To Wean or Not to Wean: The Role of Autologous Reconstructive Surgery in the Natural History of Pediatric Short Bowel Syndrome on Behalf of Italian Society for Gastroenterology, Hepatology and Nutrition (SIGENP).

Pediatric Short Bowel Syndrome (SBS) can require prolonged parenteral nutrition (PN). Over the years, SBS management has been implemented by autologous gastrointestinal reconstructive surgery (AGIR). The primary objective of the present review was to assess the effect of AGIR on weaning off PN. We also evaluated how AGIR impacts survival, the need for transplantation (Tx) and the development of liver disease (LD). We conducted a systematic literature search to identify studies published from January 1999 to the present and 947 patients were identified. PN alone was weakly associated with higher probability of weaning from PN (OR = 1.1, p = 0.03) and of surviving (OR = 1.05, p = 0.01). Adjusting for age, the probability of weaning off PN but of not surviving remained significantly associated with PN alone (OR = 1.08, p = 0.03). Finally, adjusting for age and primary diagnosis (gastroschisis), any association was lost. The prevalence of TX and LD did not differ by groups. In conclusion, in view of the low benefit in terms of intestinal adaptation and of the not negligible rate of complications (20%), a careful selection of candidates for AGIR should be required. Bowel dilation associated with failure of advancing EN and poor growth, should be criteria to refer for AGIR.

STEP improves long-term survival for pediatric short bowel syndrome patients: A Markov decision analysis.

INTRODUCTION: Increasingly, for pediatric patients with short bowel syndrome (SBS), intestinal lengthening procedures such as serial transverse enteroplasty (STEP) are being offered with the hope of improving patients' chances for achieving enteral autonomy. However, it remains unclear to what extent STEP reduces the long-term need for intestinal transplant or improves survival. METHODS: Based on existing literature, a decision analytic Markov state transition model was created to simulate the life of 1,000 pediatric SBS patients. Two simulations were modeled: 1) No STEP: patients were listed for transplant once medical management failed and 2) STEP: patients underwent STEP therapy and subsequent transplant listing if enteral autonomy was not achieved. Sensitivity analysis of small bowel length and anatomy was completed. Base case patients were defined as neonates with a small bowel length of 30cm. RESULTS: For base case patients with an ostomy and a NEC SBS etiology, STEP was associated with increased rates of enteral autonomy after 10 years for patients with an ICV (53.9% [STEP] vs. 51.1% [No STEP]) and without an ICV (43.4% [STEP] vs. 36.3% [No STEP]). Transplantation rates were also reduced following STEP therapy for both ICV (17.5% [STEP] vs. 18.2% [No STEP]) and non-ICV patients (20.2% [STEP] vs. 22.1% [No STEP]). 10-year survival was the highest in the (+) STEP and (+) ICV group (85.4%) and lowest in the (-) STEP and (-) ICV group (83.3%). CONCLUSIONS: For SBS patients, according to our model, STEP increases rates of enteral autonomy, reduces need for intestinal transplantation, and improves long-term survival. TYPE OF STUDY: Economic/Decision Analysis or Modeling Studies LEVEL OF EVIDENCE: Level III.

Differences in methodology impact estimates of survival and dependence on home parenteral support of patients with nonmalignant short bowel syndrome.

BACKGROUND: In patients with intestinal failure (IF), who are receiving home parenteral support (HPS), variations between centers in estimates of survival and HPS dependency often reflect differences in population characteristics. However, variations in methodology and adherence to model assumptions may further contribute. OBJECTIVES: We investigated how differences in methodology affect estimates of outcomes in IF patients. METHODS: We applied different model assumptions and statistical methods to real-life outcome data from a well-characterized cohort of nonmalignant short bowel syndrome (SBS) patients. This retrospective study was based on extracts from the Copenhagen IF database and from the Danish death registry. RESULTS: Estimates of mortality varied substantially, depending on the study design and statistical method. The 5-y mortality rate obtained with the Kaplan-Meier (KM) method was estimated to be 10.1% higher if patients were only followed during HPS treatment, compared with follow-up regardless of HPS treatment. The 5-y cumulative incidence of weaning off HPS was overestimated by 4.4% when inappropriately using the KM method, instead of the cumulative incidence function. The 5-y survival rates in nonmalignant SBS-IF patients who initiated HPS were 89.1% for those aged younger than 40 y, 74.8% for patients aged 40-60 y, and 52.1% for those older than 60 y. A Cox regression analysis identified age and diagnoses other than inflammatory bowel disease as significant risk factors for mortality. For HPS dependency, bowel anatomy was significantly associated with the ability to wean off, and no patients without a colon and less than 100 cm remnant of the small bowel remained continuously weaned off and alive for one year. CONCLUSIONS: The large variations in outcomes illustrated in this study emphasize the importance of the appropriate selection of statistical methods. A comparison between studies is problematic, due to differences in the methods employed.

