|Year : 2021 | Volume
| Issue : 1 | Page : 52-56
Treatment of tibial hemimelia jones type 4 by ankle-sparing reconstruction (from birth to skeletal maturity)
Mark Eidelman, Pavel Kotlarsky
Ruth Rappoport Children's Hospital, Rambam Health Care Campus, Haifa, Israel
|Date of Submission||04-Apr-2021|
|Date of Decision||22-May-2021|
|Date of Acceptance||24-May-2021|
|Date of Web Publication||30-Jun-2021|
Dr. Pavel Kotlarsky
Ruth Rappoport Children's Hospital, Rambam Healthcare Campus, 8 Haaliya Hashniya Street, Haifa 3525408
Source of Support: None, Conflict of Interest: None
Tibial hemimelia is a relatively rare congenital preaxial longitudinal deficiency. The focus of this report is on Jones Type 4 (also called ankle diastasis). Treatment options range from amputation to tibiotalar arthrodesis with subsequent lengthening procedures. We present a case report of a patient with tibial hemimelia Jones Type 4 treated by ankle-sparing reconstruction with serial tibial- and fibular-lengthening procedures. To the best of our knowledge, this is the first report of ankle-sparing reconstruction followed until maturity.
Keywords: Ankle deformity, ankle diastasis, leg deformity, tibial deficiency, tibial hemimelia
|How to cite this article:|
Eidelman M, Kotlarsky P. Treatment of tibial hemimelia jones type 4 by ankle-sparing reconstruction (from birth to skeletal maturity). J Limb Lengthen Reconstr 2021;7:52-6
|How to cite this URL:|
Eidelman M, Kotlarsky P. Treatment of tibial hemimelia jones type 4 by ankle-sparing reconstruction (from birth to skeletal maturity). J Limb Lengthen Reconstr [serial online] 2021 [cited 2022 May 20];7:52-6. Available from: https://www.jlimblengthrecon.org/text.asp?2021/7/1/52/320058
| Introduction|| |
Tibial hemimelia is a rare congenital preaxial longitudinal deficiency, first described by Theodor Bilroth in 1861., It is the least common form of lower extremity deficiency, with an incidence of approximately one in a million live births. Children with tibial deficiency may also have grossly supinated feet, short femur, and hypoplastic distal femoral epiphysis. There are three main classifications for congenital tibial deficiency – Jones (first described in 1978 by Jones, Barnes, and Lloyd-Roberts) [Figure 1], Weber, and Paley. Although Weber and Paley classifications are more comprehensive, Jones classification is still the most popular, probably because it is the easiest to understand and remember.
|Figure 1: Jones classification of tibial hemimelia. Type 1a is a total absence of the tibia, in Type 1b, there is a remnant of the proximal tibia, in Type 2, the distal tibia is absent, in Type 3, the proximal tibia is not seen, and in Type 4, there is diastasis of the ankle|
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The focus of this report is on Jones Type 4, commonly called ankle diastasis. This is probably the most reconstructable type of tibial hemimelia; however, there is no consensus regarding the treatment of this condition. Treatment options range from Boyd or Syme amputations,, the standard treatments until recently, to tibiotalar arthrodesis with subsequent series of tibial lengthening procedures., Recently, Weber and Paley, described procedures for foot reconstruction; however, long-term outcomes of these reconstruction procedures after maturity have not been reported. We describe the result of ankle-sparing reconstruction from birth to maturity.
| Case Report|| |
The patient was born in 2001 with a short right leg and a clubfoot-like foot appearance. On the first examination at our institution at the age of 18 months [Figure 2]a, the leg length discrepancy (LLD) was 5 cm, and the foot was in rigid equinovarus position, uncorrectable by passive stretching. On radiographs, the fibula was significantly longer than the tibia and clear ankle diastasis was observed with an absence of distal lateral part of tibial epiphysis and talus wedged between the tibia and the fibula [Figure 2]b and [Figure 2]c. Projected LLD calculated using the multiplier method was 16 cm.
|Figure 2: (a-c) Clinical and radiographic appearance of the leg before initiation of treatment|
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The goals of the first surgery were creating a plantigrade and shoeable foot and restoring proper tibiofibular alignment by lengthening the tibia and shortening the fibula. These were achieved by percutaneous Achilles tendon lengthening, application of the Ilizarov apparatus with simultaneous correction of the foot alignment [Figure 3]a, resection of 25 mm of the fibula, temporary wire fixation between the proximal tibia and fibula, and 4 cm gradual tibial lengthening. After the first surgery, an appropriate proximal tibiofibular relationship was achieved, and the patient was able to wear standard shoes [Figure 3]b and [Figure 3]c. However, 2 years later, the fibula grew significantly longer again [Figure 3]. LLD was again 5 cm. Staged lengthening was performed using the Taylor Spatial Frame® (TSF, Trademark of Smith and Nephew, US Pat). First proximal tibial osteotomy and gradual lengthening were done. When the length of the tibia reached fibular length, mid-shaft fibular osteotomy was performed with fixation of the fibula to the tibia. Overall, lengthening of 5 cm was accomplished [Figure 4]a and [Figure 4]b with normal tibiofibular relationship and functional ankle despite preservation of the diastasis [Figure 4]c. To reduce the projected LLD, at the age of 12 years, a contralateral (left) epiphysiodesis of the distal femur (using 8 plates on both sides of the growth plate) and proximal tibia (by a percutaneous drilling and curettage technique) was performed [Figure 4]d. The total LLD at that time was 3 cm (left leg longer).
|Figure 3: Clinical and radiographic appearance after correction of rigid equinovarus, fibular and tibial osteotomy and first lengthening procedure. (a) Final frame configuration, (b) Clinical appearance after frame removal, (c) standing X-rays after frame removal. (d) Two years after lengthening procedure, the fibula became significantly longer|
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|Figure 4: (a and b) Staged lengthening of the tibia and fibula. (c) X-ray after lengthening. Note the symmetrical tibiofibular relationship with stable ankle. (d) X-rays at the age of 13 years, after two lengthening procedures and contralateral epiphysiodesis of the distal femur and proximal tibia. The total leg length discrepancy was 3 cm|
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At the age of 16 years, the patient had near-normal ankle function with a good range of motion (30° of plantar flexion and 15° of dorsiflexion). There were no pain and no footwear limitations. The tibiofibular relationship was preserved, and the total LLD was 40 mm [Figure 5]a, [Figure 5]b, [Figure 5]c. Since the proximal tibia was practically straight, the final lengthening procedure was performed using a Precice® magnetic nail (NuVasive, San Diego, CA, USA). To prevent dissociation between the tibia and the fibula, a transverse screw was implanted linking the bones above the ankle. Osteotomy of both bones was performed with uneventful 4 cm lengthening [Figure 5]d and [Figure 5]e. Despite the prominence of the medial malleolus, the patient had no pain, normal foot and ankle function, and refused any additional cosmetic procedure [Figure 6]a and [Figure 6]b. He reached skeletal maturity and had no LLD.
|Figure 5: (a-c) Clinical appearance and X-rays with 4 cm block to compensate for leg length discrepancy at 16 years. (d and e) X-rays after final 4 cm lengthening|
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|Figure 6: (a) Clinical picture at the age of 19 years. (b) Final X-ray at the age of 19 years, medial malleolar prominence can be seen|
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| Discussion|| |
We report a case of a patient with congenital tibial deficiency Jones Type 4 with ankle diastasis. The patient was born with rigid equinovarus foot deformity and 16 cm projected LLD. Hip, knee, and quadriceps mechanism were normal.
There are few Jones Type 4 cases described in the literature. Sedgwick and Schonecker described a patient who reached skeletal maturity after ankle mortise reconstruction by tibiotalar fusion, while 5 cm LLD was treated with a shoe lift. Schonecker et al. described 10 cases with reconstruction of the ankle in five; however, the other five patients had Syme amputation due to progressive LLD. Kalamchi and Dawe proposed performing calcaneofibular fusion when possible, and when this was not feasible, they recommended either talectomy or Boyd amputation. Tokmakova et al. described treatment of 11 patients where seven patients underwent amputation and three had ankle reconstruction with ankle fusion. Recently, several reports described ankle reconstruction surgeries involving soft tissue release procedures and ankle fusion [Table 1].
|Table 1: Treatment options described in the literature for distal tibifibular diastasis (only procedures addressing the diastasis are mentioned)|
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We did not find any description of foot reconstruction procedure that preserved ankle diastasis with follow-up until maturity. Our treatment goal was the preservation of ankle function without reduction of the diastasis (without intra-articular procedures), correction of equinovarus by gradual soft tissue distraction using the Ilizarov method, and equalization of LLD. After 19 years, the patient had reasonable ankle motion (15° dorsiflexion, 30° plantar flexion), plantigrade foot, and prominence of the medial malleolus that did not interfere with any standard shoe wear. The 13 cm LLD was treated by three lengthening procedures: first two by circular external fixators and the third by internal tibial lengthening device. The remainder of the expected LLD was avoided by contralateral epiphysiodesis. Eventually, the patient achieved normal life with good functional outcomes.
Although the alternative treatment of amputation and prosthesis would be a shorter procedure with only a single surgery, we believe that when the foot is normal, foot and ankle salvage might be possible. Recent reports describe a variety of ankle reconstruction procedures [Table 1], most commonly by ankle fusion procedures. However, while achieving a plantigrade foot, these procedures lead to loss of ankle motion. The limitation of this report is that it is impossible to predict the functional lifetime of our patient's ankle or to compare it to an alternative procedure that would reconstruct the ankle joint at the expense of range of motion. The importance of this report is to show that preservation of ankle motion is possible. Foot reconstruction can be performed using a circular external fixator and LLD can be treated by staged lengthening. Maintaining a normal tibiofibular relationship is also crucial for the success of treatment. If the function of the ankle will decline in future, ankle fusion procedure can be performed at a later stage.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The authors would like to thank JE Herzenberg for his continuous support and invaluable advice over many years.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]