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Invited commentary: Primary chylous ascites
INVITED COMMENTARY INVITED COMMENTARY
Primary chylous ascites Peter Gloviczki, MD, Rochester, Minn
Primary chylous ascites is a rare condition. The presence of milky fluid, rich in triglycerides, chylomicrons, and proteins, aspirated from the abdomen in pediatric patients makes recognition of chylous ascites relatively easy, but confirming the underlying lymphatic pathology of obstruction, agenesis, hypoplasia, or dilation with valvular incompetence is difficult, and treatment is usually challenging. Campisi et al1 in this issue of the Journal of Vascular Surgery report on excellent results after surgical treatment of primary chylous ascites in 12 patients, with a mean follow-up of 5 years (range, 3 to 7 years). One patient died 1 year after surgery of an unrelated cause, one recurrence was treated effectively with a peritoneovenous shunt, and only three patients had mild recurrences. The authors should be congratulated for this remarkable series, considering that surgery is usually palliative and cure is rare, because ablation or occlusion of all abnormal lymphatic tissue is seldom possible. These surgical results are important to demonstrate the possibility to achieve several years of disease-free interval in patients with this debilitating chronic condition. Unfortunately, it is not rare to see these young patients back in the office with recurrent disease 5 to 10 years after an initial successful surgical treatment. Recurrence is frequently less severe than the original presentation. However, the life span of some of these patients with severe lymphangiectasia, protein-loosing enteropathy, extensive bowel involvement, and chronic loss of chyle associated with malnutrition and immunologic compromise is severely reduced. Secondary chylous ascites is much more frequent than primary disease, and this is reflected in the experience of Campisi et al1 as well: only 12 of 82 patients with chylous ascites had primary disease. Most patients with secondary chylous ascites have malignancy, usually lymphoma; establishing the exact etiology early, as emphasized in an excellent review by Aalami et al,2 is imperative. Primary chylous ascites is much more frequent in the pediatric age group than in adults, and developmental abnormalities of the lymphatic system are the most important causes. The type of lymphatic abnormalities found determines management and outcome. Some respond more to surgical treatment than others, which may explain, in part, the very good results reported by Campisi et al.1 J Vasc Surg 2006;43:1297-8 0741-5214/$32.00 Copyright © 2006 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2006.02.024
Browse et al3 described the three possible causes of primary chylous ascites in detail. The first is congenital lymphangiectasia, which is caused by congenital valvular incompetence and dilation of the mesenteric or retroperitoneal lymphatics, or both. The lymphatics of all abdominal organs, the thoracic duct, and leg lymphatics, usually in one limb, can also be affected. Lymphatic disruption or transudation of chyle into the peritoneal cavity is the cause of chylous ascites. The second cause is congenital obstruction or agenesis of the thoracic duct, leading to secondary dilation and reflux of chyle into the mesenteric or retroperitoneal lymphatics. The cause of chylous ascites is the elevated abdominal lymphatic pressure, which leads to lymphatic disruption or transudation of chyle into the peritoneal cavity. Finally, the third cause is obstruction localized to mesenteric lymph nodes and lymph vessels, which can be congenital or may occur later in life, similar to primary lymphedema (precox, between age 1 and 35 years; or tarda, with onset after age of 35 years). Many of these patients have dilated mesenteric lymph vessels, with dilation of lacteals in the wall of the bowel leading to disruption and leak of chyle into the abdominal cavity and frequently into the lumen of the bowel, causing protein-loosing enteropathy. The wall of the bowel maybe severely damaged, and resection of the most involved segments is required. Recurrence of ascites is more frequent with this type. No patient in the reported series of Campisi et al1 required bowel resection. Thorough evaluation of patients is important, and