유전성 다발성 외골종 환자에서 상지 변형 = The Alignment and Deformity of the Upper Extremity in Hereditary Multiple Exostoses

국문 초록 (Abstract)

목적: 유전성 다발성 외골종 환자에서 상지에 발생한 외골종 병변의 분포와 이와 관련하여 상지의 축성 배열의 변화에 대해 조사하였다. 대상 및 방법: 2001년부터 2009년까지 유전성 다발성 외골종으로 진단받은 38명, 76예를 대상으로 하였다. 외골종 침범 수에 따라 Group A (2부위 이하), Group B (3부위 이상)로 분류하였으며, 각 그룹간 운반각 및 VAS (Visual Analogue Scale), 일상 활동의 제한 정도, 외관상 만족도를 비교 평가하였다. 결과: 외골종으로 진단받은 38명 환자 중 상지에 발생한 외골종은 23명, 43예에서 관찰되었다. 발생 부위로는 상완골 근위부 33예(30%), 척골 원위부 31예(28.2%), 요골 원위부 24예(21.8%) 이었다. Group A, B 각각 운반각은 $10.7^{\circ}$, $13.8^{\circ}$, VAS는 1.3점, 3.5점, 일상생활 능력의 제한은 8점 중 7.3점, 6.6점을 보였으며 외관상 만족한 경우는 13예, 10예였다. 결론: 유전성 다발성 외골종 환자 중 상지 변형은 65%에서 동반되었다. 침범 부위 수가 증가할 수록 운반각 및 VAS는 증가하였고, 일상생활 능력과 만족도는 감소하였다.

다국어 초록 (Multilingual 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 (${\geq}$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 $10.7^{\circ}$, $13.8^{\circ}$, 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.

참고문헌 (Reference)

1 Shin E, "Treatment of multiple hereditary osteochondromas of the forearm in children: a study of surgical procedures" 88 : 255-260, 2006

2 Boyer A, "Trait des malaidies chirurgicals et des operation quileur conviennent" 3 : 594-, 1918

3 Schmale G, "The natural history of hereditary multiple exostoses" 76 : 986-992, 1994

4 Oppenheim W, "Supracondylar humeral osteotomy for traumatic childhood cubitus varus deformity" 188 : 34-39, 1984

5 Masada K, "Operations for forearm deformity caused by multiple osteochondromatosis" 71 : 24-29, 1989

6 Black B, "Multiple hereditary exostoses. An epidemiologic study of an isolated community in Manitoba" 287 : 212-217, 1993

7 Beals R, "Metachondromatosis" 169 : 167-170, 1982

8 Fogel G, "Management of deformities of the forearm in multiple hereditary osteochondromatosis" 66 : 670-680, 1980

9 Fogel G, "Management of deformities of the forearm in multiple hereditary osteochondromas" 66 : 670-680, 1984

10 Akita S, "Long-term results of surgery for forearm deformities in patients with multiple cartilaginous exostoses" 89 : 1993-1999, 2007

11 Nawata K, "Knee deformities in multiple hereditary exostoses: a longitudinal radiographic study" 313 : 194-199, 1995

12 Solomon L, "Hereditary multiple exostosis" 43 : 292-304, 1963

13 Hennekam R, "Hereditary multiple exostoses" 28 : 262-266, 1991

14 Jaffe H, "Hereditary multiple exostoses" 36 : 335-357, 1943

15 Alvarez C, "Evaluation of the anatomic burden of patients with hereditary multiple exostoses" 462 : 73-79, 2007

16 Krooth R, "Diaphysealaclasis (multiple exostosis) on Guam" 13 : 340-347, 1961

17 Peterson H, "Deformities and problems of the forearm in chil- dren with multiple hereditary osteochondromata" 14 : 92-100, 1994

18 Matsubara H, "Correction and lengthening for deformities of the forearm in multiple cartilaginous exostoses" 11 : 459-466, 2006

19 Burrows H, "An operation for the correction of Madelung’s deformity and similar conditions" 30 : 565-572, 1937