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Chiari malformation type II (CUI C0555206) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0555206

Semantic Type: Congenital Abnormality

Semantic Type: Disease or Syndrome

GARD Definition: Chiari malformation type 2 (CM type II) is a type of Chiari malformation in which both the cerebellum and brain stem tissue extend into the foramen magnum (the hole at the skull base for passing of the spinal cord).  This form is often accompanied by a type of spina bifida called myelomeningocele, and can also be accompanied by syringomyelia, hydrocephalus, or other abnormalities. Symptoms in infants may include stridor (wheezing sound); difficulty swallowing (dysphagia); feeding difficulties; hypotonia; and weak cry. Symptoms in children and/or adults may include headache; fatigue; loss of vision; tingling extremities; nausea; dysphagia; dizziness; muscle weakness; and ataxia. Adults and adolescents who previously had no symptoms may begin to have symptoms later in life. The exact cause of the condition is not known but it appears to be due to a developmental failure of the brain stem and upper spine. The term Arnold-Chiari malformation is technically specific to type II but may sometimes be used to describe other types of Chiari malformations.
- this information is from GARD/ORDR/NCATS.

Synonyms & Abbreviations: (see Synonym Details)
Arnold Chiari malformation type II
Arnold Chiari Malformation, Type 2
Arnold Chiari Malformation, Type II
Arnold Chiari type 2
Arnold-Chiari malformation type 2
Arnold-Chiari malformation type II
Arnold-Chiari Malformation, Type 2
Arnold-Chiari Malformation, Type II
Arnold-Chiari malformation
Arnold-Chiari syndrome, type II
Arnold-Chiari syndrome
Chiari malformation type 2
Chiari malformation type II (disorder)
Chiari malformation type II
Chiari malformation, type II
Chiari type II malformation
CM2
Type II Arnold Chiari Malformation
Type II Arnold-Chiari Malformation

External Source Codes:  (none)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
CASE_SIGNIFICANCE_ID 900000000000017005 SNOMEDCT_US
CTV3ID XUWKv SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DISEASE_IDENTIFIER_ID 30392 GARD
DISEASE_IDENTIFIER_ID 30393 GARD
DISEASE_IDENTIFIER_ID 3789 GARD
DISEASE_IDENTIFIER_ID 3790 GARD
DISEASE_IDENTIFIER_ID 3791 GARD
DISEASE_IDENTIFIER_ID 3792 GARD
EFFECTIVE_TIME 20020731 SNOMEDCT_US
HAS_GARD_PAGE true GARD
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_DATE 2010-02-04T00:00:00 GARD
MIMTYPE 5 OMIM
MIMTYPEMEANING Mendelian phenotype or locus, molecular basis unknown. OMIM
MIMTYPEVALUE perc OMIM
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/9232/chiari-malformation-type-2/resources/1 GARD
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 14.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMEDID D4-9006D SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS Q07.0 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~Q07.0 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS Q07.00 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~IF HYDROCEPHALUS DUE TO ARNOLD CHIARI MALFORMATION TYPE 2 CHOOSE Q07.02 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447639009 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~2 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~IFA 23931000119104 | Hydrocephalus due to Arnold Chiari malformation type 2 (disorder) | SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~OTHERWISE TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~Q07.00 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~Q07.02 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUWKv SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~D4-9006D SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T372014 MSH
TERMUI T372015 MSH
TERMUI T751421 MSH
TERMUI T827616 MSH
TERMUI T851470 MSH
TH NLM (2000) MSH
TH NLM (2010) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0555206

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