it should include computed tomography or magnetic resonance imaging of the chest and abdomen to exclude malignancy. Although the need for “total” body lymphoscintigraphy, as suggested by Campisi et al,1 is debatable, lowerlimb lymphoscintigraphy reveals evidence of fast transport, dilated lymphatics, and potential sites of colloid extravasation in the abdomen or chest. In patients who have evidence of lower-limb swelling, we perform lymphoscintigraphy by injecting first the nonaffected site.4 Crossover reflux to the affected limb, in addition to the above-mentioned findings, will confirm the diagnosis. We agree with Browse that contrast lymphangiography is frequently useful in these patients to identify the leak and confirm lymphangiectasia.3 Campisi et al1 emphasize the benefits of laparoscopy in the diagnosis of this condition, but their series does not bring us convincing evidence about the therapeutic efficacy of laparoscopy. All their patients underwent open surgical 1297
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exploration with ablation of the abnormal lymphatic tissue, with an attempt at excision or ligation, sclerotherapy, or laser ablation of the enlarged and leaking lymphatics. Some patients underwent lymphovenous anastomoses to drain the chyle from the mesenteric lymphatic system. We have observed that anastomosing an incompetent mesenteric lacteal to a mesenteric vein results in reflux of blood from the high-pressure portal venous sytem into the incompetent lymph vessels. Therefore, we now perform lymphovenous anastomosis only with the retroperitoneal venous system, assuring the lowest possible venous pressure to hinder lymphatic flow. In addition, interposition of a saphenous vein segment with a competent valve between a dilated lymph vessel and the iliac vein will prevent any reflux of blood into the lymphatic system.5 The authors used peritoneovenous shunt in one patient only, with good early results. We reported on using the LeVeen shunt with good initial results in four of 16 patients who had primary chylous ascites.6 These shunts frequently last for several years, and we have found them especially useful in patients with diffuse extravasation of chyle and without well-defined lymphatic fistulae. Primary patency of peritoneovenous shunts ⬎5 years is exceedingly rare, but we have several patients who do well with replacement of a new shunt for a few years, with lasting benefit. Superior vena cava syndrome developed in one of our patients after placement of the shunt. Primary chylous ascites requires continuous medical and intermittent surgical or interventional treatment. Patients need strict dietary restrictions and constant manage-
ment of their hemodynamic in-balance, malnutrition, and immunologic compromise. The management algorithm suggested by Campisi et al1 has been effective in their series in controlling the disease in a select group of patients with congenital lymphangiectasia. These results are testimony to the progress that has taken place in this field. Results of ours5,6 as well as of others,2,3,7 in a mixed group of patients with different underlying lymphatic pathology suggest, however, that further efforts in this field are still essential to produce lasting benefits in patients with primary chylous ascites. REFERENCES 1. Campisi C, Bellini C, Erretta C, Zilli A, da Rin E, Davini D, et al. Diagnosis and management of primary chylous ascites. J Vasc Surg 2006;43:1244-8. 2. Aalami OO, Allen DB, Organ CH. Chylous ascites: a collective review. Surgery 2000;128:761-78. 3. Browse N, Burnand KG, Mortimer PS. Diseases of the lymphatics. London: Arnold; 2003. p. 259-92. 4. Cambria RA, Gloviczki P, Naessens JM, Wahner H. Noninvasive evaluation of the lymphatic system with lymphoscintigraphy: a prospective, semiquantitative analysis in 386 extremities. J Vasc Surg 1993;18: 773-82. 5. Gloviczki P, Noel AA. Surgical treatment of chronic lymphedema and primary chylous disorders. In: Rutherford RB (editor) Rutherford’s vascular surgery. 6th edition. Philadelphia: Elsevier; 2005. p. 2428-45. 6. Noel A, Gloviczki P, Bender CE, Whitley D, Stanson AW, Deschamps C. Treatment of symptomatic primary chylous disorders. J Vasc Surg 2001; 34:785-91. 7. Browse NL, Wilson NM, Russo F, al-Hassan H, Allen DR. Aetiology and treatment of chylous ascites. Br J Surg 1993;79:1145-50.
