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Intestinal Failure (IF) includes the loss of functional intestinal mass and the requirement of long term Parenteral Nutrition (PN) to achieve the development and growth in childhood., Objective: To evaluate the experience in a specialized unit for pediatric patients with IF, describing the clinical cha racteristics of those admitted from November 2009 to December 2019., Patients and Method: Retros pective and descriptive review from clinical records of 24 cases that matched the inclusion criteria. The following variables were recorded: gender, neonatal history, origin unit, patient age and anthropome tric diagnosis at admission to the unit, cause of IF, hospital stay, anthropometric data and parenteral nutrition dependency at discharge. In those patients with a diagnosis of short bowel syndrome (SBS), the cause of intestinal resection and the characteristics of the intestinal remnant were identified: ana tomical classification, remnant length (defining ultra-short as < 25 cm), presence of ileocecal valve, and characteristics of the colon., Results: The median age at admission was 7.8 months. Seventeen cases were preterm. Regarding IF etiology, 10 patients presented SBS, 6 patients with Intestinal Neuromus cular Disease (INMD), 7 children with SBS associated with INMD, and 1 case of intestinal lymphan giectasia. Within the SBS etiologies found in this group, intestinal atresia (8 cases) and necrotizing enterocolitis (9 patients) were the main causes with a similar proportion. Eight patients had no ileo cecal valve. According to anatomical classification, 1 case was Type I, 8 were Type II, and 8 were Type III. Related to bowel length, 3 were ultrashort, besides being Type II; in those with > 40 cm of bowel length, 7 were Type III. Overall average hospital stay was 456.4 days. Enteral autonomy was achieved in 16 patients and 8 cases required home parenteral nutrition., Conclusions: IF requires life support, PN and prolonged hospital stay. The principal etiology of IF is SBS caused by congenital intestinal atresia and necrotizing enterocolitis. Nevertheless, the high frequency of INMD could be attributed to the local protocol analysis. Most of our patients had poor prognosis factors, however, the management by a specialized team allowed the achievement of enteral autonomy in 66.7% of cases.

Background: In patients suffering intestinal failure due to short bowel, the goal of an Intestinal Rehabilitation Program is to optimize and tailor all aspects of clinical management, and eventually, wean patients off lifelong parenteral nutrition., Aim: To report the results of our program in patients suffering intestinal failure., Patients and Methods: A registry of all patients referred to the Intestinal Failure unit between January 2009 and December 2015 was constructed. Initial work up included prior intestinal surgery, blood tests, endoscopic and imaging studies. Also demographic data, medical and surgical management as well as clinical follow-up, were registered., Results: Data from 14 consecutive patients aged 26 to 84 years (13 women) was reviewed. Mean length of remnant small bowel was 100 cm and they were on parenteral nutrition for a median of eight months. Seven of 14 patients had short bowel secondary to mesenteric vascular events (embolism/thrombosis). Medical management and autologous reconstruction of the bowel included jejuno-colic anastomosis in six, enterorraphies in three, entero-rectal anastomosis in two, lengthening procedures in two, ileo-colic anastomosis in one and reversal Roux-Y gastric bypass in one. Thirteen of 14 patients were weaned off parenteral nutrition., Conclusions: Our Multidisciplinary Intestinal Rehabilitation Program, allowed weaning most of the studied patients off parenteral nutrition.

Short bowel syndrome (SBS) is characterized by a significant reduction in the effective intestinal surface by an anatomical or functional loss of the small intestine. It mainly occurs after extensive bowel resection, intestinal intrinsic disease or surgical bypass. The main complications are malabsorption, maldigestion, malnutrition, dehydratation and, potentially, lethal metabolic lesions. The treatment is based on appropiate, individualized nutritional support; however, the most recent outcomes on bowel transplantation (BT) and a great rate of survivors achieving complete digestive autonomy and able to carry out activities according to their age allow for considering BT as the first choice therapy in patients with irreversible intestinal failure in whom poor prognosis with parenteral nutrition is foreseen. In this paper the most outstanding aspects of SBS are revised., (Copyright AULA MEDICA EDICIONES 2014. Published by AULA MEDICA. All rights reserved.)

