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

Young-Woo Chung; Gi-Heon Park; Hyeong-Won Park; Sung-Taek Jung

doi:10.5292/jkbjts.2011.17.1.11

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Chung, Park, Park, and Jung: The Alignment and Deformity of the Upper Extremity in Hereditary Multiple Exostoses

Original Article

J Korean Bone Joint Tumor Soc 2011;17(1):11-16.

Published online: 7 January 2011

DOI: https://doi.org/10.5292/jkbjts.2011.17.1.11

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

Young-Woo Chung, Gi-Heon Park, Hyeong-Won Park, Sung-Taek Jung

Department of Orthopedic Surgery, Chonnam National University Medical School & Hospital, Gwangju, Korea

Correspondence to: Sung-Taek Jung, M.D., Ph.D. Department of Orthopaedic Surgery, Chonnam National University Medical School & Hospital, 671 Jebong-ro, Dong-gu, Gwangju 501-757, Korea TEL: +82-62-220-6336 FAX: +82-62-225-7794 E-mail: stjung@chonnam.ac.kr

Received 21 February 201113 May 2011 Accepted 30 May 2011

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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 (≥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^o, 13.8^o, 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.

Keywords: hereditary multiple exostoses, alignment of upper extremity

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Figure 1.

A radiograph shows whole upper extremity teleradiograph. Carrying angle is measured with two drawing axes of the arm and forearm. Line A-B passes through the lateral border of the acromion to the midpoint of the lateral and medial epicondyles of the humerus. Line C-D passes through the midpoint of the lateral and medial epicondyles of the humerus to the midpoint of the distal radial and ulnar styloid process.

Figure 2.

Masada classification(Masada et al. 1989) (A) Type I: Primary exostosis formation is in the distal portion of the ulna. (B) Type IIa: In addition to ulnar shortening, the radial head is dislocated. (C) Type IIb: The radial head is dislocated without a proximal radial exostosis. (D) Type III: Primary exostosis formation is in the metaphysis of the distal radius.

Figure 3.

(A) Radial articular angle: Angle between two constructed lines - One along the articular surface of the radius and the other perpendicular to a line that bisects the head of the radius and passes through the radial edge of the distal radial epiphysis. (B) Carpal slip: Percentage of the attachment of a lunate with the radius, determined by an axial line drawn from the center of the olecranon through the ulnar edge of the radius. (C) Ulnar shortening: Distance between a line that is perpendicular to the longitudinal axis of the forearm and a line that is constructed at the distal end of the ulna.

Figure 4.

Anatomical distributions of HME checked on upper extremity.

Table 1.

Demographic Data

	A	B	Total
Number of cases	18	25	43
Age (range)∗ 15	5.5 (5.7-25.2)	9.5 (2.9-17.1)	12.2 (2.9-25.2)
Sex
Male	6	6	12
Female	4	7	11

^∗ Values are average, years.

Table 2.

Clinical Parameters

	A	B	p-value
VAS∗	1.3 (0-3.0)	3.5 (2.0-5.0)	0.003
ADL^†	7.3 (5.0-8.0)	6.6 (3.0-8.0)	0.001
Satisfactory to cosmetic outcome	13	10

^∗ VAS, visual analogue scale;

^† ADL, activity of daily living.

Table 3.

Forearm Deformity Classified as Masada Classification

Type	No∗ of cases	Radial articular angle (degree)	Carpal slip (%)	Ulnar shortening (mm)	Carrying angle (degree)
I	25	41.9 (20.7-50.5)	44.3 (10.0-100.0)	-10.9^† (3.4-22.9)	13.4 (4.6-23.6)
II	1	47.5	80	-3.4	20.2
III	4	17.9 (14.2-32.2)	27.5 (10.0-70.0)	-3.5 (1.8-10.2)	15.6 (6.8-25.4)
Total	30	42.2 (14.2-50.5)	49.7 (10.0-100.0)	-9.1 (3.4-22.9)	12.6 (4.6-25.4)

^∗ No, number;

^† Negative means shortening.

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