[Congenital unilobar pulmonary lymphangiectasis].

Area of Science:

• Pediatric Pathology
• Pulmonary Medicine
• Neonatal Surgery

Background:

• Congenital lobar emphysema (CLE) is a rare congenital lung malformation presenting in infancy.
• Accurate diagnosis is crucial for appropriate management and understanding of neonatal respiratory distress.
• Pulmonary lymphangiectasis is a rare congenital disorder characterized by dilated lymphatic vessels in the lungs.

Observation:

• A 27-day-old male infant underwent left upper lobectomy for suspected CLE.
• Intraoperative finding of a cranial pericardial hiatus was noted.
• The infant succumbed to extrapulmonary sepsis ten days postoperatively.

Findings:

• Histologic examination revealed pulmonary lymphangiectasis, not CLE, in the resected left upper lobe.
• Pulmonary lymphangiectasis is typically diffuse, making this unilateral/unilobar case exceptionally rare.
• Alveolar rupture is hypothesized to have facilitated air entry into ectatic lymphatics, causing clinical symptoms.
• Presence of foreign-body giant cells within lymph vessels, an unusual finding in pulmonary lymphangiectasis, supports this hypothesis.

Implications:

• This case highlights the importance of thorough histologic examination for accurate diagnosis of neonatal lung abnormalities.
• The findings suggest a potential mechanism for air-related complications in congenital pulmonary lymphangiectasis.
• Understanding this rare presentation can inform differential diagnosis and management strategies for neonatal respiratory distress.