THE KNUD JANSEN LECTURE; The operative treatment of congenital limb malformation-part III
E. Marquardt *
In certain malformations of the lower limbs it is necessary to intervene surgically to make it possible for the child to walk, or to improve its walking ability. Surgical intervention is carried out at an appropriate stage in the child's development and in close co-operation with the prosthetist and orthotist.
Longitudinal deficiency tibia, total or partial
Orthopaedic-surgical treatment cannot be avoided if a child with this condition is to walk. If possible the operation is carried out at the suckling age, so that walking can start according to the child's development.
If, in the case of longitudinal deficiency tibia total, the femoral condyles and the knee capsule are normal, and if the child is to be operated on not later than in his second year of life, the Brown (1965) procedure—that is the construction of a knee joint between the femoral condyles and the head of the fibula—is indicated.
During a second operating session the distal end of the fibula is fused with the astragalus or calcaneus according to Blauth (1978) in preference to disarticulation of the ankle joint. From the third year of life, disarticulation of the knee joint is the method of choice if the tibia is totally lacking and a normal femur is present.
If the distal femur is hypoplastic, there may be a more or less serious disturbance of the growth and, after a knee disarticulation, a cone shaped, eventually mid-thigh and poor load carrying stump end. In such a case, especially if malformations of the upper limbs are present, fusion between the condyles of the femur and the head of the fibula is recommended; also between the distal end of the fibula and the astragalus or calcaneus, if necessary with ensuing partial amputation of the foot (Marquardt, 1981). Advantages in comparison with knee disarticulation are the better end bearing capacity and the self-supporting and rotation-stable fixation of the orthoprosthesis with Velcro closures which a child with a hand or arm disability may manage more easily than a knee disarticulation prosthesis (Fig. 1 , left).
In cases of longitudinal deficiency of the tibia partial there is a better chance of success if the patient is operated upon as a baby, but even for the young infant there is a good chance that a load carrying leg with a functioning knee joint can be provided by surgery. Correct interpretation of the radiological signs is essential as the proximal epiphysis of the tibia is often not visible on the X-ray (Jones et al, 1978). Meyer (1980) osteotomizes the fibula subperiosteally, preserves the periosteal tube and merges the distal fibular fragment with the chondric tibial rudiment. In Heidelberg, I transfer the proximal epiphysis of the fibula to a central position below the tibia rudimentum, stabilize with Kirschner wires and join the shaped proximal epiphysis of the fibula to a manchette of the periosteum of the tibia rudimentum and to the patellar ligament (Fig. 1, right and Fig. 2. Fig. 3, Fig. 4, Fig. 5, and Fig. 6 ). For larger tibial rudiments which can be seen on the X-ray we osteotomize the fibula closely proximal to the level of the distal end of the tibia and fuse the distal fibular fragment to the tibial stump (Fig. 7 ).
Longitudinal deficiency fibula, total or partial
Accompanying malformations are shortening, bowing of the tibia, pes equino-valgus, fibular ray deficiencies of the foot and synostosis between the astragalus and calcaneus. Moreover, in some cases there may be disturbance of the lateral part of the proximal growing plate of the tibia. There is often a dimple-like skin retraction above the bowing of the tibia, a shortening of the triceps surae, often with fibrous tissue similar to the musculus sternocleido-mastoideus in torticollis, and connective tissue tending to contracture as a result of being a rudimentum of a non-ossified fibular-anlage.
In all severe cases with more than one ray deficiency of the foot, severe shortening and bowing of the tibia, the method of choice is ankle disarticulation or the formation of a modified Boyd amputation stump combined with a corrective osteotomy of the tibia (Kruger, 1971). Contraindication for the amputation or the disarticulation is the presence of severe malformations of the upper limbs, in which case the toes are required for grasping and particularly for self-care (Fig. 8 ). In cases where toes must be retained we carry out lateral arthrolysis of the ankle and, if present, of the talo-calcaneo joint, disconnection of the valgus contracture and posterior transposition of the peroneal tendon(s), if necessary, after three-dimensional correction osteotomy of the tibia (Fig. 9 ) (Marquardt, 1981). The good results of these correlated operations have caused us recently to be more cautious with the partial foot amputation or ankle disarticulation since it has been proved that children between three years and puberty should not be amputated for psychological reasons.
