Chiari I malformation management in patients with heritable connective tissue disorders.
Chiari I Malformation
Connective tissue disease
Ehlers-danlos syndrome
Marfan syndrome
Neurosurgery
Osteogenesis imperfecta
Syringomyelia
Journal
World neurosurgery: X
ISSN: 2590-1397
Titre abrégé: World Neurosurg X
Pays: United States
ID NLM: 101747743
Informations de publication
Date de publication:
Apr 2023
Apr 2023
Historique:
received:
28
08
2022
revised:
16
02
2023
accepted:
23
02
2023
entrez:
27
3
2023
pubmed:
28
3
2023
medline:
28
3
2023
Statut:
epublish
Résumé
Chiari malformation type I (CMI) is relatively common neurosurgical condition typically treated with posterior fossa decompression. However, the management of CMI in patients with heritable connective tissue disorders (CTDs), such as Ehlers-Danlos Syndrome, Marfan Syndrome, or Osteogenesis Imperfecta, involves a unique set of perioperative challenges. This study aims to define the demographic information, comorbidities, and perioperative course of patients with concomitant CMI and CTD. Patients with CMI admitted for surgical decompression from 2008 to 2015 were captured using the National Inpatient Sample (NIS). Information was collected based on ICD-9 codes. Descriptive and regression analyses were performed in SPSS (version 26). 38,169 CMI patients, 353 of whom had CTD (0.92%), were identified. CMI patients with CTD were more likely to be female ( Patients with concurrent CTD and CMI were more likely to present with complex Chiari and associated CCI. They were also younger, more often female, and had more systemic, CNS, and joint abnormalities. As such, preoperative recognition of an underlying CTD is imperative to achieve optimal outcomes in this patient population.
Sections du résumé
Background
UNASSIGNED
Chiari malformation type I (CMI) is relatively common neurosurgical condition typically treated with posterior fossa decompression. However, the management of CMI in patients with heritable connective tissue disorders (CTDs), such as Ehlers-Danlos Syndrome, Marfan Syndrome, or Osteogenesis Imperfecta, involves a unique set of perioperative challenges.
Objective
UNASSIGNED
This study aims to define the demographic information, comorbidities, and perioperative course of patients with concomitant CMI and CTD.
Methods
UNASSIGNED
Patients with CMI admitted for surgical decompression from 2008 to 2015 were captured using the National Inpatient Sample (NIS). Information was collected based on ICD-9 codes. Descriptive and regression analyses were performed in SPSS (version 26).
Results
UNASSIGNED
38,169 CMI patients, 353 of whom had CTD (0.92%), were identified. CMI patients with CTD were more likely to be female (
Conclusions
UNASSIGNED
Patients with concurrent CTD and CMI were more likely to present with complex Chiari and associated CCI. They were also younger, more often female, and had more systemic, CNS, and joint abnormalities. As such, preoperative recognition of an underlying CTD is imperative to achieve optimal outcomes in this patient population.
Identifiants
pubmed: 36969375
doi: 10.1016/j.wnsx.2023.100173
pii: S2590-1397(23)00022-4
pmc: PMC10031113
doi:
Types de publication
Journal Article
Langues
eng
Pagination
100173Informations de copyright
© 2023 The Authors.
Déclaration de conflit d'intérêts
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Références
JAMA Facial Plast Surg. 2018 Jan 01;20(1):70-75
pubmed: 29121166
J Neurol Sci. 1990 Jan;95(1):3-28
pubmed: 2187057
Dig Surg. 2012;29(6):453-5
pubmed: 23295898
Case Rep Med. 2015;2015:583570
pubmed: 26798347
Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):181-187
pubmed: 28186368
Clin Rev Allergy Immunol. 2020 Jun;58(3):273-297
pubmed: 31267471
Plast Reconstr Surg. 1970 Jun;45(6):606-9
pubmed: 5444678
PLoS One. 2021 May 11;16(5):e0251289
pubmed: 33974636
J Neurosurg Spine. 2007 Dec;7(6):601-9
pubmed: 18074684
Bull Exp Biol Med. 2011 Apr;150(6):676-8
pubmed: 22235414
Am J Med Genet. 1988 Mar;29(3):581-94
pubmed: 3287925
Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):226-236
pubmed: 28261938
Balkan Med J. 2018 Jul 31;36(1):12-16
pubmed: 30063214
J Neurosurg. 1989 Aug;71(2):159-68
pubmed: 2746341
Eur J Hum Genet. 2013 Apr;21(4):386-90
pubmed: 22929030
Neurosurgery. 1999 May;44(5):1005-17
pubmed: 10232534
J Neurosurg Pediatr. 2019 Aug 16;:1-8
pubmed: 31419800
Acta Neurochir (Wien). 2010 Jul;152(7):1117-27
pubmed: 20440631
J Med Genet. 2010 Jul;47(7):476-85
pubmed: 20591885
Neurosurgery. 2004 Jan;54(1):65-70; discussion 70-1
pubmed: 14683542
Neurosci Biobehav Rev. 2007;31(8):1101-13
pubmed: 17599406
Neurosurg Clin N Am. 2015 Oct;26(4):501-7
pubmed: 26408058
Neuroscientist. 2008 Apr;14(2):182-94
pubmed: 18079557
Pediatr Neurol. 2020 May;106:32-37
pubmed: 32113729
World Neurosurg. 2020 Oct;142:e350-e356
pubmed: 32653513
Surg Neurol. 2009 Jul;72(1):20-35
pubmed: 19559924
Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):195-211
pubmed: 28220607
J Steroid Biochem Mol Biol. 2017 Sep;172:106-116
pubmed: 28629994
Neurosurg Rev. 2019 Dec;42(4):915-936
pubmed: 30627832
Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):8-26
pubmed: 28306229
Neurosurgery. 2015 Aug;77(2):269-73
pubmed: 25924208
Semin Arthritis Rheum. 2014 Aug;44(1):93-100
pubmed: 24507822
Orphanet J Rare Dis. 2014 Jul 23;9:109
pubmed: 25053156
Ann Rheum Dis. 2007 Oct;66(10):1369-73
pubmed: 17412739
Auton Neurosci. 2018 Dec;215:3-11
pubmed: 29523389