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1/222. ehlers-danlos syndrome type VII: clinical features and molecular defects.

    We evaluated the clinical features, molecular defects, and problems associated with the management of two patients who had type-VII ehlers-danlos syndrome and reviewed the cases of eighteen patients with this condition who had been reported on previously. The typical clinical features associated with this syndrome include bilateral congenital dislocation of the hip; severe generalized hypermobility of the joints; multiple dislocations of joints other than the hip; muscular hypotonia; and hyperelasticity, fragility, and a doughy texture of the skin. Collagen and dna analyses demonstrated that both of our patients had type-VIIB ehlers-danlos syndrome, which is caused by heterozygous new mutations of the COL1A2 gene that encodes the proalpha2(I) chain of type-I procollagen. The obligatory GT dinucleotide at the splice donor site of intron 6 was altered in both of our patients: one patient (Case 1) had an A substitution of the G nucleotide, and the other patient (Case 2) had a C substitution of the T nucleotide. Abnormal splicing resulted in the loss of the exon 6-encoded N-telopeptide, which includes the N-proteinase cleavage site. Despite multiple operative procedures, one of our patients, who was thirty-seven years old at the time of the most recent follow-up, continued to have persistent subluxation of the right hip and osteoarthritis of the left hip. Closed reduction of the dislocated hips, regardless of the type of immobilization used, was unsuccessful in all twenty patients. The results of open reduction were improved when capsulorrhaphy was combined with iliac or femoral osteotomy, or both. ( info)

2/222. A new case of NSAID-induced infertility.

    A 38-year-old woman on piroxicam for hip osteoarthritis secondary to hip dysplasia developed secondary sterility. Ova collected for in vitro fertilization were immature and failed to fertilize. A further attempt done after piroxicam discontinuation produced seven mature ova that fertilized, allowing embryo implantation. Nonsteroidal antiinflammatory drugs may induce infertility by reducing the production of prostaglandins, most notably via inhibition of the enzyme cyclooxygenase 2. The impact of nonsteroidal antiinflammatory drug therapy on reproductive function needs to be evaluated. ( info)

3/222. Congenital dislocation of the hip in the newborn. A correlation of clinical, roentgenogrhic and anatomical findings.

    This study correlates roentgenography, arthrography, morbid anatomy and histology in four newborn infants with congenital dislocation of the hip. Three died shortly after birth. In two of these, manipulative reduction was impossible. Both demonstrated secondary adaptive changes at autopsy, indicating that the dislocation had been present for some time in utero. The first had a paralytic dislocation (meningomyelocele), and the second showed no pathologic findings other than the dislocated hip. The third infant had a dislocation that was reduced at birth. The child died of pneumonia on the 15th day. At autopsy, no secondary adaptive changes were found in the hip. The fourth infant, who had undergone open reduction because of a dislocation irreducible at birth, was retrospectively found to have an intrauterine dislocation when the maternal roentgenogram was reviewed. Significant secondary adaptive changes were noted at the time of operation. ( info)

4/222. A new modification of rotationplasty in a patient with proximal femoral focal deficiency Pappas type II.

    The application of rotationplasty type B III (Winkelmann) for a patient with proximal femoral focal deficiency (PFFD) Pappas type II in a 5-year-old patient is described. The advantages for prosthetic management are discussed in comparison with other surgical options. ( info)

5/222. Computer modeling of the pathomechanics of spastic hip dislocation in children.

    Spastic muscles about the hip cause subluxation, dislocation, and lead to acetabular dysplasia. Spastic hip disease occurs when the muscles about the hip exert forces that are too high or in the wrong direction or both. To determine the role of the hip forces in the progression of spastic hip disease and the effect of both muscle-lengthening and bony reconstructive surgeries, a computerized mathematical model of a spastic hip joint was created. The magnitude and direction of the forces of spastic hips undergoing surgery were analyzed preoperatively and postoperatively to determine which procedure is best suited for the treatment of spastic hip disease. The muscle-lengthening procedures included (a) the adductor longus, (b) the psoas, iliacus, gracilis, adductor brevis, and adductor longus, and (3) the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus. The bony reconstructive and muscle-lengthening procedures included (a) lengthening the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing femoral neck anteversion from 45 to 10 degrees , (b) lengthening of the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing neck-shaft angle from 165 to 135 degrees , and (c) lengthening of the psoas, iliacus, gracilis, adductor brevis, adductor longus, semimembranosus, and semitendinosus combined with changing femoral neck anteversion from 45 to 10 degrees and neck-shaft angle from 165 to 135 degrees . Results show that a child with spastic hip disease has a hip-force magnitude 3 times that of the a child with a normal hip in the normal physiologic position. Based on this mathematical model the best to normalize the magnitude of the hip-joint reaction force, the muscles to be lengthened should include the psoas, iliacus, gracilis, adductor brevis, and the adductor longus. To normalize the direction of the hip force, the extremity should be positioned in the normal physiologic position. The impact of decreasing the femoral anteversion or femoral neck-shaft angle or both had little additional effect on the direction or magnitude of hip forces. ( info)

6/222. Untreated acetabular dysplasia of the hip in the Navajo. A 34 year case series followup.

    patients born in the Many Farms District of the Navajo Indian Reservation from 1955 to 1961 were studied. Five hundred forty-eight of the 628 infants born (87%) received clinical examinations and pelvic radiographs at some time during the first 4 years of their lives. Eighteen (3.3%) of the 548 infants examined had acetabular dysplasia. Because of traditional cultural beliefs, none of these children received medical treatment. Followup evaluations and radiographs were obtained in these 18 patients during early adolescence. In 10 of the original 18 patients followup evaluations and radiographs were obtained at an average age of 35 years. None of the dysplastic hips progressed to frank dislocation. The mean center edge angle improved from 7 degrees when the patients were 1 year of age, to 29 degrees when the patients were 12 years of age, to 30 degrees when the patients were 35 years of age. Despite overall improvement of hip measurements with maturity, eight hips in five of the 10 patients who were in their fourth decade of life and who were available for examination, had radiographic evidence of residual abnormalities. The hips in patients with subluxation during infancy were less likely to be normal as adults. The results of this 34-year followup study of untreated developmental hip dysplasia showed marked radiographic improvement in all patients during childhood; however, subtle abnormalities persisted in the radiographs of 40% of the hips. ( info)

7/222. Outcome of ultrasonographic hip abnormalities in clinically stable hips.

    A retrospective review was performed of 192 newborn hips in 112 patients referred for hip evaluation. The average age at presentation was 12.7 days, with average radiographic follow-up of 15.9 months. Inclusion criteria for our study were a normal physical examination of the hip without evidence of instability and an ultrasound examination that was considered abnormal. Pavlik harness treatment was chosen at the discretion of the treating physician. At final follow-up, dysplasia was defined as greater than two standard deviations above the mean acetabular index (AI) for age. Group I consisted of 43 hips that had Pavlik treatment, and group II consisted of 149 hips that did not receive treatment. There was no difference in these two groups with respect to risk factors for dysplasia or the initial abnormalities seen on ultrasound evaluation, although patients in group I had less coverage of the femoral head during stress maneuvers. No hip in group I and two (1.3%) hips in group II were considered dysplastic (AI > 2 SD) at final radiographic follow-up (p > 0.10). There was no correlation between the severity of the ultrasound abnormality at birth and the subsequent presence of dysplasia (p > 0.10). The two hips considered dysplastic on radiograph were not being actively treated. When the hip examination of a newborn hip younger than 1 month is normal, a screening ultrasound does not appear to predict accurately subsequent hip dysplasia. In this specific setting, an initial screening ultrasound may be too sensitive and does not appear warranted. ( info)

8/222. Avascular necrosis and the Aberdeen splint in developmental dysplasia of the hip.

    Between January 1987 and December 1988 there were 7575 births in the Swansea maternity unit. Of these 823 (10.9%) were considered to be at 'high risk' for developmental dysplasia of the hip (DDH). Static ultrasound examination was performed in each case and the results classified on the basis of the method of Graf. A total of 117 type III-IV hips in 83 infants was splinted using the Aberdeen splint. Radiographs of these hips were taken at six and 12 months. Hilgenreiner's measurements of the acetabular angle were made in all cases and the development of the femoral capital epiphysis was assessed by measuring the epiphyseal area. The effect of splintage on the acetabular angle and the epiphyseal area between the normal and abnormal splinted hips was compared. Radiographs of 16 normal infants (32 normal unsplinted hips) were used as a control group. This cohort has now been followed up for a minimum of nine years. There have been no complications as a result of splintage. The failure rate was 1.7% or 0.25 per 1000 live births. No statistical difference was found when comparing the effect of splintage on the acetabular angle and epiphyseal area between normal and abnormal splinted hips and normal unsplinted hips. Our study has shown that while the Aberdeen splint had a definite but small failure rate, it was safe in that it did not produce avascular necrosis. The current conventional view that a low rate of splintage is always best is therefore brought into question if the Aberdeen splint is chosen for the management of neonatal DDH. ( info)

9/222. Proximal femoral focal deficiency (PFFD).

    A 1-year-old child with proximal femoral focal deficiency (PFFD) is presented. The clinical spectrum and associated abnormalities is described and the diagnosis and management of this entity is discussed. ( info)

10/222. Congenital transverse deficiency of the tibia and fibula: a report of two cases.

    We report two similar, but unrelated, patients with congenital bilateral partial deficiencies of the tibia and fibula associated with intact feet. In both patients, the tibia and fibula were absent on initial radiographs, while the femur and the tarsal bones were well developed and there was bilateral teratologic dislocation of the hips. Ultrasound and magnetic resonance imaging (MRI) studies suggested the presence of cartilaginous remnants of the tibia and fibula. There were multidirectional instabilities in the knees and ankles. The clinical and radiological features of these cases are distinct from those of congenital longitudinal deficiency of the tibia, in which the fibula is always preserved, and from longitudinal deficiency of the fibula, in which the tibia is present and the foot is usually involved. We suggest that the bilateral partial deficiencies of the tibia and fibula associated with the intact foot and teratologic dislocation of the hips is a single-entity disorder, possibly categorized as an intercalary transverse deficiency of the lower limb. ( info)
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