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Table 2 Summary of included studies regarding kyphosis surgery in MPS I patients

From: Treatment of thoracolumbar kyphosis in patients with mucopolysaccharidosis type I: results of an international consensus procedure

Study Design Patients for surgery Age at surgery (years) Surgical indication Surgery type Apex Levels of fusion Kyphotic angle pre- / post-operative Complications Neuro-physiologic monitoring Brace Follow-up (years)
Abelin Genevois, JIMD, 2014 [17] Case series N = 13
Hurler
Treated between 2003 and 2010
Median age: 8 (range 3.5–15) Deformity progression and disruption spinal balance Single- stage circumferential arthrodesis.
Combined anterior and posterior approach (n = 12/13)
Not reported (NR) Two levels above and 2 levels below the apex (n = 12) Median 60° (range 30°-90°) / Median 13° (range − 14°-52°) Significant loss of correction (n = 2) ➔ revision surgery (consecutively to a previous posterior-only fusion)
Mild adjacent segment spondylolisthesis at the upper level in 50%.
Multi-modal EP or at least SSEP Postoperatively a spine cast for 3 months, in addition full time bracing for 3 months Median 2.2 (range 0.8–8.7)
Roberts et al., Bone Joint J, 2016 [52] Case series N = 7
Hurler
Treated between 2001 and 2013
Median 4.1 (range 2.8–16.8) The presence of severe, progressive thoracolumbar kyphosis > 40° Circumferential arthrodesis (n = 3)
Circumferential arthrodesis with posterior instrumentation (n = 4)
T12 (n = 2)
L1 (n = 2)
L2 (n = 1)
L1-L2 (n = 2)
T10-L3 (n = 3)
T10-L4 (n = 2)
T10-L5
T11-L4
Median 78° (range 56°- 110°)/ median 35° (range 0°-65°) Deep wound infection (n = 1), stable proximal junctional kyphosis(n = 1) SSEP and MEP No brace Median 5.8 (range 3.5–9.3)
Yasin et al., Spine, 2014 [49] Case series N = 7
Hurler
Anterior fusion using vascularized rib: mean 3 (range 2.4–3.8; n = 5)
Combined anterior and posterior instrumented fusion: 8 (n = 1)
VEPTR: 4 (n = 1)
NR Anterior fusion using vascularized rib (n = 5), combined anterior and posterior instrumented fusion (n = 1), VEPTR (n = 1) NR NR Anterior fusion: mean 60° (n = 5)/NR
Combined fusion: 84° /at FU 35°
VEPTR: 87°/ 35°
Anterior only: kyphotic deformity adjacent to the operated segment (n = 5) NR Postoperatively: braced until satisfactory healing of the graft on plain radiographs NR
Tandon et al., J Bone Joint Surg, 1996 [30] Case series N = 3
Hurler
2.8
3.5
10.3
NR Posterior spinal fusion (n = 3) L2 (n = 2) NR 80°/NR
56°/NR
40°/NR
At age 12y symptomatic cord compression at T10-T11 (n = 1) NR NR NR
Garrido et al., Eur Spine J, 2014 [53] Case series N = 3
Hurler
2.4
2.9
3
NR Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation L1 (n = 2), Th12(n = 1) T10-L3 70°/NR
65°/NR
63°/NR
Left lower lobe collapse after extubation (n = 1)
Wound infection (n = 1)
SSEP Postoperatively: Moulded Thoracolumbar Sacral Orthosis for 3 months NR
Vellodi et al., Archives of Disease in childhood, 1997 [54] Case series N = 7
Hurler
Average age of 7.6 (3.8–10.3) (n = 6)   Prophylactic posterior spinal fusion (n = 6)
Thoracolumbar decompression and fusion for cord compression (n = 1)
NR NR NR NR NR NR NR
