Anesthetic Management of a Juvenile Hyaline Fibromatosis Patient With Trismus and Cervical Movement Limitation

Anesth Prog. 2021 Jun 1;68(2):117-118. doi: 10.2344/anpr-68-01-04.

Abstract

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease characterized by the presence of tissue nodules, joint contractures, and gingival hyperplasia. With a 1-year-9-month-old female patient scheduled for a gingivectomy and excision of a lower lip mass under general anesthesia, it was anticipated that airway management would be difficult because of trismus and limited cervical movement. Intubation with video-laryngoscopic assistance could not be achieved because gingival hyperplasia and trismus prevented blade insertion and manipulation. Therefore, 2 endotracheal tubes were used: 1 used as a nasopharyngeal airway for assisted ventilation, and 1 used for intubation along with a flexible fiberoptic scope. This case demonstrated a useful method for managing ventilation and intubation in patients with JHF, particularly when the use of oral airway devices is difficult.

Keywords: Difficult intubation; Fiberoptic scope; Juvenile hyaline fibromatosis; Nasotracheal intubation; Trismus.

MeSH terms

  • Anesthetics*
  • Female
  • Humans
  • Hyaline Fibromatosis Syndrome*
  • Infant
  • Trismus / etiology
  • Trismus / therapy

Substances

  • Anesthetics