ABSTRACT

Juvenile hyaline fibromatosis (JHF) is an autosomal recessive congenital disorder characterized by slow-growing nodules on the body in the early stages of life. Abnormal tissue growth in soft tissues, limited joint mobility, and other system abnormalities may lead to airway problems in these patients and also massive blood loss during operation might occur. Preparations should be made in the intensive care unit due to potential respiratory failure after the operation. In this case report, we aimed to discuss the anesthetic management modalities in a 7 year- old JHF patient.

Keywords: Juvenile hyaline fibromatosis, anesthesia, airway management