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Neuromuscular Scoliosis Treatment & Management

  • Author: Matthew B Dobbs, MD; Chief Editor: Jeffrey A Goldstein, MD  more...
 
Updated: Dec 16, 2014
 

Medical Therapy

The goal of nonoperative and operative treatment of patients with neuromuscular scoliosis is the same: to maintain the spine in a balanced position in the coronal and sagittal planes over a level pelvis. This goal is achieved with a custom molded thoracolumbosacral orthosis (TLSO) and molded seating supports. The aim is to control the curve during spinal growth rather than to correct the spinal deformity.

Controlling the curve during spinal growth may delay the need for surgical stabilization and is possible for most patients in the juvenile years. With the onset of the pubertal growth spurt, however, control of the curve is often lost, and surgical stabilization becomes necessary.[3]

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Surgical Therapy

The surgical principles in the management of neuromuscular scoliosis differ from those in idiopathic scoliosis. Fusion is necessary at a younger age, and the fused portion of the spine is longer. Fusion to the sacrum is fairly common because many of these children do not have sitting balance or have pelvic obliquity.[4]

Combined anterior and posterior fusion is common in the treatment of patients with neuromuscular scoliosis, either because posterior elements are absent, as in myelodysplasia, or because it is necessary to gain correction in a rigid lumbar or thoracolumbar curve and achieve a spine fused in balance over a level pelvis.[5, 6, 7, 8, 9, 10] The instrumentation used is segmental, with either a multiple hook-rod system, with or without the addition of sublaminar wires, or a Luque rod and sublaminar wires or a unit rod device. When fusion to the sacrum is necessary, it can be performed with the Luque-Galveston technique or with iliac screws.[11, 12, 13, 14, 15, 16, 17]

Akbarnia et al reported on preliminary results using a magnetically controlled growing rod (MCGR) in children with progressive early onset scoliosis. The study concluded that the technique was safe and provided results similar to that achieved with standard growing rods.[18]

Albert et al retrospectively reviewed the use of polyester bands and clamps utilizing pedicle screws in a hybrid fixation construct to treat neuromuscular scoliosis in 115 pediatric patients.[19] They concluded that this technique is an excellent adjunct in the correction of spinal deformity in these patients and that sublaminar bands in a hybrid construct are safe, achieve corrections equivalent to all-pedicle screw constructs, and may decrease potential complications associated with transpedicular fixation in patients with a highly dysmorphic and osteoporotic spine.

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Preoperative Details

To ensure that the patient can tolerate reconstructive spinal surgery, a detailed preoperative history and assessment should include an evaluation of respiratory competency, cardiac status, nutrition, possible feeding difficulties, seizure disorders, urologic status, and metabolic bone disease.[20]

Patients capable of cooperating should be evaluated with pulmonary function studies. Patients with vital capacities less than 30% of the predicted reference value may require postoperative ventilatory support. Performing formal pulmonary function testing is difficult in patients with neuromuscular scoliosis because patients are often unable to cooperate.[21]

Patients with Duchenne muscular dystrophy and Friedreich ataxia should be evaluated for cardiac involvement.[22]

Poor nutritional status is strongly linked to perioperative complications in these patients. Nutritional deficiencies should be corrected preoperatively through a forced nutritional improvement schedule or postoperatively with feeding tubes. Elective placement of gastric feeding tubes 3 months preoperatively dramatically improves nutritional status. The use of total parenteral nutrition (TPN) perioperatively also can be helpful in decreasing problems with wound infections.[23, 24]

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Intraoperative Details

Intraoperative replacement of blood can be decreased with the use of a cell-saving device. The judicious use of blood products, including fresh frozen plasma and platelet and clotting factor replacements, can prevent disseminated intravascular coagulation.

Because inadequate iliac autograft is available in many of these operations, either because the iliac crest is small or because iliac fixation is used, graft augmentation with allograft or a bone graft substitute is required.

Malignant hyperthermia, characterized by muscular rigidity and increased body temperature, occurs with some frequency in certain neuromuscular disorders and is triggered by inhalational anesthetics and succinyl choline. This should be a consideration in all patients with neuromuscular conditions who are undergoing general anesthesia.

