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Liang H, Shao Z, Shi Y, Wang X. Comparison of surgical and non-surgical treatment for scoliosis in Duchenne muscular dystrophy: a systematic review and meta-analysis. Arch Orthop Trauma Surg 2025; 145:219. [PMID: 40167615 DOI: 10.1007/s00402-025-05811-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2023] [Accepted: 03/05/2025] [Indexed: 04/02/2025]
Abstract
INTRODUCTION Duchenne muscular dystrophy (DMD) is a neuromuscular disease caused by dystrophin gene mutations, which not only causes severe physical and psychological damage to the patient but also imposes a considerable economic burden on society. However, whether to aggressively surgical treatment for progressive scoliosis of DMD remains a conundrum. Therefore, we aimed to systematically evaluate the efficacy and prognosis of surgical treatment for DMD-associated scoliosis. MATERIALS AND METHODS PubMed, Embase, The Cochrane Library, China National Knowledge Infrastructure database, Wanfang database, China Science and Technology Journal Database, and China Biomedical Literature Database were searched for literature from the time of database creation until November 2022. The inclusion parameters were as follows: number of survivors, frequency of complications, preoperative, postoperative, and final follow-up Cobb's angles. Data were analyzed using RevMan 5.4.1 and Stata 12.0. RESULTS Six studies were analyzed. Survival rates were higher [OR = 3.83, 95% CI (1.91, 7.66), p = 0.0001] and Cobb's angle improved [SMD, 1.79, 95% CI (150, 2.07), p < 0.00001] in the surgical group than in the non-surgical group. The non-surgical group had a lower complication rate than the surgical group did [OR = 8.75, 95% CI (1.02,75.08), p = 0.048]. CONCLUSIONS Notably, scoliosis-correction surgery improves patient survival. Besides, scoliosis-correction surgery provides patients with more stable Cobb angle. As expected, non-surgical treatment has the advantage of reducing the occurrence of complications.
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Affiliation(s)
- Haibo Liang
- The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, 325027, China
| | - Zhenxuan Shao
- The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, 325027, China
- The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, 310009, China
| | - Yifeng Shi
- The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, 325027, China
| | - Xiangyang Wang
- The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, 325027, China.
- The Second Affiliated Hospital and Yuying Children's hospital of Wenzhou Medical University, 109 West Xueyuan Road, Wenzhou, 325027, China.
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Weintraub M, Gupta A, Khokhar A, Vives M, Kaushal N. Current Concepts in the Orthopaedic Management of Duchenne Muscular Dystrophy. J Am Acad Orthop Surg Glob Res Rev 2024; 8:01979360-202407000-00011. [PMID: 38996213 PMCID: PMC11239163 DOI: 10.5435/jaaosglobal-d-24-00099] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2024] [Revised: 04/21/2024] [Accepted: 05/22/2024] [Indexed: 07/14/2024]
Abstract
Duchenne muscular dystrophy (DMD), a genetic condition marked by progressive muscle degeneration, presents notable orthopaedic challenges, especially scoliosis, which deteriorates patients' quality of life by affecting sitting balance and complicating cardiac and respiratory functions. Current orthopaedic management strategies emphasize early intervention with corticosteroids to delay disease progression and the use of surgical spinal fusion to address severe scoliosis, aiming to enhance sitting balance, alleviate discomfort, and potentially extend patient lifespan. Despite advancements, optimal management requires ongoing research to refine therapeutic approaches, ensuring improved outcomes for patients with DMD. This review synthesizes recent findings on surgical and nonsurgical interventions, underscoring the importance of a multidisciplinary approach tailored to the dynamic needs of patients with DMD.
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Affiliation(s)
- Matthew Weintraub
- From the Department of Orthopaedic Surgery, Rutgers New Jersey Medical School, Newark, NJ
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Maeda Y, Watanabe K, Kawakami N, Ohara T, Nakamura M, Matsumoto M. Surgical treatment for lumbar hyperlordosis associated with facioscapulohumeral muscular dystrophy: A case series. J Orthop Sci 2024; 29:428-433. [PMID: 35999089 DOI: 10.1016/j.jos.2022.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Revised: 06/25/2022] [Accepted: 07/09/2022] [Indexed: 11/17/2022]
Affiliation(s)
- Yoshihiro Maeda
- Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Kota Watanabe
- Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan.
| | - Noriaki Kawakami
- Department of Orthopedics & Spine Surgery, Meijo Hospital, Japan; Department of Othopedic Surgery, Ichinomiya Nishi Hospital, Japan
| | - Tetsuya Ohara
- Department of Orthopedics & Spine Surgery, Meijo Hospital, Japan
| | - Masaya Nakamura
- Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Morio Matsumoto
- Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan
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Morales Ciancio RA, Gagliardi MJ, Tucker S, Ember T, Harris M, Broomfield E, Craven CL. Distal fusion in Duchenne scoliosis: the relevance of preoperative pelvic obliquity. A case series study. Spine Deform 2023; 11:1261-1270. [PMID: 37280469 DOI: 10.1007/s43390-023-00689-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2022] [Accepted: 04/08/2023] [Indexed: 06/08/2023]
Abstract
PURPOSE The aim of this study was to determine clinical and radiographic outcomes for Duchenne Muscular Dystrophy (DMD) patients who underwent posterior spinal fusion from T2/3 to L5 (without pelvic fixation), at this single centre. METHODS From January 2012 to January 2020, 29 consecutive DMD scoliosis patients underwent posterior spinal fusion using pedicle screws from T2/3 to L5 in a single center with a minimum of 3 years follow-up (FU). Radiologic measurements and chart review were performed. RESULTS Twenty nine patients aged 14 ± 1.5 years were included. No patient was lost to FU. All patients had significant correction in Cobb angle, pelvic obliquity (PO) and lumbar lordosis (LL), without loss of correction at last FU. The mean values for preoperative, immediate postoperative and last FU were CA 62o, 15o and 17o, PO: 21o, 8o and 9o; and LL 10o, -41o and -41o respectively. Correction in CA was independent of any variable analysed including implant density, rod diameter, traction, or bone density. Regarding PO, it was inversely related to age and independent of all other variables. Factors associated with postoperative complications were age and respiratory function. CONCLUSIONS It appears from our results that pelvic fixation might not always be required in DMD scoliosis surgery, when using pedicle screws with lowest instrumented vertebra at L5. However, larger preoperative PO values can be related with residual PO. It seems that probably related to the underlying condition, early surgery may decrease incidence of complications. LEVEL OF EVIDENCE IV.
