Continually Updated Clinical Reference
 
 
  All Sources     eMedicine     Medscape     Drug Reference     MEDLINE
 
eMedicine - Chylous Ascites : Article by

Quick Find
Authors & Editors
Introduction
Pathophysiology
Classification
Frequency and Age and Sex Distribution
Clinical Disease Associations and Pathology
Clinical Features and Complications
Laboratory Studies, Ascitic Fluid Study, and Other Diagnostic Tests
Treatment
References




Patient Education
Click here for patient education.



Author: Unnithan V Raghuraman, MD, FRCP, FACG, FACP, Consulting Staff, Department of Gastroenterology, St John Medical Center

Unnithan V Raghuraman is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy

Coauthor(s): David C Wolf, MD, FACP, FACG, AGAF, Medical Director of Liver Transplantation, Westchester Medical Center, Professor of Clinical Medicine, Division of Gastroenterology and Hepatobiliary Diseases, Department of Medicine, New York Medical College

Editors: Mounzer Al Al Samman, MD, Department of Internal Medicine, Division of Gastroenterology, Assistant Professor, Texas Tech University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; BS Anand, MD, Department of Internal Medicine, Division of Gastroenterology, Professor, Baylor University College of Medicine; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania

Author and Editor Disclosure

Synonyms and related keywords: chyloperitoneum, milky ascites, abdominal lymphatics, chyliform ascites, pseudochylous ascites, lymphomas, disseminated carcinomas, tuberculosis, lymphangiectasia, mesenteric cyst, high portal pressures, polycythemia vera, portal vein thrombosis, hepatic vein thrombosis, abdominal distension, peritoneocentesis, paracentesis

Chylous ascites is the extravasation of milky chyle into the peritoneal cavity. This can occur de novo as a result of trauma or obstruction of the lymphatic system. Moreover, an existing clear ascitic fluid can turn chylous as a secondary event.

True chylous ascites is defined as the presence of ascitic fluid with high fat (triglyceride) content, usually higher than 110 mg/dL.



Chylous ascites is a rare clinical condition that occurs as a result of disruption of the abdominal lymphatics. Multiple causes have been described, including the following:

  • Abdominal surgery
  • Blunt abdominal trauma
  • Malignant neoplasms - Hepatoma, small bowel lymphoma, small bowel angiosarcoma, and retroperitoneal lymphoma
  • Spontaneous bacterial peritonitis
  • Cirrhosis - Up to 0.5% of patients with ascites from cirrhosis may have chylous ascites.
  • Pelvic irradiation
  • Peritoneal dialysis
  • Abdominal tuberculosis
  • Carcinoid syndrome
  • Congenital defects of lacteal formation



Milky ascites is subdivided into 3 groups as follows:

  • True chylous ascites - Fluid with high triglyceride content
  • Chyliform ascites - Fluid with a lecithin-globulin complex due to fatty degeneration of cells
  • Pseudochylous ascites - Fluid that is milky in appearance due to the presence of pus

This classification is not clinically useful and has been discarded by some.



Frequency

The rarity of chylous ascites in clinical practice can be judged by the fact that only 28 cases were identified at Massachusetts General Hospital over a period of 20 years. Of these 28 patients, 4 were children. The mean age at detection in adults was 54.3 years.

An earlier study (1957) reported the incidence of chylous ascites as approximately 1 case per 187,000 hospital admissions. In subsequent years, due to more aggressive retroperitoneal and cardiothoracic surgical techniques and longer survival of cancer patients, the incidence of chylous ascites has increased to 1 case in 11,584 hospital admissions (1982).

Age and sex distribution

No differences in sex distribution have been cited, but, of the 28 patients with chylous ascites from Massachusetts General Hospital, 75% were women. Chylous ascites can occur in adult and pediatric populations. In adults, it commonly is observed in individuals aged 50-65 years.



Clinical disease associations

In adults, chylous ascites is associated most frequently with malignant conditions. These conditions particularly include lymphomas and disseminated carcinomas from primaries in the pancreas, breast, colon, prostate, ovary, testes, and kidney. Inflammatory disorders, such as tuberculosis, can infrequently be associated with chylous ascites. In children, the most common causes are congenital abnormalities, such as lymphangiectasia, mesenteric cyst, and idiopathic "leaky lymphatics." Neoplasia is an uncommon cause of pediatric chylous ascites. Recently, chylous ascites has been reported in adults in association with hepatoma, small bowel angiosarcoma, retroperitoneal lymphoma, and jejunal carcinoid.

The incidence of spontaneous chylous ascites in patients with chronic liver diseases is estimated to be 0.5%. The lymphatics rupture spontaneously as a result of high portal pressures. Chylous ascites has been reported in patients with polycythemia vera due to portal and hepatic vein thrombosis.