I am extremely sceptical about elongations of the tibia. Blauth (1978) has published favourable results, but in his publication nothing is said about the foot and the function of the ankle joint. Fig. 10 , left shows the frightening result of an elongation-osteotomy of the tibia that was carried out elsewhere when the patient was an infant; the foot is in extreme valgus contracture. Disarticulation of the upper talo-calcaneo joint accompanied by the formation of a modified Syme stump was the only acceptable solution (Fig. 10 , centre and right).
Longitudinal deficiency femur, total or partial: PFFD
Children with a unilateral subtotal deficiency of the femur (PFFD type Aitken D) can walk without surgery by using an orthoprosthesis with a SACH foot (Fig. 11 ). Sooner or later, however, cosmetic problems occur due to the foot which, being at knee level, stands out in trousers or under a skirt. In addition, cosmetic and static-dynamic difficulties occur with the knee joint which, at the level of the ischial tuberosity, becomes increasingly prominent.
Surgical procedures are; the forming of a modified Syme or Boyd stump (Kruger, 1971, 1981), the tenomyoplastic Chopart stump (Marquardt, 1973) and the Borggreve (1930) and van Nes (1950) 180° rotation-osteotomy of the shank and foot with arthrodesis of the knee joint. The tenomyoplastic Chopart disarticulation and the Borggreve-van-Nes operations pre-suppose a normal ankle joint. Thus, both operations are contraindicated in the case of a combined fibular and femoral deficiency.
The pros and cons of these operations must be carefully discussed with the parents and the patient with the help of other patients who have undergone surgery and with the aid of photographs and films. Consultation with patients who have already had the operation is most valuable so that the patient and parents can understand the transformation of the ankle joint into a knee joint by the Borggreve-van Nes operation (Fig. 12 ). The radically altered phenotype of the foot, which points backward, is well compensated for by the fluid walking pattern achieved which is comparable with that of the below-knee amputee. The foot is concealed by an orthoprosthesis of good cosmesis and for the swimming pool or beach a bathing prosthesis is provided.
We are still surprised that, about six months post-operatively, patients who have had this operation think in terms of knee movements while moving their ankle joint on the operated side—dorsiflexion causing knee flexion and plantarflexion causing knee extension.
Regarding bilateral PFFD type Aitken D, surgery is unnecessary for optimal fitting of orthoprostheses (Fig. 13, left). An absolute contraindication for amputation and the Borggreve-van Nes procedure is the case of bilateral PFFD combined with phocomelias or high level longitudinal and transverse deficiencies of the upper limbs (Fig. 13 , right).
I do not wish, in this contribution, to go into detail regarding the operative treatment of PFFD type Aitken A, B and C; it is a matter of aligning the neck of the femur by resection of the sub-trochanteric pseudarthrosis and taking advantage of the knee joint in the best way possible.
Transverse lower limb deficiencies require surgical intervention only if there is a threatened perforation by the bone through the skin of the below-knee stump (American Academy of Orthopaedic Surgeons, 1981).
I shall report in our next Congress about surgical stump elongations, for example, the elongation of an ultra-short above-knee stump to provide a reasonable length for fitting an above-knee prosthesis. The majority of the examples discussed of operative interventions, particularly for the lower limb, can provide maximum benefit for the patient only when supported by appropriate prosthetic care.
Operation, prosthetic technique, physiotherapy and ergotherapy are bound into the same rehabilitative concept with the goal of reaching, or at least facilitating, mastery of the patient's everyday life, integration into school, occupation and participation in social life, including sports and leisure activities.
