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Lower Extremity

Use of Paley Classification and SUPERankle Procedure in the Management of Fibular Hemimelia

Kulkarni, Ruta M.; Arora, Nitish; Saxena, Sagar; Kulkarni, Sujay M.; Saini, Yadwinder; Negandhi, Rajiv

Author Information
Journal of Pediatric Orthopaedics: October 2019 - Volume 39 - Issue 9 - p e708-e717
doi: 10.1097/BPO.0000000000001012
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Abstract

Fibular hemimelia is partial or total aplasia of the fibula.1 It is associated with defects of the femur, tibia, ankle, and foot.2 It is the most common deficiency involving the long bones and presents clinically as, limb length discrepancy (LLD), anteromedial bowing of the tibia, valgus deformity of the knee, equinovalgus deformities of the foot and ankle, and absent lateral rays.3–5

The classifications4–6 are descriptive of the fibular pathologic abnormality, which does not require reconstruction, and lack descriptions of tibial and ankle pathologic abnormalities, which are the focus of reconstruction.7,8 The decision to recommend either reconstructive or ablative surgery to the parents of children with fibular hemimelia is difficult and debatable in the orthopedic literature.3,7,8 Moreover, persistent or recurrent foot and ankle deformities and recurrent valgus deformities of the knee, remained unsolved9 and are the primary reason for unsatisfactory results.2 The primary aim in the management of fibular hemimelia is a stable plantigrade foot, with normal limb length and alignment,7 achieving the most effective gait. Thus, Paley classification, which is based on ankle joint morphology and stability, is helpful in planning surgical treatment.10

Reconstructive surgery involves foot deformity correction and limb length equalization. SUPERankle (SUPER is an acronym for Systematic Utilitarian Procedure for Extremity Reconstruction) is a combination of bone and soft tissue procedures developed by Dr Paley in 1996 that stabilizes the foot and addresses all of the deformities.7,8 In addition, the introduction of the Ilizarov method for limb lengthening has provided an attractive alternative to amputation. Some surgeons prefer this method because it can provide simultaneous correction of the LLD and tibial deformities.11–13

Herein, we review our total experience of deformity correction followed by Ilizarov lengthening in patients with fibular hemimelia.

METHODS

A retrospective study was conducted, after obtaining approval from our institutional ethical committee, with consent from all of the patients. All patients with fibular hemimelia surgically treated at our institution between December 2000 and December 2014 were reviewed. Patients lost to follow-up or initially surgically treated elsewhere were excluded. The final sample included 29 patients (12 boys and 17 girls). The right side was affected in 14 limb segments and the left side in 15 limb segments. Patients were classified according to the Paley classification system7,8,10 for fibular hemimelia (Table 1 and Figs. 1, 2). The number of patients for within each classification is shown in Table 2.

TABLE 1
TABLE 1:
Paley Classification
FIGURE 1
FIGURE 1:
Paley’s classification type 1: tibial shortening alone with a normal ankle (A); type 2: tibial shortening with dynamic valgus at the ankle (B). ADTA indicates anterior distal tibial angle; LDTA, lateral distal tibial angle. Reproduced with permission from Dr. Dror Paley and The Paley Advanced Limb Lengthening Institute (www.paleyinstitute.org/). The SUPERankle technique was developed by Dr. Paley in 1996. Copyright The Paley Advanced Limb Lengthening Institute, Florida. All permission requests for this image should be made to the copyright holder.
FIGURE 2
FIGURE 2:
Paley classification type 3a: fixed equinovalgus at the ankle joint (A); type 3b: Fixed equinovalgus at the subtalar joint (B); type 3c: fixed equinovalgus at the combined ankle and subtalar joint (C); talar type (D). LDTA indicates lateral distal tibial angle. Reproduced with permission from Dr. Dror Paley and The Paley Advanced Limb Lengthening Institute (www.paleyinstitute.org/). The SUPERankle technique was developed by Dr. Paley in 1996. Copyright The Paley Advanced Limb Lengthening Institute, Florida. All permission requests for this image should be made to the copyright holder.
TABLE 2
TABLE 2:
The Number of Cases in Each Paley Type Category and the Associated Surgical Procedure
FIGURE 3
FIGURE 3:
Clinical picture (A) and orthoradiogram (B) of a 5-year-old male child with Paley type 2 fibular hemimelia with 3 cm of tibial shortening.
FIGURE 4
FIGURE 4:
X-ray of the same patient as in Figure 3 after treatment with tibial lengthening using the Ilizarov method as an external fixator (A). Clinical picture showing dynamic equinovalgus at the ankle (B), corrected with supramalleolar osteotomy and k wire pinning (C).
FIGURE 5
FIGURE 5:
Clinical picture showing the follow-up of the patient in Figures 3 and 4 at 15 years of age (A), with a stable ankle shown from the posterior (B) and anterior (C) views.

