Kyphosis In Myelomeningocele Research Articles

Surgical limits, pitfalls, and potential solutions in kyphectomy in myelomeningocele: three cases and systematic review of the literature.

To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic. Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients' information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies. Three cases underwent posterior vertebral column resection (pVCR) of 2-4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other. Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.

Kyphectomy in the treatment of patients with myelomeningocele

Background context Myelomeningocele kyphosis is a complex disorder that usually requires surgical intervention. Many complications can occur as a result of this disorder and its treatment, but only surgical correction offers the possibility of restoring spinal alignment. Purpose The purpose of this retrospective study was to summarize the surgical results, complications, and short-term and midterm outcomes for surgical correction of severe kyphosis using a consistent surgical technique. Study design This was a retrospective review of our database of pediatric patients with myelomeningocele and lumbar kyphosis who underwent kyphectomy with the use of the Warner and Fackler technique. Patient sample Eleven pediatric kyphectomy cases performed by a single surgeon from 1984 to 2009 were reviewed. Outcome measures Outcome measures include imaging, kyphotic angle measurement, and physical examination. Methods Patients underwent the Warner and Fackler technique of posterior-only kyphectomy and bayonet-shaped anterior sacral fixation. Results The mean extent of kyphosis was 115.6° (range, 77–176°) preoperatively with a correction to 13.0° (range, 0–32°) postoperatively, and a reduction with an average of 102.6° (range, 65–160°), for an 88.7% correction. On an average, 2.0 (range, 1–6) vertebrae were resected. Immediately postoperatively and at follow-up, with an average of 67.2 months (range, 8–222 months), the average kyphosis angle was 13.0° (range, 0–32°). All patients undergoing the procedure were unable to lie supine preoperatively. All patients postoperatively could lie in the supine position. The functional outcome in patients and caretakers was rated very favorably because all patients and caretakers who provided feedback (9 of 11) reported that they were satisfied with the procedure and would undergo the procedure again if given the choice. Conclusions This technique has become the most effective surgical reconstruction in myelomeningocele kyphosis. Although significant complications can occur during and after the procedure, most patients had satisfactory postoperative outcomes and restoration of sagittal balance with high patient and parent satisfaction

Kyphectomy in children with myelomeningocele.

Patients with myelomeningocele and rigid lumbar and thoracolumbar kyphosis face substantial functional difficulties with sitting and lying supine and are prone to skin breakdown over the gibbus and risk of infection. Kyphectomy, along with cordotomy and segmental spinal instrumentation down to the pelvis, is one alternative that can provide reliable correction of the deformity but also can maintain that correction over a period of time. We determined the fusion rates, deformity correction and maintenance, and perioperative complications of kyphectomy with long segmental spinal instrumentation using the Warner and Fackler technique. We retrospectively reviewed the charts and radiographs of 33 patients with myelomeningocele who had kyphectomy with segmental spinal instrumentation down to the pelvis between 1991 and 2006. The average age at surgery was 7.6 years (range, 3-19 years). Twenty-one patients had a minimum 2-year followup (average, 7.0 years; range, 2.4-15.7 years). The average preoperative kyphosis of 124° (range, 75°-210°) improved at last followup to 22° (range, 3°-55°) with an average correction of 81% (range, 59%-98%). We identified 17 postoperative complications. Wound and skin complications were most common; 11 secondary surgeries were performed in 10 patients. Surgery for myelomeningocele kyphosis is technically demanding and carries substantial risk. Kyphectomy and posterior spinal fusion and instrumentation with the Warner and Fackler technique allow correction and maintenance of sagittal alignment.

