Airway infantile hemangioma
Synonyms: Airway infantile haemangioma · Laryngotracheal angioma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy
- Status
- active
A rare benign vascular tumor characterized by rapid growth after birth (followed by spontaneous partial regression over the course of years), potentially leading to life-threatening airway obstruction due to the subglottic location. Patients present with respiratory symptoms including biphasic stridor, recurrent croup, cyanosis, apnea, and sternal and intercostal retractions. The tumor may be accompanied by cutaneous hemangiomata, especially in the lower facial ("beard") distribution.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Airway infantile hemangioma | Airway infantile haemangioma · Laryngotracheal angioma |
| fr | Hémangiome infantile des voies respiratoires | Angiome laryngotrachéal |
| de | Infantiles Hämangiom der Atemwege | Angiom, laryngotracheales |
| es | Hemangioma infantil de las vías respiratorias | Angioma laringotraqueal |
| it | Emangioma infantile delle vie aeree | Angioma laringotracheale · Emangioma infantile delle vie aeree |
| nl | Infantiel hemangioom van luchtwegen | Laryngotracheaal angioom |
| pt | Angioma laringotraqueal | |
| pl | Naczyniak krtaniowo-tchawiczy | = en |
| cs | Laryngotracheální angiom | = en |
| uk | Інфантильна гемангіома дихальних шляхів | Ангіома ларинготрахеальної артерії |
| zh | 喉气管血管瘤 | |
| tr | Laringotrakeal anjiyom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D18.0 | NTBT | attributed | validated |
| MONDO | 0015317 | E | validated | |
| UMLS | C3274531 | E | validated | |
| GARD | 19893 | 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:156249Larynx anomaly
rare developmental anomalies during embryogenesis; rare otorhinolaryngological diseases - ORPHA:210589Rare infantile hemangioma
rare developmental anomalies during embryogenesis; rare circulatory system diseases; rare neoplastic 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
13 annotations · validated 2019-06-25| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0010307 | Stridor | |
| Frequent (79-30%) | ||
| HP:0012735 | Cough | |
| HP:0011968 | Feeding difficulties | |
| HP:0030864 | Intercostal retractions | |
| HP:0002098 | Respiratory distress | |
| Occasional (29-5%) | ||
| HP:0000961 | Cyanosis | |
| HP:0000750 | Delayed speech and language development | |
| HP:0000329 | Facial hemangioma | |
| HP:0001609 | Hoarse voice | |
| HP:0030828 | Wheezing | |
| Very rare (<4-1%) | ||
| HP:0002104 | Apnea | |
| HP:0002360 | Sleep abnormality | |
| HP:0002013 | Vomiting | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 29706817[PMID]_21880377[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/137935
- This record as JSON: /data/d/137935.json