J Korean Bone Joint Tumor Soc.  2011 Jun;17(1):11-16. 10.5292/jkbjts.2011.17.1.11.

The Alignment and Deformity of the Upper Extremity in Hereditary Multiple Exostoses

• Chung YW
• Park GH
• Park HW
• Jung ST

• KMID: 2137038
• DOI: http://doi.org/10.5292/jkbjts.2011.17.1.11

Abstract

PURPOSE
This study was aimed to analyze the incidence and the anatomical distributions of HME (Hereditary Multiple Exostoses) on upper limbs and its related change in alignment of the upper limbs in HME patients.
MATERIALS AND METHODS
Thirty eight patients who had been diagnosed HME between 2001 and 2009, were categorized into two groups; (1) group A (1-2 involvements); (2) group B (> or =3 involvements). We checked the carrying angle, VAS (Visual Analogue Scale), limitations in daily activities, cosmetic satisfaction according to the number of exostoses invasion.
RESULTS
Among the 38 patients, 23 patients (43 cases) had exostoses in the upper limbs. The locations of exostoses in the upper limbs were proximal humerus in 33 cases (30%), distal ulna in 31 cases (28.2%), and distal radius in 24 cases (21.8%). The carrying angle of group A and B was 10.7degrees, 13.8degrees, VAS was 1.3, 3.5, and the limitations in daily activities was 7.3, 6.6 of 8 points. The cosmetic satisfactory cases were 13 and 10 cases, respectively.
CONCLUSION
The deformity in upper limbs was observed in 65% of the HME patients. As the number of invasion increases, carrying angle and VAS were increased but limitations in daily activities and cosmetic satisfaction were decreased.

Keyword

hereditary multiple exostoses; alignment of upper extremity

MeSH Terms

Congenital Abnormalities
Cosmetics
Exostoses
Exostoses, Multiple Hereditary
Humans
Humerus
Incidence
Lifting
Radius
Ulna
Upper Extremity
Cosmetics

Reference

1. Boyer A. Trait des malaidies chirurgicals et des operation quileur conviennent. Paris: ve Migneret. 1814; 3:594.

2. Beals R. Metachondromatosis. Clin Orthop Relat Res. 1982; 169:167–70.

3. Jaffe H. Hereditary multiple exostoses. Arch Pathol. 1943; 36:335–57.

4. Alvarez C, De Vera M, Heslip T, Casey B. Evaluation of the anatomic burden of patients with hereditary multiple exostoses. Clin Orthop Relat Res. 2007; 462:73–9.

5. Burrows H. An operation for the correction of Madelung's deformity and similar conditions. Proc Roy Soc Med. 1937; 30:565–72.

6. Shin E, Jones N, Lawrence J. Treatment of multiple hereditary osteochondromas of the forearm in children: a study of surgical procedures. J Bone Joint Surg Br. 2006; 88:255–60.

7. Hennekam R. Hereditary multiple exostoses. J Med Genet. 1991; 28:262–6.

8. Krooth R, Macklin M, Hilbish T. Diaphysealaclasis (multiple exostosis) on Guam. Am J Hum Genet. 1961; 13:340–7.

9. Peterson H. Deformities and problems of the forearm in children with multiple hereditary osteochondromata. J Peiatr Orthop. 1994; 14:92–100.

10. Solomon L. Hereditary multiple exostosis. J Bone Joint Surg Br. 1963; 43:292–304.

11. Black B, Dooley J, Pyper A, Reed M. Multiple hereditary exostoses. An epidemiologic study of an isolated community in Manitoba. Clin Orthop Relat Res. 1993; 287:212–7.

12. Matsubara H, Tsuchiya H, Sakurakichi K, Yamashiro T, Watanabe K, Tomita K. Correction and lengthening for deformities of the forearm in multiple cartilaginous exostoses. J Orthop Sci. 2006; 11:459–66.

13. Schmale G, Conrad E, Raskind W. The natural history of hereditary multiple exostoses. J Bone Joint Surg Am. 1994; 76:986–92.

14. Oppenheim W, Clader T, Smith C, Bayer M. Supracondylar humeral osteotomy for traumatic childhood cubitus varus deformity. Clin Orthop Relat Res. 1984; 188:34–9.

15. Masada K, Tsuyuguchi Y, Kawai H, Kawabata H, Noguchi K, Ono K. Operations for forearm deformity caused by multiple osteochondromatosis. J Bone Joint Surg Br. 1989; 71:24–9.

16. Nawata K, Teshima R, Minamizaki T, Yamamoto K. Knee deformities in multiple hereditary exostoses: a longitudinal radiographic study. Clin Orthop Relat Res. 1995; 313:194–9.

17. Fogel G, McElfresh E, Peterson H, Wocklund P. Management of deformities of the forearm in multiple hereditary osteochondromatosis. J Bone Joint Surg Am. 1980; 66:670–80.

18. Akita S, Murase T, Yonenobu K, Shimada K, Masada K, Yoshikawa H. Long-term results of surgery for forearm deformities in patients with multiple cartilaginous exostoses. J Bone Joint Surg Am. 2007; 89:1993–9.

19. Fogel G, McElfresh E, Peterson H, Wicklund P. Management of deformities of the forearm in multiple hereditary osteochondromas. J Bone Joint Surg Am. 1984; 66:670–80.