Rustgi A K, Rotolo F S, Peete W P, Vollmer R T, Meyers W C
Surgery. 1985 Jun;97(6):714-20.
Primary lymphedema of the extremities, abdomen, or chest is an unusual and difficult clinical problem with few guidelines for management. A case is reported of lymphedema acquired at the age of 61 years, with associated massive chylous ascites and chylothorax. No underlying condition was discovered and the patient was found to have hypoplastic lymphatics by lymphangiography. Initial management consisted of extremity elevation, diuresis, and repeated paracenteses and thoracenteses. A peritoneojugular shunt provided temporary relief. Surgical pleurodesis combined with intensive diuresis has given prolonged relief of his symptoms allowing him to return to a functional life. Diagnostic and therapeutic guidelines for the management of this unusual condition are suggested.
四肢、腹部或胸部的原发性淋巴水肿是一个不常见且棘手的临床问题，几乎没有管理指南。本文报告了一例61岁时获得性淋巴水肿病例，伴有大量乳糜性腹水和乳糜胸。未发现潜在疾病，淋巴管造影显示患者淋巴管发育不全。初始治疗包括抬高肢体、利尿以及反复进行腹腔穿刺和胸腔穿刺。腹腔颈静脉分流术提供了暂时缓解。手术性胸膜固定术联合强化利尿使患者症状得到长期缓解，使其能够恢复正常生活。本文提出了针对这种罕见病症的诊断和治疗指南。
