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For: Hahn F, Hauser D, Espinosa N, Blumenthal S, Min K. Scoliosis correction with pedicle screws in Duchenne muscular dystrophy. Eur Spine J 2007;17:255-61. [PMID: 18057966 DOI: 10.1007/s00586-007-0558-9] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/03/2007] [Revised: 10/21/2007] [Accepted: 11/15/2007] [Indexed: 11/29/2022]

For:	Hahn F, Hauser D, Espinosa N, Blumenthal S, Min K. Scoliosis correction with pedicle screws in Duchenne muscular dystrophy. Eur Spine J 2007;17:255-61. [PMID: 18057966 DOI: 10.1007/s00586-007-0558-9] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/03/2007] [Revised: 10/21/2007] [Accepted: 11/15/2007] [Indexed: 11/29/2022]

Number

Cited by Other Article(s)

Geiger F, Eberl J, Wirries A, Forth A, Hammad A. The indication of fusion to the pelvis in neuromuscular scoliosis is based on the underlying disease rather than on pelvic obliquity. 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 2023;32:4063-4072. [PMID: 37750950 DOI: 10.1007/s00586-023-07943-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/05/2023] [Revised: 08/24/2023] [Accepted: 09/04/2023] [Indexed: 09/27/2023]

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]

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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Miyagi M, Saito W, Mimura Y, Nakazawa T, Imura T, Shirasawa E, Uchida K, Ikeda S, Kuroda A, Inoue S, Yokozeki Y, Tanaka Y, Akazawa T, Takaso M, Inoue G. Posterior Spinal Fusion Surgery for Neuromuscular Disease Patients with Severe Scoliosis Whose Cobb Angle Was over 100 Degrees. MEDICINA (KAUNAS, LITHUANIA) 2023;59:1090. [PMID: 37374294 DOI: 10.3390/medicina59061090] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/30/2023] [Revised: 05/26/2023] [Accepted: 06/02/2023] [Indexed: 06/29/2023]

Abstract

Background and objectives: Patients with neuromuscular diseases usually have progressive neuromuscular scoliosis (NMS), requiring invasive surgery. Some patients present with severe scoliosis at the time of consultation and are difficult to treat. Posterior spinal fusion (PSF) surgery combined with anterior release and pre- or intraoperative traction would be effective for severe spinal deformities but would be invasive. This study aimed to evaluate the outcomes of PSF-only surgery for patients with severe NMS with a Cobb angle > 100°. Materials and Methods: Thirty NMS patients (13 boys and 17 girls; mean age 13.8 years) who underwent PSF-only surgery for scoliosis with a Cobb angle > 100° were included. We reviewed the lower instrumented vertebra (LIV), duration of surgery, blood loss, perioperative complications, preoperative clinical findings, and radiographic findings, including Cobb angle and pelvic obliquity (PO) in the sitting position pre- and postoperatively. The correction rate and correction loss of the Cobb angle and PO were also calculated. Results: The mean duration of surgery was 338 min, intraoperative blood loss was 1440 mL, preoperative %VC was 34.1%, FEV1.0 (%) was 91.5%, and EF was 66.1%. There were eight cases of perioperative complications. The Cobb angle and PO correction rates were 48.5% and 42.0%, respectively. We divided the patients into two groups: the L5 group, in which the LIV was L5, and the pelvis group, in which the LIV was the pelvis. The duration of surgery and PO correction rate in the pelvis group were significantly higher than those in the L5 group. Conclusions: Patients with severe NMS demonstrated severe preoperative restrictive ventilatory impairments. PSF surgery without anterior release or any intra-/preoperative traction showed satisfactory outcomes, including acceptable scoliosis correction and improved clinical findings, even in patients with extremely severe NMS. Instrumentation and fusion to the pelvis for severe scoliosis in patients with NMS showed good PO correction and low correction loss of Cobb angle and PO, but a longer duration of surgery.

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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]

Rüwald JM, Upenieks J, Ositis J, Pycha A, Avidan Y, Rüwald AL, Eymael RL, Schildberg FA. Pediatric Scoliosis Surgery-A Comprehensive Analysis of Treatment-Specific Variables and Trends in Latvia. MEDICINA (KAUNAS, LITHUANIA) 2020;56:E201. [PMID: 32344764 PMCID: PMC7230999 DOI: 10.3390/medicina56040201] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/03/2020] [Revised: 04/20/2020] [Accepted: 04/21/2020] [Indexed: 12/02/2022]

Abstract

Background and Objectives: There are currently no data available regarding pediatric scoliosis surgery in Latvia. The aim of this article is to present treatment specific variables, investigate their interrelation, and identify predictors for the length of stay after surgical pediatric scoliosis correction. Materials and Methods: This retrospective study included all surgical pediatric scoliosis corrections in Latvia for the years 2012 to 2016. Analyzed parameters were chosen to portray the patients' demographics, pathology, as well as treatment specific variables. Descriptive, inferential, and linear regression statistics were calculated. Results: A total of 69 cases, 74% female and 26% male, were identified. The diagnostic subgroups consisted of 62% idiopathic (IDI) and 38% non-idiopathic (non-IDI) scoliosis cases. Non-IDI cases had significantly increased operation time, hospital stay, Cobb angle before surgery, and instrumented levels, while IDI cases showed significantly higher Cobb angle percentage correction. For all operated cases, the operation time and the hospital stay decreased significantly over the investigated time period. Early post-operative complications (PCs) occurred in 15.9% of the cases and were associated with increased hospital stay, instrumented levels, and Cobb angle before surgery. The linear regression analysis revealed that operation time and the presence of PCs were significant predictors for the length of the hospital stay. Conclusions: This is the first study to provide comprehensive insight into pediatric scoliosis surgery since its establishment in Latvia. Our regression model offers clinically applicable predictors and further underlines the significance of the operation length on the hospital stay. These results build the foundation for international comparison and facilitate improvement in the field.