Outcomes of Children With Chronic Intestinal Failure: Experience Over 2 Decades at a Tertiary Paediatric Hospital.

BACKGROUND AND AIMS: The aim of the study was to aid decisions on prognosis and transplantation; this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne. METHODS: Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011. RESULTS: A total of 51 children were included. Forty-two (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome, and 4 (8%) had congenital enteropathies. Median small bowel length for patients with SBS was 45 cm (interquartile range 30-80) or 23.9% of the expected length for age (interquartile range 17.0%-40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2-29.2) with intestinal failure-associated liver disease (IFALD) being the only predictor (P = 0.001). Out of 50 children 21 (42%) had IFALD. Children who were premature (P = 0.013), had SBS (P = 0.038), and/or frequent sepsis (P = 0.014) were more likely to develop IFALD. PN weaning occurred in 27 of 35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (P = 0.025), preservation of the ileocecal valve (P = 0.013) and colon (P = 0.011) being predictors. None of 5 (0%) patients with chronic intestinal pseudo-obstruction syndrome and 2 of 4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both P < 0.001). CONCLUSIONS: Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

Long term outcomes of intestinal rehabilitation in children with neonatal very short bowel syndrome: Parenteral nutrition or intestinal transplantation.

BACKGROUND & AIMS: Intestinal rehabilitation is the preferred treatment for children with short bowel syndrome (SBS) whatever the residual bowel length, and depends on the accurate management of long-term parenteral nutrition (PN). If nutritional failure develops, intestinal transplantation (ITx) should be discussed and may be life-saving. This study aimed to evaluate survival, PN dependency and nutritional status in children with neonatal very SBS on PN or after ITx, in order to define indications and timing of both treatments. PATIENTS AND METHODS: This retrospective cross-sectional study enrolled 36 children with very SBS (<40 cm) who entered our intestinal rehabilitation program from 1987 to 2007. RESULTS: All the children on long-term PN (n = 16) survived with a follow-up of 17 years (9-20). Six of them were eventually weaned off PN. Twenty children underwent ITx: eight children died (40%) 29 months (0-127) after Tx. The others 12 patients were weaned off PN 73 days (13-330) after Tx. Follow-up after transplantation was 14 years (6-28). Seven out of 8 (88%) patients with a history of gastroschisis required ITx. Patients who required ITx had longer stoma duration. CONCLUSION: Survival rate of children with very short bowel was excellent if no life-threatening complications requiring transplantation developed. Gastroschisis and delayed ostomy closure are confirmed as risk factor for nutritional failure. Intestinal rehabilitation may allow a total weaning of PN before adulthood. A follow-up by a multidisciplinary team is necessary to avoid PN complications in order to minimize indications for ITx.

Three-Year Prospective Follow-up of Potential Pediatric Candidate for Intestinal Transplantation.

Intestinal transplantation (ITx) is a treatment for refractory intestinal failure (IF). However, the indications for and timing of ITx are still controversial because the course of IF is unknown. We performed a prospective multi-institutional cohort study to identify the prognostic factors for referral to an ITx facility. Patients under 18 years of age in Japan who suffered from IF and had received parenteral nutrition for longer than 6 months were enrolled in this study. They were followed up for 3 years. Seventy-two patients were followed. The mean age at the beginning of the study was 7.0 years. Diagnoses were short gut syndrome (n = 25), motility disorder (n = 45), and other (n = 2). The overall 3-year survival rate was 95%. The 3-year survival rate was 86% in patients with intestinal-failure-associated liver disease (IFALD) (n = 6) compared to 97% in those without IFALD (n = 66) (P = .0003). Furthermore, the 3-year survival rates of patients who did and did not meet the criteria for ITx were 82% (n = 11) and 97% (n = 62), respectively (P = .034). Six (44%) of 14 patients whose performance status (PS) was ≥3 at enrollment were dead or still had a PS ≥ 3 at 3 years. This study indicates that IFALD is a poor prognostic factor in pediatric patients with IF. Our indication for ITx, namely the presence of IFALD or loss of more than 2 parenteral nutrition access sites, seems to be applicable.

