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Chiari malformation type IV

MedGen UID:
148203
•Concept ID:
C0750932
•
Congenital Abnormality
Synonyms: Arnold Chiari malformation type IV; Chiari malformation type 4
SNOMED CT: Chiari malformation type IV (253187005)
 
Monarch Initiative: MONDO:0022716

Professional guidelines

PubMed

Chiari Malformation (Update on Diagnosis and Treatment).
Rosenblum JS, Pomeraniec IJ, Heiss JD
Neurol Clin 2022 May;40(2):297-307. Epub 2022 Mar 31 doi: 10.1016/j.ncl.2021.11.007. PMID: 35465876Free PMC Article
Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia.
Haffner D, Emma F, Eastwood DM, Duplan MB, Bacchetta J, Schnabel D, Wicart P, Bockenhauer D, Santos F, Levtchenko E, Harvengt P, Kirchhoff M, Di Rocco F, Chaussain C, Brandi ML, Savendahl L, Briot K, Kamenicky P, Rejnmark L, Linglart A
Nat Rev Nephrol 2019 Jul;15(7):435-455. doi: 10.1038/s41581-019-0152-5. PMID: 31068690Free PMC Article
Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation.
Giner J, Pérez López C, Hernández B, Gómez de la Riva Á, Isla A, Roda JM
Neurologia (Engl Ed) 2019 Jun;34(5):318-325. Epub 2016 Dec 9 doi: 10.1016/j.nrl.2016.09.010. PMID: 27939111

Recent clinical studies

Etiology

Imaging in Chiari I Malformation.
Pindrik J, McAllister AS, Jones JY
Neurosurg Clin N Am 2023 Jan;34(1):67-79. doi: 10.1016/j.nec.2022.08.006. PMID: 36424066
Arnold-Chiari Malformation: Core Concepts.
Fons K, Jnah AJ
Neonatal Netw 2021 Aug 1;40(5):313-320. doi: 10.1891/11-T-704. PMID: 34518383
Chiari Malformation in Children.
Albert GW
Pediatr Clin North Am 2021 Aug;68(4):783-792. doi: 10.1016/j.pcl.2021.04.015. PMID: 34247709
Chiari Malformations.
Mancarella C, Delfini R, Landi A
Acta Neurochir Suppl 2019;125:89-95. doi: 10.1007/978-3-319-62515-7_13. PMID: 30610307
Chiari Headache.
Mehta A, Chilakamarri P, Zubair A, Kuruvilla D
Curr Pain Headache Rep 2018 Jun 14;22(7):49. doi: 10.1007/s11916-018-0702-8. PMID: 29904826

Diagnosis

Arnold-Chiari Malformation: Core Concepts.
Fons K, Jnah AJ
Neonatal Netw 2021 Aug 1;40(5):313-320. doi: 10.1891/11-T-704. PMID: 34518383
The Chiari I malformation.
McClugage SG, Oakes WJ
J Neurosurg Pediatr 2019 Sep 1;24(3):217-226. doi: 10.3171/2019.5.PEDS18382. PMID: 31473667
Chiari Malformations.
Mancarella C, Delfini R, Landi A
Acta Neurochir Suppl 2019;125:89-95. doi: 10.1007/978-3-319-62515-7_13. PMID: 30610307
Chiari Headache.
Mehta A, Chilakamarri P, Zubair A, Kuruvilla D
Curr Pain Headache Rep 2018 Jun 14;22(7):49. doi: 10.1007/s11916-018-0702-8. PMID: 29904826
Chiari I Malformation: Adult and Pediatric Considerations.
Leonard JR, Limbrick DD Jr
Neurosurg Clin N Am 2015 Oct;26(4):xiii-xiv. doi: 10.1016/j.nec.2015.07.002. PMID: 26408069

Therapy

Chiari Malformation Type 1 in Adults.
Zisakis A, Sun R, Pepper J, Tsermoulas G
Adv Tech Stand Neurosurg 2023;46:149-173. doi: 10.1007/978-3-031-28202-7_8. PMID: 37318574
Fetal Repair of Neural Tube Defects.
Lee SY, Papanna R, Farmer D, Tsao K
Clin Perinatol 2022 Dec;49(4):835-848. Epub 2022 Oct 9 doi: 10.1016/j.clp.2022.06.004. PMID: 36328602
The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature.
Saletti V, Farinotti M, Peretta P, Massimi L, Ciaramitaro P, Motta S, Solari A, Valentini LG
Neurol Sci 2021 Dec;42(12):4965-4995. Epub 2021 Sep 30 doi: 10.1007/s10072-021-05565-9. PMID: 34591209
Chiari I malformation in patients with RASopathies.
Han Y, Chen M, Wang H
Childs Nerv Syst 2021 Jun;37(6):1831-1836. Epub 2021 Jan 6 doi: 10.1007/s00381-020-05034-2. PMID: 33409618
Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia.
Haffner D, Emma F, Eastwood DM, Duplan MB, Bacchetta J, Schnabel D, Wicart P, Bockenhauer D, Santos F, Levtchenko E, Harvengt P, Kirchhoff M, Di Rocco F, Chaussain C, Brandi ML, Savendahl L, Briot K, Kamenicky P, Rejnmark L, Linglart A
Nat Rev Nephrol 2019 Jul;15(7):435-455. doi: 10.1038/s41581-019-0152-5. PMID: 31068690Free PMC Article