Posterior Kyphectomy for Myelomeningocele With Anterior Placement of Fixation: A Retrospective Review

Kyphosis in myelomeningocele is a rare and difficult problem. Many strategies have been used with no single procedure universally agreed on. Techniques involving anterior and posterior fixation may provide better fusion. We describe a novel procedure for anteroposterior kyphectomy in patients with myelomeningocele. Apical posterior kyphectomy is followed by the insertion of two rods distally into the vertebral bodies and sacrum. Sublaminar wires are placed superiorly and the kyphosis is reduced by sequential tightening. We determined kyphosis correction and intraoperative blood loss for this new procedure. We retrospectively reviewed 22 patients (average age, 7.6 years [range, 2-17 years]) who underwent apical kyphectomy from 1982 to 2008. Charts were examined and radiographs measured preoperatively, immediately postoperatively, and at final followup. Followup averaged 6.4 years (range, 0-14 years) with 19 patients having at least 2 years of followup. Kyphosis decreased from a mean of 123° (range, 79°-163°) preoperatively to 40° (range, 13°-92°) immediately postoperatively and was a mean of 60° (range, 14°-126°) at final followup. Operating time was 248 minutes (range, 180-345 minutes), estimated blood loss was 765 mL (range, 140-2100 mL), and length of stay was 14 days (range, 1-57 days). Ten of the 22 patients had complications with eight requiring reoperation. This anteroposterior kyphectomy provided a high level of kyphosis correction, which was largely maintained over the study period. Blood loss, surgical time, and complication rates were acceptable as compared with other techniques reported in the literature.

Combination of Luque Instrumentation With Polyaxial Screws in the Treatment of Myelomeningocele Kyphosis

Rigid congenital kyphosis in myelomeningocele is associated with an important morbidity with skin breakdown, recurrent infection, and decreased function. Kyphectomy is the classic treatment to restore spinal alignment; however, surgery is associated with morbidity and long-term complications. The purpose of this retrospective study was to examine the authors' experience using combination of Luque instrumentation with posterolaterally placed polyaxial screws in the treatment of myelomeningocele kyphosis. From June 1999 to June 2003, 7 patients were treated and followed up for an average of 68.6 months. The average age at the time of the operation was 7 year and 1 month. All patients underwent vertebral excision from just above the apex of vertebral deformity to realign the sagittal deformity. Posterolaterally placed polyaxial screws were used in combination with segmental Luque instrumentation. Kyphotic deformity averaged 104 degrees before surgery, 15.2 degrees after surgery, and 18.5 degrees at the latest follow-up. The average loss of correction was 3.3 degrees. The average blood loss was 611 mL. Complications occurred in 2 of 7 patients were superficial wound breakdown and deep wound infection that required rotational flap closure. Kyphectomy with posterior instrumentation by using Luque technique in the combination with polyaxial screws is reliable method for correcting rigid kyphotic deformity in patients with myelomeningocele. Rigidity of the construct, greater correction capacity, and low profile instrumentation by the help of posterolateral insertion of the polyaxial screws and wiring were the distinct advantages of this technique.

Kyphectomy in myelomeningocele with a modified Dunn-McCarthy technique followed by an anterior inlayed strut graft.

Rigid congenital kyphosis in myelomeningocele is associated with an important morbidity with skin breakdown, recurrent infection, and decreased function. Kyphectomy is the classic treatment to restore spinal alignment; however, surgery is associated with an important morbidity and long-term correction is uncertain. The authors retrospectively reviewed 9 patients with a mean age of 8.8 years who underwent a two stage surgical procedure: first a posterior kyphectomy with a modified Dunn-McCarthy fixation consisting of lumbar pedicle screws and long S-shape rods buttressing the anterior sacrum. Then a second stage done several weeks later consisting of a thoraco-abdominal approach to the spine with an inlay strut graft classically from T10-S1. The mean follow-up was 34 months (range 1-5 years). The kyphosis was corrected from a mean of 110 degrees of Cobb angle (range 70-130 degrees) to 15 degrees after surgery (45-0 degrees). There was no instrumentation failure, no loss of correction and no pseudarthrosis. Complications consisted of one intra-operative cardiac arrest fortunately reversible, a wound necrosis, one deep venous thrombosis and one late aseptic bursitis on the posterior hardware. Congenital kyphosis in myelomeningocele can be treated successfully with an initial posterior approach correction and instrumentation followed by an anterior approach allowing for anterior inlay impacted structural graft. The authors believe that this technique improves biomechanical and biological fusion mass anteriorly and will prevent late instrumentation failure and loss of correction.

A kyphectomy technique with reduced perioperative morbidity for myelomeningocele kyphosis.

