Intestinal Failure: The Long and Short of the Matter
T
he strategy for management of intestinal failure (IF) has >3 months. Patients with an underlying diagnosis of necroexpanded significantly since Wilmore and Dudrick retizing enterocolitis (NEC), care at an IF site without an associported the first infant successfully receiving total parenated transplant center, and an intact ileo-cecal valve attained teral nutrition (TPN) in 1968.1 Previously, children with enteral autonomy. When the authors analyzed only patients significant intestinal resections had little hope for long-term with intraoperative measurements of residual small bowel survival, especially in the setting of progreslength, residual small bowel length (cm) in See related articles, p 29 sive cholestasis.2,3 One of the major innovaaddition to the prior risk factors NEC and and p 35 tions in the field has been the institution of IF care at a non-intestinal transplant center comprehensive intestinal rehabilitation programs (IRPs) were significantly associated with enteral autonomy. The PIFthat offer a multi-disciplinary approach incorporating experCON cohort was derived from patients cared for during the tise in medical and surgical therapies. These programs are time period 2000-2007. Given the improvement in patient surcomposed of pediatric gastroenterologists, surgeons, regisvival, ability to be liberated from TPN, and changes reflected in tered nurses and nurse practitioners, pharmacists, and social the intestinal transplant waiting list and mortality, an analysis workers dedicated to providing cutting edge care. Infants and of a more recent cohort is likely to demonstrate even higher children are monitored meticulously for growth, electrolyte rates of enteral autonomy and transplant-free survival.12 and fluid balance, and the development of cholestasis. MediMultiple reports have demonstrated the importance of recal costs for care of these children have been estimated at sidual bowel length on outcome.3,6 The data regarding approximately $500 000 $250 000 in the first year of life.4 impact of the ileo-cecal valve on adaptation has been more variable.6,7 The impact of IF-care at a non-intestinal transOver the last 10 years, there has also been a shift in the paradigm of TPN management in attempting to minimize plant center is an intriguing variable examined in this study. cholestasis, reduce the number of central line associated I would caution readers not to assume that IRPs with transbloodstream infections (CLABSI), and increase utilization plant centers simply proceed with transplant rather than unof autologous bowel reconstruction surgery with excellent dertake a potentially lengthy intestinal rehabilitation process, success.5-7 Data from 2012 reveal that intestinal transplant especially given the potential referral bias to those centers. As the authors point out, patients cared for at centers with assopatients with 5-year survival had 10- and 15-year conditional ciated intestinal transplant centers have shorter remnant survival of 75% and 61%, respectively.8 United Network of length and are more likely to be premature with very low Organ Sharing data reveal that the number of pediatric intesbirth weight. Avitzur et al described the outcomes with retinal transplants peaked in 2007 at 111, but in 2014 there were gard to intestinal transplant in an IRP-associated transplant only 56 performed.9 This decline in the number of transcenter,5 finding that patients in the time period 2006-2009 plants is likely due to earlier referrals to IRPs with expertise in caring for children with significant liver dysfunction, mulwere less likely to be listed for intestinal transplant, more tiple episodes of sepsis, and the growing application of autollikely to be removed from the waiting list because of clinical ogous bowel reconstruction.5,10 improvement, and had a decrease in mortality prior to transplant than those cared for prior to this time period. This supIn this edition of The Journal, two articles address outcomes ports the notion that global improvements in medical and in infants with IF cared for within a large consortium and a sinsurgical management, whether occurring at transplant or gle center. First, Khan et al describe the cumulative incidence of non-transplant IRPs, can improve outcomes. achieving enteral autonomy and identified patient and instituThe second article by Raphael et al13 addresses the natural tional characteristics associated with enteral autonomy.11 This cohort was derived from the Pediatric Intestinal Failure Conhistory of growth patterns in infants with IF and potential sortium (PIFCON), 14 IRPs throughout the country with risk factors for suboptimal growth in a single center retroexpertise in managing complex patients with IF. IF was defined spective cohort of 51 infants. This study is unique in that it as congenital or acquired gastrointestinal disease during inspecifically addresses infants with early onset IF and the effect fancy with TPN dependence >60 days. Enteral autonomy on growth within the first year of the initial intestinal insult. was defined as parenteral nutrition discontinuation for The description of the cohort is likely similar to other large IRPs throughout the country, although the proportion of their patients receiving Omegaven (59%) is likely higher CLABSI Central line associated bloodstream infections than average. Omegaven is a non-Food and Drug IF Intestinal failure IRP NEC PIFCON SBBO TPN
Intestinal rehabilitation program Necrotizing enterocolitis Pediatric Intestinal Failure Consortium Small bowel bacterial overgrowth Total parenteral nutrition
The author declares no conflicts of interest. 0022-3476//$ - see front matter. Copyright ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2015.04.037
6
Vol. 167, No. 1 July 2015 Administration approved intravenous lipid fish oil, comprised of omega-3 polyunsaturated fatty acids, utilized on a compassionate use basis protocol throughout the country because of its potential ability to reverse parenteral nutrition associated liver disease.14,15 The exact mechanism by which this product works is not clear but it does have recognized anti-inflammatory and anti-oxidant properties.16 These authors describe a U-shaped curve with regards to both weight for age Z-score and length for age Z-score during the initial months after enrollment in this IRP. Despite initial normal weight for age Z-score and length for age Z-score, growth plateaued at 6 months of age and then normalized at approximately 1 year. In the multivariable regression analysis, both NEC and having $2 CLABSI were independent risk factors for poor growth. Surprisingly, residual bowel length and severity of liver disease did not affect growth variables. One might expect patients with ultra-short bowel syndrome,