Field et al., J Bone Joint Surg, 1994 [6] Case series N = 6
Hurler
Average age of 7.6 (3.8 to 10.3) NR Posterior spinal fusion (n = 6) L1 or L2 NR NR NR NR NR NR
Polgreen et al., Bone Marrow Transplantation, 2009 [55] Case series N = 5
Hurler
Reported in one patient: 5 NR Spinal fusion (n = 2), anterioposterior spinal fusion (n = 1), posterior spinal fusion (n = 2), anterior spinal fusion (n = 1) NR T9 – L3
T10-L2
T11 - L3
T7 -L3
1 not reported
NR NR NR Adjacent kyphosis to operated segment T1-T8 NR
Schmidt et al., Orphanet Journal of Rare diseases, 2016 [33] Case series N = 2
Hurler
7.8
14.3
Symptoms, not further specified (n = 1) Posterior fusion (n = 2) NR NR 90°/ NR
(n = 1)
NR NR No NR
Hopwood et al., JIMD, 1993 [56] Case series N = 2
Hurler
7
10.3
NR Posterior spinal fusion (n = 1)
Posterior spinal fusion using the patient’s own left anterior iliac spine plus donor bone. (n = 1)
NR NR NR NR NR NR NR
Malm et al., Acta Paediatrica, 2008 [57] Case series N = 2
Hurler
NR NR Spinal fusion
Dorsal spinal fusion and ventral spinal fusion
NR Spinal fusion: T8 and L4
Dorsal spinal fusion: T10 to L4 and ventral spi- nal fusion: T12 - L3
NR NR NR NR NR
Souillet et al., Bone marrow transplantation, 2003 [58] Case series N = 2
Hurler
11.2 and 12.2
3.4
NR Posterior spinal fusion and anterior spinal fusion (n = 1)
Posterior spinal fusion (n = 1)
NR NR NR NR NR No NR
Stoop et al., JIMD reports, 2012 [45] Case series N = 1
Hurler
Treated between 2003 and 2011
NR NR Short segment posterior fusion after pedicle subtraction osteotomy of L1 NR Th12 to L3 80°/ 12° Kyphosis superior to the spondylodesis; 64°➔ revision of the spondylodesis at the level of T11-L4 and superior extension with a growing rod system; 14° NR No. NR
Yasuda et al., MGM reports, 2015 [59] Case report N = 1
Hurler
13 NR Arthrodesis. Thoracolumbar spinal fusion surgery, dual fusion rods with pedicle screws extended from T5- L3 with overlying graft material L2 T5-L3 NR NR NR NR NR
Bekmez et al., J Pediatr Orthop,2016 [43] Case report N = 1
Hurler
Treated between 2008 and 2011
8 Deformity progression of > 10 °/ 12 months and disruption of the sagittal balance despite brace treatment Posterior only L1 T10- L3 52°/ 15° Distal junctional Kyphosis of 18 ° SSEP and MEP Postoperatively, thoracolumbar sacral orthosis, for 4 months. 5.1
Makler et al., Surg Neurol Int, 2017 [60] Case report N = 1
Hurler
6 NR Anterior release and posterior spinal fusion L1 NR Complete resolution of gibbus deformity Preoperative work up revealed a Chiari I malformation and a syringomelia NR NR NR
Dalvie et al., Spine,2001 [44] Case report N = 1
Hurler
5 NR Anterior instrumented correction and fusion NR T11-L2 59°/18° NR NR NR 0.4
Pauchard et al., JIMD reports, 2014 [61] Case report N = 1 4 NR Two-stage surgical correction was planned, with initial posterior instrumentation of T12-L2, followed by anterior graft. After first surgery a complication ➔leading to removal of the hardware. T12 T12-L2 90°/NA Asymmetric paraplegia with partial sensory loss SSEP and MEP NR NR
  1. Abbreviations: VEPTR Vertical expandable prosthetic titanium rib, SSEP somatosensory- evoked potentials, MEP motor evoked potentials