Clinical Reasoning: An unusual case of papilledema after orthotopic liver transplantation

Abstract

A 59-year-old, right-handed woman presented with a 7-day history of insidious onset of progressive blurring of vision of the right eye. She had undergone orthotopic liver transplantation 15 months previously due to non-A non-B fulminant hepatic failure. Her postoperative period was complicated by 2 episodes of acute rejection which responded to methylprednisolone, daclizumab, and change of immunosuppressive therapy from tacrolimus to cyclosporine. Three months prior she had developed nonspecific symptoms of weight loss, malaise, and anorexia. On examination she was alert and oriented. Visual acuity was 6/60 on the right and 6/36 on the left. Color vision was impaired bilaterally as tested with the Ishihara pseudoisochromatic plates. There was enlargement of both blind spots and severe constriction of fields with relative preservation of inferior nasal fields only. Ophthalmoscopic examination and fluorescein angiography showed bilateral pale swollen optic discs with peripapillary hemorrhages and venous engorgement (figure). The remaining neurologic examination was normal. Figure Photographs of the optic disc of the right and left eyes, showing bilateral pale swollen discs with flame-shaped hemorrhages and venous engorgement of the vessels ### Questions for consideration: 1. What are the possible differential diagnoses? 2. Which initial investigations would you recommend? The initial differential diagnosis for optic neuropathies with disc swelling included raised intracranial pressure, malignant hypertension, bilateral compressive thyroid ophthalmopathy, bilateral optic nerve tumors, bilateral simultaneous anterior ischemic optic neuropathy, idiopathic intracranial hypertension, and infective and inflammatory conditions. Twenty-four–hour ambulatory blood pressure monitoring was normal. Free thyroxin and thyroid stimulating hormone levels were normal. Thyroid antibodies were negative. Inflammatory markers were normal. Gadolinium-enhanced MRI of brain, orbits, pituitary, and optic nerves revealed increased signal uptake in the left optic nerve only, excluding …