T
he strategy for management of intestinal failure (IF) has >3 months. Patients with an underlying diagnosis of necroexpanded significantly since Wilmore and Dudrick retizing enterocolitis (NEC), care at an IF site without an associported the first infant successfully receiving total parenated transplant center, and an intact ileo-cecal valve attained teral nutrition (TPN) in 1968.1 Previously, children with enteral autonomy. When the authors analyzed only patients significant intestinal resections had little hope for long-term with intraoperative measurements of residual small bowel survival, especially in the setting of progreslength, residual small bowel length (cm) in See related articles, p 29 sive cholestasis.2,3 One of the major innovaaddition to the prior risk factors NEC and and p 35 tions in the field has been the institution of IF care at a non-intestinal transplant center comprehensive intestinal rehabilitation programs (IRPs) were significantly associated with enteral autonomy. The PIFthat offer a multi-disciplinary approach incorporating experCON cohort was derived from patients cared for during the tise in medical and surgical therapies. These programs are time period 2000-2007. Given the improvement in patient surcomposed of pediatric gastroenterologists, surgeons, regisvival, ability to be liberated from TPN, and changes reflected in tered nurses and nurse practitioners, pharmacists, and social the intestinal transplant waiting list and mortality, an analysis workers dedicated to providing cutting edge care. Infants and of a more recent cohort is likely to demonstrate even higher children are monitored meticulously for growth, electrolyte rates of enteral autonomy and transplant-free survival.12 and fluid balance, and the development of cholestasis. MediMultiple reports have demonstrated the importance of recal costs for care of these children have been estimated at sidual bowel length on outcome.3,6 The data regarding approximately $500 000 $250 000 in the first year of life.4 impact of the ileo-cecal valve on adaptation has been more variable.6,7 The impact of IF-care at a non-intestinal transOver the last 10 years, there has also been a shift in the paradigm of TPN management in attempting to minimize plant center is an intriguing variable examined in this study. cholestasis, reduce the number of central line associated I would caution readers not to assume that IRPs with transbloodstream infections (CLABSI), and increase utilization plant centers simply proceed with transplant rather than unof autologous bowel reconstruction surgery with excellent dertake a potentially lengthy intestinal rehabilitation process, success.5-7 Data from 2012 reveal that intestinal transplant especially given the potential referral bias to those centers. As the authors point out, patients cared for at centers with assopatients with 5-year survival had 10- and 15-year conditional ciated intestinal transplant centers have shorter remnant survival of 75% and 61%, respectively.8 United Network of length and are more likely to be premature with very low Organ Sharing data reveal that the number of pediatric intesbirth weight. Avitzur et al described the outcomes with retinal transplants peaked in 2007 at 111, but in 2014 there were gard to intestinal transplant in an IRP-associated transplant only 56 performed.9 This decline in the number of transcenter,5 finding that patients in the time period 2006-2009 plants is likely due to earlier referrals to IRPs with expertise in caring for children with significant liver dysfunction, mulwere less likely to be listed for intestinal transplant, more tiple episodes of sepsis, and the growing application of autollikely to be removed from the waiting list because of clinical ogous bowel reconstruction.5,10 improvement, and had a decrease in mortality prior to transplant than those cared for prior to this time period. This supIn this edition of The Journal, two articles address outcomes ports the notion that global improvements in medical and in infants with IF cared for within a large consortium and a sinsurgical management, whether occurring at transplant or gle center. First, Khan et al describe the cumulative incidence of non-transplant IRPs, can improve outcomes. achieving enteral autonomy and identified patient and instituThe second article by Raphael et al13 addresses the natural tional characteristics associated with enteral autonomy.11 This cohort was derived from the Pediatric Intestinal Failure Conhistory of growth patterns in infants with IF and potential sortium (PIFCON), 14 IRPs throughout the country with risk factors for suboptimal growth in a single center retroexpertise in managing complex patients with IF. IF was defined spective cohort of 51 infants. This study is unique in that it as congenital or acquired gastrointestinal disease during inspecifically addresses infants with early onset IF and the effect fancy with TPN dependence >60 days. Enteral autonomy on growth within the first year of the initial intestinal insult. was defined as parenteral nutrition discontinuation for The description of the cohort is likely similar to other large IRPs throughout the country, although the proportion of their patients receiving Omegaven (59%) is likely higher CLABSI Central line associated bloodstream infections than average. Omegaven is a non-Food and Drug IF Intestinal failure IRP NEC PIFCON SBBO TPN
Intestinal rehabilitation program Necrotizing enterocolitis Pediatric Intestinal Failure Consortium Small bowel bacterial overgrowth Total parenteral nutrition
The author declares no conflicts of interest. 0022-3476//$ - see front matter. Copyright ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2015.04.037
6
Vol. 167, No. 1 July 2015 Administration approved intravenous lipid fish oil, comprised of omega-3 polyunsaturated fatty acids, utilized on a compassionate use basis protocol throughout the country because of its potential ability to reverse parenteral nutrition associated liver disease.14,15 The exact mechanism by which this product works is not clear but it does have recognized anti-inflammatory and anti-oxidant properties.16 These authors describe a U-shaped curve with regards to both weight for age Z-score and length for age Z-score during the initial months after enrollment in this IRP. Despite initial normal weight for age Z-score and length for age Z-score, growth plateaued at 6 months of age and then normalized at approximately 1 year. In the multivariable regression analysis, both NEC and having $2 CLABSI were independent risk factors for poor growth. Surprisingly, residual bowel length and severity of liver disease did not affect growth variables. One might expect patients with ultra-short bowel syndrome,
