scholarly journals Congenital Short Bowel Syndrome: A Case Report and Review of the Literature

2008 ◽  
Vol 22 (1) ◽  
pp. 71-74 ◽  
Author(s):  
Mohammed Hasosah ◽  
Daniel A Lemberg ◽  
Eric Skarsgard ◽  
Richard Schreiber
Keyword(s):  
Small Bowel ◽  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Rare Condition ◽  
Normal Growth ◽  
Upper Gastrointestinal Endoscopy ◽  
Term Survival ◽  
Short Bowel ◽  
Upper Gastrointestinal

Congenital short bowel syndrome (SBS) is a rare condition of the newborn, with several reports demonstrating high mortality. A six-week-old boy presented with chronic diarrhea and failure to thrive. An upper gastrointestinal endoscopy showed a straight duodenum, and multiple small bowel biopsies were histologically normal. An upper gastrointestinal series showed malrotation. At laparotomy, the small bowel was 50 cm in length, confirming the diagnosis of congenital SBS. Parenteral nutrition was initiated and enteral feeding with an amino acid-based formula containing long-chain fatty acids was introduced early and gradually advanced. At the last follow-up examination at 24 months, he was thriving on a regular diet, with normal growth and development. Long-term survival of children with congenital SBS is now possible if enteral feeds are introduced early to promote intestinal adaptation, with subsequent weaning off parenteral nutrition.

scholarly journals Short bowel syndrome in infancy: recent advances and practical management

2020 ◽  
pp. flgastro-2020-101457
Author(s):  
Elena Cernat ◽  
Chloe Corlett ◽  
Natalia Iglesias ◽  
Nkem Onyeador ◽  
Julie Steele ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Intestinal Failure ◽  
Rare Condition ◽  
Oral Feeding ◽  
Short Bowel ◽  
Intestinal Continuity ◽  
Gastrointestinal Reconstruction ◽  
Lipid Reduction

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.

Short bowel syndrome in infants: the critical role of luminal nutrients in a management program

2014 ◽  
Vol 39 (7) ◽  
pp. 745-753 ◽  
Author(s):  
Claude C. Roy ◽  
Véronique Groleau ◽  
Lise Bouthillier ◽  
Marjolain Pineault ◽  
Maxime Thibault ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Critical Role ◽  
Normal Growth ◽  
Management Program ◽  
Nutrition Status ◽  
Electrolyte Balance ◽  
High Turnover ◽  
Short Bowel

Short bowel syndrome develops when the remnant mass of functioning enterocytes following massive resections cannot support growth or maintain fluid–electrolyte balance and requires parenteral nutrition. Resection itself stimulates the intestine’s inherent ability to adapt morphologically and functionally. The capacity to change is very much related to the high turnover rate of enterocytes and is mediated by several signals; these signals are mediated in large part by enteral nutrition. Early initiation of enteral feeding, close clinical monitoring, and ongoing assessment of intestinal adaptation are key to the prevention of irreversible intestinal failure. The length of the functional small bowel remnant is the most important variable affecting outcome. The major objective of intestinal rehabilitation programs is to achieve early oral nutritional autonomy while maintaining normal growth and nutrition status and minimizing total parenteral nutrition related comorbidities such as chronic progressive liver disease. Remarkable progress has been made in terms of survivability and quality of life, especially in the context of coordinated multidisciplinary programs, but much work remains to be done.

scholarly journals RARE CASE OF SHORT BOWEL SYNDROME

2019 ◽  
Vol 49 ◽  
pp. 6-8
Author(s):  
Azmaiparashvili G. აზმაიფარაშვილი გ. ◽  
Tomadze G. თომაძე გ. ◽  
Megreladze A. მეგრელაძე ა.
Keyword(s):  
Small Intestine ◽  
Small Bowel ◽  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Rare Case ◽  
Postoperative Management ◽  
Hepatic Flexure ◽  
Postoperative Treatment ◽  
Extensive Resection ◽  
Short Bowel

Short bowel syndrome is characterized by malabsorption following extensive resection of the small bowel. It may occur after resection of more than 50% and is certain after resection of more than 70% of the small intestine, or if less than 100 cm of small bowel remains.  Successful postoperative management of short bowel syndrome has been discussed. Patient was operated because of cancer of hepatic flexure of large bowel with invasion in stomach, pancreas, retroperitoneal space, mesentery of small bowel. Right sided colectomy and excessive resection of small bowel with limphodissection was performed and only 80 cm of small bowel was left together with the left part of the colon. Ileotransversoanastomosis was performed. After the adequate course of chemotherapy and partial parenteral nutrition patient’s general condition became satisfactory. Patient started to gain weight. Adequate postoperative treatment determined postoperative period without surgical and nutritional complication.

