Surgical management of neuromuscular scoliosis: approaches, pitfalls and outcomes

Cover Page

Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription Access

Abstract

Background. Scoliosis is a common problem among neuromuscular disorders with increased incidence of morbidity and mortality. The management of neuromuscular scoliosis (NMS) is complex and requires a cooperative multi-disciplinary team to provide meticulous perioperative care. Conservative treatment, such as bracing, can delay but not replace surgical correction. However, surgery has the risk of higher perioperative complication rates in this patient population.

Aim. It is a retrospective study to evaluate clinical outcome, approaches, pitfalls in management of NMS patients undergoing surgical correction.

Materials and methods. We retrospectively evaluated patients with NMS who underwent surgery for scoliosis from 2004 to 2018. Sixty-eight patients were included in the study after meticulous preoperative checkup. In most cases an single-pedicle screw construct was used, but in a few others a hybrid construct was used. A follow up was performed to make a clinical and radiological assessment and recorded all measurements and any complications. A telephone questionnaire was used for functional evaluation and patient/parent satisfaction with surgery. Only 52 patients could participate in the questionnaire.

Results. The mean follow up period was 43.28 months, and the mean age at the time of surgery was 14.29 years. The mean preoperative Cobbs angle was 71.7°, while that of final follow-up was 34.6°. The mean Cobbs correction percentage was 53.25%. For correction of fixed pelvic obliquity ≥15°, a sacropelvic extension was done in 60.29% of cases. Complications occurred in 39.71% of operated cases; chest related in 36.11% (of all complications), hardware-related 16.67%, visceral complications (as paralytic ileus) in 13.89%, decubitus ulcer and delayed wound healing in 13.89%, deep wound infection in 8.33%, CNS complications (as status epilepticus) in 8.33%, and death in 2.78% (one case). The results of the questionnaire indicated favorable functional outcomes and patient/family satisfaction with surgery.

Conclusion. Despite the perioperative difficulties seen in patients with NMS, patients who had relatively higher postoperative morbidity and mortality, most patients/parents were satisfied with the results of the spinal deformity surgery. The patients/parents would recommend surgery to other patients with similar disorders.

About the authors

Elsayed E. Negm

Tanta University

Author for correspondence.
Email: drsayednegm@gmail.com
ORCID iD: 0000-0003-4021-1585

MD, Assistant lecturer of the Orthopedic Department, Faculty of Medicine

Egypt, Tanta

Vinay Saraph

Medical University of Graz

Email: vinay.saraph@meduni-graz.at
ORCID iD: 0000-0003-0863-9792

MD, Senior consultant of the Pediatric Orthopedic Unit, Department of Pediatric Surgery

Austria, Graz

Mohamed S. Said

Tanta University

Email: profmshafik1@outlook.com
ORCID iD: 0000-0003-1510-0584

MD, Professor and senior consultant of the Orthopedic Department, Faculty of Medicine

