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The Future of Small Bowel Transplantation

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The Future of Small Bowel Transplantation Archives ofDisease in Childhood 1995; 72: 447-451 447 CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.72.5.447 on 1 May 1995. Downloaded from The future of small bowel transplantation Deirdre A Kelly, John A C Buckels Although transplantation of solid organs such Indications for small bowel as kidney and liver have been established since transplantation the 1970s and 1980s, the future of small bowel Small bowel transplantation should be con- transplantation as treatment for intestinal fail- sidered for all children with irreversible ure is only just evolving. Early clinical experi- intestinal failure who are dependent on par- ence was almost universally unsuccessful but enteral nutrition for survival and in whom all recent developments in surgical technique, attempts to improve intestinal adaptation have immunosuppression, and postoperative man- been unsuccessful. Potential candidates may agement have improved outcome with a 50% require small bowel transplantation alone or survival in at least 100 operations.' combined with a liver transplant. Small intestinal transplantation was first The current indications for isolated small demonstrated to be technically feasible in bowel transplantation include: (1) loss of animals in 1902,24 and in humans in the vascular access with normal liver function and 1980s,5-7 but it was not until the Pittsburg histology and (2) intractable gastrointestinal group initiated a programme of small bowel fluid loss. The current indications for com- transplantation using the novel immuno- bined small bowel/liver transplantation are: (1) suppressant agent FK5068-12 that significant irreversible liver disease secondary to TPN; (2) progress was made. loss of vascular access with evidence of signifi- cant liver disease; and (3) inborn errors of liver metabolism leading to small bowel failure (for Intestinal failure example, protein C, S deficiency).24 25 Intestinal failure is defined as the inability of At the moment it is hard to justify small the small intestine to absorb sufficient solute to bowel transplantation in those patients who are maintain nutrition and/or positive fluid and successfully managed on TPN, although it is electrolyte balance.'3 It is more common in possible that in the future social, psychological, http://adc.bmj.com/ children, and the main causes are congenital and cost pressures may increase the indications abnormalities of the intestine and the short gut for transplantation. The contraindications syndrome secondary to neonatal surgery for a include uncontrolled sepsis or malignancy else- number of conditions (table). where in the body, HIV infection and severe Current treatment for intestinal failure is cerebral, cardiac, or respiratory disease. total parenteral nutrition (TPN) and many Congenital immunodeficiency may be a par- patients may be successfully maintained in this ticular contraindication because of the devel- on October 4, 2021 by guest. Protected copyright. way by specialist units.l4-16 Although success- opment of severe graft versus host disease ful provision of TPN at home has greatly (GVHD) from donor lymphocytes contained improved the outcome and quality of life for within the intestinal graft.26 Currently there children with short bowel syndrome, morbidity are no age or size limitations, although in prac- remains significantl7-'9 and the financial cost tice donor availability may preclude intestinal high.20 There are many complications which transplantation in very small infants. include cholestasis, cholelithiasis, pulmonary embolism, loss of vascular access, and recur- rent catheter sepsis.21-23 Patient selection and preparation Death in childhood is related to the develop- TIMING ment of liver dysfunction, as more than As quality of life may be acceptable for many 40% ofpatients with complete intestinal failure children maintained on parenteral nutrition will develop severe liver disease within two the timing of this operation may be difficult. Liver Unit, Birmingham years.22 Furthermore, the difficulty in obtaining suit- Children's Hospital, able age and size matched donors for very Ladywood Middleway, small children means a lengthy wait for a suit- Birmingham B16 8ET Causes ofintestinalfailure in childhood able organ. In Pittsburg 37% of patients died D A Kelly while awaiting small bowel transplantation.9 Short gut syndrome secondary to neonatal surgery for: Department of Intestinal atresia We have recently evaluated 17 children Surgery, Queen Gastroschisis (aged 4 months to 13 years) for small bowel Elizabeth Hospital, Volvolus Birmingham Necrotising enterocolitis transplantation. Ten children were referred J A C Buckels Aganglionosis with liver disease and seven with