The lumbar sacropelvis in 11 patients with myelomeningocele and kyphosis was treated with a subtraction kyphectomy technique and posterior instrumentation. The results of this procedure in the 11 patients were evaluated and compared with previous results. To examine critically their experience using the subtraction (decancellation) vertebrectomy technique combined with posterior instrumentation for myelomeningocele kyphosis, the authors reviewed the charts of 18 myelomeningocele patients who underwent surgery for lumbar kyphosis between 1994 and 1998. The benefits of restoring sagittal spinal alignment in myelomeningocele patients with severe lumbar kyphosis deformity to achieve postural stability and improved sitting balance generally are accepted. The optimal method of deformity correction, the extent of instrumentation, and the role of limited arthrodesis remain undefined. Of the 18 patients considered, 11 met the inclusion criteria of having undergone reconstruction using a subtraction (decancellation) vertebrectomy technique, preservation of the thecal sac, limited arthrodesis with posterior transpedicular lumbosacral instrumentation, and a minimum follow-up evaluation of 2 years. The study considered the age of the patient, number of levels fused, estimated blood loss, preoperative deformity, immediate postoperative correction, magnitude of correction, and maintenance of correction at latest follow-up assessment. The average age at the time of the index procedure was 6 years (range, 3-12 years). The average preoperative kyphosis was 88 degrees (range, 50-149 degrees ). Immediately after surgery, the average curve measurement was 3 degrees lordosis (range, 50 degrees to 50 degrees ). The average magnitude of postoperative sagittal plane deformity correction was 91 degrees (range, 43-126 degrees ). Finally, the magnitude of correction maintained at the final follow-up assessment averaged 66 degrees (range, 22-114 degrees ). This represented an average loss of correction at 2 years of 24 degrees (range, 0-84 degrees ). There were no deaths, episodes of acute-onset hydrocephalus, vascular complications, or chronic deep wound infections. The subtraction (decancellation) vertebrectomy technique with preservation of the dural sac is a safe and efficacious technique for correction and stabilization of myelomeningocele kyphosis in young patients. Morbidity is reduced, as compared with that of excision techniques. Restoration of sagittal alignment at the time of initial correction and stabilization to achieve a balanced spine led to acceptable results.

Sagittal static imbalance in myelomeningocele patients: improvement in sitting ability by partial and total gibbus resection.

The progression of kyphosis in myelomeningocele is independent of skeletal growth and requires early operative correction and stabilization to prevent a loss of sitting ability. In severe cases, only vertebrectomy makes it possible to achieve correction, stability and skin-closure without tension. In 14 patients with myelomeningocele gibbus, kyphectomy was performed, removing two vertebral bodies on average. The average kyphosis angle decreased from 128 degrees to 81 degrees, enabling most of the patients to participate again in social life by restoring wheelchair mobility. Nevertheless, a significantly higher complication rate was found compared to other correctional operations, lengthening the average hospital stay to 41 days. Special problems arose from trophic disorders of the skin and soft tissue and from the dystrophic muscles below the level of neural malfunction. In three cases, kyphosis reappeared cranial to the fused segments, requiring ventral stabilization. With respect to increasing kyphosis angle, an early intervention should be aimed at. A secondary operation can be necessary, if surgery is performed without taking care of the growth potential.

Orthopedic and neurosurgical treatment of severe kyphosis in myelomeningocele.

Kyphosis in myelomeningocele is characterized by a complex pattern of problems during development and therapy. On the one hand, decompensation of upright posture leads to loss of sitting ability and social integration; on the other hand, accompanying malformations and trophic alterations threaten the physical integrity and performance. Neurologic function, cerebrospinal fluid (CSF) circulation, skeletal deformity and the urinary transport system need to be kept in mind and need to be treated with cooperation between the different specialties. Especially during serious surgical interventions such as spinal surgery, neither the nervous system nor the kidneys must be ignored. Sixteen patients underwent kyphectomy in the Orthopedic Department of the University of Mainz between 1993 and 1997, all of them supervised by the Neurosurgical Department. In 13 cases, transversal myelotomy was performed. No insufficiency of CSF circulation was seen; neither were there any CSF fistulae. Particular problems arose from the skin and soft tissue above the gibbus, the lack of muscles and the regeneration deficiency caused by trophic disorders. Therefore, a significantly higher complication rate was found than with other correctional operations.

