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.

Multifocal Best's disease: The importance of genetic tests. / Enfermedad de Best multifocal: la importancia de los test genéticos.

OBJECTIVES AND METHODS: Our objective is to describe a multifocal vitelliform presentation of Best's disease. The lesions in this disease may vary in size and shape, some may be a disc diameter in size, and often have some irregularity in their contour. The case is described of a 21-year-old male patient referred to our ophthalmology department due to a progressive loss of vision. His poor visual acuity was confirmed, and a complete examination was performed, in which macular flecks were observed, with yellow pigment arranged in oval distribution near their periphery. Due to the suspicion of Best's multifocal disease, genetic tests were performed. Multifocal vitelliform disease with the same features as those in Best's disease occurs most frequently in patients with a normal electro-oculogram (EOG), and a normal family history. CONCLUSION: Best's multifocal disease must be suspected in case of multiple vitelliruptive lesions close to the posterior pole. Genetic testing is essential for its diagnosis.

[Webino syndrome caused by meningovascular syphilis. A rare entity with an unexpected cause]. / Síndrome de webino secundario a sífilis meningovascular. Una entidad excepcional con una etiología inesperada.

CASE REPORT: The patient is a 57-year-old obese and hypertensive male. His chief complaints were double vision and dizziness, with mild exodeviation in both eyes in primary gaze position in the ocular motility examination, but more predominant in the left eye. The exotropia was noticeably more evident on the attempted upgaze. On horizontal gaze, the abducting eye deviated fully, but the adducting eye did not cross the midline. Nystagmus in the abducting eye and convergence impairment were found. Pupil size and testing were normal. Ataxia and areflexia were also present. Bilateral internuclear ophthalmoplegia was suspected and imaging and laboratory tests were performed. The CAT scan showed a right occipital hypo-attenuated lesion. In the MRI scan, a mesencephalic subacute ischemic lesion was found, involving the medial rectus sub-nuclei. Blood and cerebrospinal fluid test for syphilis were positive. DISCUSSION: Bilateral internuclear ophthalmoplegia is a very uncommon -and difficult to diagnose- condition. In the reported case the lesion involved the medial rectus sub-nuclei. This fact could explain the exotropia in the primary gaze position, and supports that is not possible to exclude the involvement of the medial rectus sub-nuclei in the webino syndrome. The rapid identification of the pathology contributed to the better prognosis of the patient.

[Optical coherence tomography in vitelliform macular dystrophy type 2]. / Tomografía de coherencia óptica en la distrofia macular viteliforme tipo 2.

CASE REPORT: We report the case of a 10-year-old boy with a progressive bilateral vitelliform macular dystrophy, and his father with terminal stage disease in both eyes; we studied the development and progression of this condition with optical coherence tomography. DISCUSSION: Optical coherence tomography is a useful noninvasive tool that complements other diagnostic modalities and improves the follow up assessment. It provides additional information on the morphology of the lesion as well as identifying secondary changes in the adjacent retina. It also demonstrates the location of any yellowish material under the sensory retina.

[Sialidosis type I. Two cases in a family]. / Sialidosis tipo I. A propósito de dos casos en una familia.

CASE REPORT: We present two cases of sialidosis type 1 in two brothers, 28 and 30 years of age. Both have seizures and severe gait instability. Ophthalmoscopic examination showed a cherry-red spot in both eyes. Fibroblast culture revealed a marked reduction in neuraminidase with no alteration in galactosidase, confirming that our patients have sialidosis type I. CONCLUSIONS: We highlight the ease with which this rare syndrome can remain undetected and how a simple ophthalmoscopic examination is able to reveal the diagnosis.