Three Cases of Bilateral Congenital Spear Cataract Involvement in Three Members of the Same Family

Abstract

GirişKonjenital katarakt doğumda var olan lens opasiteleridir ve dünyada çocuklarda önlenebilir kör-lük nedenlerinden en sık görülenidir (1, 2). Dolayısıyla zamanında teşhis edilip tedavisinin dü-zenlenmesi önemlidir. Biz de literatürde çok sık görülmeyen otozomal dominant geçişli mızrak katarakt tanılı hastaların çocuklarının da göz muayenelerinin ayrıntılı yapılıp, katarakt dışında ambliyopi ve/veya şaşılık açısından da değerlendirilmesi gerektiğini düşünerek anneanne, anne ve kızından oluşan bir ailedeki bilateral mızrak katarakt olgularını sunmak istedik. Olgu SunumuOlgu 1: 79 yaşında bayan hasta. Sağ ve sol görmesi gözlük ile 0,3 logMAR unit olarak saptandı. Ön segment muayenesinde bilateral, uçları sivri, mızrak şekilli, lens nükleusundan lens korteksine doğru uzanmış kristal gibi parlayan lens opasiteleri saptandı (Resim 1). Fundus doğaldı. Göz içi basınçları normal seviyede idi. Çocukluktan beri aynı düzeyde görmesi olduğunu belirtti. Katarakt operasyonu düşünülmedi. Hasta takibe alındı. The aim of this study was to present bilateral spear cataracts in a grandmother, mother, and daughter of the same family. A 79-year old female patient. The best corrected visual acuity was 0.3 logMAR units in the right and left eyes. An anterior segment examination revealed a bilateral spear cataract. The posterior segment was normal. Intraocular pressure was within the normal range. As the patient said that she has had the same level of vision acuity since childhood, cataract surgery was not considered. Follow-up was recommended. A 45-year-old female presented to our clinic for a routine eye examination. The best corrected visual acuity was 0.4 logMAR units in the right eyes and 0.1 logMAR units in the left eye. During the slit lamp examination, bilateral congenital spear cataracts were detected. Bilateral intraocular pressure levels were within the normal range. An 8-year-old female presented to our clinic for a routine eye examination. Evaluation of crystalline lenses revealed bilateral congenital spear cataracts. After cycloplegic examination was performed, refractive errors in the right and left eyes were detected. These were (+1.00 α 90°) and +1.00 (-2.50 α 5°), respectively. The best corrected visual acuity was found to be 0.05 logMAR units in the right eye and 0.2 logMAR units in the left eye. Against the possibility of amblyopia of the left eye, patching of the right eye and follow-up were recommended. Opacities involve the outer layer of the embryonic nucleus and inner layer of the infantile nucleus. It is thought that they are crystals containing tyrosine and calcium sulfate. They disturb the vision and are prone to carry an autosomal dominant trait. Our cases comprise a grandmother, mother, and daughter of the same family. Due to this type of cataract, a high rate of lenticular astigmatism comorbidity is commonly seen. Keywords: Congenital cataract, spear cataract, genetic switchAynı aileden üç olguda konjenital bilateral mızrak katarakt olgusunu sunmayı amaçladık. 79 yaşında bayan hasta. Sağ ve sol görmesi g...