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.

The role of posterior corneal power in 21st century biometry: A review.

PURPOSE: Intraocular lens (IOL) calculation and biometry have evolved significantly in recent decades. However, present outcomes are still suboptimal. Our objective is to summarize the results reported in the literature with regard to a new variable, the value of the relationship between anterior and posterior corneal curvature in the biometric calculation of IOL power. METHODS: We have created a narrative revision of the existing evidence regarding the posterior to anterior corneal curvature ratio in IOL calculation. RESULTS: The corneal posterior/anterior ratio (P/A ratio), also called Gullstrand ratio, has a standard deviation of 2.4% in normal people, hence causing a possible IOL power miscalculation error of up to 0.75 diopters (D). This error is magnified in pathological corneas or in those with previous refractive surgery. Including the P/A ratio in the IOL formula reduces errors in the calculation of IOL power. CONCLUSIONS: Measurement of the posterior corneal surface should be recommended prior to IOL calculation, given the demonstrated results regarding the P/A ratio for IOL power calculation. Regarding toric IOL calculation, we suggest incorporation of all internal astigmatic vectors, for instance, posterior corneal surface, IOL tilt induced toricity, and retinal astigmatism. All of these factors may improve surgical outcomes.

Síndrome de Haberland con afectación ocular bilateral / Haberland syndrome with bilateral ocular involvement

El síndrome de Haberland o lipomatosis encefalocraneocutánea es un síndrome muy infrecuente caracterizado por la tríada clásica de afectación cutánea, ocular y del sistema nervioso central. Fue descrito por primera vez en 1970 por Haberland y Perou, habiéndose descrito unos 60 casos desde entonces. Presentamos un caso de un varón de 14 semanas diagnosticado de síndrome de Haberland con afectación ocular bilateral en forma de coloboma palpebral y coristomas

Haberland syndrome or encephalocutaneous lipomatosis is a very uncommon syndrome that is characterised by changes in the skin, eye, and central nervous system. It was first described in 1970 by Haberland and Perou, with about 60 cases having been reported since then. A case is reported of a 14-week-old male diagnosed with Haberland syndrome with bilateral ocular involvement in the form of palpebral coloboma and choristomas

Haberland syndrome with bilateral ocular involvement. / Síndrome de Haberland con afectación ocular bilateral.

Haberland syndrome or encephalocutaneous lipomatosis is a very uncommon syndrome that is characterised by changes in the skin, eye, and central nervous system. It was first described in 1970 by Haberland and Perou, with about 60 cases having been reported since then. A case is reported of a 14-week-old male diagnosed with Haberland syndrome with bilateral ocular involvement in the form of palpebral coloboma and choristomas.

Tamoxifen maculopathy: The importance of screening and long follow-up.

We report a case of a patient treated with tamoxifen 20mg daily as hormone therapy for breast cancer. On regular ophthalmological follow-up, tamoxifen maculopathy was detected on SD-OCT (Spectral Domain Optic Coherence Tomography, Carl Zeiss Meditec®), so the medication was discontinued. Despite discontinuation of the medication, the maculopathy progressed over time. We have been following our patient for seven years. Tamoxifen may produce a toxic maculopathy which may progress despite discontinuation of the medication. We consider our case interesting, given the lengthy follow-up of the patient with sequential SD-OCT images. To the best of our knowledge, our case represents the longest follow-up period for a patient with tamoxifen maculopathy. Moreover, we would like to stress the importance of screening in asymptomatic patients on this medication, in order to detect early pathological signs.

Traumatismo craneal abusivo (síndrome del niño zarandeado) / Abusive head trauma (shaken baby syndrome)

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Malformación venolinfática orbitaria (linfangioma) resuelta con bleomicina intralesional / Orbital venous lymphatic malformation (lymphangioma) healed with intra-lesional bleomycin

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