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Affiliation(s)
| | | | - Stewart Tucker
- Orthopaedic and Spine Department, Great Ormond Street Hospital, London, WC1N 3JH, UK
| | - Thomas Ember
- Orthopaedic and Spine Department, Great Ormond Street Hospital, London, WC1N 3JH, UK
| | - Mark Harris
- Orthopaedic and Spine Department, Great Ormond Street Hospital, London, WC1N 3JH, UK
| | - Edel Broomfield
- Orthopaedic and Spine Department, Great Ormond Street Hospital, London, WC1N 3JH, UK
| | - Claudia L Craven
- Orthopaedic and Spine Department, Great Ormond Street Hospital, London, WC1N 3JH, UK
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Roberts S, Arshad A, Tsirikos AI. Surgical and long-term functional outcomes of patients with Duchenne muscular dystrophy following spinal deformity correction. World J Orthop 2023; 14:411-426. [PMID: 37377990 PMCID: PMC10292064 DOI: 10.5312/wjo.v14.i6.411] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2022] [Revised: 03/29/2023] [Accepted: 05/15/2023] [Indexed: 06/19/2023] Open
Abstract
BACKGROUND Life expectancy in patients with Duchenne muscular dystrophy (DMD) has improved due to advances in medical care. DMD patients develop progressive spinal deformity after loss of ambulatory function and onset of wheelchair dependence for mobility. There is limited published data on the effect of spinal deformity correction on long-term functional outcomes, quality of life (QoL), and satisfaction in DMD patients.
AIM To investigate the long-term functional outcomes following spinal deformity correction in DMD patients.
METHODS This was a retrospective cohort study from 2000-2022. Data was collected from hospital records and radiographs. At follow-up, patients completed the muscular dystrophy spine questionnaire (MDSQ). Statistical analysis was performed by linear regression analysis and ANOVA to analyse clinical and radiographic factors significantly associated with MDSQ scores.
RESULTS Forty-three patients were included with mean age 14.4 years at surgery. Spino-pelvic fusion was performed in 41.9% of patients. Mean surgical time was 352.1 min and mean blood loss was 36% of estimated total blood volume. Mean hospital stay was 14.1 d. Postoperative complications occurred in 25.6% of patients. Mean preoperative scoliosis was 58°, pelvic obliquity 16.4°, thoracic kyphosis 55.8°, lumbar lordosis 11.1°, coronal balance 3.8 cm, and sagittal balance + 6.1 cm. Mean surgical correction of scoliosis was 79.2% and of pelvic obliquity was 80.8%. Mean follow-up was 10.9 years (range: 2-22.5). Twenty-four patients had died at follow-up. Sixteen patients completed the MDSQ at mean age 25.4 years (range 15.2-37.3). Two patients were bed-ridden and 7 were on ventilatory support. Mean MDSQ total score was 38.1. All 16 patients were satisfied with the results of spinal surgery and would choose surgery again if offered. Most patients (87.5%) reported no severe back pain at follow-up. Factors significantly associated with functional outcomes (MDSQ total score) included greater duration of post-operative follow-up, age, scoliosis postoperatively, correction of scoliosis, increased lumbar lordosis postoperatively, and greater age at loss of independent ambulation.
CONCLUSION Spinal deformity correction in DMD patients leads to positive long-term effects on QoL and high patient satisfaction. These results support spinal deformity correction to improve long-term QoL in DMD patients.
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Affiliation(s)
- Simon Roberts
- Department of Spinal Surgery, Leeds Teaching Hospitals NHS Trust, Leeds LS1 3EX, United Kingdom
| | - Ayesha Arshad
- Scottish National Spine Deformity Centre, Royal Hospital for Children and Young People, Edinburgh EH16 4TJ, United Kingdom
| | - Athanasios I Tsirikos
- Scottish National Spine Deformity Centre, Royal Hospital for Children and Young People, Edinburgh EH16 4TJ, United Kingdom
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Chou SH, Li WW, Lu CC, Lin KL, Lin SY, Shen PC, Tien YC, Huang HT. Hybrid versus total sublaminar wires in patients with spinal muscular atrophy undergoing scoliosis surgery. BMC Musculoskelet Disord 2021; 22:867. [PMID: 34635092 PMCID: PMC8507395 DOI: 10.1186/s12891-021-04737-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2021] [Accepted: 09/20/2021] [Indexed: 11/10/2022] Open
Abstract
Background Early versions of spinal muscular atrophy (SMA) scoliosis correction surgery often involved sublaminar devices. Recently, the utilization of pedicle screws has gained much popularity. Pedicle screws are generally believed to provide additional deformity correction, but pedicle size and rotational deformity limit their application in the thoracic spine, resulting in a hybrid construct involving pedicle screws and sublaminar wire. Studies of the efficacy of hybrid instrumentation in SMA scoliosis are often limited by the scarcity of the disease itself. In this study, we aimed to compare the surgical outcomes between hybrid constructs involving pedicle screws and sublaminar wire and sublaminar wire alone in patients with SMA scoliosis. Methods We retrospectively reviewed the clinical records and radiographic assessments of patients with SMA scoliosis who underwent corrective surgery between 1993 and 2017. The radiographic assessments included deformity correction and progressive changes in the major curve angle, pelvic tilt (PT) and coronal balance (CB). The correction of deformities was observed postoperatively and at the patient’s 2-year follow-up to test the efficacy of each type of construct. Results Thirty-three patients were included in this study. There were 14 and 19 patients in the wiring and hybrid construct groups, respectively. The hybrid construct group demonstrated a higher major curve angle correction (50.5° ± 11.2° vs. 36.4° ± 8.4°, p < 0.001), a higher apical vertebral rotation correction (10.6° ± 3.9° vs. 4.8° ± 2.6°, p < 0.001), and a reduced progression of the major curve angle at the 2-year follow-up (5.1° ± 2.9° vs. 8.7° ± 4.8°, p < 0.001). A moderate correlation was observed between the magnitude of correction of the apical vertebral rotation angle and the major curve (r = 0.528, p = 0.002). Conclusion This study demonstrated that hybrid instrumentation can provide a greater magnitude of correction in major curve and apical rotation as well as less major curve progression than sublaminar wire instrumentation alone in patients with SMA scoliosis. Level of evidence III Supplementary Information The online version contains supplementary material available at 10.1186/s12891-021-04737-0.
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Affiliation(s)
- Shih-Hsiang Chou
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.,Orthopaedic Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan.,Regeneration Medicine and Cell Therapy Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Wen-Wei Li
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Cheng-Chang Lu
- Orthopaedic Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan.,Regeneration Medicine and Cell Therapy Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan.,Departments of Orthopedics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.,Department of Orthopaedics, Kaohsiung Municipal Siaogang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Kun-Ling Lin
- Department of Obstetrics and Gynecology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.,Department of Obstetrics and Gynecology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Sung-Yen Lin
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.,Orthopaedic Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan.,Regeneration Medicine and Cell Therapy Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan.,Departments of Orthopedics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Po-Chih Shen
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.,Orthopaedic Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yin-Chun Tien
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.,Departments of Orthopedics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Hsuan-Ti Huang
- Department of Orthopaedic Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan. .,Orthopaedic Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan. .,Regeneration Medicine and Cell Therapy Research Center, Kaohsiung Medical University, Kaohsiung, Taiwan. .,Departments of Orthopedics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan. .,Department of Orthopedics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, No. 100, Shiquan 1st Rd., Sanmin Dist., Kaohsiung City, Taiwan.
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Saito W, Inoue G, Shirasawa E, Imura T, Nakazawa T, Miyagi M, Uchida K, Matsuda H, Akazawa T, Takaso M. Risk factors associated with prolonged intensive care unit stay after posterior spinal fusion for Duchenne muscular dystrophy. J Orthop Sci 2021; 26:332-336. [PMID: 32331988 DOI: 10.1016/j.jos.2020.03.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2019] [Revised: 02/25/2020] [Accepted: 03/15/2020] [Indexed: 11/25/2022]
Abstract
BACKGROUND The perioperative complication rate for spinal fusion in Duchenne muscular dystrophy (DMD) remains high and sometimes prolonged perioperative intensive care is needed. We investigated preoperative and intraoperative risk factors associated with prolonged intensive care unit (ICU) stay after posterior spinal fusion. METHODS We reviewed the records of 49 consecutive DMD patients who underwent posterior spinal fusion. Instrumentation was performed from T4 to L5 (46 cases) or to the ilium (3 cases). We recorded the preoperative Cobb angle and perioperative clinical data from patient records. Patients were divided into two groups (ICU stay 0 or 1 day, 2 days or longer). Chi-square and t tests were used for univariate analysis. Factors with p < 0.05 in the univariate analysis were entered into a multilevel logistic regression analysis. RESULTS The average age of patients at surgery was 14.2 years (range 11-20 years). Their preoperative Cobb angle was 79.2° (range 40°-154°). Average operative time was 325.3 min (range 225-507 min). The average estimated blood loss (EBL) was 1673.0 ml (range 500-3785 ml). Eight patients stayed in the ICU for 2 days or longer for postoperative monitoring and treatment. Univariate analysis found statistical difference between the two groups in preoperative Cobb angle, Thoracolumbar kyphosis, %VC, operation time, and EBL during surgery. After multiple logistic regression analysis, Cobb angle and EBL was identified as an independent factor. The patients with a larger Cobb angle and greater EBL had a higher rate of prolonged ICU stay in interquartile range-based comparison. CONCLUSIONS The present study suggests the preoperative Cobb angle and intraoperative EBL could be predictors for postoperative course in posterior spinal fusion for DMD patients.
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Affiliation(s)
- Wataru Saito
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Gen Inoue
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.
| | - Eiki Shirasawa
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Takayuki Imura
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Toshiyuki Nakazawa
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Masayuki Miyagi
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Kentaro Uchida
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Hiromi Matsuda
- Department of Anesthesiology, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Tsutomu Akazawa
- Department of Orthopaedic Surgery, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa, 216-8511, Japan
| | - Masashi Takaso
- Department of Orthopedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
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Faldini C, Viroli G, Fiore M, Barile F, Manzetti M, Merlini L, Ruffilli A. Surgical treatment of scoliosis in Ullrich Congenital Muscular Dystrophy: a case series of 3 patients. Intractable Rare Dis Res 2021; 10:75-80. [PMID: 33996351 PMCID: PMC8122316 DOI: 10.5582/irdr.2020.03162] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Scoliosis in Ullrich Congenital Muscular Dystrophy (UCMD) is very common, with a reported incidence of more than 50%, and it is rapidly progressive. There are no previous studies which specifically focus on scoliosis surgery in UCMD patients. This article reports three cases of scoliosis surgery in UCMD, focusing on operative course, clinical and radiological results achieved, fusion area and complications, with a 2-year follow-up. The surgical technique adopted for vertebral arthrodesis included: high-density pedicle screw systems, asymmetric rods contouring and direct vertebral rotation. The summary results shown a significative correction of the coronal deformity, with a reduction of the mean Cobb angle from 49° to 25° post-operatively. Mean pelvic tilt remained stable, while L5-tilt showed a decrease from 10° to 6°. Mean screw density was 1.92. None of the patients required extended fixation to S2. No major complications were reported, and patients maintained their pre-operative walking ability. All the patients reported a subjective improvement in quality of life, with a better sitting comfort. In conclusion, posterior spinal fusion with high-density pedicle screw systems and direct vertebral rotation may be safe and effective in surgical correction of scoliosis in UCMD. If pelvic obliquity and L5-tilt are less than 15°, could be possible to achieve an optimal spinal and pelvic balance even without sacral or pelvic fixation.
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Affiliation(s)
- Cesare Faldini
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
| | - Giovanni Viroli
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
| | - Michele Fiore
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
- Address correspondence to:Michele Fiore, 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), via G. C. Pupilli 1, 40136, Bologna, Italy. E-mail:
| | - Francesca Barile
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
| | - Marco Manzetti
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
| | - Luciano Merlini
- Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
| | - Alberto Ruffilli
- 1st Orthopaedic and Traumatologic Clinic, IRCCS Istituto Ortopedico Rizzoli (IOR), Bologna, Italy
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Saito W, Inoue G, Shirasawa E, Imura T, Nakazawa T, Miyagi M, Kawakubo A, Uchida K, Kotani T, Akazawa T, Takaso M. Limitations of posterior spinal fusion to L5 for flaccid neuromuscular scoliosis focusing on pelvic obliquity. Spine Deform 2021; 9:559-565. [PMID: 33006744 DOI: 10.1007/s43390-020-00214-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/13/2020] [Accepted: 09/21/2020] [Indexed: 11/25/2022]
Abstract
STUDY DESIGN Retrospective comparison based on the degree of pelvic obliquity (PO). PURPOSE To assess the controversial indications for and limitations of ending the instrumentation for posterior spinal fusion (PSF) at L5 in patients with flaccid neuromuscular scoliosis (fNMS). METHODS We reviewed the cases of 45 patients with progressive spinal deformity as a result of fNMS treated by PSF to L5 and followed for an average of 4 years postoperatively with adequate clinical and radiological data. Anterior-posterior and lateral whole spine radiographs were evaluated. We divided patients into two groups based on the degree of pelvic obliquity (PO) at the final follow-up. Radiographic data from the two groups were analyzed to identify the indications and limitations of this surgical method focusing on PO. RESULTS Preoperatively, there were significant differences between the two groups in Cobb angle, PO, thoracolumbar kyphosis, and lumbar lordosis (LL) while sitting; Cobb angle and LL while supine (Supine Cobb, and Supine LL); and major curve flexibility. Multivariate logistic regression analysis identified Supine Cobb and Supine LL as independent risk factors for residual PO at the final follow-up (Supine Cobb: odds ratio, 1.1; 95% confidence interval 1.0-1.2, Supine LL: odds ratio, 0.9; 95% confidence interval 0.8-1.0). CONCLUSION Patients with larger preoperative Cobb angle and smaller LL while supine may not achieve adequate spine and pelvic correction and this may lead to deterioration in the PO over time, even after spinal fusion ending at L5.
