Images in Clinical Medicine

Congenital Pulmonary Lymphangiectasia

Cornelia Hagmann, M.D., and Thomas M. Berger, M.D.

N Engl J Med 2003; 349:e21November 27, 2003

• Slide

Article

A female infant was born at 40 weeks and 3 days of gestation, weighing 2970 g. The Apgar scores were 8, 10, and 10 at 1, 5, and 10 minutes, respectively. Within one hour after birth, severe respiratory distress with profound cyanosis developed while the infant was breathing room air, and intubation and mechanical ventilation were required. An initial chest radiograph showed normal lung volumes with diffuse, bilateral nodular changes, and a subsequent chest radiograph obtained on the 17th day of life showed that these changes had become more prominent (Panel A). Surfactant and antibiotics were administered. Lung compliance remained poor, and high-frequency oscillatory ventilation was begun. Congenital heart disease was ruled out, and cultures of blood and tracheal aspirates were unrevealing. After surfactant protein B deficiency was ruled out, an open-lung biopsy was performed, on the 18th day of life. On gross examination, the lung had an irregular surface with scattered nodular changes (Panel B). On microscopical examination, there was subpleural and septal cystic lymphangiectasia (L, Panel C; hematoxylin and eosin, ×150). A denotes artery. These findings are consistent with a diagnosis of congenital pulmonary lymphangiectasia, which is a uniformly fatal disease when it manifests in the newborn period. The infant died on the 20th day of life.

Cornelia Hagmann, M.D.
Thomas M. Berger, M.D.
Children's Hospital of Lucerne, CH-6000 Lucerne 16, Switzerland

Citing Articles (5)

Citing Articles

1. 1

   Zivanit Ergaz, Benjamin Bar-Oz, Ido Yatsiv, Ilan Arad. (2009) Congenital chylothorax: Clinical course and prognostic significance. Pediatric Pulmonology 44:8, 806-811
   CrossRef

2. 2

   Minseob Eom, Yoo Duk Choi, Youn Shin Kim, Mee-Yon Cho, Soon-Hee Jung, Han Young Lee. (2007) Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases. Journal of Korean Medical Science 22:4, 740
   CrossRef

3. 3

   Shir-Jing Ho, Tim A Brighton. (2006) Ximelagatran, direct thrombin inhibitor, oral anticoagulants, thromboprophylaxis. Vascular Health and Risk Management 2:1, 49-58
   CrossRef

4. 4

   E.M. Dempsey, G.M. Sant'Anna, R.L. Williams, R.T. Brouillette. (2005) Congenital pulmonary lymphangiectasia presenting as nonimmune fetal hydrops and severe respiratory distress at birth: Not uniformly fatal. Pediatric Pulmonology 40:3, 270-274
   CrossRef

5. 5

   (2004) Congenital Pulmonary Lymphangiectasia. New England Journal of Medicine 350:9, 948-948
   Full Text