• T Iber, S Klösel, B Schoenes, and K Zacharowski.
• Klinik für Anästhesiologie, Intensivmedizin und Schmerztherapie, Klinikum der Johann Wolfgang Goethe-Universität Frankfurt/Main, Theodor-Stern-Kai 7, 60590 Frankfurt am Main, Deutschland. Thomas.Iber@kgu.de
• Anaesthesist. 2010 Jun 1;59(6):535-8.

AbstractFibromyalgia ossificans progressiva (FOP) is a severely disabling disorder of connective tissue characterized by congenital malformation of the toes, fingers and vertebrae associated with progressive ossification of striated muscles. Anesthetic management of these patients involves preferably general anesthesia as local or regional anesthesia should be avoided due to possible heterotopic ossification. Airway management is determined by the age of the patient and the progression of the disease. Only a few cases in the literature have reported the anesthetic management of FOP patients and to our knowledge only one case has been published on pediatric patients. In adult, cooperative patients awake fiberoptic intubation is recommended, as ankylosis of the temporo-mandibular joint is the most important clinical feature for anesthesia. As demonstrated and discussed in this case report of a 2-year-old boy, fiberoptic intubation after induction of general anesthesia should be preferred in pediatric patients. Puncture of a vein should be non-traumatic, i.m. injections strictly avoided and careful positioning and padding are needed. Every effort should be made to avoid situations stimulating new heterotopic ossification due to its substantial effect on the quality of life of FOP patients.

22 keywords...

hide…