Fibromatosis hialina juvenil y el reto de la vía aérea: reporte de un caso y revisión de la literatura

Introduction: Juvenile hyaline fibromatosis (JHF), also referred to as ANTXR2-related hyaline fibromatosis syndrome, is a rare autosomal recessive disease that can compromise the airway through gingival hyperplasia, hyaline nodules, and cervical and temporomandibular limitation. Case presentation: A 15-year-old adolescent with JHF (juvenile hyaline fibromatosis) and a mouth opening of 1 cm, scheduled for excision of dorsal tumors, in whom an anticipated difficult airway was planned. Inhalational induction with preservation of spontaneous ventilation was chosen, mask ventilation was confirmed before neuromuscular blockade, and intubation was achieved using a combined videolaryngoscopy and video-fiberoptic technique, successful on the first attempt and without complications; extubation was performed under a difficult airway protocol, with neuromuscular reversal and close postoperative monitoring for three hours during the patient’s stay in the post-anesthesia care unit (PACU), with no stridor, airway edema, or need for reintubation. Discussion: Published evidence on airway management in JHF is limited to isolated case reports without standardized protocols; the anesthetic decisions in this case were grounded in preserving spontaneous ventilation and a stepwise rescue plan consistent with current difficult airway guidelines, and surgical airway access is not recommended as a first-line option. Conclusion: An individualized anesthetic plan encompassing intubation strategy, rescue plan, and staged extubation is essential in JHF patients; this case illustrates the feasibility of a combined approach without supporting claims of risk reduction or protocol standardization.