Thoracolumbar kyphosis in patients with ...



Thoracolumbar kyphosis in patients with mucopolysaccharidoses: clinical outcomes and predictive radiographic factors for progression of deformitySimon B. Roberts, MSc, MRCS(Ed);Ross Dryden, Medical Student;Athanasios I. Tsirikos, MD, FRCS, PhD.Study conducted at the Scottish National Spine Deformity Centre, Royal Hospital for Sick Children, Edinburgh, United Kingdom.Address all correspondence to:Athanasios I. Tsirikos, MD, FRCS, PhDConsultant Orthopaedic and Spine Surgeon,Honorary Clinical Senior Lecturer University of Edinburgh,Scottish National Spine Deformity Centre,Sciennes Road, Edinburgh, EH9 1LF, United Kingdom.Email: atsirikos@, Tel. number: 0044-131-662-1265, Fax number: 0044-131-536-0924.Conflict of Interest Statement: No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.Word Count: 4351 (abstract and text)AbstractClinical and radiological data were reviewed for all mucopolysaccharidoses (MPS) patients with thoracolumbar kyphosis managed non-operatively or operatively in our institution. 16 patients were included (8 female-8 male). Nine patients had Hurler, 5 Morquio and 2 Hunter syndrome. Six patients were treated non-operatively (mean age at presentation 6.3 years; kyphosis progression +1.5o/year; follow-up 3.1 years) and 10 patients operatively (mean age at presentation 4.7 years; kyphosis progression +10.8o/year; follow-up 8.2 years) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities.In the surgical group (mean age at surgery 6.6 years; mean post-operative follow-up 6.3 years), mean preoperative thoracolumbar kyphosis of +74.3o was corrected to mean +28.6o post-operatively, relating to a mean deformity correction of 66.9%. Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis >+38o at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity.In conclusion, severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise.IntroductionMucopolysaccharidoses (MPS) are a group of rare genetic disorders characterised by deficiency in enzymes required for glycosaminoglycan catabolism. Accumulation of glycosaminoglycans leads to progressive cellular damage, multiple organ failure, and reduced life expectancy. Musculoskeletal manifestations are a prominent early feature of MPS. In particular, thoracolumbar kyphosis develops in up to 80% of MPS patients.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj52YW4gZGVyIExpbmRlbjwvQXV0aG9yPjxZZWFyPjIwMTE8

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ADDIN EN.CITE.DATA 1 The advent of haematopoetic stem cell transplantation and enzyme replacement therapy has significantly improved morbidity and mortality in MPS, with improvement of most antecedent organ damage.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5Tb3VpbGxldDwvQXV0aG9yPjxZZWFyPjIwMDM8L1llYXI+

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ADDIN EN.CITE.DATA 9-12 Following medical treatment, MPS patients are, therefore, increasingly developing progressive and severe thoracolumbar kyphosis requiring surgical intervention.There is limited evidence guiding the strategy for surgical stabilisation of thoracolumbar kyphosis in MPS. In 2004, Dalvie et al. ADDIN EN.CITE <EndNote><Cite><Author>Dalvie</Author><Year>2001</Year><RecNum>581</RecNum><DisplayText><style face="superscript">13</style></DisplayText><record><rec-number>581</rec-number><foreign-keys><key app="EN" db-id="a9zfesefqp9zpxeft5qp9rdbat9aa9zfsxts">581</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Dalvie, S. S.</author><author>Noordeen, M. H.</author><author>Vellodi, A.</author></authors></contributors><auth-address>Great Ormond Street Hospital for Children, London, United Kingdom.</auth-address><titles><title>Anterior instrumented fusion for thoracolumbar kyphosis in mucopolysaccharidosis</title><secondary-title>Spine (Phila Pa 1976)</secondary-title><alt-title>Spine</alt-title></titles><periodical><full-title>Spine (Phila Pa 1976)</full-title><abbr-1>Spine</abbr-1></periodical><alt-periodical><full-title>Spine (Phila Pa 1976)</full-title><abbr-1>Spine</abbr-1></alt-periodical><pages>E539-41</pages><volume>26</volume><number>23</number><keywords><keyword>Adolescent</keyword><keyword>Child</keyword><keyword>Child, Preschool</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Kyphosis/*etiology/*surgery</keyword><keyword>Lumbar Vertebrae/*surgery</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidoses/*complications</keyword><keyword>*Orthopedic Fixation Devices</keyword><keyword>Retrospective Studies</keyword><keyword>*Spinal Fusion</keyword><keyword>Thoracic Vertebrae/*surgery</keyword><keyword>Treatment Outcome</keyword></keywords><dates><year>2001</year><pub-dates><date>Dec 1</date></pub-dates></dates><isbn>0362-2436 (Print)&#xD;0362-2436 (Linking)</isbn><accession-num>11725253</accession-num><urls><related-urls><url> reported the first series of MPS patients with thoracolumbar kyphosis treated by anterior instrumented fusion with good correction and no complications. Yasin et al.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5ZYXNpbjwvQXV0aG9yPjxZZWFyPjIwMTQ8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 6 described good correction of kyphosis following excision of the apical vertebra and adjacent discs with anterior autologous bone grafting and posterior instrumented fusion extending 3 levels proximal and distal to the apical vertebra. Genevois et al.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5BYmVsaW4gR2VuZXZvaXM8L0F1dGhvcj48WWVhcj4yMDE0

