Título

PAPILLOPHLEBITIS IN CONGENITAL PRIMARY LYMPHOEDEMA : CASE REPORT

Objetivo

Describe a case of papillophlebitis in a woman with congenital primary lymphoedema.

Relato do Caso

A 29-year-old woman previously diagnosed with congenital primary lymphoedema developed papillophlebitis in her left eye. She had a previous Optical Coherence Tomography (OCT) scan showing macular swelling, previously treated with three intravitreal injections, resulting in documented progression to foveal cysts. She consulted in our service with a complaint of visual acuity loss in her left eye. Fundoscopy showed retinal hemorrhages and increased venous tortuosity and dilatation. She underwent one intravitreal injection with dexamethasone. The visual acuity improved from 20/60 to 20/40. OCT maintained discrete perifoveal cysts.

Conclusão

Papillophlebitis is rare condition that may present as non-ischemic and incomplete central retinal vein occlusion.The disease is defined by venous congestion and optic disc edema, led by compression of the central retinal vein. Albeit poorly understood, these findings have been hypothesized to have an inflammatory pathophysiology. Meanwhile, primary lymphoedema is a condition associated with the pathological development of the lymphatic vessels, resulting in excessive retention of lymphatic fluid in the interstitial compartment. Chronic inflammation occurs in lymphoedema because of lymphatic congestion and lack of oxygenation. The underlying vascular condition might have triggered the papillophlebitis in this case.  To our knowledge, this is the first report describing papillophlebitis in a patient with diagnosis of congenital primary lymphoedema.