Introduction: The intensive care of patients at home had probed important beneficialness for the patient and the Health System. There are very few experiences of this kind of care from the Public Hospitals., Objective: To develop a social-sanitary analysis of the feasibility of the implementation of HPN on patients with short bowel syndrome (SBS) from a Public Hospital., Material and Methods: Patients hospitalized between 1985-2009 were included. We analyzed: age, residual intestine length (RIL), time between de indication and the beginning of HPN, HPN duration, treatment modality and clinical outcome. Social determinants: home place, habitat conditions, employment conditions, educational level, social security and Low Socioeconomic Status (LSS). The group were divided in two: 1- patients with feasibility of HPN when it was prescribed; 2- patients without feasibility of HPN., Results: 61 patients were included, RIL x: 21.7 ± 11.6 cm. The HPN was feasible (G1) in 32 patients (52.4%) and no feasible (G2) in 29 (47.6%). The home treatment modality was in self-caring 25 (81%) and with nurses support 7 (19 %). The social determinants associated with the HPN feasibility were: more than one takecare (p 0.03), educational level (p 0.01), adequate habitat conditions (p 0.02) and Low Socioeconomis Status (LSS) (p 0.07). 17 patients reached intestinal adaptation (28%), 6 (10%) were transplanted, 19 (31%) died and 19 (31%) are actually on HPN., Conclusion: The HPN realized from the Public Hospital is feasible. Different social determinants were observed. The care of this group of patients must be done by an interdisciplinary group including general aspects of the child and the family.

Among patients with short bowel syndrome, surgical small intestine lengthening techniques are employed to increase the absorptive surface. Among these, serial transverse enteroplasty involves transecting the bowel transversally, preserving the blood supply of the small intestine and creating a longer segment of bowel. We report a 51-year-old woman with a short bowel syndrome and multiple hospital admissions for complications. She was subjected to a serial transverse enteroplasty, increasing small intestinal length from 140 to 180 cm. During the postoperative period, she presented intra abdominal blood collections and a septic episode with bacterial endocarditis. One month after the operation, total parenteral nutrition was discontinued and nutritional and fluid balances were achieved using exclusively the oral route. During the ambulatory follow up, the patient continues with exclusive oral feeding and five bowel movements per day.

Intestinal failure remains a cause of morbidity and mortality in children and adults. Currently intestinal failure patients must be evaluated by a multidisciplinary team in order to optimize parenteral support, to determine the chances for rehabilitation or the need for transplantation. Intestinal transplant has been the last abdominal organ to evolve. Indications and procedures have been refined and standardized; new immunosuppressive drugs and the experience of treating these patients in a limited number of centres have reduced complications and improved survival. Forty years after the first attempt, intestinal transplant can now be successfully performed in Latin-America. The aims of this review are to update the current status of intestinal transplant, to highlight new developments and the evolution in our continent and to provide the basic knowledge in the management of intestinal failure patients for nutritionists, gastroenterologists and surgeons.

Small bowel transplantation is associated with a patient survival at one and five years, of 80% and 63%, respectively. We report a 36 year-old female with short bowel syndrome, subjected to the first small bowel transplantation performed in Chile. A cadaveric gran was used. Immunosuppression was achieved by means of alemtuzumab, tacrolimus, sirolimus, micofenolate mofetil and steroids. Serial endoscopies and biopsies were performed during seven months after transplantation. The most important late complications were a drug induced renal failure, infections caused by opportunistic agents and a gastrointestinal bleeding probably induced by drugs. After 29 months of follow up, the patient is ambulatory, on oral diet only and with no evidence of graft rejection.

Introduction: Intestinal transplantation is the only long-range treatment option for patients with intestinal failure. We report an exceptional case of isolated intestinal transplantation with the implant in a non-anatomical position., Clinical Case: The patient was a thirty-eight-year-old man (60 kg weight, 180 cm height, 18.3 body mass index) with intestinal failure and home parenteral nutrition due to a short-bowel syndrome for which intestinal transplantation was indicated. The patient had a vascular malformation with the cava vein located left to the aorta, and the intestine was implanted with a 180 masculine rotation around the mesenteric axis, so that the implant s superior mesenteric artery and vein matched the recipient s cava and aorta. Postoperative follow-up was excellent and the patient was discharged after six weeks with a 10-kg gain in body weight., Discussion: This non-anatomical intestinal implantation of the small bowel, previously unreported, offers technical advantages over other options. Adequate intestinal function represents a unique model to prove the viability of intestinal implants in a non-anatomical position.

