Der kongenitale tibiale Reduktionsdefekt : neue Erkenntnisse in Diagnostik, Klassifikation und Therapie der kongenitalen Tibiaaplasie/-hypoplasie unter besonderer Berücksichtigung der Klassifikation und der extremitätenerhaltenden Therapie nach Weber

摘要

In this thesis the previous knowledge and understanding shall be documented and complemented with regard to diagnostics, classification and therapy of the congenital tibial diminution defect (congenital tibia aplasia - CAT). So as an introduction a detailed representation of the present state of science is carried out followed by a comparison with a group of own patients containing 66 people. In this work a detailed literature summary is included reflecting the international bibliography of the last 170 years. Special attention has been layed on diagnostics, classification, and therapy of the congenital tibia aplasia. In the first part of this thesis it was possible to show that a classification, even for complex deformaties caused by the tibial diminution defect, should be both, clinically simply applicable and of prognostic relevance as well. The classification after Weber (Prof. Dr. Med. Michael Weber) is portrayed in detail. Under the premise of the use of modern imaging methods a reliable classification of the congenital tibia aplasia is granted including all types of deformities and – for the first time - considering particularly also possible cartilaginous tibial moldings. Applying this method in combination with a score for the first time this classification has a prognostic significance. For clarification this classification method after Weber has been applied, comparing it with the well accepted classification models after Jones et al. and Kalamchi et Dawe addressing 66 patients (98 extremities) using their criteria. It was possible to demonstrate that by only using Weber´s classification method all extremities could be divided up thus excluding a misdiagnosis by use of modern radiological techniques and that, despite its simple applicability, the variable shaping of accompanying deformities can be associated. In the second part of this thesis the extremities conserving therapy of the congenital tibia aplasia after Weber was represented in detail in the view of looking at a quota of 24 patients. Among others a detailed analysis of the therapy progression is carried with regard to the number of operative interventions, stationary clinic stays and complications. Special attention has been layed on the therapy of the total tibia aplasia by means of application of the knee-joint reconstruction after Weber. Over several years, movement size and axial ratios of 14 limbs which had been treated with a knee-joint reconstruction after Weber were watched and evaluated during the therapy. It could be illustrated that the limb conserving therapy after Weber is applicable even for severe forms of aplasia.