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[Anesthesiologic problems in patients with fibrodysplasia ossificans progressiva]
1Anästhesieabteilung, Regionalspital Davos.
Insights
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder causing bone to form in muscles and connective tissues. This case highlights fiberoptic intubation as a safe anesthetic technique for FOP patients with airway challenges.
Area of Science:
- Anesthesiology
- Medical Genetics
Background:
- Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder characterized by progressive heterotopic ossification of soft tissues.
- There is a lack of established anesthetic protocols for FOP patients, posing significant management challenges.
- Patients with FOP often present with complex airway issues, including limited jaw mobility and cervical spine rigidity.
Observation:
- A 49-year-old female patient with FOP required surgical amputation due to a resistant infection.
- The patient exhibited severe limitations in movement, a small mouth opening, poor dentition, a history of aspiration, and restrictive lung function.
- She had undergone two surgical procedures necessitating anesthesia.
Findings:
- Fiberoptic nasal intubation in an awake patient was identified as the optimal airway management strategy due to the patient's specific anatomical limitations and risk of atlanto-axial subluxation.
- General anesthesia with propofol/fentanyl/N2O/O2 and etomidate/fentanyl/ethrane/N2O/O2, without muscle relaxation, was successfully employed during both procedures.
- Significant hypotension occurred during both anesthetics but responded well to fluid resuscitation.
Implications:
- This case underscores the critical importance of meticulous pre-anesthetic assessment and tailored airway management strategies in patients with FOP.
- Fiberoptic intubation is a valuable technique for securing the airway in FOP patients with challenging anatomy.
- Further research and case reports are needed to expand the evidence base for anesthetic management in this rare condition.
Abstract:
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 abnormalities 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 O2. 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 cesarean section [21]. Newer publications [13, 20] promote fiberoptic intubation and general anaesthesia with or without muscle relaxation. In this case propofol/fentanyl/ N2O/O2 without muscle relaxation was used for the first operation and etomidate/fentanyl/ethran/ N2O/O2 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.