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

• Published in: Anesthesia progress Vol. 68; no. 2; pp. 117 - 118
• Main Authors: Yasuda, Asako, Miyazawa, Noriko, Inoue, Emiko, Imai, Tomoaki, Shionoya, Yoshiki, Nakamura, Kiminari
• Format: Journal Article
• Language: English
• Published: United States American Dental Society of Anesthisiology 01-06-2021
• Subjects: Anesthetics; Brief Communications from the Japanese Dental Society of Anesthesiology; Female; Humans; Hyaline Fibromatosis Syndrome; Infant; Trismus - etiology; Trismus - therapy; Fiberoptic scope; Trismus; Juvenile hyaline fibromatosis; Difficult intubation; Nasotracheal intubation
• ISSN: 0003-3006; 1878-7177
• DOI: 10.2344/anpr-68-01-04

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.