OPERATIVE TECHNIQUES

Among the 29 patients, reconstruction procedures were performed in 27 patients. Two patients (1 type 3A and 1 type 3C) underwent Syme amputation as a first choice of treatment. The procedures used for the different Paley types are shown in Table 2. Type 3 patients were treated using an a la carte SUPERankle procedure7–10 in 2 stages (Figs. 6–10).

FIGURE 6
FIGURE 6:
Clinical picture (A) of a 5-year-old male child with Paley type 3b fibular hemimelia with tibial shortening and anteromedial bowing (B). A magnetic resonance imaging shows that the os calcis is translated laterally to the talus (C).
FIGURE 7
FIGURE 7:
The deformity in Figure 6 was treated with partial resection of the fibular anlage, peroneal lengthening, and tendo-Achilles lengthening. An x-ray showing the anteroposterior (A) and lateral view (B) of the tibia, wherein a subtalar osteotomy was performed and fixed with k wires. Further treatment with lengthening using the Ilizarov method as an external fixator was performed. An x-ray showing regenerate callus formation at the corticotomy site in anteroposterior (C) and lateral views (D).
FIGURE 8
FIGURE 8:
Clinical picture (A) of the same patient as in Figures 6 and 7 at 12 years follow-up. Orthoradiogram (B) showing internal rotation of the tibia, genu valgum, and a shortening of 5 cm.
FIGURE 9
FIGURE 9:
The rotation, genu valgum, and shortening in Figure 8 were treated with derotational osteotomy and lengthening of the tibia. An x-ray of the patient in the anteroposterior (A) and lateral views (B) with osteotomized tibia and stabilized using the Ilizarov method. Clinical pictures (C, D) of the patient showing both patellas facing forward with Ilizarov fixation in situ.
FIGURE 10
FIGURE 10:
Orthoradiogram (A) of a patient at 14 years follow-up with 1 cm of shortening and 5 degrees of internal rotation deformity. Clinical picture of a patient at the same time showing internal rotation deformity (D) which is well compensated by external rotation at the hip joint (B). The patient is able to squat (E) and sit cross-legged (G) with a full range of motion at the knee and appreciably good motion at the ankle (C). The patient has 1 cm of shortening with a stable ankle (F).

In the first stage, a longitudinal lateral incision was made and the interosseous membrane and fibular anlage were dissected up to the level of the diaphyseal procurvatum deformity of the tibia. The fibrous fibular anlage was partially removed, up to the level of the apex of the tibial deformity, and the peroneal and Achilles tendons were lengthened. The peroneal and posterior tibial nerves were decompressed through separate incisions. Osteotomy was then performed according to the different Paley types as shown in Table 2. Finally, once the foot deformity was corrected, lengthening and tibial deformity correction was performed using the Ilizarov technique in the second stage. The associated deformities and their management are shown in Table 3.

