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May 1996

Benign Intracranial Hypertension in Infants With Cystic Fibrosis

Author Affiliations

Division of Pediatric Respiratory Medicine Department of Pediatrics The Hospital for Sick Children 555 University Ave Toronto, Ontario Canada M5G 1X8

Arch Pediatr Adolesc Med. 1996;150(5):551-552. doi:10.1001/archpedi.1996.02170300105022

A bulging fontanelle in an infant is a worrying finding. The major causes would include infections, hydrocephalus, intracranial bleeding, and other space-occupying lesions. Less ominous, but also to be considered in the differential diagnosis, is pseudo-tumor cerebri, which is characterized by symptoms and signs of increased intracranial pressure without evidence of a mass lesion or hydrocephalus.1,2 We report on four infants with cystic fibrosis (CF) in whom pseudo-tumor cerebri developed shortly after the initiation of therapy.

Report of Patients. During the 3-year period, 1990 through 1993, 53 infants were assigned a diagnosis of CF at The Hospital for Sick Children, Toronto, Ontario. Four (7.7%) of these infants had evidence of increased intracranial pressure after diagnosis and are described herein. A diagnosis of CF was established in each patient on the basis of two consecutively elevated sweat chloride levels.

Patient 1. A 4-month-old female infant was referred with a history