Correction of lumbosacral hyperlordosis in achondroplasia
Department of Orthopaedic Surgery, Yonsei University, Sŏul, Seoul, South KoreaClinical Orthopaedics and Related Research (Impact Factor: 2.77). 10/2003; DOI: 10.1097/01.blo.0000081936.75404.a4
Anterior bulging of the abdomen and posterior protrusion of the buttocks are externally visible deformities reflecting lumbosacral hyperlordosis. Imbalance in pelvic femoral muscles may account for this posture. Despite the clinical significance of hyperlordosis, its surgical treatment has not been well-described. In the current preliminary study, the authors compare two techniques used at the authors' institution for lower limb lengthening, one of which affects the correction of lumbosacral hyperlordosis. Ten patients had bilateral lower extremity lengthening procedures. Seven patients had bilateral tibial lengthening and three patients had combined femoral and tibial lengthening. Ring external fixators were used. Correction of hyperlordosis was assessed by comparing four radiographs with measurements in the sagittal plane obtained preoperatively with those at the latest followup. In the femoral lengthening group, the average preoperative lumbar lordosis angle was 18 degrees, the lumbosacral joint angle was 12 degrees, the sacral inclination angle was 58.3 degrees, and the sacrohorizontal angle was 31 degrees. The mean changes at the latest followup were: lumbar lordosis angle (+1 degree), lumbosacral joint angle (+0.3 degrees), sacral inclination angle (-19 degrees), and sacrohorizontal angle (-15 degrees). In the tibia lengthening group, all parameters were relatively unaltered at the last followup compared with their preoperative levels. Tibial lengthening had no effect on lumbosacral hyperlordosis. However, femoral lengthening resulted in an improved apparent lumbosacral hyperlordosis, although the lumbar lordosis angle was not changed significantly. The change in sacrum tilting provides a likely explanation for the improvement in cosmetic hyperlordosis observed in patients who have had femoral lengthening.
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ABSTRACT: Purpose of review: Significant advances have been made in understanding the genetics and molecular mechanisms involved in skeletal dysplasias. Short stature and limb deformities continue to be major skeletal manifestations in patients with skeletal dysplasias. These are often the cause of significant disability in these patients. This article reviews the recent advances in the correction of angular limb deformities and limb lengthening in this group of patients. Recent developments: Surgical techniques continue to evolve and technology continues to improve in the field of deformity correction and limb lengthening. Basic science research is focused on understanding the factors involved in maturation of the regenerate bone formed during distraction osteogenesis. Hydroxyapatite-coated external fixator screws have led to decreased loosening of the screws and increased stability of external fixators. Improvements in techniques of internal fixation have led to the use of intramedullary nails for distraction osteogenesis. Early recognition of complications, especially nerve compression syndromes, and their aggressive treatment have resulted in significant reduction in the morbidity associated with these procedures. With advances in total joint arthroplasty and the availability of custom components as well as modular prostheses, the results of joint replacement in these patients continue to improve. Summary: Our knowledge and understanding of the processes involved in the pathogenesis of skeletal dysplasia continue to improve, as does the technology for intramedullary devices for lengthening. Hydroxyapatite coating of Schanz screws and earlier identification of potentially devastating complications such as nerve injury have resulted in reduced morbidity during limb lengthening and deformity corrections in patients with skeletal dysplasia who are at high risk for complications.Current Opinion in Orthopaedics 11/2004; 15(6):399-403. DOI:10.1097/01.bco.0000146123.54631.af
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ABSTRACT: Die häufigsten Formen des Kleinwuchses sind die Achondroplasie und Hypochondroplasie. Beiden gemein ist die stark verminderte Körpergröße. Zur Diagnose führt bei der Achondroplasie die klinische Untersuchung. Die Hypochondroplasie scheint manchmal unentdeckt zu bleiben und wird häufiger auch erst im Vorschulalter diagnostiziert. Betroffene mit einer Achondroplasie haben mit zahlreichen orthopädischen Problemen zu rechnen, die Auswirkungen auf das tägliche Leben haben. Hierzu sind in erster Linie Veränderungen im okzipitozervikalen Übergang oder eine Spinalkanalstenose zu rechnen. Hinzukommen noch Achsendeformitäten und teils ausgeprägte Bandlaxitäten, die die körperliche Leistungsfähigkeit beeinträchtigen und erhebliche Probleme bei der Bewältigung des Alltags mit sich bringen können. Wichtiger als mögliche und inzwischen auch standardisierte Verlängerungsoperationen sind sicherlich die Korrekturen von Fehlstellungen, wenn auch immer noch ein Teil der Betroffenen sich mit dem Gedanken einer Verlängerung trägt. Zur Analyse von Beinachsendeformitäten ist die CORA-Methode hilfreich und sollte unbedingt eingesetzt werden. Achondroplasia and hypochondroplasia are the most common forms of short stature. The leading sign is the diminished body height. Most cases of achondroplasia can be detected by clinical examination. Hypochondroplasia is more often not diagnosed until preschool age. In achondroplasia many different orthopaedic problems can arise, which influence the ADL. Mainly pathologic alterations of the occipitocervical region or a stenosis of the medullary cavity should be expected. In the limbs a bow-leg deformity and hyperlaxity of the ligaments often can be seen, which in some cases restrict walking capacity. Even if many people of short stature contemplate a lengthening procedure, the correction of deformities is much more important. The CORA method should be used in planning any osteotomy.Der Orthopäde 12/2007; 37(1):40-48. DOI:10.1007/s00132-007-1182-4 · 0.36 Impact Factor
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ABSTRACT: Achondroplasia and hypochondroplasia are the most common forms of short stature. The leading sign is the diminished body height. Most cases of achondroplasia can be detected by clinical examination. Hypochondroplasia is more often not diagnosed until preschool age.In achondroplasia many different orthopaedic problems can arise, which influence the ADL. Mainly pathologic alterations of the occipitocervical region or a stenosis of the medullary cavity should be expected. In the limbs a bow-leg deformity and hyperlaxity of the ligaments often can be seen, which in some cases restrict walking capacity. Even if many people of short stature contemplate a lengthening procedure, the correction of deformities is much more important. The CORA method should be used in planning any osteotomy.Der Orthopäde 02/2008; 37(1):40-8. · 0.36 Impact Factor
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