rhombencephalosynapsis

ORPHA: 59315

Overview

Rhombencephalosynapsis (RS) is a rare malformation of the cerebellum characterised by the association of agenesis (total or partial) of the vermis and fusion of the cerebellar hemispheres

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 rhombencephalosynapsis, sourced from HPO and Orphanet clinical annotations.

Abnormality of the uterusNarrow mouthHydrocephalusMacrocephalyAbnormality of the faceMicroretrognathiaHypertelorismAnteverted naresAbnormality of the eyeAbnormality of visionIntellectual disabilityAtaxiaAbnormality of the cardiovascular systemAnal atresiaEsophageal atresiaVentriculomegalyAganglionic megacolonAgenesis of cerebellar vermisTracheoesophageal fistulaShort noseFinger syndactylyFusion of the cerebellar hemispheresShort phalanx of fingerComplete duplication of thumb phalanxPolydactylyFusion of the left and right thalamiAbnormal renal morphologyAbnormal dentate nucleus morphologySepto-optic dysplasiaPosteriorly rotated ears

Classification & Codes

Orphanet Code

ORPHA:59315
rhombencephalosynapsis
OrphanetORPHA:59315
Treatments0 drug(s)
Symptoms on record30 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO