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Case Report

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

Patrick Schober, MD

Ralf Krage, MD

Deirdre Thone, MD

Stephan A. Loer, MD, PhD

Lothar A. Schwarte, MD, PhD

In this case report, we describe the successful use of ultrasound-guided regionalanesthesia in progressive fibrodysplasia ossificans (stone man disease), a conditioncommonly regarded as a contraindication for regional anesthesia. A patient withadvanced fibrodysplasia ossificans progressiva presented with osteomyelitis of afoot and was scheduled for resection of the infected bones and soft tissue. Ultrasoundimaging allowed us to identify the obscured anatomic landmarks for ankle blockanesthesia and to restrict the injection of local anesthetics to the epifascial tissue andsubcutaneous compartment. With this ankle block, the patient uneventfully underwentsurgery without need for additional sedative or analgesic drugs.(Anesth Analg 2009;109:98890)

Fibrodysplasia ossificans progressiva (FOP), the socalled stone man disease, is termed the most cata-strophic disease of ectopic bone formation in hu-mans.1,2 The disease is characterized by grotesqueheterotopic ossifications occurring both spontane-ously and after minimal trauma leading to completeimmobilization by a second skeleton.1,2 Medicalmanagement may markedly and irreversibly aggra-vate this disease.2,3 Hyperostosis boosts may be trig-gered by intramuscular or intraoral injections of localanesthetics.46 Growing interest in FOP and relateddisorders is stimulated by recent publications aboutthe underlying pathophysiology.711

Anesthetic management in FOP is challenging. Becauseneedle trauma regularly induces bone formation,4 6 FOPis regarded until now as a relative contraindication forregional anesthesia. Although subcutaneous injectionshave a low ossifying potential, bone formation regu-larly occurs after minimal trauma to muscles andconnective tissues, such as ligaments, i.e., structureswhich are regularly affected by regional anesthesiatechniques.

General anesthesia is often recommended in FOP.However, airway management may be complicated

by severe cranial and cervical ankylosis12,13

requiringfiberoptic intubation.1416 Fiberoptic intubation must

be performed with utmost care because airway instru-

mentation may also result in ossification. In addition,patients with FOP frequently present with a restrictivelung function16 due to a rigid thorax. The impairmentin lung function may increase the risk of postoperativepulmonary complications.14 Thus, fiberoptic intubationwith general anesthesia also includes several disadvan-tages and risks in this specific patient population.

Because strict subcutaneous injections have a lowbone-forming potential in FOP,4 superficial nerveblockades with local anesthetics17 may be a safe alter-native to general anesthesia. We present a case ofultrasound-guided ankle-foot block in a patient with

FOP scheduled for forefoot osteomyelitis surgery.

CASE DESCRIPTIONA 33-year-old woman (38-kg bodyweight) with advanced

FOP presented with progressive osteomyelitis originatingfrom the fifth digit of her right foot. Both feet were severelymutilated. Her right foot and ankle were additionally hy-peralgesic, hyperthermic, and markedly edematous (Fig. 1).Repeated attempts to cure the osteomyelitis by oral andintravenous antibiotic therapy only limited the systemicinflammatory responses temporarily but failed to cure thefocus. A surgical resection of the infected bone and tissuestructures was planned.

Preoperative evaluation revealed a complete active andpassive immobility of the temporomandibular joint and cervi-cal spine. A reduction in thorax mobility by ossified costover-tebral joints and intercostal musculature, as documented byradiograph and computed tomography imaging, contributedto a restrictive pulmonary disease. Her respiratory conditionwas complicated by recurrent recent episodes of pneumonia.

