Developmental lymphatic disorders of the thorax

Department of Radiology, State University of New York Health Science Center, Syracuse 13210.

Developmental disorders that involve the lymphatic channels of the thorax, although rare, are important and must be distinguished from the more common causes of chest masses or diffuse lung disease.

There are four major types of developmental lymphatic disorders that affect the thorax: lymphangiectasis, characterized by congenital anomalous dilatation of pulmonary lymph vessels; localized lymphangioma, a rare and benign, usually cystic, lesion characterized by masslike proliferation of lymph vessels; diffuse lymphangioma, a proliferation of vascular, mainly lymphatic, spaces in which visceral and skeletal involvement are common; and lymphangioleiomyoma, which involves a haphazard proliferation of smooth muscle in the lungs and dilatation of lymphatic spaces.

These characteristic findings can be seen with radiographic studies as well as with histologic evaluation. The discovery of one of these lymphatic disorders may prompt an investigation for associated congenital anomalies, including Noonan syndrome, asplenia, Gorham syndrome, and tuberous sclerosis.

PMID: 1749850 [PubMed - indexed for MEDLINE]

Related Abstract

Thoracic lymphatic disorders.

Davis KK, Berry GJ, Raffin TA, Faul JL.

Division of Pulmonary and Critical Care Medicine, Stanford University School of Medicine, Stanford, California 94305-5236, USA.

Thoracic complications of lymphatic disorders can culminate in respiratory failure and death and should be considered in any patient with a lymphatic disease and clinical or radiographic evidence of chest disease. Congenital lymphatic disorders are being increasingly recognized in the adult population. The spectrum of thoracic manifestations of lymphatic disorders ranges from incidental radiographic findings to diffuse lymphatic disease with respiratory failure. This article serves to review some recent advances that allow improved diagnosis and management of thoracic lymphatic disorders. Herein, we describe their anatomical and physiologic effects, the time course of their progression, and the therapies that are currently available. The management of malignant (cancerous) lymphatic disorders of the thorax is beyond the scope of this paper.

Publication Types:
Review

PMID: 15609812 [PubMed - indexed for MEDLINE]