2017 - CIRTA

Introduction:  Parenteral Nutrition (PN) is a lifesaving treatment for patients with Intestinal Failure. PN is usually administered through a central venous catheter placed in one of 6 main veins:  the right/ left internal jugular, right/left subclavian and right/left femoral. The high blood flow in these vessels allows proper mixture of macronutrients to prevent complications of high osmolar solution. Current Medicaid guidelines recognize loss of 2 access sites as an indication for intestinal transplant.

Methods:  We describe a cohort of pediatric Intestinal Failure (IF) patients evaluated at our center who underwent upper and lower extremity Venous Doppler Ultrasound and MRV of the Neck/Chest/Abdomen and Pelvis to assess Central Venous Line Associated Thrombosis.

Result: From January 2013 to December 2016 we evaluated 51 children with intestinal failure. Of these, 41 patients had concomitant studies, dopplers and MRV, to evaluate presence of thrombosis.  The age at evaluation ranged from 6 months to 19 years. 26 patients were male. ~60% (26 of 41) of the patients had Short Bowel Syndrome leading to IF. Other etiologies for IF included Dysmotility Syndromes (n=10), Microvillus Inclusion Disease (n=2), Thrombosis (n=1) and graft failure (n=2).
Venous Doppler Ultrasound identified thrombosis in 14 patients (~34%) ranging from 1-3 sites. MRV identified thrombosis in 28 patients (~68%) ranging from 1-6 sites involved. There were 14 patients (~34%) where no thrombosis was found by means of Venous Doppler but thrombosis was documented by means of MRV.  Three patients were urgently listed for transplant with appeal due to significant loss of access identified on MRV not identified on Venous Doppler.  Two patients were denied for transplant given insufficient access available identified on MRV; Venous Doppler Ultrasound on these patients did not recognize severity of thrombosis.  There were no adverse effects of sedation administered during imaging modalities. 

Discussion: Over the last decade, new surgical modalities and lipid sparing therapies have advanced the field of IF allowing patients to remain transplant-free for a longer period of time.  However, controversy remains in prevention and treatment of Central Line Associated bacterial and fungal infections leading to early removal of central lines with the potential for thrombosis formation thereafter.  Given venous accessibility is imperative to long term PN administration, screening modalities to identify thrombosis should be instituted as part of every Intestine Rehabilitation Program.  Early recognition of thrombosis is important as loss of significant access may preclude candidacy for transplantation. Our data suggest that MRV should be considered the gold standard modality as compared to Venous Doppler Ultrasound despite presumed risk associated with sedation.