Introduction
Ilizarov frames provide a versatile fixation system for the management of bony deformities, fractures and their complications. The frames give stability, soft tissue preservation, adjustability and functionality allowing bone to realise full osteogenic potential. In cases of congenital deformity, most structures in question are rudimentary, malformed or even absent. Deformity correction and lengthening in such cases requires thorough examination of the patient, alongside extensive investigations to chalk out the abnormal anatomy. Here, we present a case of deformity correction in a case of right tibial hemimelia in adult.
Case Presentation
A 17-year-old female patient presented with congenital deformity of right leg and foot. She had complaints of callosities on the lateral aspect of the foot, as that was her weight bearing portion. Diagnosis of tibial hemimelia was made upon history and radiological findings. This tibial hemimelia of Paley type 2C.1, 2 The hips, knee, and left ankle joint was normal (Figure 1, Figure 2).The neurological examination of both lower limbs was normal. The patient wanted a plantigrade foot and limb lengthening to alleviate the back pain and contralateral knee pain.
Figure 3
Intraoperative 1st stage correction: A): rudimentary TA, B): osteotomy marking, C): anterolateral wedge osteotomy, D): closed wedge osteotomy, E): plantigrade foot, F): slotted plate
Figure 6
X-rays after 3rd stage surgery: A): proximal rings removed, B): distal locking of slotted plate
Treatment was staged as follows:
Anterolateral close wedge osteotomy fixed with a distal end radius (DER) plate and tibial lengthening with 6 cm slotted plate and corticotomy
Revision of ankle correction by plate removal and Ilizarov fixator application to foot
Strayers release
Ilizarov removal
Prior to surgery, an MRI scan and doppler study was undertaken to delineate aberrant vessels and structures which might pose risk during surgery. It suggested an altered course of dorsalis pedis artery and posterior tibial artery. The nerves were normal in course. Posterior tibial tendon, peroneal tendons and tendoachilles were rudimentary distally. We marked the dorsalis pedis artery and posterior tibial artery with a doppler prior to incision.
An anterolateral incision on the distal tibia was taken after inducing the patient under spinal anaesthesia. The soft tissues were carefully dissected, and distal tibia was approached. Image Intensifying Television (IITV) was used to check the position of the wedge to be excised and marked with K-wires. With a mini saw the predetermined wedge was removed and deformity was corrected. The distal pulses were palpable despite the 1 cm of shortening caused. A DER T-plate 3(H) was contoured, and the osteotomy was fixed. The wound was closed. The surgery was continued proximally and as a standard procedure the limb lengthening with slotted plate was performed.3, 4 Postoperative period was uneventful, however after one week the lateral skin callosity started to blacken with a superficial eschar. It was manged by regular dressing, and it healed well. Gradual distraction at the speed of 1 mm/ day was initiated after 10th day and progressed smoothly (Figure 3, Figure 4).
The foot deformity needed further correction to plantigrade, thus one month later it was revised to an Ilizarov foot frame by removing the plate placed in situ. Further correction was possible and plantigrade foot was achieved (Figure 5). As the lengthening continued, the patient experienced pain, which was increasing daily. Thus, a third surgery of Strayers release was performed. She was walking bearing full weight on the fixator thereafter. 3 months after achieving 6 cm of lengthening, the distal screws of the slotted plate were locked and Ilizarov fixator was removed (Figure 6).
The foot ring was removed 1 month thereafter, and a protective above knee cast was applied for 3 weeks (Figure 7).
Results
Deformity correction achieved. Total duration of treatment was of 5 months. Good regenerate was visible along the treatment. 6 cm of lengthening was achieved in 3 months. Ring fixator for foot was kept for 3 months and was removed 1 month after the tibial rings were removed. Patient is walking with a plantigrade foot, and a shoe raise to accommodate the remaining of 6 cm of tibial shortening (Figure 8, Figure 9). Complications like pain and skin necrosis were encountered during the treatment. They were dealt with effectively.
Discussion
Deformity correction in congenital cases is very challenging. Correction in such patients is difficult as the structures are rudimentary5, 6, 7 and the behaviour of neurovascular structures cannot be predicted in acute correction.8 Hence, stage wise correction is advised. Correction in our patient was achieved over a period of 5 months in 4 stages of surgery. Many authors suggest below knee amputation for cases of tibial hemimelia, but according to Paley,1, 9 correction of foot deformity to plantigrade and lengthening gives a functional limb. An article by Yadav enumerated the result of femoral-fibular-calcaneal fusion at early age,10 but in this case, as the ipsilateral knee and femur were normal and ankle already fused, the correction of deformity with length sufficed. It can be thus stated, that here amputation was avoided and functional limb was salvaged by the given treatment.11 One article by Balci. H states the importance of knee stability for deciding the treatment course.12 Many authors now believe reconstruction can be the new road for tibial hemimelia.11, 13, 14 Certain complications should be anticipated and addressed like pain, compartment syndrome, nerve injuries and so on.15, 16