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Affiliation(s)
- Wataru Saito
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Gen Inoue
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.
| | - Eiki Shirasawa
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Takayuki Imura
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Toshiyuki Nakazawa
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Masayuki Miyagi
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Ayumu Kawakubo
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Kentaro Uchida
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
| | - Toshiaki Kotani
- Department of Orthopedic Surgery, Seirei Sakura Citizen Hospital, Sakura, Japan
| | - Tsutomu Akazawa
- Department of Orthopedic Surgery, St. Marianna University School of Medicine, Kawasaki, Japan
| | - Masashi Takaso
- Department of Orthopaedic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
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10
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Intraoperative neurophysiological monitoring during scoliosis surgery in patients with Duchenne muscular dystrophy. EUROPEAN SPINE JOURNAL : OFFICIAL PUBLICATION OF THE EUROPEAN SPINE SOCIETY, THE EUROPEAN SPINAL DEFORMITY SOCIETY, AND THE EUROPEAN SECTION OF THE CERVICAL SPINE RESEARCH SOCIETY 2020; 29:2029-2035. [PMID: 32440770 DOI: 10.1007/s00586-020-06458-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/26/2019] [Accepted: 05/10/2020] [Indexed: 10/24/2022]
Abstract
PURPOSE Little is known about the reliability and value of intraoperative neurophysiological monitoring (IONM) in patients with Duchenne muscular dystrophy (DMD) undergoing scoliosis correction surgery. The aim of this study was to investigate the feasibility of IONM and the cortical excitability in these patients. METHODS Fifteen patients with DMD and scoliosis and 15 patients with adolescent idiopathic scoliosis (AIS) underwent scoliosis correction surgery with the use of IONM. IONM consisted of transcranial electrical stimulation motor evoked potential (Tc-MEP) and somatosensory evoked potential (SSEP) monitoring. The highest Tc-MEP amplitudes were collected to test the feasibility. Preoperative compound muscle action potentials (CMAPs) and transcranial magnetic stimulation (TMS)-MEPs were recorded to test the cortical excitability. SSEPs were scored as elicitable or not elicitable. RESULTS Tc-MEP amplitudes were significantly lower in the DMD group for both the gastrocnemius and tibialis anterior muscles. However, the abductor hallucis muscle had similar amplitudes in both the DMD as the AIS group. TMS/CMAP and Tc-MEP/CMAP ratios were similar in the DMD and AIS group (P = 0.126 and P = 0.792 respectively). CONCLUSIONS Tc-MEP and SSEP monitoring is feasible, particularly when Tc-MEPs are recorded from the abductor hallucis muscle in patients with DMD. Similar TMS/CMAP and Tc-MEP/CMAP ratios show that there were no differences observed in cortical excitability between the groups. IONM seems a feasible and valuable neurophysiological tool to signal possible surgically induced damage to the spinal cord during scoliosis correction surgery in patients with DMD.
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Abstract
BACKGROUND The natural history of scoliosis in Duchenne muscular dystrophy (DMD) is progressive and debilitating if neglected. The purpose of this study was to evaluate outcomes related to spinal deformity surgery in patients with DMD over a 30-year period. METHODS This was a single center retrospective study of all operatively treated scoliosis in DMD patients over 30 years. Minimum follow-up was 2 years. Owing to changes in instrumentation over time, patients were divided into 2 groups: Luque or pedicle screws (PS) constructs. Radiographic, perioperative variables, pulmonary function test (preoperatively and postoperatively), and complication data were evaluated. RESULTS There were 60 subjects (Luque: 47, PS: 13). The Luque group was on average 13 years old, 53 kg, and had 7 years of follow-up. Coronal Cobb was 31±12 degrees preoperatively, 16±11 degrees at first postoperatively, and 21±15 degrees at final follow-up (P≤0.001). Pelvic obliquity was 7±6 degrees preoperatively, 5±5 degrees at first postoperatively (P=0.43), and 5±4 degrees at final follow-up (P=0.77). The majority of this group was fused to L5 (45%) or the sacrum (49%). The PS group was on average 14 years old, 65 kg, and had 4 years of follow-up. Coronal Cobb was 43±19 degrees preoperatively, 12±9 degrees at first postoperatively (P≤0.001), and 12±8 degrees at final follow-up. Pelvic obliquity was 6±5 degrees preoperatively, 3±3 degrees at first postoperatively (P=0.06), and 2±2 degrees at final follow-up (P=0.053). The majority were fused to the pelvis (92%). Both groups' pulmonary function declined with time. Both groups had high complication rates (Luque 68%; PS group 54%). The Luque group had more implant-related complications (26%); the PS group had a higher rate of early postoperative infections (23%). CONCLUSIONS Over a 30-year period of operative treatment of scoliosis in DMD, both PS constructs and Luque instrumentation improved coronal Cobb. The PS group had improved and maintained pelvic obliquity. Both groups had high complication rates. LEVEL OF EVIDENCE Level IV-therapeutic.
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Putzier M, Groß C, Zahn RK, Pumberger M, Strube P. [Characteristics of neuromuscular scoliosis]. DER ORTHOPADE 2017; 45:500-8. [PMID: 27197823 DOI: 10.1007/s00132-016-3272-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
Usually, neuromuscular scolioses become clinically symptomatic relatively early and are rapidly progressive even after the end of growth. Without sufficient treatment they lead to a severe reduction of quality of life, to a loss of the ability of walking, standing or sitting as well as to an impairment of the cardiopulmonary system resulting in an increased mortality. Therefore, an intensive interdisciplinary treatment by physio- and ergotherapists, internists, pediatricians, orthotists, and orthopedists is indispensable. In contrast to idiopathic scoliosis the treatment of patients with neuromuscular scoliosis with orthosis is controversially discussed, whereas physiotherapy is established and essential to prevent contractures and to maintain the residual sensorimotor function.Frequently, the surgical treatment of the scoliosis is indicated. It should be noted that only long-segment posterior correction and fusion of the whole deformity leads to a significant improvement of the quality of life as well as to a prevention of a progression of the scoliosis and the development of junctional problems. The surgical intervention is usually performed before the end of growth. A prolonged delay of surgical intervention does not result in an increased height but only in a deformity progression and is therefore not justifiable. In early onset neuromuscular scolioses guided-growth implants are used to guarantee the adequat development. Because of the high complication rates, further optimization of these implant systems with regard to efficiency and safety have to be addressed in future research.
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Affiliation(s)
- M Putzier
- Centrum für Muskuloskeletale Chirurgie, Klinik für Orthopädie, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland.
| | - C Groß
- Klinik für Orthopädie und Unfallchirurgie, Helios Klinikum Emil von Behring, Walterhöferstraße 11, 14165, Berlin, Deutschland
| | - R K Zahn
- Centrum für Muskuloskeletale Chirurgie, Klinik für Orthopädie, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland
| | - M Pumberger
- Centrum für Muskuloskeletale Chirurgie, Klinik für Orthopädie, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland
| | - P Strube
- Klinik für Orthopädie der Friedrich-Schiller-Universität Jena, Campus Waldkrankenhaus "Rudolf Elle" GmbH, Klosterlausnitzer Straße 81, 07607, Eisenberg, Deutschland
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Archer JE, Gardner AC, Roper HP, Chikermane AA, Tatman AJ. Duchenne muscular dystrophy: the management of scoliosis. JOURNAL OF SPINE SURGERY 2016; 2:185-194. [PMID: 27757431 DOI: 10.21037/jss.2016.08.05] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
This study summaries the current management of scoliosis in patients with Duchenne Muscular Dystrophy. A literature review of Medline was performed and the collected articles critically appraised. This literature is discussed to give an overview of the current management of scoliosis within Duchenne Muscular Dystrophy. Importantly, improvements in respiratory care, the use of steroids and improving surgical techniques have allowed patients to maintain quality of life and improved life expectancy in this patient group.
