Biomedical subjects
Publications and source records attributed to G Fabry.
After several round table discussions between the members of the Belgian Association of Pediatric Orthopedics (BAPO) a plan of treatment of congenital dislocation of the hip was adopted. The purpose of this publication, is to propose this plan as a basis for a general approach to the problem of congenital dislocation of the hip (CDH).
The technique of Ludloff is particularly indicated in treating congenital dislocation of the hip, under the age of two. The procedure is relatively simple, provides direct access to the most important pathology, limited blood loss and a good cosmetic result. In the period between 1981 and 1984, 26 congenital dislocations of the hip were treated in our department, by the Ludloff technique. A re-evaluation of these cases was performed after a period of 4 years and 6 months. 23 hips were concentrically reduced; there were 2 redislocations, 1 deep infection and 2 cases of avascular necrosis, type I.
This work presents early findings in the articular cartilage of dog knees in experimental hemarthrosis produced by injections of whole blood, red blood cells, white blood cells, serum, and iron citrate. The effect of coagulation and synovectomy was also studied. The fixed-charge density determination has proved to be a reliable, easy, and rapid method for finding - very early in the process - quantitative changes in cartilage ground substance. Changes in the proteoglycan (PG) content of articular cartilage matrix take place after a certain duration and intensity of hemarthrosis, after which loss of ground substance is repeatedly and consistently observed. After some time (6 months, and culminating after 14 months in our study) a reparative reaction takes place. In experimental hemarthrosis of longer duration this healing reaction seems to be overwhelmed by the degrading forces, with probable further evolution to the full-blown pathology. The cellular components of the blood seem to play a major role. Prophylactic synovectomy seems to be effective only in preventing further bleeding. Coagulation of the intra-articular blood does not alter its deleterious effect. Finally, a small iron ligand alone, although proven in vitro to penetrate into cartilage, is not capable of producing matrix changes.
Twenty-eight posterior tibial tendon transfers through the interosseous membrane were performed to correct spastic equinovarus. All patients improved their gait, and 82% discarded their brace. Foot striking at the beginning of the stance phase was good or satisfactory in 82%; 68% had a neutral position of the heel. The overall score was good in 68%, satisfactory in 21%, and poor in 11%. The main requirements for obtaining good results with this type of transfer are: no fixed varus deformity preoperatively, age at operation between 5 and 10 years, and reinsertion near the midline of the foot.
The purpose of this study is to compare preoperative and postoperative back pain problems in 182 patients operated on for idiopathic scoliosis. Preoperatively 32% of the patients presented with back pain, increasing to 66% postoperatively. Minor degrees of back pain increased (from 45 to 65%) and more severe complaints decreased (from 55 to 35%). Moderate to severe back pain increases from 0 to 46%, together with lower hook placement from L1 to L5. It is concluded that, ideally, the lower limit of the fusion should not go beyond L1 or L2. Lower fusions are prone to give more back pain, with major problems, however, only in a minority of cases (7% in fusions to L4-L5).
The clinical triad, described by Klippel and Feil in 1912 and consisting in a short neck, a low dorsal hairline and restricted neck mobility, is the result of synostosis of several cervical vertebrae, which may or may not be deformed. In recent years, however, even more attention has been given to associated urological, neurological, cardiopulmonary and locomotor deformities, which significantly increase the morbidity. A multidisciplinary approach and treatment are therefore required. We have studied 18 patients and examined 6 additional records. Of these 24 cases, 18 showed associated deformities. In 14 cases surgery was necessary, including cervical fusion for hypermobility in 3 cases, fusion to treat scoliosis in 5 cases, one cervical rib resection and one Sprengel deformity correction. In addition 3 patients needed cardiac surgery and one patient had a ureter reinserted because of vesico-ureteral reflux.
In this report we describe the occurrence of severe mesomelic shortening of the forearms due to hypoplasia of the ulnae with severe radial bowing. In contrast to the mesomelic dysplasias, i.e. Langer type of mesomelic dwarfism, this apparently autosomal dominantly inherited skeletal anomaly occurred as an isolated anomaly without concomitant involvement of shanks or other parts of the skeleton, and did not influence final adult height.
