Table 5.

Treatment of Manifestations in Individuals with Retinal Vasculopathy with RVCL-S

Manifestation/ConcernTreatmentConsiderations/Other
Visual impairment Retinal laser therapyCan prevent & slow down progression of retinal involvement that causes visual impairment
Macular edema Bevacizumab
Elevated eye pressure /
Glaucoma 		Standard treatment
Hypertension Standard treatmentMay prevent further damage to involved organs
Renal insufficiency Standard treatment of hypertensionRefer to nephrologist.
Renal replacement therapy (incl transplantation) may be considered in severe cases for those w/end-stage renal disease.
Liver function abnormalities There is no treatment for liver function abnormalities.Monitor to prevent complications associated w/liver fibrosis & to exclude other causes. 1
Cerebral vascular
edema 		Consider corticosteroid therapy. 2May reduce cerebral vasogenic edema but does not appear to treat the underlying lesion
Migraine Standard treatment
Seizure disorder Standard treatment w/antiepileptic drugsRefer to neurologist.
Hypothyroidism Thyroid replacement therapy
Anemia Standard treatmentIntravenous iron therapy (& in rare cases blood transfusion) may be necessary.
Raynaud phenomenon Standard treatment
Psychiatric concerns Standard treatmentRefer to psychiatrist or neuropsychologist.
1.

When not properly monitored, affected individuals are frequently given incorrect diagnoses and often receive advice to stop drinking alcohol or stop their medication, while this is not required.

2.

Intravenous methylprednisolone followed by an oral corticosteroid treatment

From: Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations

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