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Affiliation(s)
- James E Archer
- The Royal Orthopaedic Hospital, Northfield, Birmingham, West Midlands, UK; ; Heartlands Hospital, Bordesley Green East, Birmingham, West Midlands, UK
| | - Adrian C Gardner
- The Royal Orthopaedic Hospital, Northfield, Birmingham, West Midlands, UK
| | - Helen P Roper
- Heartlands Hospital, Bordesley Green East, Birmingham, West Midlands, UK
| | | | - Andrew J Tatman
- Birmingham Children's Hospital, Steelhouse Lane, Birmingham, UK
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Burow M, Forst R, Forst J, Hofner B, Fujak A. Perioperative complications of scoliosis surgery in patients with Duchenne muscular dystrophy and spinal muscular atrophy, focussing on wound healing disorders. Int J Neurosci 2016; 127:479-485. [PMID: 27251216 DOI: 10.1080/00207454.2016.1196688] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/21/2022]
Abstract
PURPOSE Patients with Duchenne muscular dystrophy (DMD) or spinal muscular atrophy (SMA), both neuromuscular diseases, sustain spinal scoliosis in the course of their disease. To reduce the concomitant major morbidity and to improve their quality of life, patients require surgical spine stabilization. This can lead to complications like respiratory, cardiac or neurological complications or wound healing disorders (WHD). To find out the different complexities and risk factors increasing the chance to develop a WHD, the inpatient database was analyzed. METHODS We performed a retrospective statistical study. Therefore, we analyzed the inpatient database of 180 patients (142 DMD and 38 SMA patients). The focus was on WHD. To figure out the risk factors leading to WHD, we conducted a logistic regression. RESULTS Cardiac complications occurred most frequently, followed by pulmonary complications and neurological lesions. Fifty-seven out of 180 patients developed a WHD. In 23 cases the WHD was aseptic, in the other 34 cases dermal organisms, Pseudomonas species and intestinal organisms were responsible. By means of the logistic regression, we were able to identify two more risk factors, in addition to diagnosis and gender, for developing a WHD in our patients: the year of surgery and the direction of pelvic tilt. CONCLUSIONS Most common complications following scoliosis surgery are respiratory and cardiac complications. WHD is a severe complication that implies a prolonged therapy. Some risk factors for developing WHD could be identified in this analysis. Specifically, these were the date of surgery and the direction of pelvic tilt.
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Affiliation(s)
- Mareike Burow
- a Department of Orthopaedic Surgery , Friedrich-Alexander-Universität Erlangen-Nürnberg , Erlangen , Germany
| | - Raimund Forst
- a Department of Orthopaedic Surgery , Friedrich-Alexander-Universität Erlangen-Nürnberg , Erlangen , Germany
| | - Jürgen Forst
- a Department of Orthopaedic Surgery , Friedrich-Alexander-Universität Erlangen-Nürnberg , Erlangen , Germany
| | - Benjamin Hofner
- b Department of Medical Informatics, Biometry and Epidemiology , Friedrich-Alexander-Universität Erlangen-Nürnberg , Erlangen , Germany
| | - Albert Fujak
- a Department of Orthopaedic Surgery , Friedrich-Alexander-Universität Erlangen-Nürnberg , Erlangen , Germany
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Nedelcu T, Georgescu I. Evaluation of the Unit Rod surgical instrumentation in Duchenne scoliosis. A retrospective study. J Med Life 2016; 9:437-443. [PMID: 27928452 PMCID: PMC5141408] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/31/2022] Open
Abstract
The article represents a retrospective clinical and radiological study. Objectives. Evaluating the safety and efficiency of the surgical treatment by using the Unit Rod for scoliosis in adolescents and children presenting Duchenne's muscular dystrophy. Summary. Surgical management of myopathic scoliosis still causes controversies regarding the timing of surgery (patient's age), the pelvic inclusion in the arthrodesis or the advantages of surgery over the conservatory treatment. The patients are very fragile and a long surgery with massive blood loss could lead to serious complications. Unit Rod instrumentation is simple, confers excellent stability and has a low rate of complications. Methods. This is a retrospective clinical and radiological study with a medium follow-up of 6.9 years including 13 patients diagnosed with Duchenne myopathy. All investigated patients were non-ambulatory at the time of surgery and have been treated by the Unit Rod technique at the University Hospital of Rouen between 2002 and 2008. Spinal fusion was, in all cases, realized from T2 to pelvis. Galveston technique of pelvic fixation and Luque's sublaminar wire instrumentation of the spine were used. Results. The results obtained with this treatment and post-surgery complications were analyzed and compared with those from literature. The advantages of this technique consist mostly in a good and stable pelvic fixation, a short interventional time, a minimal blood loss and few complications. Cobb angle correction is similar to that obtained by other surgical procedures. Conclusions. Using the Unit Rod instrumentation of scoliosis in Duchenne's muscular dystrophy is safe, has excellent outcomes, brings post-surgery improvements, and has minor intra and post-surgery complications. The low cost of this treatment could make it a first choice for medical health systems with financial problems.
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Affiliation(s)
- T Nedelcu
- Paediatric Orthopaedics Department, General Hospital, Le Havre, France
| | - I Georgescu
- Paediatric Orthopaedics Department, Paediatric Physical Rehabilitation Centre
Bois Larris, Lamorlaye, France
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Abstract
BACKGROUND Scoliosis in patients with Duchenne muscular dystrophy (DMD) is usually progressive and is treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most patients with DMD and scoliosis. This is an updated review, and an updated search was undertaken in which no new studies were found for inclusion. OBJECTIVES To determine the effectiveness and safety of spinal surgery in patients with DMD with scoliosis. We intended to test whether spinal surgery is effective in increasing survival and improving respiratory function, quality of life, and overall functioning, and whether spinal surgery is associated with severe adverse effects. SEARCH METHODS On 16 June 2015 we searched the Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, and CINAHL Plus. We also searched ProQuest Dissertation and Thesis database (January 1980 to June 2015), the National Institutes of Health Clinical Trials Database (6 January 2015), and the WHO International Clinical Trials Registry Platform (17 June 2015), and checked references. We imposed no language restrictions. SELECTION CRITERIA We planned to include controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in patients with DMD in the review. The control interventions would have been no treatment, non-operative treatment, or a different form of spinal surgery. DATA COLLECTION AND ANALYSIS We used standard methodological procedures expected by The Cochrane Collaboration. Two review authors independently examined the search results and evaluated the study characteristics against inclusion criteria in order to decide which studies to include in the review. MAIN RESULTS Of the 49 relevant studies we found, none met the inclusion criteria for the review because they were not clinical trials, but prospective or retrospective reviews of case series. AUTHORS' CONCLUSIONS Since no randomized controlled clinical trials were available to evaluate the effectiveness of scoliosis surgery in patients with DMD, we can make no good evidence-based conclusion to guide clinical practice. Patients with scoliosis should be informed as to the uncertainty of benefits and potential risks of surgery for scoliosis. Randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of quality of life, functional status, respiratory function, and life expectancy.