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Postoperative Details

Postoperative care for these patients is demanding. Attention must be paid to pulmonary support, fluid status, and nutrition in addition to the elements of routine postoperative monitoring.[25]

Patients should be mobilized as rapidly as possible for a return to preoperative ambulatory and functional status. Because of the secure fixation obtained with segmental fixation systems and the lower functional demands of these patients, postoperative immobilization is rarely needed.

The postoperative images below depict the same patient shown preoperatively above.

Neuromuscular scoliosis. Postoperative clinical pi Neuromuscular scoliosis. Postoperative clinical picture of young male with severe scoliosis secondary to quadriplegic cerebral palsy.
Neuromuscular scoliosis. Postoperative anteroposte Neuromuscular scoliosis. Postoperative anteroposterior spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy at 2-year follow-up.
Neuromuscular scoliosis. Postoperative lateral spi Neuromuscular scoliosis. Postoperative lateral spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy at 2-year follow-up.
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Follow-up

Hospital stays are usually 7-10 days. Modifications in the child's wheelchair should be made as soon as possible to accommodate the new sitting position. The number of hours spent upright each day should be gradually increased.

The wound should be assessed 3 weeks postoperatively. Radiographs should be obtained 6 weeks postoperatively and again 3 and 6 months after surgery.

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Complications

Because of the multitude of medical comorbidities of these patients, the complication rate after surgery is high. Some complications, however, are more common or significant than others and are included in this discussion. These include respiratory problems, ileus, nutritional problems, hip problems, and crankshaft phenomenon.

  • Respiratory problems: A child with neuromuscular disease often has some degree of intercostal paralysis and a poor cough reflex. As a result, the incidence of postoperative pneumonia is high. To minimize this problem, attention to postoperative respiratory care is essential. It is common to leave the endotracheal tube in place 1 or 2 days after the operation.
  • Ileus: Intestinal hypomotility may persist, necessitating prolonged parenteral support.
  • Nutritional problems: When intestinal motility returns postoperatively but the child cannot tolerate oral feedings, a feeding tube can be passed into the stomach or duodenum to allow nutritional support until oral feeding is tolerated.
  • Hip problems: Hip subluxation, dislocation, and contracture are frequent among patients who do not walk. Parents and caregivers should be told that the hip position might appear worse after the operation when contractures are present preoperatively. Gentle hip range of motion can be started postoperatively, but no stretching is allowed. These restrictions are in effect until the fusion is solid to avoid putting the sacral fixation in jeopardy.
  • Crankshaft phenomenon: Continued anterior spinal growth in the presence of a solid posterior fusion can occur in these children because many of them undergo fusion at a young age. Crankshaft phenomenon can be prevented with anterior fusion. However, the prospect of adding an anterior approach to an operation on a patient with respiratory compromise must be considered.
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Outcome and Prognosis

With care in surgical technique and adequate postoperative care, complications can be minimized. The patient can return to the preoperative functional level with a successful surgical result, which consists of a solidly fused spine in balance in the coronal and sagittal planes over a level pelvis.

Myung et al conducted a retrospective review of the use of posterior-only spinal instrumentation and fusion to the pelvis with iliac screws in 41 patients with neuromuscular scoliosis (mean age, 14 years).[26] The fixation in the pelvis failed in 12 of the 41 (29%). No failures occurred if there were at least six screws in L5, S1, and pelvis (0/7); if there were fewer than six screws in L5, S1, and pelvis, the failure rate was 35% (12/34).

When traditional iliac screws with connectors to rods were used, all constructs had fewer than 6 screws in L5, S1, and pelvis.[26] Only one failure occurred when S2 iliac screws were used, but that failure was without clinical consequence. The mean time from surgery to failure was 18 months (range, 1-49). The authors concluded that not placing bilateral pedicle screws at L5 and S1, in addition to two iliac screws, was associated with a 35% early failure rate of pelvic fixation.

