Lengthening reconstruction surgery versus amputation
Amputation remains the most common option presented to parents with children who are born with FH. Why is amputation offered as the main treatment option? Performing an amputation at the level of the ankle joint (Syme’s amputation) gives a nice round stump with the heel pad as a weightbearing surface. That combined with modern prosthetics leads to unrestricted excellent function. As we all saw demonstrated in the 2012 London Olympics, amputees, and even bilateral below-the-knee amputees such as Oscar Pistorius, fitted with advanced prosthetics can even compete at the highest level. There is no question that a patient with FH who undergoes a Syme’s amputation and good prosthetic fitting and who has access to a technologically advanced prosthesis and prosthetic care on a regular basis (most children need a new prosthesis each year) will function normally for almost any activity. It is not uncommon to see video clips of children skateboarding, rock-climbing and performing individual and team sports following a below-knee amputation.
Nevertheless, if an amputation could be avoided and the foot and ankle and leg reconstructed to nearly normal function comparable to that afforded by a below-the-knee prosthetic, most parents and most individuals will choose to have the reconstruction. I do not think anybody wants to give up their foot or ankle unless there are no good alternatives.
When pediatric orthopedic surgeons are asked if they would amputate the foot if all that was wrong with the leg was a foot or ankle deformity such as club foot or many other childhood foot deformities, the answer is universally “no”. Despite this, the results of some clubfoot treatments leave the child with chronic pain and a stiff deformed foot that might be better treated by amputation and prosthetic fitting. When pediatric orthopedic surgeons are asked if they would amputate the leg of a child with no foot deformity and just a leg length discrepancy, the answer is almost universally “no”. When pediatric orthopedic surgeons are asked if they will amputate the leg of a child with a combination of foot deformity and a leg length discrepancy, the answer is frequently “yes”. The logic of this does not follow since for a foot deformity the recommendation is to correct the foot and for a leg length discrepancy the recommendation is to lengthen the leg; therefore, should not the recommendation for a foot deformity with a leg length discrepancy be to correct the foot deformity and lengthen the leg?
Most authors agree that lengthening is the preferred treatment for patients with mild to moderate leg length discrepancy with mild foot deformities (Paley types 1 and 2). The controversial cases are those that include more severe foot deformities (Paley types 3 and 4) and greater leg length discrepancies due to more severe tibial growth inhibition or combined femoral and tibial discrepancy. Syme’s or Boyd amputation has been the conventional recommendation for these more severe cases [
37]. The justification for amputation for the more severe cases has been the failure of most surgeons to obtain satisfactory results after limb lengthening [
38‐
40]. No one would dispute that amputation with prosthetic fitting requires fewer surgical interventions and fewer days of hospitalization and is associated with a lower complication rate. Furthermore, no one would dispute that with the availability of modern prosthetics, limb length equalization with excellent function can be achieved reliably in patients who have undergone Syme’s or Boyd amputation [
28,
37,
41]. This does not prove, however, that the best treatment for severe cases of FH is amputation with prosthetic fitting. Excellent function could also be obtained if amputation and prosthetic fitting were used to treat clubfoot, ankle arthritis or other disabling foot conditions. This is a testimony to the excellence of modern prosthetics and nothing more.
The challenge, therefore, is not to compare the function achieved in cases of Syme’s or Boyd amputation with that achieved in cases of lengthening—but rather to improve the results of lengthening and foot reconstruction in FH [
42]. Why are the results that are reported by many authors so poor [
42]? Is it because these cases are unreconstructable or is it because of fundamental errors in the treatment strategy used? An analysis of the unsatisfactory results reported in different series in the literature [
36‐
38,
42] makes it clear that the overriding factor associated with poor results is recurrent or residual foot and tibial deformities—and not the inability to obtain equalization of limb length. The few series in which good results were obtained, even in severe cases of FH, reported that the final result was a stable plantigrade foot [
43‐
46]. The total amount of discrepancy can always be equalized by serial moderate-sized lengthenings rather than by one very large lengthening. The foot deformity can be treated by various methods, including soft tissue and bone procedures. If these fail, ankle arthrodesis is a very successful way of permanently stabilizing the foot [
43,
45]. It is clear that ankle arthrodesis should not be the indication for amputation. Therefore, because the worst-case analysis in stabilizing and correcting the foot deformity is ankle arthrodesis, there is no reason that the foot cannot be made stable in a plantigrade position.
