Kommerell diverticulum
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
Kommerell diverticulum (KD) is a developmental anomaly of the aortic arch characterized by a diverticulum at the proximal descending aorta of left or right arch configuration that gives rise to an aberrant subclavian artery. KD is primarily asymptomatic but may become symptomatic secondary to dilatation of KD, atheroma and fibrotic changes in paratracheal or paraesophageal tissue, presenting with signs of tracheal compression (more common in children), esophageal compression (dysphagia lusoria; more common in patients with a right sided aortic arch), chest pain, or blood pressure difference in the upper limbs. KD may also predispose toward aortic dissection or rupture.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Kommerell diverticulum | |
| fr | Diverticule de Kommerel | |
| de | Kommerell-Divertikel | |
| es | Divertículo de Kommerell | |
| it | Diverticolo di Kommerell | |
| nl | Divertikel van Kommerell | |
| pt | Divertículo de Kommerell | |
| pl | Uchyłek Kommerella | |
| cs | Kommerellův divertikl | |
| uk | Дивертикул Коммереля | |
| zh | Kommerell憩室 | |
| tr | Kommerell divertikülü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q25.4 | NTBT | attributed | validated |
| ICD-11 | LA8B.2Y | NTBT | index | validated |
| MONDO | 0020415 | E | validated | |
| UMLS | C0265885 | E | validated | |
| MedDRA | 10081282 | 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:1132Aortic arch defects
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99077
- This record as JSON: /data/d/99077.json