TABLE 3
TABLE 3:
Associated Deformities and their Treatment

POSTOPERATIVE MANAGEMENT AND METHOD OF EVALUATION

Weight-bearing was permitted, with mobilization of the knee and ankle joint, the day after the Ilizarov procedure. Distraction was started on postoperative day 5 and continued at a rate of 1 mm/d. Once the desired length was achieved, the regenerate was allowed to consolidate. The Ilizarov fixator was removed after the consolidation phase. Once the deformity of foot was corrected, the patients used an ankle-foot orthosis brace until skeletal maturity to prevent recurrence. The final results were evaluated clinically, radiologically, and objectively using the ASAMI scoring system1,14 (Table 4).

TABLE 4
TABLE 4:
ASAMI Scoring

Statistical Analysis

After verifying the normality of the data, parametric tests of significance were performed. A paired t test was performed to compare the LLD before and after surgery. χ2 tests were performed to evaluate associations between qualitative variables.

RESULTS

The mean age at the first surgery was 4.06±2.40 years (range: 1 to 9 y). The study demonstrated a statistically significant relationship (P=0.03) between the age at the first surgery and recurrent foot deformities.

The mean follow-up duration was 9.37±2.02 years (range: 5 to 14 y). The mean LLD at the initial presentation was 3.55±1.08 cm (range: 2 to 5.5 cm), which was significantly improved to a mean of 1.01±1.09 cm (range: 0 to 3 cm) at the latest follow-up (P=0.015). Limbs were equalized to within 1 cm in 18 of 27 (66%) patients. Two patients had a shortening of 1.5 cm. Seven patients had a shortening of >2 cm. The mean external fixator duration was 7.74 ±2.62 months (range: 3 to 14 mo). The mean healing index was 1 cm/mo.

The results in terms of the ASAMI scoring system are shown in Table 5.

TABLE 5
TABLE 5:
Paley Type and the Resultant Distribution of Patients in Each Type, as Evaluated by ASAMI Scoring

Complications

A pin tract infection was the most common complication, followed by recurrent foot deformity (Table 6 and Fig. 11). Two type 2 patients and 4 type 3 patients had recurrence of an equinovalgus deformity.

TABLE 6
TABLE 6:
Complications and their Management
FIGURE 11
FIGURE 11:
Thirteen of 29 cases did not experience complications. The percentile representations of all the complications are depicted with respect to cases without complications.

DISCUSSION

Congenital deficiency of the fibula and associated anomalies of the lower limb represent a well-recognized clinical entity. Previous results2,15,16 have shown that lengthening and deformity correction is the preferred treatment for patients with mild to moderate leg length discrepancy with mild foot deformities (Paley types 1 and 2). Controversial cases include those with more severe foot deformities (Paley types 3 and 4) and greater leg length discrepancies.8

The techniques used in the present study had 2 variations from the original SUPERankle procedure as described by Paley.8 Paley practiced a combined SUPERankle procedure with lengthening in a single surgery, with complete resection of the fibular anlage. The current authors advocate a 2-staged procedure: deformity correction with the SUPERankle procedure followed by a lengthening procedure. The lengthening procedure was usually performed 1 to 2 years after the first procedure and was never performed before the age of 3 years. This variation helps in reducing limb lengthening complications and the incidence of ankle stiffness.7,8,17 In addition, complete resection of the fibular anlage does not completely eliminate proximal tibia valgus and is not necessary as 10 to 15 degrees varusization of the tibia at the end of the lengthening procedure can balance out the proximal tibia valgus that may occur with incomplete anlage resection.8 Moreover, complete anlage resection has a theoretical risk of injury to the common and deep peroneal nerves. The present study had only 1 case of recurrent genu valgum (Figs. 8, 9), which was treated during subsequent lengthening. Therefore, we recommend a partial resection of the fibular anlage up to the level of the apex of the deformity.

