Autologous gastrointestinal reconstruction surgery for short bowel syndrome: the cornerstone for intestinal rehabilitation.

PURPOSE OF REVIEW: Intestinal failure (IF) evolved from being the last recognized organ failure, to become one of the most progressive fields in terms of therapeutic alternatives and results. Short bowel syndrome (SBS) is the main cause of IF in adults and children. The use of surgery allowed patients with unfavorable anatomy type and length to be wean off parenteral nutrition. We aim to evaluate its current impact on intestinal rehabilitation. RECENT FINDINGS: Autologous gastro-intestinal reconstructive surgery (AGIRS), including bowel lengthening contributes by converting patient's anatomy to a more favorable one, improving quality of life, and modifying the natural history of the disease, allowing to recover intestinal autonomy in approximately 70% of the adults and 50% of the children's with SBS-IF. The current use of postsurgical medical rehabilitation strategies including the use of enterohormones complement the path to sufficiency, increasing the chances of success in both age group of patients. SUMMARY: The development of AGIRS has changed the outcome of SBS-IF patients, becoming the main surgical procedure prescribed in multidisciplinary units, allowing to enhance the number of patients achieving intestinal autonomy throughout rehabilitation, leaving transplantation as the last surgical alternative.

Minimal Enteral Nutrition to Improve Adaptation After Intestinal Resection in Piglets and Infants.

BACKGROUND: Minimal enteral nutrition (MEN) may induce a diet-dependent stimulation of gut adaptation following intestinal resection. Bovine colostrum is rich in growth factors, and we hypothesized that MEN with colostrum would stimulate intestinal adaptation, compared with formula, and would be well tolerated in patients with short bowel syndrome. METHODS: In experiment 1, 3-day-old piglets with 50% distal small intestinal resection were fed parenteral nutrition (PN, n = 10) or PN plus MEN given as either colostrum (PN-COL, n = 5) or formula (PN-FORM, n = 9) for 7 days. Intestinal nutrient absorption and histomorphometry were performed. In experiment 2, tolerance and feasibility of colostrum supplementation were tested in a pilot study on 5 infants who had undergone intestinal resection, and they were compared with 5 resected infants who served as controls. RESULTS: In experiment 1, relative wet-weight absorption and intestinal villus height were higher in PN-COL vs PN (53% vs 23% and 362 ± 13 vs 329 ± 7 µm, P < .05). Crypt depth and tissue protein synthesis were higher in PN-COL (233 ± 7 µm, 22%/d) and PN-FORM (262 ± 13 µm, 22%/d) vs PN (190 ± 4 µm, 9%/d, both P < .05). In experiment 2, enteral colostrum supplementation was well tolerated, and no infants developed clinical signs of cow's milk allergy. CONCLUSION: Minimal enteral nutrition feeding with bovine colostrum and formula induced similar intestinal adaptation after resection in piglets. Colostrum was well tolerated by newly resected infants, but the clinical indication for colostrum supplementation to infants subjected to intestinal resection remains to be determined.

Management of short bowel syndrome in postoperative very low birth weight infants.

Short bowel syndrome is a potentially devastating morbidity for the very low birth weight infant and family with a high risk for mortality. Prevention of injury to the intestine is the ideal, but, if and when the problem arises, it is important to have a systematic approach to manage nutrition, use pharmaceutical strategies and tools to maximize the outcome potential. Safely maximizing parenteral nutrition support by providing adequate macronutrients and micronutrients while minimizing its hepatotoxic effects is the initial postoperative strategy. As the infant stabilizes and starts to recover from that initial injury and/or surgery, a slow and closely monitored enteral nutrition approach should be initiated. Enteral feeds can be complemented with medications and supplements emerging as valuable clinical tools. Engaging a multidisciplinary team of neonatologists, gastroenterologists, pharmacists, skilled clinical nutrition support staff including registered dietitians and nutrition support nurses will facilitate optimizing each and every infant's long term result. Promoting intestinal rehabilitation and adaptation through evidence-based practice where it is found, and ongoing pursuit of research in this rare and devastating disease, is paramount in achieving optimal outcomes.

