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Chiari malformation type III

MedGen UID:
148202
•Concept ID:
C0750931
•
Congenital Abnormality
Synonyms: Arnold Chiari malformation type III; Chiari malformation type 3
SNOMED CT: Arnold Chiari type 3 (253186001); Chiari malformation type III (253186001)
 
HPO: HP:0025661
Monarch Initiative: MONDO:0022715

Definition

A type of Chiari malformation that involves herniation of the hindbrain (cerebellum with or without the brainstem) into a low occipital or high cervical meningoencephalocele. [from HPO]

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

Diagnosis

Chiari type III malformation associated with Klippel-Feil syndrome, a case report with a narrative review of the literature.
Althomali MH, Aljohani OI, Sabbagh AJ
Childs Nerv Syst 2024 Feb;40(2):581-586. Epub 2023 Oct 28 doi: 10.1007/s00381-023-06198-3. PMID: 37897525
Chiari malformation type III treated with primary closure.
Jaggi RS, Premsagar IC
Pediatr Neurosurg 2007;43(5):424-7. doi: 10.1159/000106397. PMID: 17786013

Prognosis

Chiari type III malformation associated with Klippel-Feil syndrome, a case report with a narrative review of the literature.
Althomali MH, Aljohani OI, Sabbagh AJ
Childs Nerv Syst 2024 Feb;40(2):581-586. Epub 2023 Oct 28 doi: 10.1007/s00381-023-06198-3. PMID: 37897525
Chiari malformation type III and results of surgery: a clinical study: report of eight surgically treated cases and review of the literature.
Işik N, Elmaci I, Silav G, Celik M, Kalelioğlu M
Pediatr Neurosurg 2009;45(1):19-28. Epub 2009 Feb 17 doi: 10.1159/000202620. PMID: 19221459

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