J Korean Med Sci.  2007 Aug;22(4):740-745. 10.3346/jkms.2007.22.4.740.

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases

• Eom M
• Choi YD
• Kim YS
• Cho MY
• Jung SH
• Lee HY

• KMID: 1127098
• DOI: http://doi.org/10.3346/jkms.2007.22.4.740

Abstract

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

Keyword

Congenital Pulmonary Lymphangiectasis; Lymphatics; Autopsy

MeSH Terms

Humans
Infant, Newborn
Lung/pathology
Lung Diseases/congenital/*pathology
Lymphangiectasis/congenital/*pathology
Lymphatic System/pathology
Male

Figure

• 

  Fig. 1 Both lungs have an irregular surface with scattered nodular changes along the visceral pleura (A). A low-power view of lungs shows a network of cystic dilatation of lymphatics in the sub-pleural area (B) and peri-bronchovascular area (C) (H&E, ×40). On the high-power view, the cystic spaces are lined by one-layer of flattened cuboidal cells, and these cells and vascular endothelial cells reveal positive immunohistochemical reactivity for CD31 (D) (IHC, ×400).

• 

  Fig. 2 The pleural surfaces are carpeted with numerous tiny cystic spaces of variable sizes (A). There are widespread ectasia of lymphatics in the pleura, the interlobular septa (B), and peri-bronchovascular area (C) (H&E, ×40). On immunohistochemical stains, the flat cells lining lymphatic space are positive for D2-40 (D) (IHC, ×400).

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