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ADDIN EN.CITE.DATA 14 recorded successful fusion and correction of thoracolumbar kyphosis in MPS patients managed by anterior corpectomies and discectomies, anterior reconstruction with autologous tibial strut graft, and short posterior instrumented fusion. The optimal surgical strategy to achieve acceptable correction and stabilisation of thoracolumbar deformity in MPS patients remains undetermined.The principal indications for surgical treatment of thoracolumbar kyphosis in MPS patients include a progressively severe deformity and impending or evident neurological dysfunction. In 2014, Yasin et al.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5ZYXNpbjwvQXV0aG9yPjxZZWFyPjIwMTQ8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 6 suggested that a kyphosis angle greater than +45o at initial presentation could predict subsequent progression of deformity greater than 10o. It is unclear whether other patient factors such as age at diagnosis or commencement of haematopoetic stem cell transplantation or enzyme replacement therapy also affect subsequent progression of thoracolumbar kyphosis.The aims of our study were: 1) to investigate the efficacy of combined antero-posterior fusion with or without posterior segmental pedicle screw instrumentation in the treatment of progressively severe thoracolumbar kyphosis in MPS; 2) to investigate patient factors predictive of kyphosis progression and the development of a severe thoracolumbar deformity.Patients and MethodsWe retrospectively reviewed prospectively-collected data of all patients with MPS presenting with thoracolumbar kyphosis between 2001 and 2013 in our centre. All patients met the following inclusion criteria: biochemically-confirmed diagnosis of MPS; patient information comprising demographics, clinical manifestations of MPS, and haematopoetic stem cell transplantation or enzyme replacement therapy; radiographic data available from initial assessment to final follow-up; and operative details including pre- and post-operative radiographic parameters, and peri-operative complications.The study cohort comprised 16 patients (8 female-8 male) including 9 patients with Hurler (type I MPS), 5 with Morquio (type IV MPS), and 2 with Hunter syndrome (type II MPS). Mean follow-up was 6.2 years (range: 1 to 11.8), at which stage 6 patients (38%) were skeletally mature. Our patients were divided into 2 groups: patients managed non-operatively throughout available follow-up (n = 6), and those treated by surgical stabilisation of spinal deformity (n = 10). The demographic characteristics, extra-spinal manifestations, and spinal features of all patients are shown in Tables 1 & 2. Radiological parameters of thoracolumbar kyphosis and associated scoliosis were measured using the Cobb method ADDIN EN.CITE <EndNote><Cite><Author>Cobb</Author><Year>1948</Year><RecNum>495</RecNum><DisplayText><style face="superscript">15</style></DisplayText><record><rec-number>495</rec-number><foreign-keys><key app="EN" db-id="a9zfesefqp9zpxeft5qp9rdbat9aa9zfsxts">495</key></foreign-keys><ref-type name="Book Section">5</ref-type><contributors><authors><author>Cobb, J.R.</author></authors></contributors><titles><title>Outline for the study of scoliosis</title><secondary-title>Instructional course lectures</secondary-title></titles><volume>5</volume><section>American Academy of Orthopaedic Surgeons</section><dates><year>1948</year></dates><publisher>Ann Arbor: JW Edwards</publisher><urls></urls></record></Cite></EndNote>15 on an electronic system by 2 authors (AIT, SBR) based on consensus agreement regarding anatomical landmarks as to the extent of the thoracolumbar deformity. The same landmarks were used for measurements on consecutive radiographs. Thoracic kyphosis was measured between T2 and T12 vertebrae. Deformity correction was determined as follows: Correction Rate (%) = (pre-operative Cobb angle– post-operative Cobb angle) / (pre-operative Cobb angle) x 100. Baseline magnetic resonance imaging of the whole neural axis was performed in all patients to assess for cranio-cervical junction or intra-spinal anomalies. No intraspinal anomalies were detected on radiological assessment. Flexion-extension radiographs were also performed in all patients to assess for atlanto-axial instability. If detected patients were referred to the regional neurosurgical unit for consideration of occipito-cervical fusion prior to surgical stabilisation of thoracolumbar kyphosis.Patients with thoracolumbar kyphosis <+40o and no neurological deficits, or in whom surgery was contraindicated (Tables 2 & 3; patient 3 – surgery contraindicated due to severe cognitive and functional impairment subsequent to perinatal cerebral infarction), were managed with observation. Bracing was not attempted, as this is ineffective to control the deformity and often poorly tolerated in patients with MPS.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5XaGl0ZTwvQXV0aG9yPjxZZWFyPjIwMTE8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 13,16 Indications for surgical stabilisation in our group of patients included the presence of severe, progressive thoracolumbar kyphosis >+40o in order to prevent neurological complications and back pain.Surgical technique. Surgery comprised single-stage circumferential arthrodesis performed through a thoraco-abdominal retroperitoneal and a midline posterior spinal approach. The fibro-cartilaginous extents of the abnormal apical vertebrae were excised and complete discectomies were performed across the levels of the deformity. The apical defect following the corpectomy was bridged with autologous rib strut grafts and morsellised rib graft was placed into the intervening disc spaces. If the thoracolumbar kyphosis remained inflexible after the anterior spinal release upon testing intraoperatively (n = 4; 40% of operated patients), a long rib strut graft was placed anteriorly under compression between the proximal and distal end vertebrae to be included in the fusion to achieve fusion with positional correction [non-vascularised rib strut in 3 patients (Figure 1) - vascularised rib strut in one patient]. Posterior arthrodesis was then performed with autologous and allograft bone across the extent of deformity. Instrumentation was used in patients in whom the thoracolumbar kyphosis became flexible and correctable after the extensive anterior release as assessed intra-operatively (ability to manually correct the kyphosis at completion of anterior discectomies and apical vertebral body resection) (Figure 2; n = 6; 60% of operated patients). The posterior instrumented arthrodesis included the cephalad end vertebra of the kyphosis and caudally the first lordotic lumbar disc to prevent the development of distal junctional deformity. The fusion extended distally to L3 or L4 in order to preserve maximum motion segments as all patients retained walking capacity at the time of surgery. In only one patient (patient 9, Table 4) the lowest vertebra was selected at L5 as revision instrumented arthrodesis was required