Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation

Liu Dongming; Xiong Tingliang; Zhang Ziqi; Wang Xiaochen; Zhang Guowei; Yang Hua; Ji Zhisheng; Lin Hongsheng

doi:doi:10.11648/j.ijn.20200402.18

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Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation

Liu Dongming, Xiong Tingliang, Zhang Ziqi, Wang Xiaochen, Zhang Guowei, Yang Hua, Ji Zhisheng, Lin Hongsheng

Published in International Journal of Neurosurgery (Volume 4, Issue 2)

Received: 1 December 2020 Accepted: 14 December 2020 Published: 25 December 2020

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Abstract

Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. "Scoliosis correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw fixation" was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.

Published in	International Journal of Neurosurgery (Volume 4, Issue 2)
DOI	10.11648/j.ijn.20200402.18
Page(s)	63-67
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Marfan Syndrome, Scoliosis, Spinal Deformity, Spinal Correction

References

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[2]	Gray JR, Bridges AB, Faed MJ, Pringle T, Baines P, Dean J, Boxer M: Ascertainment and severity of Marfan syndrome in a Scottish population. J Med Genet 1994, 31 (1): 51-54.
[3]	Loeys B, De Backer J, Van Acker P, Wettinck K, Pals G, Nuytinck L, Coucke P, De Paepe A: Comprehensive molecular screening of the FBN1 gene favors locus homogeneity of classical Marfan syndrome. Hum Mutat 2004, 24 (2): 140-146.
[4]	Loeys B, Nuytinck L, Delvaux I, De Bie S, De Paepe A: Genotype and phenotype analysis of 171 patients referred for molecular study of the fibrillin-1 gene FBN1 because of suspected Marfan syndrome. Arch Intern Med 2001, 161 (20): 2447-2454.
[5]	Mizuguchi T, Collod-Beroud G, Akiyama T, Abifadel M, Harada N, Morisaki T, Allard D, Varret M, Claustres M, Morisaki H et al: Heterozygous TGFBR2 mutations in Marfan syndrome. Nat Genet 2004, 36 (8): 855-860.
[6]	Sakai H, Visser R, Ikegawa S, Ito E, Numabe H, Watanabe Y, Mikami H, Kondoh T, Kitoh H, Sugiyama R et al: Comprehensive genetic analysis of relevant four genes in 49 patients with Marfan syndrome or Marfan-related phenotypes. Am J Med Genet A 2006, 140 (16): 1719-1725.
[7]	Singh KK, Rommel K, Mishra A, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M: TGFBR1 and TGFBR2 mutations in patients with features of Marfan syndrome and Loeys-Dietz syndrome. Hum Mutat 2006, 27 (8): 770-777.
[8]	Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, Hilhorst-Hofstee Y, Jondeau G, Faivre L, Milewicz DM et al: The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010, 47 (7): 476-485.
[9]	Pepe G, Giusti B, Sticchi E, Abbate R, Gensini GF, Nistri S: Marfan syndrome: current perspectives. Appl Clin Genet 2016, 9: 55-65.
[10]	Dean JC: Marfan syndrome: clinical diagnosis and management. Eur J Hum Genet 2007, 15 (7): 724-733.
[11]	Jones KB, Erkula G, Sponseller PD, Dormans JP: Spine deformity correction in Marfan syndrome. Spine (Phila Pa 1976) 2002, 27 (18): 2003-2012.
[12]	Kurucan E, Bernstein DN, Ying M, Li Y, Menga EN, Sponseller PD, Mesfin A: Trends in spinal deformity surgery in Marfan syndrome. Spine J 2019, 19 (12): 1934-1940.
[13]	Guala A, Teixido-Tura G, Rodriguez-Palomares J, Ruiz-Munoz A, Dux-Santoy L, Villalva N, Granato C, Galian L, Gutierrez L, Gonzalez-Alujas T et al: Proximal aorta longitudinal strain predicts aortic root dilation rate and aortic events in Marfan syndrome. Eur Heart J 2019, 40 (25): 2047-2055.
[14]	Esfandiari H, Ansari S, Mohammad-Rabei H, Mets MB: Management Strategies of Ocular Abnormalities in Patients with Marfan Syndrome: Current Perspective. J Ophthalmic Vis Res 2019, 14 (1): 71-77.
[15]	von Kodolitsch Y, De Backer J, Schuler H, Bannas P, Behzadi C, Bernhardt AM, Hillebrand M, Fuisting B, Sheikhzadeh S, Rybczynski M et al: Perspectives on the revised Ghent criteria for the diagnosis of Marfan syndrome. Appl Clin Genet 2015, 8: 137-155.
[16]	Child AH: Non-cardiac manifestations of Marfan syndrome. Ann Cardiothorac Surg 2017, 6 (6): 599-609.
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APA Style