Short bowel syndrome: treatment options / Síndrome Intestino Curto: abordagens terapêuticas

Introduction: Short bowel syndrome (SBS) refers to the malabsorptive state that occurs following extensive intestinal resection and is associated with several complications. Methods: The research for this review was conducted in the Pubmed database. Relevant scientific articles dated between 1991 and 2015 and written in Portuguese, Spanish or English were selected. Results: Several therapies, including nutritional support, pharmacological options and surgical procedures have been used in these patients. Conclusions: Over the last decades new surgical and pharmacological approaches emerged, increasing survival and quality of life (QoL) in patients with SBS. All SBS patients ought to have an individualized and multidisciplinary care that promotes intestinal rehabilitation.

Introdução: A Síndrome do Intestino Curto (SIC) resulta da perda da capacidade de absorção do intestino após resseção intestinal extensa e está associada a diversas complicações. Métodos: Esta revisão foi realizada com base em artigos científicos originais pesquisados na base de dados MEDLINE via Pubmed, na língua portuguesa, inglesa e espanhola, com o limite temporal de 1991 a 2015. Resultados: O tratamento instituído pode ser a nível nutricional, farmacológico ou cirúrgico. Conclusões: Ao longo das últimas décadas surgiram novas abordagens terapêuticas cirúrgicas e não-cirúrgicas que melhoraram a sobrevivência e a qualidade de vida (QoL) destes pacientes. Deve-se estabelecer uma abordagem multidisciplinar e individualizada para garantir a melhor reabilitação.

Radiation enteritis leading to intestinal failure: 1994 patient-years of experience in a national referral centre.

BACKGROUND/OBJECTIVES: Chronic radiation enteritis (RE) has been reported in up to 20% of patients receiving pelvic radiotherapy and can lead to intestinal failure (IF), accounting for 3.9% of new registrants for home parenteral nutrition (HPN) in the UK annually. Our aim is to report nutritional and survival outcomes for patients with RE referred to a national IF unit. SUBJECTS/METHODS: A retrospective study of all new admissions over a 13-year period at the Intestinal Failure Centre, Manchester, UK. Data are presented as median (range). RESULTS: Twenty-three (3.8%) of 611 patients were admitted with IF secondary to RE. The primary site of malignancy was genitourinary in 17 (74%) patients. Radiotherapy was administered 9.5 (1-42) years previously. Patients underwent 2 (1-5) laparotomies prior to intestinal failure unit (IFU) admission. Twelve (52%) patients were admitted with intestinal obstruction and 11 (48%) with intractable weight loss and/or high output fistulae/stomas. Additional conditions contributing to IF were noted in 11 (48%) patients. Twenty-two (96%) patients had 2 (1-5) laparotomies prior to IFU referral. At discharge, 5 (22%) patients resumed oral diet without the need for artificial nutrition support, 3 (13%) required enteral feeding and 13 (56%) commenced HPN. The 10-year survival of the patient cohort was 48.2%. CONCLUSIONS: Surgical intervention is infrequently required, whereas the majority of patients with IF secondary to RE require long-term HPN. The judicious use of surgery in selected patients, coupled with an aggressive medical strategy to detect and treat contributing factors, and optimal enteral feeding may allow a modest proportion of patients with IF secondary to RE to achieve independence from PN.

Autologous gastrointestinal reconstructive surgery: complement or alternative to intestinal transplantation.

PURPOSE OF REVIEW: Autologous intestinal reconstructive surgery has evolved over the past 3 decades from rescue to main surgical procedure in the multidisciplinary approach to short bowel syndrome (SBS) patients with intestinal failure. The purpose of this review is to clarify the actual place of intestinal reconstructive surgical techniques in the management of intestinal failure related to SBS and their relationship with intestinal transplantation. RECENT FINDINGS: Recent reports from centers of excellence in intestinal rehabilitation underline the efficacy and safety of autologous intestinal reconstructive surgery in patients with SBS. Outcome parameters as survival, parenteral nutrition weaning, and clinical conditions were improved in SBS patients treated by gastrointestinal reconstructive surgery. SUMMARY: Autologous intestinal reconstructive procedures are pivotal to achieve enteral autonomy in patients with intestinal failure related to SBS. They should be considered mutually supportive and not antagonistic to intestinal transplantation.

