LA04.0

ICD-11 MMS 2026-01

Hydrocephalus with stenosis of the aqueduct of Sylvius

Description

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).

Index terms

  • Hydrocephalus with stenosis of the aqueduct of Sylvius
  • Atresia of the aqueduct of Sylvius
  • Bicker-Adams syndrome
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  • Stenosis of the aqueduct of Sylvius
  • anomaly of aqueduct of sylvius
  • aqueduct of Sylvius stricture
  • aqueduct of sylvius septum
  • congenital aqueduct of Sylvius occlusion
  • congenital obstruction of aqueduct of Sylvius
  • congenital stenosis of aqueduct of Sylvius
  • deformity of aqueduct of sylvius
  • hydrocephalus with aqueduct of Sylvius stricture

Inclusions

  • Stenosis of the aqueduct of Sylvius

Exclusions from above levels

  • KA64.0 – Hydrocephalus due to congenital toxoplasmosis
  • LA02.00 – Myelomeningocele with hydrocephalus
  • LA03 – Arnold-Chiari malformation type II
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  • LA07.4 – Arnold-Chiari malformation type I
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA04.Y – Other specified congenital hydrocephalus
  • LA04.Z – Congenital hydrocephalus, unspecified

Foundation entity

Hydrocephalus with stenosis of the aqueduct of Sylvius – http://id.who.int/icd/entity/1284135636

Part of: LA04: Congenital hydrocephalus › 20: Developmental anomalies