Awwad et al conducted a retrospective analysis to evaluate the safety and efficacy of maximum-width segmental sacropelvic fixation to correct severe pelvic obliquity in 20 patients with neuromuscular scoliosis (mean age, 13 years).[27] All 20 patients underwent spinal fusion with instrumentation extending to the pelvis; 14 underwent primary operations; and six had undergone previous spinal fusion above the pelvis requiring extension to the pelvis. The mean preoperative Cobb angle was 84° (range, 56°–135°), corrected to 41° (range, 8°–75°) postoperatively.

At the final follow-up, the mean spinal curve remained at 42° (range, 10°-75°).[27] The mean preoperative pelvic obliquity was 42° (range, 15°–105°), which was corrected by 78% to 9° (range, 0°-49°) postoperatively, with a pelvic obliquity of 10° (range, 2°-49°) at final follow-up. The authors concluded that maximum-width segmental sacropelvic fixation, utilizing iliosacral screws and/or iliac screws, provides superior correction of severe pelvic obliquity in patients with neuromuscular scoliosis.

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Future and Controversies

In patients requiring combined anterior and posterior spinal fusion, the issue of whether to perform these fusions as staged or same-day surgery remains unsettled. Combined anterior-posterior procedures facilitate spinal correction and a higher union rate in the neuromuscular population. The question of morbidity associated with same-day versus that associated with staged procedures has not been fully resolved.[28]

Intraoperative monitoring has become a standard of care for spinal deformity surgeries. The combination of somatosensory and motor evoked potentials is widely accepted to be accurate and effective in detecting neurologic deficit in most patients during spine surgery. However, the success of this form of monitoring in the patient with neuromuscular scoliosis is still a matter of debate.[29]

The intraoperative use of halo-femoral traction aids in the correction of pelvic obliquity and is becoming more widely used.[30]

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Contributor Information and Disclosures
Author

Matthew B Dobbs, MD Associate Professor, Department of Orthopaedic Surgery, Washington University School of Medicine

Matthew B Dobbs, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Lawrence G Lenke, MD Jerome J Gilden Professor of Orthopedic Surgery, Section of Spinal Surgery, Director of Residency Program, Washington University School of Medicine; Chief of Spinal Surgery, Department of Orthopedic Surgery, St Louis Shriners Hospital

Lawrence G Lenke, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Scoliosis Research Society, American Medical Association, American Orthopaedic Association, American Spinal Injury Association, Missouri State Medical Association, North American Spine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

William O Shaffer, MD Orthopedic Spine Surgeon, Northwest Iowa Bone, Joint, and Sports Surgeons

William O Shaffer, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, Kentucky Medical Association, North American Spine Society, Kentucky Orthopaedic Society, International Society for the Study of the Lumbar Spine, Southern Medical Association, Southern Orthopaedic Association

Disclosure: Received royalty from DePuySpine 1997-2007 (not presently) for consulting; Received grant/research funds from DePuySpine 2002-2007 (closed) for sacropelvic instrumentation biomechanical study; Received grant/research funds from DePuyBiologics 2005-2008 (closed) for healos study just closed; Received consulting fee from DePuySpine 2009 for design of offset modification of expedium.

Chief Editor

Jeffrey A Goldstein, MD Clinical Professor of Orthopedic Surgery, New York University School of Medicine; Director of Spine Service, Director of Spine Fellowship, Department of Orthopedic Surgery, NYU Hospital for Joint Diseases, NYU Langone Medical Center

Jeffrey A Goldstein, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Orthopaedic Association, North American Spine Society, Scoliosis Research Society, Cervical Spine Research Society, International Society for the Study of the Lumbar Spine, AOSpine, Society of Lateral Access Surgery, International Society for the Advancement of Spine Surgery, Lumbar Spine Research Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from NuVasive for consulting; Received royalty from Nuvasive for consulting; Received consulting fee from K2M for consulting; Received ownership interest from NuVasive for none.

Additional Contributors

Lee H Riley III, MD Chief, Division of Orthopedic Spine Surgery, Associate Professor, Departments of Orthopedic Surgery and Neurosurgery, Johns Hopkins University School of Medicine

Disclosure: Nothing to disclose.

References
  1. Benites BD, Traina F, Duarte AD, Lorand-Metze IG, Costa FF, Saad ST. Increased expression of APAF-1 in low-risk myelodysplastic syndrome: a possible role in the pathophysiology of myelodysplasia. Eur J Haematol. 2010 Mar 23. [Medline].

