Spinal Fusion in Patients With Classic Amyoplasia and General Arthrogryposis.


Journal

Journal of pediatric orthopedics
ISSN: 1539-2570
Titre abrégé: J Pediatr Orthop
Pays: United States
ID NLM: 8109053

Informations de publication

Date de publication:
01 Oct 2023
Historique:
medline: 12 9 2023
pubmed: 28 7 2023
entrez: 28 7 2023
Statut: ppublish

Résumé

Arthrogryposis multiplex congenita is a group of conditions characterized by joint contractures affecting 2 or more joints. This study describes results of spinal fusion in patients with classic amyoplasia and general arthrogryposis. IRB approved retrospective review of patients with a diagnosis of classic amyoplasia and general arthrogryposis who had a primary definitive posterior spinal fusion between 1990 and 2017 at a single pediatric institution. Patients with distal and syndromic arthrogryposis were excluded as well as patients treated with growth-sparing spinal instrumentation. The Modified Clavien-Dindo-sink (MCDS) classification system was used to describe postoperative complications. Over the 28-year period, 342 patients were diagnosed with amyoplasia and general arthrogryposis. Among the 342 patients, 60 (18%) had scoliosis, and 22 (6% of the cohort and 37% of those with scoliosis) were treated surgically. Six patients had growth-sparing techniques, initial fusion elsewhere, or ˂1 year of follow-up, leaving 15 patients. Of the 15, 9 (60%) had a posterior spinal fusion (PSF) and 6 (40%) had a combined anterior spinal fusion (ASF)/PSF. The ASF/PSF group was significantly younger at surgery, had a greater American Society of Anesthesiologists status, longer surgery duration, and lower implant density. The average preoperative major coronal deformity in ASF/PSF patients (108 degrees) was greater than patients treated with PSF alone (88 deg). There were 11 complications in 7 patients, with the most common being deep infection requiring reoperation (5/11, 45%). There was 1 instance (1/11, 9%) of each: prolonged intensive care unit admission (>72 h), superficial wound dehiscence, symptomatic implants requiring removal/revision, coronal plane progression requiring extension of fusion, recurrent pneumothorax requiring return to OR, and pseudoarthrosis leading to implant failure (without revision). Complications occurred in 1/9 (11%) PSF-only patients and 6/6 (100%) ASF/PSF patients with all 6 ASF/PSF patients requiring at least 1 reoperation. The average coronal correction was 48% in the PSF-only group and 28% in the ASF/PSF group. Complication rates after spinal fusion for scoliosis in arthrogryposis multiplex congenita patients are high, especially in patients undergoing ASF/PSF, deep infection is common, and major coronal plane curve correction is modest. II Retrospective Study.

Sections du résumé

BACKGROUND BACKGROUND
Arthrogryposis multiplex congenita is a group of conditions characterized by joint contractures affecting 2 or more joints. This study describes results of spinal fusion in patients with classic amyoplasia and general arthrogryposis.
METHODS METHODS
IRB approved retrospective review of patients with a diagnosis of classic amyoplasia and general arthrogryposis who had a primary definitive posterior spinal fusion between 1990 and 2017 at a single pediatric institution. Patients with distal and syndromic arthrogryposis were excluded as well as patients treated with growth-sparing spinal instrumentation. The Modified Clavien-Dindo-sink (MCDS) classification system was used to describe postoperative complications.
RESULTS RESULTS
Over the 28-year period, 342 patients were diagnosed with amyoplasia and general arthrogryposis. Among the 342 patients, 60 (18%) had scoliosis, and 22 (6% of the cohort and 37% of those with scoliosis) were treated surgically. Six patients had growth-sparing techniques, initial fusion elsewhere, or ˂1 year of follow-up, leaving 15 patients. Of the 15, 9 (60%) had a posterior spinal fusion (PSF) and 6 (40%) had a combined anterior spinal fusion (ASF)/PSF. The ASF/PSF group was significantly younger at surgery, had a greater American Society of Anesthesiologists status, longer surgery duration, and lower implant density. The average preoperative major coronal deformity in ASF/PSF patients (108 degrees) was greater than patients treated with PSF alone (88 deg). There were 11 complications in 7 patients, with the most common being deep infection requiring reoperation (5/11, 45%). There was 1 instance (1/11, 9%) of each: prolonged intensive care unit admission (>72 h), superficial wound dehiscence, symptomatic implants requiring removal/revision, coronal plane progression requiring extension of fusion, recurrent pneumothorax requiring return to OR, and pseudoarthrosis leading to implant failure (without revision). Complications occurred in 1/9 (11%) PSF-only patients and 6/6 (100%) ASF/PSF patients with all 6 ASF/PSF patients requiring at least 1 reoperation. The average coronal correction was 48% in the PSF-only group and 28% in the ASF/PSF group.
CONCLUSION CONCLUSIONS
Complication rates after spinal fusion for scoliosis in arthrogryposis multiplex congenita patients are high, especially in patients undergoing ASF/PSF, deep infection is common, and major coronal plane curve correction is modest.
LEVEL OF EVIDENCE METHODS
II Retrospective Study.

Identifiants

pubmed: 37503867
doi: 10.1097/BPO.0000000000002483
pii: 01241398-990000000-00333
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e751-e756

Informations de copyright

Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.

Déclaration de conflit d'intérêts

The authors declare no conflicts of interest.

Références

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Yingsakmongkol W, Kumar SJ. Scoliosis in arthrogryposis multiplex congenita: results after nonsurgical and surgical treatment. J Pediatr Orthop. 2000;20:656–661.
Siebold RM, Winter RB, Moe JH. The treatment of scoliosis in arthrogryposis multiplex congenita. Clin Orthop Relat Res. 1974;103:191–198.
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Greggi T, Martikos K, Pipitone E, et al. Surgical treatment of scoliosis in a rare disease: arthrogryposis. Scoliosis. 2010;5:24.
Xu L, Chen Z, Qiu Y, et al. Case-matched comparative analysis of spinal deformity correction in arthrogryposis multiplex congenita versus adolescent idiopathic scoliosis. J Neurosurg Pediatr. 2018;23:22–29.
Verhofste BP, Emans JB, Miller PE, et al. Growth-friendly spine surgery in arthrogryposis multiplex congenita. J Bone Joint Surg Am. 2021;103:715–726.
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Bevan WP, Hall JG, Bamshad M, et al. Arthrogryposis multiplex congenita (amyoplasia): an orthopaedic perspective. J Pediatr Orthop. 2007;27:594–600.
Wahlig B, Poppino K, Jo CH, et al. Arthrogryposis multiplex congenita: a 28-year retrospective study. Dev Med Child Neurol. 2022;64:476–480.
Dodwell ER, Pathy R, Widmann RF, et al. Reliability of the modified clavien-dindo-sink complication classification system in pediatric orthopaedic surgery. JB JS Open Access. 2018;3:e0020.

Auteurs

Dietrich Riepen (D)

UT Southwestern Medical Center.

Emily E Lachmann (EE)

Texas Scottish Rite Hospital for Children, Dallas, TX.

Brian Wahlig (B)

Mayo Clinic, Department of Orthopedic Surgery, Rochester, MN.

David C Thornberg (DC)

Texas Scottish Rite Hospital for Children, Dallas, TX.

Karl E Rathjen (KE)

UT Southwestern Medical Center.
Texas Scottish Rite Hospital for Children, Dallas, TX.

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