Introduction

Cutaneovisceral angiomatosis with thrombocytopenia (CAT), also known as multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), is a rare vascular malformation. CAT can be a multi-organ disease with associated life-threatening bleeding. Liver cirrhosis with nodular formation is a rarely reported association. We describe a patient with CAT/MLT who suffered from recurrent GI bleeding, found to have decompensated liver cirrhosis who underwent liver transplantation with resolution of GI bleeding. 

Methods

Case: A 13-year-old female with CAT presented with recurrent GI bleeding. Endoscopy showed visceral lesions throughout the GI tract. She required therapeutic endoscopic interventions. Subsequently, she was found to have decompensated, cirrhotic nodular liver with large ascites on CT scan. She underwent liver transplantation in March of 2022. Her liver explant showed regenerative nodules without evidence of malignancy.

Results

The patient is currently 15 months from transplant with stable graft function. Prior to transplant, she was on sirolimus, given prior success with use of mTOR inhibitors in disease regression. Post-transplant, she has been maintained on tacrolimus for immunosuppression with no recurrence of GI bleeding.

Conclusion

Currently, there is no consensus with management of CAT. To date, there is one similar case of a patient with CAT who also presented with acute liver failure; her recurrent GI bleeding resolved following liver transplant. The mechanism that leads to resolution of GI bleeding after liver transplant is unclear. Patients with CAT should undergo thorough hepatic investigation. Liver transplant may be an optimal choice for CAT patients who present with both hepatic and GI manifestations of disease.