Liu Dongming, Xiong Tingliang, Zhang Ziqi, Wang Xiaochen, Zhang Guowei, et al. (2020). Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. International Journal of Neurosurgery, 4(2), 63-67. https://doi.org/10.11648/j.ijn.20200402.18

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ACS Style

Liu Dongming; Xiong Tingliang; Zhang Ziqi; Wang Xiaochen; Zhang Guowei, et al. Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. Int. J. Neurosurg. 2020, 4(2), 63-67. doi: 10.11648/j.ijn.20200402.18

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AMA Style

Liu Dongming, Xiong Tingliang, Zhang Ziqi, Wang Xiaochen, Zhang Guowei, et al. Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. Int J Neurosurg. 2020;4(2):63-67. doi: 10.11648/j.ijn.20200402.18

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@article{10.11648/j.ijn.20200402.18,
  author = {Liu Dongming and Xiong Tingliang and Zhang Ziqi and Wang Xiaochen and Zhang Guowei and Yang Hua and Ji Zhisheng and Lin Hongsheng},
  title = {Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation},
  journal = {International Journal of Neurosurgery},
  volume = {4},
  number = {2},
  pages = {63-67},
  doi = {10.11648/j.ijn.20200402.18},
  url = {https://doi.org/10.11648/j.ijn.20200402.18},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijn.20200402.18},
  abstract = {Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. "Scoliosis correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw fixation" was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.},
 year = {2020}
}

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TY  - JOUR
T1  - Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation
AU  - Liu Dongming
AU  - Xiong Tingliang
AU  - Zhang Ziqi
AU  - Wang Xiaochen
AU  - Zhang Guowei
AU  - Yang Hua
AU  - Ji Zhisheng
AU  - Lin Hongsheng
Y1  - 2020/12/25
PY  - 2020
N1  - https://doi.org/10.11648/j.ijn.20200402.18
DO  - 10.11648/j.ijn.20200402.18
T2  - International Journal of Neurosurgery
JF  - International Journal of Neurosurgery
JO  - International Journal of Neurosurgery
SP  - 63
EP  - 67
PB  - Science Publishing Group
SN  - 2640-1959
UR  - https://doi.org/10.11648/j.ijn.20200402.18
AB  - Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. "Scoliosis correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw fixation" was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.
VL  - 4
IS  - 2
ER  -

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Author Information

Liu Dongming

Department of Orthopedics, the First Affiliated Hospital of Jinan University, Guangzhou, China
Xiong Tingliang

The Second People's Hospital of Panyu Guangzhou, Guangzhou, China
Zhang Ziqi

School of Medicine, Jinan University, Guangzhou, China
Wang Xiaochen

School of Medicine, Jinan University, Guangzhou, China
Zhang Guowei

Department of Orthopedics, the First Affiliated Hospital of Jinan University, Guangzhou, China
Yang Hua

Department of Orthopedics, the First Affiliated Hospital of Jinan University, Guangzhou, China
Ji Zhisheng

Department of Orthopedics, the First Affiliated Hospital of Jinan University, Guangzhou, China
Lin Hongsheng

Department of Orthopedics, the First Affiliated Hospital of Jinan University, Guangzhou, China

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Plain Text BibTeX RIS

APA Style

Liu Dongming, Xiong Tingliang, Zhang Ziqi, Wang Xiaochen, Zhang Guowei, et al. (2020). Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. International Journal of Neurosurgery, 4(2), 63-67. https://doi.org/10.11648/j.ijn.20200402.18

