Chylaskos bei Lymphangioleiomyomatose: Peritoneo-venöse Ableitung mittels Denver-Shunt^*

Abstract

Chylous ascites, due to a lymphangiomyomatosis and resistant to conservative therapy, occurred in a 30 year-old female. A distinct subjective and clinical improvement could be attained with the aid of a peritoneo-venous anastomosis employing a Denver-Shunt. With the preconditions that the implantation is correct and that it is reliably managed, we regard the Denver-Shunt system as a recommendable approach to the treatment of chylous ascites not controllable by conservative measures.

Peritoneovenous Shunt After Failure of Octreotide Treatment for Chylous Ascites in Lymphangioleiomyomatosis

“…That is why we finally decided to implant a peritoneovenous shunt. This therapeutic approach had been reported successfully in a few cases of LAM [2,9,10]. The risks of thrombosis or infection of the shunt in refractory ascites are well recognized [11], but to our knowledge no report mentions greater complication rates for chylous ascites.…”

Lymphangioleiomyomatosis: A Disease Involving the Lymphatic System

“…Successful instillation of a peritoneo-venous shunt for intractable chylous ascites has also been reported by several groups. 78,92 Rapamycin, an mTOR inhibitor, is an attractive possibility since constitutively activated mTOR due to the loss of TSC1=TSC2 function is the pathogenic basis of LAM. Sirolimus, a type of rapamycin, has been successfully used for the treatment of TSC-associated diseases including LAM and AML.…”