Javier Mateo

Watchmakers’ glaucoma: Watchmakers’ glaucoma

We present the first reported case of an ‘apparent’ unilateral glaucoma in the eye used by a watchmaker with a monocular loupe. We hypothesise that it was caused by the pressure from the orbicularis oculi muscle contraction associated with holding it in position for long periods of time. We believe that users of this kind of optical instrument should avoid wearing it always in the same eye or using it for long periods of time and should be monitored closely by routine ophthalmic examinations.

Unilateral tacrolimus-associated optic neuropathy after liver transplantation

Background: Tacrolimus has been associated with several ocular adverse effects, such as optic neuropathy. Methodology/principal findings: A 56-year-old woman noted sudden, severe, painless visual loss in her left eye. She had undergone liver transplantation for alcoholic related cirrhosis 6 months before. Her chronic immunosuppressive regimen consisted of prednisone and tacrolimus at dosage of 1.5 mg orally once daily. Consequently, the patient developed a left optic neuropathy. Conclusion/significance: We report the first case of unilateral optic neuropathy associated with oral tacrolimus medication. Surgeons and ophthalmologists must evaluate ocular symptoms in the post-transplantation period, and suspicion should be maintained even if unilaterality or asymmetry of symptoms against a toxic etiology.

The contribution of optical coherence tomography in neuromyelitis optica spectrum disorders

Neuromyelitis optica spectrum disorders (NMOSD) comprises a group of central nervous system disorders of inflammatory autoimmune origin that mainly affect the optic nerves and the spinal cord and can cause severe visual and general disability. The clinical signs are similar to those of multiple sclerosis (MS), with the result that it is often difficult to differentiate between the two, thus leading to misdiagnosis. As the treatment and prognosis of NMOSD and MS are different, it is important to make an accurate and early diagnosis of NMOSD. Optical coherence tomography (OCT) is a non-invasive technique that enables a quantitative study of the changes that the optic nerve and the macula undergo in several neurodegenerative diseases. Many studies have shown that some of these changes, such as retinal nerve fiber layer thinning or microcystic macular edema, can be related to alterations in the brain due to neurodegenerative disorders. The purpose of this mini-review is to show how OCT can be useful for the diagnosis of NMOSD and follow-up of affected patients, as well as for the differential diagnosis with MS.

Other Neurological Disorders: Migraine, Neurosarcoidosis, Schizophrenia, Obstructive Sleep Apnea-Hypopnea Syndrome (OSAHS)

Optical coherence tomography (OCT) is a relatively recent, reproducible, noninvasive, noncontact in vivo imaging technique similar to ultrasonography except that it uses infrared wavelengths and has a sensitivity of 8–10 μm. Numerous studies have demonstrated a thinning in retinal nerve fiber layer (RNFL) measurements in neurodegenerative disorders including multiple sclerosis, Alzheimer’s disease, and Parkinson’s disease. In this chapter we present an update on the use of OCT in other neurological disorders, such as migraine, neurosarcoidosis, schizophrenia and obstructive sleep apnea-hypopnea syndrome (OSAHS).

Ophthalmological Manifestations in Acute Lymphoblastic Leukemia

The aim of the present chapter is to review the different ophthalmological signs and symptoms that can be observed in acute lymphoblastic leukemia and the importance of the examination of these patients by an ophthalmologist whenever an ocular affectation is suspected. Acute lymphoblastic leukemia is a malignant neoplasm caused by the proliferation of poorly differenciated precursors of the lymphoid cells, which are known as blast cells. Blast cells replace the normal elements of the bone marrow, decreasing the production of normal blood cells and, therefore, causing anemia, thrombocytopenia and neutropenia. They can also infiltrate other organs, such as liver, spleen, lymph nodes or, less frequently, central nervous system. (Florensa et al, 2006; Ribera & Ortega, 2003; Sharma et al, 2004) Acute lymphoblastic leukemia is the most common type of leukemia in children, although it is also seen in adult patients. If blood test results are abnormal or the doctor suspects leukemia despite normal cell counts, a bone marrow aspiration and biopsy are the next steps. Treatment is based on chemotherapy, radiotherapy and bone marrow transplantation. (Ribera & Ortega, 2003; Ortega, 2006; Ribera, 2006) The dominant clinical feature of these diseases is usually bone marrow failure caused by accumulation of blast cells although any organ can be infiltrated. Furthermore, signs and symptoms of acute lymphoblastic leukemia can be secondary to the toxicity of chemotherapy and/or radiotherapy, graft versus host reaction following bone marrow transplantation, or infections due to immunosuppression. They can include fever, weakness, fatigue, breathlessness, opportunistic infections, weight loss, anorexia, easy bruising and bleeding, thrombosis, edema of the lower limbs and the abdomen, swollen liver or spleen, lymphadenopathy, or bone pain. (Florensa et al, 2006; Ribera & Ortega, 2003; Ortega, 2006) Ophthalmological signs in patients suffering from leukemia were first described as “leukemic retinopathy” by Liebreich in 1863. (Campos-Campos et al, 2004; Guyer et al, 1989) Reports of patients with acute lymphoblastic leukemia presenting with visual symptoms as the initial sign of the disease are rare (Kim et al, 2010). However, ocular changes in acute lymphoblastic leukemia are common. They have been reported to occur in up to 90% of patients with this disease (Kincaid & Green, 1983; Mesa, 2003).

