Celiac artery compression syndrome
Synonyms: Dunbar syndrome · MALS · Median arcuate ligament syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare disease caused by compression of the celiac axis by an abnormally shaped arcuate ligament (the part of the diaphragm in which both pillars join in the midline around the aorta). Patients have recurrent abdominal pain, anorexia and weight loss. The pain is epigastric, and diarrhea or constipation may be present as well. Onset of pain will usually, although not always, be after food intake, and may be associated with nausea and emesis. Other symptoms may include lassitude, exercise intolerance and vomiting. Occasionally, a patient may show an abdominal murmur upon auscultation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Celiac artery compression syndrome | Dunbar syndrome · MALS · Median arcuate ligament syndrome |
| fr | Syndrome de compression du tronc coeliaque | Syndrome de Dunbar · Syndrome du ligament arqué médian |
| de | Arteria-coeliaca-Kompressions-Syndrom | Dunbar-Syndrom · Ligamentum arcuatum medianum-Syndrom · MALS |
| es | Síndrome de compresión de la arteria celíaca | MALS · Síndrome de Dunbar · Síndrome del ligamento arcuato medio |
| it | Sindrome da compressione del tronco celiaco | |
| nl | Compressie van truncus coeliacus-syndroom | Ligamentum arcuatum medianum-syndroom · MALS · Mediaan gebogen ligament-syndroom · Syndroom van Dunbar |
| pt | Síndrome de compressão do tronco celíaco | |
| pl | Zespół ucisku pnia trzewnego | = en |
| cs | Syndrom komprese truncus coeliacus | Syndrom komprese celiakální arterie · Syndrom ligamentum arcuatum medianum · Dunbarův syndrom |
| uk | Синдром здавлення чревної артерії | Синдром Данбара · MALS · Синдром серединної дугоподібної звязки |
| zh | 腹腔动脉压迫综合征 | Dunbar综合征 |
| tr | Çölyak arter kompresyon sendromu | MALS · Dunbar sendromu · Median arkuat ligament sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I77.4 | E | specific | validated |
| ICD-11 | BD52.5 | E | specific | validated |
| MONDO | 0017388 | E | validated | |
| MONDO | 17388 | E | validated | |
| UMLS | C1861783 | E | validated | |
| GARD | 12308 | E | validated | |
| MedDRA | 10009838 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:165711Rare abdominal surgical disease
rare abdominal surgical diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | 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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/293208
- This record as JSON: /data/d/293208.json