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Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.
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PMID:Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. 1132 44

Short bowel syndrome is a spectrum of malnutrition resulting from inadequate bowel length. In infant and pediatric patients, the most common causes are necrotizing enterocolitis, abdominal wall defects, jejunal ileal atresia, and mid gut volvulus. There appear to be regional variations in etiology. Since the publication of Wilmore's classic monograph in 1972, there have been significant improvements in monitoring and nutritional support. In the modern era, survival rate ranges from 80% to 94%, and the presence or absence the ileal cecal valve appears to not impact on mortality rate, but does significantly affect the length of time on total parenteral nutrition TPN. The most common morbidities remain sepsis, both central line related and bacterial overgrowth, and TPN cholestasis. Long-term recovery of these children often is remarkably normal, but there is a 10% to 15% incidence of neurologic and developmental defects. The clinical and ethical considerations around the care of infants with 20 to 40 cm of residual bowel remains controversial, as does the place of intestinal transplantation, especially in patients developing gut failure in infancy. Perioperative surgical decision making plays a critical role in the long-term outcome of these patients. This chapter presents an overview of the current status of care and outcome in this difficult population; these topics are further expanded in subsequent chapters.
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PMID:Short bowel syndrome in infants and children: an overview. 1132 5

Intestinal transplantation has emerged as a feasible alternative in the treatment of children with short gut syndrome. The challenges in the management of these patients include maintaining a tight balance between the degree of immunosuppression necessary to prevent graft-versus-host disease and rejection. At the same time, this amount of immunosuppression is associated with a high risk for lymphoproliferative disorders and intestinal-derived sepsis. Current 3-year patient and graft survival rates are 55% and 50%, respectively. The indications, morbidity, and timing for referral are discussed.
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PMID:Intestinal transplantation for children with short bowel syndrome. 1132 11

Sepsis in short bowel syndrome (SBS) is due in part to bacterial translocation (BT). Parenteral nutrition (PN) is often necessary in SBS and promotes BT. The presence of ileocecal valve (ICV) has been considered as a good prognostic factor in the outcome of this children. The aim of this study was to asses the effect of the presence or absence of ICV and cecum in five different models of gut resection in the rat. Fifty-five adult Wistar rats were randomly assigned to one of five groups: Group 1 (N = 14): standard rat chow + 80% small bowel resection. Group 2 (N = 10): standard rat chow + 80% small bowel resection including cecum. Group 3 (N = 10): standard rat chow + 80% small bowel resection including ICV. Group 4 (N = 11): NP + 80% small bowel resection. Group 5 (N = 10): NP + 80% small bowel resection including ICV and cecum. Ten days after surgery they were sacrificed and mesenteric lymph nodes (MLN), spleen and peripheral (PBL) and portal blood (POBL) specimens were recovered and cultured. Groups 3 (without ICV, with cecum) and 5 (without ICV, without cecum) showed 60% BT in MLN and POBL, and groups 1 and 4 (with ICV, without cecum) 93% and 91% respectively (p < 0.05). In PBL, group 3 (without ICV, with cecum) showed also less BT than groups 1 and 4 (10% vs 43% and 55% respectively, p < 0.05) and group 5 (without ICV and cecum) had less BT than groups 1, 2 and 4 (0% vs 43%, 30% and 55%, p < 0.01). In conclusion, these results suggest that the absence of ICV decreases BT and that the cecum does not seems to play a role on his.
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PMID:[Bacterial translocation associated with short bowel: role of ileocecal valve and cecum]. 1148 Jan 92

The treatment of children with intestinal failure should be predicated upon three overriding goals: 1) to keep the patient well nourished by parenteral nutrition (TPN), 2) to minimize the fecal loss of fluid, electrolytes, and nutrients, and 3) to enhance the natural process of intestinal adaptation whenever possible. The first goal is relatively easy to accomplish in the short- or intermediate-term, but difficult to accomplish for more than a few years because of recurrent septicemia, loss of venous access, and cholestatic liver disease. The risks of sepsis and loss of venous access can be minimized through meticulous central line care and the use of appropriate antibiotics when indicated. Cycling TPN and limiting parenteral protein intake sometimes ameliorates cholestasis. The second goal is only partially achievable regardless of the cause of intestinal failure. Fluid and electrolyte secretion often can be reduced but not normalized with antisecretory drugs. Bacterial overgrowth can be treated with the judicious use of antibiotics. The third goal generally can be accomplished only in a subpopulation of patients with surgically created short bowel. In these children, a satisfactory increase in surface area can occur only if nutrients are delivered directly into the bowel lumen. The trophic effects of glutamine, growth hormone, and other hormones remain to be universally accepted. Surgical bowel lengthening or bowel tapering can sometimes enhance intestinal function among patients with short bowel syndrome. If medical or nontransplantation surgical management of intestinal failure is unsuccessful, and the patient develops irreversible TPN-associated complications, transplantation of the intestine should be strongly considered.
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PMID:Small Intestinal Failure in Children. 1156 Jul 89

Advancements in medical technology over the last decades have greatly benefited perioperative care of newborns undergoing major surgical interventions. Yet, a proportion of these babies will not survive, and doctors are forced to face the difficult ethical question of whether, in cases of severe congenital malformations or acquired diseases, the expected quality of life justifies the decision to continue, withhold, or withdraw treatment. In a tripartite approach, the authors present their relevant experiences with these newborns in the pediatric surgical department of the Sophia Children's Hospital (SCH). First the authors evaluated the mortality pattern and causes of death in surgical neonates over 2 periods (1986 through 1990 and 1996 through 2000). The mortality rate was the same, 10%. Pattern of mortality was classified into 3 groups: nonpreventable, permissible, and preventable death. The most striking difference between both mortality groups existed between the percentage of preventable deaths, 14.5% in the earlier period, versus 5% in the most recent period. Half of the preventable deaths in the former period occurred in relation to postoperative, infectious treatment complications, such as inadequate sepsis management. These errors were not seen anymore in our recent evaluation. Second, the authors studied the physical and psychosocial adjustment of former patients, particularly those operated on for digestive tract anomalies. The follow-up period encompasses from 8 to 12 years showing that the physical functioning of these children was relatively good. With respect to their cognitive and psychosocial functioning it seems justified to conclude that they are at risk for lower cognitive functioning, learning problems, and possibly lower educational levels. Third, the authors decided that ethical questions related to surgical treatment of necrotizing enterocolitis (NEC) actually fits within the scope of this article. They hypothesized that a particular minimum birth weight could be a decisive factor for refraining from surgery on ethical grounds in the treatment of this particular disease. A total of 116 patients with NEC were identified over a 5-year study period. Seventy-five fell into the lowest birth weight group (<1,500 g). The operative mortality rate (21 patients) increased with decreasing birth weight: group A, 29% (A1, 31%; A2, 26%); group B, 23%; and group C, 0%. The authors were particularly interested in the long-term follow-up of the A1 group (BW < 1,000 g), and 18 of the 30 survivors of this group were available for follow-up. There were 2 survivors with short bowel syndrome. Early assessment and neurodevelopmental outcome, at least 2 years after surgery, showed that in 14 of these 18 (78%) there were no major handicaps. The other 4 patients had one or more major handicaps (cerebral palsy, mental and/or visual handicap, and hearing loss). NEC-related mortality after surgery is highest for patients with birth weight less than 1,000 g (31%). However, in view of the overall good 2-year follow-up results of the survivors, we feel unable to define a minimum birth weight that, as such, could serve as a cutoff point for deciding to forbear, on ethical grounds, surgical treatment for NEC. The picture represented by our tripartite investigation of life and death in and after the intensive care unit period serves as a mirror and truthfully reflects ethical questions of our medical practices.
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PMID:The neonate with major malformations: experiences in a university children's hospital in the Netherlands. 1168 96

