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Ultrasound-Guided Ankle Block in Stone Man Disease, Fibrodysplasia Ossificans Progressiva

Schober, Patrick MD; Krage, Ralf MD; Thöne, Deirdre MD; Loer, Stephan A. MD, PhD; Schwarte, Lothar A. MD, PhD

doi: 10.1213/ane.0b013e3181ac1093
Analgesia: Regional Anesthesia: Case Report

In this case report, we describe the successful use of ultrasound-guided regional anesthesia in progressive fibrodysplasia ossificans (stone man disease), a condition commonly regarded as a contraindication for regional anesthesia. A patient with advanced fibrodysplasia ossificans progressiva presented with osteomyelitis of a foot and was scheduled for resection of the infected bones and soft tissue. Ultrasound imaging allowed us to identify the obscured anatomic landmarks for ankle block anesthesia and to restrict the injection of local anesthetics to the epifascial tissue and subcutaneous compartment. With this ankle block, the patient uneventfully underwent surgery without need for additional sedative or analgesic drugs.

From the Department of Anesthesiology, VU University Medical Center (VUmc), Amsterdam, The Netherlands.

Accepted for publication February 25, 2009.

No conflict of interest for any of the authors.

Reprints will not be available from the author.

Address correspondence to Lothar A. Schwarte, MD, PhD, DESA, EDIC, Department of Anesthesiology, VU University Medical Center (VUmc) De Boelelaan 1117 1081 HV Amsterdam, The Netherlands. Address e-mail to L.Schwarte@vumc.nl.

Fibrodysplasia ossificans progressiva (FOP), the so called “stone man disease,” is termed the most catastrophic disease of ectopic bone formation in humans.1,2 The disease is characterized by grotesque heterotopic ossifications occurring both spontaneously and after minimal trauma leading to complete immobilization by a “second skeleton.”1,2 Medical management may markedly and irreversibly aggravate this disease.2,3 Hyperostosis boosts may be triggered by intramuscular or intraoral injections of local anesthetics.4–6 Growing interest in FOP and related disorders is stimulated by recent publications about the underlying pathophysiology.7–11

Anesthetic management in FOP is challenging. Because needle trauma regularly induces bone formation,4–6 FOP is regarded until now as a relative contraindication for regional anesthesia. Although subcutaneous injections have a low ossifying potential, bone formation regularly occurs after minimal trauma to muscles and connective tissues, such as ligaments, i.e., structures which are regularly affected by regional anesthesia techniques.

General anesthesia is often recommended in FOP. However, airway management may be complicated by severe cranial and cervical ankylosis12,13 requiring fiberoptic intubation.14–16 Fiberoptic intubation must be performed with utmost care because airway instrumentation may also result in ossification. In addition, patients with FOP frequently present with a restrictive lung function16 due to a rigid thorax. The impairment in lung function may increase the risk of postoperative pulmonary complications.14 Thus, fiberoptic intubation with general anesthesia also includes several disadvantages and risks in this specific patient population.

Because strict subcutaneous injections have a low bone-forming potential in FOP,4 superficial nerve blockades with local anesthetics17 may be a safe alternative to general anesthesia. We present a case of ultrasound-guided ankle-foot block in a patient with FOP scheduled for forefoot osteomyelitis surgery.

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CASE DESCRIPTION

A 33-year-old woman (38-kg bodyweight) with advanced FOP presented with progressive osteomyelitis originating from the fifth digit of her right foot. Both feet were severely mutilated. Her right foot and ankle were additionally hyperalgesic, hyperthermic, and markedly edematous (Fig. 1). Repeated attempts to cure the osteomyelitis by oral and intravenous antibiotic therapy only limited the systemic inflammatory responses temporarily but failed to cure the focus. A surgical resection of the infected bone and tissue structures was planned.

Figure 1

Figure 1

Preoperative evaluation revealed a complete active and passive immobility of the temporomandibular joint and cervical spine. A reduction in thorax mobility by ossified costovertebral joints and intercostal musculature, as documented by radiograph and computed tomography imaging, contributed to a restrictive pulmonary disease. Her respiratory condition was complicated by recurrent recent episodes of pneumonia.

In view of the anticipated complexity in airway management, ventilation and weaning, avoidance of airway instrumentation appeared desirable. Although we were aware that locoregional anesthesia techniques are relatively contraindicated in FOP,18 we planned a modified ankle block to avoid general anesthesia. Because experience with regional anesthesia in these patients is limited, we performed a preoperative provocation test with bupivacaine the week before surgery. After informed consent, a subcutaneous depot of 5 mL bupivacaine 0.5% and a depot of 5 mL saline (as control) were injected into corresponding spots of the left and right dorsal forearm, respectively. The injections were performed strictly subcutaneously under ultrasound guidance (MicroMaxx with linear array probe HFL-38; 6–13 MHz, SonoSite, Bothell, WA) and pen marked for subsequent reevaluation. Ultrasound investigation of the two corresponding forearm sites before and after the subcutaneous injections and 1 week later revealed no signs of ossifying boosts. Therefore, we regarded subcutaneous injections of bupivacaine as a safe technique in our patient.

The extent of forefoot inflammation was unclear. Therefore, blockade of all five nerves at the ankle level was planned. The performance of this block was complicated by the inability to move the ankle and foot in a neutral position. Superficial anatomical landmarks such as palpable malleoli or arteries19 were absent because of the severe mutilations and the current inflammatory swelling (Fig. 1). Sonography, however, allowed us to identify the landmarks and selective injection of local anesthetics into the subcutaneous tissue (Fig. 2). During injection, needle contact to muscles, tendons, and bones was avoided. Under standard anesthetic monitoring plain bupivacaine 0.5% was injected into the tibial nerve site (∼5 mL) and the deep peroneal nerve site (∼5 mL) using a 25-mm needle (Terumo® Neolus, Leuven, Belgium). A strict subcutaneous field block was used to anesthetize the superficial peroneal, sural, and saphenous nerve (∼15 mL). One-half hour after completion of the block, the patient was transferred to the operating room and uneventfully underwent surgery. There was no need for additional sedative or analgesic drugs. The injection sites were reevaluated 1 week later with ultrasound, revealing no tissue inflammation, tissue necrosis or other signs of new ossification.

Figure 2

Figure 2

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DISCUSSION

Anesthetic management in patients with stone man disease is complicated by several aspects. Because minimal trauma caused by needles may induce bone formation, regional anesthesia is often regarded as relatively contraindicated. Ectopic bone formation occurs locally after minimal trauma to skeletal muscles and connective tissues, such as ligaments and other structures frequently affected by regional anesthesia techniques.

Ultrasound guidance allowed us to identify the relevant anatomical landmarks19 in our patient in whom regular anatomy was obscured by osteomyelitis-induced swelling and the preexisting mutilation of the skeleton. In addition, ultrasound guidance allowed us to strictly limit the local anesthetic injections to the epifascial tissue layer, which is crucial in patients with FOP to prevent further episodes of bone formation.4,20–24

Although our approach is obviously restricted to regional anesthesia techniques not requiring needle penetration through muscle and connective tissue (i.e., ankle block), our case report demonstrates that in selected cases regional anesthesia techniques are feasible in patients with FOP and can be performed safely when using ultrasound guidance.

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