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Affiliation(s)
- Daniel KL Cheuk
- The University of Hong Kong, Queen Mary HospitalDepartment of Pediatrics and Adolescent MedicinePokfulam RoadHong Kong SARChina
| | - Virginia Wong
- The University of Hong Kong, Queen Mary HospitalDepartment of Pediatrics and Adolescent MedicinePokfulam RoadHong KongChina
| | - Elizabeth Wraige
- Evelina Children's Hospital, St Thomas' HospitalDepartment of Paediatric NeurologyLambeth Palace RoadLondonUKSE1 7EH
| | - Peter Baxter
- Sheffield Children's HospitalRyegate Children's CentreWestern BankSheffieldSouth YorkshireUKS10 2TH
| | - Ashley Cole
- Sheffield Children's HospitalOrthopaedics DepartmentWestern BankSheffieldUKS10 2TH
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Cawley DT, Carmody O, Dodds MK, McCormack D. Early limited instrumentation of scoliosis in Duchenne muscular dystrophy: is a single-rod construct sufficient? Spine J 2015; 15:2166-71. [PMID: 26070283 DOI: 10.1016/j.spinee.2015.06.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2014] [Revised: 05/02/2015] [Accepted: 06/01/2015] [Indexed: 02/03/2023]
Abstract
BACKGROUND CONTEXT Correction of scoliotic deformity in Duchenne muscular dystrophy (DMD) is performed to maintain adequate seating posture and facilitate comfort. Delaying surgery can predispose to greater morbidity as DMD exhibits progressive cardiorespiratory compromise. Early limited instrumentation may provide a solution to optimize patients with this condition. PURPOSE The aim was to assess outcomes for a cohort of DMD patients who had posterior single-rod instrumentation and bilateral spinal fusion of their neuromuscular scoliotic deformity. STUDY DESIGN This was a retrospective cohort study. PATIENT SAMPLE Forty-one consecutive patients were included. OUTCOME MEASURES Perioperative morbidity, seating outcomes, pulmonary function, deformity correction, and instrumentation integrity were assessed. METHODS Clinical and radiographic review was performed. RESULTS No perioperative mortality or neurologic deterioration was encountered. Total surgical time was 96 minutes, mean total blood loss was 2.3l, mean intensive care unit stay was 41 hours, and overall length of stay was 11 days. Mean Cobb angle improved from 24.3° to 15.6°, pelvic obliquity improved from 7° preoperatively to 5° postoperatively. Three patients had failure of fixation at a mean of 3.5 years. Forced vital capacity was 60% preoperatively and 56% at 1 year, forced expiratory volume/1 second was 67% and 62% at 1 year postoperatively. Seating and posture was satisfactory in all these patients. CONCLUSIONS The authors advocate early operative intervention using a limited instrumentation technique in patients with DMD to maintain seating balance and minimize perioperative morbidity.
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Affiliation(s)
- Derek T Cawley
- Department of Trauma & Orthopaedic Surgery, The Children's University Hospital, Temple St, Dublin 1, Ireland
| | - Olan Carmody
- Department of Trauma & Orthopaedic Surgery, The Children's University Hospital, Temple St, Dublin 1, Ireland
| | - Michael K Dodds
- Department of Trauma & Orthopaedic Surgery, The Children's University Hospital, Temple St, Dublin 1, Ireland
| | - Damian McCormack
- Department of Trauma & Orthopaedic Surgery, The Children's University Hospital, Temple St, Dublin 1, Ireland.
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Corticosteroid Treatment Impact on Spinal Deformity in Duchenne Muscular Dystrophy. INTERNATIONAL SCHOLARLY RESEARCH NOTICES 2014; 2014:965235. [PMID: 27382620 PMCID: PMC4897302 DOI: 10.1155/2014/965235] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/21/2014] [Accepted: 08/24/2014] [Indexed: 01/03/2023]
Abstract
Duchenne muscular dystrophy is a progressive disease with loss of ambulation at around 9-10 years of age, followed, if untreated, by development of scoliosis, respiratory insufficiency, and death in the second decade of life. This review highlights the natural history of the disease, in particular, with regard to the development of the spinal deformity and how this complication has been modified by surgical interventions and overall by corticosteroid treatment. The beneficial effect of corticosteroids may have also an impact on the clinical trial design of the new emerging causative therapies.
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Incidence and the risk factors of spinal deformity in adult patient after spinal cord injury: a single center cohort study. EUROPEAN SPINE JOURNAL : OFFICIAL PUBLICATION OF THE EUROPEAN SPINE SOCIETY, THE EUROPEAN SPINAL DEFORMITY SOCIETY, AND THE EUROPEAN SECTION OF THE CERVICAL SPINE RESEARCH SOCIETY 2014; 24:203-8. [DOI: 10.1007/s00586-014-3534-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/15/2014] [Revised: 08/20/2014] [Accepted: 08/21/2014] [Indexed: 10/24/2022]
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Posterior spinal fusion to sacrum in non-ambulatory hypotonic neuromuscular patients: sacral rod/bone graft onlay method. J Child Orthop 2014; 8:229-36. [PMID: 24728975 PMCID: PMC4142883 DOI: 10.1007/s11832-014-0581-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2013] [Accepted: 03/20/2014] [Indexed: 02/03/2023] Open
Abstract
PURPOSE A retrospective study involving 65 non-ambulatory patients with hypotonic neuromuscular scoliosis has assessed the effectiveness of a sacral rod/bone onlay technique for extending spinal fusion to the sacrum. METHODS To extend posterior spinal fusion to the sacrum, we used either 1 Harrington rod and 1 Luque L rod with sublaminar wires in 14 patients (Group 1) or two rods with sublaminar wires in 51 patients (Group 2) along with abundant autograft and allograft bone covering the ends of the rods. RESULTS Diagnoses were Duchenne muscular dystrophy 53, spinal muscular atrophy 4, myopathy 3, limb girdle muscular dystrophy 2, infantile FSH muscular dystrophy 1, cerebral palsy 1, and Friedreich ataxia 1. Mean age at surgery was 14.3 years (±2.2, range 10.9-25.2). Radiographic follow-up (2 years post-surgery or greater) was 6.4 years (±4.4, range 2-25.3). Using the onlay technique, all patients fused with no rod breakage or pseudarthrosis. For the entire series, the mean pre-operative scoliosis was 54.7° (±31.1, range 0°-120°) with post-operative correction to 21.8° (±21.7, range 0°-91°) and long-term follow-up 24° (±22.9, range 0°-94°). For pelvic obliquity, pre-operative deformity was 17.3° (±11.3, range 0°-51°) with post-operative correction to 8.9° (±7.8, range 0°-35°) and long-term follow-up 10.1° (±8.1, range 0°-27°). Five required revision at a mean of 3.3 years post-original surgery involving rod shortening at the distal end. One of these had associated infection. CONCLUSION Lumbosacral stability and long-term sitting comfort have been achieved in all patients. Problems can be minimized by positioning the rods firmly against the sacrum at the time of surgery with a relatively short extension beyond the L5-S1 junction. The procedure is valuable in hypotonic non-ambulatory neuromuscular patients whose immobility enhances the success rate for fusion due to diminished stress at the lumbosacral junction. It is particularly warranted for those with osteoporosis and a small, deformed pelvis. Considerable weight loss and lengthy rods not closely apposed to the sacrum at the time of surgery played a major role in patients needing revision.
