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.

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.

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 / 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.

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.

Refined Multidisciplinary Protocol-Based Approach to Short Bowel Syndrome Improves Outcomes.

OBJECTIVE: Management of short bowel syndrome (SBS) has significantly evolved recently. We present our single-center, 25-year experience focusing on the implementation of a refined multidisciplinary SBS care protocol. METHODS: This is a retrospective review of the patients with SBS treated at our tertiary center from 1988 to 2014, with either <25% short bowel remaining or duration of parenteral nutrition (PN) >3 months. Patients with primary intestinal motility disorders were excluded. Clinical characteristics, including intestinal anatomy, markers of cholestasis, and catheter-related infections (CRIs), were analyzed. The implementation of a refined modern uniform management protocol in 2009 divided the cohort into 2 subgroups, whose outcomes are compared. RESULTS: Forty-eight patients with SBS were identified (median gestational age 33 weeks). Of them, 22 were born between 2009 and 2014. The main causes of SBS were necrotizing enterocolitis (46%) and midgut volvulus (23%). Median remaining small bowel length was 36 cm. The overall survival was 23 of 26 (88%) before 2009 and 21 of 22 (95%) thereafter, whereas none were transplanted. Duration of PN shortened from a median of 15 to 6 months (P = 0.0015) in the latter cohort, whereas frequency of autologous intestinal reconstruction surgery (31%) remained unchanged. Frequency of neonatal cholestasis was similar in both groups (75%), but cleared in all after 2009. Before 2009, 2 patients died of progressive cholestatic liver failure. The CRI rates decreased from 1.7 to 0.7 per 1000 catheter-days between 2000-2008 and 2009-2014, respectively (P = 0.0178). CONCLUSIONS: Uniform refined multidisciplinary approach decreased the duration of PN and CRI rates with high transplant-free survival and avoidance of liver failure, although the frequency of transient neonatal cholestasis remained unchanged.

Enteral autonomy in pediatric short bowel syndrome: predictive factors one year after diagnosis.

PURPOSE: This study examined predictors of achieving enteral autonomy among pediatric short bowel syndrome (SBS) patients remaining on parenteral nutrition (PN) beyond one year. METHODS: A retrospective single-institution study of 171 pediatric SBS patients (defined as ≥50% small bowel (SB) loss or ≥60 days of PN with onset before 6 weeks of age) was performed. Multivariate Cox proportional hazards analysis was conducted, with subgroup analysis of patients on PN for ≥1 year (n=59). Primary outcome was successful wean from PN. RESULTS: Over a follow-up of 4.1±4.8 years, 64.3% of children weaned from PN. Mortality was 15.2%. Presence of ≥10% expected SB length (hazard ratio [HR] 6.48, p=0.002) or an ileocecal valve (ICV; HR, 2.86, p<0.001) predicted PN weaning. Of those on PN ≥1 year, the wean rate was 50.8%, and ICV no longer predicted weaning (p=0.153). Predictors among those on PN ≥1 year were: ≥10% expected SB length (HR, 8.27, p=0.010), intestinal atresia (HR, 4.26, p=0.011), and necrotizing enterocolitis (NEC, HR, 2.84, p=0.025). CONCLUSIONS: SBS children on PN ≥1 year continue to wean from PN, and those with ≥10% of predicted SB length, NEC, or atresia are more likely to do so. These findings may help direct management and advice for these challenging patients.

Salvage surgery due to bowel obstruction in advanced or relapsed ovarian cancer resulting in short bowel syndrome and long-life total parenteral nutrition: surgical and clinical outcome.

OBJECTIVE: Salvage surgery for patients with highly advanced or relapsed epithelial ovarian cancer (EOC) complicated by bowel obstruction and resulting in short bowel syndrome (SBS) constitutes a therapeutic dilemma. Our aim was to evaluate surgical and clinical outcome in these highly palliative situations. METHODS: We evaluated all patients with EOC who underwent salvage extraperitoneal en bloc intestinal resection with terminal ileostomy or jejunostomy resulting in SBS and total parenteral nutrition owing to bowel obstruction between May 2003 and January 2012 in our institution. RESULTS: Thirty-seven patients were identified (median age, 58 years; range, 22-71 years), 3 (8.1%) with primary and 34 (91.6%) with relapsed EOC. Five patients (13.5%) were platinum sensitive. Median residual intestinal length was 70 cm (range, 10-180 cm); 21 patients (56.8%) had a residual intestinal length less than 1 m. Operative 30-day mortality and major morbidity rates were 10% and 51%, respectively. Median overall survival was 5.6 months (range, 0.1-49 months). One-year and 2-year overall survival rates were 18.3% (95% confidence interval, 5.1%-31.5%) and 8.1% (95% confidence interval, 0%-18.0)%, respectively. Within a median follow-up period of 5 months (range, 0.2-49 months), 4 patients (10.8%) are still alive. No significant differences in survival were seen between patients with or without major complications, tumor residuals, or residual intestinal length of less than 1 m versus greater than 1 m. CONCLUSIONS: Salvage palliative surgery in EOC due to bowel obstruction resulting in SBS and in need of long-life total parenteral nutrition is associated with high morbidity rates and low overall survival. These surgeries should ideally be performed only in a multidisciplinary setting with adequate infrastructure and possibility of home care support. Conservative management should be the route of action in the absence of acute abdomen or intestinal perforation.