Fundus autofluorescence: the key in the diagnosis of maternally inherited diabetes and deafness

We report an interesting case of maternally inherited diabetes and deafness (MIDD), in which fundus autofluorescence aided in the diagnosis. MIDD is a rare genetic disorder, maternally transmitted, characterised by a point mutation of the mitochondrial DNA (mtDNA) at position 3243 that disrupts the mitochondrial respiratory chain and thus, affects metabolically active organs such as pancreatic islets, cochlea or retina. It has an average age onset of 34–40 years and accounts for 0.5 to 2.8 per cent of all cases of diabetes. In more than 80 per cent of cases, it is associated with bilateral macular pattern dystrophy.

Effect of mitochondrial haplogroups on ranibizumab response in neovascular age-related macular degeneration patients: a pilot study

of 52 lm became apparent within the first year and remained at 250 102 lm on average in year 5 (Fig. 1E). We report on a representative number of eyes complicated by nAMD and treated with a personalized PRN regimen for at least 5 years without discontinuation. Moderate visual decline was achieved through an individualized monitoring and retreatment scheme based on empiric variable intervals for each patient. Advantages of this strategy imply a direct response to activity independent of an early or late stage disease while reducing expenses of antiVEGF treatment and maximizing safety. Our adopted method – though reactive in treatment modality – is somewhat between monthly PRN and proactive treat and extend. Only a few comparative reviews have been published until today with short-term efficacy outcomes (Chin-Yee et al. 2016; Danyliv et al. 2017). In conclusion, our real-world data confirm the potential benefit of a personalized PRN regimen. It combines a fairly low injection rate with an acceptable clinical effort.

Optical Coherence Tomography in Patients with Chronic Migraine: Literature Review and Update

Migraine is a chronic disease characterized by unilateral, pulsating, and often moderate-to-severe recurrent episodes of headache with nausea and vomiting. It affects approximately 15% of the general population, yet the underlying pathophysiological mechanisms are not fully understood. Optical coherence tomography (OCT) is a safe and reproducible diagnostic technique that utilizes infrared wavelengths and has a sensitivity of 8–10 μm. It can be used to measure thinning of the retinal nerve fiber layer (RNFL) in some neurological disorders. Although ophthalmologists are often the first specialists to examine patients with migraine, few studies have addressed the involvement of the optic nerve and retino-choroidal structures in this group. We reviewed the literature on the etiological and pathological mechanisms of migraine and the relationship between recurrent constriction of cerebral and retrobulbar vessels and ischemic damage to the optic nerve, retina, and choroid. We also assessed the role of OCT for measuring peripapillary RNFL thickness and macular and choroidal changes in migraine patients. There is considerable evidence of cerebral and retrobulbar vascular involvement in the etiology of migraine. Transitory and recurrent constriction of the retinal and ciliary arteries may cause ischemic damage to the optic nerve, retina, and choroid in patients with migraine. OCT to assess the thickness of the peripapillary RNFL, macula, and choroid might increase our understanding of the pathophysiology of migraine and facilitate diagnosis of retino-choroidal compromise and follow-up of therapy in migraine patients. Future studies should determine the usefulness of OCT findings as a biomarker of migraine.

Jugular venous thrombosis secondary to idiopathic myelofibrosis: a rare cause of bilateral optic disc swelling.

We would like to report the very uncommon case of a bilateral optic disc oedema that appeared after the patient suffered a jugular vein thrombosis secondary to an idiopathic myelofibrosis. Idiopathic myelofibrosis is a chronic myeloproliferative disorder, in which the bone marrow is progressively substituted by connective fibrous tissue due to the elevated level of fibroblast growth factor, produced by megakaryocytes. Idiopathic myelofibrosis is clinically characterised by extramedullary haematopoiesis with hepatosplenomegaly, anaemia, weight loss, bone pain, infections or coagulopathy. The most common ocular findings in idiopathic myelofibrosis are retinal haemorrhages, although other signs can be found, depending on the blood-cell alterations. Bone marrow biopsy shows various degrees of fibrosis and nests of megakaryocytes. Patients usually receive symptomatic treatment and in some cases, chemotherapy.

Response to Re: Blinking abnormalities in watchmaker's glaucoma pathogenesis

the directly observable pulse patterns are synchronous with the cardiac pulse and are also related to blinking and eye movement. Each systole induces an expansion of choroidal volume in the closed space of the eye, producing a transient increase in IOP that has been estimated at 3.0 mmHg and voluntary blinking induces pressure increments of five to 10 mmHg. It is thought that aqueous outflow works as a pump, propelled by these physiological IOP spikes. Histological studies of Schlemm’s canal indicate that this aqueous pump may be due to the presence of one-way valves. This continuous physiological massage could also help to propel venous blood out of the eye and could have a direct role in optic nerve head nourishment. To understand the power of blinking we should take into consideration that it is estimated that a normal person blinks thousands of times each day. We hypothesise that wearing the loupe or developing a floppy lid could induce glaucoma by preventing these continuous and beneficial IOP changes. These pressure spikes are probably too short to induce optic nerve damage but may have a role in optic nerve nourishment or aqueous humour dynamics. Against this theory, it could be stated that chronic facial paralysis has not been linked to glaucoma; however, to the best of our knowledge, this topic has not been investigated. A study on the effect of chronic facial paralysis on the optic nerve fibres could cast some light upon this topic.