Outcomes associated with the reconstruction procedures are favorable. For example, Paley et al18 compared 22 patients treated with the SUPERankle procedure, combined with lengthening, to an age-matched group of patients who underwent Syme amputation; the results demonstrated no difference in function between the 2 groups. The big advantage of reconstruction surgery is that patient retains a sensate foot that can feel the ground, thereby providing balance and proprioception.7,8 However, Birch et al19 classified fibular hemimelia according to the number of rays of the foot and recommended amputation for most cases with less than 3 rays. The present study had 2 patients with 2 rays who underwent primary amputation as there was not enough surface area for a stable plantigrade foot.

The present study did not compare limb reconstruction to amputation because the current challenge involves improving the results of lengthening.7,8 An analysis of literature15,20,21 suggests that unsatisfactory results after a salvage procedure are mainly related to recurrent or residual foot deformities.8 For example, Naudie et al2 achieved satisfactory results in only 4 of 10 cases after lengthening and the reason for unsatisfactory outcomes involved residual or recurrent foot and tibial deformities.8 Cheng et al22 reported a similar experience in a small prospective group of 4 cases of lengthening, with unsatisfactory results secondary to recurrent tibial and foot deformities.

The current study revealed a statistically significant relationship between the age at the first surgery and recurrence of foot deformities. Hence, early treatment is important in reducing recurrence and unsatisfactory results. Five of 12 patients with an age at the first surgery of more than 5 years had recurrence, while only 1 of 15 patients with an age at the first surgery of less than 5 years had recurrence. Paley8 prefers to perform this procedure when the patient is between 18 and 24 months of age, performing lengthening at the same time. We have performed SUPERankle as early as 12 months, with lengthening performed as a next-staged procedure. Furthermore, genu valgum at the knee contributes to recurrent deformities at the ankle. As there is usually no subtalar joint present, genu valgum cannot be compensated by a mobile subtalar joint.7,8 It is therefore important to identify and treat knee valgus to improve the results of the foot correction and to help prevent recurrent ankle valgus.7,8 Despite the above precautions, recurrence is still a problem. The theoretical explanation is that by reorienting the ankle joint via supramalleolar osteotomy, the distal tibial growth plate is maloriented. This could lead to gradual recurrence, which can be addressed at the time of next staged lengthening.7,8

Previous studies have shown the effectiveness of lengthening for fibular hemimelia. Using gait analysis Johnson and Haideri23 showed that, successful lengthening resulting in plantigrade feet and well-aligned tibia, is associated with better ankle push-off strength and better knee flexion strength compared with that in patients who underwent Syme amputation.8 Furthermore, Catagni et al1 reviewed 32 patients with type 3 fibular hemimelia treated with successive lower limb lengthenings and deformity correction using the Ilizarov method; nearly equal limb length and a plantigrade foot were achieved in half of the patients. Similarly, Jawish and Carlioz11 reported good correction of the foot in 60% of cases of fibular hemimelia treated by lengthening. In addition, Paley7,8 (in his unpublished results presented at AAOS 1999, Anaheim, CA) was able to achieve good or excellent functional results, including the desired goal of lengthening, in 36 of 38 lengthened legs.8

Similarly, the present study had favorable results. According to the ASAMI scoring system, 15 of 27 cases had excellent results. Among patients classified as Paley type 1, the majority had excellent ASAMI scores and all had at least a good score. In contrast, among Paley type 3a cases, some patients had poor and fair scores. In addition, none of the type 3c cases had a score of more than fair. However, as the number of cases within each type was small, an association between Paley type and ASAMI scores could not be verified statistically. Two patients with ankle fusion were rated as fair. The ankle fusion was performed as a successful way of permanently stabilizing the foot12 and the patients did not require any additional surgeries for the ankle or foot. In addition, the present study had excellent follow-up duration, with a mean of 9.37 years and a maximum of 14 years.

In the present study, 18 of 27 patients had limbs equalized to within 1 cm. Two patients had a shortening of 1.5 cm. These patients did not require any additional surgeries and were managed with the help of a shoe rise. Patients with a shortening of more than 2 cm were lengthened again after a minimum gap of 2 years.

