Treatment of symptomatic primary chylous disorders
Treatment of symptomatic primary chylous disorders
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DOI：
10.1067/mva.2001.118800
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发表时间：
2001-11-01
影响因子：
4.3
通讯作者：
Deschamps, C
中科院分区：
文献类型：
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作者：
Noel, AA;Gloviczki, P;Deschamps, C
目的：原发性乳糜性疾病(PCD)很少见。扩张的淋巴管破裂(淋巴管扩张症)可能导致乳糜性腹水、乳糜胸，或通过下肢或生殖器的乳糜皮瘘漏乳糜漏。乳糜会通过功能不全的淋巴管回流，导致淋巴水肿。方法：回顾分析1976年1月1日至2000年8月31日收治的35例PCDS患者的临床资料。结果：男15例，女20例，平均年龄29岁；16例(46%)患者有乳糜性腹水，19例(54%)患者有乳糜胸(20例)，其中10例(29%)两者都有。在16例患者中，乳糜流至盆腔或下肢淋巴管导致淋巴水肿(14例，88%)或淋巴漏(11例，69%)。主要症状为肢体浮肿(19例，54%)、呼吸困难。(17，49%)，阴囊或阴唇浮肿(15，43%)，或腹胀(13，37%)。原发淋巴管扩张症23例(66%)，与临床症状或其他病理改变相关12例(黄甲综合征4例，淋巴管肌瘤病3例，不明原因3例，Prasad综合征(低丙种球蛋白血症、淋巴结病、肺功能不全)1例，胸管囊肿1例)。21例(60%)患者接受了26次手术。术前CT扫描15例，磁共振成像3例，淋巴核素扫描12例，淋巴管造影14例。15例患者因乳糜性腹水或盆腔反流接受了18次手术。腹膜后/肠系膜淋巴管切除加或不加硬化治疗10例(56%)，淋巴静脉吻合或移植4例(22%)，腹膜静脉分流术3例(17%)，子宫切除术1例(6%)。6名患者接受了8次乳糜胸手术，其中包括开胸去皮质手术。胸膜固定术4例，胸腔镜术1例，胸导管结扎术2例，胸导管囊肿切除术1例。术后平均随访54个月(0.3~276个月)。早期并发症包括伤口感染3例，肝酶升高1例，腹膜静脉分流闭塞合并无名静脉闭塞1例。所有患者术后症状均有改善，但4例(19%)术后症状复发，平均25个月(1~43个月)。3例患者术后行淋巴核素扫描，证实淋巴转运改善，反流减少。术后12年死亡1例，死亡原因与PCD无关。超过一半的PCD患者需要手术治疗，对于有明显PCD症状的患者应考虑手术治疗。淋巴血管造影术被推荐用于确定淋巴渗漏的解剖和位置，特别是在计划进行淋巴静脉移植的情况下。所有患者术后均有初步受益，三分之二的患者在手术治疗后显示出持久的临床改善。
Purpose: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymph vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or genitalia. Chyle may reflux through incompetent lymphatics, causing lymphedema. To assess the efficacy of surgical treatment, we reviewed our experience.Methods: The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively.Results: Fifteen men and 20 women (mean age, 29 years; range, I day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, mid 19 (54%) had chylothorax (20 patients), and of these, 10 (29%) had both. In 16 patients, reflux of chyle into the pelvic or lower limb lymphatics caused lymphedema (14, 88%) or lymphatic leak through cutaneous fistulae (11, 69%). Presenting symptoms included lower-limb edema (19, 54%), dyspnea. (17, 49%), scrotal or labial edema (15, 43%), or abdominal distention (13, 37%). Primary lymphangiectasia presented alone in 23 patients (66%), and it was associated with clinical syndromes or additional pathologic findings in 12 (yellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad syndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficiency) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography scan in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures for chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymphatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were peritoneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients underwent eight procedures for chylothorax, including thoracotomy with decortication. and pleurodesis (4 procedures), thoracoscopic decortication (I patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (I patient). Postoperative mean follow-up was 54 months (range, 0.3-276). Early complications included wound infections in 3 patients, elevated liver enzymes in 1, and peritoneovenous shunt occlusion with innominate vein occlusion in 1. All patients improved initially, but four (19%) had recurrence of symptoms at a mean of 25 months (range, 1-43). Three patients had postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from causes unrelated to PCD.Conclusions. More than half of the patients with PCDs require surgical treatment, and surgery should be considered in patients with significant symptoms of PCD. Lymphangiography is recommended to determine anatomy and the site of the lymphatic leak, especially if lymphovenous grafting is planned. All patients had initial benefit postoperatively and two thirds of patients demonstrated durable clinical improvement after surgical treatment.