More than a simple headache: pseudotumor cerebri in patients with sickle cell disease ▸

Children with sickle cell disease (SCD) often have headache, a symptom that can be related to the patient’s anemia or cerebrovascular disease. Headache can also be caused by pseudotumor cerebri (PC), or false brain tumor, which is characterized by the buildup of cerebrospinal fluid in the space surrounding the brain. PC is often associated with obesity, and untreated, can lead to blindness.

These authors describe three children with SCD who presented with headache. On ophthalmologic exam, bilateral papilledema was noted in all three patients, which led to a diagnosis of PC. The children received acetazolamide therapy, and symptoms resolved in two patients and improved in the third. None of the children had permanent visual deficits.

Comment ▸

Early recognition of papilledema in patients with SCD and headache can expedite treatment and prevent vision loss. We may tend to attribute all of a patient’s symptoms to his or her known diagnosis; in these children, such an assumption could have delayed diagnosis and resulted in loss of vision. This report highlights the importance of keeping one’s clinical and diagnostic skills in shape, even in an age of protocol-based clinical care. With increased childhood and adolescent obesity, PC may become more common in the pediatric population.

Susan Jay, MD, and Walter M. Jay, MD

Dr. Walter M. Jay is the John M. Krasa Professor of Ophthalmology at Loyola University Medical Center, Maywood, Illinois.

Published in Journal Watch Pediatrics and Adolescent Medicine May 10, 2004

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