hydrocephalus with stenosis of the aqueduct of sylvius

ORPHA: 2182

Overview

historical term for L1 syndrome

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with hydrocephalus with stenosis of the aqueduct of sylvius, sourced from HPO and Orphanet clinical annotations.

HydrocephalusCoarse facial featuresStrabismusNystagmusAdducted thumbSeizureSpasticityAgenesis of corpus callosumAbsent septum pellucidumHoloprosencephalyJoint stiffnessAqueductal stenosisIncreased intracranial pressureHemiplegia/hemiparesisSevere intellectual disability

Classification & Codes

Orphanet Code

ORPHA:2182
hydrocephalus with stenosis of the aqueduct of sylvius
OrphanetORPHA:2182
Treatments0 drug(s)
Symptoms on record15 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO