Pediatric lymphangiectasia: an imaging spectrum.

Area of Science:

• Pediatric Radiology
• Lymphatic Imaging
• Vascular Malformations

Background:

• Lymphangiectasia is a rare lymphatic dysplasia characterized by lymphatic dilation.
• It commonly affects central conducting lymphatics, pulmonary, and intestinal networks.
• Advances in lymphatic interventions increase reliance on imaging for disease characterization.

Purpose of the Study:

• To describe patient demographics, underlying conditions, and imaging features of pediatric lymphangiectasia.
• To correlate imaging findings with pathology and patient outcomes over a 10-year period.
• To evaluate the utility of cross-sectional imaging in managing lymphangiectasia.

Main Methods:

• Retrospective review of pathology database (2002-2012) for pathologically proven lymphangiectasia.
• Inclusion of patients who underwent cross-sectional imaging.
• Review of medical records for demographics, conditions, treatments, and outcomes.

Main Results:

• Thirteen children (1 month-16 years) identified with pulmonary, intestinal, or diffuse lymphangiectasia.
• Distinct imaging findings for pulmonary (septal thickening, effusions) and intestinal (bowel wall thickening) involvement.
• Infantile/diffuse cases had high mortality; later/segmental cases showed improvement; lymphatic duct ligation was successful in three children.

Conclusions:

• Lymphangiectasia is a complex disorder with diverse presentations and outcomes.
• Cross-sectional imaging differentiates segmental from diffuse disease and identifies dilated central lymphatics.
• Imaging findings guide interventions like ligation or occlusion for potential benefit.