X-linked retinoschisis: About a case.

X-linked retinoschisis (XLR) is a cause of retinal degeneration that affects males at an early age. X-linked disorders classically affect only males. We present the case of a 10-year-old female with the full spectrum of the pathology. BCVA 0.7 OU. Optical coherence tomography (OCT) showed bilateral foveal alteration with cystic appearance. The genetic study identified the variant c.644A>T (p.Glu215Gly) in the RS1 gene in homozygosis, associated with retinoschisis with X-linked recessive mode of inheritance. XLR is a condition that has a great variety in the severity of the disease and there is no correlation between the latter and the progression of the pathology. The disease has been described in a limited number of females mainly in families with high degree of consanguinity.

Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis. / Manifestaciones neuroftalmológicas como complicación de una infección por Mycoplasma pneumoniae y desarrollo posterior de una encefalitis aguda diseminada.

The purpose of this article is to describe two paediatric neuro-ophthalmological clinical cases caused by a systemic infection due to Mycoplasma pneumoniae (M. pneumoniae). The cases are two girls aged 14 and 12 seen in the Emergency Department: The first one had internuclear ophthalmoplegia and second with loss of vision and headache. They had no other neurological foci. Magnetic resonance imaging showed hyperintense plaques in both, suggestive of a demyelinating disease. One month later, the neuro-ophthalmological symptoms resolved, with normal follow-up magnetic resonance imagings. The diagnosis was acute disseminated encephalitis secondary to M. pneumoniae. The diagnosis was made using PCR (gold standard) and/or IgM in serology. It is important to think about this possible aetiology in cases of suggestive demyelinating disease. There is controversy about the role of antibiotics and on whether corticosteroids are contemplated. In conclusion, M. pneumoniae must be a differential diagnosis in acute neuro-ophthalmological disorders in children.

Oculomotor nerve palsy in herpes zoster ophthalmicus: Presentation of 4 cases. / Parálisis oculomotoras en el contexto de una reactivación del virus varicela-zóster: a propósito de 4 casos.

Varicella zoster virus (VZV) infection has a high prevalence worldwide. Within the multiple ophthalmologic manifestations that VZV can cause, sudden diplopia is among them. A review is presented of four clinical cases of 3rd and 4th oculomotor nerve palsies in herpes zoster ophthalmicus. A review is also presented of the physiopathology and most important clinical manifestations. Imaging tests are essential in order to rule out complications. Systemic antiviral administration is the correct treatment.

Topical brinzolamide in congenital nystagmus: A retrospective study. / Brinzolamida tópica en el nistagmo congénito: Estudio retrospectivo.

OBJECTIVE: To evaluate the effect of treatment with topical brinzolamide on visual acuity and nystagmus intensity in patients with congenital nystagmus. MATERIAL AND METHODS: A retrospective study was designed in which the clinical records of 14 patients with congenital nystagmus were reviewed. All patients underwent a complete ophthalmological examination and a Perea video-oculography (VOG) before, and three days after, initiation of treatment with topical brinzolamide (Azopt). Five expert researchers evaluated the intensity of nystagmus by video before and after treatment. Finally, the subjective improvement of the patients was recorded. RESULTS: Statistically significant differences were found in pre- and post-treatment binocular near visual acuity. A slight increase in the frequency of nystagmus was found, which was statistically significant with the horizontal gaze to the left and with the gaze downwards (P=.04, P=.03, respectively). The kappa index concordance between the researchers evaluating the intensity of nystagmus was 0.014. Only two of the patients noticed improvement in visual acuity, and one patient noticed improvement in the aesthetic aspect. CONCLUSIONS: In spite of an improvement in nystagmus, it was slight, not cosmetically appreciable by patients in most cases, and was not related to a significant improvement in visual acuity or in patient quality of life. Further studies are needed to evaluate the effects of topical brinzolamide, and to establish potential therapeutic indications in nystagmus.

[Study of the retinal nerve fiber layer in childhood strabismus]. / Estudio de la capa de fibras nerviosas de la retina en el estrabismo infantil.

OBJECTIVE: To assess possible differences in the retinal nerve fiber layer (RNFL) thickness between children with strabismus and controls. This study also compared esotropia with exotropia cases, and dominant eyes with non-dominant eyes. METHOD: 31 children with esotropia, 17 children with exotropia and 32 controls were studied. The peripapillary RNFL average thickness was determined in 4 different areas (inferior, superior, nasal and temporal) using optical coherence tomography. Statistical analysis was performed a) between the strabic children and the control group, b) between the esotropia and exotropia groups, and c) between the dominant eyes and the non-dominant eyes of the strabic children. RESULTS: No statistically significant differences in RNFL thickness were found in any of these statistical comparisons. CONCLUSIONS: From this study, no evidence of changes in RNFL thickness, associated to the existence of strabismus, were found.

[Peripheral homonymous hemianopia. A case report]. / Hemianopsia homónima periférica. A propósito de un caso.

CASE REPORT: A 63-year-old male with a history of hypertension, hypercholesterolemia and chronic smoking, presented with a loss of the temporal visual field of right eye (RE). A restriction of the temporal field of RE and also of the nasal field of left eye was clinically suspected when the patient was tested by confrontation. A kinetic perimetry confirmed the existence of a right homonymous hemianopia that was very eccentric in both eyes (beyond the central 50 degrees). Brain magnetic resonance images revealed a well-circumscribed infarction in the ventral portion of the left calcarine fissure that was consistent with the patient's visual field loss. DISCUSSION: Peripheral homonymous hemianopsia is a rare condition that is caused by a partial infarction of the most ventral striate cortex. Kinetic perimetry provided the most helpful information about the referred scotomas.

Manifestaciones neuroftalmológicas como complicación de una infección por Mycoplasma pneumoniae y desarrollo posterior de una encefalitis aguda diseminada / Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis

El objetivo es describir dos cuadros clínicos neuroftalmológicos en niños por infección sistémica por Mycoplasma pneumoniae (M. pneumoniae). Se presentan los casos de dos niñas de 14 y 12 años que acudieron a urgencias: la primera con oftalmoplejía internuclear y la segunda con pérdida de visión y cefalea. No presentaban otra focalidad neurológica. En la imagen de resonancia magnética se evidenciaron placas hiperintensas en ambas, sugerentes de cuadro desmielinizante. Al mes, los síntomas neuroftalmológicos se resolvieron y las resonancias magnéticas de control fueron normales. El diagnóstico fue encefalitis diseminada aguda secundaria a M. pneumoniae. El diagnóstico se hace por PCR (gold standard) y/o IgM en serología. Es importante pensar en esta posible etiología ante casos sugerentes de enfermedad desmielinizante. Existe controversia sobre el papel de los antibióticos y si se contemplan los corticoides. Como conclusión, M. pneumoniae debe ser diagnóstico diferencial en afectaciones neuroftalmológicas agudas en niños

The purpose of this article is to describe two paediatric neuro-ophthalmological clinical cases caused by a systemic infection due to Mycoplasma pneumoniae (M. pneumoniae). The cases are two girls aged 14 and 12 seen in the Emergency Department: The first one had internuclear ophthalmoplegia and second with loss of vision and headache. They had no other neurological foci. Magnetic resonance imaging showed hyperintense plaques in both, suggestive of a demyelinating disease. One month later, the neuro-ophthalmological symptoms resolved, with normal follow-up magnetic resonance imagings. The diagnosis was acute disseminated encephalitis secondary to M. pneumoniae. The diagnosis was made using PCR (gold standard) and/or IgM in serology. It is important to think about this possible aetiology in cases of suggestive demyelinating disease. There is controversy about the role of antibiotics and on whether corticosteroids are contemplated. In conclusion, M. pneumoniae must be a differential diagnosis in acute neuro-ophthalmological disorders in children

[Residual myopia after penetrating keratoplasty for keratoconus]. / Miopía residual tras queratoplastia penetrante en queratocono.

PURPOSE: To study the results of penetrating keratoplasty for keratoconus analyzing the differences in ultimate spherical equivalent according to graft size and the trephine used in the recipient. MATERIAL AND METHODS: Retrospective study of 38 patients that were divided into three groups. Group A is composed of patients with a 0.50 mm donor-recipient difference, 0.25 mm in group B and same-sized in group C. In each group visual acuity, spheric equivalent, keratometry and other parameters were assessed. Statistical analysis was performed by comparing the means of the parameters evaluated. RESULTS: We found a better final visual acuity in group C when compared with group A, and a lower spherical equivalent when we compared groups A with C and A with B. The results were better in those groups where the donor-recipient difference was smaller. CONCLUSIONS: We believe that it is convenient to minimize the size difference between donor and recipient or to use same-sized trephines to diminish the final spheric equivalent.

