Treatment of symptomatic primary chylous disorders

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.

The clinical data of 35 patients with PCDs treated between January 1, 1976, and August 31, 2000, were reviewed retrospectively.

Fifteen men and 20 women (mean age, 29 years; range, 1 day-81 years) presented with PCDs. Sixteen (46%) patients had chylous ascites, and 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 (1 patient), ligation of thoracic duct (2 procedures), and resection of thoracic duct cyst (1 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.

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.