Modification of the L'Episcopo procedure for brachial plexus birth palsies.
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We reviewed 19 children who had undergone a new modification of the L'Episcopo procedure for obstetric brachial plexus palsy. Through an axillary approach the latissimus dorsi tendon was re-routed anteriorly to the humerus and then anastomosed to the teres major tendon routed posteriorly. At an average follow-up of four years two months, the mean increase in shoulder abduction was 26 degrees and the mean increase in external rotation was 29 degrees. No neurovascular injury or postoperative infection occurred. Two patients had complications, and five did not gain from the procedure. The modified operation was relatively easier to perform and provided excellent cosmesis. (+info)
Ehlers-Danlos syndrome with soft-tissue contractures.
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We report four patients with a form of Ehlers-Danlos syndrome associated with soft-tissue contractures from birth and skin hyperalgesia. In early infancy, these cases were thought to be forms of arthrogryposis multiplex congenita, Larsen's syndrome or Marfan's syndrome. The characteristic triad of signs of Ehlers-Danlos disease gradually appeared from four to six years of age, allowing us to establish the correct diagnosis. We discuss the differential diagnosis of these connective-tissue disorders and the problems of the orthopaedic treatment of the associated joint deformities. Ehlers-Danlos syndrome is a heterogeneous group of metabolic diseases of collagen and our cases constitute a group which is distinct from the conventional types. (+info)
Permissive effect of fibronectin on collagen gel contraction mediated by bovine trabecular meshwork cells.
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PURPOSE: The effect of fibronectin on the contractility of trabecular meshwork (TM) cells was investigated. METHODS: The contractility of bovine TM cells was evaluated by culture of the cells in a collagen gel and measurement of the change in the diameter of the gel under various conditions. The formation of stress fibers and the localization of integrin alpha5 and beta1 chains (which together form a fibronectin receptor) in bovine TM cells were investigated by laser confocal microscopy of cells stained with phalloidin and antibodies to the integrin subunits. RESULTS: The addition of fibronectin to collagen gels containing bovine TM cells induced marked gel contraction in a time- and concentration-dependent manner. Cytochalasin D (an inhibitor of microfilament formation) and the peptide GRGDSP (Gly-Arg-Gly-Asp-Ser-Pro), a fibronectin receptor antagonist, each inhibited this effect of fibronectin, whereas nocodazole (an inhibitor of microtubule polymerization) and the control peptide GRGESP (Gly-Arg-Gly-Glu-Ser-Pro) did not. Furthermore, fibronectin induced the spreading of cells, the formation of actin stress fibers, and the expression of integrin alpha5 in the collagen gel-embedded TM cells. CONCLUSIONS: Fibronectin promotes collagen gel contraction mediated by bovine TM cells. Moreover, the formation of actin stress fibers and upregulation of integrin alpha5 appear to contribute to this permissive effect of fibronectin. The interaction of fibronectin with TM cells may thus be a determinant of the contractility of TM tissue. (+info)
Restrictive dermopathy in two sisters.
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Restrictive dermopathy (RD) is a very rare and lethal congenital skin disease. It is inherited by an autosomal recessive pattern with characteristic features of abnormally rigid skin, generalized joint contractures (arthrogryposis), and dysmorphic facies consisting of downward slanting eyes, a small pinched nose, low-set ears, a fixed open mouth in the O-position, and micrognathia. We report on 2 siblings from consecutive pregnancies affected with RD. They died of possible sepsis and respiratory insufficiency at 6 and 8 days after birth, respectively. This kind of stiff skin defect may lead to a fetal akinesia/hypokinesia deformation sequence, which causes the facial abnormalities of RD, as presented in these cases. (+info)
Outcome after later combined brachial plexus and shoulder surgery after birth trauma.
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Of 22 infants aged between 11 and 29 months who underwent a combined reconstruction of the upper brachial plexus and shoulder for the sequelae of a birth injury, 19 were followed up for two or more years. The results were evaluated using a modified Gilbert scale. Three patients required a secondary procedure before follow-up. Three patients had a persistent minor internal rotation contracture. All improved by at least two grades on a modified Gilbert scale. (+info)
Clinical analysis of the trigger thumb of childhood.
