Monitoring and Management of Fibrodysplasia Ossificans Progressiva: Current Perspectives.

Fibrodysplasia ossificans progressiva (FOP), sometimes known as myositis ossificans progressiva, is an ultra-rare disease in which bone is formed in muscular tissue, tendons and ligaments. This is known as heterotopic ossification (HO). FOP is caused by a heterozygous mutation in the highly conserved ACVR1/ALK2 gene which affects about 1 in 1.5-2 million individuals. At birth, patients with the predominant R206H mutation only exhibit a bilateral hallux valgus. During childhood, heterotopic bone formation develops in a typical pattern, affecting the axial muscles first before appendicular body parts are involved. HO can start spontaneously but is often elicited by soft tissue trauma or medical procedures. After soft tissue injury, an inflammatory process called a flare-up can start, followed by the formation of HO. HO leads to a limited range of motion, culminating in complete ankylosis of nearly all joints. As a result of HO surrounding the thorax, patients often suffer from thoracic insufficiency syndrome (TIS). TIS is the most common cause of a limited life expectancy for FOP patients, with a median life expectancy of 56 years. Management is focused on preventing soft-tissue injury that can provoke flare-ups. This includes prevention of iatrogenic damage by biopsies, intramuscular injections and surgery. Anti-inflammatory medication is often started when a flare-up occurs but has a poor basis of evidence. Several forms of potential treatment for FOP are being researched in clinical trials. Progression of the disease is monitored using CT and 18F-NaF PET/CT combined with functional assessments. Patients are regularly evaluated for frequently occurring complications such as restrictive lung disease. Here, we review the current management, monitoring and treatment of FOP.

When Limb Surgery Has Become the Only Life-Saving Therapy in FOP: A Case Report and Systematic Review of the Literature.

Fibrodysplasia ossificans progressiva (FOP) is a rare disease in which heterotopic ossification (HO) is formed in muscles, tendons and ligaments. Traumatic events, including surgery, are discouraged as this is known to trigger a flare-up with risk of subsequent HO. Anesthetic management for patients with FOP is challenging. Cervical spine fusion, ankylosis of the temporomandibular joints, thoracic insufficiency syndrome, restrictive chest wall disease, and sensitivity to oral trauma complicate airway management and anesthesia and pose life-threatening risks. We report a patient with FOP suffering from life-threatening antibiotic resistant bacterial infected ulcers of the right lower leg and foot. The anesthetic, surgical and postoperative challenges and considerations are discussed. In addition, the literature on limb surgeries of FOP patients is systemically reviewed. The 44 year-old female patient was scheduled for a through-knee amputation. Airway and pulmonary evaluation elicited severe abnormalities, rendering standard general anesthesia a rather complication-prone approach in this patient. Thus, regional anesthesia, supplemented with intravenous analgosedation and N2O-inhalation were performed in this case. The surgery itself was securely planned to avoid any unnecessary tissue damage. Postoperatively the patient was closely monitored for FOP activity by ultrasound and [18F]PET/CT-scan. One year after surgery, a non-significant amount of HO had formed at the operated site. The systematic review revealed seventeen articles in which thirty-two limb surgeries in FOP patients were described. HO reoccurrence was described in 90% of the cases. Clinical improvement due to improved mobility of the operated joint was noted in 16% of the cases. It should be noted, though, that follow-up time was limited and no or inadequate imaging modalities were used to follow-up in the majority of these cases. To conclude, if medically urgent, limb surgery in FOP is possible even when general anesthesia is not preferred. The procedure should be well-planned, alternative techniques or procedures should be tested prior to surgery and special attention should be paid to the correct positioning of the patient. According to the literature recurrent HO should be expected after surgery of a limb, even though it was limited in the case described.

Cancellations of (helicopter-transported) mobile medical team dispatches in the Netherlands.

BACKGROUND: The trauma centre of the Trauma Center Region North-West Netherlands (TRNWN) has consensus criteria for Mobile Medical Team (MMT) scene dispatch. The MMT can be dispatched by the EMS-dispatch centre or by the on-scene ambulance crew and is transported by helicopter or ground transport. Although much attention has been paid to improve the dispatch criteria, the MMT is often cancelled after being dispatched. The aim of this study was to assess the cancellation rate and the noncompliant dispatches of our MMT and to identify factors associated with this form of primary overtriage. METHODS: By retrospective analysis of all MMT dispatches in the period from 1 July 2006 till 31 December 2006 using chart review, we conducted a consecutive case review of 605 dispatches. Four hundred and sixty seven of these were included for our study, collecting data related to prehospital triage, patient's condition on-scene and hospital course. RESULTS: Average age was 35.9 years; the majority of the patients were male (65.3%). Four hundred and thirty patients were victims of trauma, sustaining injuries in most cases from blunt trauma (89.3%). After being dispatched, the MMT was cancelled 203 times (43.5%). Statistically significant differences between assists and cancellations were found for overall mortality, mean RTS, GCS and ISS, mean hospitalization, length and amount of ICU admissions (p < 0.001). All dispatches were evaluated by using the MMT-dispatch criteria and mission appropriateness criteria. Almost 26% of all dispatches were neither appropriate, nor met the dispatch criteria. Fourteen missions were appropriate, but did not meet the dispatch criteria. The remaining 318 dispatches had met the dispatch criteria, of which 135 (30.3%) were also appropriate. The calculated additional costs of the cancelled dispatches summed up to a total of 34,448 euro, amounting to 2.2% of the total MMT costs during the study period. CONCLUSION: In our trauma system, the MMT dispatches are involved with high rates of overtriage. After being dispatched, the MMT is cancelled in almost 50% of all cases. We found an undertriage rate of 4%, which we think is acceptable. All cancellations were justified. The additional costs of the cancelled missions were within an acceptable range. According to this study, it seems to be possible to reduce the overtriage rate of the MMT dispatches, without increasing the undertriage rate to non-acceptable levels.