Neurorretinitis estrellada idiopática de Leber: conflictos diagnósticos y terapéuticos

Resumen

Presentamos 3 pacientes con clínica de disminución de agudeza visual, papilitis y desarrollo de estrella macular al cabo de unas semanas. En todos ellos, las pruebas complementarias arrojaron resultados irrelevantes. Dado este contexto, fueron diagnosticados de neurorretinitis estrellada idiopática de Leber (NEIL) y se pautó tratamiento con corticoides, así como antibióticos en 2 de ellos. Evolucionaron favorablemente, aunque con signos de atrofia papilar en los ojos afectos.

El diagnóstico de la neurorretinitis estrellada idiopática de Leber puede resultar complejo, dado el amplio espectro de enfermedades que descartar. A esto se suma un período de tiempo en el que puede no haber aparecido la estrella macular, motivo que no debe hacer excluir su diagnóstico. El uso de antibióticos o corticoides como medida terapéutica es controvertido dado su buen curso clínico, aunque parece razonable en casos severos. Otro punto controvertido es el uso rutinario de pruebas complementarias, principalmente aquellas que pueden resultar agresivas o caras, que deben ser utilizadas de forma racional.

Abstract

Three cases of patients with decreased visual acuity and papillitis at onset with subsequent macular star development after a few weeks are presented. Complementary tests were unremarkable in all included patients. Based on this clinical context, they were diagnosed with Leber's idiopathic stellate neuroretinitis, were treated with corticosteroids, as well as with antibiotics in 2 cases. All patients showed favourable outcomes, although signs of papillary atrophy were observed in the affected eyes.

Leber's idiopathic stellate neuroretinitis diagnosis can be challenging due to the wide spectrum of conditions that have to be ruled out. In addition, macular star may appear later on, which should not exclude its diagnosis. Use of antibiotics and/or corticosteroids is controversial considering its benign nature, but should be considered in selected severe cases. Moreover, the routine use of complementary tests should be carefully evaluated, mainly those that can be aggressive and/or expensive, which should be rationally used.