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Abstract
BACKGROUND Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. This is an updated review and an updated search was undertaken in which no new studies were found. OBJECTIVES To determine the effectiveness and safety of spinal surgery in people with Duchenne muscular dystrophy with scoliosis. We intended to test whether spinal surgery is effective in increasing survival, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects. SEARCH METHODS We searched the specialized registers of the Cochrane Neuromuscular Disease Group (31 July 2012), MEDLINE (January 1966 to July 2012), EMBASE (January 1947 to July 2012), CENTRAL (2012, Issue 7 in the Cochrane Library), CINAHL Plus(January 1937 to July 2012), Proquest Dissertation and Thesis Database (January 1980 to July 2012), and the National Institute of Health Clinical Trials Database (July 2012). No language restrictions were imposed. SELECTION CRITERIA We planned to include controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in people with Duchenne muscular dystrophy in the review. The control interventions would have been no treatment, non-operative treatment, or a different form of spinal surgery. DATA COLLECTION AND ANALYSIS Two authors independently examined the search results and evaluated the study characteristics against inclusion criteria to decide which ones would be included in the review. MAIN RESULTS On searching, 47 studies were relevant but none met the inclusion criteria for the review, because they were not clinical trials but prospective or retrospective reviews of case series. AUTHORS' CONCLUSIONS Since there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery in people with Duchenne muscular dystrophy, no evidence-based recommendation can be made for clinical practice. People with scoliosis should be informed about the uncertainty of benefits and potential risks of surgery for scoliosis. Randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of quality of life, functional status, respiratory function and life expectancy.
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Affiliation(s)
- Daniel K L Cheuk
- Department of Pediatrics and Adolescent Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China.
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Mattila M, Jalanko T, Puisto V, Pajulo O, Helenius IJ. Hybrid versus total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis: a comparative study with matched cohorts. ACTA ACUST UNITED AC 2012; 94:1393-8. [PMID: 23015567 DOI: 10.1302/0301-620x.94b10.29383] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
We compared the clinical, radiological and quality-of-life outcomes between hybrid and total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis. A matched comparison using prospectively collected data was undertaken. A total of 66 patients underwent posterior or anteroposterior correction and fusion with hybrid (n = 33, mean age at surgery 15.8 years (9.10 to 19.6)) or total pedicle screw instrumentation (n = 33, mean age 14.7 years (7.0 to 20.7)) with a minimum follow-up of two years. The major curve pre-operatively was a mean of 87° (SD 29, 25° to 141°) and 81° (SD 18, 47° to 116°) in the hybrid and total pedicle screw groups, respectively (p = 0.29) and at a minimum of two years it was 33° (SD 20; 2° to 87°) and 20° (SD 12; 1° to 55°), respectively (p = 0.0016). The mean correction of the major curve was 59% (41% to 88%) in the hybrid and 75% (43% to 99%) in the total pedicle screw groups at two-year follow-up (p = 0.0011). The mean operating time was 7.45 hours (SD 2.18) and 6.04 hours (SD 1.71) in the hybrid and total pedicle screw groups, respectively (p = 0.001), and the mean intra-operative blood loss was 3760 ml (SD 2790) and 1785 ml (SD 1110), respectively (p = 0.001). Total pedicle screw instrumentation provided shorter operating times, less blood loss and better correction of the major curve compared with hybrid constructs in patients undergoing surgery for neuromuscular scoliosis.
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Affiliation(s)
- M Mattila
- Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland
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The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy. EUROPEAN SPINE JOURNAL : OFFICIAL PUBLICATION OF THE EUROPEAN SPINE SOCIETY, THE EUROPEAN SPINAL DEFORMITY SOCIETY, AND THE EUROPEAN SECTION OF THE CERVICAL SPINE RESEARCH SOCIETY 2012. [PMID: 23179984 DOI: 10.1007/s00586-012-2585-4] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
PURPOSE Posterior instrumented spinal fusion is indicated for progressive scoliosis that develops in Duchenne muscular dystrophy (DMD) patients. Whilst spinal fusion is known to improve quality of life, there is inconsistency amongst the literature regarding its specific effect on respiratory function. Our objective was to determine the effect of scoliosis correction by posterior spinal fusion on respiratory function in a large cohort of patients with DMD. Patients with DMD undergoing posterior spinal fusion were compared to patients with DMD not undergoing surgical intervention. METHODS An observational study of 65 patients with DMD associated scoliosis, born between 1961 and 2001: 28 of which underwent correction of scoliosis via posterior spinal fusion (Surgical Group) and 37 of which did not undergo surgical intervention (Non-Surgical Group). Pulmonary function was assessed using traditional spirometry. Comparisons were made between groups at set times, and by way of rates of change over time. RESULTS There was no correlation between the level of respiratory dysfunction and the severity of scoliosis (as measured by Cobb angle) for the whole cohort. The Surgical Group had significantly worse respiratory function at a comparable age pre-operatively compared to the Non-Surgical Group, as measured by per cent predicted forced vital capacity (p = 0.02) on spirometry. The rate of decline of forced vital capacity and per cent predicted forced vital capacity was not slowed following surgery compared to the non-operated cases. There was no significant difference in survival between the two groups. CONCLUSIONS Severity of scoliosis was not a key determinant of respiratory dysfunction. Posterior spinal fusion did not reduce the rate of respiratory function decline. These two points suggest that intrinsic respiratory muscle weakness is the main determinant of decline in respiratory function in DMD.
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Spinal management of Duchenne muscular dystrophy in children. CURRENT ORTHOPAEDIC PRACTICE 2012. [DOI: 10.1097/bco.0b013e318266f55e] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Rodini CO, Collange LA, Juliano Y, Oliveira CS, Isola AM, Almeida SB, Misao MH. Influência da adequação postural em cadeira de rodas na função respiratória de pacientes com distrofia muscular de Duchenne. FISIOTERAPIA E PESQUISA 2012. [DOI: 10.1590/s1809-29502012000200002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
O objetivo deste estudo foi avaliar a influência da adequação postural em cadeira de rodas na função respiratória de pacientes com distrofia muscular de Duchenne (DMD). Participaram 12 pacientes com diagnóstico de DMD e que possuíam cadeira de rodas adaptada com idade variando de 10 a 22 anos. Cada indivíduo foi avaliado na própria cadeira de rodas e em uma cadeira de rodas padrão, ou seja, sem reclinação ou tilt. As cadeiras dos participantes possuíam adaptações no encosto e no assento, confeccionados de acordo com as especificidades de cada paciente. A avaliação consistiu em mensurar o volume minuto (VM), volume corrente (VC), capacidade vital forçada (CVF), pressões inspiratória (PImax) e expiratória (PEmax) máximas e pico de fluxo expiratório (PFE). Para análise dos dados, foi utilizado o teste t pareado, adotando-se o nível de significância de 0,05. As adaptações resultaram em melhores valores estatisticamente significativos de todos os parâmetros respiratórios: VM (8.963,3 e 10.762,5 mL/min; p=0,028), VC (319,1 e 433,6 mL; p=0,005), CVF (1.476,3 e 1.850 mL; p=0,005), PImax (-41,2 e -51,2 cmH2O; p=0,022), PEmax (29,6 e 36,7 cmH2O; p=0,004) e PFE (162,1 e 185 L/min; p=0,018). Nossos resultados sugerem que a adequação postural em cadeira de rodas influenciou positivamente a função respiratória de pacientes com DMD.