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Schur M, Andras LM, Murgai R, Siddiqui AA, Gonsalves NR, Sponseller PD, Emans JB, Vitale MG, Skaggs DL. Pelvic Obliquity Correction in Distraction-Based Growth Friendly Implants. Spine Deform 2019;7:985-991. [PMID: 31732012 DOI: 10.1016/j.jspd.2019.03.003] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/21/2018] [Revised: 02/25/2019] [Accepted: 03/15/2019] [Indexed: 11/17/2022]

Abstract

DESIGN

Multicenter retrospective review.

OBJECTIVE

To evaluate radiographic outcomes and complication rates of patients treated with distraction based implants and pelvic fixation with either screws (sacral-alar-iliac [SAI] screws or iliac screws) or hooks (S hook iliac fixation).

SUMMARY OF BACKGROUND DATA

Multiple options exist for pelvic fixation in distraction-based growing rod systems; however, limited comparative data are available.

METHODS

Early-onset scoliosis (EOS) patients of all diagnoses with distraction-based implants that had pelvic fixation from 2000 to 2013 were reviewed from two EOS multicenter databases. Patients were divided into two groups by type of pelvic fixation: (1) screw group (SAI screws or iliac screws) or (2) S hooks. Exclusion criteria were as follows: index instrumentation ≥10 years old and follow up <2 years. A total of 153 patients met the inclusion criteria. Mean age at index surgery was 6.1 years (range 1.0-9.9 years) and mean follow-up was 4.9 years.

RESULTS

Pelvic fixation in the 153 patients was as follows: screw group = 42 and S hook group = 111. When comparing patients with >20° of initial pelvic obliquity, the screw group had significantly more correction; mean 26° ± 13° for the screw group versus mean 17° ± 7° in the S hook group (p = .039). There was no significant difference in change in T1-S1 length (40 vs. 39 mm, p = .89) or correction of Cobb angle (30° vs. 24°, p = .24). The total complication rate for the screw group was 14% (6/42) versus 25% (28/111) in the S hook group, though this did not achieve significance (p = .25). The most common complications were device migration (13), implant failure (8), and implant prominence (4) for S hooks and implant failure (3), implant prominence (2), and device migration (1) for the screw group.

CONCLUSION

In distraction-based growth-friendly constructs, pelvic fixation with screws achieved better correction of pelvic obliquity than S hooks. Complications were almost twice as common with S hooks than screws, though this did not reach statistical significance.

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Matsumoto M, Miyagi M, Saito W, Imura T, Inoue G, Nakazawa T, Shirasawa E, Uchida K, Akazawa T, Takahira N, Takaso M. Perioperative Complications in Posterior Spinal Fusion Surgery for Neuromuscular Scoliosis. Spine Surg Relat Res 2018;2:278-282. [PMID: 31435534 PMCID: PMC6690101 DOI: 10.22603/ssrr.2017-0075] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2017] [Accepted: 02/09/2018] [Indexed: 11/05/2022] Open

Abstract

Introduction

Patients with neuromuscular disorders sometimes show progressive spinal scoliosis. The surgery for neuromuscular scoliosis (NMS) has high rates of complications. In this study, we elucidated the perioperative complications in patients with NMS.

Methods

We included 83 patients with NMS (58 boys and 25 girls; 61 with muscular dystrophy, 18 with spinal muscular atrophy, and 4 others) who had undergone posterior fusion surgery for scoliosis. We evaluated the perioperative complications (within 3 months), age at time of surgery, operative time, blood loss, preoperative %VC and FEV_1.0 (%) for pulmonary function, and preoperative ejection fraction (EF) for cardiac function.

Results

There were 5 (6%) major complications, including pneumonia and a cardiovascular complication requiring intensive care unit (ICU) care, and 15 (18%) minor complications including viral enteritis and a urinary tract infection. Overall, there were 20 (24%) complications. Three of the 5 major complications were pulmonary. The mean age at the time of surgery was 13.7 y, operative time was 304 min, and blood loss was 1530 ml. The mean preoperative %VC was 41%, FEV_1.0 was 91%, and EF was 60%. When we separated the patients into a group with major complications (n = 5) and a group without major complications (n = 78), the preoperative %VC in the group with major complications (23%) was significantly lower than that in the group without (42%) (p < 0.05). However, operative time, blood loss, preoperative FEV_1.0 (%) and EF between the two groups were not significantly different (p > 0.05).

Conclusions

Compared with the previous findings of the perioperative complication rate (45%-74%) for NMS, the complication rate was remarkably low in this case series. Because of advances in medical skills, including anesthesia and surgical instruments, surgery for NMS appears to be safe. However, patients with NMS with complications demonstrated severe restrictive ventilatory impairment preoperatively. Therefore, we should be vigilant for perioperative pulmonary complications especially in patients with NMS and preoperative severe restrictive ventilatory impairment.

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Segmental Pedicle Screw Instrumentation and Fusion Only to L5 in the Surgical Treatment of Flaccid Neuromuscular Scoliosis. Spine (Phila Pa 1976) 2018;43:331-338. [PMID: 29095413 DOI: 10.1097/brs.0000000000000996] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]

Abstract

STUDY DESIGN

A retrospective cohort study was performed.

OBJECTIVE

The purpose of this study was to determine the efficacy and safety of stopping segmental pedicle screw instrumentation constructs at L5 in the treatment of neuromuscular scoliosis.

SUMMARY OF BACKGROUND DATA

Duchenne muscular dystrophy and spinal muscular atrophy are flaccid neuromuscular disorders in which gradual deterioration is the hallmark and have a lot of characteristics in common despite differences in etiology. Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of flaccid neuromuscular scoliosis and recommended to correct pelvic obliquity. However, the caudal extent of instrumentation and fusion in the surgical treatment of flaccid neuromuscular scoliosis has remained a matter of considerable debate and there have been few studies on the use of segmental pedicle screw instrumentation for flaccid neuromuscular scoliosis.

METHOD

From 2005 to 2007, a total of 27 consecutive patients with neuromuscular disorders (20 Duchenne muscular dystrophy and 7 spinal muscular atrophy), aged 11 to 17 years, underwent segmental pedicle screw instrumentation and fusion only to L5. Assessment was performed clinically and with radiologic measurements. Minimum 2-year follow-up was required for inclusion in this study.

RESULTS

Twenty patients were enrolled in this study. No patient was lost to follow-up. All patients had L5 tilt of less than 15° and a coronal curve with apex L2 or higher preoperatively. Preoperative coronal curve averaged 70° (range: 51°-88°), with a postoperative mean of 15° (range: 5°-25°) and 17° (range: 6°-27°) at the last follow-up. The pelvic obliquity improved from 15° (range: 9°-25°) preoperatively to 5° (range: 3°-8°) postoperatively and 6° (range: 3°-8°) at the last follow-up. The L5 tilt improved from 9° (range: 2°-14°) preoperatively to 2° (range: 0°-4°) postoperatively and 2° (range: 0°-5°) at the last follow-up. Physiologic sagittal plane alignment was recreated after surgery and maintained long-term. There was no significant loss of correction of coronal curve and pelvic obliquity. There was no major complication.

CONCLUSION

Segmental pedicle screw instrumentation and fusion to L5 was safe and effective in patients with flaccid neuromuscular scoliosis with apex L2 or higher and minimal L5 tilt of less than 15°. Segmental pedicle screw instrumentation ending at L5 offered the ability to correct spinal deformity and pelvic obliquity initially, intermediate and even long-term, with no major complications. This method in appropriate patients can be a viable alternative to instrumentation and fusion to the sacrum/pelvis in the surgical treatment of flaccid neuromuscular scoliosis.

LEVEL OF EVIDENCE

N/A.

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Expert's comment concerning Grand Rounds case entitled "Successful Surgery for a Neuromuscular Scoliosis Patient by Pulmonary Rehabilitation with Forced Vital Capacity Below 30%" by Kai Han et al. (ESJO-D-16-01272R4). 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 2018;27:2076-2078. [PMID: 29302741 DOI: 10.1007/s00586-017-5449-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/02/2017] [Accepted: 09/11/2017] [Indexed: 10/18/2022]

Surgical Correction of Scoliosis in Patients With Duchenne Muscular Dystrophy: 30-Year Experience. J Pediatr Orthop 2017;37:e464-e469. [PMID: 26756984 DOI: 10.1097/bpo.0000000000000717] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]

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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Perioperative Evaluation of Respiratory Muscle Strength after Scoliosis Correction in Patients with Duchenne Muscular Dystrophy. Asian Spine J 2017;11:787-792. [PMID: 29093790 PMCID: PMC5662863 DOI: 10.4184/asj.2017.11.5.787] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/15/2017] [Revised: 02/22/2017] [Accepted: 02/23/2017] [Indexed: 11/08/2022] Open

Abstract

Study Design

Retrospective cohort study.

Purpose

To investigate the effect of spinal correction on respiratory muscle strength in patients with Duchenne muscular dystrophy (DMD).

Overview of Literature

Several studies have reported that scoliosis correction in patients with DMD does not improve pulmonary function. In these studies, pulmonary function was evaluated using the traditional spirometric values of percent vital capacity (%VC) and percent forced vital capacity (%FVC). However, traditional spirometry may not be suitable for patients with DMD because the results can be influenced by patient fatigue or level of understanding. Therefore, we evaluated respiratory function focusing on respiratory muscle strength using maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP), in addition to %VC and %FVC.