Pregnancy in an intestinal transplant recipient.

BACKGROUND: Intestinal transplantation is a relatively new form of therapy for short gut syndrome. Pregnancy after intestinal transplantation is rare. CASE: A 26-year-old small bowel transplant recipient presented for prenatal care. She previously had undergone bariatric surgery and later experienced small bowel necrosis and resection. The resulting short gut syndrome was treated with an isolated small bowel transplant. Medications during this pregnancy included prednisone, esomeprazole, diphenoxylate-atropine, ascorbic acid, tacrolimus, and magnesium supplementation. Throughout her pregnancy, her creatinine level was elevated. Labor was induced at 39 3/7 weeks and resulted in a spontaneous vaginal delivery of a healthy female neonate. Twelve weeks after delivery, the mother was admitted for a rejection reaction that was treated successfully. CONCLUSION: A successful pregnancy in an intestinal transplant recipient resulted in delivery of a healthy term newborn.

Chronic rejection in a small bowel transplant with successful revision of the allograft by segmental resection: case report.

An 8-year-old girl was admitted for severe electrolyte imbalance and for hyponatremic seizure. In July 2005, at 3 years of age, she underwent isolated small-bowel transplantation of 100 cm ileum from her father. Her own bowel was only 50 cm of proximal jejunum which had been directly connected to the anus due to extended total aganglionosis. The graft was placed into the middle of her remaining bowel, using the splenic artery and vein as feeding vessels with saving of the spleen. Daclizumab induction and tacrolimus monotherapy were applied for immunosuppression. Two acute cellular rejection episodes, E on day 10 and 4 years after transplantation, were successfully treated with OKT-3 and recombinant antithymocyte globulin, respectively. However, because of intermittent bowel dysfunction, she was hospitalized several times for hydration and metabolic care. On admission, her abdomen was moderately distended, and a simple abdominal film showed a fixed dilated loop. Colonoscopy could not pass the narrowed lumen, with stiffness at the anastomosis between the graft and the distal bowel. Endoscopic biopsy at the entrance to the stricture showed a nonspecific inflammatory reaction with fibrosis. Similar findings on a gastrograffin enema suggested chronic rejection (CR). On laparotomy, an irregularly narrowed fibrotic loop was noticed at the distal part of the graft, proximal to the anastomosis. We performed a 20-cm segmental resection with an end-to-end anastomosis. Histopathologic findings showed CR with fibrosis and hyalinization of the entire bowel wall and vessel walls with mild cellular infiltrations. She recovered in 10 days. The graft may have been saved, but intermittent requirement of hydration over the following months suggested progressive graft dysfunction. A case of segmental involvement of CR with subsequent successful graft salvage by partial resection is rare in the literature.

Prevention and reversal of intestinal failure-associated liver disease in premature infants with short bowel syndrome using intravenous fish oil in combination with omega-6/9 lipid emulsions.

Although premature infants with short bowel syndrome are at the highest risk of developing intestinal failure-associated liver disease (IFALD), they have great capacity for intestinal growth and adaptation if IFALD can be prevented. Conventional soybean oil-based intravenous lipid emulsions have been associated with IFALD. This study presents data on 5 premature neonates with short bowel syndrome treated with a combination of parenteral fish oil- and olive/soybean-based lipid emulsion for periods ranging between 7 and 17 months. Despite an enteral tolerance of less than 50% in 4 of these patients during their first year of life, direct bilirubin levels normalized while on this combination of ClinOleic (Baxter, Maurepas, France)/Omegaven (Fresenius Kabi, Bad Homburg, Germany) at a 1:1 ratio. None of our patients developed irreversible IFALD even though all of them were premature, had undergone multiple major surgical procedures, and had experienced several episodes of sepsis. Thus far, we have not seen any adverse effects of this mixed lipid emulsion in these preterm infants. All 5 patients are growing and developing well and have normal liver function.

Elements of successful intestinal rehabilitation.

PURPOSE: The optimal therapy for intestinal failure (IF) is unknown. The results of a systematic, protocol-driven management strategy by a multidisciplinary team are described. METHODS: Intestinal failure was defined as bowel length of less than 40 cm or parenteral nutrition (PN) for more than 42 days. A multidisciplinary team and protocol to prevent PN-associated liver disease (PNALD) were instituted in 2006. Data were gathered prospectively with consent and ethics board approval. RESULTS: From 1998 to 2006, 33 patients were treated (historical cohort) with an overall survival of 72%. Rotating prophylactic antibiotics for bacterial overgrowth were given to 27% of patients; 6% had lipid-sparing PN, and none received fish oil-based lipids. Median time to intestinal rehabilitation was 7 ± 3.1 months, and 27% of patients who developed PNALD died. From 2006 to 2009, 31 patients were treated. Seventy-seven percent received PAB; 60%, lipid-sparing PN; and 47%, parenteral fish oil emulsion. Eighty-seven percent weaned from PN at 3.9 ± 3.8 months, and no patients developed PNALD with 100% survival. Novel lipid therapies were associated with changes in essential fatty acid profile and one case of clinical essential fatty acid deficiency. CONCLUSION: The institution of a multidisciplinary team and a protocol-driven strategy to prevent PNALD improves survival in IF. Further studies are recommended.

Parenteral but not enteral omega-3 fatty acids (Omegaven) modulate intestinal regrowth after massive small bowel resection in rats.

BACKGROUND: The purpose of the present study was to evaluate the effects of ω-3 fatty acids (Omegaven) on early intestinal adaptation in rats with short bowel syndrome (SBS). METHODS: Male Sprague-Dawley rats were randomly assigned to 1 of 4 groups: sham rats underwent bowel transection; SBS rats underwent 75% bowel resection; SBS-O ω-3 rats underwent bowel resection and were treated with oral Omegaven given by gavage; and SBS-I ω-3 rats underwent bowel resection and were treated with Omegaven given intraperitoneally. Rats were killed on day 14. Parameters of intestinal adaptation (bowel and mucosal weight, mucosal DNA and protein, villus height and crypt depths, cell proliferation and apoptosis) were determined at time of death. Real-time polymerase chain reaction was used to determine the level of Bax and Bcl-2 messenger RNA (mRNA). Statistical analysis was performed using Kruskal-Wallis test followed by post hoc test, with P < .05 considered statistically significant. RESULTS: Oral ω-3 supplementation did not significantly change intestinal regrowth. In contrast, parenteral ω-3 in rats that underwent resection resulted in higher bowel and mucosal weights, mucosal DNA and protein in ileum, villus height in ileum, crypt depth in jejunum and ileum, and greater rates of cell proliferation in jejunum and ileum compared with SBS animals. The initial decreased levels of apoptosis corresponded with the early decrease in Bax and increase in Bcl-2 mRNA levels. CONCLUSIONS: Parenteral but not enteral Omegaven augments and accelerates structural bowel adaptation in a rat model of SBS. Increased cell proliferation and decreased apoptosis reflect increased cell turnover in Omegaven-treated animals.

[Parenteral nutrition in the treatment of short bowel syndrome].

Relevance of nutritive support problems at patients with SRL is shown in experiences of RSSC members named by B.V. Petrovski RAMS. This article demonstrates feasibility of artificial therapeutic nutrition and role of parenteral nutrition, clinical and social rehabilitation at patients with SRL.

Ingestion of ferrous sulfate increases ferremia in patients with short bowel syndrome.

