congenital pulmonary lymphangiectasia

MeSH: C537727ORPHA: 2414

Contents

1. Overview
2. Available Treatments
3. Clinical Presentation
4. Classification & Codes

Overview

Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung and characterized by pulmonary subpleural, interlobar, perivascular, and peribronchial lymphatic dilatation

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with congenital pulmonary lymphangiectasia, sourced from HPO and Orphanet clinical annotations.

CyanosisGrowth delayAscitesCongestive heart failurePulmonic stenosisSplenomegalyHydrops fetalisGastroesophageal refluxPulmonary arterial hypertensionRespiratory distressPleural effusionHepatomegalyTricuspid regurgitationChronic pulmonary obstructionChylopericardiumCough

Classification & Codes

MeSH Code

C537727

Orphanet Code

ORPHA:2414

congenital pulmonary lymphangiectasia

MeSH	`C537727`
Orphanet	`ORPHA:2414`
Treatments	0 drug(s)
Symptoms on record	16 signs
Status	published

Factual Authority

Last Updated3/20/2026

Clinical DataHPO · Orphanet

Drug DataFDA · EMA · CDSCO