loss ofvenous Microvillus inclusion disease access. of children 11 died Correspondence to: Autoimmune enteropathy In this group pre- Dr Kelly. operatively (seven were too ill to place on the 448 Kelly, Buckels transplant list and four died waiting for a isolated small bowel transplantation or com- donor. The children with the highest plasma bined liver and small bowel transplantation. bilirubin concentration at the time of evalua- Current experience suggests that combined tion had the shortest duration of survival.27 liver and small bowel transplantation may Arch Dis Child: first published as 10.1136/adc.72.5.447 on 1 May 1995. Downloaded from Thus it is important to refer children for small reduce the chances of intestinal rejection post- bowel transplantation as soon as hepatic dys- operatively.7 It is possible that patients with function becomes apparent or there are diffi- early liver disease may have progression of culties with vascular access in order to allow TPN cholestasis postoperatively particularly if sufficient time for assessment and obtaining a there are problems with establishing intestinal donor. function28 and thus the liver should be replaced if significantly affected. It is important to ensure that complications Assessment related to either liver or intestinal failure are The assessment for small bowel transplanta- medically managed and sepsis is effectively tion focuses on establishing: (1) hepatic and treated. It is our practice to start selective bowel intestinal function; (2) patent vessels; and (3) decontamination (polymyxin B, gentamicin, physical and psychological preparation of child and amphotericin administered orally) once and family. children are placed on the waiting list. GASTROINTESTINAL FUNCTION Donor procurement and operative It is necessary to establish that intestinal failure technique is irreversible. The precise anatomy, motility, Donor procurement and operative technique and function of the remaining gastrointestinal are now well established.29 30 tract should be assessed. Gastric emptying Grafts must be ABO blood group com- studies and upper and lower gastrointestinal patible and preferably matched for endoscopy including histology of the bowel cytomegalovirus status. Selection of donor size should be performed. The capacity of the peri- is of critical importance as previous surgery in toneal cavity should be measured as this may the recipient often leads to extensive adhesions be a limiting factor in donor selection. and contraction of the peritoneal cavity so that the ideal donor should weigh approximately 20% less than the recipient. For isolated small HEPATIC FUNCTION bowel transplantation the entire small bowel It is important to assess the extent of together with the right colon is removed with fibrosis/cirrhosis and portal hypertension, its vascular pedicle comprising the superior especially in patients without overt liver mesenteric artery and origin of the portal vein disease. (fig 1). Recent experience has demonstrated reasons vascular to in the colon with For technical the supply advantages including right http://adc.bmj.com/ the liver needs to be determined by ultrasonic the small bowel in terms of fluid and short radiology. The presence or absence of gall chain fatty acid absorption. In combined grafts stones and common bile duct stones should be the same bowel is removed but the portal vein established by ultrasound or endoscopic retro- is left in continuity with the liver (fig 2). In grade cholangiopancreatography. both situations the organs are perfused with University of Wisconsin Solution which will allow preservation for up to 10 hours. VASCULAR ACCESS In isolated small bowel grafts the superior on October 4, 2021 by guest. Protected copyright. The presence of patent vessels and vascular mesenteric artery is anastomosed to the aorta, thrombosis is usually established using usually via a conduit of donor iliac artery, and Doppler ultrasound, although angiography venous drainage established by anastomosing may be required. the donor portal vein to the side of the recipi- ent portal vein. The upper end of the graft is GENERAL ASSESSMENT Anthropometry to measure nutritional status and careful cardiac and pulmonary assessment Superior mesenc t vpin ! tI/sSuperior mesenteric to establish incidence of pulmonary embolism from central venous lines23 is essential. PSYCHOLOGICAL ASSESSMENT As small bowel transplantation remains a new procedure in the UK it is important that the family are honestly appraised of the risks and complications of this procedure in order to make an informed decision. The psychological preparation of the child is paramount and should include preparation for a stoma and Figure 1 Isolated small bowel transplantation. The donor isolated small bowel is removed with the right colon. The ileostomy. superior
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