raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:400

Cystic echinococcosis

Synonyms: Hydatid cyst · Hydatid disease · Hydatidosis

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

A rare parasitic disease characterized by slowly enlarging cysts of the liver, lungs and other organs which often go unnoticed for years. They are caused principally by the larval form (also called metacestode) of Echinococcus granulosus sensu lato tapeworm. The adult form of this tapeworm parasitizes the intestine of dogs. Cystic echinococcosis (formerly hydatidosis) generally affects large domestic herbivores and humans are dead-end hosts, infected through contact with herding dogs or through ingestion of food contaminated with canine excrement.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCystic echinococcosisHydatid cyst · Hydatid disease · Hydatidosis
frEchinococcose kystiqueHydatidose · Kyste hydatique · Maladie hydatique
deZystische EchinokokkoseHydatidenzyste · Hydatidose
esEquinococosis quísticaEnfermedad hidatídica · Hidatidosis · Quiste hidatídico
itEchinococcosi cisticaIdatidosi · Malattia idatidea · Cisti idatidea
nlCystische echinokokkoseCysteuze echinokokkose · Hydatidencyste · Hydatidenziekte · Hydatidose · Hydatidosis
ptEquinococose quísticaDoença hidática · Hidatidose · Quisto hidático
plBąblowica= en
csCystická echinokokózaHydatidová nemoc · Hydatidóza
ukКістозний ехінококозКіста гідатиди · Хвороба Гідатіда · Гідратидоз
zh囊型包虫病棘球蚴病
trKistik ekinokokkozHidatidoz · Hidatik hastalığı

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10B67.0BTNTspecificvalidated
ICD-10B67.1BTNTspecificvalidated
ICD-10B67.2BTNTspecificvalidated
ICD-10B67.3BTNTspecificvalidated
ICD-10B67.4BTNTspecificvalidated
ICD-10B67.5BTNTspecificvalidated
ICD-10B67.6BTNTspecificvalidated
ICD-10B67.7BTNTspecificvalidated
ICD-10B67.8BTNTspecificvalidated
ICD-10B67.9BTNTspecificvalidated
ICD-111F73.ZNTBTindexvalidated
MONDO0018408Evalidated
MeSHD004443Evalidated
UMLSC4553297Evalidated
GARD2764Evalidated
MedDRA10014096Evalidated

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

2 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 100 0001 / 100 000EuropeValue and classValidated[EXPERT]
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET

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

41 annotations · validated 2020-04-15
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0001407Hepatic cysts
HP:0001880Increased total eosinophil count
Frequent (79-30%)
HP:0002611Cholestatic liver disease
HP:0410019Epigastric pain
HP:0012378Fatigue
HP:0010702Increased circulating immunoglobulin concentration
HP:0001824Weight loss
Occasional (29-5%)
HP:0011458Abdominal symptom
HP:0031983Abnormal pulmonary thoracic imaging finding
HP:0000775Abnormality of the diaphragm
HP:0025615Abscess
HP:0100845Anaphylactic shock
HP:0005230Biliary tract obstruction
HP:0003155Elevated circulating alkaline phosphatase concentration
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0030948Elevated gamma-glutamyltransferase level
HP:0002240Hepatomegaly
HP:0002904Hyperbilirubinemia
HP:0005948Multiple pulmonary cysts
HP:0032445Pulmonary cyst
HP:0032101Unusual infection
Very rare (<4-1%)
HP:0001627Abnormal heart morphology
HP:0031630Abnormal subpleural morphology
HP:0000478Abnormality of the eye
HP:0003011Abnormality of the musculature
HP:0001732Abnormality of the pancreas
HP:0002585Abnormality of the peritoneum
HP:0045058Abnormality of the testis size
HP:0000925Abnormality of the vertebral column
HP:0002099Asthma
HP:0012062Bone cyst
HP:0010576Intracranial cystic lesion
HP:0031700Invasive parasitic infection
HP:0000952Jaundice
HP:0011355Localized skin lesion
HP:0012578Membranous nephropathy
HP:0000138Ovarian cyst
HP:0100592Peritoneal abscess
HP:0000107Renal cyst
HP:0030423Splenic cyst
HP:0001025Urticaria

Run the differential on the 2 obligate / very frequent terms

Annotation source: 26677245[PMID]_14575976[PMID]

7

Sources for this card