scholarly journals Congenital short bowel syndrome: a rare cause of neonatal intestinal obstruction

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052095472
Author(s):  
Mostafa Zain ◽  
Mansour Abdelkader ◽  
Ahmed Azab ◽  
Mostafa Kotb
Keyword(s):  
Short Bowel Syndrome ◽  
Failure To Thrive ◽  
Gastrointestinal Disorder ◽  
Chronic Diarrhoea ◽  
Small Bowel Length ◽  
Short Bowel ◽  
Ileocecal Junction ◽  
Intestinal Length ◽  
Neonatal Intestinal Obstruction ◽  
Ladd’S Procedure

Congenital short bowel syndrome (CSBS) is an uncommon gastrointestinal disorder in which an unclear aetiology causes considerable intrauterine reduction in small bowel length. As a result of reduced absorptive intestinal length, chronic diarrhoea, vomiting, and consequently, failure to thrive are likely. We report a case of CSBS in a 26-day-old girl who had malrotation and a short bowel with a length of bowel from the pylorus to the ileocecal junction of approximately 40 cm. The patient underwent Ladd’s procedure, but she is still dependent on parenteral nutrition.

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 Closed Gastroschisis

2017 ◽  
Vol 6 (3) ◽  
pp. 61 ◽  
Author(s):  
Mohammed Abdel-Latif ◽  
Mohamed Hisham Soliman ◽  
Khaled Mohaned El-Asmar ◽  
Mohamed Abdel-Sattar ◽  
Ibrahim M Abdelraheem ◽  
...  
Keyword(s):  
Intestinal Obstruction ◽  
Short Bowel Syndrome ◽  
Intestinal Atresia ◽  
Rare Entity ◽  
Short Bowel ◽  
Neonatal Intestinal Obstruction

Closed gastroschisis is a rare entity usually associated with intestinal atresia and short bowel syndrome. We report two cases of closed gastroschisis presenting with neonatal intestinal obstruction and para-umbilical evisceration without an abdominal defect.

Preliminary In Vivo Experimental Validation of SMA Based Bowel Extender for Short Bowel Syndrome

2009 ◽  
Author(s):  
Brent Utter ◽  
Diann Brei ◽  
Jonathan Luntz ◽  
Daniel Teitelbaum ◽  
Manabu Okawada ◽  
...  
Keyword(s):  
Wireless Communication ◽  
Short Bowel Syndrome ◽  
Medical Condition ◽  
Ex Vivo ◽  
Tensile Loading ◽  
Battery Life ◽  
Small Bowel Length ◽  
Short Bowel ◽  
Surgical Implantation

Short Bowel Syndrome is a serious medical condition caused by insufficient small bowel length resulting in significantly high rates of morbidity and mortality. The limited success of current therapies has prompted the investigation of a new treatment approach based on mechanotransduction — the process through which mechanical tensile loading on the bowel induces longitudinal growth. To enable clinically relevant mechanotransduction growth studies in large animals, such as pigs, a fully implantable and instrumented bowel extender device based on a Shape Memory Alloy (SMA) ratchet was developed and validated in benchtop and ex vivo tests. These devices, however, must also be validated against the unique in vivo environment which presents challenges such as sealing, battery life, surgical implantation, signal attenuation from tissue, and isolating the measurement of tensile loading on the bowel wall. This paper extends the earlier development work to in vivo validation experiments within live pigs. A brief summary of the bowel extender architecture and operation is provided along with earlier ex vivo results that established device limits for in vivo testing. The wireless communication rate was updated to extend battery life and new surgical implantation procedures and lengthening schemes were developed. Two bowel extenders were tested in in vivo experiments ranging from 2.5 to 4.5 days with data collected to validate the wireless communication, SMA ratcheting and load/displacement measurements, confirming that the bowel extender successfully operates in vivo. More importantly, the bowel extenders successfully induced significant growth, which is promising for future studies comparing different lengthening schemes for optimal growth and the development of a clinical device for treating short bowel syndrome in humans.

