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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVChiari malformation
- CROGVChiari malformation type III
- CROGVChiari malformation type IV
- CROGVChiari type I malformation
- CROGVChiari type II malformation

Abnormal brain morphology
- Abnormal hindbrain morphology
  - Abnormal metencephalon morphology
    - Abnormal cerebellum morphology
      - Cerebellar malformation
        Chiari malformation
        Chiari malformation type III

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: 35465876 Free 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: 31068690 Free 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

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Recent clinical studies

Diagnosis

Chiari malformation type III and its viability. Case report and literature review.

Vázquez Sufuentes S, Esteban García J, Casado Pellejero J, Curto Simón B, Fustero de Miguel D
Neurochirurgie 2024 Nov;70(6):101585. Epub 2024 Aug 7 doi: 10.1016/j.neuchi.2024.101585. PMID: 39121943

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

See all (3)