Çocuklarda Skleral Melanositoz
Özet
Skleral melanositoz, çocukluk çağında sıkça karşılaşılan, skleranın gri-mavi veya siyah renkte konjenital melanositik hiperpigmentasyonu olarak tanımlanmaktadır. Mezoderm ve nöral krest kökenli fibröz bir yapı olan sklera, gözü koruyan dış katmandır. Melanositik lezyonlar; dendritik, nevüs veya fuziform melanositlerden oluşur ve melanositoz fuziform tipte yer alır. Özellikle Asya toplumlarında yaygın görülen bu durum, Çin'deki çalışmalara göre 6 yaş civarında pik yapmakta, çoğunlukla bilateral seyretmekte ve ergenlik sonrası sıklığı azalabilmektedir. Ayırıcı tanıda; malign transformasyon riski taşıyan, trigeminal sinir boyunca pigmentasyon yapan Ota nevüs, episkleral boşlukta pigment birikimi olan Axenfeld sinir halkası ve nadir görülen mavi nevüsler dikkate alınmalıdır. Ota nevüs, skleral melanositozdan farklı olarak alacalı pigmentasyon ve glokom riski ile ayrılır. Çocukluk döneminde pigmentli skleral lezyonlarda malignite riski düşük olsa da, morbi̇di̇te ve mortaliteyi azaltmak adına hekimlerin dikkatli bir klinik takip yapması ve melanositik lezyonlar konusunda farkındalık sahibi olması kritik önem taşır.
Scleral melanocytosis is defined as a congenital melanocytic hyperpigmentation of the sclera appearing in gray-blue or black colors, frequently encountered in childhood. The sclera, a fibrous structure of mesoderm and neural crest origin, is the outer layer that protects the eye. Melanocytic lesions consist of dendritic, nevus, or fusiform melanocytes, with melanocytosis categorized under the fusiform type. Particularly common in Asian populations, this condition peaks around age 6 according to studies in China, is mostly bilateral, and its frequency may decrease after adolescence. In differential diagnosis, the Ota nevus, which carries a risk of malignant transformation and causes pigmentation along the trigeminal nerve, the Axenfeld nerve loop characterized by pigment accumulation in the episcleral space, and rare blue nevi must be considered. Ota nevus differs from scleral melanocytosis by its mottled pigmentation and risk of glaucoma. Although the risk of malignancy in pigmented scleral lesions during childhood is low, it is critically important for physicians to conduct careful clinical follow-up and maintain awareness regarding melanocytic lesions to reduce potential morbidity and mortality.
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