Background: Some surgical pathologies eventually require intestinal resection. This may lead to an extended procedure such as leaving 30 cm of proximal jejunum and left and sigmoid colon. One of the most important consequences of this type of resection is "intestinal failure" or short bowel syndrome. This complex syndrome leads to different metabolic and water and acid/base imbalances, as well as nutritional and immunological challenges along with the problem accompanying an abdomen subjected to many surgical procedures and high mortality. Many surgical techniques have been developed to improve quality of life of patients., Methods: We designed a non-transplant surgical approach and performed the procedure on two patients with postoperative short bowel syndrome with <40 cm of proximal jejunum and left colon., Results: There are a variety of non-transplant surgical procedures that, due to their complex technique or high mortality rate, have not resolved this important problem. However, the technique we present in this work can be performed by a large number of surgeons. The procedure has a low morbimortality rate and offers the opportunity for better control of metabolic and acid/base balance, intestinal transit and proper nutrition., Conclusions: We consider that this technique offers a new alternative for the complex management required by patients with short bowel syndrome and facilitates their long-term nutritional control.

Management of short bowel syndrome has to be undertaken by a multidisciplinary team including gastroenterology, surgery, and nutrition, among other specialties. The final therapeutic goal is favoring intestinal adaptation by the bowel so that the patient may be weaned from parenteral nutrition. Anatomical factors influencing on adaptation are very important for deciding the best surgical option. The type of procedure will defined by age, length and functionality of the remnant bowel, the existence of intestinal dilation, the presence of bacterial overgrowth, and the presence or absence of parenteral nutrition-related complications, among others. Early closure of the stomas, if present, and correction of stenoses, if possible with stricturoplasties and with remodeling or intestinal plicature if needed, are among the basic principles of surgical management. Interventions aimed at decreasing the transit time such as the creation of valves, interposition of anti-peristaltic segments or large bowel are poorly accepted. The most accepted techniques are those elongating the bowel that may be applied only on dilated bowels. Although experience is greater with Bianchi's technique, short-term outcomes of serial transversal enteroplasty (STEP) are promising. STEP is simpler and may be applied even to previously elongated bowels by the Bianchi's technique.

Background: Children cause of intestinal failure is short-bowel syndrome. It provokes an altered absorption of nutrients and makes patients to be dependent on parenteral nutrition (PN) while they wait or not for an intestinal transplantation, with its side effects. It is crucial to achieve the maximum efficiency of remaining intestine. Many surgical techniques have been led to reduce stasis of dilated small intestine and improve the mucosal surface area for absorption., Methods: Six patients have presented intestinal failure because of a surgical resection during newborn period. 2 gastroschisis, 2 intestinal atresias (Apple-peel), 1 necrotizing enterocolitis (NEC) and 1 midgut volvulus. 4 preserve ileocecal valve (ICV): 2 Apple-peel, 1 NEC and the midgut volvulus. The shortest length of bowel after resection were 12cm without ICV (gastroschisis) and 18cm with ICV in a preterm newborn of 24 weeks of gestational age (midgut volvulus). Tapering and plication have been done in Apple-peel cases. No complementary surgical techniques have been necessary in NEC and volvulus. Gastroschisis cases had 12 and 40 cm of small bowel without ICV. In the first one, during newborn period an intestinal lengthening according to Bianchi was done, followed by sequential transversal enteroplasty (STEP), partial gastrectomy and plication of 1st and 2nd duodenal portion. In the second, an STEP was done. All patients have received cycled and optimized PN (COPN) in our centre, only one case (gastroschisis) proceeding from another centre had received standard PN and developed a great hepatic affectation., Results: 2 Apple-peel have been adapted to normal enteral nutrition after 3 and 18 months of COPN, such as NEC and volvulus (3-6 months). One gastroschisis (12cm) has a normal hepatic function with free oral nutrition and home COPN at 23 months. The other one (40cm) has COPN and started enteral nutrition 1 month after surgery, although its hepatic function remains altered., Conclusions: Parenteral nutrition is essential for these patients to survive. We would like to enhance the importance of COPN in order to preserve hepatic function. Surgical procedures aim to avoid stasis and bacterial overgrowth and improve intestinal motility. Different techniques may be used alone or sequentially. The purpose of this management is to achieve nutritional autonomy or increase waiting time before intestinal transplantation.