due to proximal and distal junctional kyphosis developing following previous limited anterior apical fusion performed at another centre. Bilateral paediatric segmental pedicle screw/hook instrumentation was used in all patients undergoing instrumented spinal arthrodesis. Intra-operative spinal cord monitoring was performed recording transcranial motor and cortical/cervical somatosensory evoked potentials, which remained stable throughout in all patients. The postoperative protocol included immediate transfer of all patients to the intensive care unit, weaning from invasive to non-invasive ventilatory support as soon as possible and subsequent transfer to regular ward care. All patients were immobilised in a custom-moulded plaster jacket for 4 months post-operatively until fusion was evident on radiographs, and were permitted to ambulate as able.Statistical analysis. Demographics, chronology of medical therapy, and radiological parameters between non-operated/operated patients and non-instrumented/instrumented fusions were compared using the two-tailed Student’s t-test. Chi-squared test was used to analyse nominal data between groups. Receiver-Operator Characteristics (ROC) analysis was used to evaluate the initial kyphosis severity predictive of subsequent progression of deformity. Statistical significance was set at p<0.05. All statistical analyses were performed using SPSS v 21.0.0 software (SPSS Inc., Chicago, Illinois).ResultsOur cohort comprised 16 MPS patients with thoracolumbar kyphosis and mean age at clinical presentation 5.3 years (range: 0.4 to 14.4 years) of which 6 patients were managed non-operatively and 10 patients were treated surgically. There was no significant difference in the distribution of MPS sub-type between patients treated non-operatively or operatively (X2=2.110, p=0.35). All patients with Hurler syndrome had received haematopoetic stem cell transplantation at mean age 1.2 years (range: 0.6 to 1.6). Patients with Hunter or Morquio syndrome were all treated by enzyme replacement therapy, commencing at mean age 6.9 years (range: 5 to 9.5), except 3 patients for whom enzyme replacement therapy was not available from the regional health boards. Occipito-cervical fusion for atlanto-axial instability was performed in 4 patients (3 Morquio; 1 Hurler) prior to management of thoracolumbar kyphosis.The characteristics of spinal deformity in patients managed non-operatively are shown in Table 3. Mean age at presentation was 6.3 years (range: 0.4 to 13). Mean extent of thoracolumbar kyphosis was 6.2 vertebrae (range: 4 to 9). The thoracolumbar kyphosis progressed from mean +30.5o (range: +8 to +60o) to +35.3o (range: +15 to +75o) over a mean follow-up of 3.1 years (range: 1 to 5.1). The mean rate of kyphosis progression was +1.5o per year. A patient (patient 3, Table 3) with severe thoracolumbar kyphosis and untreated Hunter syndrome continued non-operative management (+60o kyphosis at presentation progressing to +75o at follow-up) as he had suffered a perinatal cerebral infarct with resultant hemiplegia and surgical intervention was contra-indicated following discussion with the patient’s family and the multidisciplinary team. Two patients had an associated thoracic and/or thoracolumbar scoliosis, which was also kept under monitoring (Table 3).The characteristics of spinal deformity and peri-operative complications in patients treated surgically are shown in Table 4. Mean age at initial presentation was 4.7 years (range: 2.4 to 16.8). Mean extent of thoracolumbar kyphosis was 4.3 vertebrae (range: 3 to 6). Mean thoracolumbar kyphosis at presentation was +54.6o (range: +36 to +95o), progressing to mean +74.3o (range: +42 to +110o) prior to surgical stabilisation over a mean 1.9 years pre-operative follow-up (range: 1.1 to 3.2). The mean rate of kyphosis progression was +10.8o per year prior to surgical treatment. The mean age at surgery was 6.6 years (range 2.4 to 16.8) and mean post-operative follow-up was 6.3 years (range: 3.5 to 10.3). The mean post-operative thoracolumbar kyphosis was +28.6o (range: 0 to +65o), corresponding to a mean correction of 66.9% (range: 31 to 100%). Patients treated by un-instrumented arthrodesis (Figure 1) had a greater mean pre-operative kyphosis [+92.3o (range: +55 to +110o) compared to +62.3o (range: +37 to +78o); p=0.04], and less absolute [+39o (range: +27 to +55o) compared to +50.2o (range: +37 to +64o); p=0.09] and percentage correction of kyphosis [42.7% (range: 30.9 to 50%) compared to 83.1% (range: 55.1 to 100%); p=0.002], compared to patients who underwent an instrumented arthrodesis (Figure 2). There was no significant loss of kyphosis correction between immediate post-operative and latest follow-up radiographs for all patients. Thoracic kyphosis improved from mean -0.7o (range: -50 to +14o) pre-operatively to mean +20.7o (range: -15 to +37o) postoperatively. Two patients had a thoracolumbar and 2 a thoracic scoliosis in association with the thoracolumbar kyphosis. The thoracolumbar scoliosis was corrected from mean +35.5o (range: +26 to +45o) pre-operatively to mean +7.5o (range: 0 to +15o) post-operatively; the thoracic scoliosis improved spontaneously from mean +29o (range: +25 to +33o) pre-operatively to mean +17o (range: +14 to +20o) post-operatively following correction of the thoracolumbar kyphosis.Prior to spinal surgery, 2 patients required establishment of an elective tracheotomy. Mean surgical time was 170 minutes (range: 150 to 240), and mean intra-operative blood loss was 387 mls (range: 200 to 1000). Three patients developed surgical complications in our cohort. One patient developed an early deep wound infection treated with surgical debridement and intravenous antibiotics, permitting retention of instrumentation with no subsequent loss of deformity correction. The same patient also developed proximal junctional kyphosis which remained stable at follow-up and did not require extension of the fusion. Another patient who underwent an un-instrumented fusion developed non-union at the apex of the kyphosis posteriorly treated with posterior re-grafting using allograft bone followed by application of a custom-moulded plaster jacket for 3 months. The only patient with Hunter syndrome treated operatively developed a stable grade