Congenital kyphosis in myelomeningocele

To determine the effect of cordotomy on the function of the bladder during surgical correction of congenital kyphosis in myelomeningocele, we reviewed 13 patients who had this procedure between 1981 and 1996. The mean age of the patients at operation was 8.9 years (3.7 to 16) and the mean follow-up was 4.8 years (1.3 to 10.8). Bladder function before and after operation was assessed clinically and quantitatively by urodynamics. The mean preoperative kyphosis was 117° (52 to 175) and decreased to 49° (1 to 89) immediately after surgery. At the latest follow-up, a mean correction of 52% had been achieved. Only one patient showed deterioration in bladder function after operation. Eight out of the nine patients who had urodynamic assessment had improvement in bladder capacity and compliance, and five showed an increase in urethral pressure. One patient developed a spastic bladder and required subsequent surgical intervention. Cordotomy, at or below the level of the kyphosis, allows excellent correction of the structural deformity.

Congenital kyphosis in myelomeningocele. The effect of cordotomy on bladder function.

To determine the effect of cordotomy on the function of the bladder during surgical correction of congenital kyphosis in myelomeningocele, we reviewed 13 patients who had this procedure between 1981 and 1996. The mean age of the patients at operation was 8.9 years (3.7 to 16) and the mean follow-up was 4.8 years (1.3 to 10.8). Bladder function before and after operation was assessed clinically and quantitatively by urodynamics. The mean preoperative kyphosis was 117 degrees (52 to 175) and decreased to 49 degrees (1 to 89) immediately after surgery. At the latest follow-up, a mean correction of 52% had been achieved. Only one patient showed deterioration in bladder function after operation. Eight out of the nine patients who had urodynamic assessment had improvement in bladder capacity and compliance, and five showed an increase in urethral pressure. One patient developed a spastic bladder and required subsequent surgical intervention. Cordotomy, at or below the level of the kyphosis, allows excellent correction of the structural deformity.

Modified luque instrumentation after myelomeningocele kyphectomy.

Treatment of congenital kyphosis in myelomeningocele is a difficult problem. Current thinking supports kyphectomy and postoperative internal fixation. Since 1989, vertebral resection with modified Luque fixation has been the procedure of choice for correction of myelomeningocele kyphotic deformity at the author's institution. The study objective was to evaluate long-term results with this technique. Most investigators agree that kyphotic deformity in myelomeningocele should be treated with vertebral resection. There is less uniform consensus about postoperative fixation. Reports in the literature support fixation with modified segmental instrumentation. Sixteen patients, observed for an average of 57.2 months (range, 36-94 months), underwent vertebral resection from the proximal aspect of the apical vertebra cephalad into the compensatory lordotic curve. Fixation was segmental instrumentation wired to the thoracic spine and anterior to the sacrum. The average blood loss was 1121 mL (range, 450-2580 mL). Kyphotic deformity averaged 111 degrees before surgery (range, 75-157 degrees), 15 degrees after surgery (range, -18-36 degrees) and 20 degrees at latest follow-up (range, -17-83 degrees). Loss of correction was 6 degrees (range, 0-27 degrees). Postoperative immobilization was with a thoracolumbosacral orthosis for 18 months. Complications occurring in 8 of the 16 patients were transient headache, superficial wound breakdown, supracondylar femur fractures, and one late infection secondary to skin breakdown that necessitated early rod removal, resulting in some loss of correction. Kyphectomy is an excellent method of correcting rigid kyphotic deformity in the patient with myelodysplasia. Segmental spinal instrumentation provided three distinct advantages: rigidity of the construct, greater correction of the deformity and low-profile instrumentation.

Development of pathological lumbar kyphosis in myelomeningocele.

We analysed the cases of lumbar kyphosis in 151 (21%) of a series of 719 patients with myelomeningocele. Three different types were distinguished: paralytic, sharp-angled and congenital. In a cross-sectional and partly longitudinal study the size and magnitude of the kyphosis, the apex of the curve and the level of paralysis of each group were recorded and statistically analysed. Paralytic kyphosis (less than 90 degrees at birth) occurred in 44.4% and increased linearly during further development. Sharp-angled kyphosis (90 degrees or more at birth) was present in 38.4% and also showed a linear progression. In both types, progression seemed to depend also on the level of paralysis. Congenital kyphosis occurred in 13.9% and we could find no significant factor which correlated with progression.

