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Benign Intracranial Hypertension in Infants With Cystic Fibrosis

Peter Bikangaga, MD, FRCPC; Gerard J Canny, MD, FRCPC
Arch Pediatr Adolesc Med. 1996;150(5):551-552. doi:10.1001/archpedi.1996.02170300105022.
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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


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