Abdominal surgery is a common cause of chylous ascites. The surgical procedures most frequently associated with chylous ascites are resection of abdominal aortic aneurysm and retroperitoneal lymph node dissection. In one series of 329 patients with testicular cancer who underwent postchemotherapy retroperitoneal lymph node dissection, 7% of patients developed chylous ascites. Chylous ascites is also described after splenorenal shunt surgery.

Pathology

Dietary chylomicrons are absorbed in the small intestines and gradually pass along larger omental lymphatics to the cisterna chyli located anterior to the second lumbar vertebra. The cisterna is joined by the descending thoracic, right and left lumbar, and liver lymphatic trunks, and, collectively, these form the thoracic duct, which passes through the aortic hiatus and courses through the right posterior mediastinum and eventually enters the venous system. The thoracic duct carries lymphatic drainage from the entire body, except for the right side of the head and neck, right arm, and right side of thorax. Chylous effusions develop when these channels are injured or obstructed.

Based on animal experiments, Blalock concluded that obstruction of the thoracic duct alone is not sufficient to cause chylous ascites. Patients with a limited reserve of anastomotic channels are at greater risk of developing persistent ascites when obstruction or injury of the lymphatic channels occurs.



Clinical features

Abdominal distension is the most common symptom in patients with chylous ascites. Other clinical features include abdominal pain, anorexia, weight loss, edema, weakness, nausea, dyspnea, weight gain, lymphadenopathy, early satiety, fever, and night sweats. Fever, night sweats, and lymphadenopathy usually are observed in patients with lymphoma. Often, features of the primary illness, such as cirrhosis or of an associated malignancy, dominate the clinical picture. Rarely, it can present as acute peritonitis.

Complications

Sepsis is the most common complication, and sudden death has been reported in patients with chylous ascites. The prognosis in adult patients with chylous ascites is poor due to its association with malignancy and severe liver disease. However, pediatric patients and those with postsurgical and posttraumatic chylous ascites have a favorable prognosis.



Laboratory Studies

Routine laboratory tests may show hypoalbuminemia, lymphocytopenia, anemia, hyperuricemia, elevated alkaline phosphatase and liver enzymes, and hyponatremia. Usually, serum cholesterol and triglyceride levels are normal.

Abnormal liver enzymes are more common in patients with disseminated carcinoma than in patients with lymphoma or nonmalignant disorders. Anemia is common in patients with neoplasia.

The diagnosis of chylous ascites is made by peritoneocentesis and analysis of the ascitic fluid.

Ascitic fluid study

  • Color usually is white or milky.
  • Specific gravity is 1.010-1.054.
  • Total fat content is 4-40 g/L.
  • Triglyceride level is elevated in all patients. Typically, chylous ascites is diagnosed when the ascites triglyceride level is greater than 110 mg/dL. Levels as high as 8100 mg/dL have been described.
  • Glucose and amylase levels usually are normal.
  • Cholesterol level usually is low.
  • Leukocyte count generally is high, from 232-2560 cells/mm3, usually with a marked lymphocytic predominance.
  • Total protein content varies from 1.4-6.4 g/dL, with a mean of 3.7 g/dL. This variation reflects changes in serum proteins and dietary habits.
  • Microbiologic cultures usually are negative.
  • Gross milkiness of the ascitic fluid corresponds poorly with absolute triglyceride levels because turbidity also reflects the size of the chylomicrons.

Other diagnostic tests

  • CT scan
  • Lymph node biopsy
  • Laparotomy
  • Lymphangiography
  • Barium studies of the gastrointestinal tract
  • Bone marrow examination
  • Intravenous pyelography

CT scan, lymph node biopsy, and laparotomy carry the highest yield of diagnostic information. The role of MRI is not well defined. Lymphangiography can transiently worsen chylous ascites due to the oily contrast medium used for the test.



Because chylous ascites is a manifestation rather than a disease by itself, the prognosis depends on the treatment of the underlying disease or cause.

Supportive measures can relieve the symptoms. These measures include repeated peritoneocentesis, diuretic therapy, salt and water restriction, elevation of legs with use of supportive stockings, and dietary measures.

A low-fat diet with medium-chain triglyceride supplementation can reduce the flow of chyle into the lymphatics. Typically, medium-chain triglyceride oil is administered orally at a dose of 15 mL 3 times per day at meals. However, this approach is frequently not successful. One recent case report described the successful use of orlistat (Xenical) in a patient who had difficulty complying with a low-fat diet.

If chylous ascites persists despite dietary management, the next step may involve bowel rest and the institution of total parenteral nutrition. Bowel rest and total parenteral nutrition are postulated to be beneficial in patients with posttraumatic or postsurgical chylous ascites.

Paracentesis can result in immediate symptom relief; however, reaccumulation of fluid usually follows, and patients may require repeated paracentesis. Some authorities have advocated large-volume paracentesis. Morbidity from a single tap usually is low, but complications, such as peritonitis and hemorrhage, can occur. Transfusion of albumin and/or RBCs during paracentesis may help prevent hypovolemia in patients with hypoalbuminemia or anemia.

