raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:645393

Hemi-myeloschisis

Synonyms: Split cord malformation associated with myeloschisis

Type
Clinical subtype
Group
Subtype of disorder
Inheritance
Unknown
Status
active

A very rare form of composite dysraphism characterized by the presence of a split cord malformation and a myeloschisis on one of the two hemicords. Hemicords can be in a single dural sac or in two separated dural sacs. Other spinal cord malformations can be associated. Due to the comparable prognosis it is considered as a subtype of myeloschisis.

1

Names in Orphadata languages

10 of 12 languages
LangNameSynonyms
enHemi-myeloschisisSplit cord malformation associated with myeloschisis
frHémi-myéloschisisMoelle épinière dédoublée associée à un myéloschisis
deHemimyeloschisisSplit-Cord-Malformation mit assoziierter Myeloschisis
esHemimielosquisisMalformación del cordón espinal hendido asociada con mielosquisis
itEmi-mieloschisiMalformazione da fissurazione della corda spinale con mieloschisi
nlHemi-myeloschisis'Split cord'-malformatie geassocieerd met myeloschisis · Hemimyeloschisis
ptHemi-mielosquiseEspinha bífida associada a mielosquise
plRozszczep rdzenia kręgowego z ekspozycją zewnętrzną jednej jego części= en
csHemi-myeloschíza= en
ukГемімієлошизисРозщеплення пуповини, повязане з мієлошизисом

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
MONDO0958351Evalidated
UMLSC5816709Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

  • ORPHA:645398Myeloschisis
    rare developmental anomalies during embryogenesis; rare genetic diseases; rare neurological diseases

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card