Methods

We retrospectively reviewed 16 patients with DMD who underwent spinal correction surgery between 2006 and 2011 at Kitasato University Hospital. All patients were males, and the mean age was 13.5 years. Respiratory muscle strength was evaluated using MIP, MEP, and SNIP. Measurements were obtained preoperatively and at 1 and 6 months postoperatively, and %VC and %FVC were obtained preoperatively and within 6 months postoperatively.

Results

The mean preoperative and postoperative %VC values were 54.0% and 51.7%, whereas the mean %FVC values were 53.9% and 53.2%, respectively. The mean MIP, MEP, and SNIP values obtained preoperatively and at 1 and 6 months postoperatively were as follows: MIP, 40.5, 42.7 and 47.2 cm H₂O; MEP, 26.0, 28.0, and 29.0 cm H₂O; and SNIP, 33.4, 33.0, and 33.0 cm H₂O; respectively. The mean MIP and MEP values significantly improved postoperatively. There were no significant differences in SNIP, %VC, or %FVC preand postoperatively.

Conclusions

By focusing on respiratory muscle strength, our results suggest that scoliosis correction in patients with DMD might have a favorable effect on respiratory function.

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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]

The Thoracic Lordosis Correction Improves Sacral Slope and Walking Ability in Neuromuscular Scoliosis. Clin Spine Surg 2016;29:E413-20. [PMID: 27171667 DOI: 10.1097/bsd.0b013e318294368e] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]

Abstract

STUDY DESIGN

Retrospective study.

OBJECTIVE

To report on neuromuscular patients with preserved walking ability, but forward bending of the body due to thoracic lordosis, and to suggest thoracic lordosis correction as the surgical treatment.

SUMMARY OF BACKGROUND DATA

It is an established fact that lumbar lordosis or pelvic parameter is directly related to thoracic sagittal balance. However, the reverse relationship has not been fully defined yet. Loss of thoracic kyphosis results in positive sagittal balance, which causes walking difficulty. Neuromuscular patients with thoracic lordosis have not been reported yet, and there have been no reports on their surgical treatments.

METHODS

This study analyzed 8 patients treated with thoracic lordosis correction surgery. Every patient was diagnosed with muscular dystrophy. In thoracic lordosis correction surgery, anterior release was performed in the first stage and posterior segmental instrumentation was performed in the second stage. Radiographic parameters were compared and walking ability was evaluated with gait analysis. All patients were classified according to the modified Rancho Los Amigos Hospital system preoperatively and 2 years postoperatively to evaluate functional ability. The average follow-up period was 2.9 years.

RESULTS

Before surgery, the mean thoracic sagittal alignment was -2.1-degree lordosis, the mean Cobb angle and sacral slope increased to 36.3 and 56.6 degrees, respectively. The anterior pelvic tilt in gait analysis was 29.3 degrees. At last follow-up after surgery, the mean thoracic sagittal alignment changed to 12.6-degree kyphosis, and the Cobb angle and sacral slope decreased to 18.9 and 39.5 degrees, respectively. Lumbar lordosis and the sacral slope showed significant positive correlation (P<0.001). The improvement in thoracic lordosis showed a significant correlation to the preoperative flexibility of the major curve (P=0.028). The anterior pelvic tilt in gait analysis improved to 15.4 degrees. The functional ability improved in 2 (50%) of 4 patients in class 2 and maintained in remaining 6 patients 2 years after surgery.

CONCLUSIONS

Thoracic lordosis correction surgery in neuromuscular scoliosis patients with thoracic lordosis improved the sacral slope in the standing position and the anterior pelvic tilt in gait. Sagittal imbalance was compensated by the spinopelvic mechanism, and back and hip extensor muscles seem to play a major role in this compensation.

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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: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]

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]

Chua K, Tan CY, Chen Z, Wong HK, Lee EH, Tay SKH, Ong HT, Goh DYT, Hui JHP. Long-term Follow-up of Pulmonary Function and Scoliosis in Patients With Duchenne's Muscular Dystrophy and Spinal Muscular Atrophy. J Pediatr Orthop 2016;36:63-9. [PMID: 26642076 DOI: 10.1097/bpo.0000000000000396] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]

Abstract

BACKGROUND

Spine surgery for neuromuscular scoliosis in patients with Duchenne's Muscular Dystrophy (DMD) and Spinal Muscular Atrophy (SMA) remained controversial. This study aimed to review the long-term results of spine surgery and its effect on pulmonary function in these patients.

METHODS

A retrospective review was conducted for the above patients who had undergone surgery from 1990 to 2006 in a tertiary hospital. Their yearly lung function tests, clinical records, and x-ray films before and after surgery were reviewed. All patients had at least 2 lung function tests performed before surgery and at least 3 lung function tests performed after surgery. Records of perioperative pulmonary infections that resulted in hospital admissions were also retrieved from the hospital computer system.