II spondylolisthesis at the adjacent segment distal to the instrumentation; as the patient remained asymptomatic, this was managed conservatively to skeletal maturity and the spondylolisthesis showed no evidence of deterioration. Post-operative medical complications included chest infection in 3 patients, prolonged ileus and need for tracheotomy for prolonged mechanical ventilation in one patient each. Mean hospital stay was 31 days (range: 10 to 142). All patients had been ambulant pre-operatively and there was no alteration in ambulatory status post-operatively and at follow-up. Mean post-operative follow-up was 6.3 years (range: 3.5 to 10.3), at the conclusion of which the mean age of operated patients was 14.5 years (range: 7 to 24). The demographics, surgical techniques, radiographic outcomes and peri-operative complications in our cohort of MPS patients with thoracolumbar deformity are compared with previously published series in Table 5. Variables at initial presentation that may predict progression to severe thoracolumbar kyphosis and need for surgical stabilisation were investigated. Significant association was found for neither age at presentation (p=0.836) nor age at commencement of haematopoetic stem cell transplantation or enzyme replacement therapy (p=0.513). The size of thoracolumbar kyphosis at initial clinical presentation was associated with progression to severe deformity and need for surgical treatment (p<0.001). ROC analysis demonstrated that an initial thoracolumbar kyphosis >+38o predicts progression to severe deformity and need for surgical correction with 90% sensitivity and 83% specificity (AUC = 0.892).DiscussionWe believe this is one of the largest reported series reviewing the outcomes of treatment for thoracolumbar kyphosis in patients with MPS and contains the longest reported follow-up after non-operative management or single-stage circumferential arthrodesis, with 38% of the study cohort reaching skeletal maturity. Consensus treatment recommendations have not yet emerged due to the limited reports of both the natural history and management of thoracolumbar kyphosis in patients with MPS. In our series, there was similar distribution of patients with each MPS subtype (I, II, and IV) in the non-operative and operative groups. 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ADDIN EN.CITE.DATA 6,17The surgical techniques described for correction of thoracolumbar deformity in patients with MPS include anterior instrumented fusion, anterior fusion using vascularised rib graft, and combined anterior/posterior instrumented fusion.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5EYWx2aWU8L0F1dGhvcj48WWVhcj4yMDAxPC9ZZWFyPjxS

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ADDIN EN.CITE.DATA 6,13,14 Dalvie et al. ADDIN EN.CITE <EndNote><Cite><Author>Dalvie</Author><Year>2001</Year><RecNum>581</RecNum><DisplayText><style face="superscript">13</style></DisplayText><record><rec-number>581</rec-number><foreign-keys><key app="EN" db-id="a9zfesefqp9zpxeft5qp9rdbat9aa9zfsxts">581</key></foreign-keys><ref-type name="Journal Article">17</ref-type><contributors><authors><author>Dalvie, S. S.</author><author>Noordeen, M. H.</author><author>Vellodi, A.</author></authors></contributors><auth-address>Great Ormond Street Hospital for Children, London, United Kingdom.</auth-address><titles><title>Anterior instrumented fusion for thoracolumbar kyphosis in mucopolysaccharidosis</title><secondary-title>Spine (Phila Pa 1976)</secondary-title><alt-title>Spine</alt-title></titles><periodical><full-title>Spine (Phila Pa 1976)</full-title><abbr-1>Spine</abbr-1></periodical><alt-periodical><full-title>Spine (Phila Pa 1976)</full-title><abbr-1>Spine</abbr-1></alt-periodical><pages>E539-41</pages><volume>26</volume><number>23</number><keywords><keyword>Adolescent</keyword><keyword>Child</keyword><keyword>Child, Preschool</keyword><keyword>Female</keyword><keyword>Humans</keyword><keyword>Kyphosis/*etiology/*surgery</keyword><keyword>Lumbar Vertebrae/*surgery</keyword><keyword>Male</keyword><keyword>Mucopolysaccharidoses/*complications</keyword><keyword>*Orthopedic Fixation Devices</keyword><keyword>Retrospective Studies</keyword><keyword>*Spinal Fusion</keyword><keyword>Thoracic Vertebrae/*surgery</keyword><keyword>Treatment Outcome</keyword></keywords><dates><year>2001</year><pub-dates><date>Dec 1</date></pub-dates></dates><isbn>0362-2436 (Print)&#xD;0362-2436 (Linking)</isbn><accession-num>11725253</accession-num><urls><related-urls><url> described good correction of kyphosis in MPS patients treated by anterior-only instrumented fusion, though this series included predominantly patients with Morquio syndrome and less severe kyphosis. Although no complications were described, the duration of post-operative follow-up was limited. Anterior fusion using vascularised rib graft has also been described for MPS patients with thoracolumbar kyphosis but is associated with a high rate of post-operative complications and progression of deformity.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5ZYXNpbjwvQXV0aG9yPjxZZWFyPjIwMTQ8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 6 This was also the experience in our study in which a single patient was managed by anterior reconstruction using a vascularised rib graft; the patient required prolonged post-operative ventilatory support and a tracheotomy, underwent re-grafting surgery to address an apical non-union, and developed long-term chest wall disruption across the harvesting site. In contrast, non-vascularised rib grafting permits placement of a strut across the deformity, while the defect at the donor rib site gradually heals as the vascular supply across the rib bed is preserved. This produces, in our experience, much less disruption of the chest wall when compared to a vascularised rib which creates a permanent thoracic defect across the harvesting site. The disadvantage of a non-vascularised rib graft is that it takes longer to consolidate to the vertebral bodies and therefore postoperative support in the form of a custom-moulded spinal jacket is recommended. Posterior-only fusion is also associated with a higher risk of pseudoarthrosis. By comparison with the management of other skeletal dysplasias, surgical correction of developmental kyphosis secondary to failure of anterior vertebral body formation may be optimally achieved by anterior spinal release with strut graft correction and circumferential arthrodesis with the use of posterior instrumentation.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5NY01hc3RlcjwvQXV0aG9yPjxZZWFyPjIwMDE8L1llYXI+

