Outcomes of intralesional osteotomy and distraction osteogenesis for limb length equalization in Ollier's disease.

Limb length discrepancy and deformities resulting from Ollier's disease are challenging to treat and have increased complications. We aimed to assess the safety of intralesional osteotomy for distraction osteogenesis and report the results of guided growth as a method of deformity correction in such conditions. We retrospectively reviewed 13 patients (eight boys and five girls), 28 segments (12 femora and 16 tibias), treated using Ilizarov circular ring fixator in one center. Nine patients had an oblique plane deformity, whereas four had a coronal plane deformity. Femoral shortening ranged from three to 11 cm. Tibial shortening ranged from 3.5 to 12 cm. Intralesional osteotomy was carried out in all patients, and guided growth (hemiepiphysiodesis) was used in seven segments (25%). The median age was 11 years (6-14 years) at surgery, with a median follow-up of 4.5 years (3-18 years). The median achieved lengthening in the femur was 7 cm (5-11 cm) and in the tibia was 5 cm (3-9 cm). The average Bone Healing Index (BHI) for the femur was 32 days/cm (28-38 days/cm), and for the tibia was 36 days/cm (28-40 days/cm). Before frame removal, the mechanical axis was restored to the knee joint center in all cases. Normal radiographic bone regeneration was evident in all cases. Hemiepiphysiodesis successfully corrected the angular deformities. Intralesional osteotomy for distraction osteogenesis is well-tolerated and reliable in Ollier's disease. Radiological normal bone was formed at the distraction site. Guided growth is also a reproducible method for deformity correction in Ollier's disease, similar to other conditions.

Are internal lengthening devices effective and associated with less complications compared to other lengthening devices? A systematic review and meta-analysis.

Internal lengthening devices became more popular than external fixators. This was attributed to their effectiveness and lower complication rates. However, this is an assumption that is not on the basis of solid scientific evidence. Therefore, this study systematically reviewed the quality and strength of evidence supporting femoral internal lengthening devices' effectiveness and their associated complications. Six electronic databases (PubMed, Cochrane, Sage, Embase, Web of Science and Scopus) were searched from inception until 20 June 2020. Further, all included articles' bibliographies were searched manually. After screening of retrieved articles, five studies were eligible for qualitative and quantitative synthesis. Studies were assessed by two independent assessors using a modified 'assessment of the methodological quality both of randomized and nonrandomized studies of health care interventions'. Further, meta-analysis was conducted to compare the procedure's effectiveness, in terms of healing index and major complications incidence. Our findings showed internal validity as the major source of bias whereas external validity was the least. For healing index, there is limited evidence that internal lengthening devices lower healing index by 0.45 months/cm (95% CI, -0.62 to -0.28; P < 0.01) compared to LON/external fixators. The incidence of major complications, that were directly related to the procedures, did not differ between internal lengthening devices and other fixators (risk ratio=0.97; 95% CI, 0.39-2.44; P < 0.95). This review provides evidence that supports lower healing index and similar complications associated with internal lengthening devices compared to other procedures of femoral lengthening. However, the evidence is very limited to draw a solid conclusion.

Hip Arthroplasty with a Preceding Contralateral Pelvic Support Osteotomy: 7- and 21-Year Follow-up: A Case Report.

CASE: This is a case of 27-year-old woman with bilateral hip dysplasia who had been treated with a pelvic support osteotomy (PSO) on 1 side and a contralateral total hip replacement (THR). Good clinical outcomes for both hips were achieved after 21 and 7 years after PSO and THR, respectively. To the best of our knowledge, these 2 different methods have not been previously reported in 1 patient. CONCLUSION: Total hip arthroplasty in presence of contralateral PSO may be offered as a viable treatment option for advanced osteoarthritis to restore hip range of motion and achieve satisfactory clinical results.

Limb lengthening history, evolution, complications and current concepts.

Limb lengthening continues to be a real challenge to both the patient and the orthopaedic surgeon. Although it is not a difficult operative problem, there is a long and exhausting postoperative commitment which can jeopardize early good results. I aim to review the history, evolution, biology, complications and current concepts of limb lengthening. Ilizarov's innovative procedure using distraction histeogenesis is the mainstay of all newly developing methods of treatment. The method of fixation is evolving rapidly from unilateral external fixator to ring fixator, computer assisted and finally lengthening intramedullary nails. The newly manufactured nails avoid many of the drawbacks of external fixation but they have their own complications. In general, the indications for limb lengthening are controversial. The indications have been extended from lower limb length inequality to upper extremity lengthening, including humeral, forearm and phalangeal lengthening. A wide range in frequency of complications is recorded in the English literature, which may reach up to 100% of cases treated. With developing experience, cosmetic lengthening has become possible using external or internal lengthening devices with an acceptable rate of problems.Level of evidence: V.

