摘要:

Osteogenesis Imperfecta (OI) is characterized by bone fragility. At least seven discrete types have been described ranging from mild disease to a lethal form. In a large number of cases, mutations in one of the two genes encoding type I collagen have been found. In forms recently described (types V, VI, VII), such mutations have been excluded. In two other forms, (Bruck, and osteoporosis – pseudoglioma syndromes) defects in other proteins have been characterized. In OI, bone fragility stems from: decreased bone mass, disturbed organization of bone tissue, and altered bone geometry (size and shape). Histologic studies have shown that increased bone turnover is the rule in OI bone. This justifies using bisphosphonates in order to reduce osteoclast mediated bone resorption. Initial results are encouraging. Cyclical intravenous pamidronate administration reduces bone pain and fracture incidence, and increases bone density and level of ambulation, with minimal side effects. Effects on bone include increase in size of vertebral bodies and thickening of cortical bone. These results allow for more efficacious corrective surgery using intramedullary rodding of the long bones and paravertegral instrumentation. Specific occupational and physiotherapy programs are integral parts of the treatment protocol. This multidisciplinary approach will prevail until strategies aiming at the correction of the basic defect(s) will have come to fruition.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

Osteogenesis imperfecta is one of the most common groups of inherited disorders of connective tissue. Hyperplastic callus formation in patients with osteogenesis imperfecta after fracture or surgery is a rare occurrence that has often been misdiagnosed as osteosarcoma. Previous series reported that hyperplastic callus formation is more often present in osteogenesis imperfecta male patients, with white sclerae, and a negative family history of the disorder. This is the first time that this complication has been presented in three female siblings, with a positive family history of osteogenesis imperfecta type IV. An association between osteogenesis imperfecta type IV and hyperplastic callus formation is unclear. This association might, however, be a separate, specific subtype of osteogenesis imperfecta, with an unknown inheritance pattern.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

We conducted a retrospective, clinical and radiological study of fixation with a single screw in slipped upper femoral epiphysis. Forty children (53 hips) who had a slipped upper femoral epiphysis were managed by in-situ pinning with a single cannulated screw. The average age of these patients was 12.7 years and their average weight was above the 50th centile at the time of diagnosis. There were three acute slips, eight acute-on-chronic slips and 35 chronic slips. They were followed for an average of 2 years. Fifty-eight percent of patients had an excellent clinical result, 36% a good result, 4% a fair result and 2% a poor result. The complication rate was 5.3%. Our experience with single cannulated screw fixation of slipped upper femoral epiphysis is encouraging. No complications resulted from the choice of the fixation device.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The purpose of this prospective study was to determine if a connecting bar prevented mechanical failure of hip spicas. Eighty-two hip spicas were applied to 70 patients between April 2000 and June 2001 following open or closed reduction for developmental dysplasia of the hip. Bilateral hip surgery was undertaken in 12 patients. Thirty-six spicas were applied with a bar whilst 46 were without. The technique of application was similar in all patients. Overall nine (11%) hip spicas failed (mechanically) prematurely and had to be replaced in hospital under general anaesthesia. All nine were inpatients whose spica did not have a connecting bar (P<0.005). The rate of spica revision increased with the age of the patient. All parents of failed spica cases (nine cases) preferred the new spica with a bar to the previous one without, because they agreed that the bar made the spica more secure. The bar did not hamper toileting or handling.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The development of acetabular dysplasia in the growing child has been postulated to be due to abnormal forces on the acetabular rim, such as when the hip is in an adducted position. In this case report we document the development of acetabular dysplasia, as measured radiographically, in a child with a perinatal contralateral high above-knee amputation.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The authors analysed the status of screening and prevention of developmental dysplasia of the hip in the Slovak Republic on the basis of the European Pediatric Orthopaedic Society questionnaire. The questionnaire was sent to 31 heads of orthopaedic departments, with 23 (74%) responses. According to the responses received, in the Slovak Republic a neonatologist or orthopaedic surgeon, depending on availability, makes the first clinical examination of the hips up to the fifth day after delivery. Every instability, relative shortening of one of the lower limbs, or ‘high-risk’ baby is referred immediately to the orthopaedic surgeon. Sonographic examination up to 7 days after delivery is performed in six departments. In another 11 departments, two or more sonographic examinations are performed on each baby. Authors support the simple static ultrasound as an effective screening test for developmental dysplasia of the hip, that should be applied to the whole population and not simply to the at risk group.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The inherent ability of pediatric metaphyseal radius fractures to heal and remodel made us question the need for immediate anatomic reduction under conscious sedation. We believe that isolated closed distal radius fractures with 15° of angulation and 1 cm of shortening will heal well and remodel completely without clinical or functional sequelae. Time and expense can be decreased by splinting and follow-up without the need for immediate anatomic reduction in the emergency room. In order to answer this question, we retrospectively evaluated 34 pediatric metaphyseal wrist fractures that lost position after attempted reduction and healed in their angulated or shortened position. We looked at the time to healing, time to remodeling and any residual clinical or functional deficits. We then did a comparison cost analysis with time matched patients who had complete but minimally displaced fractures of the distal radius that were treated by immediate splinting with orthopaedic follow-up. Our results showed that skeletally immature patients with open physes, isolated injuries, dorsovolar and radioulnar angulations less than 15° and less than 1 cm of shortening will heal and be out of cast within an average of 6 weeks and completely remodel within an average of 7.5 months. The average time in the emergency room was 2 h less with no reduction. The cost of the emergency room visit with attempted reduction was 50% more than splinting with early referral (US$536 versus US$270). None of our patients had significant clinical deformities or residual functional deficits.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