I have shown only some small elements; a lot of work still lies ahead. In preparing ourselves for this responsible task, Knud Jansen has pointed out that the way is by multidisciplinary co-operation.
I wish to thank my co-workers in the Orthopaedic Workshop, in the Occupational and Physiotherapy Departments, in Psychology and Education and finally, our patients and their parents for their wonderful co-operation. I also wish to express my gratitude to Mr. H. Brünler for the photographs used in this contribution.
Aitken, G. T. (1969). Proximal femoral focal deficiency—definition classification, and management. In: Proximal femoral focal deficiency: A congenital anomaly, Publication No 1734, National Academy of Sciences, Washington, D.C.
American Academy of Orthopaedic Surgeons (1981). Atlas of limb prosthetics: Surgical and prosthetic principles. C.V. Mosby Co., St. Louis, Toronto, London.
Blauth, W., and Hepp W. R. (1978). Die angeborenen Fehlbildungen an den unteren Gliedmaßen. In: Chirurgie der Gegenwart, Band 5, Bewegungsorgane Verantwortlicher Fach-redakteur: P. P. Rickham, Zürich. Verlag: Urban und Schwarzenberg, München-Wien-Baltimore, 1-50.
Borggreve, J. (1930). Kniegelenksersatz durch das in der Beinlängsachse um 180 Grad gedrehte Fußgelenk. Z. Orthop. Chir. 28, 175.
Brown, F. W. (1965). Construction of the knee joint in congenital total absence of the tibia (paraxial hemimelia tibia): Preliminary report. J. Bone & Jt. Surg. 47-A(4), 695-704.
Brown, F. W. (1971). The Brown operation for total hemimelia tibia. In: Selected lower limb anomalies, surgical and prosthetics management, Ed. Aitken, G. T. National Academy of Sciences, Washington, D.C. 20-28.
Jones, B., Barnes, J. and Lloyd-Roberts G.C. (1978). Congenital aplasia and dysplasia of the tibia with intact fibula. J. Bone & Jt. Surg, 60-B (1), 31-39
Kruger, L. M. (1971). Fibular hemimelia. In: Selected lower limb-anomalies. Ed. Aitken, G. T. Washington, D C.
Kruger, L. M. (1981). Lower limb deficiencies. In: Atlas of limb prosthetics, surgical and prosthetic principles. American Academy of Orthopedic Surgeons, C. V. Mosby, St. Louis, Toronto, London, 522-552.
Marquardt, E. (1981). The multiple limb-deficient child. In: Atlas of limb prosthetics, surgical and prosthetic principles, American Academy of Orthopedic Surgeons. C. V. Mosby, St. Louis, Toronto, London, 627-630.
Marquardt, E. (1973). Die Chopart-Exartikulation mit Tenomyoplastic. Z. Orthop. 111, 584-586.
Meyer, L. C. Sehayik, R. I., and Davis, H. (1980). The telescoping bifurcation synostosis in the treatment of incomplete longitudinal tibial deficiency Inter-Clin. Inf. Bull. XVII (7), 1-8.
Van-Nes, C. P., and Meyer, L. C. (1950). Rotationsplasty for congenital defects of the femur. J. Bone & Jt. Surg. 32—B 12.
Additional bibliography for Part I of The operative treatment of congenital limb malformation, which was published in Vol. 4, No. 3.
Buck-Gramcko, D. (1975). Congenital malformations of the hand. Scandinavian J. Plastic Reconstructive Surg. 9,190-198.
Buck-Gramcko, D. (1975). Operative Behandlung angeborener Fehlbildungen der hand, Handchirurgie 7,53-67.
All correspondence to be addressed to: Prof. E. Marquardt, Leiter der Abteiling für Dysmelie und technische Orthopädie, Orthopädische Klinik und Poliklinik der Universität Heidelberg, 6900 Heidelberg 1, Germany.