Distraction osteogenesis of fourth brachymetatarsia.

BACKGROUND: Brachymetatarsia is a rare congenital or developmental condition that results in a short metatarsal. The condition most commonly affects the fourth metatarsal of young and adolescent females. It does not usually produce a functional problem. However, it may produce a significant cosmetic problem especially in young women. The authors present their experience in gradual distraction of the fourth metatarsal to tackle this problem in adults. METHODS: That was done using a monolateral frame in 11 feet of female patients with an average age of 23 years. Evaluation depended upon the achievement of the target length, angulation, pain, and satisfaction of the patient. RESULTS: There were eight excellent and three good results after an average follow up of 2.6 years. Aside from nine cases of mild pin-tract infection, complications were few and minor. CONCLUSIONS: We recommend distraction osteogenesis as the treatment of choice for brachymetatarsia of the fourth toe.

Treatment of posttraumatic radial club hand with distraction lengthening.

The clinical and radiological results of distraction lengthening in cases with posttraumatic radial club hand were evaluated. Five patients (3 men and 2 women, with average age of 21 years) with traumatic radial shortening (3 patients had nonunited fracture of distal end radius with bone loss and 2 patients had short radius after malunited fracture of radius or growth arrest of distal radial epiphysis, the average shortening was 4.2 cm) were treated with distraction lengthening using Ilizarov fixator and hybrid fixation technique. Patients were evaluated clinically for hand function and appearance, and radiologically for union. The average follow-up was 25 months. The preplanned length was achieved in all cases with good union and no bone graft was needed, clinically there was much improvement in hand function (the range of motion of the wrist increased and the power of hand grip increased) and appearance. Pin tract infection occurred in 3 cases, broken pin in 1 case, and stiff wrist in 1 case. These complications were treated and did not affect the final results. Acquired radial club hand is difficult to treat. Treatment with distraction lengthening is a good option with excellent results and mild complications that did not affect the overall results.

Dyggve-Melchior-Clausen syndrome: clinical, genetic, and radiological study of 15 Egyptian patients from nine unrelated families.

INTRODUCTION: Dyggve-Melchior-Clausen (DMC) syndrome is a rare autosomal recessive type of skeletal dysplasia. It is characterized by the association of progressive spondyloepimetaphyseal dysplasia (SEMD), microcephaly, mental retardation (MR), and coarse facies. The radiographic appearance of generalized platyspondyly with double-humped end plates and the lace-like appearance of iliac crests are pathognomonic and distinctive of DMC syndrome. The disorder results from mutations in the DYM gene mapped in the 18q12-12.1 chromosomal region. MATERIALS AND METHODS: In this report, we studied 15 Egyptian cases with DMC syndrome from nine unrelated families. We aimed to emphasize the characteristic clinical and radiological features in order to differentiate the condition from other SEMDs and mucopolysaccharidosis (MPS). Patients were subjected to detailed history taking, three-generation family pedigree analysis, complete physical examination, anthropometric measurements, quantitative estimation, and two-dimensional electrophoresis of glycosaminoglycans in the urine and measurement of α-l-iduronidase and galactose-6-sulfatase enzyme activities to exclude Hurler and Morquio diseases (MPS type I and MPS type IVA), respectively. Other investigations were carried out whenever indicated. All patients were the offspring of consanguineous apparently normal parents. Positive family history and similarly affected sibs were noted, confirming the autosomal recessive inheritance pattern of the syndrome. Short stature, microcephaly, variable degree of MR, and coarse facies were constant features. The frequency of characteristic orthopedic and radiological findings was reported. Orthopedic surgical intervention was carried out for two patients. CONCLUSIONS: The study concluded that DMC syndrome may be more frequent in Egypt than previously thought, especially due to misdiagnosis. Characteristic facial dysmorphism, body habitus, and pathognomonic radiological signs suggest the diagnosis and differentiate it from other types of SEMDs and MPS for proper genetic counseling and management.

Ultrasound screening of the infant's hip: introduction of a new combined angle.

This study evaluates the most widely used sonographic measurements for judging an infant's hip morphology. The alpha and beta angles of Graf, the bony coverage percentage of Morin and a new combined angle were analysed. This latter angle records the direction of the whole acetabulum (cartilagenous and bony) as one unit. One-hundred sonograms were examined independently by two observers to calculate the interobserver and intraobserver limits of agreement. The new combined angle proved to be the most reproducible measurement. Graf's alpha angle was the second most reliable measurement followed by the bone coverage percentage. The relationship between the combined angle and alpha angle was statistically significant with a correlation coefficient of 0.70 (P<0.001).