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ADDIN EN.CITE.DATA 14 Thus, in our series of MPS patients, the preferred surgical technique was to perform a circumferential arthrodesis supported by either an anterior strut graft in patients with rigid thoracolumbar kyphosis or posterior instrumentation in patients in whom thoracolumbar deformity became almost fully correctable after the anterior release. Deformities in which the thoracolumbar kyphosis remained inflexible after the extensive anterior release were supported by an additional long anterior rib strut graft between the proximal and distal end vertebrae of the fusion. This permitted positional correction with the strut graft keyed into the vertebrae under compression in order to promote fusion. Posterior instrumentation was not used in this group of patients as this would not have increased the ability to significantly correct the rigid kyphosis. Posterior instrumentation in this case would have also been at a high risk of pull-out failure and become prominent under the skin in the presence of a severe residual sagittal deformity. In contrast, if the thoracolumbar kyphosis became flexible after the anterior release posterior instrumentation was used in order to maximally correct the deformity and maintain stability of the thoracolumbar junction to enhance a circumferential fusion. We included the proximal end vertebra of the kyphosis, as well as the first lordotic disc distally within the area of the fusion in order to reduce the risk of junctional add-on deformity and preserve growth of the spine by limiting the fusion segments. We performed preoperative whole spinal MRI and CT across the levels of the deformity in all patients but no preoperative bone mineral density scans. In the presence of very small pedicles, thoracic pedicle hooks instead of screws were placed at the proximal end of the construct to resist pull-out failure and supplement the pedicle screws at adjacent levels.There is no consensus regarding the relative timing of reconstructive hip surgery and spinal stabilisation in patients with MPS. Pain and arthrosis associated with hip dysplasia, as well as deformity and risk of neurological compromise associated with progressive thoracolumbar kyphosis must be assessed in all patients and a decision on relative timing of interventions made dependent on the immediacy of each on an individual patient basis.In our cohort of patients, the thoracolumbar kyphosis was managed by combined antero-posterior spinal arthrodesis at a similar mean age at surgery but with a greater preoperative sagittal deformity when compared to previously reported MPS cohorts (Table 5). Differences between series in the mean pre-operative kyphotic angle and age at time of surgery are likely due to variation in the proportion of MPS subtypes, indications for surgery, and whether or not other interventions, such as reconstructive hip surgery, were scheduled before or after spinal surgery.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5BYmVsaW4gR2VuZXZvaXM8L0F1dGhvcj48WWVhcj4yMDE0

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ADDIN EN.CITE.DATA 14 Despite our patients developing a greater kyphosis pre-operatively, we achieved a mean kyphosis correction of 66.9%, which is comparable to the 60 to 70% correction reported in previous series. As can be expected, patients whose thoracolumbar deformity became flexible after completion of the anterior stage of the procedure and therefore underwent instrumented arthrodesis posteriorly had a less severe kyphotic deformity pre-operatively, and achieved greater kyphosis correction, than patients in whom the deformity was rigid who underwent an un-instrumented circumferential arthrodesis. In our cohort, no patient had failure of instrumentation and correction of kyphosis was maintained at follow-up in all patients. Circumferential arthrodesis also achieved improvement in thoracic kyphosis and associated thoracic or thoracolumbar scoliosis.Despite good correction of thoracolumbar deformity in our cohort, a few patients developed post-operative complications. Significant medical and anaesthetic risks are associated with spinal surgery in patients with MPS.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5BYmVsaW4gR2VuZXZvaXM8L0F1dGhvcj48WWVhcj4yMDE0

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ADDIN EN.CITE.DATA 14 In our cohort, respiratory complications were frequent with 2 patients requiring a pre-operative tracheotomy, and 3 patients developing post-operative pulmonary infections. This was reflected in the mean duration of hospital stay of 31 days in our cohort. One patient developed a deep wound infection that resolved with early management and had no effect on maintenance of deformity correction. One patient who underwent an un-instrumented arthrodesis developed non-union at the apex of the deformity posteriorly. Fusion was achieved by a revision re-grafting procedure. The only other complication was the development of grade II spondylolisthesis at the distal adjacent segment in a patient with Hunter syndrome. This was reported as a relatively frequent complication by Genevois et al.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5BYmVsaW4gR2VuZXZvaXM8L0F1dGhvcj48WWVhcj4yMDE0

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ADDIN EN.CITE.DATA 14 (3 of 13 patients; 23%), with only one patient requiring revision surgery. The patient in our series was kept under close monitoring to skeletal maturity with no deterioration in spondylolisthesis or neurological function. Interestingly, mild proximal adjacent segment spondylolisthesis occurred in 50% of patients in a previous series, but did not require revision surgery.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5BYmVsaW4gR2VuZXZvaXM8L0F1dGhvcj48WWVhcj4yMDE0