Risk factors for parenteral nutrition-dependence and mortality with the short bowel syndrome: a 10-year retrospective study in Thailand.

BACKGROUND AND OBJECTIVES: Short bowel syndrome (SBS) is a rare and life-threatening disease. Few studies have investigated risk factors for parenteral nutrition (PN)-dependence and death in SBS. Accordingly, the aim of this study was to investigate the risk factors for PN-dependence and long-term mortality in SBS. METHODS AND STUDY DESIGN: This retrospective study reviewed and evaluated children and adults who were diagnosed with SBS at King Chulalongkorn Memorial Hospital from October 2005 to January 2015. Age, causes of SBS, length of remnant bowel, type of anastomosis, types of nutrition support, SBS-associated complications, PN-dependence rate, duration of PN-dependence, mortality rate, and causes of death were evaluated. RESULTS: Twenty-two adults and 19 children were reviewed. The median follow-up time was 48 months. At the end of follow-up, PNdependence rate was 51.2%. The residual colon >=50% group had a significantly lower PN-dependence rate, with a hazard ratio of 0.36 (95% CI: 0.14-0.93; p=0.03). The most frequent cause of death was infection, with the highest percentage of mortality occurring within the first 2 years after surgery. The mortality rate was 53.8% and the residual colon >=50% group had a significantly lower mortality rate, with a hazard ratio of 0.36 (95% CI: 0.14- 0.88; p=0.03). CONCLUSIONS: PN-dependence and death occurred in about half of all patients. Residual colon >=50% was significantly associated with lower death rate and PN-dependence. The crucial role of colon in continuity as a protective factor should be investigated further in prospective studies.

Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984-2017.

BACKGROUND: Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. METHODS: All children with IF who required parenteral nutrition (PN) >2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. RESULTS: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0.4-3.5) years were identified. Causes of IF were short bowel syndrome (SBS; n = 78), primary intestinal motility disorders (n = 14), and congenital intestinopathies (n = 8). Patients with SBS had median 40 (25-60) cm of small bowel remaining. Overall, Kaplan-Meier 5- and 10-year weaning-off estimates were 67% (95% CI, 57-77) and 73% (95% CI, 63-84), respectively. Weaning off PN was predicted by remaining bowel anatomy, multidisciplinary treatment era, and absence of immune deficiency. Catheter-related bloodstream infections decreased from 1.4 to 0.6/1000 PN days (P = .0003) with systematic use of taurolidine locks. None had progressive liver disease. Thirty-one percent of patients with SBS underwent autologous intestinal reconstructive surgery. Five patients received and 2 were listed for isolated intestinal transplantation. Eight patients died, and overall 15-year survival rate estimate was 91% (95% CI, 85-98). CONCLUSIONS: Despite reassuring rates of survival and weaning off PN, long-term PN failed in 14% of patients solely because of catheter complications in the recent era. Achievement of enteral autonomy in those with the shortest remaining small bowel and functional cause of IF remains challenging.

Outcomes in patients with short bowel syndrome after autologous intestinal reconstruction: Does etiology matter?

BACKGROUND: Short bowel syndrome (SBS) is the most common cause of intestinal failure in children. Many factors have been investigated in an attempt to define which parameters influence most survival and ability to wean off parenteral nutrition (PN). The aim of this study was to investigate if aetiology of SBS affects the outcomes in paediatric patients treated with autologous gastrointestinal reconstructive surgery. METHODS: All children with SBS who underwent autologous gastrointestinal reconstructive surgery between 2002 and 2012 were retrospectively reviewed and outcome measures were recorded. RESULTS: Forty-three patients were divided into 4 groups according to aetiology (gastroschisis, volvulus, necrotizing enterocolitis (NEC), intestinal atresia). No significant differences were found among groups regarding survival and median age at surgery. The volvulus group had a lower pre-operative bowel length in comparison with gastroschisis and intestinal atresia and the lowest percentage of patients off PN (30%). Gastroschisis had the lowest rate of preserved ileocaecal valve (10%), while intestinal atresia had the highest (66%). For children who weaned off PN, intestinal atresia had also the longest time to achieve enteral autonomy (14.5months), while NEC had the shortest (3.5months), followed by gastroschisis (8.5months). None of the patients needed transplant. CONCLUSIONS: In our experience it does not appear that diagnosis is significantly related to outcome and this is consistent with the conclusions of other reviews. However, it should be noted that in our series patients with volvulus had the worse outcome in terms of weaning off PN when compared with intestinal atresia. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: II.