BackgroundMany surgical procedures are used for the correction of cubitus varus deformity in children. Even if the primary indication is cosmetic, but many poor results have been described. The technique of a supracondylar medial opening wedge osteotomy with external fixation is presented and preliminary results in four cases are reported.TechniqueAn antero-medial approach is chosen with exposition and anterior transposition of the ulnar nerve. Under image intensifier control, insertion of four Schanz screws is performed from medial to lateral. In between, an incomplete medial osteotomy is performed obliquely (in the antero-posterior as well as in the medio-lateral plane), leaving a small part of the radial humeral cortex intact. The osteotomy is opened until varus and, if present, hyperextension deformation are corrected. Then, the fixator system is applied.ResultsAll osteotomies healed primarily. Mean valgus correction of 21.75° was achieved; in only one patient additional flexion modification of 17° was necessary. At a follow-up of at least 2 years clinically three out of four presented symmetric elbow position, one slight residual varus deformity was noted in spite of 20° of valgization due to important valgus position of the contralateral side. No lateral bulging of the corrected elbow was observed.ConclusionThe described technique is an alternative to other procedures, with special regard to the cosmetic outcome as well as to the control of correction for valgus and flexion.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The Taylor spatial frame is a new external ring fixation system developed for correction of axial, sagittal, planar and rotational deformities of the extremities. Between August 1999 and October 2000 five children were treated by this system. Four had shortened and deformed femurs after fracture, osteomyelitis or a congenital short femur. One patient had a pseudoarthrosis of the tibia. Mean corrections were leg lengthening 5.9 cm (1.7–7.2), valgus correction 10.6° (0–19), lateral translation 12.6 mm (0–33) and rotation 10°. The mean healing index was 48.4 days/cm. In all patients the bones fused without major complications. We recommend the Taylor spatial frame for corrections of complex limb deformities.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

We report a case of physeal arrest secondary to extravasation of an intravenous infusion in a neonate. The ankle was reconstructed according to the Gruca operation followed by callus distraction lengthening.

ISSN:0271-6798    

出版商:OVID    

年代:2003

数据来源: OVID