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A 14-year-old boy presented with the clinical and radiological features of rickets. Serum inorganic phosphate levels were constantly low, whereas serum calcium and parathyroid hormone levels were within the normal range. Laboratory investigation did not show any evidence for vitamin-D deficiency, chronic renal insufficiency, Fanconi syndrome, tubular acidosis, hepatic disease or intestinal malabsorption. A family study comprising 34 members over four generations revealed 10 other individuals to be affected and the mode of inheritance to be autosomal dominant. In addition to hypophosphataemia and normocalcaemia, the disease is characterized by elevated serum 1,25 dihydroxyvitamin D levels and hypercalciuria. This hereditary syndrome of renal hypophosphataemia differs from the common familial X-linked hypophosphataemia and the recently described autosomal recessive hypophosphataemic rickets with hypercalciuria by its dominant mode of inheritance; it differs from hypophosphataemic non-rachitic bone disease by the elevated serum 1,25 dihydroxyvitamin D levels and hypercalciuria.
This study reports on the late results of septic arthritis of the hip in 29 children, after an average follow-up of 12 years 7 months. The purpose of the study is to show the severe destruction caused by infection in children's hips and to reemphasize the necessity for early diagnosis and treatment. The 29 patients were divided into two groups according to age (0-4 weeks and 1 month-3 years of age) because of the different prognoses. Functional and anatomical evaluations of the hips were performed. In some cases there was no correlation between the functional results and the anatomical situation of the joint. Our results showed that the prognosis is worse in young children and also in cases with associated osteomyelitis of the proximal femur. Eleven of the 16 neonates were premature. Late reconstructive surgery is difficult and does not always give the desired functional and anatomical improvement. The most important factor influencing the end result, however, is the time between beginning of symptoms and treatment; that is the only factor, also, we can change to improve the ultimate outcome of the disease.
We present here a follow-up study concerning Legg-Calvé-Perthes. This study about 71 hips shows that the Catterall grouping, age and "at risk signs" are still to be considered as very important prognostical factors. A definitive and indispensable scheme of treatment has not yet been advanced, especially because the right etiology still remains an obscure point. The cited "classical" prognostic factors however remain all-important.
This study reports on the early findings in experimental hemarthrosis. Biochemical and histological analyses show the first signs of cartilage degeneration secondary to the presence of blood in the joint, and this for a certain duration. The lower limits of amount and duration, necessary to produce a reaction in synovium and cartilage are determined. Evidence is also given that the production of degradative enzymes, by the synovium especially, plays an important role, in the early phase of cartilage degeneration. A few hypotheses are discussed.
The results of 64 posterior fusions for scoliosis are presented. Also the first detection of the curves and the preoperative treatment is discussed. Details are given about the immediate and late postoperative complications and about the correction loss. Interesting too, is an investigation of more subjective information, concerning the postoperative physical and social status. These last problems are not very often discussed in studies of scoliosis.
A partial deletion of the short arm of chromosome 9 is reported in a female newborn and a 12.5 year-old male. The features expressed by both patients, and especially the peculiar type of the craniofacial dysmorphism, confirm the existence of a typical clinical syndrome associated with this partial autosomal monosomy.
A literature review is presented about triple arthrodesis and Lambrinudi arthrodesis including indications, techniques, complications, and a more extensive review of some specific indications. Forty-eight patients were operated on between 1961 and 1977, 25 of whom were reviewed at follow-up. Our follow-up study shows a rather high rate of pseudarthrosis, with however a normal failure rate. According to these findings transfixation of the bones with K-wires is to be considered.
The Dubowitz syndrome has been recognized during the past few years as a new, distinct intrauterine growth-retardation syndrome with autosomal recessive inheritance. One new patient is presented, a 10 1/2-year-old slightly mentally retarded girl.
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