Pediatric Short Bowel Syndrome: Predicting Four-Year Outcome after Massive Neonatal Resection.

OBJECTIVES: The aim of this study was to ascertain predictors of survival, liver disease (LD), and enteral autonomy 48 months after resection in neonatal short bowel syndrome (SBS) patients with residual small bowel length (SBL) ≤40 cm. PATIENTS AND METHODS: Medical records of all SBS patients followed up between 1996 and 2016 were retrospectively reviewed. Survival rate, prevalence of LD, and of enteral autonomy were evaluated. RESULTS: Forty-seven patients were included, and 43 were still alive at the end of the study period, with cumulative 48-month survival of 91.5%. Twenty-one (45%) patients developed LD, all within the first 6 months. On the final follow-up visit, three (6%) patients were still jaundiced and progressed toward end-stage LD. LD prevalence was higher in patients with recurrent bloodstream infections (odds ratio [OR] 5.4, 95% confidence interval [CI] 1.5-19.3). Of the 43 surviving patients, 22 (51%) had enteral autonomy 48 months after resection. The probability of weaning off parenteral nutrition (PN) was strongly correlated with the remaining SBL. CONCLUSION: Survival of patients who have undergone neonatal massive small bowel resection has improved in recent years. Multidisciplinary strategies can improve the course of LD, but not the probability of weaning off PN, which seems to be strongly dependent on the anatomical profile of residual bowel. Therefore, the primary surgical approach should be as conservative as possible to gain even small amounts of intestinal length, which may be crucial in promoting intestinal adaptation.

Long-term outcomes of pediatric intestinal failure.

Management of pediatric intestinal failure has evolved in recent decades, with improved survival since the advent of specialized multidisciplinary intestinal failure centers. Though sepsis and intestinal failure associated liver disease still contribute to mortality, we now have growing data on the long-term outcomes for this population. While intestinal adaptation and parenteral nutrition weaning is most rapid during the first year on parenteral support, achievement of enteral autonomy is possible even after many years as energy and protein requirements decline dramatically with age. Intestinal transplant is an option for patients experiencing complications of long-term parenteral nutrition who are expected to have permanent intestinal failure, but outcomes are hindered by immunosuppression-related complications. Much of the available data comes from single center retrospective reports, with variable inclusion criteria, intestinal failure definitions, and follow-up durations; this limits the ability to analyze outcomes and identify best practices. As most children now survive long-term, the focus of management has shifted to the avoidance and management of comorbidities, support of normal growth and development, and optimization of quality of life for these medically and surgically complex patients.

Predictors of a successful outcome for infants with short bowel syndrome: a 30-year single-institution experience.

PURPOSE: Short-bowel syndrome (SBS) is associated with high morbidity and mortality. We conducted this study to establish the predictors of survival and weaning off parenteral nutrition (PN). METHODS: We reviewed the medical records of 16 SBS infants treated at our institution within a 30-year period. SBS was defined as a residual small-bowel length (RSBL) of <75 cm. Loss of the ileocecal valve (ICV), cholestasis (D-Bil >2.0 mg/dl), enterostomy, and RSBL were all evaluated. Kaplan-Meier analysis was used to analyze the predictors. RESULTS: The mean RSBL was 34.9 ± 22.9 cm. Six patients died (37.5%) and nine patients were weaned off PN (56.3%). Significant differences were observed in cholestasis (p < 0.03), enterostomy (p < 0.01), an absolute RSBL of <30 cm (p < 0.04), and a percentage of expected RSBL of <10% (p < 0.04) as survival predictors. Significant differences were also observed for cholestasis (p < 0.01), loss of the ICV (p < 0.04), an absolute RSBL of <20 cm (p < 0.01), and a percentage of expected RSBL of <10% (p < 0.03) as predictors of weaning off PN. CONCLUSION: These predictors may help us select the optimal treatments for pediatric patients with SBS.

