Lymphatic disorders (Proceedings)

Chylothorax

The etiology of chylothorax is often not identified. Although trauma is listed as a potential cause in some sources, there is no evidence that it would be responsible for persistent chylothorax. Causes included R-CHF, thoracic lymphangiectasia, generalized lymphangiectasia, cranial mediastinal masses, fungal granulomas, venous thrombi, or congenital abnormalities of the thoracic duct.

Breed predispositions include the Afghan hound (middle-aged), Shiba Inu (<1 year of age), and Oriental cat breeds.

Suggested medical therapy typically includes low-fat diets, thoracocentesis as needed, and Rutin (50-100 mg/kg TID PO). The success of any one of these is questionable. Spontaneous resolution may occur without therapy. Rutin is a benzopyrone extracted from plants. Its mechanism of action is unknown but it is suspected to reduce leakage of fluid from blood vessels, increase proteolysis in extravascular sites, and enhance macrophage phagocytosis of chyle.

Suggested surgical therapy includes thoracic duct ligation with pericardiectomy. Complete ligation of the thoracic duct is difficult without mesenteric lymphangiography. This author (Harkin) believes that continuous pleural evacuation following surgery is important for guaranteeing success in addition to pericardectomy and TD ligation. Palliative procedures have also been developed (pleuroperitoneal shunting) but are often fraught with complications and high expense.

In their study on thoracic duct ligation with concurrent cisterna chyli ablation (Hayashi K, Sicard G, et al. Cisterna chili ablation with thoracic duct ligation of chylothorax: results in eight dogs. Vet Surg 2005;34:519-523.), the authors stated that the goal of TD ligation is to block the flow of chyle to the area of leakage and result in the formation of new lymphaticovenous anastomoses within 5-14 days to permanently divert chyle from the leaking thoracic duct and thoracic cavity. They point out that TDL reportedly results in complete resolution 50-60% of the time. This failure could be related to development of collateral lymphatics that bypass the ligature, failure to ligate all channels of thoracic duct, or ligation rostral to site of leakage. The authors proposed that ablation of the CC (the cisterna chyli is an elongated saccular reservoir receiving lymph from the lumbar and mesenteric lymphatic trunks that then empties into the thoracic duct at the level of the dorsal diaphragm) would prevent recurrence of chylothorax. They looked retrospectively at 8 dogs with idiopathic chylothorax, three which had received Rutin to no avail. One dog never resolved and was euthanized at 2 months; 1 dog had effusion for one month, then no additional effusion for 4 months when it died suddenly; and 6 dogs had good long-term outcome with no additional fluid accumulation. Whether CCA made the difference in these dogs is unknown without a comparison population of TDL alone. The efforts made to identify all branches of the TD may have had the most significant contribution to success.

In their evaluation of pleuroperitoneal shunts (Denver shunt) (Smeak DD, Birchard SJ, McLoughlin MA, et al. Treatment of chronic pleural effusion with pleuroperitoneal shunts in dogs: 14 cases (1985-1999). J Am Vet Med Assoc 2001;219:1590-1597.) the authors noted short term complications of obstruction from tube kink, infection around external pump (2), pump chamber dislodgement from thorax, pain on pumping, pump obstruction, owner compliance (2), and acute collapse just after discharge (1). The long-term complications included pump obstruction (3), marked abdominal distension (3), pump dislodgement (1), pyothorax (1), peritonitis (2), pleural compartmentalization (1), and owner compliance (1). Three dogs were reported not to have long-term complications, although one was euthanized because rate of fluid accumulation exceeded rate at which it could be removed and in one dog fluid accumulation stopped 6 weeks after removal of thymoma. Given the expense of the shunt itself (>$800), failure to resolve fluid accumulation, and complications, I would not recommend this procedure to any client.

In this author's opinion, concurrent TDL and pericardiectomy are the preferred surgery. In one study (Fossum TW, Mertens MM, Miller MW, et al. Thoracic duct ligation and pericardectomy for treatment of idiopathic chylothorax. J Vet Intern Med 2004;18:307-310.), in which 10 dogs and 10 cats with idiopathich chylothorax were treated (TDL and PC were performed simultaneously in 13; PC was performed 2 and 9 months later in 2 dogs; 1 dog and 1 cat had PC without TDL; in 2 the PC was performed after TDL elsewhere and one had repeated TDL; and other combinations of the above...) pleural fluid accumulation resolved in 10/10 dogs and 8/10 cats. Fibrosing pleuritis was evident in 5 cats and 1 dog. Decortication was performed in 2 cats, both of which developed persistent post-op pneumothorax (resolved in both).

Lymphangiectasia

Intestinal lymphangiectasia is one of several causes of protein-losing enteropathy. Either primary or secondary pathologic dilation of intestinal lymphatics and subsequent loss of lymph into the intestines, resulting in panhypoproteinemia, lympopenia, and hypocholesterolemia. Lymphangiectasia is far more common in the dog than in the cat, although it has been reported in the cat, including 3 of 43 cats in one retrospective study (these 3 cats did not have a PLE and their lesions were not as severe as is seen in dogs). The most common clinical signs are diarrhea (100% of dogs in some studies), anorexia, lethargy, vomiting, and weight loss. The onset can be described as acute or chronic and the diarrhea can be either small or large bowel. Ascites is obvious in about half of the patients on original presentation and body condition is variable. The classic laboratory findings of panhypoproteinemia, leukopenia, and hypocholesterolemia is seen less frequently than expected (approximately 40-60% of cases), although hypoalbuminemia is more consistent (~80%) and likely reflects the severity of disease. Another common finding is low ionized calcium (100% in one study, even though total calcium (corrected) was low in only 50%). In a recent retrospective study on full-thickness biopsies from dogs with chronic G signs evaluated by a pathology service (Vet Pathol 2006;43:1000-3) from 64 dogs, lymphangiectasia was the most common finding (38 dogs: 29 had transmural LA) without accompanying inflammation.

Primary lymphangiectasia is a congenital disorder and typically is limited to intestines, although a more widespread lymphatic disorder can be seen with accompanying chylothorax. The disease manifests in adulthood, but development of lipogranulomas may be a precipitating factor for the late onset of symptoms (as opposed to puppyhood). Commonly affected breeds include Yorkshire and Maltese terriers and the Norwegian lundehund.

Secondary lymphangiectasia is a consequence of obstructed lymphatics, which can be localized to the intestinal tract in the case of various inflammatory bowel diseases (especially lymphoplasmacytic enteritis), or may be secondary to right-sided heart disease, intra-abdominal or intra-thoracic neoplasia, disease of the thoracic duct, fungal disease with lymphadenitis, or any other disease that disrupts lymphatics.

Treatment is directed at the underlying cause, if present and found. For those dogs with idiopathic lymphangiectasia (presumed congenital), corticosteroids may still be beneficial if secondary inflammation from leaking lymph is inducing lipogranuloma formation. A diet that is restricted in fat is still recommended, although the true value of this is questionable. Ultimately, dogs with primary lymphangiectasia have a poor prognosis and are either euthanized due to progressive loss in body condition or intractable diarrhea or develop MODS from severe malnutrition.