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ACS Style

Liu Dongming; Xiong Tingliang; Zhang Ziqi; Wang Xiaochen; Zhang Guowei, et al. Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. Int. J. Neurosurg. 2020, 4(2), 63-67. doi: 10.11648/j.ijn.20200402.18

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AMA Style

Liu Dongming, Xiong Tingliang, Zhang Ziqi, Wang Xiaochen, Zhang Guowei, et al. Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation. Int J Neurosurg. 2020;4(2):63-67. doi: 10.11648/j.ijn.20200402.18

Copy | Download

@article{10.11648/j.ijn.20200402.18,
  author = {Liu Dongming and Xiong Tingliang and Zhang Ziqi and Wang Xiaochen and Zhang Guowei and Yang Hua and Ji Zhisheng and Lin Hongsheng},
  title = {Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation},
  journal = {International Journal of Neurosurgery},
  volume = {4},
  number = {2},
  pages = {63-67},
  doi = {10.11648/j.ijn.20200402.18},
  url = {https://doi.org/10.11648/j.ijn.20200402.18},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijn.20200402.18},
  abstract = {Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. "Scoliosis correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw fixation" was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.},
 year = {2020}
}

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TY  - JOUR
T1  - Scoliosis Correction Surgery for a Patient with Marfan Syndrome with Severe Scoliosis and Restricted Lung Ventilation
AU  - Liu Dongming
AU  - Xiong Tingliang
AU  - Zhang Ziqi
AU  - Wang Xiaochen
AU  - Zhang Guowei
AU  - Yang Hua
AU  - Ji Zhisheng
AU  - Lin Hongsheng
Y1  - 2020/12/25
PY  - 2020
N1  - https://doi.org/10.11648/j.ijn.20200402.18
DO  - 10.11648/j.ijn.20200402.18
T2  - International Journal of Neurosurgery
JF  - International Journal of Neurosurgery
JO  - International Journal of Neurosurgery
SP  - 63
EP  - 67
PB  - Science Publishing Group
SN  - 2640-1959
UR  - https://doi.org/10.11648/j.ijn.20200402.18
AB  - Objective: Marfan syndrome is a multi-system disease caused by genetic mutations that can cause damage to multiple systems in the body. With the development of the disease, the scoliosis of patients with Marfan syndrome will be further aggravated, which will eventually seriously affect the patient's cardiopulmonary function and lead to a serious decline in the patient's quality of life. The aim is to summarize the experience of spinal orthopedic surgery in a patient with Marfan syndrome and severe scoliosis with limited pulmonary ventilation. Method: In November 2019, a 13-year-old male with Marfan syndrome with severe scoliosis and restricted lung ventilation was admitted. The full-length positive and lateral X-rays of the spine before the operation showed that the spine was S-shaped scoliosis, double thoracic curve (thoracic curve + lumbar curve), upper vertebrae T4, lower vertebrae T11, thoracic T4-T11 structural curve, thoracic Cobb angle of bend is 42.4°, the lumbar bend is compensatory, Cobb angle of lumbar bend is 33.2° The lung function indicates that the forced end-expiratory volume/forced vital capacity (FEV1/FVC) in the first second is 70%. "Scoliosis correction + interlaminar bone graft fusion + posterior T4-T11 pedicle screw fixation" was performed on the patient under general anesthesia with tracheal intubation. Result: The full-length positive and lateral X-rays of the spine at 1 week, 1 month, 3 months, and 1 year after surgery showed that the internal fixation position was good, and the Cobb angle remained basically unchanged. There was basically no change in the Cobb angle of the thoracic curve (10.2°, 10.8°, 10.9°, 10.5°, respectively) and the Cobb angle of the lumbar curve (13.1°, 13.6°, 13.8°, 13.9, respectively) at each follow-up time point after surgery. The lung function at 1 year after surgery suggested that FEV1/FVC was 80%. No sensorimotor abnormalities and other complications occurred during the follow-up. Conclusion: Scoliosis correction surgery is feasible and safe for patients with Marfan syndrome combined with severe scoliosis deformity and restricted lung ventilation. It can improve the patient's appearance, at the same time improve the patient's cardiopulmonary function, and solve the problem of the continued increase in the number of cobb angles of scoliosis. Surgery improves the quality of life of patients as a whole.
VL  - 4
IS  - 2
ER  -

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