Abstract
A syndrome with cholestasis in infancy and recurrent cholestasis and edema in the lower extremities in adulthood was presented in this Society in 1967. Further studies in our now 22 patients with this syndrome have shown that these children have abnormal lymph vessels in the legs before development of edema, and the cause of the edema is probably a congential lymph vessel hypoplasia. A liver lymphangiography with Au108 in one adult patient with no cholestasis at time of examination failed to show the normal clearance of the radioactive gold to the thoracic lymph nodes. To study the relationship between liver lymph flow and bile flow, an experimental animal model was devised: all lymph nodes draining the liver were obstructed in cats while control animals were sham-operated. In one group of cats, the bile flow was studied shortly after the lymph obstruction, in another group 1 day later, and in yet another group 2 days later. The bile flow was the same in the lymph-obstructed and the sham-operated groups of cats studied on the day of lymph obstruction; in the lymph-obstructed cats studied 24 hr after lymph obstruction the bile flow was about 70% higher and the bile acid excretion more than 100% higher than in the sham-operated group. Forty-eight hours after the lymph obstruction, the bile flow was only slightly elevated in the lymph-obstructed group. Following the bile flow study an electron-dense material (lanthanum) was injected in the common bile duct, and biopsies from different parts of the liver were studied in the electron microscope. The injected lathanum could be traced from the bile canaliculi to the intercellular spaces and to the Disse's spaces. The findings of this intimate relationship, both anatomically and functionally between the liver lymphatics and the biliary tree, compared with the clinical findings of abnormal peripheral lymphatics and an abnormal liver lymphangiography, indicate that a lymph anomaly in the liver may be the cause of the cholestais in these patients.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Aagenaes, O., Sigsted, H., Cuderman, B. et al. 47. Clinical and experimental relationship between liver lymphatics and bile flow. Pediatr Res 5, 93 (1971). https://doi.org/10.1203/00006450-197102000-00052
Issue Date:
DOI: https://doi.org/10.1203/00006450-197102000-00052