Ein Beitrag zur operativen Behandlung der Osteogenesis imperfecta im Kindesalter

Summary

Two boys, 5 and 7 years old, suffering from osteogenesis imperfecta, with extreme deformation of the bones of the legs were subjected to surgery. Both were unable to stand or walk.

The purpose of the operation was to correct the position of the bones, to prevent contracture of muscles and relative shortening of vessels and nerves, and finally to support stability of bones by internal fixation.

To correct the position of the bones osteotomy was performed at locations and angles determined by drawings copied from X-ray-pictures. In one case 8 osteotomies were necessary, 2 on each femur and 2 on each tibia. The pieces were stabilized by Rush-Pins or very thin Küntscher-nails.

Though fractures occurring after the operation required renewed bloodless correction, the results were on the whole satisfactory: the boys were able to stand and even to walk, supporting themselves on pieces of furniture; the intervals between new fractures become longer, and the bone structure became more compact.

The present state allows the conclusion that the earlier surgical correction is performed in children with osteogenesis imperfecta the better chance they have of social rehabilitation.

Zusammenfassung

An Hand von zwei Fällen wird über eine operative Stellungskorrektur der unteren Extremitäten bei Osteogenesis imperfecta berichtet. Die Ergebnisse hinsichtlich der Stellungsverbesserung sind gut, hinsicht lich der Belastbarkeit zufriedenstellend. Röntgenologisch ist die Knochenstruktur deutlich verbessert. Die Operation sollte möglichst früh vorgenommen werden, um den betroffenen Kindern eine möglichst normale schulische Entwicklung zu ermöglichen.