Objective: To compare two intestinal lengthening procedures in an experimental dog model., Background: Intestinal lengthening is one of the methods for gastrointestinal reconstruction used for treatment of short bowel syndrome. The modification to the Bianchi's technique is an alternative. The modified technique decreases the number of anastomoses to a single one, thus reducing the risk of leaks and strictures. To our knowledge there is not any clinical or experimental report that studied both techniques, so we realized the present report., Methods: Twelve creole dogs were operated with the Bianchi technique for intestinal lengthening (group A) and other 12 creole dogs from the same race and weight were operated by the modified technique (Group B). Both groups were compared in relation to operating time, difficulties in technique, cost, intestinal lengthening and anastomoses diameter., Results: There were no statistical difference in the anastomoses diameter (A = 9.0 mm vs. B = 8.5 mm, p = 0.3846). Operating time (142 min vs. 63 min) cost and technique difficulties were lower in group B (p < 0.0001). Intestinal lengthening was greater in group B (p = 0.0006). At the end of surgery as well as four hours later, all except one of the anastomoses (of Group B) and intestinal segments had good blood supply and were patent along their full length., Conclusion: Bianchi technique and the modified technique offer two good reliable alternatives for the treatment of short bowel syndrome. The modified technique improved operating time, cost and technical issues.

Background: More than two thirds of all intestinal transplantations (ITx) performed around the world correspondent to recipients younger than 18., Objective: To analyze our 5-year experience in pediatric ITx., Patients: We assessed the outcome of the 19 children included in list out of 41 patients considered for ITx from 1997. The main cause of intestinal failure was short bowel syndrome (14) followed by intestinal motility disorders (3) and congenit disorders of intestinal epithelium (CDIE) (2). The median of age, at the moment of including in the list, was 0.9 years (range 0.4-17) and median of weight was 6.4kg (range 0.4-29.3). Ten children were included for liver and small bowel transplantation (LSBTx), 7 to isolated small bowel (SBTx), and 2 for multivisceral transplantation (MVTx). Indications for SBx were hepatic fibrosis/cirrhosis (10), hepatic fibrosis in evolution (5) (to avoid later LSBTx), intractable diarrhoea (1), recurrent line infections (1), lost of central vein access (1), and bad quality of life in one., Results: Five children died in the waiting list, after a median time of 325 days (range 19-581). Seven remain in the waiting list (median 139 days, range 30-778). In 3 of these the indication changed from SBTx to LSBTx because of progression to end stage liver disease. Six children recieved seven grafts (1 MVTx, 4 LSBTx, 2 SBx) after a median time in the waiting list of 352 days (range 66-732). Six out of seven grafts achieved normal function and all survivals reached full digestive autonomy after Tx. We had to rejection episodes, one with good response to medical treatment and one that required removal of intestinal allograft and later LSBTx. Two children died 1 because of problem not related to the procedure (hemorrage following liver biopsy) and one girl died 29 months after transplant due to post-transplantation lymphoproliferative disease., Conclusions: ITx is a realistic alternative in our country for children with intestinal failure. The main problems are immunologic (rejection, lynphoproliferative and disease) Shortage of small weight donors is a dramatic limitation that prompts the discussion of surgical alternatives.

The objective of this study was to compare the clinical and analytical repercussion of a new type of intestinal valve -which can be used in both massive resections and right hemicolectomies requiring the elimination of the ileocecal valve- on two historical series of patients: one group with ileocolic resections and end-to-end anastomoses (EE), and one group with Ricotta valves. We compared 23 patients with ileocolic resection and end-to-end anastomosis, 15 with Ricotta's valve, and 20 patients with a new valve made with a small intestinal invagination. There were no statistically significant differences in baseline characteristics among patients. Patients with the new valve showed less relevant weight loss and fewer stools at 3 months and one year when compared to patients with Ricotta's valve, and particularly those with ileocolic resection. Moreover, with the new valve technique there was neither colonization of the ileal mucosa nor bacterial overgrowth. In conclusion, in massive intestinal resections and right hemicolectomies, including the ileocecal valve, the new valve technique would be of choice.

