Volkmann’s ischaemic contracture of the upper extremity – raising a red flag in the setting of developing countries

INTRODUCTION

Volkmann’s ischaemic contracture (VIC) is a devastating complication of the compartment syndrome due to prolonged ischaemia in muscle compartments resulting in tissue necrosis and irreversible changes in muscles, nerves, and vascular endothelium. It can lead to permanent disability with a wide range of presenting symptoms, from mild to severe contractures with various forms of function impairment of the affected muscle compartment (Fig. 1). It was first described in 1881 by Richard von Volkmann as a paralysis resulting from interrupted blood supply by the application of tight bandages to the injured extremity [1]. Since then, many articles were published concerning the aetiology of the VIC, showing us that several other different causes of VIC existed. The most commonly described aetiology involves supracondylar fractures or any other fracture in the arm, elbow, or forearm. Many rare entities such as an iatrogenic injury due to attempted sampling from the radial artery, bleeding disorders, drugs, infections, and many more have been described, as well [2,3].

Several methods of treatment have been proposed ranging from conservative to surgical [2,4]. Young age of onset and mild contractures may respond to stretching and splinting. A variety of osseous and soft tissue surgical procedures have been used to treat severe VIC. The treatment of the VIC is very complex and needs considerable expertise. Even with advanced reconstruction, it is not possible to reanimate the normal function of the hand in severe cases (Fig. 2, 3). Prevention is, therefore, of utmost importance to recognize and bear in mind while treating the patients.

Many VIC patients were encountered in our practice, coming from the far western provinces of Nepal. Patients from every province were analysed to see what the aetiology of VIC is and to analyse how it can be prevented.

Fig. 1: Case of Volkmann’s ischaemic contracture, “severe” according to Tsuge´s classification
A) Preoperative state; B) Postoperative result

METHODS

A retrospective analysis was conducted in patients with established VIC of upper extremity admitted and treated during 6 years between 2013 to 2018 by a single surgeon at HRDC Hospital, Janagal, Nepal. Age, sex, district of origin, cause of VIC, duration of injury, grade of VIC and quick DASH score at presentation were extracted from the patients’ documentation [5]. The grade of VIC was classified as mild, moderate, and severe according to Tsuge’s classification (Tab. 1) [6,7]. All patients with established VIC of the upper extremity with clear aetiology and the district of origin were included. It was also noted whether the fasciotomy to achieve compartment release at the time of the acute compartment syndrome had been performed. These characteristics of VIC from each province of Nepal were compared in Tab. 2 and the map of occurrence was drawn (Fig. 2). This research was conducted according to the Helsinki Declaration of 1975, as revised in 2000. All patient data were anonymized before conducting further analysis.

Fig. 2: Surgical treatment of Volkmann’s ischaemic contracture by tendon transfers and tendon lengthening
A) Preoperative; B1) Release of contracted muscles; B2) Image from surgery after tendon transfers and lengthening

Fig. 3: Postoperative state of the case presented in Fig. 2

Tab. 1: Tsuge’s classification – Volkmann’s ischaemic contracture [6-7]

Tab. 2: Exact data from every Nepali province are shown together with its number of inhabitants

RESULTS

We analysed 47 patients presenting with VIC of upper extremity during the period from 2013 to 2018. All these patients fulfilled our inclusion criteria. The average age was 11.59 (ranging between three and 34 years). The sex ratio was 59.57% (28 patients) males and 40.43% (19 patients) females. The leading causes of VIC were a tight cast applied by the health care personnel for minor traumatic injuries (distal radius fracture, contusions, etc.) in 53.19% (25 patients) and tight bandage applied by the patient, a family member or local traditional healer in 44.68% (21 patients). Of those 21 patients, two patients (4.26%) applied a tight bandage to treat snakebite injury. The supracondylar fracture was the cause of VIC in 2.13% (1 patient). The distribution of patients as per their place of origin is shown in Tab. 2. The patients were mainly from province 6 – 29.79% (14 patients), province 3 – 23.40% (11 patients) and province 1 – 12.77% (6 patients) (Fig. 4).

Figure 4: Map of Nepali provinces showing the occurrence of Volkmann’s ischaemic contracture
Notes: Δ – the place of the hospital where the data were obtained; ² – marks the capital – Kathmandu.

The average duration of the injury at the time of presentation was 57.8 months, ranging from 12 months to 216 months. The VIC was classified as per Tsuge’s classification. There were three mild (6.4%), 35 moderate (74.5%) and nine severe (19.1%) cases of VIC. Only 14 cases (29.78%) had undergone compartment release at the time of the acute compartment syndrome. The average Quick DASH score at the time of presentation was 48.5, ranging from 18.2 to 77.3. As most of the patients had a moderate to severe type of VIC, surgical management was offered to 44 patients (93.6%). Out of these, only 10 patients (22.7%) underwent surgical management; physiotherapy and splinting were used in the remaining patients.

