Biomedical subjects
Publications and source records attributed to M Matĕjícek.
A case of multilocular pigmented villonodular synovitis in a child is described. It is an uncommon disorder, slowly progressive. Investigation, diagnosis and treatment are very difficult.
Skeletal dysplasia represents a wide range of congenital diseases diagnosed mostly on the basis of a clinical and x-ray picture, exceptionally on the basis of the finding of biochemical changes of metabolism (e.g. mucopolysaccharidosis). The author presents a clinical and x-ray picture of a skeletal disease in two gypsy children (brother and sister) which differed from any other regularly described congenital bone dysplasia. The mentioned finding most of all resembled a rarely described disease (or rather only theoretically presupposed) in which there occur combined symptoms of spondylo-, epi-, spondylometa- and physeal dysplasia (= spondylo-epimetaphyseal dysplasia). Apart from the clinical picture the author presents also the philosophy of therapeutical tactics. Optimal treatment depends on the experience of the physician, the clinical picture of the patient and his/her ability as well as the ability of the people round him/her to cope with the postoperative rehabilitation. The treatment must aim at ensuring for the patient the greatest possible independence and self-sufficiency, self-service and adequate work--this all of course according to his/her mental abilities, which unfortunately are due to the skeletal dysplasia usually reduced.
The authors deal with etiology, pathological anatomy and clinical finding in inborn high-riding scapula--the so-called Sprengel deformity. What is the best treatment tactics? Is the conservative treatment more suitable than the surgical one, or vice versa? By comparing their own experience with the data in the specialized literature the authors advocate a strictly individualized approach to each affected patient. After determining the diagnosis of Sprengel deformity it is necessary to evaluate the position of the scapula and the extent of the limitation of the range of motion of the respective shoulder-girdle. At the same time it is essential to consider the ability of the child and his parents to cooperate in the period of the postoperative rehabilitation. The rehabilitation carried out properly in the postoperative period is equally important as the perfect performance of the surgery. In more severe grades of affection combined with greater functional limitation of the ipsilateral shoulder the authors recommend the surgical treatment using the Green procedure with additional subperiosteal osteotomy of the ipsilateral clavicle (morcellation). The aim of the operation consists in the reduction of the scapula with simultaneous extraperiosteal release of most muscles inserted on the scapula. At the same time the os omovertebrale, if present, is always removed. The congruency of the anterior surface of the scapula and the thoracic wallis achieved by the resection of the supraspinatus part of the scapula. The proper position of the reduced scapula is ensured both by the reinsertion of muscle insertions to new, functionally more advantageous parts and by the fixation of the interior angle of the scapula in a newly created "pocket" in m. latissimus dorsi. Immediately after operation the proper position of the scapula is ensured by wire cerclage, distally anchored to the contralateral spica cast. The authors evaluate a group of 7 patients operated on at the Orthopaedic Clinic of the Institute for Further Education of Physicians, Teaching Hospital at Bulovka in Prague in the period of 1980-1987. Ensuing from the evaluation is the fact that in all deformities of III grade according to Cavendish the results achieved were satisfactory. On the other hand, the deformities of IV grade are difficult to treat even by operation. Only in one case the result was partially successful. In the conclusion the authors recommend a timely screening of the deformity, its timely operation (up to the age of 4 years) and indication carefulness in elder children.
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