Egypt, Tanta

References

  1. Vialle R, Thevenin-Lemoine C, Mary P. Neuromuscular scoliosis. Orthop Traumatol Surg Res. 2013;99(1 Suppl):S124-139. https://doi.org/10.1016/ j.otsr.2012.11.002.
  2. McCarthy RE. Management of neuromuscular scoliosis. Orthop Clin North Am. 1999;30(3):435-449, viii. https://doi.org/10.1016/s0030-5898(05)70096-1.
  3. Allam AM, Schwabe AL. Neuromuscular scoliosis. PMR. 2013;5(11):957-963. https://doi.org/10.1016/ j.pmrj.2013.05.015.
  4. Berven S, Bradford DS. Neuromuscular scoliosis: causes of deformity and principles for evaluation and management. Semin Neurol. 2002;22(2):167-178. https://doi.org/10.1055/s-2002-36540.
  5. Halawi MJ, Lark RK, Fitch RD. Neuromuscular scoliosis: current concepts. Orthopedics. 2015;38(6):e452-456. https://doi.org/10.3928/01477447-20150603-50.
  6. Brooks JT, Sponseller PD. What’s new in the management of neuromuscular scoliosis. J Pediatr Orthop. 2016;36(6):627-633. https://doi.org/10.1097/BPO. 0000000000000497.
  7. Lebel DE, Corston JA, McAdam LC, et al. Glucocorticoid treatment for the prevention of scoliosis in children with Duchenne muscular dystrophy: long-term follow-up. J Bone Joint Surg Am. 2013;95(12):1057-1061. https://doi.org/10.2106/JBJS.L.01577.
  8. Tokala DP, Lam KS, Freeman BJ, Webb JK. Is there a role for selective anterior instrumentation in neuromuscular scoliosis? Eur Spine J. 2007;16(1):91-96. https://doi.org/10.1007/s00586-006-0105-0.
  9. Mattila M, Jalanko T, Puisto V, et al. Hybrid versus total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis: a comparative study with matched cohorts. J Bone Joint Surg Br. 2012;94(10):1393-1398. https://doi.org/10.1302/0301-620X.94B10.29383.
  10. Sarwark J, Sarwahi V. New strategies and decision making in the management of neuromuscular scoliosis. Orthop Clin North Am. 2007;38(4):485-496, v. https://doi.org/10.1016/j.ocl.2007.07.001.
  11. Nordon DG, Lugao AF, Machado LC, et al. Correlation between the degree of correction of neuromuscular scoliosis and patient quality of life. Clinics. 2017;72(2):71-80. https://doi.org/10.6061/clinics/2017(02)02.
  12. Wimmer C, Wallnofer P, Walochnik N, et al. Comparative evaluation of luque and isola instrumentation for treatment of neuromuscular scoliosis. Clin Orthop Relat Res. 2005;439:181-192. https://doi.org/10.1097/01.blo.0000173252.95130.cb.
  13. Bowen RE, Abel MF, Arlet V, et al. Outcome assessment in neuromuscular spinal deformity. J Pediatr Orthop. 2012;32(8):792-798. https://doi.org/10.1097/BPO.0b013e318273ab5a.
  14. Karol LA. Scoliosis in patients with Duchenne muscular dystrophy. J Bone Joint Surg Am. 2007;89 Suppl 1:155-162. https://doi.org/10.2106/JBJS.F.00506.
  15. Kotwicki T, Jozwiak M. Conservative management of neuromuscular scoliosis: personal experience and review of literature. Disabil Rehabil. 2008;30(10):792-798. https://doi.org/10.1080/09638280801889584.
  16. Kotwicki T, Durmala J, Czubak J. Bracing for neuromuscular scoliosis: orthosis construction to improve the patient’s function. Disabil Rehabil Assist Technol. 2008;3(3):161-169. https://doi.org/10.1080/ 17483100801905900.
  17. Weinstein SL. The pediatric spine: principles and practice. Philadelphia: Lippincott Williams & Wilkins; 2001.
  18. Tsirikos AI, Chang WN, Dabney KW, Miller F. Comparison of one-stage versus two-stage anteroposterior spinal fusion in pediatric patients with cerebral palsy and neuromuscular scoliosis. Spine (Phila Pa 1976). 2003;28(12):1300-1305. https://doi.org/10.1097/01.BRS.0000065572.10824.AB.
  19. Hopf CG, Eysel P. One-stage versus two-stage spinal fusion in neuromuscular scolioses. J Pediatr Orthop B. 2000;9(4):234-243. https://doi.org/10.1097/01202412-200010000-00005.
