Anaesthesiological problems in patients with fibrodysplasia ossificans progressiva

Fibrodysplasia ossificans progressiva (FOP) is a rare, congenital disease of the striated muscular system, ligaments and fascia; it leads to complete ossification in adult life. The disease usually begins in the first decade of life and is accompanied by abnormities of the hands and feet that have already begun to occur at birth. There is no effective therapy. Although some of these patients have to undergo an operation, there is very little information available on anaesthetic procedures. Case report. A 49-year-old-woman came to the anaesthetic ward for amputation of her left foot with the diagnosis of fibrodysplasia ossificans progressive (FOP) and an infection resistant to any antibiotic treatment. Except for her left elbow, she could not move any of her extremities. Her back and neck were stiff (Figs. 1, 2), she could not open her mouth more than 2.5 cm, and her teeth were full of cavities and loose (Fig. 3). Aspiration of gastric content some time ago was known. Lung function showed a restrictive pattern. She suffered from kyphoscoliosis and needed chronic nasal administration of O-2. With this therapy the arterial blood gas analysis was normal. Right bundle-branch block on the electrocardiogram was found. She was in a wheelchair and was completely dependent on the help of others. She was taking no medicine at the time of admission. A second operation was necessary because of reactive bone proliferation with a danger of skin perforation. Discussion. This rare and congenital disease was first described in 1648 by G. Patin [14] and again in 1736 by J. Freke [10]. Since then, the progressive ossification of striated muscles, ligaments and facia has been described more than 600 times. Despite this fact, descriptions of anaesthetic procedures are rare. Because of the neck stiffness, the small mouth opening, the poor teeth and the recently observed history of aspiration, fiberoptic nasal intubation when the patient is awake was found to be the best choice. The possibility of atlanto-axial subluxation in such cases [2, 4] favored this procedure. Other authors have used blind nasal intubation [11], ketamine without intubation [18], or even local anaesthesia for a cesarian section [21]. Newer publications [13, 20] promote fiberoptic intubation and general anaesthesia with or without muscle relaxation. In this case propofol/fentanyl/N2O/O-2 without muscle relaxation was used for the first operation and etomidate/fentanyl/ethran/N2O/O-2 without muscle relaxation for the second procedure. During both anaesthesias important hypotension with good response to a generous volume supply was seen. The patient recovered well. Unfortunately, she died a few weeks later from suicide. The goal of this case report is to emphasize the value of the fibrobronchoscope in patients with FOP.