RESULTS

Forty patients were reviewed: 29 with DMD, 11 with SMA. The mean follow-up period was 11.6 years. For patients with DMD, the mean correction of Cobb's angle from surgery was 34.1 degrees. The rate of decline of the predicted forced vital capacity preoperatively was 7.80% per year, and was reduced to 4.26% per year postoperatively (P<0.001). For patients with SMA, the mean correction of Cobb's angle from surgery was 44.1 degrees. The rate of decline of the predicted forced vital capacity preoperatively was 5.31% per year, and was reduced to 1.77% per year postoperatively (P<0.001). For both DMD and SMA patients, the difference between the rate of preoperative and postoperative pulmonary infections that resulted in hospital admission were, however, not significant (P=0.433 and 0.452, respectively).

CONCLUSIONS

Scoliosis surgery in patients with DMD and SMA results in a long-term decreased rate of decline in pulmonary function over a follow-up period of more than 10 years. The level of the apical vertebrae of the scoliosis did not demonstrate a significant trend on the pulmonary function. The frequency of chest infections did not improve by scoliosis surgery.

LEVEL OF SIGNIFICANCE

Level III—Retrospective study.

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Cheuk DKL, Wong V, Wraige E, Baxter P, Cole A. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev 2015;2015:CD005375. [PMID: 26423318 PMCID: PMC7273403 DOI: 10.1002/14651858.cd005375.pub4] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/10/2022]

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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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]

You JE, Lee HY, Kim K. Comparison of pulmonary function and back muscle strength according to the degree of spinal curvature of healthy adults. J Phys Ther Sci 2015;27:1787-9. [PMID: 26180321 PMCID: PMC4499984 DOI: 10.1589/jpts.27.1787] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2015] [Accepted: 02/26/2015] [Indexed: 11/24/2022] Open

Khirani S, Bersanini C, Aubertin G, Bachy M, Vialle R, Fauroux B. Non-invasive positive pressure ventilation to facilitate the post-operative respiratory outcome of spine surgery in neuromuscular children. 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;23 Suppl 4:S406-11. [DOI: 10.1007/s00586-014-3335-6] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/19/2014] [Revised: 04/24/2014] [Accepted: 04/24/2014] [Indexed: 10/25/2022]

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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Multiaxial high-modularity spinopelvis (HMSP) fixation device in neuromuscular scoliosis: a comparative 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 2013;23:543-9. [PMID: 24346017 DOI: 10.1007/s00586-013-3048-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/15/2013] [Revised: 09/25/2013] [Accepted: 09/25/2013] [Indexed: 10/25/2022]

Scoliosis in Duchenne muscular dystrophy (DMD). Neuromuscul Disord 2013;23:611-7. [DOI: 10.1016/j.nmd.2013.05.003] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]

Cheuk DKL, Wong V, Wraige E, Baxter P, Cole A. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev 2013:CD005375. [PMID: 23450561 DOI: 10.1002/14651858.cd005375.pub3] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/07/2022]

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. 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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Vialle R, Thévenin-Lemoine C, Mary P. Neuromuscular scoliosis. Orthop Traumatol Surg Res 2013;99:S124-39. [PMID: 23337438 DOI: 10.1016/j.otsr.2012.11.002] [Citation(s) in RCA: 92] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 10/29/2012] [Indexed: 02/02/2023]

Mohanty RK, Tripathi A, Lenka P, Equebal A, Kumar R. The effect of a spinal orthosis on posture and cardio-respiratory functions in progressive myopathic scoliosis. Prosthet Orthot Int 2012;36:450-5. [PMID: 22549821 DOI: 10.1177/0309364612442678] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]

Dayer R, Ouellet JA, Saran N. Pelvic fixation for neuromuscular scoliosis deformity correction. Curr Rev Musculoskelet Med 2012;5:91-101. [PMID: 22430864 DOI: 10.1007/s12178-012-9122-2] [Citation(s) in RCA: 41] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]

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]

Hong JY, Suh SW, Park JH, Hur CY, Hong SJ, Modi HN. Changes in level of the conus after corrective surgery for scoliosis: MRI-based preliminary study in 31 patients. Clin Orthop Surg 2011;3:24-33. [PMID: 21369475 PMCID: PMC3042166 DOI: 10.4055/cios.2011.3.1.24] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2010] [Accepted: 06/08/2010] [Indexed: 11/06/2022] Open

Abstract

BACKGROUND

Detection of postoperative spinal cord level change can provide basic information about the spinal cord status, and electrophysiological studies regarding this point should be conducted in the future.

METHODS

To determine the changes in the spinal cord level postoperatively and the possible associated factors, we prospectively studied 31 patients with scoliosis. All the patients underwent correction and posterior fusion using pedicle screws and rods between January 2008 and March 2009. The pre- and postoperative conus medullaris levels were determined by matching the axial magnetic resonance image to the sagittal scout image. The patients were divided according to the change in the postoperative conus medullaris level. The change group was defined as the patients who showed a change of more than one divided section in the vertebral column postoperatively, and the parameters of the change and non-change groups were compared.

RESULTS

The mean pre- and postoperative Cobb's angle of the coronal curve was 76.80° ± 17.19° and 33.23° ± 14.39°, respectively. Eleven of 31 patients showed a lower conus medullaris level postoperatively. There were no differences in the pre- and postoperative magnitude of the coronal curve, lordosis and kyphosis between the groups. However, the postoperative degrees of correction of the coronal curve and lumbar lordosis were higher in the change group. There were also differences in the disease entities between the groups. A higher percentage of patients with Duchenne muscular dystrophy had a change in level compared to that of the patients with cerebral palsy (83.3% vs. 45.5%, respectively).