  2. Dane B, Dane C, Aksoy F, Cetin A, Yayla M. Arthrogryposis multiplex congenita: analysis of twelve cases. Clin Exp Obstet Gynecol. 2009. 36(4):259-62. [Medline].

  3. Sarwark J, Sarwahi V. New strategies and decision making in the management of neuromuscular scoliosis. Orthop Clin North Am. 2007 Oct. 38(4):485-96, v. [Medline].

  4. Barsdorf AI, Sproule DM, Kaufmann P. Scoliosis surgery in children with neuromuscular disease: findings from the US National Inpatient Sample, 1997 to 2003. Arch Neurol. 2010 Feb. 67(2):231-5. [Medline].

  5. Bridwell KH, O''Brien MF, Lenke LG, et al. Posterior spinal fusion supplemented with only allograft bone in paralytic scoliosis. Does it work?. Spine. 1994 Dec 1. 19(23):2658-66. [Medline].

  6. Brook PD, Kennedy JD, Stern LM, et al. Spinal fusion in Duchenne''s muscular dystrophy. J Pediatr Orthop. 1996 May-Jun. 16(3):324-31. [Medline].

  7. Gersoff WK, Renshaw TS. The treatment of scoliosis in cerebral palsy by posterior spinal fusion with Luque-rod segmental instrumentation. J Bone Joint Surg Am. 1988 Jan. 70(1):41-4. [Medline].

  8. Sussman MD, Little D, Alley RM, McCoig JA. Posterior instrumentation and fusion of the thoracolumbar spine for treatment of neuromuscular scoliosis. J Pediatr Orthop. 1996 May-Jun. 16(3):304-13. [Medline].

  9. Mercado E, Alman B, Wright JG. Does spinal fusion influence quality of life in neuromuscular scoliosis?. Spine. 2007 Sep 1. 32(19 Suppl):S120-5. [Medline].

  10. Gregg FO, Zhou H, Bertrand SL. Treatment of neuromuscular scoliosis with posterior spinal fusion using the galveston procedure: retrospective of eight years of experience with unit rod instrumentation. J Long Term Eff Med Implants. 2012. 22(1):11-5. [Medline].

  11. Rinsky LA. Surgery of spinal deformity in cerebral palsy. Twelve years in the evolution of scoliosis management. Clin Orthop. 1990 Apr. (253):100-9. [Medline].

  12. Sarwahi V, Sarwark JF, Schafer MF, et al. Standards in anterior spine surgery in pediatric patients with neuromuscular scoliosis. J Pediatr Orthop. 2001 Nov-Dec. 21(6):756-60. [Medline].

  13. Westerlund LE, Gill SS, Jarosz TS, et al. Posterior-only unit rod instrumentation and fusion for neuromuscular scoliosis. Spine. 2001 Sep 15. 26(18):1984-9. [Medline].

  14. Phillips JH, Gutheil JP, Knapp DR Jr. Iliac screw fixation in neuromuscular scoliosis. Spine. 2007 Jun 15. 32(14):1566-70. [Medline].

  15. Modi H, Suh SW, Song HR, Yang JH. Accuracy of thoracic pedicle screw placement in scoliosis using the ideal pedicle entry point during the freehand technique. Int Orthop. 2008 Mar 21. [Medline].

  16. Viswanathan A, Johnson KK, Whitehead WE, Curry DJ, Luerssen TG, Jea A. Hybrid spinal constructs using sublaminar polyester bands in posterior instrumented fusions in children: a series of 5 cases. Neurosurgery. 2010 May. 66(5):862-7; discussion 867. [Medline].

  17. Keeler KA, Lenke LG, Good CR, Bridwell KH, Sides B, Luhmann SJ. Spinal Fusion for Spastic Neuromuscular Scoliosis: Is Anterior Releasing Necessary When Intraoperative Halo-Femoral Traction Is Used?. Spine (Phila Pa 1976). 2010 Apr 13. [Medline].