Limb lengthening with an external fixator alone is fraught with complications during the long external fixator period.24 Early removal of fixator will decrease most of the associated complications and can be done with the help of a lengthening over plate and recently designed slotted plates by the current author.25 Delayed union of the corticotomy site was due to frame instability, associated with a grade 2 pin tract infection and wire loosening. This was treated with debridement and wire exchange, thereby stabilizing the frame. Three patients had knee subluxation and was associated with femoral lengthening.24 This was treated by reducing the knee joint with the addition of a tibial ring.

The main limitation of the present study is that, due to relative rarity of this condition and small sample size, a comparison in functional outcomes and complications between the different Paley types was not possible.

CONCLUSIONS

Our long-term follow-up clearly suggests that limb reconstruction according to the Paley classification is an excellent option in the management of fibular hemimelia. Our 2-staged procedure of SUPERankle followed by lengthening with a minimum gap of 1 to 2 years helps in reducing the complications of limb lengthening and ankle stiffness. Performing the first surgery at an earlier age (below 5 y) plays a significant role in preventing recurrent foot deformities. Nevertheless, the surgeon must be thoroughly trained to get the desired result.

REFERENCES

1. Catagni MA, Radwan M, Lovisetti L, et al. Limb lengthening and deformity correction by the Ilizarov technique in type III fibular hemimelia: an alternative to amputation. Clin Orthop Relat Res. 2011;469:1175–1180.
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8. Paley D. Surgical reconstruction for fibular hemimelia. J Child Orthop. 2016;10:557–583.
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11. Jawish R, Carlioz H. [Conservation of the foot in the treatment of longitudinal external ectromelia]. Rev Chir Orthop Reparatrice Appar Mot. 1991;77:115–120.
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14. Shahid M, Hussain A, Bridgeman P, et al. Clinical outcomes of the Ilizarov method after an infected tibial non union. Arch Trauma Res. 2013;2:71–75.
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17. Herzenberg JE, Shabtai L, Standard SCSabharwal S. Fibular hemimelia: principles and techniques of management. Pediatric Lower Limb Deformities: Principles and Techniques of Management. Cham: Springer International Publishing; 2016:427–454.
18. Paley DBJ, Specht S, Ward S, et al. Limb reconstruction or amputation for severe fibular deficiency: a two-center comparison. Presented at the Annual Meeting of the American Academy of Orthopaedic Surgeons; February 14–19, 2011; San Diego.
19. Birch JG, Lincoln TL, Mack PW, et al. Congenital fibular deficiency: a review of thirty years’ experience at one institution and a proposed classification system based on clinical deformity. J Bone Joint Surg Am. 2011;93:1144–1151.
20. Patel M, Paley D, Herzenberg JE. Limb-lengthening versus amputation for fibular hemimelia. J Bone Joint Surg Am. 2002;84-A:317–319.
21. Birch JG, Walsh SJ, Small JM, et al. Syme amputation for the treatment of fibular deficiency. An evaluation of long-term physical and psychological functional status. J Bone Joint Surg Am. 1999;81:1511–1518.
22. Cheng JC, Cheung KW, Ng BK. Severe progressive deformities after limb lengthening in type-II fibular hemimelia. J Bone Joint Surg Br. 1998;80:772–776.
23. Johnson CE, Haideri NFHerring JA, Birch JG. Comparison of functional outcome in fibular deficiency treated by limb salvage versus Syme’s amputation. The Child With a Limb Deficiency. Rosemont: American Academy of Orthopaedic Surgeons; 1998:173–177.
24. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res. 1990;250:81–104.
25. Kulkarni R, Singh N, Kulkarni GS, et al. Limb lengthening over plate. Indian J Orthop. 2012;46:339–345.
Keywords:

fibular hemimelia; SUPERankle procedure; Paley classification; Ilizarov

Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc