Tratamiento del síndrome de Duane vertical

Case reportWe report 3 cases with a vertical incomitance in upgaze, narrowing of palpebral fissure, and pseudo-overaction of both inferior oblique muscles. Surgery consisted of an elevation of both lateral rectus muscles with an asymmetrical weakening. A satisfactory result was achieved in 2 cases, whereas a Lambda syndrome appeared in the other case.DiscussionThe surgical technique of upper-insertion with a recession of both lateral rectus muscles improved vertical incomitance in 2 of the 3patients; however, a residual deviation remains in the majority of cases.     

Manifestaciones neurooftalmológicas del síndrome de POEMS

The cases is presented of a 38 year-old male with a constitutional syndrome, fever, multiple swollen lymph nodes, and hepatosplenomegaly of 2 months onset. There was also mention of headache, bilateral blurred vision, and myiodesopsias. Best correct visual acuity was 20/50 and 20/200. The anterior segment was unremarkable. The fundus of both eyes showed raised and erased discs, accompanied by serous detachment, greater in the left eye. Complementary studies of both eyes showed a visual field with increased blind spot; fluorescein angiography indicated late disc hyperfluorescence. The electroretinogram showed compromise of cones and rods, and the visual evoked potential detected alteration in the perception and conduction of stimuli. The imaging studies were essentially normal. During his hospital admission he presented with paraparesis, renal failure, endocrinopathy, skin alterations, and osteoblastic lesions with negative biopsies. Monoclonal gammopathy was documented, and the diagnosis of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes) syndrome was made. The chemotherapy started with dexamethasone/melphalan, with a good response at 3 months.     

Variante de la técnica de Nishida para la corrección quirúrgica del síndrome de déficit monocular de la elevación

Monocular elevation deficiency (MED) is characterized by unilateral limitation of supraductions, similar in adduction and abduction, in addition to hypotropia and ptosis. We describe a case of a 62-year-old woman with long-standing left ptosis who was initially operated with a frontal suspension technique. On subsequent examinations, a MED of that eye was found. The passive duction test was positive, so the inferior rectus was recessed. In addition, a modified Nishida technique was performed, consisting of the scleral anchorage of the superior edges of the rectus, medial and lateral, 12 mm from the corneal limbus in the superonasal and superotemporal quadrants, respectively. Postoperatively, the left eye presented a minimal hypotropia of 3 PD. This modified technnique used here on the horizontal rectus muscles, turned out to be effective and safe for the correction of MED.     

Síndrome de oftalmoplejía dolorosa,más allá del síndrome de Tolosa Hunt. Una serie de casos

Painful ophthalmoplejía syndrome is characterised by the presence of peri-orbital or peri-cranial pain that is accompanied by paralysis of the oculomotor nerves. The differential diagnosis is broad, and requires a rigorous study. Three clinical cases of patients with painful ophthalmoplejía syndrome are described, in which multiple extension studies were required to obtain an aetiological diagnosis. Painful ophthalmoplejía syndrome is a complex disorder that has multiple causes, including infections, inflammatory, and tumour processes. Biopsy should be considered in those cases in which non-invasive studies are inconclusive.     

Micropunción estromal anterior como tratamiento del síndrome de Brown-McLean

Case reportThe Brown-McLean syndrome is defined by peripheral corneal edema sparing the central cornea. We report a patient with bullous annular keratopathy following phacoemulsification and implantation of posterior chamber intraocular lens. The uncorrected and best corrected visual acuity was 20/30. The patient required the use of a bandage contact lens for almost 5 years to relieve the ocular discomfort.DiscussionAnterior stromal puncture was performed using a cystotome. Two weeks after the procedure the ocular discomfort disappeared and the use of bandage contact lens was no longer required. Anterior stromal puncture is a simple procedure that is performed under the slit lamp, and can alleviate the symptoms in patients with Brown-McLean syndrome.     

Diagnóstico clínico y genético de un caso de síndrome de Meretoja e intervención mediante lifting frontotemporal

A 56-year-old male with family background of corneal dystrophy presents with poor subjective vision. Biomicroscopy reveals bilateral reticular stromal dystrophy and facial inspection shows signs of muscle dysfunction, such as eyebrow ptosis, weakness and sagging of the frontal muscles, redundant skin on the forehead and skin hyperelasticity.The patient is referred to plastic surgery for evaluation of the frontal muscle involvement, undergoing a frontotemporal lifting procedure. On the other hand, genetics confirms the pathogenic variant c.640G>A (p.Asp214Asn) in the GSN gene, encoding gelsolin, a mutation associated with Finnish-type familial amyloidosis or Meretoja syndrome.     

Edad retiniana como biomarcador predictivo del grado de retinopatía diabética

ObjectiveTo apply artificial intelligence techniques, through deep learning algorithms, for the development and optimization of a system for predicting the age of a person based on a color retinography, and to study a possible relationship between the evolution of retinopathy diabetes (RD) and premature aging of the retina.MethodsA convolutional network was trained to calculate the age of a person based on a retinography. Said training was carried out on a set of retinographies of patients with diabetes previously divided into 3 subsets (training, validation and test). The difference between the chronological age of the patient and the biological age of the retina was defined as the retinal age gap.ResultsA set of 98,400 images was used for the training phase, 1000 images for the validation phase and 13,544 for the test phase. The retinal gap of the patients without RD was 0.609 years and that of the patients with RD was 1905 years (p < 0.001), with the distribution by degree of RD being: mild RD 1541 years; moderate RD 3017 years; RD severe 3117 years, and proliferative RD 8583 years.ConclusionsThe retinal age gap shows a positive mean difference between diabetics with RD versus those without RD, and it increases progressively, according to the degree of RD. These results could indicate the existence of a relationship between the evolution of the disease and premature aging of the retina.     