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Trigger thumb of childhood, termed congenital trigger thumb, is a pathology of the flexor pollicis longus tendon with an unknown etiology. In this study, treatment outcomes of 47 trigger thumbs of 36 children were evaluated. There were 18 males and 18 females with a mean age of 34 months (9 months-13 years). Average age of recognition of pathology by the family was 20.5 months (0-8 years). In seven of 11 bilateral cases pathology was recognized simultaneously while in the other four, diagnosis was made at different times. We used conservative treatment for all patients under three years of age, which was unsuccessful. Thus, surgical relase was performed in all cases. In the mean follow-up of seven years (range 5-15), contracture and palpable nodules disappeared. In conclusion, we believe trigger thumb in childhood should be treated surgically and that the term "congenital trigger thumb" should be changed to "developmental trigger thumb". (+info)
New method of limb deformities correction in children.
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A new "bloodless" technique (Ilizarov) was used to correct 36 limb deformities in 29 children. There were six leg length discrepancies, five achondroplasias, four deformed feet, five joint contractures, one rotational deformity of tibia, and in three the apparatus was used as an external fixator after corrective osteotomy. Lengthening was accomplished in 15 of the 16 procedures (93%). Average increase in femur length was 10 cm (32%), in tibial length 7.5 cm (30%), in humerus 11 cm (40%). Bony union was achieved in two out of five pseudoarthroses. Four deformed feet were fully corrected. Joint contractures were corrected in four out of five. The complication rate is as high as in other methods but with the Ilizarov apparatus, longer segments of bone were lengthened and more complex deformities were treated. Complications lessened as experience was gained. (+info)
A new method to measure post-traumatic joint contractures in the rabbit knee.
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A new device and method to measure rabbit knee joint angles are described. The method was used to measure rabbit knee joint angles in normal specimens and in knee joints with obvious contractures. The custom-designed and manufactured gripping device has two clamps. The femoral clamp sits on a pinion gear that is driven by a rack attached to a materials testing system. A 100 N load cell in series with the rack gives force feedback. The tibial clamp is attached to a rotatory potentiometer. The system allows the knee joint multiple degrees-of-freedom (DOF). There are two independent DOF (compression-distraction and internal-external rotation) and two coupled motions (medial-lateral translation coupled with varus-valgus rotation; anterior-posterior translation coupled with flexion-extension rotation). Knee joint extension-flexion motion is measured, which is a combination of the materials testing system displacement (converted to degrees of motion) and the potentiometer values (calibrated to degrees). Internal frictional forces were determined to be at maximum 2% of measured loading. Two separate experiments were performed to evaluate rabbit knees. First, normal right and left pairs of knees from four New Zealand White (NZW) rabbits were subjected to cyclic loading. An extension torque of 0.2 Nm was applied to each knee. The average change in knee joint extension from the first to the fifth cycle was 1.9 deg +/- 1.5 deg (mean +/- sd) with a total of 49 tests of these eight knees. The maximum extension of the four left knees (tested 23 times) was 14.6 deg +/- 7.1 deg, and of the four right knees (tested 26 times) was 12.0 deg +/- 10.9 deg. There was no significant difference in the maximum extension between normal left and right knees. In the second experiment, nine skeletally mature NZW rabbits had stable fractures of the femoral condyles of the right knee that were immobilized for five, six or 10 weeks. The left knee served as an unoperated control. Loss of knee joint extension (flexion contracture) was demonstrated for the experimental knees using the new methodology where the maximum extension was 35 deg +/- 9 deg, compared to the unoperated knee maximum extension of 11 deg +/- 7 deg, 10 or 12 weeks after the immobilization was discontinued. The custom gripping device coupled to a materials testing machine will serve as a measurement test for future studies characterizing a rabbit knee model of post-traumatic joint contractures. (+info)