Refinements in the Treatment of Volkmann Ischemic Contracture of the Forearm: A Thematic Review

Authors: Milan Stevanovic, Frances E. Sharpe

Abstract

Volkmann contracture of the forearm is a devastating complication of muscle ischemia. It is most commonly associated with trauma and a sequela of compartment syndrome. In the last few decades, much has improved in our ability to treat these patients. Our preferred treatments are presented along with representative case studies that illustrate the functional gains that can be achieved with a focus on moderate and severe contractures. Refinements in care that have evolved over the past 40 years of experience in treating these patients are presented.

INTRODUCTION

More than 150 years ago, Richard von Volkmann reported on a contracture of the forearm that would later become eponymously associated with his name.^1^ Volkmann contracture can cause devastating effects on wrist and hand function. Historically, treatment was predominately concerned with the correction of contractures more than the restoration of function. The advent of microsurgery and the ability to transfer muscles as a functional unit has radically changed the landscape for treatment options. This review will present a brief history and description of Volkmann contracture, and current methods of treatment for moderate and severe established Volkmann contracture of the forearm.

HISTORICAL PERSPECTIVE

In his 1870 publication, while writing on infantile paralysis (polio), Volkmann described a contracture of the

“ … The fingers become drawn into the palm. The wrist is at the same time slightly flexed. Extension of the fingers is opposed by more or less resistance but if the wrist be flexed strongly, the fingers may be passively extended … only one of the patients suffered from ‘infantile paralysis’. In the other, the palsy was caused by too tight bandaging of a fractured forearm.”^1^

In 1881, von Volkmann ascribed irreversible contractures of the flexor muscles of the hand to ischemic processes in the forearm, believing that the problem was caused by massive venous stasis and simultaneous arterial insufficiency secondary to overly tight bandages.^2–5^ (See figure, Supplemental Digital Content 1, which shows a portrait of Richard von Volkmann 1830–1889, http://links.lww.com/PRSGO/D14.) In 1906, Hildebrand was the first to refer to this condition as Volkmann ischemic contracture.

Over the next 100 years, much would be done to identify and understand the cause of Volkmann contracture. Specifically, compartment syndrome was identified as the causative etiology, and many investigators undertook the study of compartment syndrome. This culminated with the development of the unified concept of compartment syndrome, which is still accepted today.^6,7^

The early identification and treatment of compartment syndrome with emergent fasciotomy decreased the incidence of Volkmann contracture. However, cases still occur and cause devastating effects on limb function. Although the incidence has markedly decreased, the treatment of established Volkmann contracture changed little over the ensuing 120 years. Treatment typically included excision of the infarcted muscle to try to correct contracture, but it did little to restore function. Tendon transfers could be used to replace some function, but the donor musculotendinous units were often compromised by the initial event or would have to work through a scarred soft-tissue bed. Nerve dysfunction associated with compartment syndrome was recognized, but little was done to treat this component of the deficit. Two very small series of patients were treated with a flexor origin muscle slide. Despite functional improvement, this did not gain popularity as a treatment option.^8,9^ The advancement of microsurgical techniques, and specifically of functional free muscle transfer, has dramatically changed the outlook for these patients.

CLINICAL DESCRIPTION

Volkmann ischemic contracture is the consequence of prolonged ischemia and irreversible tissue necrosis resulting from compartment syndrome. During the ischemic event, the most vulnerable muscle group is the deep flexor compartment of the forearm, which includes the flexor digitorum profundus (FDP) muscle and the flexor pollicis longus (FPL). In the mildest form of ischemic contracture, these muscles are affected, and often only a portion of these muscles.^10–12^ The flexor digitorum superficialis, pronator teres, and wrist flexors are involved with more severe or prolonged ischemia. In the most severe cases, ischemia and subsequent necrosis of the extensor compartment can occur. Nerve involvement can be mild to severe, depending on the extent of ischemia and fibrosis. Nerve involvement in Volkmann ischemic contracture is likely a combination of the initial ischemic event and the late mechanical effects of fibrosis (Fig. 1).^13^

Established Volkmann contracture has a different presentation than acute compartment syndrome. Patients with Volkmann contracture do not have pain but rather have deformity and dysfunction resulting from the prior ischemic event and subsequent muscle fibrosis. There is a broad clinical spectrum of presentation, as each case represents a different extent of muscle necrosis and nerve injury. The muscle fibrosis and neurological deficits lead to deformity of the joints distal to the site of ischemia. Deformity progresses over weeks to months following the ischemic event and then stabilizes. In children, the deformity will progress until skeletal maturity; fibrotic muscles are unable to lengthen at the rate of bone growth. Even when contractures are treated, the affected extremity is shorter due to the tethering of growth across the physis and the ischemic effect on the physis. In the pediatric population, recurrent contracture is common due to the differential growth of bone and fibrotic muscle.