Peritoneovenous shunting has been used successfully in small numbers of patients with chylous ascites. However, shunt failure is common. Surgical revision of the shunt may be necessary.

Postsurgical chylous ascites usually resolves with supportive therapy. Early reoperation is indicated when the site of leakage is apparent and if the patient is a good operative candidate. Case reports now describe the laparoscopic treatment of chylous leaks, using suture ligation and fibrin glue to control the leak.

Use of TIPS to successfully treat chylous ascites related to cirrhosis has been reported.

Spontaneous healing of a chylous leak has been noted after lymphangiography performed to identify a lymph leak prior to a planned surgical therapy.

Multiple case reports describe the use of octreotide, a somatostatin analog, in the management of chylous ascites, typically at a dose of 100 mcg administered subcutaneously 3 times per day. Somatostatin receptors have been described in the lymphatic vessels of the intestine. In 1999, one group postulated that octreotide was effective in managing chylous ascites because it helped to decrease lymph flow through these vessels.

Malignant chylous ascites requires specific therapy directed at the primary cause and also supportive therapy. These therapies may include chemotherapy, radiation, and surgery. Laparotomy and ligation of the leaking lymphatics, resection of the leaking small bowel segment, and removal of an obstructing tumor all have been attempted with varying degrees of success. Transient success also has been achieved with peritoneovenous shunts. Laparotomy should not be used in pediatric patients with chylous ascites unless the condition is unresponsive to conservative therapy and a lesion that can be corrected by surgery is apparent.



  • Ablan CJ, Littooy FN, Freeark RJ. Postoperative chylous ascites: diagnosis and treatment. A series report and literature review. Arch Surg. Feb 1990;125(2):270-3. [Medline].
  • Ayers R. Chylous ascites and jejunal carcinoid: a diagnostic challenge. ANZ J Surg. Jul 2005;75(7):618-9. [Medline].
  • Berzigotti A, Magalotti D, Cocci C, et al. Octreotide in the outpatient therapy of cirrhotic chylous ascites: a case report. Dig Liv Dis. 2006;38:138-42.
  • Burdette WJ. Management of chylous extravasation. AMA Arch Surg. Jun 1959;78(6):815-28; discussion 828-31. [Medline].
  • Chen J, Lin RK, Hassanein T. Use of Orlistat (Xenical) to treat chylous ascites. J Clin Gastroenterol. 2005;39:831-3.
  • Evans JG, Spiess PE, Kamat AM, et al. Chylous ascites after post-chemotherapy retroperitoneal lymph node dissection: review of the M.D. Anderson experience. J Urol. 2006;176:1463-7.
  • Jensen EH, Weiss CA 3rd. Management of chylous ascites after laparoscopic cholecystectomy using minimally invasive techniques: a case report and literature review. Am Surg. 2006;72:60-3.
  • Kypson AP, Onaitis MW, Feldman JM, Tyler DS. Carcinoid and chylous ascites: an unusual association. J Gastrointest Surg. Sep-Oct 2002;6(5):781-3. [Medline].
  • Lamblin A, Mulliez E, Lemaitre L. [Acute peritonitis: a rare presentation of chylous ascites]. Ann Chir. Feb 2003;128(1):49-52. [Medline].
  • Malagelada JR, Iber FL, Linscheer WG. Origin of fat in chylous ascites of patients with liver cirrhosis. Gastroenterology. Nov 1974;67(5):878-86. [Medline].
  • Nix JT, Albert M, Dugas JE, Wendt DL. Chylothorax and chylous ascites; a study of 302 selected cases. Am J Gastroenterol. Jul 1957;28(1):40-53; discussion, 53-5.
  • Press OW, Press NO, Kaufman SD. Evaluation and management of chylous ascites. Ann Intern Med. Mar 1982;96(3):358-64. [Medline].
  • Weinstein LD, Scanlon GT, Hersh T. Chylous ascites. Management with medium-chain triglycerides and exacerbation by lymphangiography. Am J Dig Dis. Jul 1969;14(7):500-9. [Medline].
  • Widjaja A, Gratz KF, Ockenga J, et al. Octreotide for therapy of chylous ascites in yellow nail syndrome. Gastroenterology. 1999;116:1017-8.
  • Yamagami T, Masunami T, Kato T, et al. Spontaneous healing of chyle leakage after lymphangiography. Br J Radiol. Sep 2005;78(933):854-7. [Medline].
  • de Vries GJ, Ryan BM, de Bievre M, et al. Cirrhosis related chylous ascites successfully treated with TIPS. Eur J Gastroenterol Hepatol. Apr 2005;17(4):463-6. [Medline].

Chylous Ascites excerpt

Article Last Updated: Feb 8, 2007