Tibial Hemimelia Treatment, Symptoms and Causes
Tibial Hemimelia Treatment
Tibial hemimelia is a rare congenital lower limb deficiency presenting with a wide spectrum of malformation, deficiency, and duplication. It present with variety of presentation including sever leg & foot deformity, limb shortening, weakness in quadriceps & absent cruciate ligaments and patella. It can be unilateral or bilateral. Presentation in tibial hemimelia is very wide. It can present with variety of presentation ranging from hypo plastic tibia to complete absence of tibia. Incidence of tibial hemimelia is one per millions. Dr Drohr Paley has classified tibial hemimelia according to deficiency.
Type 1: hypo plastic non deficient tibia with valgus knee joint
Type 2: dysplastic ankle with presence of both proximal & distal physis
Type 2a: well formed both proximal & distal tibial physis, equinovarus deformity of foot
Type 2b: delta tibia, bracket physis, malorientation of ankle & knee joint.
Type 2c: delayed or absent ossification (part or all) of tibia with dysplastic ankle joint
Type 3: presence of knee joint, absence of distal tibial plafond.
Type 3a: absence tibial plafond, medial and lateral
Type 3 b: same as type 3A with skin cleft separating tibia & fibula.
Type 4: distal tibia aplasia
Type 4a: complete absence of distal tibia from diaphysis, knee joint present.
Type 4 b: epiphysis of proximal tibia and knee joint present but absent proximal physis
Type 5 : complete tibial aplasia
Type 5a: Complete absence of tibia, patella present;
Flexion contracture of knee, equino-varus contracture of
Dislocated foot and ankle
Type 5B: Complete absence of tibia, no patella present;
Flexion contracture of knee, auto-centralized fibula,
Quadriceps present, knee capsule present
Type 5C: Complete absence of tibia, no patella present;
Flexion contracture of knee, dislocated fibula, quadriceps
Absent, no knee capsule present.
We follow the principle of Drohr Paley in reconstruction of these deformities. Treatment plan is based on classification given by Drohr Paley. In type 1valgus deformity of knee is corrected by hemiepiphyiodesis and limb lengthening by illizarov. In variety with intact knee joint and tibia, length and alignment of leg is restored in staged manner it include illizarov distraction to correct the deformity, tibialization of fibula and fibula- talar arthrodesis followed by limb lengthening. In child with complete absence of tibia & with presence of patella we use the procedure of brown arthroplasty in which patella was utilized to make knee joint & tibia was made use of fibula. with absent of patella and non functioning quadriceps knee disarticulation is needed. Usually first surgery is being done at the age 1.5 year. Serial limb lengthening may be required. In earlier years gold standard for treatment was the limb amputation with prosthetic fitting. With the drohr paley approach complete reconstruction can be done in most of this child and they can have palntigrade foot and equal limb length.
Tibial hemimelia is congenital absence of tibia. Its severity ranges from a minor deficiency in length to complete absence of tibia. Infant can present with sever deformity & shortening of leg, equinovarus deformity of foot & instability of knee & ankle joint. Drohr paley has classified this problem in five variety on the basis of tibial length deficiency in progressive manner & knee problem. In mild to moderate length discrepancy lengthening of tibia, tibialization of fibula, ankle fusion along with a lengthening of tibia is required. In sever deficiency, reconstruction or fibula femoral fusion of knee joint and stabilization of ankle joint on distal end of fibula can be done in some cases but in few cases amputation & artificial prosthesis is required. Child will need repeated lengthening and continuous monitoring. Usually, surgical reconstruction is being done between the age of 1.5 to 2 year. All these new surgical techniques is based on the concept of Drohr paley. Trishla foundation under the supervision of Dr. Jitendra Kumar Jain, a pediatric orthopedic surgeon has given new lease of life in many children affected by this problem.