Cortical Visual Impairment as a Manifestation of Hepatic Encephalopathy in a Young Child: A Case Report

Abstract

Cortical visual impairment (CVI) is an extremely rare manifestation of hepatic encephalopathy (HE), which refers to neuropsychiatric abnormalities as a result of impaired detoxification function of liver. A 16-month-old female Syrian refugee who developed jaundice 4 weeks before referral to our hospital was diagnosed with hepatitis A based on her clinical presentation at a local facility. Overtime, her jaundice worsened, her lower limbs became edematous, and the family noticed sleep disturbances and irritability. On admission to a local hospital, she was deeply jaundiced and irritable, but showed no stigmata of chronic liver dysfunction. During hospitalization, the patient lost the ability to track objects and eventually her sight. However, the results of fundoscopic and neurological examinations were normal, except for a fine tremor. Her laboratory workup showed elevated liver enzymes and ammonia, and a prolongation of prothrombin time. Brain imaging revealed an abnormal signal intensity which is suggestive of metabolic etiology. The patient was diagnosed with CVI secondary to HE and transferred to the pediatric intensive care unit. After a few days of HE management, ammonia levels dropped and vision recovered. In conclusion, CVI may indicate HE in children.