Hope for Severely Disabled Children
Weber’s Limb Construction Techniques
Tibial Hemimelia (tibial reduction deficiency) is a very rare birth defect that occurs in every one million births. This disease is characterized by different amounts of reduction of tibial bone up to a complete missing tibial bone. This malformation can appear with a cartilaginous anlage (remnant of missing tibial part made of cartilage) or without. With the occurrence of the missing tibial part the adjacent joint (knee or ankle) is non-existent too. Children with this rare birth defect are not able to walk on the affected leg or if both legs are affected are not able to walk at all. The usual therapy for such children is to support walking with orthoprosthetics or after amputation of the affected limb with prosthetics. This disease is the most challenging disease to treat for Orthopaedic surgeons.
For these severely disabled children Prof. Dr. Michael Weber has developed proprietary techniques not only to prevent amputations, but also to improve enormously the function of the limbs and therefore life quality of the children. Dr. Weber has invented his so called “Transformations Surgery” and “Booster Surgery” for these type of malformations.
Weber’s-Transformation-Surgery means the conversion of a useless anatomical structure of the disabled limb into a useful anatomical structure. For example, the transformation of the fibula into the function of a tibia, or a knee cap (patella) replacing the part of a missing joint, see figs. 2-4. Once an anatomical structure has been transformed into a useful function, automatically, this structure develops the form intended. This extraordinary principle of the “form follows the function” works only in childhood. Therefore, the younger the children so better their biological capacities and so better the biological response and outcome to Dr. Weber’s-Transformation-Surgery.
Weber’s-Booster-Surgery means the enhancement of sleeping growth potentials of the cartilaginous anlage. Prof. Weber was the first in history who discovered the existence of the cartilaginous anlage in tibial hemimelia. This cartilaginous anlage is a remnant of the developing tibia in the fetus and has still growth potential, if 2 you know how to stimulate it. If there is no stimulation the cartilaginous anlage waste away. Prof. Dr. Weber found a way to stimulate the growth potential of the cartilaginous anlage by bringing them into contact with their joint partners. With this unique technique the cartilaginous anlage formulates the missing joint part, its own growth plate and later maturates into a tibial bone. The use of the cartilaginous anlage in tibial hemimelia is a breakthrough in the construction of missing joints and recovery of the original growth plates of the affected limbs.
With this technique Dr. Weber has given the malformed limb the chance to complete the missing parts in the fetal growth. This spectacular technique can be used to construct the missing tibial parts and the joints for five different types of tibial hemimelia (Types IIIa, IVa, Va, VIa and VIIa of Weber-Classification). Prof. Weber has treated worldwide the most patients with this rare disease. According to his classification of tibial hemimelia Prof. Dr. Weber has invented a unique technique for each of the 12 different types. These techniques are the most successfully worldwide. For the discovery of this innovative techniques Prof. Weber was awarded by the German and the European Society for Paediatric Orthopaedics.
Fig. 1. Weber-Classification of tibial hemimelia characterized in seven types and five subgroups (a = with cartilaginous anlage, b = without cartilaginous anlage) according to the severity of the malformation. Black = bone, blue = cartilaginous anlage and red circle = tibial defect without cartilaginous anlage. Hypoplasia = all limb structures present but underdeveloped. Diastasis = malformation of ankle joint where the foot is pushed between the two bones of the lower leg. Distal Aplasia = the lower part of the tibia is missing. Proximal Aplasia = the upper part of 4 the tibia is missing. Bifocal Aplasia = the upper and lower part of tibia is missing only a central part of tibia is present. Agenesia = total missing of tibia.
Fig. 2. Schematic drawing of knee construction transforming knee cap into tibial plateau, the knee capsule – beside its original function – into collateral ligaments and the fibula into tibia. (1.) Drawing of the incisions (red dotted lines a, b, c) into capsule (blue circle) required for creation of two visor flaps based medially and laterally. The quadriceps tendon is lengthened in Z-plastic manner and sutured end to end after visor flaps shift. (2.) In order to bring the knee cap into the position of a tibial plateau, the visor flaps have to be crossed contra rotating and sutured. The knee cap is connected to the fibular head.
Fig. 3. The transformation of fibula into tibia and the knee cap (red) into tibial plateau (joint) demonstrated in a lateral view of the limb. (a.) The X-ray shows the right leg before surgery at the age of 12 months. Note the small size of the fibula in comparison to my index finger. (b.) Schematic drawing of the limb before surgery. No tibia is present, there is no knee joint and ankle joint and the fibula has no function. (c.) After soft tissue lengthening with a ring fixator the fibular head can be placed under the femur and the foot under the lower end of the fibula with an acute maneuver via surgery. (d.) At the same surgery the knee cap shift via the double visor flaps is performed bringing the knee cap into position of a tibial plateau. Now the knee cap can work as a joint partner for the femur. The knee cap is fixed to the fibula. The foot is stabilized under the lower end of fibula. (e.) The Xray of the same leg one year after surgery. Note the tremendous size increase of the fibula (“form follows function”) in comparison to my index finger.
