congenital pulmonary lymphangiectasia
MONDO:0009933Mondo
Findings
No curated finding names congenital pulmonary lymphangiectasia yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung and characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation.
Definition from the Mondo Disease Ontology (MONDO:0009933), read 2026-09-29. CC BY 4.0.
- Onset and course
- Antenatal onset
HPO, annotations 2026-09-02
Features
23 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- BronchodysplasiaHPOHP:0006533
- 1 of 1 reported patient
- ChylothoraxHPOHP:0010310
- 3 of 3 reported patients
- LymphedemaHPOHP:0001004
- 3 of 3 reported patients
- Nonimmune hydrops fetalisHPOHP:0001790
- 3 of 3 reported patients · Antenatal onset
- Pulmonary lymphangiectasiaHPOHP:0006521
- 3 of 3 reported patients · Congenital onset
- CyanosisHPOHP:0000961
- Very frequent (80% to 99% of cases)
- Pleural effusion
Show the remaining 11
- CoughHPOHP:0012735
- Frequent (30% to 79% of cases)
- Gastroesophageal refluxHPOHP:0002020
- Frequent (30% to 79% of cases)
- Growth delayHPOHP:0001510
- Frequent (30% to 79% of cases)
- Hydrops fetalisHPOHP:0001789
- Frequent (30% to 79% of cases)
- HepatomegalyHPOHP:0002240
- Occasional (5% to 29% of cases)
- Pulmonary arterial hypertensionHPOHP:0002092
- Occasional (5% to 29% of cases)
Where it sits
Other names
3 names
Resolves to: congenital pulmonary lymphangiectasia
- Also called
- congenital pulmonary lymphangiectasisDiffuse Pulmonary Lymphangiomatosispulmonary lymphangiomatosis