To further assess these differences, we have employed Fisher’s exact test for pairwise comparison of the occurrences of VIC patients compared to the rest of the provinces’ populations between provinces 3, 6 and the rest (grouped cases from provinces 1, 2, 4, 5, and 7). Province 6 had statistically significantly more VIC cases per inhabitant than province 3 and the rest (p<0.001 is significant even after Bonferroni correction for multiple hypothesis testing for three hypotheses). Furthermore, province 6 had statistically significantly more cases when compared with each province separately (p<0.005). A marginally statistically significant difference was found between province 3 and the rest (province 6 excluded) with p=0.0398 for Fisher’s exact test with a one-sided alternative.

DISCUSSION

Forty-one (87.2%) of our patients were children below 18 years. Fractures in children are often managed conservatively with plaster. This is validated by our results showing that the leading cause of VIC in Nepal population is a tight cast in 53.19%, followed by tight bandage in 44.68%. Two cases of tight bandage application were due to snake bite injury, believing that it would prevent the venom from spreading into the circulation. It is important to note the snake bite injury did not cause VIC by itself in this case but the wrongly applied tight bandage for its treatment did. Cases of snake bites directly leading to VIC have been, nevertheless, described in the literature; these cases were caused due to the necrotoxic venom in Crotailinae (CZ: chřestýšovití) [8]. The application of a tight cast and bandage was the major cause of VIC in our study in contrast to trauma as described in most of the studies [9,10]. Therefore, we provide recommendations for the prevention in these scenarios, which can be used for educational purposes (Fig. 3).

Our study clearly shows the arising problem and helps to predict the provinces in Nepal on which our attention with educational programs should be focused. A similar study needs to be done for every country where such a problem exists. There are several limitations to our study. It is possible that patients from far provinces are seeking healthcare elsewhere in the neighbouring countries. Patients from province 4 have a closer centre with good reputation and many of them probably seek healthcare there. Patients in the capital are probably divided into more hospitals. The problem of underdeveloped countries is the lack of a reporting system and well-made documentation, which make a multi-centre study hard to conduct. Nevertheless, addressing this problem, which is not described in previous literature, and raising awareness is the first step to decrease those alarming numbers of VIC.

There is a tremendous variability of clinical presentations of VIC based on the extent of muscle necrosis and nerve injury [7]. It may range from a mild contracture with no pain or functional disability to a severe contracture with deformity, pain, and gross dysfunction. Amongst several classification systems [7,11–14], we used the most common classification system of Tsuge (1975) [7]. Tsuge classified established VIC as mild, moderate, and severe types, according to the extent of muscle involvement. We used quick DASH score to assess preoperative function to determine how badly the VIC affects the daily function of the affected person. Even though DASH score has limited value in paediatric population, it is by and large the simplest method of determining the hand function.

We find it alarming that the largest sample came from province 6 which has the lowest number of inhabitants (resulting in a statistically significant difference in the occurrence of VIC cases) and is accessible with much more difficulty than provinces 1–5. A further analysis to find the reasons for such numbers should be attempted and the educational program started as soon as possible. Moreover, a good quality healthcare can be obtained in Pokhara in province 4 (see Fig. 2 for better understanding of Nepal geography). The only other statistically significant difference was observed between province 3 and the rest (province 6 excluded), but that was expected as the hospital where the cases were observed is found in this province.

Over the years, VIC became a rare entity in developed countries. In 1967, Eichler published an article documenting a period of ten years when 38 new cases were diagnosed at Mayo Clinic [9]. In 1979, Mubarak published an article in Toronto stating that VIC incidence was not decreasing over the 21 years during which they admitted 19 patients with upper extremity VIC [10]. On the other hand, in our practice 47 patients were treated with upper extremity VIC in six years by a single surgeon. This discrepancy itself is highly disturbing let alone the fact that the numbers of patients in Eichler’s and Mubarak’s studies were from an area of major teaching hospitals and the studies are 40 years old. It is both interesting and alarming that our aetiology differs from other mentioned studies. Our findings are consistent with the first description of VIC by Volkmann as an iatrogenic injury. In 1909, Thomas analysed 107 cases in his review to prove that other aetiologies besides tight bandages and cast even existed [15]. On the other hand, 102 years later in a review published by Kaylani, there were no patients with VIC due to tight cast or bandage [8]. The only iatrogenic injury reported was caused by IV infiltration, tourniquet and unspecific category postsurgery. It seems hard to believe that such a discrepancy exists and a possible bias may result from the unwillingness to report bad results. Another possibility is that what is reported as VIC due to the fracture is actually caused by a tight cast. Special attention should be given to the spontaneous compartment syndrome, which was reported only 21 times in the literature, and due to its typical late diagnosis it might also be a risk for VIC development. Most of such cases are a combination of minor trauma and anticoagulation therapy or deficit, or rare systemic diseases (systemic sclerosis, Ehler-Danlos syndrome, McAdler disease [16]).

Casting should be done by someone who is experienced and skilled in its application or under the supervision of such a person [17]. In the case any junior orthopaedic resident is not sure about how to put on the cast and supervision is not available, diagnosis is not confirmed, or the patient has much swelling at presentation, it is better to apply the splint during a second look, which also enables the patient or their guardian in case of children to loosen the splint if it gets tight.