The Use of Multiple Serial Transverse Enteroplasty (STEP) Procedures for the Management of Intestinal Atresia and Short Bowel Syndrome

2013 ◽  
Vol 79 (8) ◽  
pp. 826-828 ◽  
Author(s):  
Varun K. Bhalla ◽  
Walter L. Pipkin ◽  
Robyn M. Hatley ◽  
Charles G. Howell
Keyword(s):  
Small Bowel ◽  
Short Bowel Syndrome ◽  
Intestinal Transit Time ◽  
Small Bowel Length ◽  
Short Bowel ◽  
Jejunal Atresia ◽  
Repeat Procedure ◽  
Step Procedure ◽  
Primary Procedure ◽  
Serial Transverse Enteroplasty

The serial transverse enteroplasty procedure (STEP) was introduced as a bowel-lengthening procedure to reduce complications related to short bowel syndrome (SBS). Although some have described it as a useful adjunct to the Bianci procedure, others have acknowledged it as a primary procedure. We present a case of jejunal atresia in which two STEP procedures were performed 7 months apart to increase small bowel length. A 1-day-old, term girl presented with a known bowel obstruction diagnosed in utero. A laparotomy revealed a Type IIIb jejunal atresia with no remaining small bowel or cecum. A STEP procedure with an end jejunostomy and ascending colon mucous fistula lengthened the small bowel from 35 to 50 cm. A repeat procedure 7 months later lengthened it to 89 cm. The STEP procedure results in slower intestinal transit time and increases enterocytes contact with oral intake. We performed it during our initial exploration to increase small bowel size by 30 per cent. A repeat procedure 7 months later increased length to 89 cm. The use of multiple, staged STEP procedures avoided the need for bowel transplantation and long-term total parenteral nutrition dependence, demonstrating its effectiveness as a primary procedure for the surgical management of SBS.

Intestinal Failure and Short Bowel Syndrome in Crohn’s Disease: A Systematic Review

2021 ◽  
pp. 77-86
Author(s):  
Hayslan Theobaldo Boemer ◽  
Ana Valéria Garcia Ramirez ◽  
Durval Ribas Filho
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Short Bowel Syndrome ◽  
Intestinal Failure ◽  
Intestinal Secretion ◽  
Surgical Interventions ◽  
Short Bowel ◽  
Intestinal Length ◽  
Intestinal Rehabilitation ◽  
Long Term Prognosis

Crohn's disease (CD) is an inflammatory, chronic and progressive disease that affects the digestive tract. Despite optimized drug therapy, the risk of multiple surgical interventions over the years is high, leading the patient to develop short bowel syndrome (SBS). Thus, adequate management in the postoperative period directly interferes with the long-term prognosis. Initially, most of these patients, due to hydro electrolytic disorders and absorptive incapacity inherent in SBS, will need parenteral nutritional support. According to the patient's residual digestive profile and according to nutritional management (oral, enteral, and/or parenteral), the intestine will evolve in its adaptive capacity. During this period, control agents are used for motility and intestinal secretion and, if necessary, GLP-2 agonists (intestinotrophic). In cases refractory to these treatments, we can still indicate surgical procedures to control motility, increasing intestinal length, and, finally, transplantation. CD is recurrent, and patients with SBS need a multidisciplinary approach with continuous monitoring to provide better intestinal rehabilitation and consequent quality of life.

scholarly journals Adult zebrafish intestine resection: a novel model of short bowel syndrome, adaptation, and intestinal stem cell regeneration

2015 ◽  
Vol 309 (3) ◽  
pp. G135-G145 ◽  
Author(s):  
K. A. Schall ◽  
K. A. Holoyda ◽  
C. N. Grant ◽  
D. E. Levin ◽  
E. R. Torres ◽  
...  
Keyword(s):  
Stem Cell ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Intestinal Stem Cell ◽  
Brdu Incorporation ◽  
In Vivo Model ◽  
In Vivo Models ◽  
Short Bowel ◽  
Intestinal Length

Loss of significant intestinal length from congenital anomaly or disease may lead to short bowel syndrome (SBS); intestinal failure may be partially offset by a gain in epithelial surface area, termed adaptation. Current in vivo models of SBS are costly and technically challenging. Operative times and survival rates have slowed extension to transgenic models. We created a new reproducible in vivo model of SBS in zebrafish, a tractable vertebrate model, to facilitate investigation of the mechanisms of intestinal adaptation. Proximal intestinal diversion at segment 1 (S1, equivalent to jejunum) was performed in adult male zebrafish. SBS fish emptied distal intestinal contents via stoma as in the human disease. After 2 wk, S1 was dilated compared with controls and villus ridges had increased complexity, contributing to greater villus epithelial perimeter. The number of intervillus pockets, the intestinal stem cell zone of the zebrafish increased and contained a higher number of bromodeoxyuridine (BrdU)-labeled cells after 2 wk of SBS. Egf receptor and a subset of its ligands, also drivers of adaptation, were upregulated in SBS fish. Igf has been reported as a driver of intestinal adaptation in other animal models, and SBS fish exposed to a pharmacological inhibitor of the Igf receptor failed to demonstrate signs of intestinal adaptation, such as increased inner epithelial perimeter and BrdU incorporation. We describe a technically feasible model of human SBS in the zebrafish, a faster and less expensive tool to investigate intestinal stem cell plasticity as well as the mechanisms that drive intestinal adaptation.