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ADDIN EN.CITE.DATA 14 These complications highlight the risk of developing wound healing problems and segmental instability producing spondylolisthesis adjacent to the extents of fusions in patients with MPS. MPS patients may be particularly susceptible to such complications as they frequently develop a severe flexed posture with marked positive sagittal imbalance and fixed hip flexion deformity, as well as due to the underlying connective tissue disorder and inherent immunological compromise.Significant variation in the rate of progression of thoracolumbar kyphosis in patients with MPS has previously been reported.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5ZYXNpbjwvQXV0aG9yPjxZZWFyPjIwMTQ8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 6 We found that only the magnitude of kyphosis at initial presentation was significantly associated with progression to severe deformity and need for surgical treatment. We identified that initial thoracolumbar kyphosis >+38o predicted deformity progression and need for surgical intervention with high sensitivity and specificity. Our finding is similar to that of Yasin et al.PEVuZE5vdGU+PENpdGU+PEF1dGhvcj5ZYXNpbjwvQXV0aG9yPjxZZWFyPjIwMTQ8L1llYXI+PFJl

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ADDIN EN.CITE.DATA 6 who found an initial kyphosis angle of +45o to be predictive of a progressive deformity. Differences in the subtypes of MPS and proportion of non-operative and operative patients included within each series may account for the small variability in predictive thresholds. Additional factors are likely to contribute to the natural history of thoracolumbar kyphosis progression and future investigation in larger surgical MPS cohorts will permit more accurate prediction of deformity progression and prophylactic intervention. Our results indicate that patients presenting with a thoracolumbar kyphosis >+38o should be considered for surgical stabilisation, in addition to patients presenting with a progressive deformity.Our study has limitations. It is a retrospective analysis of clinical and radiological data. Although the follow-up of patients managed non-operatively and operatively was longer in our cohort, only 38% (n = 6) of our patients had reached skeletal maturity at the available final follow-up. Further studies following patients through to skeletal maturity will allow a more complete understanding of the outcomes of non-operative and surgical interventions for thoracolumbar kyphosis in MPS. Our study reported clinical and radiological outcomes, but patient function and carer/relative satisfaction were not directly assessed. Further studies to develop and validate functional outcome scores and to assess satisfaction using quality of life scores specific to MPS may be beneficial adjuncts in assessing treatments for spinal deformity. Finally, MPS are a heterogeneous group of disorders and larger cohorts examining outcomes of treatment for spinal deformity according to subtype will be useful to further optimise indications, timing and strategies for surgical intervention.In conclusion, thoracolumbar kyphosis in patients with MPS may follow a variable course. Patients with MPS have significant medical co-morbidities and anaesthetic risks. Successful surgery depends on applying the optimum procedure at the most appropriate time. Progressive thoracolumbar kyphosis and the need for surgical treatment is best predicted by an initial kyphosis angle >+38o. Patients that develop progressively severe thoracolumbar kyphosis can be adequately treated by combined antero-posterior arthrodesis, with the use of a supportive long anterior rib strut graft in patients whose deformity remains rigid after anterior release, and the use of posterior instrumentation in patients in whom the deformity becomes flexible allowing for maximum kyphosis correction. This is an effective strategy for treating severe thoracolumbar kyphosis in patients with mucopolysaccharidoses. In our series, circumferential spinal arthrodesis resulted in satisfactory deformity correction, which was maintained at follow-up. Patients with MPS may be particularly susceptible to post-operative complications including wound infections and adjacent segment instability due to the underlying connective tissue disorder and immunological compromise, as well as the frequently pre-existing marked positive global sagittal imbalance.AcknowledgementThe authors would like to acknowledge Mr Christopher I. Adams, Consultant Orthopaedic and Spine Surgeon in our Unit who contributed one surgical patient in the current series.References ADDIN EN.REFLIST 1. van der Linden MH, Kruyt MC, Sakkers RJ, de Koning TJ, Oner FC, Castelein RM. Orthopaedic management of Hurler's disease after hematopoietic stem cell transplantation: a systematic review. J Inherit Metab Dis 2011;34-3:657-69.2. Souillet G, Guffon N, Maire I, Pujol M, Taylor P, Sevin F, Bleyzac N, Mulier C, Durin A, Kebaili K, Galambrun C, Bertrand Y, Froissart R, Dorche C, Gebuhrer L, Garin C, Berard J, Guibaud P. Outcome of 27 patients with Hurler's syndrome transplanted from either related or unrelated haematopoietic stem cell sources. Bone Marrow Transplant 2003;31-12:1105-17.3. Clarke LA, Wraith JE, Beck M, Kolodny EH, Pastores GM, Muenzer J, Rapoport DM, Berger KI, Sidman M, Kakkis ED, Cox GF. Long-term efficacy and safety of laronidase in the treatment of mucopolysaccharidosis I. Pediatrics 2009;123-1:229-40.4. Muenzer J, Beck M, Eng CM, Giugliani R, Harmatz P, Martin R, Ramaswami U, Vellodi A, Wraith JE, Cleary M, Gucsavas-Calikoglu M, Puga AC, Shinawi M, Ulbrich B, Vijayaraghavan S, Wendt S, Conway AM, Rossi A, Whiteman DA, Kimura A. Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome. Genet Med 2011;13-2:95-101.5. Weisstein JS, Delgado E, Steinbach LS, Hart K, Packman S. Musculoskeletal manifestations of Hurler syndrome: long-term follow-up after bone marrow transplantation. J Pediatr Orthop 2004;24-1:97-101.6. Yasin MN, Sacho R, Oxborrow NJ, Wraith JE, Williamson JB, Siddique I. Thoracolumbar kyphosis in treated mucopolysaccharidosis 1 (Hurler syndrome). Spine (Phila Pa 1976) 2014;39-5:381-7.7. Heppner JM, Zaucke F, Clarke LA. Extracellular matrix disruption is an early event in the pathogenesis of skeletal disease in mucopolysaccharidosis I. Mol Genet Metab 2015;114-2:146-55.8. Xing EM, Knox VW, O'Donnell PA, Sikura T, Liu Y, Wu S, Casal ML, Haskins ME, Ponder KP. The effect of neonatal gene therapy on skeletal manifestations in mucopolysaccharidosis VII dogs after a decade. Mol Genet Metab 2013;109-2:183-93.9. Tomatsu S, Almeciga-Diaz CJ, Montano AM, Yabe H, Tanaka A, Dung VC, Giugliani R, Kubaski F, Mason RW, Yasuda E, Sawamoto K, Mackenzie W, Suzuki Y, Orii KE, Barrera LA, Sly WS, Orii T. Therapies for the bone in mucopolysaccharidoses. Mol Genet Metab 2015;114-2:94-109.10. Tomatsu S, Sawamoto K, Almeciga-Diaz CJ, Shimada T, Bober MB, Chinen Y, Yabe H, Montano AM, Giugliani R, Kubaski F, Yasuda E, Rodriguez-Lopez A, Espejo-Mojica AJ, Sanchez OF, Mason RW, Barrera LA, Mackenzie WG, Orii T. Impact of enzyme replacement therapy and hematopoietic stem cell transplantation in patients with Morquio A syndrome. Drug Des Devel Ther 2015;9:1937-53.11. Pastores GM, Meere PA. Musculoskeletal complications associated with lysosomal storage disorders: Gaucher disease and Hurler-Scheie syndrome (mucopolysaccharidosis type I). Curr Opin Rheumatol 2005;17-1:70-8.12. Dornelles AD, de Camargo Pinto LL, de Paula AC, Steiner CE, Lourenco CM, Kim CA, Horovitz DD, Ribeiro EM, Valadares ER, Goulart I, Neves de Souza IC, da Costa Neri JI, Santana-da-Silva LC, Silva LR, Ribeiro M, de Oliveira Sobrinho RP, Giugliani R, Schwartz IV. Enzyme replacement therapy for Mucopolysaccharidosis Type I among patients followed within the MPS Brazil Network. Genet Mol Biol 2014;37-1:23-9.13. Dalvie SS, Noordeen MH, Vellodi A. Anterior instrumented fusion for thoracolumbar kyphosis in mucopolysaccharidosis. Spine (Phila Pa 1976) 2001;26-23:E539-41.14. Abelin Genevois K, Garin C, Solla F, Guffon N, Kohler R. Surgical management of thoracolumbar kyphosis in mucopolysaccharidosis type 1 in a reference center. J Inherit Metab Dis 2014;37-1:69-78.15. Cobb JR. Outline for the study of scoliosis. In: Instructional course lectures. Vol. 5: Ann Arbor: JW Edwards, 1948.16. White KK. Orthopaedic aspects of mucopolysaccharidoses. Rheumatology (Oxford) 2011;50 Suppl 5:v26-33.17. Field RE, Buchanan JA, Copplemans MG, Aichroth PM. Bone-marrow transplantation in Hurler's syndrome. Effect on skeletal development. J Bone Joint Surg Br 1994;76-6:975-81.18. McMaster MJ, Singh H. The surgical management of congenital kyphosis and kyphoscoliosis. Spine (Phila Pa 1976) 2001;26-19:2146-54; discussion 55.19. Ain MC, Browne JA. Spinal arthrodesis with instrumentation for thoracolumbar kyphosis in pediatric achondroplasia. Spine 2004;29-18:2075-80.20. Auregan JC, Odent T, Coyle RM, Miladi L, Wicart P, Dubousset J, Le Merrer M, Padovani JP, Glorion C. Ischiovertebral dysplasia: a retrospective analysis of 30 consecutive cases pointing out the specifics and risks of the spine management. Spine (Phila Pa 1976) 2014;39-9:E564-75.21. Mason DE, Sanders JO, MacKenzie WG, Nakata Y, Winter R. Spinal deformity in chondrodysplasia punctata. Spine (Phila Pa 1976) 2002;27-18:1995-2002.22. Saraph VJ, Bach CM, Krismer M, Wimmer C. Evaluation of spinal fusion using autologous anterior strut grafts and posterior instrumentation for thoracic/thoracolumbar kyphosis. Spine (Phila Pa 1976) 2005;30-14:1594-601.23. Kim YJ, Otsuka NY, Flynn JM, Hall JE, Emans JB, Hresko MT. Surgical treatment of congenital kyphosis. Spine (Phila Pa 1976) 2001;26-20:2251-7.Table 1. Demographics, extra-spinal features and previous operations in our MPS patients.Patient NoMPS TypeAge at Presentation (years)GenderMedical TherapyAge BMT/ERT commencedExtraspinal Features and Previous Operations1Hurler1.08FemaleBMT1.42Macrocephaly, mild coarctation of aorta, hypoplastic and lateralised hips2Hurler0.42FemaleBMT0.66Hearing impairment, obstructive sleep apnoea, inguinal hernia repair, joint contractures3Hunter13MaleNoneN/APerinatal cerebral infarct causing hemiplegia, learning difficulties, hearing impairment, mitral regurgitation, sleep apnoea, umbilical hernia repair4Morquio12.91MaleNoneN/AHigh tetraplegia following previous occipito-cervical fusion5Morquio3.91FemaleERT5None6Morquio6.5MaleERT9.5Hemi-epiphysiodesis for genu valgum7Hurler4.83MaleBMT0.58Sleep apnoea, inguinal hernia repair, bilateral carpal tunnel release8Hurler5.66MaleBMT1Learning difficulties, mitral regurgitation, bilateral carpal tunnel decompression, bilateral hernia repair, bilateral genu valgum, bilateral congenital talipes equinovarus9Hurler14.42MaleBMT1.6Mitral regurgitation, bilateral carpal tunnel decompression, bilateral hernia repair, bilateral hip dysplasia, bilateral hemi-epiphysiodesis for genu valgum10Hurler1.5FemaleBMT1Learning difficulties, hearing impairment, suppraglottoplasty, tracheo-bronchal malacia, adrenal insufficiency, bilateral genu valgum11Hurler0.91FemaleBMT1.42Macrocephaly, developmental delay, cardio-myopathy and mitral regurgitation, bilatera umbilical hernia repair, bilateral hip dysplasia12Hurler1.91MaleBMT1.33Hyposplenism13Hurler1.25FemaleBMT1.42Aortic valve stenosis, hypoadrenalism, umbilical hernia repair14Hunter12.5MaleERT5.5Learning difficulties, hearing impairment, mitral stenosis, gastro-oesophageal reflux, bilateral hip dysplasia, bilateral congenital talipes equinovarus15Morquio1.83FemaleNoneN/AMitral regurgitation, left hip dislocation16Morquio2.75FemaleERT7.58Sleep apnoea, bilateral hip dysplasia, bilateral genu valgumTable 2. Spinal features in our MPS patients.Patient NoMPS TypeSpinal DeformityManagement of Spinal DeformityCervical Spine FeaturesThoracolumbar Spine Features1HurlerThoracolumbar kyphosisNon-operativeNoneHypoplastic beaked L2 vertebra2HurlerThoracolumbar kyphosisNon-operativeDynamic cranio-cervical junction narrowing affecting CSF flowHypoplastic beaked L3 vertebra3HunterThoracolumbar kyphosis, left thoracolumbar scoliosisNon-operativeNarrowing of cranio-cervical junctionHypoplastic beaked L1 & L2 vertebrae4MorquioThoracolumbar kyphosisNon-operativePrevious occipito-cervical fusion for occipito-cervical instabilityHypoplastic vertebrae T11-L25MorquioThoracolumbar kyphosisNon-operativeHypoplastic odontoid process, with hyperplastic peri-odontoid soft tissue, mild cervical spine instabilityHypoplastic beaked T12 vertebra, elliptical L1 & L2 vertebra6MorquioThoracolumbar kyphosis, right thoracic scoliosis, left thoracolumbar scoliosisNon-operativeNoneHypoplastic beaked T11, vertebral platyspondyly at