CONCLUSIONS

The conus medullaris level changed postoperatively in the patients with severe scoliosis. Overall, the postoperative degree of correction of the coronal curve was higher in the change group than that in the non-change group. The degrees of correction of the coronal curve and lumbar lordosis were related to the spinal cord level change after scoliosis correction.

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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

Chechik O, Fishkin M, Wientroub S, Ovadia D. A new pelvic rod system for the surgical correction and fixation of pelvic obliquity in pediatric neuromuscular scoliosis. J Child Orthop 2011;5:41-8. [PMID: 22295048 PMCID: PMC3024490 DOI: 10.1007/s11832-010-0318-y] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2010] [Accepted: 11/30/2010] [Indexed: 02/03/2023] Open

Surgical correction of spinal deformity in patients with congenital muscular dystrophy. J Orthop Sci 2010;15:493-501. [PMID: 20721717 DOI: 10.1007/s00776-010-1486-9] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2010] [Accepted: 03/11/2010] [Indexed: 02/09/2023]

Abstract

BACKGROUND

Congenital muscular dystrophy (CMD), among the myopathic disorders is one form of flaccid neuromuscular disorder (NMD). Patients with NMD frequently develop progressive spinal deformity. For NMD patients who have a severe spinal deformity, sitting is often difficult and is accompanied by pain and breakdown of the skin. Spinal deformity surgery in these patients has been highly effective in stabilizing the spine, maintaining upright, comfortable sitting balance, and improving patients' quality of life. However, many studies have reported significant rates of peri/postoperative complications in these patients. To our knowledge, there has been no study on the results of spinal deformity surgery in patients with CMD. The purpose of this study was to review the clinical and radiological results of spinal deformity surgery in this group of patients with CMD.

METHODS

Between 2004 and 2007, a total of 10 CMD patients underwent scoliosis surgery. There were three patients with Fukuyama CMD, three with Ullrich CMD, and 4 with nonsyndromic CMD (merosin-negative). They were nonambulatory. All the patients had standard posterior spinal fusion and pedicle-screw-alone fixation from T3 or T4 to L5 for spinal deformity. Our inclusion criteria required that each patient (1) had considerable difficulty with sitting balance and pain or breakdown of the skin due to scoliosis; (2) was able to ventilate his or her lung autonomously; (3) was not ventilator-dependent; and (4) did not have cardiac failure. Sufficient informed consent was important, and the decision to perform surgery was made by the patient/family with sufficient preoperative informed consent. Patients were trained with inspiratory muscle training (IMT) using an inspiratory muscle trainer (Threshold IMT) for 6 weeks prior to surgery Cardiac function was assessed preoperatively. Pulmonary function tests were performed preoperatively and postoperatively. Radiographic assessments were performed on sitting anteroposterior (AP) and lateral radiographs. These assessments were made periodically. The Cobb angles of the curves and spinal pelvic obliquity (SPO) on the coronal plane, thoracic kyphosis, and lumbar lordosis were measured. The preoperative AP radiograph and side-bending films were examined to determine flexibility. Patients' and parents' satisfaction were surveyed by a self-completed questionnaire at the last follow-up.

RESULTS

Percent forced vital capacity (%FVC) increased from a mean of 30% before IMT to a mean of 34% the day before surgery. The preoperative scoliosis was 75 degrees (range 61 degrees -95 degrees ). The scoliotic curvature on preoperative side-bending films was 19 degrees (range 11 degrees -28 degrees ). All patients were extubated on the day of surgery. No patients developed cardiac or respiratory complications. The scoliotic curvature was 18 degrees (range 10 degrees -25 degrees ) immediately after surgery, and 19 degrees (range 12 degrees -27 degrees ) at the last follow-up. The pelvic obliquity improved from a mean of 17 degrees (range 14 degrees -20 degrees ) preoperatively to a mean of 6 degrees (range 4 degrees -9 degrees ) postoperatively and to 7 degrees (range 4 degrees -10 degrees ) at the last follow-up. Balanced sitting posture was achieved and maintained. On the sagittal plane, good reconstruction of sagittal plane alignment was recreated and maintained. There were no major complications or deaths. All patients/parents completed the outcome satisfaction questionnaire. Eight patients/parents were very satisfied and two were satisfied.

CONCLUSIONS

Pedicle-screw-alone fixation and fusion to L5 was safe and effective in CMD patients with scoliosis of <95 degrees and pelvic obliquity of <20 degrees . Scoliosis curves were flexible (75% correction) on side-bending films preoperatively. Curve correction and maintenance of correction in the coronal and sagittal plane was excellent. The pelvic obliquity significantly improved. Balanced sitting posture was achieved and maintained in all patients. Our patients with CMD spinal deformity and a moderately and severely decreased FVC could be operated on safely and successfully with general anesthesia. All patients were extubated in the operating room. There were no major complications or deaths. We believe a FVC of <30% alone is not a predisposition to pulmonary complications. However, cardiomyopathy might be a determining risk of mortality, and we believe surgery for these patients should be avoided. Patients' and parents' satisfaction was high.