Primary chylous ascites Peter Gloviczki, MD, Rochester, Minn
Primary chylous ascites is a rare condition. The presence of milky fluid, rich in triglycerides, chylomicrons, and proteins, aspirated from the abdomen in pediatric patients makes recognition of chylous ascites relatively easy, but confirming the underlying lymphatic pathology of obstruction, agenesis, hypoplasia, or dilation with valvular incompetence is difficult, and treatment is usually challenging. Campisi et al1 in this issue of the Journal of Vascular Surgery report on excellent results after surgical treatment of primary chylous ascites in 12 patients, with a mean follow-up of 5 years (range, 3 to 7 years). One patient died 1 year after surgery of an unrelated cause, one recurrence was treated effectively with a peritoneovenous shunt, and only three patients had mild recurrences. The authors should be congratulated for this remarkable series, considering that surgery is usually palliative and cure is rare, because ablation or occlusion of all abnormal lymphatic tissue is seldom possible. These surgical results are important to demonstrate the possibility to achieve several years of disease-free interval in patients with this debilitating chronic condition. Unfortunately, it is not rare to see these young patients back in the office with recurrent disease 5 to 10 years after an initial successful surgical treatment. Recurrence is frequently less severe than the original presentation. However, the life span of some of these patients with severe lymphangiectasia, protein-loosing enteropathy, extensive bowel involvement, and chronic loss of chyle associated with malnutrition and immunologic compromise is severely reduced. Secondary chylous ascites is much more frequent than primary disease, and this is reflected in the experience of Campisi et al1 as well: only 12 of 82 patients with chylous ascites had primary disease. Most patients with secondary chylous ascites have malignancy, usually lymphoma; establishing the exact etiology early, as emphasized in an excellent review by Aalami et al,2 is imperative. Primary chylous ascites is much more frequent in the pediatric age group than in adults, and developmental abnormalities of the lymphatic system are the most important causes. The type of lymphatic abnormalities found determines management and outcome. Some respond more to surgical treatment than others, which may explain, in part, the very good results reported by Campisi et al.1 J Vasc Surg 2006;43:1297-8 0741-5214/$32.00 Copyright © 2006 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2006.02.024
Browse et al3 described the three possible causes of primary chylous ascites in detail. The first is congenital lymphangiectasia, which is caused by congenital valvular incompetence and dilation of the mesenteric or retroperitoneal lymphatics, or both. The lymphatics of all abdominal organs, the thoracic duct, and leg lymphatics, usually in one limb, can also be affected. Lymphatic disruption or transudation of chyle into the peritoneal cavity is the cause of chylous ascites. The second cause is congenital obstruction or agenesis of the thoracic duct, leading to secondary dilation and reflux of chyle into the mesenteric or retroperitoneal lymphatics. The cause of chylous ascites is the elevated abdominal lymphatic pressure, which leads to lymphatic disruption or transudation of chyle into the peritoneal cavity. Finally, the third cause is obstruction localized to mesenteric lymph nodes and lymph vessels, which can be congenital or may occur later in life, similar to primary lymphedema (precox, between age 1 and 35 years; or tarda, with onset after age of 35 years). Many of these patients have dilated mesenteric lymph vessels, with dilation of lacteals in the wall of the bowel leading to disruption and leak of chyle into the abdominal cavity and frequently into the lumen of the bowel, causing protein-loosing enteropathy. The wall of the bowel maybe severely damaged, and resection of the most involved segments is required. Recurrence of ascites is more frequent with this type. No patient in the reported series of Campisi et al1 required bowel resection. Thorough evaluation of patients is important, and it should include computed tomography or magnetic resonance imaging