From an experimental procedure, intestinal transplantation (ITx) has evolved over the last 10 yr into a treatment option for patients suffering from short bowel syndrome and who develop life-threatening complications from total parenteral nutrition (TPN) (e.g. liver dysfunction, line sepsis, shortage of venous access, etc.). One-year survival rates are approximately 70% and thus similar to lung Tx. However, the intestine remains the most challenging abdominal organ to transplant. This is because of the severe immune response (mostly rejection) that is produced, and therefore the need for profound immunosuppression with its attendant complications (sepsis, lymphoma, direct drug toxicity). Unlike other organs, graft loss as a result of acute rejection can occur late after transplantation (more than 1 yr post-transplant). With regard to the actual immunosuppressive regimens, considerable experience in patient management is required to optimize outcome of those complex transplants, which are permanently at risk of rejection and infection. ITx remains an unfinished product, and the application of ITx to patients doing well on TPN warrants further research in the understanding of the rejection process, in the development of less toxic and more efficient immunosuppressive protocols, and in the development of immunomodulatory strategies, to better control rejection and thereby reduce the need for immunosuppression.
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PMID:Recent advances and future prospects in intestinal and multi-visceral transplantation. 1173 71

Parenteral nutrition is a life-saving therapy for patients with intestinal failure. It may be associated with transient elevations of liver enzyme concentrations, which return to normal after parenteral nutrition is discontinued. Prolonged parenteral nutrition is associated with complications affecting the hepatobiliary system, such as cholelithiasis, cholestasis, and steatosis. The most common of these is parenteral nutrition-associated cholestasis (PNAC), which may occur in children and may progress to liver failure. The pathophysiology of PNAC is poorly understood, and the etiology is multifactorial. Risk factors include prematurity, long duration of parenteral nutrition, sepsis, lack of bowel motility, and short bowel syndrome. Possible etiologies include excessive caloric administration, parenteral nutrition components, and nutritional deficiencies. Several measures can be undertaken to prevent PNAC, such as avoiding overfeeding, providing a balanced source of energy, weaning parenteral nutrition, starting enteral feeding, and avoiding sepsis.
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PMID:Parenteral nutrition-associated liver complications in children. 1183 58

Liver injury is associated with parenteral nutrition therapy. Severity of injury varies from minimal and transient increases in liver-related blood tests to biliary cirrhosis and liver failure. Severe parenteral nutrition-related liver disease is usually confined to patients who have undergone massive intestinal resection. In these patients, early sepsis appears to cause initial liver injury, and recurring sepsis and inflammation, local or systemic, may result in its perpetuation and progression. Liver disease associated with parenteral nutrition is not necessarily related either to duration of parenteral nutrition or to delayed intestinal feeding. However, treatment includes enteral nutrition to promote enterohepatic circulation of bile acids and management of inflammation and sepsis, including control of intestinal bacterial overgrowth. Restriction of intravenous lipid emulsions may be important. The clinical picture of advanced liver failure related to short bowel syndrome differs from liver failure with an anatomically normal gastrointestinal tract. In the former, hyperbilirubinemia, hepatosplenomegaly, and functional hypersplenism dominate the clinical picture, and severe ascites and esophageal variceal hemorrhage are unusual. Early referral of these patients for intestinal and/or liver transplantation may provide the best chance for long-term survival.
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PMID:Prevention of parenteral nutrition-associated liver disease in children. 1190 41

In this review the current status of home parenteral nutrition is analysed, with respect to the predictability of weaning from nutritional support and the risk of developing major complications associated with the technique, the loss of vascular access and liver disease. These two complications were evaluated because they represent the more important indication for intestinal transplantation, the availability of which has changed the perspectives of patients and of physicians. Analysis of outcomes from the largest series allows the identification of patients who could be weaned from parenteral nutrition. Important prognostic factors in patients affected by short bowel syndrome are the length and type of the remnant and the time to tolerate enteral feeding. The main complications of therapy are sepsis, thrombosis, nutrient imbalances and liver disease. Sepsis and thrombosis could lead to line replacement and the loss of vascular access. Sepsis no longer represents a major cause of death, but it is a frequent complication. In some patients, it is difficult to assess the risk factors for sepsis, which is possibly related to a poorer outcome. The care of gut failure appears to be the best preventative measure for the occurrence of cholestatic liver disease, but further studies are needed to define the eventual role of lipid emulsion and of specific nutrient deficiency. The quality of life still remains to be studied: because home parenteral nutrition in children has a longer duration, its analysis is mandatory.
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PMID:Outcome and quality of life in paediatric home parenteral nutrition. 1195 57


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