CLASSIFICATION

Several classification systems have been described for Volkmann contracture of the forearm. All classifications are limited by the variability in the extent of both muscle and nerve involvement.^11,12,14,15^ Although patterns of necrosis associated with muscle compression have some consistency, fracture location or the etiology of the compartment syndrome also plays a role in the pattern of necrosis.

Our preferred classification system is that of Tsuge.^11^ Established Volkmann contracture is divided into mild, moderate, and severe types, according to the extent of muscle involvement. The Tsuge category of severe contractures includes cases of moderate tissue necrosis that are exacerbated by fixed joint contractures, a scarred soft-tissue envelope, or failed surgery. Within each classification type, there is a wide range of clinical presentations. The heterogeneity of presentation makes it difficult to apply a specific treatment based solely on the classification system and confounds meaningful outcome and comparison studies. (See figure, Supplemental Digital Content 2, which shows mild-type contracture, http://links.lww.com/PRSGO/D15.) (See figure, Supplemental Digital Content 3, which shows moderate-type contracture, http://links.lww.com/PRSGO/D16.) (See figure, Supplemental Digital Content 4, which shows severe-type contracture, http://links.lww.com/PRSGO/D17.)

Variations on Volkmann Contracture

Two distinct entities should be reviewed. One is neonatal Volkmann contracture, which is an in utero compression or ischemic event resulting in partial or complete limb necrosis. Unlike neonatal compartment syndrome, which requires emergent fasciotomy, there is no emergent treatment for neonatal Volkmann contracture. (See figure, Supplemental Digital Content 5, which shows neonatal compartment syndrome and neonatal Volkmann, http://links.lww.com/PRSGO/D18.) The etiology of neonatal Volkmann is unknown but thought to result from both extrinsic and intrinsic factors. Extrinsic factors include oligohydramnios, multiple gestation, fetal macrosomia, abnormal in utero posture, difficult extractions, maternal diabetes, and excessive maternal weight gain. Intrinsic factors include a hypercoagulable state, leading to intravenous or intra-arterial thrombosis.^16^ The presentation varies depending on the time of ischemia relative to the time of delivery, ranging from wrist and finger flexion contracture without skin changes to evidence of skin and muscle necrosis of varying severity. Determining if there is still a component of compartment syndrome is critical. If present, the neonate requires emergent surgery with fasciotomy and excision of necrotic tissue. Necrotic tissue without signs of acute compartment syndrome can be debrided electively within the first 2–4 weeks, provided that the neonate is stable for surgery and general anesthesia. Contractures without tissue necrosis can be addressed throughout development.^17–20^

The other entity is a mimic of Volkmann contracture, described as pseudo-Volkmann contracture. Distinguishing Volkmann contracture from pseudo-Volkmann contracture can be difficult. The patient history of fracture as well as timing and severity of pain helps in distinguishing these diagnoses. Pseudo-Volkmann is most commonly caused by tethering by scar or entrapment of the FDP or the FPL to the fractures of the radius or ulna. Other causes include adhesions around internal fixation plates, or muscle scarring secondary to infection. Most commonly, a pseudo-Volkmann will mimic a mild contracture.^21–23^ At surgical exploration, the muscles will seem normal but adherent to the bone. A myotenolysis or tenolysis alone will usually allow for correction of the contracture.

TREATMENT

Treatment is based on patient age, contracture severity, and associated injuries and/or neurological involvement. Diagnostic imaging including radiographs of the uninvolved limb provide information on fracture healing and alignment and growth discrepancy compared with the uninvolved limb. Magnetic resonance imaging (MRI) can evaluate the extent of muscle damage within each compartment and may augment clinical findings. MRI may assist surgical planning and provide prognostic information to the patient and their family. MRI can also help distinguish between Volkmann contracture and pseudo-Volkmann contracture. Magnetic resonance angiography or traditional angiography is useful for patients who have severe involvement who are anticipated to need a functional free muscle transfer, helping to plan the recipient vessel anastomosis site and assess perfusion to the hand.

Treatment is tailored to the severity of muscle and nerve involvement. Intervention may require a combination of muscle and joint contracture releases, tendon transfers, joint fusions, and free functional muscle transfers. Surgery is customized to the functional losses and the remaining donor muscles that are available for transfer.

