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Ann Thorac Surg 1985;40:398-401
© 1985 The Society of Thoracic Surgeons

Articles

Pleuropulmonary and Skeletal Lymphangiomatosis with Chylothorax and Chylopericardium

Muhammad A.K. Bhatti, M.D., John W. Ferrante, M.D., Isaac Gielchinsky, M.D., John C. Norman, M.D.*

From the Department of Surgery, Muhlenberg Hospital, Plainfield, NJ, and the Department of Thoracic and Cardiovascular Surgery, Newark Beth Israel Medical Center, Newark, NJ.

Accepted for publication January 11, 1985.

* Address reprint requests to Dr. Norman, Department of Thoracic and Cardiovascular Surgery, Newark Beth Israel Medical Center, 201 Lyons Ave, Newark, NJ 07112

Congenital lymphangiomatosis of lung and bone, with or without chylothorax, is a rare but often fatal systemic lymphatic malformation. In those who survive infancy and early childhood, parietal pleurectomy with excision of lymphatic lakes and ligation of the thoracic duct can be successful. Two patients with lymphangiomatosis are described, 1 with chylothorax and chylopericardium with generalized skeletal lesions and the other with pleuropulmonary lesions and chylothorax. Both were successfully treated with parietal pleurectomy, excision of lymphatic lakes, and ligation of lymphatics, including the thoracic duct. To our knowledge, the triad of generalized skeletal lymphangiomatosis, chylopericardium, and chylothorax has not been previously reported.




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