Mirizzi syndrome
Synonyms: Extrinsic biliary compression syndrome
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare biliary tract disease characterized by external compression and subsequent obstruction of an extrahepatic biliary duct by one or more gallstones in the cystic duct or the gallbladder. Patients may present with acute or chronic cholecystitis with right upper abdominal pain, nausea, and vomiting, jaundice, or cholangitis. Cholecystobiliary or -enteric fistulae can arise due to chronic inflammation and ulceration.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mirizzi syndrome | Extrinsic biliary compression syndrome |
| fr | Syndrome de Mirizzi | Syndrome de compression extrinsèque de la voie biliaire principale |
| de | Mirizzi-Syndrom | Extrinsische Gallenwegskompression |
| es | Síndrome de Mirizzi | Síndrome de compresión biliar extrínseca |
| it | Sindrome di Mirizzi | |
| nl | Syndroom van Mirizzi | Extrinsieke biliaire compressie-syndroom |
| pt | Síndrome Mirizzi | |
| pl | Zespół Mirizziego | = en |
| cs | Mirizziho syndrom | = en |
| uk | Синдром Міріцці | Синдром зовнішньої компресії жовчних шляхів |
| zh | 米里齐综合征 | 胆道外压迫综合症 |
| tr | Mirizzi sendromu | Ekstrinsik biliyer kompresyon sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K80.8 | NTBT | attributed | not yet validated |
| ICD-11 | DC11.Y | NTBT | index | validated |
| MONDO | 0043330 | E | validated | |
| MeSH | D057792 | E | validated | |
| UMLS | C0267878 | E | validated | |
| GARD | 10177 | 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:101941Rare biliary tract disease
rare hepatic 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 | Validated | ORPHANET_29494098[PMID] |
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
20 annotations · validated 2021-06-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001081 | Cholelithiasis | |
| HP:0011980 | Cholesterol gallstones | |
| HP:0040319 | Dark urine | |
| HP:0003155 | Elevated circulating alkaline phosphatase concentration | |
| HP:0002904 | Hyperbilirubinemia | |
| HP:0000952 | Jaundice | |
| Occasional (29-5%) | ||
| HP:0011848 | Abdominal colic | |
| HP:0003270 | Abdominal distention | |
| HP:0002039 | Anorexia | |
| HP:0025143 | Chills | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0410019 | Epigastric pain | |
| HP:0001945 | Fever | |
| HP:0002018 | Nausea | |
| HP:0001733 | Pancreatitis | |
| HP:0001649 | Tachycardia | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0100889 | Abnormality of the ductus choledochus | |
| HP:0030154 | Gallbladder perforation | |
Annotation source: 29494098[PMID]_28653000[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/521219
- This record as JSON: /data/d/521219.json