CASE REPORT

A 47-year old female with no known comorbidities presented with progressive breathlessness. Initial evaluation revealed a left sided pleural effusion. Analysis showing lymphocytic predominant exudative, milky white fluid with high triglyceride contents, leading to the differentials of chylothorax. There was recurrent collections despite of repeated drainage. Lymphangioscintigraphy showed no lymphatic leak from the thoracic duct. PET-CT ruled out the presence of malignancy.

VATS showed a thick walled cyst in the pleural cavity with Thoracic duct splayed over it. The cyst was excised after clipping off the thoracic duct. Histopathological examination showed features of lymphangioma - cystic variety. 

DISCUSSION 

Lymphangioma is congenital malformation of lymphatic system. They represent the remnants of lymphatic tissues that failed  to connect to efferent channels or get sequestrated into portions of lymphatic sacs. Intrathoracic lymphatic abnormalities could be broadly divided into intrapulmonary  (lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndromes) or extrapulmonary lesions. Intrapulmonary lesions are extremely rare and Only 17 case reports have been published in the international literature to date¹. What we initially thought to be a chylothorax, turned out to be a rare case of intrathorasic lymphangioma. 

REFERENCES

1. Limmer S, Krokowski M, Kujath P. Pulmonary lymphangioma. Ann Thorac Surg. 2008 Jan;85(1):336-9.