Search

Background: When total parenteral nutrition (TPN) is not an option, intestinal transplantation is the sole treatment for patients with end-stage intestinal failure to increase the chance of long-term survival. However, in 20-33% of patients, abdominal wall-related complications occur after isolated intestinal transplantation., Case Description: The patient is a 24-year-old woman with ultra-short bowel syndrome, caused by a severely complicated history of Crohn's disease. After 5 years of TPN, the patient was referred for intestinal transplantation. In addition, an abdominal wall transplant was required due to an abdominal wall defect, extensive scarring of the abdominal wall and lack of free space within the abdomen. Therefore, a combined intestinal and abdominal wall transplantation was performed. Six months after transplantation the patient has a sufficient abdominal wall, a normal body mass index and no longer requires any feeding lines., Conclusion: This case report describes the first combined intestinal and abdominal wall transplantation in the Netherlands and in the Eurotransplant region in a patient with end-stage intestinal failure and loss of abdominal domain.

Objective: To evaluate an intestinal rehabilitation program, introduced in 2001, for children who developed short bowel syndrome (SBS) after extensive intestinal resection., Design: Retrospective cohort study., Method: General information on disease status and outcome of intestinal rehabilitation plus demographic data on all SBS patients, treated in the programme between 2001 and 2009, were collected. The outcome measures were intestinal autonomy, intestinal and/or liver transplantation and survival., Results: A total of 10 boys and 9 girls, born at a median gestational age of 36 weeks, were treated according to intestinal rehabilitation programme guidelines. Eight of them had been referred to our centre from elsewhere, 3 times as many as in 1991-2000. The causes of SBS were: intestinal atresia (n = 3), gastroschisis (n = 2), volvulus (n = 9), necrotising enterocolitis (n = 3) or strangulation (n = 2). Median remaining small-intestinal length was 35 cm (range: 10-70 cm). In 14 patients the ileocecal valve was still present and in all 19 patients at least 25% of the colon remained. The median follow-up period was 25 months (range: 50 days-9 years). Following total parenteral nutrition lasting a median 138 days (range: 41 days-11 years), 16 patients (84%) reached intestinal autonomy. Central venous catheter-related complications occurred in all of the patients; liver function disorders in 13 (68%) and failure to thrive in 5 (26%). One patient underwent an intestinal lengthening procedure. None of the patients needed an intestinal transplantation, but one patient underwent a liver transplantation for intestinal failure associated liver disease. Overall mortality was 11%: 2 patients died of abdominal sepsis., Conclusion: The specialised intestinal rehabilitation programme led to intestinal autonomy in 84% of the patients with SBS.

Patients with intestinal failure, predominantly caused by short-bowel syndrome, have impaired quality of life due to the frequent development of complications. Dietary modifications have an established role in the treatment of short-bowel syndrome. Treatment of short-bowel syndrome includes optimising the balance of fluids and nutrients in the presence of reduced absorption. The population is heterogeneous due to differences in anatomical structure and the functional status of the remaining intestine. Diet must therefore be tailored to the individual patient. Determining the appropriate amount of carbohydrates is based on the presence of the colon, because carbohydrates are processed in the colon by bacterial fermentation. Patients with a jejunostomy rapidly become dehydrated because they lose more sodium and fluids than are taken up enterally. The jejunum rapidly absorbs solutions with high salt concentrations, such as the WHO-recommended oral rehydration solution. Replacement of long-chain fatty acids with water-soluble medium-chain fatty acids increases the energy intake in patients with short-bowel syndrome and a colon. Extra attention should be given to electrolytes, trace elements and vitamins. Patients with short-bowel syndrome and a colon are at risk for oxalate nephropathy. For these patients, a low oxalate diet is recommended. With these interventions, many patients with intestinal failure will ultimately become independent of total parenteral nutrition.

Small bowel transplantation for intestinal failure is no longer an experimental procedure, but an accepted treatment for patients where total parenteral nutrition (TPN) therapy for intestinal failure is unsuccessful. Early referral for screening for small bowel transplantation should be considered in patients with permanent intestinal failure who have occlusion of more than 2 major veins, frequent line-related septic episodes, impairment of liver function or an unacceptable quality of life. With the increased experience in post-transplant patient care and newer forms of induction (thymoglobulin, IL-2 receptor antagonists) and maintenance (tacrolimus) therapies, the 1-year graft survival has increased to 65% for isolated and to 59% for liver/small bowel transplantation and is further improving. Rejection, bacterial, fungal and viral (Cytomegalovirus, Epstein-Barr-virus) infections, post-transplant lymphoproliferative disease and graft versus host disease are the most common complications after intestinal transplantation. Although most of the long-term survivors are TPN-independent and have a good quality of life, the risk of the procedure and long-term adverse effects ofimmunosuppressive medication limits small bowel, or liver/small bowel transplantation only to patients with severe complications of TPN therapy.

Three children, 2 boys aged 5 and 4 years, and 1 girl aged 2 years, who suffered from intestinal failure as a result of a short bowel syndrome and who were dependent on total parenteral nutrition (TPN), were screened for small bowel transplantation. The girl also had progressive liver failure. Each child had a clear indication for either isolated small bowel or combined small bowel/liver transplantation. All three children were enrolled in an intestinal rehabilitation program because they were referred early. The intestinal rehabilitation consisted of a systematic analysis of the absorptive capacity of the residual intestine, optimizing enteral and parenteral feeding, prevention of catheter sepsis and further deterioration of liver function. The rehabilitation was performed in an in-patient setting. All three children were partially weaned from TPN, while their growth improved. None of the children were consequently listed for small bowel transplantation. When a child is initially referred for small bowel transplantation, intestinal rehabilitation should be considered as part of an intestinal transplantation program.