of the chest and abdomen to exclude malignancy. Although the need for “total” body lymphoscintigraphy, as suggested by Campisi et al,1 is debatable, lowerlimb lymphoscintigraphy reveals evidence of fast transport, dilated lymphatics, and potential sites of colloid extravasation in the abdomen or chest. In patients who have evidence of lower-limb swelling, we perform lymphoscintigraphy by injecting first the nonaffected site.4 Crossover reflux to the affected limb, in addition to the above-mentioned findings, will confirm the diagnosis. We agree with Browse that contrast lymphangiography is frequently useful in these patients to identify the leak and confirm lymphangiectasia.3 Campisi et al1 emphasize the benefits of laparoscopy in the diagnosis of this condition, but their series does not bring us convincing evidence about the therapeutic efficacy of laparoscopy. All their patients underwent open surgical 1297
JOURNAL OF VASCULAR SURGERY June 2006
1298 Gloviczki
exploration with ablation of the abnormal lymphatic tissue, with an attempt at excision or ligation, sclerotherapy, or laser ablation of the enlarged and leaking lymphatics. Some patients underwent lymphovenous anastomoses to drain the chyle from the mesenteric lymphatic system. We have observed that anastomosing an incompetent mesenteric lacteal to a mesenteric vein results in reflux of blood from the high-pressure portal venous sytem into the incompetent lymph vessels. Therefore, we now perform lymphovenous anastomosis only with the retroperitoneal venous system, assuring the lowest possible venous pressure to hinder lymphatic flow. In addition, interposition of a saphenous vein segment with a competent valve between a dilated lymph vessel and the iliac vein will prevent any reflux of blood into the lymphatic system.5 The authors used peritoneovenous shunt in one patient only, with good early results. We reported on using the LeVeen shunt with good initial results in four of 16 patients who had primary chylous ascites.6 These shunts frequently last for several years, and we have found them especially useful in patients with diffuse extravasation of chyle and without well-defined lymphatic fistulae. Primary patency of peritoneovenous shunts ⬎5 years is exceedingly rare, but we have several patients who do well with replacement of a new shunt for a few years, with lasting benefit. Superior vena cava syndrome developed in one of our patients after placement of the shunt. Primary chylous ascites requires continuous medical and intermittent surgical or interventional treatment. Patients need strict dietary restrictions and constant manage-
ment of their hemodynamic in-balance, malnutrition, and immunologic compromise. The management algorithm suggested by Campisi et al1 has been effective in their series in controlling the disease in a select group of patients with congenital lymphangiectasia. These results are testimony to the progress that has taken place in this field. Results of ours5,6 as well as of others,2,3,7 in a mixed group of patients with different underlying lymphatic pathology suggest, however, that further efforts in this field are still essential to produce lasting benefits in patients with primary chylous ascites. REFERENCES 1. Campisi C, Bellini C, Erretta C, Zilli A, da Rin E, Davini D, et al. Diagnosis and management of primary chylous ascites. J Vasc Surg 2006;43:1244-8. 2. Aalami OO, Allen DB, Organ CH. Chylous ascites: a collective review. Surgery 2000;128:761-78. 3. Browse N, Burnand KG, Mortimer PS. Diseases of the lymphatics. London: Arnold; 2003. p. 259-92. 4. Cambria RA, Gloviczki P, Naessens JM, Wahner H. Noninvasive evaluation of the lymphatic system with lymphoscintigraphy: a prospective, semiquantitative analysis in 386 extremities. J Vasc Surg 1993;18: 773-82. 5. Gloviczki P, Noel AA. Surgical treatment of chronic lymphedema and primary chylous disorders. In: Rutherford RB (editor) Rutherford’s vascular surgery. 6th edition. Philadelphia: Elsevier; 2005. p. 2428-45. 6. Noel A, Gloviczki P, Bender CE, Whitley D, Stanson AW, Deschamps C. Treatment of symptomatic primary chylous disorders. J Vasc Surg 2001; 34:785-91. 7. Browse NL, Wilson NM, Russo F, al-Hassan H, Allen DR. Aetiology and treatment of chylous ascites. Br J Surg 1993;79:1145-50.