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.
The Taylor spatial frame uses the slow correction principles of the Ilizarov system but adds a six-axis deformity analysis incorporated within a computer program. To evaluate its use in our community, we used it in lengthening and deformity correction of the lower limbs to treat 22 cases from 1999 to 2001. There were 14 females and eight males (average age 16.5 years). Our target was lengthening in eight cases, correction of deformities in eight and both in six. The results were excellent in 18 cases, good in two, and fair in two. Despite the cost, patient profile and a steep learning curve, our results were encouraging but less favorable than with the Ilizarov external fixator.
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 methodsIn 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 a-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.
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