PubMed2003
A case of Larsen's syndrome in a boy is presented. The syndrome is characterized by flattened facies, multiple congenital dislocations and foot deformities as a result of connective tissue maldevelopment during gestation. The importance of early intensive conservative therapy is emphasized. In the case reported, conservative therapy led, in the first stage, to the management of subluxation of both hips and improvement in left foot position and in the state of both knees. Subsequently, dislocations of the right and the left knee joint were surgically treated at 16 and 20 months, respectively, by reduction and transfixation with Kirschner's wires according to Niebauer and King. After six weeks of plaster immobilization, the wires were removed and the knee position was corrected with laminate splints. The authors point out that the syndrome is often associated with cardiovascular anomalies. Although these were not present in the case reported, due to the repeated occurrence of pericardial exudates from 2 years of the child' age, it was not possible to manage his left foot (pes equinovarus) surgically earlier than at 36 months. For that procedure, the method of posteroplantar release was used. The authors also discuss the occurrence of vertebral anomalies in Larsen's syndrome that may lead to cervical kyphosis and, potentially, to cervical spine instability. In our case, the signs of kyphosis were observed in the C1-C3 region but magnetic resonance imaging did not show any signs of either myelopathy or narrowing of the spinal canal. The intensive conservative and surgical treatment with subsequent thorough rehabilitation enabled the patient to stand up and walk without any support or orthotics. The fact that the boy was able to integrate in healthy children's playgroups can be considered a great achievement of orthopedic therapy.