Intestinal bowel lengthening in children with short bowel syndrome: systematic review of the Bianchi and STEP procedures.

BACKGROUND: It has been thirty years since Bianchi introduced the technique that made intestinal lengthening possible. The last three decades have seen lengthening procedures established as vital components of intestinal rehabilitation programs. The goal of the present study was to use a systematic literature review to determine patient outcomes for the two most commonly used lengthening procedures, the Bianchi procedure and the serial transverse enteroplasty procedure (STEP). METHODS: Pubmed and Embase were searched using the terms "intestinal lengthening" and "bowel lengthening." Patient outcomes were extracted from each relevant journal article on the basis of a set proforma. The results were combined to create overall mean outcomes. Mean outcomes were also calculated separately for the Bianchi procedure and STEP. Significance was tested with the independent t-test. RESULTS: Overall survival for the last thirty years is 83%. However, survival for the last fifteen years has been 89%, with no significant difference between the two procedures. The Bianchi procedure has a higher rate of weaning patients who were static on parenteral nutrition with conservative measures: 55% versus 48%. In addition, the Bianchi procedure was associated with a higher rate of patients receiving transplants: 10% versus 6%. The STEP has a higher rate of complication. Length of follow-up is significantly longer for the Bianchi procedure: 76 versus 22 months. The impact that this differential could have had on our results must be considered. CONCLUSIONS: Outcomes for intestinal lengthening procedures are very good, and increasingly so. However, further analysis is required in order to fully understand the relative strengths and weaknesses of each procedure.

Long-term outcome after extensive intestinal resection for chronic radiation enteritis.

INTRODUCTION: Management of chronic radiation enteritis is often controversial, particularly due to the risk of short bowel syndrome. METHODS: One hundred and seven chronic radiation enteritis patients with short bowel syndrome were studied retrospectively between 1980 and 2009. Survival and home parenteral nutrition dependence rates were evaluated with univariate and multivariate analysis. RESULTS: The survival probabilities were 93%, 67% and 44.5% at 1, 5 and 10 years, respectively. On multivariate analysis, survival was significantly decreased with residual neoplastic disease (HR=0.21 [0.11-0.38], p<0.001), an American Society of Anesthesiologists score >3 (HR=0.38 [0.20-0.73], p=0.004) and an age of chronic radiation enteritis diagnosis >60 years (HR=0.45 [0.22-0.89], p=0.02). The actuarial home parenteral nutrition dependence probabilities were 66%, 55% and 43% at 1, 2 and 3 years, respectively. On multivariate analysis, this dependence was significantly decreased when there was a residual small bowel length >100 cm (HR=0.35 [0.18-0.68], p=0.002), adaptive hyperphagia (HR=0.39 [0.17-0.87], p=0.02) and the absence of a definitive stoma (HR=0.48 [0.27-0.84], p=0.01). CONCLUSION: The survival of patients with diffuse chronic radiation enteritis after extensive intestinal resection was good and was mainly influenced by underlying comorbidities. Almost two-thirds of patients were able to be weaned off home parenteral nutrition.

Determinants of home parenteral nutrition dependence and survival of 268 patients with non-malignant short bowel syndrome.

BACKGROUND & AIMS: Short bowel syndrome (SBS) is a rare and severe condition where home parenteral nutrition (HPN) dependence can be either permanent or transient. The timing of HPN discontinuation and the survival, according to SBS characteristics, need to be further reported to help plan pre-emptive intestinal transplantation and reconstructive surgery. METHODS: 268 Non-malignant SBS patients have been followed in our institution since 1980. HPN dependence and survival rate were studied with univariate and multivariate analysis. RESULTS: Median follow-up was 4.4 (0.3-24) years. Actuarial HPN dependence probabilities were 74%, 64% and 48% at 1, 2 and 5 years, respectively. In multivariate analysis, HPN dependence was significantly decreased with an early (<6 mo) plasma citrulline concentration >20 µmol/l, a remaining colon >57% (4/7) and a remnant small bowel length >75 cm. Among the 124 patients who became HPN independent, 26.5% did so more than 2 years after SBS constitution. CONCLUSIONS: This study indicates that long-term HPN is required in 47% of SBS patients started on this therapy. HPN independence is significantly associated with the remnant small bowel length, remaining colon and early plasma citrulline concentration. Noteworthy, HPN dependence could be reversed until 5 years after SBS constitution.

Intestinal transplantation: indications and prospects.

Intestinal transplantation (IT) can involve small bowel transplantation alone, or be associated with liver or multivisceral transplantation. Although IT is the radical treatment for intestinal failure, home parenteral nutrition (PN) remains the treatment of choice for this disease. Indications for IT are still debated. A recent study showed that early referral for IT is recommended for patients with life-threatening combined liver and intestinal failure or for patients with invasive intra-abdominal desmoid tumors. In the same study, no survival benefit was shown for patients undergoing IT for ultra-short bowel or major complications related to the PN catheter; indications still need to be fully assessed. While short-term outcomes for IT have improved dramatically (one-year survival for small bowel-alone IT is now 80% versus 0-28% in the 1980s), long-term outcomes have not improved much since the introduction of Tacrolimus in the 1990s: five-year survival still does not exceed 60%. Some prospective developments could improve these results: the use of multivisceral grafts, the use of Sirolimus and Thymoglobulins in the immunosuppressive treatment, or the use of new biochemical markers for early diagnosis of graft rejection.