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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

Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery.
Dien Esquivel MF, Gupta N, Wilson N, O'Brien CA, Gladkikh M, Barrowman N, Bijelić V, Tu A
Childs Nerv Syst 2022 Nov;38(11):2119-2128. Epub 2022 Aug 12 doi: 10.1007/s00381-022-05644-y. PMID: 35962220
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 I malformation in children-the natural history.
Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA Jr
Childs Nerv Syst 2019 Oct;35(10):1793-1799. Epub 2019 Jul 30 doi: 10.1007/s00381-019-04310-0. PMID: 31363830
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
Complex Chiari Malformations in Children: Diagnosis and Management.
Brockmeyer DL, Spader HS
Neurosurg Clin N Am 2015 Oct;26(4):555-60. Epub 2015 Aug 4 doi: 10.1016/j.nec.2015.06.002. PMID: 26408065

Diagnosis

Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery.
Dien Esquivel MF, Gupta N, Wilson N, O'Brien CA, Gladkikh M, Barrowman N, Bijelić V, Tu A
Childs Nerv Syst 2022 Nov;38(11):2119-2128. Epub 2022 Aug 12 doi: 10.1007/s00381-022-05644-y. PMID: 35962220
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 1 deformity in children: etiopathogenesis and radiologic diagnosis.
Raybaud C, Jallo GI
Handb Clin Neurol 2018;155:25-48. doi: 10.1016/B978-0-444-64189-2.00002-0. PMID: 29891063

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
The Management of Idiopathic and Refractory Syringomyelia.
Gallo P, Kaliaperumal C
Adv Tech Stand Neurosurg 2022;45:317-338. doi: 10.1007/978-3-030-99166-1_10. PMID: 35976455
Posterior fossa decompression for children with Chiari I malformation and hydrocephalus.
Koueik J, DeSanti RL, Iskandar BJ
Childs Nerv Syst 2022 Jan;38(1):153-161. Epub 2021 Oct 20 doi: 10.1007/s00381-021-05377-4. PMID: 34671850
Chiari I malformation-neuropsychological functions and quality of life.
Almotairi FS, Hellström P, Skoglund T, Nilsson ÅL, Tisell M
Acta Neurochir (Wien) 2020 Jul;162(7):1575-1582. Epub 2019 Apr 6 doi: 10.1007/s00701-019-03897-2. PMID: 30955083
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

Histological and intraoperative evaluations of the arachnoid in patients with Chiari I malformation.
Heidary M, Respondek M, Klekamp J
Acta Neurochir (Wien) 2021 Jan;163(1):219-225. Epub 2020 Oct 6 doi: 10.1007/s00701-020-04582-5. PMID: 33025091
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 malformation in children-the natural history.
Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA Jr
Childs Nerv Syst 2019 Oct;35(10):1793-1799. Epub 2019 Jul 30 doi: 10.1007/s00381-019-04310-0. PMID: 31363830
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

Sella Volume and Posterior Fossa Morphometric Measurements in Chiari Type 1.
Bas G, Ozkara E, Ozbek Z, Naderi S, Arslantas A
Turk Neurosurg 2023;33(2):290-295. doi: 10.5137/1019-5149.JTN.40088-22.4. PMID: 36799276
Chiari 1 Formation Redefined-Clinical and Radiographic Observations in 388 Surgically Treated Patients.
Goel A, Jadhav D, Shah A, Rai S, Dandpat S, Vutha R, Dhar A, Prasad A
World Neurosurg 2020 Sep;141:e921-e934. Epub 2020 Jun 17 doi: 10.1016/j.wneu.2020.06.076. PMID: 32562905
Chiari I malformation in children-the natural history.
Chatrath A, Marino A, Taylor D, Elsarrag M, Soldozy S, Jane JA Jr
Childs Nerv Syst 2019 Oct;35(10):1793-1799. Epub 2019 Jul 30 doi: 10.1007/s00381-019-04310-0. PMID: 31363830
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
Chiari Malformations and Syringohydromyelia in Children.
Poretti A, Boltshauser E, Huisman TA
Semin Ultrasound CT MR 2016 Apr;37(2):129-42. Epub 2015 Dec 10 doi: 10.1053/j.sult.2015.12.001. PMID: 27063663

Recent systematic reviews

Surgical management of Chiari II malformation: a systematic review of literature.
Vencio RCC, Lino-Filho AM, Bonfim RCF, Pereira NM, Franco CL, Ribeiro PRJ, Morais BA
Childs Nerv Syst 2024 Jul;40(7):2093-2100. Epub 2024 Mar 27 doi: 10.1007/s00381-024-06368-x. PMID: 38536450
Posterior fossa decompression with duraplasty in Chiari malformation type 1: a systematic review and meta-analysis.
Tam SKP, Brodbelt A, Bolognese PA, Foroughi M
Acta Neurochir (Wien) 2021 Jan;163(1):229-238. Epub 2020 Jun 23 doi: 10.1007/s00701-020-04403-9. PMID: 32577895
Comparison of Results Between Posterior Fossa Decompression with and without Duraplasty for the Surgical Treatment of Chiari Malformation Type I: A Systematic Review and Meta-Analysis.
Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B
World Neurosurg 2018 Feb;110:460-474.e5. Epub 2017 Nov 11 doi: 10.1016/j.wneu.2017.10.161. PMID: 29138073
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
The case for duraplasty in adults undergoing posterior fossa decompression for Chiari I malformation: a systematic review and meta-analysis of observational studies.
Förander P, Sjåvik K, Solheim O, Riphagen I, Gulati S, Salvesen Ø, Jakola AS
Clin Neurol Neurosurg 2014 Oct;125:58-64. Epub 2014 Jul 21 doi: 10.1016/j.clineuro.2014.07.019. PMID: 25087160

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