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J Thorac Cardiovasc Surg 2007;133:1664-1665
© 2007 The American Association for Thoracic Surgery

Brief Communication

Diffuse lymphangiomatosis: Are there any clinical or therapeutic standards?

Charalambos Zisis, MD, PhD, FETCSa,*, Konstantinos Spiliotopoulos, MDa, Marios Patronis, MDa, George Filippakis, MDa, Grigoris Stratakos, MD, PhDb, George Tzelepis, MD, PhDc, Ion Bellenis, MD, PhD, FETCSa

a Department of Thoracic and Vascular Surgery, Evangelismos Hospital, Athens, Greece
b Department of Intensive Care and Chest Diseases, Medical School, University of Athens, Athens, Greece
c Department of Pathophysiology, Medical School, University of Athens, Athens, Greece.

Received for publication December 24, 2006; accepted for publication February 7, 2007.

* Address for reprints: Charalambos Zisis, MD, PhD, FETCS, 17a, Patriarchou Grigoriou str, 166 74-Glyfada, Athens, Greece. (Email: chzisis@hol.gr2).

The first 20% of the full text of this article appears below.

One of a variety of clinical disorders representing congenital developmental malformations of the lymphatic system, lymphangiomatosis describes the presence of multiple lymphangiomas. It is associated with other lymphatic abnormalities, and 75% of cases involve multiple organs. It most commonly presents in childhood, with predilection for thoracic and neck lesions, with up to 75% of patients having bony involvement.1Go

Clinical Summary

A 23-year-old man was referred to our department for a large left-sided pleural effusion representing a chylothorax as demonstrated after thoracocentesis. He had had persistent cough for months without dyspnea or hemoptysis. His medical history included small bowel resection at the age of 6 years for acute intestinal obstruction owing to intestinal lymphangiomatosis. The computed tomographic scan revealed diffuse abnormal mediastinal, retrocrural, and peritoneal lymphatic tissue with enlarged, nonhomogeneous spleen (Figure 1).


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  		Figure 1. Peritoneal lymphangiomatosis with enlarged, patchy affected spleen.

 
A drain was initially inserted in the left side of the chest . . . [Full Text of this Article]






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