several levels7HurlerThoracolumbar kyphosisOperativeNoneHypoplastic beaked T12 vertebra, anterior spinal subluxation T11/T128HurlerThoracolumbar kyphosis, thoracolumbar scoliosisOperativePrevious occipito-cervical fusion for C1-C2 instabilityHypoplastic beaked L1 vertebra9HurlerThoracolumbar kyphosis (add-on junctional kyphosis)OperativeHyperplasia of peri-odontoid soft tissue with impingement of cordHypoplastic beaked L1 vertebra, previous antero-posterior fusion for thoracolumbar kyphosis10HurlerThoracolumbar kyphosisOperativeNoneMultiple vertebral platyspondyly, short and narrow pedicles, hypoplastic and beaked L2 vertebra11HurlerThoracolumbar kyphosis, right thoracic scoliosisOperativeNoneHypoplastic beaked L1 & L2 vertebrae, T12-L1 segmental instability12HurlerThoracolumbar kyphosisOperativeNoneHypoplastic beaked T12 vertebra, T12-L1 spondylolisthesis13HurlerThoracolumbar kyphosis, left thoracic scoliosisOperativeNoneHypoplastic beaked L1 & L2 vertebra, T11/T12 and T12/L1 spondylolisthesis14HunterThoracolumbar kyphosis, left thoracolumbar scoliosisOperativeNoneHypoplastic beaked T12 vertebra, T11/T12 segmental instability, bilateral L5 isthmic spondylolysis15MorquioThoracolumbar kyphosisOperativePrevious occipito-cervical fusion for C1-C2 instabilityHypoplastic beaked L1 & L2 vertebrae, grade II spondylolisthesis T12/L116MorquioThoracolumbar kyphosisOperativeAbsent odontoid process, cranio-cervical junction stenosis, previous occipito-cervical fusion for C1-C2 instabilityHypoplastic beaked T12 vertebraTable 3. Spinal deformity in our MPS patients managed conservatively.Patient NoMPS TypeAge at Presentation (years)Spinal DeformityExtent of DeformityInitial Deformity Cobb AngleFinal Deformity Cobb AngleFollow-Up (years)1Hurler1.08Thoracolumbar kyphosisT12-L3303312Hurler0.42Thoracolumbar kyphosisT12-L4303013Hunter13Thoracolumbar kyphosisLeft thoracolumbar scoliosisT8-L4T6-L36015752234Morquio12.91Thoracolumbar kyphosisT7-L327275.15Morquio3.91Thoracolumbar kyphosisT9-L18154.16Morquio6.5Thoracolumbar kyphosisRight thoracic scoliosisLeft thoracolumbar scoliosisT9-L1T11-L3T5-T102826173233304.5Table 4. Spinal deformity in our MPS patients treated surgically.Patient NoMPS TypeAge at Presentation (years)Age at Surgery (years)Spinal DeformityExtent of DeformitySurgical Procedure (extent)Initial Deformity Cobb AnglePre-operative Deformity Cobb AnglePost-operative Deformity Cobb AngleSurgical ComplicationsPost-Operative Follow-Up (years)7Hurler4.86.8Thoracolumbar kyphosisT10-L2Circumferential arthrodesis (T10-L3)9511065None7.28Hurler5.77.7Thoracolumbar kyphosis Thoracolumbar scoliosisT11-L3 T11-L3Circumferential arthrodesis (T10-L4)78 1494 2665 15None9.39Hurler14.416.8Thoracolumbar kyphosis (proximal & distal junctional kyphosis after anterior arthrodesis at other centre)T12-L4Circumferential arthrodesis with posterior instrumentation (T10-L5)607835None4.610Hurler1.52.8Thoracolumbar kyphosisT11-L3Circumferential arthrodesis with posterior instrumentation (T11-L4)44560Deep wound infection managed by debridement & IV antibiotics; stable proximal junctional kyphosis3.511Hurler0.94.1Thoracolumbar kyphosis Thoracic scoliosisT10-L3 T5-T9Circumferential arthrodesis (T10-L4)45 19110 2555 14None6.612Hurler1.93.1Thoracolumbar kyphosisT10-L2Circumferential arthrodesis with posterior instrumentation (T10-L3)606222None5.113Hurler1.32.4Thoracolumbar kyphosis Thoracic scoliosisT12-L2 T11-L3Circumferential arthrodesis with posterior instrumentation (T10-L3)44 2269 3312 20None5.814Hunter12.513.7Thoracolumbar kyphosis Thoracolumbar scoliosisT11-L3 T11-L3Circumferential arthrodesis with posterior instrumentation (T9-L4)42 3068 454 0Adjacent distal segment grade II spondylolisthesis4.515Morquio1.83.8Thoracolumbar kyphosisT11-L3Circumferential arthrodesis with posterior instrumentation (T10-L4)36420None6.216Morquio2.84.3Thoracolumbar kyphosisT10-L1Circumferential arthrodesis (T9-L4)405528Non-union at apex of kyphosis managed by posterior re-grafting10.3Table 5. Comparison of our study with published series of spinal stabilisation in MPS patients.6,9,10Series Author (Year)Number of patientsMPS Types (n=)Mean Age at Surgery (Years)Surgical TechniqueMean Pre-operative TL Kyphosis (degrees; range)Mean Post-operative TL Kyphosis (degrees; range)Mean Operative Correction of TL Kyphosis (%; range)Mean Post-operative Follow-up (years; range)Major ComplicationsDalvie (2001)7Morquio (4), Martoux-Lamy (2), Hurler (1)8.7 (4 - 14)Anterior segmental instrumented arthrodesis52.5 (42 - 64)15 (3 - 29)70.6 (45.5 - 93.3)1.2 (0.4 - 3)NoneGenevois (2014)14Hurler (14)7.8 (3.5 - 15)Combined antero-posterior arthrodesis57.5 (30 - 90)14.1 (-14 - +52)66 (30.8 - 100)3.7 (0.8 - 8.7)Adjacent segment listhesis (n = 3), transient paraperesis (n=1), progressive scoliosis (n=1)Yasin (2014)7Hurler (7)4.1 (2.4 - 10)Anterior vascularised rib graft (n = 5). Combined antero-posterior instrumented arthrodesis (n = 1). VEPTR (n = 1)67.3 (N/A)35 (combined arthrodesis & VEPTR cases only)59.5 (58.3 - 59.8; combined arthrodesis & VEPTR cases only)0.8 (0.5 - 1; combined arthrodesis and VEPTR cases only)Proximal junctional kyphosis after anterior-only surgery (n = 3)Current Study (2015)10Hurler (7), Hunter (1), Morquio (2)6.6 (2.4 – 16.8)Combined antero-posterior arthrodesis +/-posterior instrumentation74.3 (37 - 110)28.6 (0 - 65)66.9 (31 - 100)6.3 (3.5 - 10.3)Proximal junction kyphosis (n = 1); deep wound infection (n = 1); adjacent distal segment spondylolisthesis (n = 1); non-union at kyphosis apex (n = 1)Figure legends.Figure 1. Radiographs of patient 7 (Table 4) showing pre-operative a) postero-anterior (PA) and b) lateral radiographs of the spine with 110o thoracolumbar kyphosis (T10-L2). Post-operative c) PA and d) lateral radiographs at 2 years following un-instrumented circumferential arthrodesis (T10-L3) show correction of kyphosis to 65o.Figure 2. Radiographs of patient 12 (Table 4) showing pre-operative a) postero-anterior (PA) and b) lateral radiographs of the spine with 62o thoracolumbar kyphosis (T10-L2). Post-operative c) PA and d) lateral radiographs at 2 years following circumferential arthrodesis with the use of posterior instrumentation (T10-L3) achieved correction of kyphosis to 22o. ................
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