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Multicenter Study
. 2021 Mar 1;41(3):182-189.
doi: 10.1097/BPO.0000000000001720.

Intraoperative Neurological Monitoring in Lower Limb Surgery for Patients With Mucopolysaccharidoses

Andrew G Georgiadis  1   2 , Trever M Koester  1 , William G Mackenzie  3 , Nickolas J Nahm  3 , Susan A Novotny  1   4 , Kenneth J Rogers  3 , Breana R Siljander  1   2 , Ted C Sousa  5   6 , Klane K White  5 , Kevin R Walker  1   2
Affiliations
Multicenter Study

Intraoperative Neurological Monitoring in Lower Limb Surgery for Patients With Mucopolysaccharidoses

Andrew G Georgiadis et al. J Pediatr Orthop. .

Abstract

Background: There are reports of spinal cord injury (SCI) occurring after lower extremity (LE) surgery in children with mucopolysaccharidoses (MPS). Intraoperative neurological monitoring (IONM) has been adopted in some centers to assess real-time spinal cord function during these procedures. The aim of this investigation was to review 3 specialty centers' experiences with MPS patients undergoing LE surgery. We report how IONM affected care and the details of spinal cord injuries in these patients.

Methods: All pediatric MPS patients who underwent LE surgery between 2001 and 2018 were reviewed at 3 children's orthopaedic specialty centers. Demographic and surgical details were reviewed. Estimated blood loss (EBL), surgical time, positioning, use of IONM, and changes in management as a result of IONM were recorded. Details of any spinal cord injuries were examined in detail.

Results: During the study period, 92 patients with MPS underwent 252 LE surgeries. IONM was used in 83 of 252 (32.9%) surgeries, and intraoperative care was altered in 17 of 83 (20.5%) cases, including serial repositioning (n=7), aggressive blood pressure management (n=6), and abortion of procedures (n=8). IONM was utilized in cases with larger EBL (279 vs. 130 mL) and longer operative time (274 vs. 175 min) compared with procedures without IONM. Three patients without IONM sustained complete thoracic SCI postoperatively, all from cord infarction in the upper thoracic region. These 3 cases were characterized by long surgical time (328±41 min) and substantial EBL (533±416 mL or 30.5% of total blood volume; range, 11% to 50%). No LE surgeries accompanied by IONM experienced SCI.

Conclusions: Patients with MPS undergoing LE orthopaedic surgery may be at risk for SCI, particularly if the procedures are long or are expected to have large EBL. One hypothesis for the etiology of SCI in this setting is hypoperfusion of the upper thoracic spinal cord due to prolonged intraoperative or postoperative hypotension. IONM during these procedures may mitigate the risk of SCI by identifying real-time changes in spinal cord function during surgery, inciting a change in the surgical plan.

Level of evidence: Level III-retrospective comparative series.

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Conflict of interest statement

The authors declare no conflicts of interest.

References

    1. Williams N, Challoumas D, Eastwood DM. Does orthopaedic surgery improve quality of life and function in patients with mucopolysaccharidoses? J Child Orthop. 2017;11:289–297.
    1. White KK, Jester A, Bache CE, et al. Orthopedic management of the extremities in patients with Morquio A syndrome. J Child Orthop. 2014;8:295–304.
    1. Kanazawa T, Yasunaga Y, Ikuta Y, et al. Femoral head dysplasia in Morquio disease type A: bilateral varus osteotomy of the femur. Acta Orthop Scand. 2001;72:18–21.
    1. Thawrani DP, Walker K, Polgreen LE, et al. Hip dysplasia in patients with Hurler syndrome (mucopolysaccharidosis type 1H). J Pediatr Orthop. 2013;33:635–643.
    1. Lewis JR, Gibson PH. Bilateral hip replacement in three patients with lysosomal storage disease: mucopolysaccharidosis type IV and Mucolipidosis type III. J Bone Joint Surg Br. 2010;92:289–292.

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