Complication of lengthening and the role of post-operative care, physical and psychological rehabilitation among fibula hemimelia

Abstract

There is a clear clinical need for efficient physiotherapy and rehabilitation programs during and after bone lengthening and reconstruction for gaining the optimal effect and also prevention or treatment of lengthening side effects. Pin tract infection is the most prevalent side effect during lengthening which could be prevented and treated initially via proper wound care. Muscle contractures are typically a consequence of the generated tension on the distracted muscle. It can be managed by physiotherapy initially and surgically in later severe stages. Furthermore, it is essential to avoid muscle contracture development, which is the demonstration of the imbalanced muscle appeals on the joint to inhibit the following subluxation. The knee is the furthermost affected joint by the aforementioned problem due to the inherent lack of ligamentous and bony stability. Joint stiffness is the other possible unfavorable effect of lengthening. It happens because of extensive muscle contractures or may possibly be attributed to rigidity of the joint following the amplified pressure on the joint surface during the process of lengthening. Physiotherapy and occupational therapy including endurance and strength exercise as well as stretching play an important role during the rehabilitation periods for the prevention and also the treatment of muscle contracture and the following deformity and also joint stiffness. Likewise, the effect of mental and physical rehabilitation programs should not be overlooked.

Key Words: Fibular hemimelia, Rehabilitation, Reconstruction, Lengthening

Core Tip: Difficulties that occur during limb lengthening are not avoidable. Pin tract infections are considered the most common adverse effect of lengthening which should be prevented through prophylaxis and treated as soon as possible when occurred. Muscle contractures are usually a result of the tension generated on the muscle due to distraction. It can be managed by physiotherapy initially and surgically in later severe stages. Subluxation or dislocation during lengthening may have been attributed to the preexisting joint instability or imbalanced muscle tension that developed during lengthening. It often happens in the knee joint posteriorly.

INTRODUCTION

Fibular hemimelia (FH) is considered the most common long bones deficiency and includes an extensive spectrum of anomalies, from a slight partial fibular shortening to a whole lack of fibula, and associated defects of the foot, and ankle, tibia, femur, and even hand abnormality[1].

With respect to the numerous deformities among fibular insufficiency as well as its relationship with postaxial hypoplasia, decision-making and strategies of treatment must be personalized[2]. Treatment choices consist of non-surgical management with shoe lift and orthotic devices; limb preservation, which includes limb elongation strategies; or amputation followed by lifetime prosthetic use. The aforementioned recommended managements are principally hooked on the harshness of the amount of limb length discrepancy, the foot deformity and deficiency, and the potential for achieving the functional and plantigrade foot[3]. The optimal treatment of FH has been controversial for a long time. Amputation along with prosthetic fitting has been recommended as an effective method in the management of FH in early studies and they also use to believe that patients may be more satisfied with amputation and prosthesis with no need for multiple surgeries to correct the deformities and then lengthen the usual major limb length discrepancies[4].

Unfavorable side effects have inundated limb-lengthening procedures since the first introduction for lengthening of the lower limb in 1905[5]. High obstacle rates, mostly bone-healing related, turn into the hallmark of the conservatively accepted aforementioned technique[6]. Along with the introduction of physiological-based approaches to elongation established by Ilizarov[7] in 1988 and according to the biology and physiology of soft tissue as well as bone regeneration under the tension and stretching condition, bone healing problems and complications have developed substantially less and the treatment goals are mostly achieved[8].

It did not take long before the great revolution of Ilizarov's principles of external fixation, became the main pillar of reconstruction and lengthening. This improved the results of limb preservation and reconstruction presenting an attractive alternative to amputation[9]. Over time and as surgeons become more interested in limb preservation, lengthening, and reconstruction, several studies have been conducted on the effects of post-operative care and rehabilitation on the final outcome of reconstructive surgical approaches[10,11]. Since the Ilizarov technique is slow and gradual through daily physiologic changes in the soft tissues and bone, many potential difficulties develop during the distraction as well as fixation periods. Some of the aforementioned difficulties could be solved during the lengthening procedure[6]. In this perusal, we reviewed the literature to find the current post-operative care and rehabilitation programs during and after limb lengthening and reconstruction.

PIN TRACT INFECTION

Pin tract infections are considered the most common adverse effect of lengthening or even an almost inevitable obstacle in using external apparatus with various rates of 5% to 100% which is the result of pin tract insertion[12,13] (Figure 1). It happens because the natural barrier, the skin, and residues open, providing a communication, vehiculated through the pins, between the bone and the surroundings. It can ultimately cause osteomyelitis, loosening, and fixation loss[14]. Moreover, rehabilitation may lead to motion at the pin-skin interface, aggregating the risk for pin tract infection[15].

Figure 1 Pin tract infection. A: Healthy healing of pin tract site; B: Infection of pin tract site.

Treatment and prophylaxis of pin track infection are influenced by different parameters[14]. Pins and holes care includes gentle softening and massage of the crust by moistening it with saline, following the use of alcohol, and the application of an antiseptic which usually consists of chlorhexidine or povidone-iodine[16,17]. Some other authors have also used tribromophenate, paraffin ointment, bismuth, silver sulfadiazine, polyhexamethylene biguanide, or hydrogen peroxide[18-21]. Protection with dry gauze finalizes the care[14]. Dry nailing makes the environment of the pining site unfavorable for germs to grow. The frequency of the aforementioned care is usually every two days, even though it varies between once a week and twice a day[14].

It has been suggested that the prophylactic prudence including the release of the skin tension at the pin-site as well as cautious pin-site overhaul and care by the parents will help in the prevention and control of the infections and their development to a complication[3,22-26]. Likewise, including the knee in the Ilizarov frame prevent moving the proximal ring to the cutaneous or subcutaneous tissue of the posterior thigh or popliteal fossa and further wound infections[27].

MUSCLE CONTRACTURE

Muscle contracture is frequently a consequence of the over tension on the muscle following distraction[28]. They often have a tendency to happen in the over-powering muscle group. It goes back to the strength difference between extensors and flexors[29]. Knee flexion and ankle plantarflexion contracture during the lengthening of the tibia, the most common site of lengthening among fibula hemimelia, occurs because of the triceps surae muscle’s resistance to lengthening because of their large muscle mass and strength[30]. In some cases, who have a simultaneous proximal focal deficiency, which is accompanied by fibular hemimelia in 50% to 80%, the second stage of lengthening from femoral bone is desirable[31]. In these cases, the hamstrings are the bulkiest and largest muscle group. The hamstring contracture leads to the moving back of the leg (hip extension) and the knee to bend (knee flexion)[32].

The muscles that cross two joints are frequently involved in contractures because they contain fibers of numerous extents as opposed to muscles crossing a single joint and have fibers of the same extent[33] (Figure 2). The aforementioned statement would cause tension deviations in similar muscles. Muscle tension is a mechanism of stimulating regeneration in muscle during the lengthening procedure. Muscles that pass through two joints may contain a variance proportion of histogenesis in comparison to muscles crossing a single joint[34]. Moreover, it may be attributed to the differences in the maximum histogenesis rate and potential between bone and muscle[35].

Figure 2 Lateral view of the tibia and associated muscles. A: Before lengthening. Gastrosoleus muscles and Achilles tendon; B: After lengthening of tibia. Ideally the muscle should lengthen to the same extent as the bone; C: Knee flexion contracture. This occurs when knee extension is not maintained throughout lengthening. A relative shortening of the muscle to the new bone length has been produced; D: Equinus contracture of ankle. This occurs when ankle dorsiflexion is not maintained during lengthening.

The other etiologic concern is the muscles or tendons' transfixion by the pins of the apparatus[36]. It would be amplified via transfixed pins, the longitudinal clump of numerous pins in the single plane as well as the thick pins’ diameter. Transfixed fascia and tendons lead to joint motion restriction more than transfixed muscles[36].

Therefore, muscle contracture prevention is an inevitable portion of the elongation process. The initial preventive methods include fixation across joints, splinting, and physiotherapy[37]. Physiotherapy has been expected to motivate passive stretching movements for the most involved muscles. Moreover, it is not enough to stretch the muscle only at one end, due to the fact the aforementioned muscle typically crosses two joints. For instance, regarding the triceps surae, while the knee is flexed, the foot should be motivated to dorsiflex maximally. Afterward, the knee would be extended passively, as the ankle is dorsiflexed[38,39]. The patient must be instructed and motivated to repeat the aforementioned movements during the day[40]. Electrical stimulation, as well as active exercises, are approved to improve muscle regeneration[41]. There are several reports which have shown the positive role of continuous passive motions (CPM). Many studies have demonstrated the encouraging effect of passive motion in improving the knee flexion contracture[42,43]. CPM has also some proven hopeful effects in plantar flexion contracture of the ankle which is frequently seen following tibia lengthening[44].

The principles of contracture prevention would work if they have been maintained for at least six hours each day. It is far from the mind that most cases would exercise their muscles that much[45,46].

Trigger point dry needling is suggestive management that would reduce the pain during the physiotherapy and stretching process. This hypothesis has been supported by several investigations demonstrating that muscle damage identified by magnetic resonance imaging after the application of dry needling over the spasmed muscle was associated with post-needling induced pain[42]. They have associated the presence of post-needling with the number of local twitch responses elicited during the needling therapy. Moreover, Gattie et al[47] conducted an interesting meta-analysis that displayed low to moderate evidence suggesting that dry needling, when applied by physical therapists, is superior to each treatment solely. This may reduce the pain and so fascinate the stretching progression.

SUBLUXATION/ DISLOCATION

Dislocation or subluxation of the adjacent joint frequently occurs during lengthening[48]. It may have been attributed to the preexisting joint instability. For example, the anterior cruciate ligament deficiency almost always occurs in the fibula hemimelia state due to femoral notch narrowing[49,50]. The imbalanced muscle pressure that developed during elongation might conduce subluxation, even in the lack of preoperative instability; this particularly happens in the knee[51,52]. Therefore, it is fundamental to avoid muscle contracture presentation.

The knee is the most frequently affected joint because of the inherent deficiency of bony and ligamentous stability[28]. It also should be mentioned that if there is a body segment with a hypomobility (deficit of movement), the nearest segment (joint) will assume that deficit creates hypermobility (increased movement, and therefore the possibility of subluxation) thus creating a compensatory mechanism automatically and involuntarily by the patient.

The subluxated joint may be improved using stretching physiotherapy of the distorting muscle force. It is worth mentioning that physiotherapy would help in the early stages and among milder cases. Traction is another way of management[53]. For the cases of complete dislocation as well as the more severe and persistent subluxation, the apparatus should be extended across the joint to distract and then relocate it gradually or immediately. Moreover, tendon or capsular releases or reapplication of the Ilizarov may be needed[54].

JOINT STIFFNESS

Joint rigidity is usually known as a late-onset complication[55]. This happens because of long-term muscle contractures or can be attributed to rigidity of the joint following the amplified pressure on the joint surface during the elongation process[56]. If a joint is being assumed at the risk of residual rigidity while the elongation device was still on, it should be extended across the joint, and the joint can be distracted afterward. Following that the device can be used to mobilize the joint prior to its removal[57]. The severity of adverse effects can be different according to the created functional limitation. Apparently, 15° loss of ankle dorsiflexion or knee extension is much more significant in comparison to the same motion limitation in ankle plantar flexion and knee flexion[58].

PSYCHOLOGICAL AND MENTAL REHABILITATION

Many experienced pediatric orthopaedics have stated that quality of life will increase as the child grows up[22,59]. The higher quality of life in adults may be attributed to psychological reasons and musculoskeletal development[60]. To describe more precisely, children and their parents are generally under the impression that surgery will make their appearance, daily function, and other physical aspects normal and like healthy individuals[61]. They sometimes confuse this defect with a simple bone fracture and expect it to be completely repaired with surgery. But as these patients age, they will become more realistic about their malformation and learn to adjust their lives to their disease[22]. Therefore, more involved in routine daily activity and report a higher quality of life when being surveyed. Through much literature review, we have come to the conclusion that educating parents about the fact that FH has a range of problems and that the patients who choose any management method will never be as skeletal as their peers are ultimately effective in their satisfaction[61].

CONCLUSION

Limb salvage and lengthening is a speedily growing approach to orthopaedic surgery among bone deficient cases. There are some unavoidable adverse effects. Pin Tract Infection could be extensively prevented via post-operative wound care and cleaning. Muscle contracture could be prevented and managed by physiotherapy in the early stages and surgically in later severe stages. Subluxation/ dislocation would be treated by stretching the deforming muscle force during the physiotherapy procedure. Moreover, if a joint is being assumed at the risk of residual rigidity while the elongation device was still on, it should be extended across the joint, and the joint can be distracted afterward. Following that the device can be used to mobilize the joint prior to its removal. Therefore, the aforementioned issues during or after lengthening could be prevented extensively through proper physiotherapy and occupational therapy including endurance and strength exercise as well as stretching programs. Psychological Rehabilitation would also improve the long-term quality of life and patients' satisfaction.