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J Thorac Cardiovasc Surg 1994;108:594-595
© 1994 Mosby, Inc.

LETTERS TO THE EDITOR

Lymphangiomatosis of the mediastinum with chylothorax and chylopericardium: Role of radiation treatment

Department of Cardiac Surgery and Radiation Therapy
Fountain Valley Regional Hospital
Fountain Valley, CA 92708

To the Editor:

We report a case of symptomatic lymphangiomatosis of the mediastinum in which refractory chylothorax and chylopericardium developed. In view of the young age of the patient and the benign nature of the disease, radiotherapy was given reluctantly and resulted in prompt resolution.

A 36-year-old smoker with chest pain, dyspnea, and cough had a left hilar mass. At thoracotomy in December 1987, a 4 cm x4 cm multilocular cystic mass filled with chyle was excised. Histologic examination showed cavernous cystic spaces with foci of chronic inflammation and fibrosis with lymphoid aggregate. Subsequent computed tomographic (CT) scan of the abdomen showed massive retroperitoneal and mesenteric lymphadenopathy. Bone scan and skull radiograph showed possible foci of metastatic uptake. Lymphangiogram revealed lymph nodes replaced by an infiltrative process suggestive of possible malignancy. In February 1988, a laparotomy showed a massive retroperitoneal process identical to that in the chest.

Between March and May 1988, the patient presented with marked shortness of breath, recurrent chylothorax, and chylopericardium that necessitated multiple thoracenteses, chest tube drainages, and pericardiocenteses. On each occasion between 1.5 and 2 L of chyle was drained. The most serious event was pericardial tamponade. To obviate recurrent admission and life-threatening recurrent pericardial tamponade, we seriously considered administering radiation therapy to the mediastinum and lymph node–bearing abdomen after the patient refused any surgical intervention. Ten fractionated dose of 2000 rad of radiation was given. The patient made a remarkable improvement over the next 2 weeks. Over the next 12 months he had no symptoms and the chest radiograph and CT scan of the chest and the abdomen were remarkably improved.

Lymphangiomas of the generalized variety that involve the mediastinum, retroperitoneum, and skeletal system are rare. Groves and Effler ¹ were the first to report the association of skeletal lymphangiomatosis and chylopericardium. Skeletal involvement is rare and can be both disfiguring and fatal. ²

Our patient had lytic lesions of the skull, but he refused bone biopsy for definitive diagnosis. Cough and dyspnea, although nonspecific, are the usual findings in patients with chylothorax and chylopericardium. Rarely, chylopericardium may be asymptomatic. ³ Even lymphangiograms can be misleading; for example, in our case lymphangioma was mistaken for lymphoma or metastatic replacement of retroperitoneal lymph nodes. Laparotomy may have been avoided, but we were not prepared to overlook a possible malignant process. Lymphoceles in the postoperative setting have been diagnosed and treated by percutaneous techniques with CT. These postoperative CT scans shows debris within the lymphoceles that are hypoechoic to anechoic. We were not able to make this diagnosis on CT scan because postoperative lymphoceles usually occur in the setting of lymph node dissection, with renal transplantation and vascular surgery. Thoracic involvement usually can be managed with excision of the pleura and pericardium and ligation of the thoracic duct. However, thoracic duct ligation is no guarantee for successful control of chylothorax.

When the thoracic duct is not ligated, fatal chylopericardium may occur. The alternative method of treatment, including sclerosant agents, namely sodium morrhuate, tetracycline, and bleomycin fat emulsion, have been used. But these alternative forms of treatment cannot be tried in the pericardial space because constrictive pericarditis may result. Our concern was the application of radiation in a benign disease, such as lymphangioma. Radiation has been used in the control of skeletal destruction by lymphangioma. ² Only two other reports have described the use of radiation as an alternative mode of treatment to control recurrent chylothorax. ^4,5 Fractionated dose of radiotherapy is justified in patients with persistent, recurrent, and life-threatening conditions, as in our case. More important, the patient must consent to such an unconventional treatment and realize the potential for constrictive pericarditis and malignant transformation of previously irradiated lymphangiomas. Any patient with chylothorax of unknown origin should have lymphangiography followed by CT to delineate the extent of the abnormality and thus avoid unnecessary surgery that may be life threatening.

Because of the generalized nature of the disease and the potential for constrictive pericarditis and malignant transformation after radiation, follow-up in these patients must be life-long.

References

1. Groves LK, Effler DB. Primary chylopericardium. N Engl J Med 1954;250:520-3.
   
2. Watts MA, Gibbons JA, Aaron BR. Mediastinal and osseous lymphangiomatosis: case report in review. Ann Thorac Surg 1982;34:324-8.[Abstract/Free Full Text]
   
3. Goldstein MR, Benchimol A, Cornell W, Long D. Chlyopericardium with multiple lymphangioma of bone. N Engl J Med 1962;280:1034-7.
   
4. Bell KA, Simon BK. Chylothorax and lymphangiomas of bone: unusual manifestations of lymphatic disease. South Med J 1978;71:459-60.[Medline]
   
5. Johnson DW, Klazynski PT, Gordon WH. Mediastinal lymphangioma and chylothorax: the role of radiotherapy. Ann Thorac Surg 1986;41:325-38.[Abstract/Free Full Text]
   

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