Morbus Charcot-Marie-Tooth - Case-report, Orthopaedic Therapy

Overview

The authors present the case-history of a girl with hereditary motor sensory neuropathy - morbus Charcot--Marie-Tooth type 2 axonal, whom they admitted to care at the orthopaedic clinic at the age of 9 years. The patientwhere the first manifestations of the disease started at the age of 3 years and already at the age of 6 years she hadsevere deformities of the feet was treated almost up the age of 10 years conservatively and surgery was hotrecommended.Surgery of the soft tissues, consecutively on both feet markedly improved the condition or even achievedplantigrade position of the left foot, on the right foot after an interval of 1.5 years progression of the defect calledfor surgery of the bones in the tarsal region.The authors draw attention to the difference in morbus Charcot-Marie-Tooth type 1 - demyelinization and inparticular type 1A which ismost frequent, where as a rule the deformities of the feet in children were not somarkedand the progression was not so rapid.The authors describe the most frequently used surgical tactics in these patients and mention triple desis asa definite operation in progressive neuromuscular diseases.The authors present favourable results after surgery of both feet in a female patient with morbus Charcot-Marie-Tooth with achieved verticalization, though the operation is not yet final and the patient will have anotheroperation of the bones in the area of the left foot. As, with regard to age, only a partial resection is involved, it maybe assumed that after termination of growth a triple desis will have to be performed.

Key words:
morbus Charcot-Marie-Tooth, demyelinization type, axonal, pes equinovarus, surgical treatment