Objective: To evaluate the functional response, morbidity and histostructural changes in rats enterectomized and without cecum using two types of syngenic enteral transplants., Material and Methods: Controlled randomized surgical-therapeutic trial. Four groups of male Lewis rats 8-10 weeks old underwent the following procedures: 1. Lethal enteral resection (n = 10). 2. Lethal enteral resection + total yeyuno-ileal transplant (n = 28). 3. Lethal enteral resection + distal segmentary of 40% and cecum transplant (n = 32). 4. Control group (n = 10)., Results: 11% of the transplanted animals died due to technical failures; both transplanted groups had a similar proportion of late complications, mostly enteral obstruction. A persistent diarrhea was observed in 20% of the yeyuno-ileal transplanted group, but no significant differences were found between the two groups concerning survival, weight gain, protein and triglycerides serum levels, and a maltose absorption test; villus and crypt hypertrophy was observed in both grafts. The enteral graft integration was followed by structural changes similar to those found in intestinal remnants on deficit conditions after enteral resection., Conclusion: The bowel distal segmentary transplant with ileocecal valve and cecum may be a good option in cases of irreversible enteral failure, as the functional response and morbidity are similar to those found with the standard total transplant.

An isolated bowell segment (IBS) was created in rats by a two-stage procedure. Initial lateral entero-enteropexy between small and colonic bowell was performed. Six weeks later, the small bowell mesentery was divided. After four weeks, the survival animals were killed, the viability of the segment is maintained by developing vascular collateral across the coaptation between both intestinal segments, under light microscopy the willi were atrophied on 50% of animals.

Morbidity and mortality are especially increased in pediatric patients affected of short bowel syndrome in whom ileocecal valve was resected. This report concerns 13 infants and children with short gut syndrome with ileocecal valve resected treated in the last 4 years. In two of them we performed intestinal duplication and in seven an antireflux intestinal valve with a segment of bowel. With that procedure we achieved a significant shortening of the hospital stay as well as reduction in time and quantity of total parenteral nutrition. The rest of the patients were treated with conservative management.

An exceptional case of a 16-year-old boy who accidentally lost all of the small bowel (except the proximal 5 cm. of jejunum), and also 10 cm of transverse colon and a small part of the middle and upper rectum, is presented. After suffering severe medical complications derived from prolonged and uninterrupted total parenteral nutrition for more than one year, with no oral intake except fluids, the patient underwent intestinal lengthening of all the residual bowel up to the sigmoid colon, with antiperistaltic anastomosis and sigmoid J pouch with myotomy. Several months after the operation the patient returned to his normal activities and is fed with a free-diet, complemented or not with nocturnal enteral and parenteral feeding, depending on the circumstances and weight variations. Lengthening of the large bowel together with the rest of surgical gestures here performed has not been published previously.

The creation of intestinal neomucosa on grafts or materials implanted in the intestinal wall is one of the therapeutic procedures that has been tested in the short bowel syndrome. This paper presents the results obtained after intestinal implanting lyophilised dural patches in an experimental model of massive intestinal resection in rats. Data were contrasted with those of a control group subjected to massive resection without patches. The findings reveal: 1) high mortality related to the surgical technique; 2) slight improvement in the ponderal curve; 3) slowing down of intestinal transit; 4) neomucosa on the patch, with histological characteristic similar to normal. We conclude that although the creation of neomucosa on dural patches is feasible and conditions a slight improvement in the animal's nutritive status, application of the procedure is not practical in treatment for the short bowel syndrome due to the high mortality rate.

Intestinal pouches are one of the techniques for the surgical treatment of the short bowel syndrome. In a experimental study with rats, the influence of the intestinal pouches in a model of 85-90% small bowel resection are investigated. The pouches improve the transit time of the resected animal. However, there is no improvement in the weight curves after extensive small bowel resection. This negative effect is due to the establishment of a blind loop syndrome at the level of the pouch. The intestinal pouches have no place in the surgical treatment of the short bowel syndrome.

The technics of growing of small bowel neomucosa is one of the possible surgical therapies for the treatment of the short bowel syndrome. We carried out an experimental study in rats implanting patches and tubes of dura mater in the antimesenteric border of intestine. The growth of normal neomucosa is demonstrated. The animal tolerance to the dura mater patch is established in 3-4 cms. We also have proved that dura mater tubes are inefficacious. The dura mater is a good support for the growth of the small bowel neomucosa; however, it's necessary to prove their utility in the surgical treatment of short bowel syndrome.