  20. Thacker M, Hui JH, Wong HK, et al. Spinal fusion and instrumentation for paediatric neuromuscular scoliosis: retrospective review. J Orthop Surg (Hong Kong). 2002;10(2):144-151. https://doi.org/10.1177/230949900201000207.
  21. Sharma S, Wu C, Andersen T, et al. Prevalence of complications in neuromuscular scoliosis surgery: a literature meta-analysis from the past 15 years. Eur Spine J. 2013;22(6):1230-1249. https://doi.org/10.1007/s00586-012-2542-2.
  22. Turturro F, Montanaro A, Calderaro C, et al. Rate of complications due to neuromuscular scoliosis spine surgery in a 30-years consecutive series. Eur Spine J. 2017;26(Suppl 4):539-545. https://doi.org/10.1007/s00586-017-5034-6.
  23. Tsirikos AI, Chang WN, Dabney KW, et al. Life expectancy in pediatric patients with cerebral palsy and neuromuscular scoliosis who underwent spinal fusion. Dev Med Child Neurol. 2003;45(10):677-682. https://doi.org/10.1017/s0012162203001269.
  24. Mohamad F, Parent S, Pawelek J, et al. Perioperative complications after surgical correction in neuromuscular scoliosis. J Pediatr Orthop. 2007;27(4):392-397. https://doi.org/10.1097/01.bpb.0000271321.10869.98.
  25. Chidambaran V, Gentry C, Ajuba-Iwuji C, et al. A retrospective identification of gastroesophageal reflux disease as a new risk factor for surgical site infection in cerebral palsy patients after spine surgery. Anesth Analg. 2013;117(1):162-168. https://doi.org/10.1213/ANE.0b013e318290c542.
  26. Bohtz C, Meyer-Heim A, Min K. Changes in health-related quality of life after spinal fusion and scoliosis correction in patients with cerebral palsy. J Pediatr Orthop. 2011;31(6):668-673. https://doi.org/10.1097/BPO.0b013e318221093c.
  27. Suk KS, Baek JH, Park JO, et al. Postoperative quality of life in patients with progressive neuromuscular scoliosis and their parents. Spine J. 2015;15(3):446-453. https://doi.org/10.1016/j.spinee.2014.09.030.
  28. How NE, Street JT, Dvorak MF, et al. Pseudarthrosis in adult and pediatric spinal deformity surgery: a systematic review of the literature and meta-analysis of incidence, characteristics, and risk factors. Neurosurg Rev. 2019;42(2):319-336. https://doi.org/10.1007/s10143-018-0951-3.
  29. Modi HN, Suh SW, Song HR, et al. Treatment of neuromuscular scoliosis with posterior-only pedicle screw fixation. J Orthop Surg Res. 2008;3:23. https://doi.org/10.1186/1749-799X-3-23.
  30. Lowenstein JE, Matsumoto H, Vitale MG, et al. Coronal and sagittal plane correction in adolescent idiopathic scoliosis: a comparison between all pedicle screw versus hybrid thoracic hook lumbar screw constructs. Spine (Phila Pa 1976). 2007;32(4):448-452. https://doi.org/10.1097/01.brs.0000255030.78293.fd.
  31. Murphy NA, Firth S, Jorgensen T, Young PC. Spinal surgery in children with idiopathic and neuromuscular scoliosis. What’s the difference? J Pediatr Orthop. 2006;26(2):216-220. https://doi.org/10.1097/01.bpo.0000206516.61706.6e.
  32. Hod-Feins R, Abu-Kishk I, Eshel G, et al. Risk factors affecting the immediate postoperative course in pediatric scoliosis surgery. Spine (Phila Pa 1976). 2007;32(21):2355-2360. https://doi.org/10.1097/BRS.0b013e3181558393.
  33. Master DL, Son-Hing JP, Poe-Kochert C, et al. Risk factors for major complications after surgery for neuromuscular scoliosis. Spine (Phila Pa 1976). 2011;36(7):564-571. https://doi.org/10.1097/BRS.0b013e3181e193e9.
  34. Suk KS, Lee BH, Lee HM, et al. Functional outcomes in Duchenne muscular dystrophy scoliosis: comparison of the differences between surgical and nonsurgical treatment. J Bone Joint Surg Am. 2014;96(5):409-415. https://doi.org/10.2106/JBJS.M.00777.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 4. The frequency and types of postoperative complications

