes with macular holes, stage III and IV, treated with three different surgical techniques was performed. Vitrectomy was performed in 25 eyes (group 1). Vitrectomy was associated with internal limiting membrane peeling in 19 eyes (group 2), and in the remaining 87 eyes, the peeling of the retinal internal limiting membrane was assisted with indocyanine green (ICG) (group 3). The concentrations of ICG used were 5%, 2.5% and 0.5%. We compared the anatomic and functional results, and the complications which occurred. RESULTS: The overall anatomic success rate was 88.4% (114 eyes), and it was higher in group 3 (90.6%). A visual acuity improvement was achieved in 63.6% of eyes; however there were no significant differences between the groups studied. The most important complications included cataracts (51.9%), increase in intra-ocular pressure (37.2%), retinal detachment (7.8%) and retinal pigment epithelial changes (7%). These pigment epithelial changes were associated with the use of ICG-assisted peeling (p<0.05), but there was no correlation between the changes and the concentration of ICG employed. The hole size had an inverse correlation with initial and final visual acuity. An epi-retinal membrane was associated with better visual acuities and higher anatomic success. CONCLUSIONS: In our experience, the use of ICG-assisted internal limiting membrane peeling in macular hole surgery improves the anatomic success, but may lead to retinal pigment epithelial changes.

Resultados de la cirugía del agujero macular con diferentes técnicas de tratamiento / Outcomes of macular hole surgery using three different surgical techniques

Objetivo: Comparar la efectividad, eficiencia y complicaciones de las tres técnicas quirúrgicas empleadas en el tratamiento del agujero macular en el período 1998-2004 en nuestro centro. Métodos: Estudio retrospectivo, comparativo, intervencionista y no aleatorizado de 131 ojos con agujero macular idiopático en estadío III y IV, operados con tres técnicas. En 25 ojos se realizó vitrectomía simple (grupo 1), en 19 ojos se asoció rexis de la membrana limitante interna (MLI) (grupo 2), y en 87 ojos esta rexis se llevó a cabo previa tinción con verde de indocianina (ICG) (grupo 3). Las concentraciones de ICG empleadas fueron del 5%, 2,5% y 0,5%. Se compararon la recuperación anatómica y funcional y las complicaciones en los distintos grupos. Resultados: El 88,4% (114 ojos) lograron la resolución anatómica, siendo superior en el grupo 3 (90,6%). La recuperación funcional se consiguió en el 63,6%. En ambos casos no existieron diferencias entre grupos. Las complicaciones más importantes fueron: cataratas post-cirugía (51,9%), aumento de PIO (37,2%), desprendimientos de retina (7,8%) y alteraciones del epitelio pigmentario (7%). Esta última se relacionó con el uso de ICG (p<0,05), sin encontrarse diferencias en las concentraciones empleadas. El tamaño del agujero tuvo una correlación inversa con la agudeza visual (AV) inicial y post-quirúrgica. La presencia de membrana epirretiniana se asoció a mejores AV y a un mayor éxito anatómico. Conclusiones: En nuestra experiencia, la cirugía mejora la AV, siendo más efectiva si se asocia ICG para la rexis de la MLI. Sin embargo, esta tinción podría estar relacionada con las alteraciones del EPR encontradas

Purpose: To compare the effectiveness, efficiency and complications of three different surgical techniques employed for the treatment of macular holes between 1998 -2004 in our health care centre. Methods: A retrospective, comparative, and nonrandomized study of 131 eyes with macular holes, stage III and IV, treated with three different surgical techniques was performed. Vitrectomy was performed in 25 eyes (group 1). Vitrectomy was associated with internal limiting membrane peeling in 19 eyes (group 2), and in the remaining 87 eyes, the peeling of the retinal internal limiting membrane was assisted with indocyanine green (ICG) (group 3). The concentrations of ICG used were 5%, 2.5% and 0.5%. We compared the anatomic and functional results, and the complications which occurred. Results: The overall anatomic success rate was 88.4% (114 eyes), and it was higher in group 3 (90.6%). A visual acuity improvement was achieved in 63.6% of eyes; however there were no significant differences between the groups studied. The most important complications included cataracts (51.9%), increase in intra-ocular pressure (37.2%), retinal detachment (7.8%) and retinal pigment epithelial changes (7%). These pigment epithelial changes were associated with the use of ICG-assisted peeling (p<0.05), but there was no correlation between the changes and the concentration of ICG employed. The hole size had an inverse correlation with initial and final visual acuity. An epi-retinal membrane was associated with better visual acuities and higher anatomic success. Conclusions: In our experience, the use of ICGassisted internal limiting membrane peeling in macular hole surgery improves the anatomic success, but may lead to retinal pigment epithelial change (AU)

[Dual cause of blindness: chorioretinitis sclopetaria and homonymous hemianopsia]. / Doble causa de ceguera: coriorretinitis esclopetaria y hemianopsia homónima.

CLINICAL CASE: We describe the case of a man who, after a gunshot wound to the right facial region, exhibited chorioretinitis sclopetaria of the right eye and a contralateral homonymous hemianopsia. DISCUSSION: Chorioretinitis sclopetaria is a rare entity resulting from an ocular injury caused by a bullet passing through the orbit. It is not common to find the condition associated with a damaged visual field caused by the subsequent path traversed and lodging of that bullet in the brain.

Doble causa de ceguera: coriorretinitis esclopetaria y hemianopsia homónima / Dual cause of blindness: choriorretinitis sclopetaria and homonymous hemianopsia

Caso clínico: Se describe el caso de un varón quetras una agresión con arma de fuego en la regiónfacial derecha presenta una coriorretinopatía esclopetaria(CS) en ojo derecho y una heminanopsiahomónima contralateral.Discusión: La CS es una rara entidad producida porla lesión ocular por una bala al atravesar la órbita.Además es rara encontrarla asociada a una alteracióncampimétrica causada por el trayecto y el alojamientode la misma bala en el cerebro

Clinical case: We describe the case of a man who, ;;after a gunshot wound to the right facial region, ;;exhibited chorioretinitis sclopetaria of the right eye ;;and a contralateral homonymous hemianopsia. ;;Discussion: Chorioretinitis sclopetaria is a rare ;;entity resulting from an ocular injury caused by a ;;bullet passing through the orbit. It is not common to ;;find the condition associated with a damaged visual ;;field caused by the subsequent path traversed and ;;lodging of that bullet in the brain