Disorders of the Pleura |

Recurrent Exudative Pleural Effusion Due to Thoracic Lymphangiomatosis FREE TO VIEW

Satish Chandrashekaran, MBBS; Lioudmila Karnatovskaia, MD; Emir Festic, MD
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Mayo Clinic, Jacksonville, FL

Chest. 2013;144(4_MeetingAbstracts):485A. doi:10.1378/chest.1702364
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SESSION TITLE: Pleural Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Lymphangiomas are congenital errors of the lymphatic development where focal proliferation of well differentiated lymphatic tissue is seen. It is a rare disorder, often misdiagnosed and difficult to manage.

CASE PRESENTATION: 60 year-old woman, a 40 pack-year smoker, presented with shortness of breath, right pleuritic chest pain and non-productive cough. Past medical history included irritable bowel syndrome and depression. Dyspnea was present for two months, initially only upon exertion, later at rest. Physical examination was significant for decreased breath sounds and dullness to percussion in the right base. Chest Xray demonstrated right pleural effusion. Several thoracentesis were performed yielding bloody, exudative pleural effusions, which subsequently recurred. Cytology was negative except for rare mesothelial cells. Triglyceride level was 35 mg/dl. Pulmonary function testing demonstrated mild obstruction and decreased DLCO. Computed Tomography (CT) scan of the chest showed an 8 cm cystic mass extending from the subcarinal region to the level of the lower thoracic esophagus (Figure 1). Innumerable subcentimeter hypodense splenic lesions were also present. The patient was referred for surgery and subsequently a large mediastinal cyst along with multiple subpleural cysts were excised during right thoracotomy. Pathology demonstrated lymphangiomatosis with lymphangiectasia (Figure 2). Patient tolerated surgery well and remained asymptomatic on a follow up a year later.

DISCUSSION: Thoracic lymphangiomas usually present as multicystic or spongelike accumulations. Lymphangiectasis refers to pathologic dilatation of lymphatic vessels. Unlike solitary lymphangioma usually seen in neonates and young children, lymphangiomatosis presents in older individuals possibly due to its slow growth. It involves multiple organs in majority of cases, and may present with lytic bone lesions or chylothorax with or without associated chyloptysis, hemoptysis, chylopericardium or chylous ascites. Many patients develop wheezing and are often misdiagnosed with asthma. Accurate anatomic localization by CT imaging helps with management, as well as with prognosis. Surgery may be required to confirm the diagnosis and to prevent complications that could arise from the compression of surrounding structures. Incomplete resection may result in recurrence. Percutaneous sclerotherapy with doxycycline can be used in unresectable cases.

CONCLUSIONS: In patients presenting with exudative pleural effusion and cystic lesions in the thorax, lymphangiomatosis should be considered in differential diagnosis.

Reference #1: Faul JL et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000;161:1037-46.

DISCLOSURE: The following authors have nothing to disclose: Satish Chandrashekaran, Lioudmila Karnatovskaia, Emir Festic

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