Neovascular Glaucoma Secondary to Bloch-Sulzberger Syndrome Documented by Retinography and Wide Field Angiography

Aims: To describe a Neovascular Glaucoma Secondary to Bloch-Sulzberger Syndrome.Presentation of Case: A.B.F. female, 23 years old, claimed significant pain sensation and hyperemia in the left eye (LE) for 45 days, without triggering factors. She came to the consultation with a previous diagnosis of Bloch-Sulzberger Syndrome with pigmented lesions in the lower limbs since childhood, being followed up by dermatology since then.Discussion: The diagnosis of IP (Incontinentia Pigmenti) is complex because it mimics other dermatoses. The impairment in other systems, such as the ophthalmic system, with the possibility of loss of vision and functional disability is a reality. The natural history of retinal lesions is not fully understood. Vascular changes seem to start in the first weeks of life and progress after birth for weeks or months, and may stabilize at any stage, leaving numerous sequelae.Conclusions: Incontinentia pigmenti is difficult to diagnose and, at each evolutionary stage, presents many differential diagnoses. Eye injuries can lead to blindness as a consequence of ischemic events that can start soon after birth.

Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria

Aims: To describe a Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria.Presentation of Case: A 25 years old, male, student, in regular follow-up in the Hematology sector due to Paroxysmal Nocturnal Hemoglobinuria in regular use of Eculizumab. He reports a month ago that he suddenly noticed, upon waking up in the morning, blurring and decreased visual acuity in her left eye.Discussion: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of glycosylphosphatidylinositol-anchored (GPI-anchored) complement inhibitors. Most commonly, PNH is caused by the loss of PIGA function, which is necessary for GPI biosynthesis.Conclusions: Patients with hemolytic anemia, unexplained thrombosis, especially in uncommon sites, cytopenias and bone marrow failure syndromes, dysphagia, and unexplained abdominal pain should be screened for PNH. PNH patients can benefit greatly from the treatments available, with a reduction in the risk of serious sequelae and a considerable improvement in their quality of life. So that the patient continues to be followed up in the ophthalmology department, with decreased visual acuity ipsilateral to the lesion, with a relative afferent pupillary defect on that side.

Congenital Ocular Melanocytosis: Case Report

Aims: To describe Congenital Ocular Melanocytosis.Presentation of Case: LPC, 7 years old, male, brown, with no previous comorbidities, was taken to the ophthalmology outpatient clinic of the Hospital Universit醨io Ant鬾io Pedro, Brazil by parents who alleged the presence of bluish-looking lesions in the sclera of the child's right eye since birth.Discussion: Congenital Ocular Melanocytosis is a rare pathology characterized by an increase in the number, size and pigmentation of melanocytes. Its pathophysiological picture is unknown, but it is believed to be due to an alteration in the migration of melanocytes from the neural crest to the epidermis during the embryonic process. This condition can be complicated by glaucoma and uveal melanoma. Gonioscopy is essential in these cases to assess whether there is pigmentation of the trabeculae, so that the propaedeutics of investigation of glaucoma becomes essential in these patients, since 10% of cases can complicate this condition.Conclusions: Congenital Ocular Melanocytosis early in life and the importance of monitoring these patients should be emphasized. Comprehensive tests are important for early detection and treatment, in order to improve the prognosis and avoid more severe consequences than what can happen from melanocytosis.