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Scientific article
English

Intracranial hypertension and papilledema in a large cohort of pediatric patients with alagille syndrome

ContributorsRock, Nathalie; Demaret, Tanguy; Stéphenne, Xavier; Scheers, Isabelle; Smets, Francoise; Mclin, Valérie Anne; Boschi, Antonella; Sokal, Etienne M
Published inJournal of Pediatric Gastroenterology and Nutrition, vol. 71, no. 5, p. 655-662
Publication date2020
Abstract

Ophthalmic abnormalities are amongst the 5 major criteria required for a diagnosis of Alagille syndrome (ALGS), of which embryotoxon, pseudopapilledema, and hypopigmented retinopathy are the most common. Papilledema with or without intracranial hypertension (ICHT) is rarely described. We report 9 pediatric cases of ALGS with bilateral papilledema, 5 of which were diagnosed with ICHT.

Keywords
  • Alagille Syndrome/complications/diagnosis
  • Child
  • Eye Diseases
  • Hereditary/complications/diagnosis
  • Humans
  • Intracranial Hypertension/complications/diagnosis
  • Optic Nerve Diseases
  • Papilledema/etiology
Affiliation entities
Research groups
Citation (ISO format)
ROCK, Nathalie et al. Intracranial hypertension and papilledema in a large cohort of pediatric patients with alagille syndrome. In: Journal of Pediatric Gastroenterology and Nutrition, 2020, vol. 71, n° 5, p. 655–662. doi: 10.1097/MPG.0000000000002883
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Article (Published version)
accessLevelRestricted
Identifiers
Journal ISSN0277-2116
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