In view of the anticipated complexity in airway manage-ment, ventilation and weaning, avoidance of airway instru-mentation appeared desirable. Although we were awarethat locoregional anesthesia techniques are relatively contra-indicated in FOP,18 we planned a modified ankle block toavoid general anesthesia. Because experience with regionalanesthesia in these patients is limited, we performed apreoperative provocation test with bupivacaine the week

before surgery. After informed consent, a subcutaneousdepot of 5 mL bupivacaine 0.5% and a depot of 5 mL saline

From the Department of Anesthesiology, VU University MedicalCenter (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 Medi-cal Center (VUmc) De Boelelaan 1117 1081 HV Amsterdam, TheNetherlands. Address e-mail to L.Schwarte@vumc.nl.

Copyright 2009 International Anesthesia Research Society

DOI: 10.1213/ane.0b013e3181ac1093

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(as control) were injected into corresponding spots of the leftand right dorsal forearm, respectively. The injections wereperformed strictly subcutaneously under ultrasound guid-ance (MicroMaxx with linear array probe HFL-38; 6 13MHz, SonoSite, Bothell, WA) and pen marked for subse-quent reevaluation. Ultrasound investigation of the twocorresponding forearm sites before and after the subcutane-ous injections and 1 week later revealed no signs of ossifyingboosts. Therefore, we regarded subcutaneous injections ofbupivacaine as a safe technique in our patient.

The extent of forefoot inflammation was unclear. There-fore, blockade of all five nerves at the ankle level wasplanned. The performance of this block was complicated bythe inability to move the ankle and foot in a neutral position.Superficial anatomical landmarks such as palpable malleolior arteries19 were absent because of the severe mutilationsand the current inflammatory swelling (Fig. 1). Sonography,however, allowed us to identify the landmarks and selectiveinjection of local anesthetics into the subcutaneous tissue(Fig. 2). During injection, needle contact to muscles, tendons,and bones was avoided. Under standard anesthetic moni-toring plain bupivacaine 0.5% was injected into the tibialnerve 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 under-went surgery. There was no need for additional sedative oranalgesic drugs. The injection sites were reevaluated 1 weeklater with ultrasound, revealing no tissue inflammation,tissue necrosis or other signs of new ossification.

DISCUSSION

Anesthetic management in patients with stone mandisease is complicated by several aspects. Becauseminimal trauma caused by needles may induce boneformation, regional anesthesia is often regarded asrelatively contraindicated. Ectopic bone formation oc-curs locally after minimal trauma to skeletal musclesand connective tissues, such as ligaments and otherstructures frequently affected by regional anesthesiatechniques.

Ultrasound guidance allowed us to identify the rel-

evant anatomical landmarks19

in our patient in whomregular anatomy was obscured by osteomyelitis-inducedswelling and the preexisting mutilation of the skeleton.In addition, ultrasound guidance allowed us to strictlylimit the local anesthetic injections to the epifascial tissuelayer, which is crucial in patients with FOP to preventfurther episodes of bone formation.4,2024

Although our approach is obviously restricted toregional anesthesia techniques not requiring needlepenetration through muscle and connective tissue (i.e.,ankle block), our case report demonstrates that inselected cases regional anesthesia techniques are fea-

sible in patients with FOP and can be performed safelywhen using ultrasound guidance.

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Figure 1. Preoperative (A) picture(right foot, iodized and draped forsurgery) of the presented case of fi-brodysplasia ossificans progressiva(FOP). Beside the characteristic in-born malformation of the great toe,anatomy is obscured which is aggra-vated by edema. Three days afterremoval of the infectious focus (B,postoperative picture), anatomical land-marks (malleolus and arterial pulse)were still not palpable.

Figure 2. Ultrasound image recorded during the foot-ankleblock in the reported case of fibrodysplasia ossificans pro-gressiva (FOP). Note that ultrasound imaging was used tolocate the needle in the subcutaneous tissue and confinelocal anesthetic injections strictly to the subcutaneous com-partment. The images were recorded with a Sonosite Micro-

maxx, using the linear array probe HFL-38 (613 MHz, 38mm). The total tissue depth imaged is approximately 20 mm.

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