Surgical Correction of Congenital Kyphosis in Myelomeningocele

A progressive kyphosis in myelomeningocele patients may occur when the lesion is in the lower thoracic spine (i.e., a high-level lesion). Previously described techniques for this difficult problem have been fraught with problems of loss of fixation, recurrent deformity, implant failure, and wound breakdown. The technique described involves resection of the apical vertebra and proximal portion of the gibbus followed by fixation with a single U-shaped rod implanted distally into the lumbosacral spine. The rod is fixed to the thoracic spine using the Luque technique. No wires are used distally and the sacroiliac joints are not violated. This paper presents the first four patients who have undergone this form of spinal surgery. To date these patients have maintained a satisfactory result, and there has been no implant failure. Although this is a small series and with a short follow-up, the results support the use of this technique.

Surgical Correction of Congenital Kyphosis in Myelomeningocele

Surgical Correction of Congenital Kyphosis in Myelomeningocele

The Natural History of Congenital Kyphosis in Myelomeningocele

The progression of congenital lumbar kyphosis in myelomeningocele is a well-known problem, but rates of progression are not well documented in the literature. Fifty-one children with congenital kyphosis and myelomeningocele were followed for an average of 4.8 years. Minimum follow-up was 1 year. Group I (35 patients) had initial radiographs at 1 year of age or less. Group II (16 patients) had radiographs taken after the age of 1 year. Curves less than or equal to 90 degrees in Group I progressed 7.7 degrees/yr; those greater than 90 degrees progressed 12.1 degrees/yr. Curves less than or equal to 90 degrees and greater than 90 degrees progressed at similar rates, regardless of initial curve magnitude: 6.4 degrees/yr and 6.7 degrees/yr, respectively. No correlation existed among the rate of curve progression, the frequency of shunt revisions, or the presence of vertebral anomalies, aside from the dysraphism.

Management of myelomeningocele kyphosis in the older child by kyphectomy and segmental spinal instrumentation.

Kyphosis in the myelomeningocele patient population causes significant problems such as skin breakdown, recurrent infection, and decreased function. Kyphectomy has proved to be a satisfactory means of correcting the deformity, but postoperative stabilization to prevent recurrence of deformity continues to be a problem. Twelve patients with myelomeningocele kyphosis, measuring an average of 124 degrees, were managed by posterior kyphectomy and segmental spinal instrumentation. After operation, the curves measured an average of 32.8 degrees. With a follow-up period, 6-57 months, only one patient lost correction. The loss of correction was secondary to rod failure. Straight midline incision, ignoring previous incisions, can be used to approach the spine without risk of increased wound complications. Adequate immediate stable correction can be achieved by kyphectomy and segmental spinal instrumentation with anterior fixation to the pelvis that thus allows the patient to proceed more quickly to an improved functional level.

Congenital Kyphosis in Myelomeningocele

The deforming mechanisms in the congenital lumbar kyphosis of myelomeningocele are situated anteriorly. Posterior wedge resection with local fusion therefore will not prevent progression of the deformity. Nonetheless, this procedure may be the only method of affording sac closure, or providing continuity of the ulcerated integument. Definitive corrective surgery will be required later and must combat the anterior deforming forces. Resection of the apical vertebral body followed by posterior fusion with Harrington instrumentation provides a solid straight spine.

The Management of Kyphosis in Patients with Myelomeningocele

Kyphosis in myelomeningocele is a progressive condition, which can lead to severe deterioration of function and skin ulceration over the apex of the kyphus. Since bracing is impractical, surgical correction and stabilization is the recommended method of management. Three different techniques are in current use. An anterior procedure using a plate and u-bolt shackles, an anterior approach using a bone strut, and a posterior approach excising a wedge, and the spinal cord if it is functionless. We recommend the wedge excision combined with posterior Harrington compression instrumentation and spinal fusion extending well above and below the resection. Regardless of the approach used, it is the long spinal fusion both anterior and posterior, which will maintain correction.

Spine ostectomy for kyphosis in myelomeningocele.

Spine ostectomy for kyphosis in myelomeningocele.