raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:583861

Isolated mesenteric vein thrombosis

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adult, Elderly
Status
active

A rare intestinal disease characterized by isolated thrombosis of the mesenteric veins without involvement of the splenic or portal veins, potentially causing mesenteric ischemia. It may be primary/idiopathic or due to one or more local or systemic predisposing conditions, and may be acute/subacute or chronic, the latter being used for patients with symptoms lasting longer than 4 weeks but without bowel infarction, or patients without recent symptoms, where the thrombosis is an incidental finding. Symptoms depend on the extent and severity of thrombosis and often involve prolonged nonspecific abdominal complaints.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enIsolated mesenteric vein thrombosis
frThrombose isolée de la veine mésentérique
deMesenterialvenenthrombose, isolierte
esTrombosis de la vena mesentérica aislada
itTrombosi isolata della vena mesenterica
nlGeïsoleerde trombose van vena mesenterica
ptTrombose isolada da veia mesentérica
plIzolowana zakrzepica żyły krezkowej
csIzolovaná trombóza mezenterické žíly
ukІзольований тромбоз брижової вени
zh孤立性肠系膜静脉血栓
trİzole mezenterik ven trombozu

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10K55.0NTBTinclusionvalidated
ICD-11DD30.2Especificvalidated
MONDO0035009Evalidated
UMLSC0267412Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

4 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 100 0001.6 / 100 000EuropeValue and classValidated18720461[PMID]_25917534[PMID]
Annual incidence1-9 / 1 000 0000.5 / 100 000FinlandValue and classValidated25917534[PMID]
Annual incidence1-9 / 100 0002.7 / 100 000SwedenValue and classValidated18720461[PMID]
Point prevalenceUnknown—EuropeClass onlyValidated18720461[PMID]_25917534[PMID]_ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

19 annotations · validated 2024-09-26
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0002027Abdominal pain
HP:0030248Mesenteric venous thrombosis
Frequent (79-30%)
HP:0002039Anorexia
HP:0002014Diarrhea
HP:0001974Increased total leukocyte count
HP:0002018Nausea
HP:0002013Vomiting
Occasional (29-5%)
HP:0003270Abdominal distention
HP:0410282Abnormal circulating amylase concentration
HP:0001541Ascites
HP:0030151Cholangitis
HP:0001081Cholelithiasis
HP:0001945Fever
HP:0002239Gastrointestinal hemorrhage
HP:0002615Hypotension
HP:0002151Increased circulating lactate concentration
HP:6000377Intramural intestinal gas
HP:0100806Sepsis
HP:0031273Shock

Run the differential on the 2 obligate / very frequent terms

Annotation source: 29083683[PMID]_25755568[PMID]_26285602[PMID]

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Sources for this card