Tibia Hemimelia-A Rare Case Report

Dharun Kumar M^* and Venkat kiran

^*Correspondence: Dharun Kumar M, Department of Orthopaedics, Sree Balaji Medical College and Hospital, Chromepet, Chennai, Tamilnadu, India, Email:

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Introduction

Hemimelia of tibia can have wide spectrum of pathological presentations, ranging from tibial hypoplasia to absent tibia, much more than that of the congenital fibular hemimelia. Tibial hemimelia can be present unilaterally or bilaterally. Bilateral presentation accounts for about 30% of cases [1]. Hemimelia of Tibia can be related to other congenital anomalies like those affecting the same side limb like syndactyly, dysplastic radius, polydactyly [2]. It also has many syndromic associations.

Jones classification [3]

This classification is based on plain x-ray findings, divided into 4 types, ranging from the most deficient to the least deficient.

• Jones type I -Tibia is absent.

• Ia–Femoral distal epiphysis hypoplasia.

• Ib-Tibia is not seen and with normal femoral lower epiphysis.

• Jones type II-Distal tibia is absent.

• Jones type III-Proximal tibia is not seen.

• Jones type IV-Presence of diastasis (Figure 1).

Figure 1: Jones classification.

Paley proposed a new modified classification

• Paley type 1: Hypoplasia of tibia: Genu valgum with excessive growth of fibula proximal part with normal plafond.

• Paley type 2: Tibial proximal and distal epiphysis present with dysplasia of ankle from proximal aspect of tibial; dysplastic tibial plafond; associated proximal fibula over growth.

• Paley type 3: knee joint and proximal tibia, medial malleolus present, no tibial distal plafond, with diastasis of tibiofibular.

• Paley type 4: Distal tibial aplasia.

• Paley type 5: Complete aplasia of tibial.

In tibial Jones type I b or II, there are some reports having good outcomes with tibiofibular synostosis [4-7]. The use of an external fixator prior to reconstruction, contractures can be reduced6. Schoe-necker [8] recommendation for Jones types Ib and II patients was TF synostosis in combination with a Syme’s amputation. These patients were functional as B-K amputees.

Spiegel et al. [9] described about side effect of amputation, in patients with Jones type II managed with amputation (Chopart or Syme) due to irritation of prosthesis because of the overgrowth and fibular head prominence.

Jones type IV treatment of choice is ankle stabilization, arthrodesis or amputation [10,11].

Case Report

History

A 10-year-old patient came with c/o deformity and shortened left leg since birth. History of difficulty in walking. No history of developmental disorder. No syndromic features.

Clinical features

Limb length discrepancy – left leg shorter than right leg by 12cm.Distal tibia could not be palpated. Tarsal and metatarsal appears to be normal. Foot in varus deformity (Figure 2 and 3).

Figure 2: Pre OP Imaging (Left Side).

Figure 3: Pre OP Imaging (Right Side).

Surgical treatment

According to type 4 Jones, we decided to treat the patient with fibular osteotomy under limb reconstructive system (Figures 4-8).

Figure 4:Surgical treatment.

Figure 5:After 2 months: Lateral epiphysiodesis done in proximal tibia.

Figure 6:After 3 months: A tibiofibular synostosis was carried out using one screw.

Figure 7:LRS was removed after 2months.

Figure 8:2 months follow up.

Discussion

Based on the Jones classification, our case corresponds to type IV. Jones classified Tibial hemimelia into 4 types, ranging from the most to the least deficient. Type 4 is tibia shortening with diastasis. They are various surgical treatments based on the type of tibial hemimelia, such as amputation, reconstruction and deformity correction, arthrodesis of ankle, tendons repair. Jones type 4 is equivalent to Paley type 3A. Paley did ankle arthrodesis, external fixator for type 3A, whereas in our study patient underwent above mentioned procedure. The outcome proved to be effective as paleys. Hence our treatment modality can be considered as an alternative option.

Conclusion

Jones type 4 is equivalent to Paley type 3A. Paley did ankle arthrodesis, external fixator for type 3A, whereas in our study patient underwent above mentioned procedure. The outcome proved to be effective as Paleys. Hence our treatment modality can be considered as an alternative option.

References

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