Congenital pancreatic cyst
Synonyms: Neonatal congenital pancreatic cyst · True congenital pancreatic cyst
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Infancy, Neonatal
- Status
- active
A rare pancreatic disease characterized by a most commonly single, unilocular, thin-walled cystic lesion which may be located anywhere within the pancreas (but is more frequently found in the body and tail) and does not communicate with the pancreatic ductal system. Patients may be asymptomatic or present with signs and symptoms of gastrointestinal or biliary obstruction, or pancreatitis. The condition can be isolated or occur in association with other anomalies (such as von Hippel-Lindau disease or polycystic kidney disease).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital pancreatic cyst | Neonatal congenital pancreatic cyst · True congenital pancreatic cyst |
| fr | Kyste pancréatique congénital | Kyste congénital vrai du pancréas · Kyste pancréatique congénital néonatal |
| de | Pankreaszysten, kongenitale | Pankreaszysten, echte kongenitale · Pankreaszysten, kongenitale neonatale |
| es | Quiste pancreático congénito | Quiste pancreático congénito neonatal · Quiste verdadero congénito del páncreas |
| it | Cisti pancreatica congenita | Cisti pancreatica congenita neonatale · Cisti pancreatica congenita vera |
| nl | Congenitale cyste van pancreas | Echte congenitale cyste van pancreas · Neonatale congenitale cyste van pancreas |
| pt | Quisto pancreático congénito | Quisto pancreático congénito neonatal · Quisto pancreático verdadeiramente congénito |
| pl | Wrodzona torbiel trzustki | Noworodkowa wrodzona torbiel trzustki · Prawdziwa wrodzona torbiel trzustki |
| cs | Vrozené cysty pankreatu | Neonatální vrozené pankreatické cysty · Pravé vrozené pankreatické cysty |
| uk | Вроджена кіста підшлункової залози | Неонатальна вроджена кіста підшлункової залози · Істинна вроджена кіста підшлункової залози |
| zh | 先天性胰腺囊肿 | 新生儿先天性胰腺囊肿 |
| tr | Doğumsal pankreas kisti | Gerçek doğumsal pankreas kisti · Yenidoğan doğumsal pankreas kisti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q45.2 | E | specific | validated |
| ICD-11 | LB21.Y | NTBT | index | validated |
| MONDO | 0017783 | E | validated | |
| UMLS | C0341480 | 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 (2):
- ORPHA:506216Rare disorder potentially indicated for bowel transplant
rare transplant-related disorders - ORPHA:101937Rare pancreatic disease
rare gastroenterological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 10 cases | Worldwide | Case(s) | Validated | 19231513[PMID]_2202976[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | 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
6 annotations · validated 2016-06-30| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Occasional (29-5%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0002027 | Abdominal pain | |
| HP:0002039 | Anorexia | |
| HP:0000952 | Jaundice | |
| HP:0001733 | Pancreatitis | |
| HP:0002013 | Vomiting | |
Annotation source: 27088671[PMID]_19231513[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/313906
- This record as JSON: /data/d/313906.json