Cambios morfométricos de las células del endotelio corneal en el síndrome pseudoexfoliativo y glaucoma pseudoexfoliativo

ObjetiveTo evaluate the corneal endothelial morphometry and central corneal thickness (CCT) in pseudoexfoliative (PEX) eyes with and without glaucoma and to compare with normal eyes and eyes with primary open-angle glaucoma (POAG).MethodA total of 166 patients were included in this study: 36 eyes with pseudoexfoliation syndrome (PXS), 30 eyes with pseudoexfoliation glaucoma (PXG), 40 eyes with POAG, and 60 normal eyes. Corneal endothelial cell density (ECD), coefficient of variation (CV) in cell size, and percentage of hexagonal cells, were measured using a non-contact specular microscope, whereas CCT was measured with an ultrasonic pachymeter.ResultsECD and percentage of hexagonal cells were lower in PEX groups and in the POAG group compared with normal eyes, while the CV in cell size was greater. There was a tendency for greater cell loss and morphological abnormalities of the corneal endothelial cells in PXG eyes compared to PXS eyes, when all pseudoexfoliative eyes were analyzed together. Changes in endothelial cells increased with age. There were no significant differences in mean CCT between the four groups.ConclusionEndothelial cell density is significantly decreased, and pleomorphism and polymegathism of cells are increased in PEX eyes, particularly when intraocular pressure is high.     

Vasculitis retiniana y del disco óptico en el síndrome de Susac

Case Report

The case is presented of 42 year-old woman with no significant medical history, with severe headaches, nausea and vomiting, hearing loss, and alteration of mental status with disorientation and confusion. Ophthalmic examination showed optic disc hyperaemia in right eye, and focal areas of arteriolar occlusion in both eyes. Audiometry demonstrated bilateral neurosensory hypoacusis. Magnetic Resonance Imaging showed multiple small round hyperintense lesions located in the splenium, corpus callosum, basal ganglia, and white matter.

Síndrome de Alicia en el país de las maravillas

Case reportA case of Alice in Wonderland syndrome is described as the only sign of Epstein-Barr virus infection.DiscussionEpstein-Barr virus infection may include visual symptoms as the first or only signs of disease. All patients presenting with a clinical picture consistent with the Alice in Wonderland syndrome should undergo serological testing for Epstein-Barr virus infection.     

Neuropatía optica isquémica anterior no arterítica como primera manifestación de síndrome antifosfolipídico en un paciente joven

Case reportWe report the case of a young patient with unilateral anterior ischemic optic neuropathy, with no known cardiovascular risk factors and visual acuity preserved with positive anticardiolipin antibodies as a unique find.DiscussionNon-arteritic anterior ischemic optic neuropathy in the context of antiphospholipid syndrome is an uncommon finding, but it must be considered in the diagnosis of the atypical anterior ischemic optic neuropathy.     

Meibografía infrarroja y evaluación molecular de la mutación del gen p63 en un paciente mexicano con síndrome EEC

Objective

To report the finding of infrared meibography in a Mexican patient with EEC syndrome (Ectrodactyly–ectodermal dysplasia–cleft syndrome) confirmed by molecular analysis of the p63 gene.

Síndrome de Horner incompleto como signo de presentación de ependimoma del cuarto ventrículo

Case reportThe case of 44 year old male patient with palpebral ptosis and trigeminal neuralgia as presenting sign of fourth ventricle ependymoma is reported. After surgical treatment, the patient developed a residual paresis of the sixth cranial nerve.DiscussionHorner's syndrome occurs due to an alteration of the sympathetic innervations of the eye and adnexa. Some tumours may be the cause, in our case an ependymoma of the fourth ventricle, which onset exceptionally with blepharoptosis and involvement of the ophthalmic division of trigeminal nerve, due to the proximity of these nerve fibres at the brainstem.     

Alteraciones de la motilidad ocular en el síndrome de Kabuki

Kabuki syndrome is a rare genetic disorder, caused by mutation in the KMT2D or KDM6A genes, which affects several organs in the majority of patients, among which are the eyes. The most typical clinical characteristics are mental retardation, postnatal growth retardation, skeletal anomalies, and characteristic facial features. As the eyes are affected in most of the cases, ophthalmological examination is recommended for the early detection of ocular anomalies, in order to prevent visual impairment. The most frequent ocular signs are strabismus, ptosis, and refractive anomalies. A series of cases of Kabuki syndrome is described in five children, four of whom exhibited strabismus with esotropia, over action of inferior oblique muscles, and under action of superior oblique muscles associated with a V pattern. Most published papers do not report or might underestimate the ocular problems.It may be appropriate to perform orbital magnetic resonances in order to detect changes in the muscle paths that are related to the pathology of the eye movements found.     

Corticoterapia vs. corticoterapia con inmunosupresores en el tratamiento de la fase aguda del síndrome de Vogt-Koyanagi-Harada

Introduction

Vogt-Koyanagi-Harada disease is a multisystem disorder characterized by a bilateral granulomatous panuveitis. Multiple therapeutic regimens have been used to control inflammation in acute uveitic stage to prevent irreversible visual loss. The purpose of this paper is to compare the effect, on functional and anatomic outcomes, of early treatment with standard corticotherapy vs. corticotherapy plus immunosuppressive (IMT) therapy in acute Vogt-Koyanagi-Harada disease (Group A vs Group B).