Journal List > J Korean Soc Spine Surg > v.8(4) > 1035983

TOOLS
Lim, Chang, Lee, Jeong, Lim, and Lee: Scoliosis Associated with Marfan Syndrome
Original Article

Journal of Korean Spine Surg 2001;8(4):482-490.

Published online: 21 February 2001

DOI: https://doi.org/10.4184/jkss.2001.8.4.482

Scoliosis Associated with Marfan Syndrome

Chang-Kyun Lim, M.D., Bong-Soon Chang, M.D., Dong-Ho Lee, M.D., Beom-Young Jeong, M.D., Soo Taek Lim, M.D., Choon-Ki Lee, M.D.

Department of Orthopedic Surgery, College of Medicine, Seoul National University, Seoul, Korea

Address reprint requests to Choon-Ki Lee, M.D. Department of Orthopedic Surgery, College of Medicine, Seoul National University #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea Tel : 82-2-760-2336, Fax : 82-2-764-2718, E-mail : choonki@plaza.snu.ac.kr

Copyright © 2001 Korean Society of Spine Surgery

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

Objectives

To evaluate characteristics and results of treatment of scoliosis associated with Marfan syndrome

Methods

The clinical data of 57 patients diagnosed as Marfan syndrome from January 1989 to June 1999 were reviewed. Thirty one patients had major curves more than 10 degrees by Cobb's method on whole spine standing film and their curve patterns and treatment methods were analyzed. Twelve cases of Marfan scoliosis who underwent posterior instrumented fusion were compared with randomly- sampled cases with idiopathic scoliosis about flexibility, correctability and correction loss of curves.

Results

Scoliosis was identified in 31 of 57 patients (54.4%) and among them 15 patients had the curve more than 40 degrees. Twelve curves were double major, 10 were thoracic, and 8 were double thoracic. Seventy- five per cent of the 57 patients had congenital heart disease and 40% had lens dislocation. Twelve of 31 cases underwent posterior fusion with pedicle screw instrumentation. Marfan scoliosis (mean flexibility; 39% in thoracic, 52% in lumbar) were significantly less flexible than idiopathic scoliosis (60% in thoracic, 93% in lumbar) (P<0.05). Mean correctability of Marfan scolisis of operation (61% in thoracic, 55% in lumbar) was poorer than that of idiopathic scoliosis (72% in thoracic, 67% in lumbar), however, this was not significant statistically (P>0.05). Mean correction loss in Marfan syndrome was 3.2% at the mean 4.1 year followup in 9 cases.

Conclusions

The prevalence of scoliosis in Marfan syndrome shows positive correlation with severity of the disease. As compared to idiopathic scoliosis, the curve associated with Marfan syndrome was larger and less flexible. A fter posterior fusion using pedicle screw instrumentation, the correctability of Marfan curve was poorer than that of idiopathic curve, which is, however, insignificant statistically. So it was considered to be a good method to treat Marfan scoliosis.

Keywords: Marfan syndrome, Scoliosis

REFERENCES

1). Birch JG, Herring JA. Spinal deformity in Marfan syndrome, J Pediatr Orthop. 7:546–552. 1987.
2). Brenton DP, Dow CJ, Hay RL and Wynne-Davies. Ho-mocystinuria and Marfan syndrome. J Bone Joint Surg. 54-B:277–283. 1972.
3). Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, Puffenberger EG, Nathakuma EJ, Cur-ristin SM, Mayers DA, Fracomano CA. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene, Nature. 352(25):337–339. 1991.
4). Donaldson DH, Brown CW. Marfan's spinal pathol -ogy, The textbook of spinal surgery. 2nd Edition. Philadelphia: Lippincott Raven Publishers;p. 299–306. 1997.
5). Joseph KN, Kane HA, Milner RS, Steg NL, Williamson MB, Bowen JR. Orthopedic aspects of the Marfan phenotype, Clin Orthop. 277:251–261. 1992.
6). Lee CS, Chung SS, Lee SG, Son HS, Kim SM. Spinal abnormalities associated with Marfan syndrome J Korean Spine Surg. 7:365–372. 2000.
7). Lonstein JE, Carlson JM. The prediction of curve progression in untreated idiopathic scolisis during growth, J Bong Joint Surg. 66-A:1061–1071. 1984.
8). Makin M, MacEwen GE, Steel HH. Marfan syndrome and its marfanoid variant, J West Pac Orthop Assoc. 21:29–36. 1984.
9). Orcutt FV, DeWald RL. The special problems which the Marfan syndrome introduces to scoliosis, J Bone Joint Surg. 56-A:1763–1768. 1974.
10). Pyeritz RE, McKusick VA. The Marfan syndrome: dignosis and management, N Engl J Med. 305:772–777. 1979.
11). Robins PR, Moe JH, Winter RB. Scoliosis in Marfan syndrome, J Bone Joint Surg. 57-A:358–368. 1975.
12). Sponseller PD, Hobbs W, Riler LH, Pyeritz RE. The thoracolumbar spine in Marfan syndrome, J Bone Joint Surg. 77-A:867–876. 1995.
13). Sponseller PD, Sethi N, Cameron DE, Pyeritz RE. Infantile scoliosis in Marfan syndrome, spine. 22:509–516. 1997.
14). Streeten EA, Murphy EA, Pyeritz RE. Pulmonary function in the Marfan syndrome, Chest. 91(3):408–412. 1987.
15). Suk SI, Ahn JW, Kim IK, Choi IH. The Incidence of Scoliosis in Korea Part III: The Incidence of Scoliosis in the Middle and High School Students. J Korean Orthop Assoc. 15:1–6. 1980.
crossref
16). Suk SI, Kim JH, Kim WJ, Kim DS, Lee SM, Kim JH. The Influence of Segmental Pedicle Screw Fixation on Distal Fusion Level in King Type 1 Adolescent Idiopathic Scoliosis. J Korean Spine Surg. 4:273–280. 1997.
17). Suk SI, Lee CK, Kim WJ, Chung YJ, Song KY, Park YB. Segmental pedicle screw fixation in the treatment of Thoracic idiopathic scoliosis. J Korean Orthop Assoc. 30:49–58. 1995.
crossref
18). Taneja DK, Manning CW. Scoliosis in Marfan syndrome and arachnodactyly. Zorab PA, editor. ed.scoiosis. London: Academic press;p. 261–281. 1977.
19). Weistein SL. The Pediatric Spine, Principles and Prac -tice. New York: Ravin Press;p. 665–683. 1994.
20). Winter RB. Thoracic lordoscoliosis in Marfan syndrome: Report of two patients with surgical correction using rods and sublaminar wires, spine. 15(3):233–235. 1990.
21). Winter RB. The surgical treatment of scoliosis in Marfan syndrome. Zobra PA, editor. ed.Scoliosis. London: Academic press;p. 238–299. 1977.