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Takaso M, Nakazawa T, Imura T, Ueno M, Saito W, Shintani R, Takahashi K, Yamazaki M, Ohtori S, Okamoto M, Masaki T, Okamoto H, Okutomi T, Ishii K, Ueda Y. Can the caudal extent of fusion in the surgical treatment of scoliosis in Duchenne muscular dystrophy be stopped at lumbar 5? 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 2010;19:787-96. [PMID: 20213296 DOI: 10.1007/s00586-010-1347-4] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/12/2009] [Revised: 01/02/2010] [Accepted: 02/11/2010] [Indexed: 10/19/2022]

Abstract

Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of scoliosis in Duchenne muscular dystrophy (DMD) and is recommended to correct pelvic obliquity. The caudal extent of instrumentation and fusion in the surgical treatment of scoliosis in DMD has remained a matter of considerable debate, and there have been few studies on the use of segmental pedicle screw instrumentation for this pathology. From 2004 to 2007, a total of 28 patients with DMD underwent segmental pedicle screw instrumentation and fusion only to L5. Assessment was performed clinically and with radiologic measurements. All patients had a curve with the apex at L2 or higher preoperatively. Preoperative coronal curve averaged 74 degrees, with a postoperative mean of 14 degrees, and 17 degrees at the last follow-up. The pelvic obliquity improved from 17 degrees preoperatively to 6 degrees postoperatively, and 6 degrees at the last follow-up. Good sagittal plane alignment was recreated after surgery and maintained long term. In 23 patients with a preoperative L5 tilt of less than 15 degrees, the pelvic obliquity was effectively corrected to less than 10 degrees and maintained by adequately addressing spinal deformity, while five patients with a preoperative L5 tilt of more than 15 degrees had a postoperative pelvic obliquity of more than 15 degrees. Segmental pedicle screw instrumentation and fusion to L5 was effective and safe in patients with DMD scoliosis with a minimal L5 tilt (<15 degrees) and a curve with the apex at L2 or higher, both initially and long term, obviating the need for fixation to the sacrum/pelvis. Segmental pedicle screw instrumentation and fusion to L5 was safe and effective in patients with DMD scoliosis with stable L5/S1 articulation as evidenced by a minimal L5 tilt of less than 15 degrees, even though pelvic obliquity was significant. There was no major complication. With rigid segmental pedicle screw instrumentation, the caudal extent of fusion in the treatment of DMD scoliosis should be determined by the degree of L5 tilt. This method in appropriate patients can be a viable alternative to instrumentation and fusion to the sacrum/pelvis in the surgical treatment of DMD scoliosis.

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Modi HN, Suh SW, Hong JY, Cho JW, Park JH, Yang JH. Treatment and complications in flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy) with posterior-only pedicle screw instrumentation. 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 2010;19:384-93. [PMID: 19885687 PMCID: PMC2899771 DOI: 10.1007/s00586-009-1198-z] [Citation(s) in RCA: 56] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/05/2009] [Revised: 09/24/2009] [Accepted: 10/18/2009] [Indexed: 10/20/2022]

Abstract

Literature has described treatment of flaccid neuromuscular scoliosis using different instrumentation; however, only one article has been published using posterior-only pedicle screw fixation. Complications using pedicle screws in paralytic neuromuscular scoliosis has not been described before. To present results and complications with posterior-only pedicle screws, a retrospective study was carried out in 27 consecutive patients with flaccid neuromuscular scoliosis (Duchenne muscular dystrophy and spinal muscular atrophy), who were operated between 2002 and 2006 using posterior-only pedicle screw instrumentation. Immediate postoperative and final follow-up results were compared using t test for Cobb angle, pelvic obliquity, thoracic kyphosis and lumbar lordosis. Perioperative and postoperative complications were noted from the hospital records of each patient. Complications, not described in literature, were discussed in detail. Average follow-up was 32.2 months. Preoperative, immediate postoperative and final follow-up Cobb angle were 79.8 degrees , 30.2 degrees (63.3% correction, p < 0.0001) and 31.9 degrees , respectively; and pelvic obliquity was 18.3 degrees , 8.9 degrees (52% correction, p < 0.0001) and 8.9 degrees . Postoperative thoracic kyphosis remained unchanged from 27.6 degrees to 19.9 degrees (p = 0.376); while lumbar lordosis improved significantly from +15.6 degrees to -22.4 degrees lordosis (p = 0.0002). Most patients had major to moderate improvement in postoperative functional and ambulatory status compared to the preoperative status. Thirteen (48.1%) perioperative complications were noted with five major complications (four respiratory in the form of hemothorax or respiratory failure that required ventilator support and one death) and eight minor complications (three UTI, two atelectasis, two neurological and one ileus). Postoperatively, we noted complications, such as coccygodynia with subluxation in 7, back sore on the convex side in 4 and dislodging of rod distally in 1 patient making a total of 12 (44.4%) postoperative complications. Of 12 postoperative complications, 6 (50%) required secondary procedure. We conclude that although flaccid neuromuscular scoliosis can be well corrected with posterior-only pedicle screw, there is a high rate of associated complications.