Mild Type (Localized Muscle Contracture)

Therapy is beneficial to minimize contracture formation following acute compartment syndrome. In children, splinting must continue until skeletal maturity. Therapy should include maintenance of passive joint motion, preservation and strengthening of remaining muscle function, and splinting. Established mild Volkmann contracture may require surgical release. Mild contractures involve all or part of the deep flexor compartment, specifically the ring or small FDP muscle, or FPL. An isolated release of these muscles as a limited flexor origin slide or a direct release of the tendon distally with tenodesis to the adjacent functional profundus tendons will both correct the contracture and restore function. For the FPL, a one- or two-level fractional or step-cut lengthening can correct the contracture. This may weaken the muscle power, and a tendon transfer may be necessary for improved pinch strength. Alternatively, a primary tenotomy with tendon transfer may be used.^8,9,11,12,14,24,25^

Moderate Type (Deep and Superficial Flexor Compartment with Neurological Deficit)

Moderate contractures require surgical intervention. When some flexor muscle function is preserved, a procedure addressing muscle tightness can correct the contracture and still maintain flexor function. Traditionally, this has been performed as a lengthening of the muscle tendon unit. In Volkmann contracture, the muscle resting length has been shortened through scarring of the muscle unit, such that further compromise of the muscle resting length through muscle lengthening results in a greater decrease in the remaining muscle strength. Alternatively, a flexor-pronator muscle slide allows the entire muscle group to move distally, allowing for correction of the joint contracture associated with the shortened muscle while preserving the existing muscle power (Fig. 2). (See Video 1 [online], which shows the 10-year follow-up after flexor muscle origin slide demonstrating finger flexion and extension.)

Video 1shows the 10-year follow-up after flexor muscle origin slide demonstrating finger flexion and extension.1_pf282rq1Kaltura

Moderate contractures are characterized by neurological deficits. The severity of neurological deficits contributes to the patient’s hand disability and plays a role in determining surgical treatment. The median nerve is the most susceptible to injury, lying between the superficial and deep flexor muscles. Mild median nerve involvement may be treated with neurolysis combined with a flexor origin slide, with a separate release at the carpal tunnel. More severe involvement and/or involvement of the ulnar nerve is more likely to require nerve reconstruction of the scarred and infarcted nerve. Table 1 provides an outline of the surgical technique of the flexor-pronator muscle origin slide.

A flexor origin slide or muscle lengthening procedure is unlikely to provide much functional improvement in moderate contractures where there is little remaining muscle power or where a significant neurological impairment exists. A free functional muscle transfer and nerve reconstruction may provide the best chance of meaningful functional return. Adjunctive procedures such as tendon transfers or joint fusions may be necessary either as a simultaneous or staged procedure to improve functional results.

Severe Type (Superficial and Deep Flexor of the Fingers and Wrist, Extensor Muscle Compartment, and Neurological Deficits in the Median and Ulnar Nerves, Affecting Hand Function)

Past treatment for severe contractures included bone and tendon procedures to address contractures but did little to improve function. Today, severe contractures are best treated with infarct excision and functional free muscle transfers in combination with adjunctive procedures including nerve reconstruction of nonfunctional portions of the involved nerves, bone procedures for stability, and tendon transfers if available.

The gracilis muscle is the most commonly used muscle for free functional muscle transfer; it has the best length and excursion to match the finger flexors and extensors. The estimated excursion of the gracilis is 12 cm (based on a 30-cm muscle length).^26^ The estimated finger excursion needed for restoration of finger and wrist flexion is 9 cm (Figs. 3–5).^27^ (See Video 2 [online], which demonstrates independent thumb flexion and opposition.) (See Video 3 [online], which demonstrates independent thumb flexion and opposition.) Other muscles (pectoralis major, medial gastrocnemius, and latissimus dorsi) have lesser excursion and do not produce good restoration of finger flexion. Both the gracilis and latissimus dorsi myocutaneous flaps can be transferred with a large skin envelope to address soft-tissue loss in the forearm. The myocutaneous gracilis flap in particular can be harvested with a broad area of fascia that includes the entire medial thigh.^28^ This provides a broad area of tissue coverage and a robust fascial sleeve in which the muscle can glide. Appropriate marking of the muscle resting length and establishing a strong muscle origin and insertion are critical to achieving good functional results.^29,30^ Separate motor functional units of the gracilis can be used to restore independent FDP and FPL motor function (Fig. 5).^31–34^ Simultaneous median nerve reconstruction for necrotic or fibrotic nonfunctional nerve should be performed at the time of reconstruction. We prefer cabled sural nerve autograft for reconstruction. For severe-type contractures with extensive involvement of the extensor compartment, a double free muscle transfer should be considered. Therapy for muscle and joint mobilization, customized splinting, and muscle retraining remain an important part of patient care (Tables 2–4).

