Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis.
Management of refractory chylothorax in pulmonary lymphangioleiomyomatosis.
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DOI：
10.1002/rcr2.105
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发表时间：
2015-06
影响因子：
0.8
通讯作者：
Whitford, Helen M
中科院分区：
文献类型：
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作者：
Ellender, Claire M;Williams, Trevor J;Gooi, Julian;Snell, Gregory I;Whitford, Helen M
本病例报告了一例肺淋巴管肌瘤病(PLAM)非移植患者乳糜胸的成功治疗。延长的初始治疗失败了，包括完全肠外营养、胸膜引流、外科胸膜固定术和胸膜切除术。开始每天服用西罗莫司2 mg(交替服用2 mg失败)，20天后乳糜胸消失。停用西罗莫司进行腹部手术导致乳糜胸复发。西罗莫司治疗后积液迅速消退，肺功能稳定，4年内未见复发。我们的结论是，在PLAM相关性乳糜胸的治疗中，西罗莫司应该被考虑，也许在手术干预之前。
This case reports the successful management of chylothorax in a non-transplanted patient with pulmonary lymphangioleiomyomatosis (pLAM). Prolonged initial therapy failed, including total parenteral nutrition, pleural drainage, surgical pleurodesis, and pleurectomy. Commencement of sirolimus 2 mg daily (2 mg alternating days had failed) led to resolution of chylothorax after 20 days. Discontinuation of sirolimus for abdominal surgery led to recurrence of the chylothorax. Reinstitution of sirolimus led to rapid resolution of the effusion, stabilization of lung function, and there has been no recurrence in the ensuing 4 years. We conclude that sirolimus should be considered in the management of pLAM-related chylothorax, perhaps before surgical intervention.