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[Orthopedic considerations of trichorhinophalangeal syndrome type II]
F Gassel1, S Gassel, L Perlick
1Klinik und Poliklinik für Orthopädie der Rheinischen Friedrich-Wilhelms-Universität Bonn, Germany.
Introduction:
The trichorhinophalangeal syndrome type II or Langer-Giedion syndrome is regarded as a rare abnormity that is marked by a number of clinical characteristics beside multiple cartilaginous exostoses.
Results:
The deviation of the fingers within the scope of the TRPS II that is often reported in literature can not be found in the case at issue of a now 14 year old boy. The course of disease was complicated due to consecutive axis deviation of two large joints of the lower extremities being determined by the syndrome. Due to the marked exostoses in the area of the growth plate of the left knee joint a valgus deformity developed there. It was corrected with means of a temporary clamping of the growth plate. With the increasing valgus deformity of the right ankle causing a calcaneovalgus foot deformity the osteochondroma located at the distal fibula was also removed and a temporary clamping of the growth plate was carried out at the right medial malleolus. From earliest childhood repeating cartilaginous exostoses both at the extremities and the trunk attracted attention. Also strongly developed are the facial distinguishing marks which determine the typical shape of the face.
Conclusion:
By the case of a now 14 year old boy with severe orthopedic complications considerations are made concerning therapeutic principles due to the TRPS II.