Video 2demonstrates independent thumb flexion and opposition.1_be2jch0fKaltura

Video 3demonstrates independent thumb flexion and opposition.1_m32wcvo3Kaltura

Acute Compartment Syndrome with Liquefactive Muscle Necrosis

Some cases of acute compartment syndrome should be considered for immediate reconstruction, specifically when there is extensive or liquefactive muscle necrosis. Early wide excision of necrotic muscle with immediate reconstruction using a functional free muscle transfer has the ability to prevent joint contracture, prevent sclerotic strangulation of intact nerves, and allow early restoration of finger flexion (and extension if necessary). There is no need in the setting of liquefactive necrosis to wait and see what function returns. Function will not return in the setting of dead and dissolved muscles. Waiting will only add to the disability of the limb, greater fibrosis around the nerves, and greater nerve dysfunction (Fig. 6 and see Video 3 [online]).

OUTCOMES

Outcomes are difficult to assess in this group of patients. Studies are limited by small patient numbers, variability in initial presentations, varied surgical techniques, and difficulty in following patients through to skeletal maturity.^8,9,11,35–37^

Sharma and Swam reported on outcomes of the flexor origin slide in 19 patients with moderate Volkmann contracture. Seventy-eight percent resulted in good outcomes with improvement in grip, dexterity, and appearance.^37^ Griffart et al reported on outcomes in seven patients with mild contracture treated with flexor muscle origin slide. These patients had 72% of the grip strength of the contralateral hand. All patients were satisfied with surgery and would have the surgery repeated.^36^

Ultee and Hovius^38^ reported on 25 patients, whose treatment varied based on the severity of involvement. They found that all patients who had developed the contracture during childhood had a relatively shortened extremity. Substantial improvements in hand function were noted in those who underwent functional free muscle transfer. Tendon lengthening alone often resulted in recurrent contracture. Finally, in patients having sufficient remaining muscle, procedures which combined infarct excision, tenolysis, neurolysis, and tendon transfer when necessary produced good hand function.^38^ Sundararaj and Mani noted improvement in sensory function in conjunction with neurolysis. Additional procedures were done simultaneously, and little analysis of outcomes of those other procedures was given.^39^

Sabapathy et al reported on 22 patients with Volkmann contracture treated with free functional muscle transfers for finger flexor reconstruction. In their series, four of 22 (18%) required nerve reconstruction. Twenty patients recovered M3 or M4 motor function. Ten patients who had measurement of grip strength achieved 24% of the strength of the contralateral hand.^40^

CONCLUSIONS

As with many diagnoses in medicine, the best treatment for Volkmann ischemic contracture is prevention. Early recognition and prompt treatment of compartment syndrome has decreased, but not eliminated, the incidence and severity of late contracture and hand dysfunction. We have found the flexor muscle slide is our best treatment option for mild and moderate deformity. This procedure can be combined with additional reconstructive procedures to maximize functional outcome. We believe this procedure results in the best preservation of the muscle resting length and limits the scarring around the adjacent muscles. The ulnar approach as opposed to the anterior approach avoids additional scarring within the flexor compartment. Refinements in the original technique described by Page in 1923 include the use of the extended ulnar approach with a longer proximal and distal incision that allows elevation and exposure along the full length of the elbow and forearm from the ulnar side, across the interosseous membrane to the radial side of the radius. This approach also allows access to the distal brachialis muscle which can be fractionally lengthened in the setting of elbow contracture. Ulnar nerve transposition reduces risk of tension on the ulnar nerve that may occur with restoration of wrist and finger extension (Table 5).

For severe cases, functional free muscle transfer has been transformative in offering functional recovery to patients with this devastating injury. Refinements in the techniques initially described include the ability to harvest a large and reliable skin paddle by including a large swathe of fascia in the harvest and the greater saphenous vein to augment outflow. Primary wound closure is often possible with this flap. The ability to provide independent thumb and finger flexions by separating the obturator fascicle of the gracilis provides more natural hand function. Preoperative identification of nerve deficits in the median (most common) and or ulnar nerves preoperatively prompts careful inspection of these nerves for signs of necrosis that would require simultaneous nerve reconstruction below the functional muscle transfer (Table 5).

We also advocate early wide excision and functional free muscle transfer for patients with severe compartment syndrome and liquefactive necrosis. This is done within the first 1–2 weeks when the tissue bed is clean. Early intervention may limit both joint contractures and injury to the nerves, decreasing the distal problems associated with motor and sensory impairment in the hand (Table 5).

DISCLOSURE

The authors have no financial interest to declare in relation to the content of this article.

PATIENT CONSENT

Patients provided written consent for the use of their images.

Supplementary Material