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.

Visual impairment induced by prosthetic cobaltism.

The case of a 68-year-old patient with visual loss secondary to prosthetic cobaltism is reported. The degeneration of the metallic hip prosthesis can produce a systemic absorption of cobalt with cardiac, neurological, endocrine, auditory, and visual manifestations. The diagnostic suspicion is confirmed by serum cobalt measurements. Treatment with early surgery and chelating agents can lead to improvement of the visual, and the other disorders.

Afectación visual por cobaltismo protésico / Visual impairment induced by prosthetic cobaltism

Se presenta el caso de un paciente de 68 años con pérdida visual secundaria a cobaltismo protésico. La degeneración de la prótesis metálica de cadera produce una absorción sistémica de cobalto con manifestaciones cardíacas, neurológicas, endocrinas, auditivas y visuales. La sospecha diagnóstica es confirmada mediante determinaciones séricas de cobalto, y un tratamiento con cirugía precoz y agentes quelantes puede generar una mejoría visual y del resto de síntomas (AU)

The case of a 68-year-old patient with visual loss secondary to prosthetic cobaltism is reported. The degeneration of the metallic hip prosthesis can produce a systemic absorption of cobalt with cardiac, neurological, endocrine, auditory, and visual manifestations. The diagnostic suspicion is confirmed by serum cobalt measurements. Treatment with early surgery and chelating agents can lead to improvement of the visual, and the other disorders (AU)

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.

[Neuro-ophthalmological manifestations of pituitary adenomas. The usefulness of optical coherence tomography]. / Manifestaciones neurooftalmológicas de los adenomas hipofisarios. Valor de la tomografía de coherencia óptica.

INTRODUCTION: Pituitary adenomas are a frequently occurring pathology that require multidisciplinary management by several different specialists. Their neuro-ophthalmological manifestations vary widely and sometimes appear as the presenting symptom. AIM: To gather the main ophthalmological signs and symptoms of these tumours so that specialists who find themselves before any of them will suspect this pathology. DEVELOPMENT: This survey was based on the clinical experience of the neuro-ophthalmological unit at the Hospital Clinic de Barcelona, with over 350 patients who had suffered from pituitary tumours. A bibliographical search was also carried out on Medline for papers published on pituitary adenomas in the literature in both English and Spanish. We focused mainly on those that reported on the ophthalmological manifestations of such tumours. A number of articles were found dealing with isolated ophthalmological manifestations of these tumours, many of them as presenting symptoms. We also found review articles in English. Apart from oculomotor disorders and other less common findings, visual field defects stand out as the guiding symptom of this condition. CONCLUSIONS: Improved diagnostic techniques are allowing pituitary tumours to be detected at increasingly earlier stages, but cases are still seen with neuro-ophthalmological symptoms as the presenting symptoms. Familiarity with these syndromes is crucial. In addition to the clinical and visual field examination, optical coherence tomography is particularly useful for the diagnosis and follow-up of these patients. Prospective studies are needed to establish factors for predicting the visual recovery in these patients.

Manifestaciones neurooftalmológicas de los adenomas hipofisarios. Valor de la tomografía de coherencia óptica / Neuro-ophthalmological manifestations of pituitary adenomas. The usefulness of optical coherence tomography

Introducción. Los adenomas hipofisarios son una patología frecuente. Requieren un tratamiento multidisciplinario entre varios especialistas. Sus manifestaciones neurooftalmológicas son muy variadas, y en ocasiones aparecen como síntoma de presentación. Objetivo. Reunir los principales signos y síntomas oftalmológicos de estos tumores, para que todo especialista sospeche esta patología cuando se encuentre ante alguno de ellos. Desarrollo. Esta revisión se ha desarrollado sobre la base de la experiencia clínica de la unidad de neurooftalmología del Hospital Clínic de Barcelona, con más de 350 pacientes afectos de tumores hipofisarios. Asimismo, se ha realizado una búsqueda bibliográfica en Medline de los artículos publicadossobre adenomas hipofisarios en lengua castellana e inglesa. Se centró principalmente en aquéllos que se referían a las manifestaciones oftalmológicas de dichos tumores. Se encontraron numerosos artículos referentes a manifestaciones oftalmológicas aisladas de estos tumores, muchas de ellas como inicio. También se encontraron artículos de revisión en lengua inglesa.Aparte de las alteraciones oculomotoras y otros hallazgos menos frecuentes, destacan las alteraciones campimétricas como síntoma guía de esta patología. Conclusiones. Los tumores hipofisarios se detectan cada vez más temprano debido a la mejora en las técnicas diagnósticas, pero aún se observan casos con síntomas neurooftalmológicos como inicio. Resulta crucialel conocimiento de estos síndromes. Además de la exploración clínica y la campimetría, la tomografía de coherencia óptica resulta de gran valor para el diagnóstico y el seguimiento de estos pacientes. Se necesitan estudios prospectivos para establecerfactores pronósticos de recuperación visual en estos pacientes

Introduction. Pituitary adenomas are a frequently occurring pathology that require multidisciplinary management by several different specialists. Their neuro-ophthalmological manifestations vary widely and sometimes appear as the presenting symptom. Aim. To gather the main ophthalmological signs and symptoms of these tumours so that specialists who find themselves before any of them will suspect this pathology. Development. This survey was based on the clinical experience of the neuro-ophthalmological unit at the Hospital Clínic de Barcelona, with over 350 patients who had suffered from pituitary tumours. A bibliographical search was also carried out on Medline for papers published on pituitary adenomas in the literature in both English and Spanish. We focused mainly on those that reported on the ophthalmological manifestations ofsuch tumours. A number of articles were found dealing with isolated ophthalmological manifestations of these tumours, many of them as presenting symptoms. We also found review articles in English. Apart from oculomotor disorders and other less common findings, visual field defects stand out as the guiding symptom of this condition. Conclusions. Improved diagnostictechniques are allowing pituitary tumours to be detected at increasingly earlier stages, but cases are still seen with neuroophthalmological symptoms as the presenting symptoms. Familiarity with these syndromes is crucial. In addition to the clinical and visual field examination, optical coherence tomography is particularly useful for the diagnosis and follow-up ofthese patients. Prospective studies are needed to establish factors for predicting the visual recovery in these patients

[Papilledema as an indicator of POEMS syndrome]. / Papiledema asociado a síndrome de POEMS.

CASE REPORT: We present the case of a 54-year-old woman with papilledema associated to POEMS syndrome. The presence of intracranial hypertension was detected and treatment started with acetazolamide. DISCUSSION: The most common ophthalmological pathology in POEMS syndrome is papilledema, the etiology of which could be infiltrative, intracranial hypertension, inflammation or an increase of the vascular permeability. The correct diagnosis and treatment of papilledema, depending on its etiology, should permit an acceptable visual outcome to be achieved.

[Sinus syndrome, an uncommon cause of enophthalmos]. / Síndrome del seno silente, una causa infrecuente de enoftalmos.

CLINICAL CASE: A 29-year-old woman presented with a subjective sensation of ocular asymmetry from several months beforehand. Ophthalmological exam showed 3,5 mm right enophthalmos and light hypoglobus. CT (Computerized Tomography) scan showed a collapsed maxillar sinus, and a thinned inferior orbital wall. The diagnosis of silent sinus syndrome was made. An endoscopic maxillary antrostomy with uncinectomy was made with an excellent surgical outcome. DISCUSSION: Clinical features of silent sinus syndrome are described, including diagnosis, differential diagnosis and treatment.