Spontaneous intestinal perforation
Synonyms: FIP · Focal intestinal perforation · Isolated perforation · Neonatal focal intestinal perforation · SIP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare intestinal disease characterized by a single, focal intestinal perforation, associated with hemorrhagic necrosis, typically occurring at the terminal ileum, involving antimesenteric border. It may also occur in the jejunum or colon. It predominantly affects very (or extremely) low weight infants (birth weight less than 1500 g) mostly in the first week of life. Patients have healthy bowel apart from the perforation site, they present with bluish discoloration and gasless abdomen in the absence of pneumatosis intestinalis. Urinary tract infection can also be present. However they do not manifest necrotizing enterocolitis, bowel obstruction or prodromal clinical symptoms.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Spontaneous intestinal perforation | FIP · Focal intestinal perforation · Isolated perforation · Neonatal focal intestinal perforation · SIP |
| fr | Perforation intestinale spontanée | FIP · Perforation intestinale focale · Perforation intestinale néonatale focale · Perforation isolée · SIP |
| de | Spontane intestinale Perforation | FIP · Fokale intestinale Perforation · SIP |
| es | Perforación intestinal espontánea | PIE · Perforación aislada · Perforación intestinal focal · Perforación intestinal localizada |
| it | Perforazione intestinale spontanea | Perforazione intestinale focale · Perforazione isolata · SIP · FIP |
| nl | Spontane intestinale perforatie | FIP · Focale intestinale perforatie · Geïsoleerde perforatie · SIP · Spontane darmperforatie |
| pt | Perfuração intestinal espontânea | FIP · Perfuração intestinal focal · Perfuração isolada · SIP |
| pl | Samoistna perforacja jelita | = en |
| cs | Spontánní perforace střeva | = en |
| uk | Спонтанна перфорація кишечника | FIP · Фокальна перфорація кишечника · Ізольована перфорація · Неонатальна фокальна перфорація кишечника · SIP |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K63.1 | NTBT | attributed | validated |
| ICD-11 | KB84 | E | specific | validated |
| MONDO | 0957460 | E | validated | |
| UMLS | C3897004 | 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:165711Rare abdominal surgical disease
rare abdominal surgical diseases - ORPHA:117569Rare intestinal disease
rare gastroenterological 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/645793
- This record as JSON: /data/d/645793.json