OBJECTIVE: Because short bowel syndrome is associated with iron deficiency, the objective of the present study was to monitor ferremia after the ingestion of different iron compounds and doses in enterectomized patients. METHODS: This was a randomized, double-blind, cross-over study conducted in 13 patients of both sexes in the metabolic unit of Hospital das Clínicas de Faculdade de Medicina de Ribeirão Preto-Universídade de São Paulo and was divided into two stages. Three different iron compounds, ferrous sulfate (FS), sodium iron ethylenediaminetetra-acetic acid (EDTA), and dehydrated cell fraction (DCF), were studied. The patients were randomly assigned to one of four groups receiving high-dose DCF (120mg) and low-dose DCF (5mg) and the two iron compounds. The subjects (n=7) receiving high-dose DCF participated in 2 test days. All patients receiving the physiologic dose (low-dose DCF, n=6) participated in 3 test days with a 1-mo interval between compounds. After an 8-h fast, blood samples were collected at 0.25, 0.5, 1, and 2h. Serum iron curve and the sum of the area under the curve were calculated and adjusted according to a mixed-effect linear model (P<0.05). RESULTS: Serum FS levels were higher in the 120-mg group compared with the others (P<0.005). The mean areas under the curve for FS and EDTA at the doses of 120 and 5mg of elemental iron were 238, 224, 177, and 153 microg/dL, respectively. The mean area under the curve for DCF was 165 microg/dL, with no significant difference between groups. CONCLUSION: Regardless of dose, FS was the compound that resulted in higher ferremia compared with the other doses and compounds.

Colostrum supplementation restores insulin-like growth factor -1 levels and alters muscle morphology following massive small bowel resection.

BACKGROUND: Colostrum protein concentrate (CPC) contains a high level of insulin-like growth factor-1 (IGF-1). IGF-1 and IGF binding protein (IGFBPs) may play an important role during the postresection adaptation response. As smooth muscle is an important site for IGF-1 action in the intestine, this study aims to (1) investigate the effect of CPC supplementation on circulating levels and tissue expression of IGF-1, IGF-1 receptor, and IGFBPs following massive small bowel resection (MSBR), and (2) characterize the effect of CPC on the muscular adaptation response following MSBR. METHODS: Four-week-old piglets underwent either a 75% MSBR or sham operation. Piglets received either a polymeric infant formula (PIF) diet or PIF supplemented with CPC for 8 weeks. Serum was analyzed by enzyme-linked immunosorbent assay, and ileal tissue assessed by molecular and histological analysis. RESULTS: There was no difference in IGF-1 or IGFBPs mRNA among groups. CPC treatment resulted in significant increases in circulating levels of IGF-1 and IGFBPs and a concurrent increase in muscle width and the number of muscle cells, but did not alter muscle cell size. CONCLUSIONS: Strategies aimed at increasing muscular adaptation may decrease Gl transit and allow greater mucosal contact time for absorption. We have shown that CPC supplementation following resection results in increased levels of circulating IGF-1, IGFBP-2, and IGFBP-3 and muscular hypertrophy. Our results suggest that IGF-1 and its mediators may play a role in the muscular adaptation response and warrant further exploration as a treatment option for short bowel syndrome.

Review of incidence and management of chylous ascites after small bowel transplantation.

Nutrition management of intestinal transplant recipients continues to be a challenging and essential component of the early postoperative care of this patient population. The absorptive capacity of the graft can be affected by immunologic and nonimmunologic factors, including enteric lymphatic disruption, preservation injury, central denervation, viral enteritis, systemic infections, and rejection. Chylous ascites, the extravasation of milky chyle into the peritoneal fluid, defined by elevated triglycerides levels of > or = 200 mg/dL, can occur as a result of trauma, obstruction, or interruption of the lymphatic system. It seems the incidence of chylous ascites after small bowel transplantation is low; however, this may be due in part to the limitation of enteral long-chain triglycerides in the early posttransplant period of 2-6 weeks. After this time frame, clinical evidence suggests that fat assimilation normalizes. In the event that chylous ascites develop as a posttransplant complication, limitation of oral or enteral nutrition support to a very-low-fat regimen may be required, or parenteral nutrition (PN) will need to be provided until clinical status improves. Long-term posttransplant, lymphatic regeneration generally occurs and the majority of intestinal transplant recipients achieve the ultimate goal of nutrition autonomy.

Oral antibiotics attenuate bowel segment reversal-induced systemic inflammatory response and body weight loss in massively bowel-resected rats.