Fig. 4. (a.) The child has all four limbs malformed. (b.) After lengthening with a MiniRing-Fixator and performing the Weber-Transformation-Surgery (patella > tibial plateau, fibula > tibia) for creating a knee joint and a tibia on the right leg the patient has full weight bearing with very good range of motion (c.). The affected left leg was also treated successfully. Worldwide this technique is the only procedure preventing lower extremity limb amputation in children suffering from type VII-b Tibial Hemimelia. The treatment of the hands was performed later and subsequently the boy was able to play soccer (d.).
Fig. 5. Schematic drawing of operational procedure of type-Va tibial hemimelia. (a.) X-ray of a leg in 10month old girl before procedure. (b.) The tibial remnant has no contact to the joint partners of femur and foot. (c.) After cut of tibia the bone wants to heal and produces callus. The callus is soft and can be distracted (callus distraction). Both bone parts with the adhering cartilaginous anlage is transported towards their joint partners. (d.) The gap between the cartilaginous anlage of the upper tibia and the femoral condyles as well as lower tibia and the talus (bone of foot) is closed by bi-directional bone transport. (e.) The distracted callus maturates into full quality bone. (f.) X-ray of the same leg one year after first lengthening. Patient has full weight bearing, very good joint functions and can walk.
Fig. 6. Same patient as in Fig. 5. (a.) The malformed leg and foot before surgery. The additional toe has to be removed to enable the patient of wearing shoes. (b.) and (c.) Ring fixators are stabilizing the leg during lengthening process. (d.) and (e.) The X-rays show the creation of knee and ankle joint including 400% lengthening of tibial remnant after two lengthening’s.
Saeed Abdullah Mohamed Al Shehhi from Abu Dhabi, UAE is one of these children with tibial hemimelia. He was suffering of a Weber-type-IIIB tibial hemimelia of his right leg without cartilaginous anlage and severe deformities on his foot. The parents from desperately try to find a surgeon capable of doing treatments for such kind of malformation. The only answer what they got from surgeons of the region was that the leg has to be amputated. They tried everything to prevent this amputation and were willing to go to USA in order to get another treatment than amputation. The parents were amazed to find out that the specialist in USA is using successfully Before surgery During Surgery After 2. lengthening Fig. 6 Weber-Type-Va: Booster Technique 400% lengthening Before After a. b. c. d. e. knee area 9 techniques from Prof. Weber and he the inventor of these techniques is performing these specialized surgeries successfully in Dubai Healthcare City at Sulaiman Al Habib Hospital. The parents made an appointment with him and were very much relieved to see the huge collection of successful treatments of this rare disease with a long-term follow up of 25 years, the longest experience worldwide. For the first time they heard from the German Expert that an amputation is not recommended and that their boy will walk and even be able to play soccer after a couple of planned surgeries with the Special-Weber-Techniques. For the first time the parents felt to be in the best hands with their son and that their prayers were heard. They were told from Prof. Weber that the treatment will take 7 – 9 months with a fixator which ensures the stability of the leg and allowed all the corrective procedures. They were told that already with the fixator their boy will learn how to walk and after the removal of the fixator the leg will be of the same length than the healthy leg and with intensive physiotherapy he will be able to walk and run on his own with a functioning knee joint. And after more exercises he will even be able to play soccer.
All of it become true. The boy is able to walk on his own and even started playing soccer. The operated leg is protected at the moment with orthotic brace until the stability of the bone is increased and the knee has reached his full function.
Prof. Dr. Weber's Profile
Dr. Michael Weber is a world-renowned, German Board Certified Orthopaedic Surgeon as well as a Professor of Orthopaedics and Faculty Member of the internationally acclaimed University of RWTH in Aachen, Germany.
Awards & Affiliations
- 26 international honors and awards
- Honorary President of ASAMI Germany (Association for the Study and Application of the Method of Ilizarov)
- President of the German-Arabian¬Medical-Society
Special Expertise & Services
- Limb lengthening and limb reconstruction with advanced techniques (llizarov fixator, Taylor Spatial Frame and Fitbone nail)
- Cosmetic lengthening
- Minimally invasive arthroscopic surgeries
- Conservative and operative treatments in pediatric Orthopaedic.