Serial transverse enteroplasty and nipple valve construction, two life saving techniques for patients with short bowel syndrome, a report of 5 cases

Background Various abdominal pathologies end up with surgical resection of small intestine. When the small intestine remnant is too short for adequate fluid and micronutrients absorption, short bowel syndrome is diagnosed. The disabling condition needs a multidisciplinary approach to design parenteral nutrition, care for thrombotic, hepatic and infectious complications and gradually wean the patient from parenteral nutrition. Various surgical techniques have been introduced to increase absorptive mucosa and enhance the intestinal adaptation process. Serial transverse enteroplasty and nipple valve reconstruction are among the procedures, which will be discussed in the current article. Case presentation Herein, we presented 5 cases of short bowel syndrome as a consequence of abdominal laparotomies, patients were referred to our center to receive parenteral nutrition and to be prepared for the final autologous gastrointestinal reconstruction or intestinal transplantation, if indicated. Conclusion Patient’s age, performance status and bowel remnant length determines the appropriate technique for autologous gastrointestinal reconstruction. Serial transverse enteroplasty is designed to increase bowel’s length by creating zigzag patterns through dilated bowel loops. Presence of ileocecal valve is crucial to delay intestinal transit time and to prevent colonic bacterial transfer to ileum. Patient’s with ileocecal valve loss benefit from creating an artificial valve, namely, nipple valve.

Early effectivity of Teduglutide for the Treatment of short bowel Sydrome – our first Clinical experience

Background Short bowel syndrome is an uncommon malabsorptive condition most frequently caused by massive surgical resection of the small intestine. Standard of care is home parenteral nutrition. Teduglutide, glucagon like peptide 2 analog, was approved to treat patients with short bowel syndrome, who are stable following a period of post-surgery intestinal adaptation. We report two cases of patients with short bowel syndrome on home parenteral nutrition, treated with teduglutide, novel advanced therapy. We monitored the effectiveness and tolerance of the new treatment. Teduglutide increases intestinal absorption, causes cryptal hyperplasia, villous hypertrophy, angiogenesis, and allows weaning from parenteral nutrition. We confirmed the early onset of treatment effectiveness in the 4th month. Early effectiveness of treatment allowed a reduction in the volume of home parenteral nutrition with an improvement in quality of life.

Intestinal Adaptation upon Chemotherapy-Induced Intestinal Injury in Mice Depends on GLP-2 Receptor Activation

Intestinal adaptation is an important response and a natural repair mechanism in acute intestinal injury and is critical for recovery. Glucagon-like peptide 2 (GLP-2) has been demonstrated to enhance mucosal repair following intestinal damage. In this study, we aimed to investigate the role of GLP-2 receptor activation on intestinal protection and adaptation upon chemotherapy-induced intestinal injury. The injury was induced with a single injection of 5-fluorouracil in female GLP-2 receptor knockout (GLP-2R(-/-)) mice and their wild type (WT) littermates. The mice were euthanized in the acute or the recovery phase of the injury; the small intestines were analysed for weight changes, morphology, histology, inflammation, apoptosis and proliferation. In the acute phase, only inflammation was slightly increased in the GLP-2R(-/-) mice compared to WT. In the recovery phase, we observed the natural compensatory response with an increase in small intestinal weight, crypt depth and villus height in WT mice, and this was absent in the GLP-2R(-/-) mice. Both genotypes responded with hyperproliferation. From this, we concluded that GLP-2R signalling does not have a major impact on acute intestinal injury but is pivotal for the adaptive response in the small intestine.

Early effectivity of teduglutide for the treatment of short bowel sydrome – our first clinical experience

Background Short bowel syndrome is an uncommon malabsorptive condition most frequently caused by massive surgical resection of the small intestine. Standard of care is home parenteral nutrition. Teduglutide, glucagon like peptide 2 analog, was approved to treat patients with short bowel syndrome, who are stable following a period of post-surgery intestinal adaptation. Case presentation We report two cases of patients with short bowel syndrome on home parenteral nutrition, treated with teduglutide, novel advanced therapy. We monitored the effectiveness and tolerance of the new treatment. Conclusion Teduglutide increases intestinal absorption, causes cryptal hyperplasia, villous hypertrophy, angiogenesis, and allows weaning from parenteral nutrition. We confirmed the early onset of treatment effectiveness in the 4th month. Early effectiveness of treatment allowed a reduction in the volume of home parenteral nutrition with an improvement in quality of life.

Short bowel syndrome in infancy: recent advances and practical management

Short bowel syndrome (SBS) is a rare condition characterised by extensive loss of intestinal mass secondary to congenital or acquired disease. The outcomes are determined by dependency on parenteral nutrition (PN), its possible complications and factors that influence intestinal adaptation. In order to achieve the best results, patients should be managed by a specialised multidisciplinary team with the aims of promoting growth and development, stimulating intestinal adaptation and preventing possible complications. This involves timely surgical management aimed at rescuing maximum bowel length and eventually re-establishing intestinal continuity where appropriate. A combination of enteral and parenteral nutrition needs to be targeted towards maintaining a balance between fulfilling the nutritional and metabolic needs of the child while preventing or at least minimising potential complications. Enteral nutrition and establishment of oral feeding play a fundamental role in stimulating bowel adaptation and promoting enteral autonomy. Other measures to promote enteral autonomy include the chyme recycling in patients where bowel is not in continuity, autologous gastrointestinal reconstruction and pharmacological treatments, including promising new therapies like teduglutide. Strategies such as lipid reduction, changing the type of lipid emulsion and cycling PN are associated with a reduction in the rates of intestinal failure–associated liver disease. Even though vast improvements have been made in the surgical and medical management of SBS, there is still lack of consensus in many aspects and collaboration is essential.

Loss of Slfn3 induces a gender-dependent repair vulnerability after 50% bowel resection

Bowel resection accelerates enterocyte proliferation in the remaining gut that may have suboptimal absorptive and digestive capacity because of proliferation-associated decrease in functional differentiation markers. We hypothesized that although Slfn3 is an important regulator of murine enterocytic differentiation, Slfn3 would have less impact on the bowel resection adaptation where accelerated proliferation takes priority over differentiation. We assessed proliferation, cell shedding, and enterocyte differentiation markers of mucosa from resected and postoperative bowel of wild type (WT) and Slfn3 knockout (Slfn3KO) mice. Villus length and crypt depth were increased in WT mice and was even longer in Slfn3KO mice, while female Slfn3KO mice displayed even deeper crypts than both WT sexes after resection. Mitotic marker, Phh3+, and proliferation marker expression of Lgr5, FoxL1, and PDGFRα were increased after resection in male WT but this effect was blunted in male Slfn3KO mice. Cell shedding regulators Villin1 and TNFα were downregulated in female mice and male WT mice only, while Gelsolin and EGFR increased expression in all mice. Slfn3 expression increased after resection in WT mice but differentiation markers sucrase isomaltase, Dpp4, Glut2, and SGLT1 were all decreased. This suggests that enterocytic differentiation effort is incompatible with a rapid proliferation shift in intestinal adaptation. Slfn3 absence potentiates villus length and crypt depth, suggesting that the differentiating stimulus of Slfn3 signaling may restrain mucosal mass increase through regulating Villin1, Gelsolin, EGFR, TNFα, and proliferation markers. Slfn3 may therefore be an important regulator not only of "normal" enterocytic differentiation but also the response to bowel resection.

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome

Introduction The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise. Materials and Methods An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association. Results Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively; p = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A (p = 0.09 and p = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively; p = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) (p = 0.02). Conclusion Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.