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Surgical treatment of Duchenne muscular dystrophy patients in Germany: the present situation. ACTA MYOLOGICA : MYOPATHIES AND CARDIOMYOPATHIES : OFFICIAL JOURNAL OF THE MEDITERRANEAN SOCIETY OF MYOLOGY 2012; 31:21-3. [PMID: 22655513 PMCID: PMC3440800] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
In 1988, we familiarised ourselves at Poitiers with the concept of operative treatment of the lower limbs and the spine in Duchenne muscular dystrophy (DMD) patients which Yves Rideau and his collaborators had developed there in the early 1980s. Thereupon, we immediately established the techniques at our home universities, first at the Technische Universität Aachen and, from 1999 on, at the Universitätsklinikum Erlangen, Germany. Since then, we have applied the technique to more than 500 DMD patients in total by performing more than 800 operations on the lower limbs and/or spine. In support of findings reported by Professor Rideau in this issue we observed that, where patients are still ambulatory at the time of operation, the operation delays the point at which patients become wheelchair-bound by about two years. Likewise, patients receiving this treatment were/are also able to perform the Gowers' manoeuvre for around two years longer.
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Debnath UK, Mehdian SMH, Webb JK. Spinal Deformity Correction in Duchenne Muscular Dystrophy (DMD): Comparing the Outcome of Two Instrumentation Techniques. Asian Spine J 2011; 5:43-50. [PMID: 21386945 PMCID: PMC3047897 DOI: 10.4184/asj.2011.5.1.43] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/09/2010] [Revised: 12/03/2010] [Accepted: 12/06/2010] [Indexed: 11/08/2022] Open
Abstract
STUDY DESIGN A retrospective matched cohort study. PURPOSE To compare the results of combined Luque rod-sublaminar wiring (thoracic) and pedicle (lower lumbar) instrumentation (SLW) versus those with pedicle screw fixation (PS) for scoliosis correction in Duchenne muscular dystrophy (DMD). OVERVIEW OF LITERATURE PS fixation is gaining popularity. Two instrumentation systems were not compared before in a matched cohort of patients. METHODS Two groups of patients with DMD were matched according to the age at surgery, magnitude of deformity and vital capacity. Indications for surgery included loss of sitting balance, rapid decline of vital capacity and curve progression. In group 1 (22 patients) SLW fixation was used from T2/3 to pelvis or sacrum. In group 2 (18 patients) PS fixation was used from T2/3 to L5. Five patients had all level segmental PS fixations. Minimum follow-up was 2 years (range, 2 to 13 years). Radiographs, lung function tests and subjective/objective assessment were performed at standardized intervals. RESULTS Mean Cobb angle in group 1 improved from 45.3° (range, 26 to 75°) to 17.7°(range, 0 to 37°) and mean pelvic obliquity improved from 14.5° (range, 8 to 28°) to 5.6° (range, 0 to 15°). Mean Cobb angle in group 2 improved from 42.8° (range, 28 to 80°) to 7.3° (range, 0 to 20°) and mean pelvic obliquity improved from 11.2° (range, 7 to 30°) to 2.0° (range, 0 to 5°) (p < 0.05). Mean operating time and blood loss were less in group 2 (p < 0.05). In group 1, the infection rate and instrumentation failure was higher, and subjective/objective outcomes showed no significant difference between the groups. CONCLUSIONS PS fixation had superior correction and controlled pelvic obliquity without the need for pelvic fixation.
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Affiliation(s)
- Ujjwal Kanti Debnath
- The Centre for Spinal Studies and Surgery, Queens Medical Centre, Nottingham NG7 2UH, UK
| | | | - John K. Webb
- The Centre for Spinal Studies and Surgery, Queens Medical Centre, Nottingham NG7 2UH, UK
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Cheuk DKL, Wong V, Wraige E, Baxter P, Cole A, N'Diaye T, Mayowe V. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev 2007:CD005375. [PMID: 17253553 DOI: 10.1002/14651858.cd005375.pub2] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
BACKGROUND Scoliosis in people with Duchenne muscular dystrophy is usually progressive and treated with surgery. However, it is unclear whether the existing evidence is sufficiently scientifically rigorous to support a recommendation for spinal surgery for most people with Duchenne muscular dystrophy and scoliosis. OBJECTIVES The objectives of this systematic review were to determine the effectiveness and safety of spinal surgery in Duchenne muscular dystrophy patients with scoliosis. We intended to test whether spinal surgery is effective in increasing life expectancy, improving respiratory function, improving quality of life and overall functioning; and whether spinal surgery is associated with severe adverse effects. SEARCH STRATEGY We searched the specialized registers of the Cochrane Neuromuscular Disease Group and Cochrane Back Group, the Cochrane Central Register of Controlled Trials (January 2006), MEDLINE (January 1966 to January 2006), EMBASE (January 1980 to January 2006), Dissertation Abstracts International (1861 to Jan 2006), CINAHL (January 1982 to January 2006), and the National Institute of Health Clinical Trials Database (January 2006). No language restrictions were imposed. SELECTION CRITERIA Controlled clinical trials using random or quasi-random allocation of treatment evaluating all forms of spinal surgery for scoliosis in patients with Duchenne muscular dystrophy were to be included in the review. The control interventions would have been no treatment, non-operative treatment, or a different form of spinal surgery. DATA COLLECTION AND ANALYSIS Two authors examined the search results and evaluated the study characteristics against inclusion criteria to decide which ones would be included in the review. MAIN RESULTS A total of 402 studies were identified from electronic searching. Thirty-six studies were relevant but none met the inclusion criteria for the review, because they were not clinical trials but prospective or retrospective reviews of case series. AUTHORS' CONCLUSIONS Since there were no randomized controlled clinical trials available to evaluate the effectiveness of scoliosis surgery in people with Duchenne muscular dystrophy, no evidence-based recommendation can be made for clinical practice. Patients should be informed about the uncertainty of benefits and potential risks of surgery for scoliosis. Randomized controlled trials are needed to investigate the effectiveness of scoliosis surgery, in terms of patients' quality of life, functional status, respiratory function and life expectancy.
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Affiliation(s)
- D K L Cheuk
- University of Hong Kong, Department of Pediatrics and Adolescent Medicine, Pokfulam Road, Hong Kong, China.
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