Preliminary Experience With Intestinal Transplantation in Infants and Children

PEDIATRICS ◽  
1996 ◽  
Vol 97 (4) ◽  
pp. 443-448
Author(s):  
Alan N. Langnas ◽  
B. W. Shaw ◽  
Dean L. Antonson ◽  
Stuart S. Kaufman ◽  
David R. Mack ◽  
...  
Keyword(s):  
Small Bowel ◽  
Parenteral Nutrition ◽  
Total Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Gastrointestinal Disease ◽  
Intestinal Transplantation ◽  
Infants And Children ◽  
Short Bowel ◽  
In Infants And Children

Objective. This report discusses the preliminary experience with intestinal transplantation in children at the University of Nebraska Medical Center. Patients. During the past 4 years, 16 intestinal transplants have been performed in infants and children. Thirteen have been combined liver and bowel transplants, and the remainder were isolated intestinal transplants. Nearly half of the patients were younger than 1 year of age at the time of surgery, and the vast majority were younger than 5 years of age. All but one had short bowel syndrome. Results. The 1-year actuarial patient and graft survival rates for recipients of liver and small bowel transplants were 76% and 61%, respectively. Eight of 13 patients who received liver and small bowel transplants remain alive at the time of this writing, with a mean length of follow-up of 263 (range, 7 to 1223) days. Six patients are currently free of total parenteral nutrition. All three patients receiving isolated intestinal transplants are alive and free of parenteral nutrition. The mean length of follow-up is 384 (range, 330 to 450) days. Major complications have included severe infections and rejection. Lymphoproliferative disease, graft-versus-host disease, and chylous ascites have not been major problems. Conclusions. Although intestinal transplantation is in its infancy, these preliminary results suggest combined liver and bowel transplants and isolated intestinal transplantation may be viable options for some patients with intestinal failure caused by short bowel syndrome or other gastrointestinal disease in whom long-term total parenteral nutrition is not an attractive option.

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

2017 ◽  
Vol 28 (05) ◽  
pp. 455-463 ◽  
Author(s):  
Teresa Capriati ◽  
Daniela Giorgio ◽  
Fabio Fusaro ◽  
Manila Candusso ◽  
Paolo Schingo ◽  
...  
Keyword(s):  
Small Bowel ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Bloodstream Infections ◽  
Small Bowel Resection ◽  
Strongly Correlated ◽  
Small Bowel Length ◽  
Short Bowel ◽  
Intestinal Length ◽  
End Stage

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.

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

2017 ◽  
Vol 266 (2) ◽  
pp. 369-375 ◽  
Author(s):  
Maria Hukkinen ◽  
Reetta Kivisaari ◽  
Laura Merras-Salmio ◽  
Antti Koivusalo ◽  
Mikko P. Pakarinen
Keyword(s):  
Small Bowel ◽  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Short Bowel ◽  
Bowel Dilatation

Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome

1999 ◽  
Vol 117 (5) ◽  
pp. 1043-1050 ◽  
Author(s):  
Bernard Messing ◽  
Pascal Crenn ◽  
Philippe Beau ◽  
Marie Christine Boutron-Ruault ◽  
Jean-Claude Rambaud ◽  
...  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Adult Patients ◽  
Term Survival ◽  
Long Term Survival ◽  
Short Bowel

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

2021 ◽  
pp. 230
Author(s):  
◽  
◽  
Keyword(s):  
Parenteral Nutrition ◽  
Short Bowel Syndrome ◽  
Treatment Effectiveness ◽  
Intestinal Adaptation ◽  
Standard Of Care ◽  
Home Parenteral Nutrition ◽  
Short Bowel ◽  
Post Surgery ◽  
Advanced Therapy ◽  
New Treatment

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.

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