[Corneal descompensation in patients with endothelial compromise treated with topical dorzolamide]. / Descompensación corneal en pacientes con compromiso endotelial tratados con dorzolamida tópica.

PURPOSE: To investigate the possibility of corneal alterations in patients with long-term endothelial compromise with topical dorzolamide. MATERIAL AND METHODS: Retrospective descriptive study of 17 patients with penetrating keratoplasty and glaucoma associated with topical carbonic anhydrase inhibitor therapy, looking for coincidence with corneal alteration. RESULTS: Classified by ethiology, type of glaucoma and control, recording previous ophthalmological surgeries, evolution time, complications and rejection episodes. Seven patients suffered a corneal decompensation, in three of them there were signs of true reject but only four cases had edema at the beginning of dorzolamide treatment, one of them recovering after stopping dorzolamide. Risk factors were previous cataract surgery, mainly aphakia, filtering surgery and previous vitrectomy. CONCLUSIONS: Dorzolamide could have a potential negative effect on patients with endothelial compromise.

[Conjunctival marginal zone lymphoma: clinical and pathological features]. / Linfoma de la zona marginal de conjuntiva: características clínico-patológicas.

OBJECTIVE/METHODS: We present the case of a 66-years-old woman with a left pink-coloured tumor in the temporal bulbar conjunctiva which had been present for 7 months, without response to the topic treatment applied. A biopsy and an immunohistochemistry study were done to confirm the diagnosis. RESULTS/CONCLUSIONS: The biopsy showed a low-grade B-lymphoma compatible with a marginal zone lymphoma, formerly called <> (MALT) lymphoma. The conjunctival Iymphomas are rare and the majority of them are marginal zone lymphomas. It is important to differentiate this one from another ocular adnexa lymphomas, because of their low grade of association to extraocular affectation and more favourable prognosis.

[Dorzolamide: hypotensive efficacy in combination with beta-blockers. Long-term results]. / Dorzolamida: eficacia hipotensora en asociación a betabloqueantes. Resultados a largo plazo.

PURPOSE: To know, in long term, the effects in IOP of dorzolamide as adjunctive treatment to beta-blockers. To compare the hypotensory potential added to the different beta-blockers (selective, non-selective, with I.S.A.). To compare its potential as hypotensive drug versus another drugs in association to beta-blockers- pilocarpine and dipivalilepinefrina. METHODS: A descriptive-retrospective randomised study about 132 eyes with glaucoma, with 16 months of mean follow-up; divided into three groups: one of the patients treated with association to beta-blockers, and the two others patients treated with a combination of beta-blockers with pilocarpine or DPVE, in which they were substituted by dorzolamide. Student T was used for comparing the media. RESULTS: The average IOP reduction was 21.48 to 18.39 mmHg, with the addiction of dorzolamide to beta-blockers. Between the different beta-blockers, the non selective showed a higher hypotensive effect in association to dorzolamide (5.23 mmHg), more than selective (3.75 mmHg), but not significantly higher than those with I.S.A. (4.29 mmHg). In substitution of pilocarpine or DPVE, in its use associated to beta-blockers, dorzolamide showed a significantly higher efficacy, with an average IOP reduction of 4.16 mmHg by pilocarpine, and 4.33 by DPVE. CONCLUSION: Dorzolamide gets a higher hypotensive effect by adding it to patients in treatment with beta-blockers, principally with the non selective ones. In this way, it has shown a superior hypotensive effect than pilocarpine and DPVE.

[Bilateral acute retinal necrosis due to herpes simplex virus in inmunocompetent people and acyclovir resistance]. / Necrosis retiniana aguda bilateral por herpes simple en individuo inmunocompetentes y resistencia a aciclovir.

CASE REPORT: A twenty-eight year old woman with necrotitizing retinitis and herpes simplex virus type 1 isolated in aqueous humor with polymerase chain reaction (PCR). An Acyclovir and corticosteroid therapy was started with unsuccessful response, Foscarnet was added getting quiescence of lesions. DISCUSSION: Acute Retinal Necrosis Syndrome (ARNS), induced by a virus of the herpes family, could develop in immunocompetent people. A characteristic clinical case with uveitis and vitritis, white retinitis areas and occlusive vasculitis is reported. Antiviral therapy with acyclovir and antiinflammatory treatment must be established quickly. Foscarnet can effectively treat ARNS in inmunocompetent patients. In spite of therapy, this is a potentially blinding retinal disease.