Figures and Tables%

Fig. 1-A,B.
A 22-year old girl with scoliosis associated with Marfan syndrome. She underwent an operation for heart disease and had been taking Coumadin(anticoagulant). The magnitudes of right thoracic and left lumbar curves were 78˚ and 68˚ retrospectively.
jkss-8-482f1.tif
Fig. 2-C,D.
Side-bending radiographs. The thoracic and lumbar curves were corrected to 54˚ (31%) and 27˚ (60%).
jkss-8-482f2.tif
Fig. 3-E,F.
Postoperative standing radiographs. The thoracic and lumbar curves were corrected to 29° (63%) and 28° (59%).
jkss-8-482f3.tif
Table 1.
Summary of operated cases of Scoliosis Associated with Marfan Syndrome
Case Sex/Age Curve∗ Pattern Level Fusion Pre-Op.(˚) Side-Bending(˚) Post-Op.(˚)
1 M/15 ST T5-T12 T4-L1 66 30 7
2 F/15 ST T5-T10 T4-T11 52 35 11
3 F/14 DT T1-T7-L1 T2-L2 45/44 32/21 16/12
4 F/17 DT T1-T6-T11 T1-T11 55/49 40/35 28/24
5 F/13 DT T1-T7-T12 T1-L2 40/33 21/2 4/3
6 F/11 DM1 T6-T10-L3 T10-L2 35/66 20/58 27/43
7 F/13 DM1 T5-T11-L4 T5-L5 51/61 21/20 10/10
8† F/17 DM2 T4-T12-L4 T4=L4 83/60 68/27 33/28
9† F/24 DM2 T6-L1-L5 T6-L3 96/53 68/9 21/10
10‡ F/12 DM2 T6-T12-L4 T5-L4 91/83 65/39 55/46
11‡ F/23 DM2 T5-T11-L4 T4-L4 78/68 54/27 29/28
12 F/16 DM2 T5-T12-L4 T4-T12 98/62 80/21 53/46

∗ ST = single thoracic; DT = double thoracic; DM1 = double major (King 1); DM2 = double major (King 2).

† Patients done anterior release and thoracoplasty.

‡ Heparinization

Table 2.
Summary of operated cases of Idiopathic Scoliosis (sample)
Case Sex/Age Curve Pattern Level Fusion Pre-Op.(˚) Side-Bending(˚) Post-Op.(˚)
1 F/15 ST T6-L1 T5-T12 41 14 5
2 M/15 ST T5-T12 T5-T12 59 24 24
3 F/15 DT T2-L3 T1-T6-L2 47/55 35/38 17/18
4 F/13 DT T2-T12 T1-T6-T12 23/45 10/15 5/6
5 M/20 DT T2-L3 T1-T6-L2 35/49 13/0 16/12
6 F/11 DM1 T5-L3 T7-T12-L3 45/61 15/19 18/10
7 F/13 DM1 T4-L3 T4-T10-L4 34/61 15/24 10/17
8 F/15 DM2 T4-L3 T4-T11-L3 63/62 26/18 12/20
9 F/11 DM2 T5-L3 T5-T11-L4 49/47 20/7 14/8
10 F/21 DM2 T6-L3 T6-L1-L5 44/22 8/-15 12/8
11 F/12 DM2 T4-T11 T5-T12-L4 51/41 13/-6 11/26
12 M/16 DM2 T4-T12 T6-T11-L4 67/31 45/5 15/10

∗ ST = single thoracic; DT = double thoracic; DM1 = double major (King 1) DM2 = double major (King 2).

Table 3.
Accompanied diseases of Marfan Scoliosis
Disease No. of patient (%)
Heart disease 43 (75)
Lens dislocation 23 (40)
Thoracic hypokyphosis 8 (14)
Thoracic deformity 5 (8)
Spontaneous pneumothorax 2 (3.5)
Inguinal hernia 2 (3.5)
Table 4.
Curve pattern of Marfan Scoliosis
Curve pattern No. of patient
Double major 12
Single thoracic 10
(Lt. : Rt.) (2 : 8)
Double thoracic 8
Single lumbar 1
TOOLS
Similar articles