Download (103KB)
Indexing metadata
3. Fig. 5. Generalized results of responses to the first part of the questionnaire depending on the functional status of the patient and daily activity

Download (116KB)
Indexing metadata
4. Fig. 6. Summarized results of responses to the second part of the questionnaire related to patient / family satisfaction with the results of the operation

Download (140KB)
Indexing metadata
5. Fig. 1. Preoperative (a–b), immediate postoperative (c–d), and final postoperative (e–f) PA and lateral x-rays of an example case

Download (292KB)
Indexing metadata
6. Fig. 2. Preoperative (a–b), and postoperative (c–d) clinical photos of the example case

Download (282KB)
Indexing metadata
7. Fig. 3. Neuromuscular pathology among operated patients. CP: Cerebral Palsy, MMC: Myelomeningocele, SMA: Spinal muscle atrophy, NFM-1: Neurofibromatosis type 1, SCI: Spinal cord injury, HSMN: Hereditary sensory and motor neuropathy

Download (79KB)
Indexing metadata
8. Fig. 5. Summary of the responses of 1st part of the questionnaire regarding patients’ functional status, and daily activities

Download (83KB)
Indexing metadata
9. Fig. 6. Summary of the responses of the 2nd part of the questionnaire concerned with patient / family satisfaction from surgery

Download (127KB)
Indexing metadata

Copyright (c) 2020 Negm E.E., Saraph V., Said M.S.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
 


Согласие на обработку персональных данных с помощью сервиса «Яндекс.Метрика»

1. Я (далее – «Пользователь» или «Субъект персональных данных»), осуществляя использование сайта https://journals.rcsi.science/ (далее – «Сайт»), подтверждая свою полную дееспособность даю согласие на обработку персональных данных с использованием средств автоматизации Оператору - федеральному государственному бюджетному учреждению «Российский центр научной информации» (РЦНИ), далее – «Оператор», расположенному по адресу: 119991, г. Москва, Ленинский просп., д.32А, со следующими условиями.

2. Категории обрабатываемых данных: файлы «cookies» (куки-файлы). Файлы «cookie» – это небольшой текстовый файл, который веб-сервер может хранить в браузере Пользователя. Данные файлы веб-сервер загружает на устройство Пользователя при посещении им Сайта. При каждом следующем посещении Пользователем Сайта «cookie» файлы отправляются на Сайт Оператора. Данные файлы позволяют Сайту распознавать устройство Пользователя. Содержимое такого файла может как относиться, так и не относиться к персональным данным, в зависимости от того, содержит ли такой файл персональные данные или содержит обезличенные технические данные.

3. Цель обработки персональных данных: анализ пользовательской активности с помощью сервиса «Яндекс.Метрика».

4. Категории субъектов персональных данных: все Пользователи Сайта, которые дали согласие на обработку файлов «cookie».

5. Способы обработки: сбор, запись, систематизация, накопление, хранение, уточнение (обновление, изменение), извлечение, использование, передача (доступ, предоставление), блокирование, удаление, уничтожение персональных данных.

6. Срок обработки и хранения: до получения от Субъекта персональных данных требования о прекращении обработки/отзыва согласия.

7. Способ отзыва: заявление об отзыве в письменном виде путём его направления на адрес электронной почты Оператора: info@rcsi.science или путем письменного обращения по юридическому адресу: 119991, г. Москва, Ленинский просп., д.32А

8. Субъект персональных данных вправе запретить своему оборудованию прием этих данных или ограничить прием этих данных. При отказе от получения таких данных или при ограничении приема данных некоторые функции Сайта могут работать некорректно. Субъект персональных данных обязуется сам настроить свое оборудование таким способом, чтобы оно обеспечивало адекватный его желаниям режим работы и уровень защиты данных файлов «cookie», Оператор не предоставляет технологических и правовых консультаций на темы подобного характера.

9. Порядок уничтожения персональных данных при достижении цели их обработки или при наступлении иных законных оснований определяется Оператором в соответствии с законодательством Российской Федерации.

10. Я согласен/согласна квалифицировать в качестве своей простой электронной подписи под настоящим Согласием и под Политикой обработки персональных данных выполнение мною следующего действия на сайте: https://journals.rcsi.science/ нажатие мною на интерфейсе с текстом: «Сайт использует сервис «Яндекс.Метрика» (который использует файлы «cookie») на элемент с текстом «Принять и продолжить».