MANAGEMENT AND OUTCOME OF EXTREME END STAGE VENOUS ACCESS IN CHILDREN WITH INTESTINAL FAILURE (IF) ASSESSED FOR SMALL BOWEL TRANSPLANTATION (SBTX)

Abstract

3- year survival after SBTx has improved to between 73% and 88%. Impaired venous access is an indication for SBTx in children with chronic IF, whereas thrombosis of the superior vena cava (SVC) was a contra-indication to SBTx, historically. Aim: To report our experience in management of children with extreme end stage venous access. Subjects: 6 children (all males), mean age of assessment 30 months (range 13 - 52). Diagnosis: Total intestinal aganglionosis1, protracted diarrhoea1, short bowel syndrome4 of which gastroschisis2 and malrotation with mid gut volvulus2. All presented with thrombosis of the SVC and or inferior vena cava and malnutrition because of interruption of parenteral nutrition. All had venograms and/or MR angiograms to access venous access. All had a documented history of 6-18 central venous line (CVL) insertions previously. Methods: Venous access was achieved as follows: Transhepatic venous catheters (THVC) (5), Right atrial (RA) CVL via midline sternotomy4, Azygous venous system2, dilatation of left subclavian vein following passage of a guide wire and then placing a CVL to reach the RA1 and SVC dilatation, stent insertion and placement of a CVL in situ1. Complications included pleural effusion, which resolved after chest drain insertion, following RA insertion1, displacement of THVC needing repositioning2, and balloon dilatation of SVC stent after 16 and 22 months to restore patency. Outcome: 4 children with permanent IF on assessment were offered SBTx, 3 of which were transplanted and were established on full enteral nutrition while the family of one child declined the procedure (and he died 3 years later). In the remaining 2 children in whom bowel adaptation was still a possibility, attempts were made to provide adequate venous access as feeds and manipulations of intestinal motility drugs were undertaken. One child received SBTx 3 years after his first assessment, as bowel adaptation failed and the other child (the sixth subject) died in the immediate post operative period following a THVC insertion, but the cause of death is inconclusive. RA lines in transplanted children proved adequate for the management of uncomplicated transplantation, although the usual infusion protocol had to be modified to take account for the lack of access. Conclusion: It is possible to re-establish central venous access using unconventional techniques. However it is difficult and time consuming to assemble a skilled team consisting of one of more: surgeons, cardiologists, interventional radiologist, transplant anaesthetists. If SBTx is inevitable it is imperative as well as easier and safer with adequate venous access and we advocate liaison with a SBTx centre at an early stage.