Impact of multidisciplinary teams for management of intestinal failure in children.

PURPOSE OF REVIEW: To review the recent literature related to the impact of an intestinal rehabilitation program (IRP) on the management of intestinal failure in children. RECENT FINDINGS: As publication of a systematic review of pediatric IRPs in 2013, there have been four publications further describing the impact of IRPs in children with intestinal failure. The results continue to support an improvement in survival and enteral autonomy, and a decrease in complications related to liver dysfunction, central venous catheters, and transplantation. SUMMARY: Pediatric IRPs offer significant advantage to outcomes of children with intestinal failure. The literature is difficult to interpret because of methodological limitations. IRP collaboration is necessary to further advance the field.

Liver Failure From Ultra-Short Bowel Syndrome on the Intestinal Transplant Waiting List: A Retrospective Study.

BACKGROUND: Patients with intestinal failure (IF) are candidates for intestinal transplantation (ITx). In Japan, these patients have few opportunities to undergo cadaveric ITx because of low rates of organ donation. The donor criteria and recipient priority for ITx are still unknown. We reviewed our cases of IF to investigate which patients should be prioritized for ITx. METHODS: Patients with IF who were registered as candidates for cadaveric ITx between January 2010 and November 2015 in our institute were included in this retrospective study. Their data were gathered from their charts and analyzed. RESULTS: Five patients were included. Their primary diseases included total colon aganglionosis (n = 1), chronic idiopathic intestinal pseudo-obstruction syndrome (n = 2), superior mesenteric vein embolization (n = 1), and graft loss after ITx (n = 1). Two patients died of liver failure (LF) during the waiting period. The remaining three are now alive and waiting for transplantation. The lengths of the remaining intestine were more than 20 cm in living cases but less than 20 cm in fatal cases. In the fatal cases, they had several episodes of catheter-related blood stream infection, which caused LF and acute renal failure. CONCLUSIONS: We identified two patients with less than 20 cm residual small bowel who died after acute deterioration of liver function. Patients with ultra-short bowel could have a higher risk of LF. Therefore, they should be referred as soon as possible to a specialized hospital where ITx is a choice of treatment for IF.

Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit.

Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed. Results Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1-217) months and had undergone 3 (1-6) previous laparotomies that left 5 (0-9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4-175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14-715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil). Conclusion A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality.

Small Bowel Dilatation Predicts Prolonged Parenteral Nutrition and Decreased Survival in Pediatric Short Bowel Syndrome.

OBJECTIVE: To analyze risk factors and prognostic significance of small bowel (SB) dilatation in children with short bowel syndrome (SBS). BACKGROUND: In SBS, the remaining SB may dilate as part of intestinal adaptation. The impact of dilatation on parenteral nutrition (PN) dependence and survival has not been studied systematically. METHODS: SB diameter of SBS children (n = 61) was measured in contrast SB series (n = 169, median age 0.94, range 0.32-2.7 years) during 2002 to 2015, and expressed as millimeters (SB width) and as ratio to L5 vertebra height (SB diameter ratio). Linear regression was used to examine risk factors for dilatation. PN weaning and survival were analyzed with Cox proportional hazards regression. RESULTS: Maximal SB diameter ratio during follow-up was predicted by PN dependence and SB atresia, while maximal absolute SB width by birth weight, age, PN duration, and remaining bowel length. Weaning off PN was 14-fold more likely in patients with maximal SB diameter ratio <2.00 compared with >3.00 (P = 0.005), and 5.4-fold more likely when maximal SB width was <20 mm compared with >30 mm (P = 0.023). After adjustment for age, remaining SB length, and the presence of ileocecal valve, both estimates of maximal SB dilatation remained significant independent predictors for weaning off PN. When all measurements were included, the cumulative survival was worse if SB diameter ratio exceeded 2.00 (P = 0.002-0.042). CONCLUSIONS: SB dilatation predicts prolonged PN duration and decreased survival in SBS children. Measurement of maximal SB diameter standardized to L5 vertebra height may be a valuable objective tool for patient follow-up and assessment of prognosis.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program.

The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program / Tratamento da síndrome do intestino encurtado na criança: valor do Programa de Reabilitação Intestinal

Summary The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition. The currently recommended treatment includes the use of prolonged parenteral nutrition and enteral nutrition, always aimed at constant weight gain, in conjunction with surgeries aimed at elongating the dilated bowel. This set of procedures constitutes what is called an Intestinal Rehabilitation Program. This therapy was used in 16 children in periods ranging from 8 months to 7.5 years, with survival in 75% of the cases. Finally, the last resort to be used in children with complete resection of the small bowel is an intestinal transplant. However, to date there is no record of a Brazilian child that has survived this procedure, despite it being attempted in seven patients. We conclude that the results of the intestinal rehabilitation program are encouraging for the continuation of this type of treatment and stimulate the creation of the program in other pediatric care institutions.

Resumo A principal causa da falência intestinal aguda é a síndrome do intestino encurtado, decorrente, em geral, de ressecção de extensos segmentos de intestino delgado. Em consequência, os principais sintomas são diarreia aquosa, síndrome de má absorção, desnutrição crônica e óbito, caso o paciente não seja adequadamente tratado. Se o comprimento do intestino remanescente for superior a 30 cm, poderá haver adaptação completa e o paciente poderá ficar livre da nutrição parenteral. O tratamento atualmente preconizado inclui a utilização de nutrição parenteral prolongada e de nutrição enteral, objetivando sempre o ganho ponderal constante, em paralelo a cirurgias que visem ao alongamento do intestino dilatado. Esse conjunto de procedimentos constitui o que se denomina Programa de Reabilitação Intestinal. Essa terapia foi utilizada em 16 crianças, em períodos que variaram de 8 meses a 7 anos e meio, com sobrevida em 75% dos casos. O último recurso utilizado em crianças com ressecção completa do intestino delgado é o transplante intestinal. Até o momento, não há registro de criança brasileira que tenha sobrevivido a esse procedimento, a despeito de sete pacientes terem sido submetidos a ele. Os resultados do Programa de Reabilitação Intestinal nos anima a continuar com esse tipo de tratamento e estimular a criação do programa em outras instituições de atendimento pediátrico.

Change of Outcomes in Pediatric Intestinal Failure: Use of Time-Series Analysis to Assess the Evolution of an Intestinal Rehabilitation Program.

BACKGROUND: The clinical picture of pediatric intestinal failure has changed over the past 15 years, while effectiveness evolving treatment options remains unclear. This study explored evolution in care and quantified independent effects of new treatment options. STUDY DESIGN: Consecutive patients (n = 196) with neonatal or infantile intestinal failure, born between July 1996 and December 2011, were derived from an intestinal rehabilitation program (IRP) patient registry. Change over time was analyzed using multivariable Box-Jenkins method-based autoregressive integrative moving average models (ARIMA), robust linear regression, and nonparametric trend analysis. Four systematically introduced treatment options (IRP, serial transverse enteroplasty, omega-3 lipid emulsions, and ethanol locks) were evaluated. Analyses were adjusted for patient characteristics and disease severity. The primary outcome was disease-specific mortality from liver failure and sepsis. Secondary outcomes included parenteral nutrition weaning, transplantations, catheter complications, and liver disease. RESULTS: Patient characteristics remained unchanged over time, except for decreasing small bowel length (-0.5%/quarter; 95% CI -0.85, -0.16) and ICU admission time (-0.6 days/quarter; 95% CI -1.03, -0.18). Disease-specific mortality diminished significantly over time (-0.02 deaths/quarter; 95% CI -0.03, -0.01) by IRP and omega-3 lipids introduction (-0.6 deaths/quarter each, 95% CI -1.23, -0.02 and -0.77, -0.45, respectively). Serial transverse enteroplasty and ethanol locks had no significant impact. Parenteral nutrition weaning and transplantations remained unchanged, while catheter sepsis and complication rates decreased by 0.3 episodes/1,000 catheter-days each (95% CI -0.43, -0.2 and -0.45, -0.24, respectively). CONCLUSIONS: Introduction of IRP and omega-3 lipids independently decreased disease-specific mortality. For the first time, time series analysis was applied to evaluate effectiveness of treatment options in intestinal rehabilitation.

Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome.

Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3-135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter-related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome.