Pseudotumor cerebri presenting with visual failure in promyelocytic leukemia: a case report

Pseudotumor cerebri secondary to all-trans retinoic acid in acute promyelocytic leukemia is a reported but rare complication of the therapy. Most cases improve following the discontinuation of all-trans retinoic acid. There is no published literature on how to manage such patients if severe symptoms of increased intracranial pressure continue after discontinuation of the drug.

We report the case of a 16-year-old Afro-Caribbean woman with aggressive secondary pseudotumor cerebri who presented to our facility with visual failure that persisted despite discontinuation of all-trans retinoic acid. A lumbar drain was inserted for 11 days resulting in symptomatic relief of headaches and objective improvement of visual failure. Pressure settings were titrated regularly to ensure optimal symptomatic relief.

The use of a lumbar drain for continuous drainage of cerebrospinal fluid in patients with all-trans retinoic acid-induced pseudotumor cerebri resistant to all-trans retinoic acid discontinuation is a feasible management option. This method can be used when other less invasive measures have failed to improve signs and symptoms. Permanent drainage of cerebrospinal fluid with a shunt may also provide a long-term viable management strategy but the use of a lumbar drain may be preferable if the cause of pseudotumor cerebri is known to be self-limiting.