  18. Akbarnia BA, Cheung K, Noordeen H, Elsebaie H, Yazici M, Dannawi Z, et al. Next Generation of Growth-Sparing Technique: Preliminary Clinical Results of a Magnetically Controlled Growing Rod (MCGR) in 14 Patients With Early Onset Scoliosis. Spine (Phila Pa 1976). 2012 Oct 10. [Medline].

  19. Albert MC, LaFleur BC. Hybrid Fixation With Sublaminar Polyester Bands in the Treatment of Neuromuscular Scoliosis: A Comparative Analysis. J Pediatr Orthop. 2014 Jul 17. [Medline].

  20. Pruijs JE, van Tol MJ, van Kesteren RG, van Nieuwenhuizen O. Neuromuscular scoliosis: clinical evaluation pre- and postoperative. J Pediatr Orthop B. 2000 Oct. 9(4):217-20. [Medline].

  21. Rawlins BA, Winter RB, Lonstein JE, et al. Reconstructive spine surgery in pediatric patients with major loss in vital capacity. J Pediatr Orthop. 1996 May-Jun. 16(3):284-92. [Medline].

  22. Cheuk DK, Wong V, Wraige E, Baxter P, Cole A, N'Diaye T, et al. Surgery for scoliosis in Duchenne muscular dystrophy. Cochrane Database Syst Rev. 2007 Jan 24. CD005375. [Medline].

  23. Mohamad F, Parent S, Pawelek J, Marks M, Bastrom T, Faro F, et al. Perioperative complications after surgical correction in neuromuscular scoliosis. J Pediatr Orthop. 2007 Jun. 27(4):392-7. [Medline].

  24. Canavese F, Gupta S, Krajbich JI, Emara KM. Vacuum-assisted closure for deep infection after spinal instrumentation for scoliosis. J Bone Joint Surg Br. 2008 Mar. 90(3):377-81. [Medline].

  25. Hod-Feins R, Abu-Kishk I, Eshel G, Barr Y, Anekstein Y, Mirovsky Y. Risk factors affecting the immediate postoperative course in pediatric scoliosis surgery. Spine. 2007 Oct 1. 32(21):2355-60. [Medline].

  26. Myung KS, Lee C, Skaggs DL. Early Pelvic Fixation Failure in Neuromuscular Scoliosis. J Pediatr Orthop. 2014 Jul 2. [Medline].

  27. Awwad W, Al-Ahaideb A, Jiang L, Algarni AD, Ouellet J, Harold MU, et al. Correction of severe pelvic obliquity using maximum-width segmental sacropelvic screw fixation: an analysis of 20 neuromuscular scoliosis patients. Eur J Orthop Surg Traumatol. 2014 May 6. [Medline].

  28. Ferguson RL, Hansen MM, Nicholas DA, Allen BL Jr. Same-day versus staged anterior-posterior spinal surgery in a neuromuscular scoliosis population: the evaluation of medical complications. J Pediatr Orthop. 1996 May-Jun. 16(3):293-303. [Medline].

  29. Owen JH, Sponseller PD, Szymanski J, Hurdle M. Efficacy of multimodality spinal cord monitoring during surgery for neuromuscular scoliosis. Spine. 1995 Jul 1. 20(13):1480-8. [Medline].

  30. Huang MJ, Lenke LG. Scoliosis and severe pelvic obliquity in a patient with cerebral palsy: surgical treatment utilizing halo-femoral traction. Spine. 2001 Oct 1. 26(19):2168-70. [Medline].

 
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Neuromuscular scoliosis. Preoperative clinical picture of a young male with severe scoliosis secondary to quadriplegic cerebral palsy.
Neuromuscular scoliosis. Preoperative anteroposterior spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy.
Neuromuscular scoliosis. Preoperative lateral spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy.
Neuromuscular scoliosis. Postoperative clinical picture of young male with severe scoliosis secondary to quadriplegic cerebral palsy.
Neuromuscular scoliosis. Postoperative anteroposterior spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy at 2-year follow-up.
Neuromuscular scoliosis. Postoperative lateral spinal radiograph of young male with severe scoliosis secondary to quadriplegic cerebral palsy at 2-year follow-up.
 
 
 
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