Hydrocephalus with stenosis of the aqueduct of Sylvius

ICD-11 code : LA04.0;

Preferred Label : Hydrocephalus with stenosis of the aqueduct of Sylvius;

ICD-11 definition : Hydrocephalus with stenosis of aqueduct of Sylvius (HSAS) or Bickers-Adams syndrome is characterised by the association of hydrocephaly, severe intellectual deficit, spasticity and adducted thumbs, and is part of the L1 syndrome (see this term).;

ICD-11 synonym : aqueduct of sylvius septum; anomaly of aqueduct of sylvius; Atresia of the aqueduct of Sylvius; Stenosis of the aqueduct of Sylvius; congenital obstruction of aqueduct of Sylvius; Bicker-Adams syndrome; deformity of aqueduct of sylvius; hydrocephalus with aqueduct of Sylvius stricture; congenital aqueduct of Sylvius occlusion; congenital stenosis of aqueduct of Sylvius; aqueduct of Sylvius stricture;

ICD-11 inclusion : Stenosis of the aqueduct of Sylvius;

Details

Origin ID

1284135636;

UMLS CUI

C0265216;

Automatic exact mappings (from CISMeF team)
- Aqueductal stenosis [HPO term]
- Congenital obstruction of aqueduct of Sylvius (disorder) [SNOMED CT concept]
- Congenital stenosis of aqueduct of Sylvius (disorder) [SNOMED CT concept]
- Malformations of aqueduct of Sylvius [ICD-10 Sub-category]
- Stenosis of aqueduc of Sylvius [HRDO Sign]
- congenital obstruction of aqueduct of sylvius [SNOMED Notion]
- congenital stenosis of aqueduct of sylvius [SNOMED Notion]
Currated CISMeF NLP mapping
- Hydrocephalus with stenosis of the aqueduct of Sylvius [ORDO Disease]
- Hydrocephalus, congenital, X-linked [OMIM Phenotype]
ICD-10 Mapping
- Malformations of aqueduct of Sylvius [ICD-10 Sub-category]
See also inter- (CISMeF)
- Aqueduct of Sylvius anomaly (disorder) [SNOMED CT concept]
- Congenital atresia of aqueduct of Sylvius (disorder) [SNOMED CT concept]
- aqueduct of sylvius anomaly [SNOMED Notion]
Semantic type(s)
- Disease or Syndrome [UMLS semantic type]
UMLS correspondences (same concept)
- Hydrocephalus due to congenital stenosis of aqueduct of sylvius [OMIM Phenotype]
- Hydrocephalus with stenosis of the aqueduct of Sylvius [ORDO Disease]
- Hydrocephalus, X-linked [MeSH concept]
- Hydrocephalus, congenital, X-linked [OMIM Phenotype]
- X-linked hydrocephalus syndrome (disorder) [SNOMED CT concept]
- hydrocephalus, X-linked [MeSH Supplementary Concept]
- x-linked hydrocephalus syndrome [SNOMED Notion]

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