Diffuse Cerebral Edema and Impending Herniation Complicating Hepatic Encephalopathy in Hereditary Hemorrhagic Telangiectasia

Charisma Mylavarapu, Alexander J. Lu, Ethan A. Burns, Justin Samorajski, Deepa Gotur, Kelty Baker

Research output: Contribution to journalArticlepeer-review

Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease characterized by the formation of cutaneous and visceral telangiectasias and arteriovenous malformations (AVM). Multiple organs may be affected, including the nasal mucosa, skin, lungs, gastrointestinal tract, and brain. The following case highlights a unique manifestation of HHT in a patient with a gastrointestinal hemorrhage and epistaxis, resulting in hyperammonemia and diffuse cerebral edema and herniation. Clinicians should be aware of this potential complication in such patients and initiate ammonia-reducing agents early to avoid this devastating consequence.

Original languageEnglish (US)
Article number2612544
JournalCase Reports in Medicine
Volume2022
DOIs
StatePublished - 2022

ASJC Scopus subject areas

  • Medicine(all)

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