Anaesthesia and juvenile hyaline fibromatosis

Juvenile hyaline fibromatosis is a rare autosomal recessive disease characterized by large cutaneous nodules, especially around the head and neck and often involving the lips. The effects become increasingly severe with age and also include joint contractures, gingival hypertrophy and osteolytic lesions. We describe the anaesthetic management of two sisters with this disease. Safe maintenance of a patent airway is the principal anaesthetic challenge.