The adolescent child with short bowel syndrome: new onset of failure to thrive and need for increased nutritional supplementation

2010 ◽  
Vol 45 (6) ◽  
pp. 1280-1286 ◽  
Author(s):  
Eiichi A. Miyasaka ◽  
Pamela I. Brown ◽  
Shirley Kadoura ◽  
Mary Beth Harris ◽  
Daniel H. Teitelbaum
Keyword(s):  
Short Bowel Syndrome ◽  
Failure To Thrive ◽  
Nutritional Supplementation ◽  
Short Bowel ◽  
Adolescent Child ◽  
New Onset

scholarly journals Synergy of glucagon-like peptide-2 and epidermal growth factor coadministration on intestinal adaptation in neonatal piglets with short bowel syndrome

2017 ◽  
Vol 312 (4) ◽  
pp. G390-G404 ◽  
Author(s):  
David W. Lim ◽  
Crystal L. Levesque ◽  
Donna F. Vine ◽  
Mitsuru Muto ◽  
Jacob R. Koepke ◽  
...  
Keyword(s):  
Growth Factors ◽  
Growth Factor ◽  
Epidermal Growth Factor ◽  
Short Bowel Syndrome ◽  
Intestinal Adaptation ◽  
Villus Height ◽  
Short Bowel ◽  
Intestinal Length ◽  
Epidermal Growth ◽  
Glucagon Like Peptide

Glucagon-like peptide-2 (GLP-2) and epidermal growth factor (EGF) treatment enhance intestinal adaptation. To determine whether these growth factors exert synergistic effects on intestinal growth and function, GLP-2 and EGF-containing media (EGF-cm) were administered, alone and in combination, in neonatal piglet models of short bowel syndrome (SBS). Neonatal Landrace-Large White piglets were block randomized to 75% midintestinal [jejunoileal (JI) group] or distal intestinal [jejunocolic (JC) group] resection or sham control, with 7-day infusion of saline (control), intravenous human GLP-2 (11 nmol·kg−1·day−1) alone, enteral EGF-cm (80 μg·kg−1·day−1) alone, or GLP-2 and EGF-cm in combination. Adaptation was assessed by intestinal length, histopathology, Üssing chamber analysis, and real-time quantitative PCR of intestinal growth factors. Combined EGF-cm and GLP-2 treatment increased intestinal length in all three surgical models ( P < 0.01). EGF-cm alone selectively increased bowel weight per length and jejunal villus height in the JI group only. The JC group demonstrated increased intestinal weight and villus height ( P < 0.01) when given either GLP-2 alone or in combination with EGF-cm, with no effect of EGF-cm alone. Jejunal permeability of mannitol and polyethylene glycol decreased with combination therapy in both SBS groups ( P < 0.05). No difference was observed in fat absorption or body weight gain. IGF-1 mRNA was differentially expressed in JI vs. JC piglets with treatment. Combined treatment with GLP-2 and EGF-cm induced intestinal lengthening and decreased permeability, in addition to the trophic effects of GLP-2 alone. Our findings demonstrate the benefits of novel combination GLP-2 and EGF treatment for neonatal SBS, especially in the JC model representing most human infants with SBS. NEW & NOTEWORTHY Glucagon-like peptide-2 (GLP-2) and epidermal growth factor (EGF) are intestinotrophic, with demonstrated benefit in both animal models and human studies of short bowel syndrome (SBS). The current research shows that over and above known trophic effects, the combination of GLP-2 and EGF synergistically lengthens the bowel in neonatal piglet models of SBS. Most notable benefit occurred with resection of the terminal ileum, the common clinical anatomy seen in neonatal SBS and associated with least de novo lengthening postsurgery.

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