Surgical correction of scoliosis in Rett syndrome: cord monitoring and complications

Hammett, T.; Harris, Angela P.; Boreham, B.; Mehdian, S. M. H.

doi:10.1007/s00586-014-3170-9

Cited by 11 publications

(5 citation statements)

References 13 publications

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“…These results extend our understanding of interactions between scoliosis and overall clinical severity. Surgical correction of scoliosis is mostly successful but has a very high rate of complications with a prolonged hospital stay 49 . Other orthopedic symptoms include hip displacement and a high prevalence of fractures 50 – 52 , which can be diminished with intravenous bisphosphonates treatment 53 .…”

Section: Skeletal Systemmentioning

confidence: 99%

Rett syndrome: think outside the (skull) box

Borloz¹,

Villard²,

Roux³

2021

Fac Rev

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Rett syndrome (RTT) is a severe X-linked neurodevelopmental disorder characterized by neurodevelopmental regression between 6 and 18 months of life and associated with multi-system comorbidities. Caused mainly by pathogenic variants in the MECP2 (methyl CpG binding protein 2) gene, it is the second leading genetic cause of intellectual disability in girls after Down syndrome. RTT affects not only neurological function but also a wide array of non-neurological organs. RTT-related disorders involve abnormalities of the respiratory, cardiovascular, digestive, metabolic, skeletal, endocrine, muscular, and urinary systems and immune response. Here, we review the different aspects of RTT affecting the main peripheral groups of organs and sometimes occurring independently of nervous system defects.

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Section: Skeletal Systemmentioning

confidence: 99%

Rett syndrome: think outside the (skull) box

Borloz¹,

Villard²,

Roux³

2021

Fac Rev

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“…First, using monopolar stimulation with a sternal electrode as an anode was found to yield similar lower-limb responses to those yielded by bipolar stimulation, and can therefore be used to monitor spinal pathways during standard surgery for scoliosis involving arthrodesis with posterior instrumentation. Such a procedure represents an alternative method to the usual SEPs that cannot give full satisfaction in all patients or to tcMEPs when there are concerns over their use in patients with epilepsy such as children with Rett syndrome [21]. Second, where indicated, monopolar stimulation allows for the combined monitoring of the spinal cord and the brachial plexus within a single stimulation procedure.…”

Section: Discussionmentioning

confidence: 99%

Validity and utility of monopolar spinal cord stimulation in pediatric scoliosis surgery

et al. 2016

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“…As spinal surgery generally has greater length, complexity, and risk than other pediatric orthopaedic procedures 27 , one might assume that patients with Down syndrome may have greater risk of surgical complications after spinal surgery. Patients with various types of syndromic scoliosis have higher surgical risk, with higher rates of postoperative complications 20,[28][29][30][31][32][33][34][35][36][37] . Levy et al reported complication rates in syndromic cases including wound infection (14.3%), respiratory issues (14.3%), implant failure (35.7%), and pseudarthrosis (28.6%) 38 .…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Posterior Spinal Fusion in Pediatric Patients with Down Syndrome

Fraser

Krakow

Lin

et al. 2022

Journal of Bone and Joint Surgery

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Background:Trisomy 21 or Down syndrome is associated with multiple orthopaedic manifestations. Although cervical instability is the most common spinal condition associated with Down syndrome, the prevalence of scoliosis has been estimated at 4.8% to 8.7%. Very few prior studies have documented the role of spinal fusion in this population, and all have included ≤10 patients.Methods:An institutional review board-approved multicenter retrospective analysis of patients with Down syndrome treated with spinal fusion between January 2009 and December 2019 was performed by cross-referencing Current Procedural Terminology (CPT) and International Classification of Diseases, Ninth and Tenth Revisions (ICD-9 and ICD-10) codes. Patients were followed for ≥2 years, with a mean follow-up of 3.77 years. Clinical and radiographic outcomes were collected, and complications were documented using the Clavien-Dindo-Sink (CDS) classification.Results:A total of 23 patients were included: 96% had ≥1 medical comorbidities, including 16 (70%) with congenital heart disease, of whom 88% had previous cardiac surgery, and 10 (44%) with thyroid disorders. All 23 patients underwent posterior spinal fusion. The mean estimated blood loss was 617 ± 459 mL, the mean length of the surgical procedure was 290 ± 92.7 minutes, and the mean length of hospital stay was 6.03 ± 2.91 days. The major Cobb angle measured 61.7° ± 17.6°, which corrected to 19.4° ± 14.8° (68.6% correction; p < 0.001), with well-maintained correction at 2 years of 22.0° ± 10.3° (64.3% correction; p = 0.158). Thirteen (57%) of 23 patients had a change in curve of >5°. There were no intraoperative complications; however, 12 patients (52%) sustained postoperative complications (e.g., need for reoperation, implant failure, and pulmonary complications), including 6 patients with CDS type 3 or 4 (e.g., wound dehiscence, late superficial abscess, pleural effusion, pseudarthrosis, and readmission for hypoxia). Four patients (17%) required a revision surgical procedure. One patient (4%) required an unplanned intensive care unit admission.Conclusions:Although instrumented spinal fusion can effectively correct spinal deformity in these patients, complications are more frequent than in children with adolescent idiopathic scoliosis, with over half of patients sustaining a complication.Level of Evidence:Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

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Surgical correction of scoliosis in Rett syndrome: cord monitoring and complications

Cited by 11 publications

References 13 publications

Rett syndrome: think outside the (skull) box

Rett syndrome: think outside the (skull) box

Validity and utility of monopolar spinal cord stimulation in pediatric scoliosis surgery

Outcomes of Posterior Spinal Fusion in Pediatric Patients with Down Syndrome

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