Treatment of symptomatic primary chylous disorders

Purpose: Primary chylous disorders (PCDs) are rare. Rupture of dilated lymp h vessels (lymphangiectasia) may result in chylous ascites, chylothorax, or leakage of chyle through chylocutanous fistulas in the lower limbs or geni talia. 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 year s) 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%). P rimary lymphangiectasia presented alone in 23 patients (66%), and it was as sociated with clinical syndromes or additional pathologic findings in 12 (y ellow nail syndrome in 4, lymphangiomyomatosis in 3, unknown in 3, Prasad s yndrome (hypogammaglobulinemia, lymphadenopathy, and pulmonary insufficienc y) in 1, and thoracic duct cyst in 1). Twenty-one (60%) patients underwent 26 surgical procedures. Preoperative imaging included computed tomography s can in 15 patients, magnetic resonance imaging in 3, lymphoscintigraphy in 12, and lymphangiography in 14. Fifteen patients underwent 18 procedures fo r chylous ascites or pelvic reflux. Ten (56%) procedures were resection of retroperitoneal/mesenteric lymphatics with or without sclerotherapy of lymp hatics, 4 (22%) were lymphovenous anastomoses or grafts, 3 (17%) were perit oneovenous shunts, and 1 (6%) patient had a hysterectomy. Six patients unde rwent eight procedures for chylothorax, including thoracotomy with decortic ation. and pleurodesis (4 procedures), thoracoscopic decortication (I patie nt), ligation of thoracic duct (2 procedures), and resection of thoracic du ct cyst (I patient). Postoperative mean follow-up was 54 months (range, 0.3 -276). Early complications included wound infections in 3 patients, elevate d liver enzymes in 1, and peritoneovenous shunt occlusion with innominate v ein occlusion in 1. All patients improved initially, but four (19%) had rec urrence of symptoms at a mean of 25 months (range, 1-43). Three patients ha d postoperative lymphoscintigraphy confirming improved lymphatic transport and diminished reflux. One patient died 12 years postoperatively, from caus es unrelated to PCD. Conclusions. More than half of the patients with PCDs require surgical trea tment, and surgery should be considered in patients with significant sympto ms of PCD. Lymphangiography is recommended to determine anatomy and the sit e of the lymphatic leak, especially if lymphovenous grafting is planned. Al l patients had initial benefit postoperatively and two thirds of patients d emonstrated durable clinical improvement after surgical treatment.