[Anesthetic management of a patient with juvenile hyaline fibromatosis: a case report].
Juvenile hyaline fibromatosis (JHF) is a rare disease of autosomal recessive heredity characterized by multiple subcutaneous nodules, hypertrophy of the gingiva, and flexural contracture of the joints including those of the cervical vertebrae and the mandibula. It has been pointed out that the clinical features render tracheal intubation profoundly difficult. We present a case of 6-year-old girl diagnosed as JHF and anesthetized for tumor resection and reconstruction of the naso-oral region. She was significantly small for her age weight 12.3 kg and lived on a wheel-chair because of flexion contracture of the extremities. However, the cervical spinal joints were not involved and the neck could be extended in our case. With extreme care and preparation, anesthetic induction and tracheal intubation were smoothly done although a tooth was damaged by the surgeon when gauze-packing was attempted into the pharynx before the operation. Anesthesia was maintained uneventfully and satisfactorily during the surgery under anesthesia with nitrous oxide, oxygen, sevoflurane, fentanyl and vecuronium. There was no prolongation of nor abnormal reaction to the effect of vecuronium observed in this patient.
Sugahara
S
S
Department of Anesthesiology, Nippon Medical School, Tokyo.
Ikezaki
H
H
Abe
K
K
Ogawa
R
R
D000769:Anesthesia, Inhalation; D002648:Child; D005260:Female; D005350:Fibroma / Q000601:surgery*; D006801:Humans; D009055:Mouth / Q000601:surgery; D009666:Nose / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000601:surgery*