A Pleuro-Peritoneal Communication through the Diaphragm affected with Lymphangioleiomyomatosis

Abstract

Abstract

Lymphangioleiomyomatosis: A Disease Involving the Lymphatic System

“…62,66 The diaphragmatic LAM lesion can generate pleuro-peritoneal communication through LAMassociated lymphangiogenesis rather than an anatomical defect of the diaphragm. 67 On the other hand, Morimoto et al reported that chylorrhea from swollen vessels on the right diaphragm was observed by thoracoscopy in a Japanese LAM patient who had right chylothorax. 61 Chyloptysis can occur due to stasis of the lymph flow with a reflux of chyle into the tracheobronchial tree (Fig.…”

“…Chylous pleural effusion, ascites, and chyloptysis, are often contaminated with red blood cells (Fig. 1), 67 thus suggesting the presence of bloodlymphatic vessel shunts. Abundant lymphatic vessels with few blood vessels in LAM lesions may indicate that VEGF-Dmediated lymphangiogenesis suppresses angiogenesis in LAM lesions.…”

Characteristics of Patients with Lymphangioleiomyomatosis and Pleural Effusion: A Systematic Review

“…Figure 1 presents a flowchart showing the complete breakdown of the steps used in the identification of appropriate studies [9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,…”

“…The question of whether the PE was transudate or exudate was only addressed in 14 cases, and all corresponded to the latter. PF triglyceride levels were reported in 20 cases [23,25,29,32,37,45,47,49,60,63,74,77,81,82,83,84,87,88,91,101], and all had values higher than 110 mg/dl (range 190-4,600 mg/dl). The presence of chylomicrons was only specified in 3 cases [46,83,101].…”

“…Cytology and culture of PE were negative in all cases. In 44 of 69 patients (63.8%) in whom ascites was described, PE was accompanied by this condition [9,11,16,17,18,24,26,28,30,39,48,52,55,58,63,64,65,68,73,75,76,78,82,84,85,86,88,95], and in 97.3% (36/37) of the cases it was chylous. The only case of nonchylous ascites corresponded to the already mentioned patient that had a hemiperitoneum and a serous-hematic PE [48].…”