[Post-cataract surgery diplopia: etiology and treatment]. / Diplopía post-cirugía de catarata: causas y tratamiento.

PURPOSE: To know the causes and treatment of diplopia observed after cataract surgery. METHODS: We make a descriptive retrospective study on 19 cases with persistent binocular diplopia following cataract surgery. RESULTS: They were classified by aetiology in four groups: myotoxic effects in surgery act (47.4%), alteration in binocular vision (amblyopia, previous strabismus or long sensory deprivation) (47.4%), refractive alterations (5.2%) and previous disease. Initially, treatment with prisms, was tolerated on 47.4%; surgery was required on 36.8%, botulinicum toxin on 5.3% and penalty on 10.5% to avoid diplopia. Eventually, 36.8% of patients went on with diplopia, just only one lay equal, disappearing on 63.2% because binocular vision was recovered or suppressed by themselves or by penalty. CONCLUSIONS: Diplopia after cataracts surgery, is a serious complication that surgeons must take into account. In spite of the different treatments used, it is not easy to get its disappearance.

[Melanoma of the eyelid margin: clinical management and oncopathologic study]. / Melanoma del borde libre del párpado: manejo clínico y estudio onco-patológico.

OBJECTIVE/METHODS: A 57 year-old male patient with a very pigmented tumor of the margin of the upper lid and two months of evolution is presented. After its excision and histopathological study, the diagnose was nodular melanoma. RESULTS/CONCLUSIONS: We expose the treatment and clinical management of this unfrequent pathology. Different clinical features and other locations in the eyelid are discussed regarding the prognosis.

Retinosquisis ligada al X en homocigosis: A propósito de un caso / X-linked retinoschisis: About a case

La retinosquisis ligada al cromosoma X (RLX) es una causa de degeneración retiniana que afecta a varones en edades tempranas. Los desórdenes ligados al cromosoma X clásicamente afectan sólo a varones. Presentamos el caso de una mujer de 10 años de edad, con el espectro completo de la patología. MAVC 0.7 AO. En la tomografía de coherencia óptica (TCO) presentaba alteración foveal bilateral de aspecto quístico. En el estudio genético se identifica la variante c.644A>T (p.Glu215Gly) en el gen RS1 en homocigosis, asociada a retinosquisis con modo de herencia recesiva ligada al X. La RXL es una condición que tiene una gran variedad en la severidad de la enfermedad y no existe correlación entre esta última y la progresión de la patología. La enfermedad ha sido descrita en un limitado número de mujeres principalmente en familias con alto grado de consanguinidad (AU)

X-linked retinoschisis (XLR) is a cause of retinal degeneration that affects males at an early age. X-linked disorders classically affect only males. We present the case of a 10-year-old female with the full spectrum of the pathology. BCVA 0.7 OU. Optical coherence tomography (OCT) showed bilateral foveal alteration with cystic appearance. The genetic study identified the variant c.644A>T (p.Glu215Gly) in the RS1 gene in homozygosis, associated with retinoschisis with X-linked recessive mode of inheritance. XLR is a condition that has a great variety in the severity of the disease and there is no correlation between the latter and the progression of the pathology. The disease has been described in a limited number of females mainly in families with high degree of consanguinity (AU)

Parálisis oculomotoras en el contexto de una reactivación del virus varicela-zóster: a propósito de 4 casos / Oculomotor nerve palsy in herpes zoster ophthalmicus: Presentation of 4 cases

La infección por el virus varicela-zóster (VVZ) tiene una alta prevalencia a nivel mundial. De las muchas manifestaciones oftalmológicas que puede manifestar, la diplopía de aparición brusca es una de ellas. Revisamos 4 casos clínicos de mononeuritis del III y VI par craneales en el contexto de vesículas herpéticas por el VVZ y revisamos la fisiopatología y las manifestaciones clínicas más importantes. Es obligado para el oftalmólogo descartar complicaciones mediante pruebas de imagen y el correcto tratamiento con antivirales sistémicos

Varicella zoster virus (VZV) infection has a high prevalence worldwide. Within the multiple ophthalmologic manifestations that VZV can cause, sudden diplopia is among them. A review is presented of four clinical cases of 3rd and 4th oculomotor nerve palsies in herpes zoster ophthalmicus. A review is also presented of the physiopathology and most important clinical manifestations. Imaging tests are essential in order to rule out complications. Systemic antiviral administration is the correct treatment