Prognosis

Human genetics and molecular genomics of Chiari malformation type 1.
Mekbib KY, Muñoz W, Allington G, McGee S, Mehta NH, Shofi JP, Fortes C, Le HT, Nelson-Williams C, Nanda P, Dennis E, Kundishora AJ, Khanna A, Smith H, Ocken J, Greenberg ABW, Wu R, Moreno-De-Luca A, DeSpenza T Jr, Zhao S, Marlier A, Jin SC, Alper SL, Butler WE, Kahle KT
Trends Mol Med 2023 Dec;29(12):1059-1075. Epub 2023 Oct 4 doi: 10.1016/j.molmed.2023.08.013. PMID: 37802664
Orthostatic Intolerance and Chiari I Malformation.
Petracek LS, Rowe PC
Neurosurg Clin N Am 2023 Jan;34(1):43-54. doi: 10.1016/j.nec.2022.09.002. PMID: 36424063
The Chiari I malformation.
McClugage SG, Oakes WJ
J Neurosurg Pediatr 2019 Sep 1;24(3):217-226. doi: 10.3171/2019.5.PEDS18382. PMID: 31473667
Chiari I-a 'not so' congenital malformation?
Thompson DNP
Childs Nerv Syst 2019 Oct;35(10):1653-1664. Epub 2019 Jul 10 doi: 10.1007/s00381-019-04296-9. PMID: 31292759
Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.
Langridge B, Phillips E, Choi D
World Neurosurg 2017 Aug;104:213-219. Epub 2017 Apr 21 doi: 10.1016/j.wneu.2017.04.082. PMID: 28435116

Clinical prediction guides

Human genetics and molecular genomics of Chiari malformation type 1.
Mekbib KY, Muñoz W, Allington G, McGee S, Mehta NH, Shofi JP, Fortes C, Le HT, Nelson-Williams C, Nanda P, Dennis E, Kundishora AJ, Khanna A, Smith H, Ocken J, Greenberg ABW, Wu R, Moreno-De-Luca A, DeSpenza T Jr, Zhao S, Marlier A, Jin SC, Alper SL, Butler WE, Kahle KT
Trends Mol Med 2023 Dec;29(12):1059-1075. Epub 2023 Oct 4 doi: 10.1016/j.molmed.2023.08.013. PMID: 37802664
Chiari I malformation in patients with RASopathies.
Han Y, Chen M, Wang H
Childs Nerv Syst 2021 Jun;37(6):1831-1836. Epub 2021 Jan 6 doi: 10.1007/s00381-020-05034-2. PMID: 33409618
Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.
Langridge B, Phillips E, Choi D
World Neurosurg 2017 Aug;104:213-219. Epub 2017 Apr 21 doi: 10.1016/j.wneu.2017.04.082. PMID: 28435116
Clinical Outcome Measures in Chiari I Malformation.
Yarbrough CK, Greenberg JK, Park TS
Neurosurg Clin N Am 2015 Oct;26(4):533-41. Epub 2015 Aug 4 doi: 10.1016/j.nec.2015.06.008. PMID: 26408063
Clinical Presentation of Chiari I Malformation and Syringomyelia in Children.
Pindrik J, Johnston JM Jr
Neurosurg Clin N Am 2015 Oct;26(4):509-14. Epub 2015 Aug 4 doi: 10.1016/j.nec.2015.06.004. PMID: 26408059

Recent systematic reviews

Hemifacial spasm secondary to Chiari malformation type I: Systematic review with case illustration.
Hunsaker JC, Baker CM, Schmidt RH, Rahimpour S
J Clin Neurosci 2022 Jul;101:21-25. Epub 2022 May 2 doi: 10.1016/j.jocn.2022.04.038. PMID: 35512425
The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature.
Saletti V, Farinotti M, Peretta P, Massimi L, Ciaramitaro P, Motta S, Solari A, Valentini LG
Neurol Sci 2021 Dec;42(12):4965-4995. Epub 2021 Sep 30 doi: 10.1007/s10072-021-05565-9. PMID: 34591209
Chiari Malformation Type 1: A Systematic Review of Natural History and Conservative Management.
Langridge B, Phillips E, Choi D
World Neurosurg 2017 Aug;104:213-219. Epub 2017 Apr 21 doi: 10.1016/j.wneu.2017.04.082. PMID: 28435116
Acquired Chiari Malformation and Syringomyelia Secondary to Space-Occupying Lesions: A Systematic Review.
Wang J, Alotaibi NM, Samuel N, Ibrahim GM, Fallah A, Cusimano MD
World Neurosurg 2017 Feb;98:800-808.e2. Epub 2016 Nov 25 doi: 10.1016/j.wneu.2016.11.080. PMID: 27894943
A Systematic Review of Chiari I Malformation: Techniques and Outcomes.
Zhao JL, Li MH, Wang CL, Meng W
World Neurosurg 2016 Apr;88:7-14. Epub 2015 Dec 28 doi: 10.1016/j.wneu.2015.11.087. PMID: 26732952

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