BACKGROUND: Using a massively bowel-resected rat model, our previous study demonstrated that small bowel segment reversal stimulates jejunal hyperplasia but may also increase the possibility of bacterial translocation and the elevation of circulating white blood cells and serum interleukin-6 that may reduce the whole-body anabolism. The aim of this study is to investigate whether oral antibiotics might attenuate the inflammatory responses and might therefore facilitate the beneficial effects of bowel segment reversal. METHODS: Male Wistar rats (approximately 270 g) underwent a 70% small bowel resection with (REV group) or without (CON group) a 3-cm small bowel segment reversal, or underwent a sham operation (SHAM group). After surgeries, half of the animals in the REV group were given oral clindamycin plus amoxicillin (50 plus 50 mg/kg/d, ANT group) for 3 weeks. RESULTS: Oral antibiotics administration significantly attenuated the decreases in feeding efficiency (g of body weight/100 kcal diet) and increases in the circulation of white blood cells, serum nitric oxide, and interleukin-6 (1-way ANOVA, p < .05), which are associated with bowel segment reversal. In addition, antibiotics significantly increased serum concentrations of insulin-like growth factor-I, significantly decreased the total numbers of bacteria in the intestine, and tended to reduce the extent of jejunal hyperplasia in rats with bowel segment reversal. CONCLUSIONS: Our results suggest that oral antibiotics may be used as an adjuvant to attenuate the inflammatory responses and to enhance the anabolic responses in massively bowel-resected patients with bowel segment reversal.

A propósito de un caso de intestino corto en un paciente de edad avanzada / A propos of a case of short bowel in an elderly patient

La resección de grandes segmentos de intestino delgado puede causar grados variables de mala bsorción. Por lo tanto, las consecuencias de la resección dependerán de la longitud y la localización del segmento resecado, pero también de la edad y de la enfermedad que originó la resección. La instauración del Soporte Nutricional tras la resección intestinal es el principal factor estimulador de la adaptación del intestino remanente e importante para evitar la desnutrición. La relevancia de este caso se debe a que nuestro paciente con 87 años de edad y después de 10 años de cirugía, tiene un buen estado nutricional y una buena calidad de vida, adaptándose su intestino remanente a las funciones fisiológicas (AU)

Resection of long segments of the small bowel may cause variable degrees of malabsorption. Therefore, the results of the resection will depend on the length and location of the resected segment, and on the age of the patient and the underlying disease that motivated the resection. The implementation of Nutritional Support after intestinal resection is the main stimulating factor for adaptation of the remnant bowel and is important to prevent hyponutrition. The relevance of this case is due to the fact that our 87 years old patient has a good nutritional status and good quality of life 10 years after resection was performed, his remnant bowel having adapted to physiological functions (AU)

Nutrition management of small bowel transplant patients.

Nutrition therapy after small bowel or combined liver/small bowel transplantation is challenging. The objective is to restore enteral autonomy to a patient with a complex past surgical history and equally complex posttransplant immunosuppressive regimen in the context of a newly created surgical anatomy. Improved surgical techniques and immunosuppressive regimens have led to superior outcomes. Accompanying these advances is a range of nutrition issues that require specific management strategies. This review outlines the current clinical practice and decision making used to create individualized nutrition regimens for small bowel or combined liver/small bowel transplant recipients. Successful small bowel transplant outcomes require a coordinated effort from a transplant team to restore nutritional autonomy to transplant recipients and free them from parenteral nutrition.

[Results of an intestinal transplantation program in Spain. Five years later]. / Resultados de un programa de trasplante intestinal. Cinco años después.

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.

Congenital short bowel syndrome with malrotation.

Congenital short bowel syndrome (SBS) associated with malrotation and malabsorption is a very rare condition. We report on an infant girl with congenital SBS associated with malrotation and malabsorption. No polyhydraminos was noted during the regular prenatal examination. Protracted postnatal postprandial vomiting with progressive failure to thrive was noted. A laparotomy showed the small bowel was only about 20 cm in length. She eventually survived with short-term parenteral nutrition and use of oral L-glutamine supplementation. To our knowledge, this might be the shortest length of bowel loop ever reported. Currently, she is 15 months of age with a body weight of about 7 kg and good development.