Johnson and Haideri [
47], using gait analysis, showed that patients in whom lengthening has resulted in plantigrade feet and well-aligned tibiae have better ankle push-off strength and better knee flexion strength than do patients who have undergone Syme’s amputation. These authors noted that the lengthened limb, even if it was stiff and weak, was less different from its opposite normal limb than was the prosthetic side in cases of Syme’s amputation as compared with its opposite normal limb. They reported that the lengthened limb with a plantigrade foot was “clearly more functional than a prosthetic ankle”.
Naudie et al. [
39] achieved satisfactory results in only four of ten cases after lengthening. These authors compared their group with an amputation group and concluded that amputation was preferable to lengthening. The reason for the unsatisfactory outcomes was residual or recurrent foot and tibial deformities. Cheng et al. [
48], in a small prospective group of four lengthenings, had the same experience, with unsatisfactory results secondary to recurrent tibial and foot deformities. Both groups succeeded in achieving the limb lengthening amounts desired using the Ilizarov apparatus. These results using the Ilizarov apparatus are not much different from those reported by Choi et al. [
38], who used the older Wagner method. In the study of Choi et al. [
38], all of the cases of higher grades of FH had unsatisfactory results, which were attributed by the authors to rigid uncorrected equino-valgus deformity of the foot. Satisfactory results were achieved in all except one of the patients with mild FH, a patient who had a rigid equino-valgus foot. Choi et al. [
38] also concluded that the more severe grades of FH are not candidates for lengthening surgery and would be best served with amputation and prosthetic fitting.
Clearly, although limb length can be successfully corrected in most patients, if the foot deformity is left uncorrected initially or if the foot deformity recurs, the final functional outcome will be unsatisfactory [
49,
50]. This conclusion is also valid for the treatment of clubfoot and vertical talus deformities. If one examines the few series in the literature that report good functional results after limb lengthening, the predominant difference is that in the final result not only was the leg length discrepancy addressed successfully but the foot deformities were also addressed successfully.
Miller and Bell [
45] reported the outcomes of 12 lengthenings in cases of FH. At the time of final follow-up, all limbs had regained full knee motion and all feet were plantigrade. All but three limbs had regained their preoperative range of ankle motion. None of the ankles had residual instability. Despite these excellent final results, 25 complications occurred in the 12 lengthenings, and the patients required eight secondary procedures to treat and correct complications. Gibbons and Bradish [
44] lengthened ten tibiae in cases of FH. In all cases, the desired lengthening was achieved and all patients were able to wear normal shoes without orthoses. A plantigrade position was achieved in all feet without persistent ankle instability. Complications occurred in nine of the ten cases, and all were all resolved either surgically or nonoperatively. Several patients required foot deformity correction with soft tissue or bone procedures.
Perhaps the largest series in the recent literature with the longest follow-up duration is that presented by Catagni and Guerreschi [
43]. Using the modified Dal Monte classification [
12], these authors reported 32, 37 and 20 cases of grade 1, grade 2 and grade 3 FH, respectively, that were treated with lengthenings, all of which led to completed reconstruction. Of the 32 patients with grade 1 FH, 31 required only one lengthening each and one required a second lengthening. Equal leg lengths with a plantigrade foot were achieved in each of these patients. In the 37 patients with grade 2 FH, five patients required three lengthenings each, nine required two lengthenings each, and 23 required one lengthening each. At the end of the reconstruction 35 of the patients had a plantigrade, functional foot, and the remaining two patients had residual valgus deformity, requiring shoes with orthoses. No patient underwent ankle arthrodesis. Thirty-two of the 37 were ultimately able to participate in recreational sports, and five limited their activities as a result of knee stiffness or instability. In the grade 3 FH group, two patients required six stages of reconstruction (a stage referred to as a lengthening or a deformity correction), four required five stages, six required four stages, three required three stages, four required two stages and one required one stage. Of these 20 patients, eight underwent foot deformity correction as a separate procedure before the age of 3 years. The final result was that 16 feet were plantigrade, stable and asymptomatic, and five had residual valgus with stiffness, requiring an orthosis to alleviate the symptoms. Although most of the patients could bike or swim, athletic pursuits were more limited than in the grade 1 and 2 patients. There were no permanent sequelae of knee subluxation, hip subluxation, nerve injury, nonunion or osteomyelitis in any patient. All of the patients were satisfied with the functional results of their reconstruction.
Paley presented (unpublished results presented at AAOS 1999, Anaheim, California) similar results to those of these last three studies. Excellent functional results, including the desired goal of lengthening, were achieved in 36 of the 38 legs lengthened. The one patient who was rated as having achieved only a fair result had a residual equinus deformity with a painful arthritic ankle and required an ankle fusion. Many patients were involved in recreational and/or competitive athletics. All of the adults in the series were gainfully employed, including the one who required an ankle fusion. Despite complications, the final result was not related to the complication rate. Few of the complications lead to major sequelae; those that do can usually be resolved surgically [
51].
One of the other criticisms of lengthening is the psychologic impact on the child. Although lengthening is undisputedly stressful for the child and the family, two recent studies have shown that the majority of problems are transitory and remit with appropriate treatment [
52,
53] and that the lengthening treatment does not cause long-term psychologic maladjustment [
53]. Although most patients tolerate the lengthening process well, some patients do develop loss of appetite, weight loss and difficulty sleeping. A single small dose of amitriptyline before bedtime is useful in helping these patients. Lengthening should not be an excruciatingly painful experience. If a patient is complaining of a lot of pain, especially during the day while at rest, the cause of the pain should be sought. Pain may be related to pin infection, pin loosening or cutting out, frame instability, nerve entrapment/stretch, reflex sympathetic dystrophy, rupture of the regenerating bone after premature consolidation, among other causes. Appropriate treatment, such as antibiotics, pin removal, wire retensioning, slowing distraction, pin replacement and backing up of the distraction, should be administered as soon as the problem is recognized. Peroneal nerve release should be considered if evidence of peroneal nerve stretch does not respond to slowing distraction.
To minimize the psychologic impact of lengthening, serial lengthenings and surgical reconstructions should be spaced apart according to the patient’s age to allow the child as much time as possible without surgery between sessions. Regarding lengthening, this author’s protocol is to perform the first lengthening when the patient is between 1.5 and 4 years of age, the second lengthening at between 6 and 10 years of age and the final lengthening at between 12 and 14 years of age. Children between the ages of 4.5 and 6 years have the most psychologic difficulty with lengthening, whereas children 4 years and younger have the easiest time with the treatment [
21]. This author prefers to complete the last lengthening before the patient is in high school, for social reasons, if possible. Cost is another argument for reconstruction rather than amputation.
In 1988 Johnson and Haderi [
47] reported that the cost of amputation and prosthetic fitting from age 1 to 18 years was US $81,000 per patient. In 1994 Williams projected lifetime total costs to be US $373,051 per amputee [
54]. During the same time period, the cost of surgical reconstruction was $40,000–50,000 for a single surgical lengthening reconstruction. Thus, even three such reconstructions cost less than the lifetime cost per amputee. Therefore, limb salvage is more cost-effective than amputation. While prices have gone up in the last 20 years since these studies were published, they have likely increased proportionately, and the cost of surgical reconstruction today is likely still less than the lifetime cost of amputation with lifetime prosthetic costs.
Paley et al. compared 22 patients personally treated by the first author with the SUPERankle procedure combined with lengthening to an age-matched group of patients who underwent Syme’s amputation at the Dallas at Texas Scottish Rite Hospital [
36]. The results of the comparison demonstrated no difference in function between the two groups. Both groups of patients were satisfied with their results, were equally and functionally active and had no pain. Both groups assessed their function as comparable to normal. The choice is therefore that of the parents as to which procedure they prefer for their child. With lengthening reconstruction surgery using the SUPERankle and lengthening, the big advantage is that in addition to normal function, the patient retains a sensate foot that can feel the ground, thereby providing balance and proprioception. No prosthesis provides sensibility or proprioception. Furthermore, the child and later the adult with the prosthesis must have an expensive high-quality technically advanced prosthesis made every year throughout childhood and frequently every year throughout adult life. This is an important economic consideration. The total cost to health care of these many prosthetic changes is much greater than all of the medical costs related to the surgery of lengthening reconstruction surgery [
47,
54]. This does not even factor in the frequent adjustments and modifications to the prosthesis that are required, nor the intermittent skin irritation of the stump to the prosthetic that causes some pain and suffering and sometimes interrupts prosthetic use. It also does not factor in that children and adults with FH with missing knee ligaments who have added stress due to the lever arm of a prosthesis can develop secondary problems at the knee joint. As well, it does not take into account the psychologic effects of having a prosthesis, such as going to the beach and having to take off the prosthetic to get into the water, the impact on dating, or any psychologic stress to the individual with the prosthesis created by not feeling comfortable wearing short pants or skirt. With lengthening reconstruction surgery, these are not considerations that the patient has to deal with.
Patients after SUPERankle procedures and lengthening surgery are able to participate in a wide range of sports, such as baseball, football, basketball, tennis, soccer, gymnastics, rock-climbing, etc. Therefore, the decision to undergo the procedure is a personal one and not one that should be dictated by the surgeon. The option of amputation is too readily provided because of the lack of training and availability of the SHORDT and SUPERankle procedures. While every pediatric orthopedic surgeon has been trained in amputation techniques and while amputation is not a technically difficult procedure, the SHORDT and SUPERankle procedures are technically challenging operations. Since FH is a rare diagnosis (less than 1:50,000 births) and since type 3 FH, which needs to be treated using the SUPERankle procedure, is even more rare, the majority of the pediatric orthopedic surgeons do not see many of these cases. In order to become proficient with the various variations of the SUPERankle procedure, for the different Paley types, one needs to perform this operation several times a year. Most pediatric orthopedic surgeons do not see more than one or two cases of this condition in a year. Therefore, it is difficult, if not impossible, for most pediatric orthopedic surgeons to gain sufficient experience with this procedure even if they do obtain proper training. On the other hand, to be proficient in Symes amputation is far easier since there are many more indications and the procedure is simpler and more forgiving. Therefore, when one takes into account both the lack of training and experience of pediatric orthopedic surgeons and the rarity of the condition, the SUPERankle procedure will remain an obscure, underutilized operation than it should be. It is therefore less likely to be recommended to most patients. Hopefully, with greater awareness centers of excellence can develop this expertise, and it will be offered as an alternative and perhaps one day replace amputation surgery for FH.
Based on this author’s experience, there are few contraindications to lengthening. All patients should be given the option of lengthening reconstruction surgery versus amputation. If the lengthening option is not available at the treating center, patients should be offered a second opinion at a referral center that has expertise in lengthening reconstruction surgery. Socioeconomic factors may limit such second opinion options. Nevertheless, this should be the patient’s decision and not the doctor’s. There are many avenues to overcome socioeconomic limitations in today’s society. In many developing nations, amputation may be culturally unacceptable and good prosthetics unobtainable. In such situations, amputation is contraindicated. Finally, when there are upper extremity deficiencies, which make independently getting in and out of a prosthetic challenging, amputation is also contraindicated [
13].