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Two-year results for scoliosis secondary to Duchenne muscular dystrophy fused to lumbar 5 with segmental pedicle screw instrumentation. J Orthop Sci 2010;15:171-7. [PMID: 20358328 DOI: 10.1007/s00776-009-1437-5] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/13/2009] [Accepted: 11/03/2009] [Indexed: 10/19/2022]

Abstract

BACKGROUND

Instrumentation and fusion to the sacrum/pelvis has been a mainstay in the surgical treatment of scoliosis in patients with Duchenne muscular dystrophy since the development of the intrailiac post. It is recommended for correcting pelvic obliquity. However, caudal extent of instrumentation and fusion has remained a matter of considerable debate. This study was performed to determine the efficacy and safety of stopping segmental pedicle screw constructs at L5 during surgical treatment of scoliosis associated with Duchenne muscular dystrophy (DMD).

METHODS

From May 2005 to June 2007, a total of 20 consecutive patients underwent posterior spinal fusion and segmental pedicle screw instrumentation only to L5 for scoliosis secondary to DMD. All patients had progressive scoliosis, difficulty sitting, and back pain before surgery. A minimum 2-year follow-up was required for inclusion in this study. Assessment was performed clinically and with radiological measurements. The Cobb angles of the curves and spinal pelvic obliquity were measured on the coronal plane. Thoracic kyphosis and lumbar lordosis were measured on the sagittal plane. These radiographic assessments were performed before surgery, immediately after surgery, and at a 3-month interval thereafter. The operating time, blood loss, and complications were evaluated. Patients were questioned about whether they had difficulty sitting and felt back pain before surgery and at 6 weeks, 1 year, and 2 years after surgery.

RESULTS

A total of 20 patients, aged 11-17 years, were enrolled. The average follow-up period was 37 months. Preoperative coronal curves averaged 70 degrees (range 51 degrees -85 degrees ), with a postoperative mean of 15 degrees (range 8 degrees -25 degrees ) and a mean of 17 degrees (range 9 degrees -27 degrees ) at the last follow-up. Pelvic obliquity improved from 13 degrees (range 7 degrees -15 degrees ) preoperatively to 5 degrees degrees (range 3 degrees -8 degrees ) postoperatively and 6 degrees (range 3 degrees -9 degrees ) at the last follow-up. Good sagittal plane alignment was recreated and maintained. Only a small loss of correction of scoliosis and pelvic obliquity was noted. The mean operating time was 271 min (range 232-308 min). The mean intraoperative blood loss was 890 ml (range 660-1260 ml). The mean total blood loss was 2100 ml (range 1250-2880 ml). There was no major complication. All patients reported that difficulty sitting and back pain were alleviated after surgery.

CONCLUSION

Segmental pedicle screw instrumentation and fusion only to L5 is safe and effective in patients with DMD scoliosis of <85 degrees and pelvic obliquity of <15 degrees . Good sagittal plane alignment was achieved and maintained. All patients benefited from surgery in terms of improved quality of life. There was no major complication.

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Surgical management of severe scoliosis with high risk pulmonary dysfunction in Duchenne muscular dystrophy: patient function, quality of life and satisfaction. INTERNATIONAL ORTHOPAEDICS 2010;34:695-702. [PMID: 20155495 DOI: 10.1007/s00264-010-0957-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/11/2009] [Revised: 01/10/2010] [Accepted: 01/11/2010] [Indexed: 10/19/2022]

Takaso M, Nakazawa T, Imura T, Okada T, Ueno M, Fukushima K, Saito W, Sasaki A, Sakagami H, Okamoto M, Masaki T, Okamoto H, Okutomi T, Ishii M, Ueda Y. Segmental pedicle screws instrumentation and fusion to L5 for spinal deformity secondary to Duchenne muscular dystrophy: results with a minimum of 2 years follow-up. EUROPEAN JOURNAL OF ORTHOPAEDIC SURGERY AND TRAUMATOLOGY 2010. [DOI: 10.1007/s00590-010-0589-5] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]

Tian X, Li J, Sheng W, Qu D, Ouyang J, Xu D, Chen S, Ding Z. Morphometry of iliac anchorage for transiliac screws: a cadaver and CT study of the Eastern population. Surg Radiol Anat 2009;32:455-62. [DOI: 10.1007/s00276-009-0589-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2008] [Accepted: 10/23/2009] [Indexed: 11/29/2022]

Pedicle Screw-only Constructs With Lumbar or Pelvic Fixation for Spinal Stabilization in Patients With Duchenne Muscular Dystrophy. ACTA ACUST UNITED AC 2009;22:428-33. [DOI: 10.1097/bsd.0b013e3181872d74] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]

Evaluation of pelvic fixation in neuromuscular scoliosis: a retrospective study in 55 patients. INTERNATIONAL ORTHOPAEDICS 2008;34:89-96. [PMID: 19052744 DOI: 10.1007/s00264-008-0703-z] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/24/2008] [Revised: 10/01/2008] [Accepted: 10/06/2008] [Indexed: 12/26/2022]

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

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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