23 results on '"Short Bowel Syndrome surgery"'
Search Results
2. Re: Bowel Lengthening Procedures in Children with Short Bowel Syndrome: A Systematic Review.
- Author
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Nagelkerke SCJ and Bakx R
- Subjects
- Humans, Child, Digestive System Surgical Procedures methods, Short Bowel Syndrome surgery
- Abstract
Competing Interests: None declared.
- Published
- 2024
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3. Long-Term Outcome of Children with Short Bowel Syndrome Treated with a Modification of the STEP Technique Avoiding Mesenteric Defect.
- Author
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Bueno J, García-Martínez L, Redecillas S, Segarra O, and López M
- Subjects
- Child, Follow-Up Studies, Humans, Infant, Intestine, Small surgery, Retrospective Studies, Treatment Outcome, Digestive System Surgical Procedures methods, Short Bowel Syndrome surgery
- Abstract
Background: The Serial Transverse Enteroplasty Procedure (STEP) Registry has reported a 47% success to achieve enteral autonomy in pediatric short bowel syndrome (SBS). We have performed the STEP with a technical modification (MSTEP) consisting in stapler application without mesenteric defects that can also be applied to the duodenum. Our experience with this technique is described., Materials and Methods: In this study, 16 children with SBS underwent MSTEP (2005-2019). Indications were nutritional autonomy achievement ( n = 11, with duodenal lengthening in 5/11) and bacterial overgrowth treatment ( n = 5)., Results: With a median follow-up of 5.8 years (0.7-13.7 years), 5 of 11 (45%) patients achieved enteral autonomy, 4 of them with duodenal lengthening. Four of four who preserved > 50% colon, while only one of seven with < 50% of colon achieved enteral autonomy ( p < 0.05). After redo procedures, three of four attained enteral autonomy. Thus, 8 of 11 (73%) progressed to enteral autonomy, including all with duodenal lengthening. One child, already parenteral nutrition free, died due to central line sepsis. All the patients from the bacterial translocation group improved their metabolic/nutritional status, but one required subsequent enterectomy of the lengthened intestine due to multiple ulcers in the staple lines., Conclusion: The effectiveness of MSTEP to achieve enteral autonomy seems similar to the classical STEP. It can be applied to the duodenum. The retained colon length may influence the post-STEP enteral autonomy achievement., Competing Interests: None declared., (Thieme. All rights reserved.)
- Published
- 2022
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4. Bowel Lengthening Procedures in Children with Short Bowel Syndrome: A Systematic Review.
- Author
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Nagelkerke SCJ, Poelgeest MYV, Wessel LM, Mutanen A, Langeveld HR, Hill S, Benninga MA, Tabbers MM, and Bakx R
- Subjects
- Child, Child, Preschool, Humans, Infant, Infant, Newborn, Parenteral Nutrition, Prospective Studies, Retrospective Studies, Treatment Outcome, Digestive System Surgical Procedures methods, Intestines surgery, Short Bowel Syndrome surgery, Short Bowel Syndrome therapy
- Abstract
Introduction: The aims of the study are to systematically assess and critically appraise the evidence concerning two surgical techniques to lengthen the bowel in children with short bowel syndrome (SBS), namely, the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP), and to identify patient characteristics associated with a favorable outcome., Materials and Methods: MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception till December 2019. No language restriction was used., Results: In all, 2,390 articles were found, of which 40 were included, discussing 782 patients. The median age of the patients at the primary bowel lengthening procedure was 16 months (range: 1-84 months). Meta-analysis could not be performed due to the incomparability of the groups, due to heterogeneous definitions and outcome reporting. After STEP, 46% of patients weaned off parenteral nutrition (PN) versus 52% after LILT. Mortality was 7% for STEP and 26% for LILT. Patient characteristics predictive for success (weaning or survival) were discussed in nine studies showing differing results. Quality of reporting was considered poor to fair., Conclusion: LILT and STEP are both valuable treatment strategies used in the management of pediatric SBS. However, currently it is not possible to advise surgeons on accurate patient selection and to predict the result of either intervention. Homogenous, prospective, outcome reporting is necessary, for which an international network is needed., Competing Interests: L.M.W. reports other from Shire, outside the submitted work. M.A.B reports other from Shire, Norgine, Tramedico, Allergan, DAnone, Novalac, Friesland Campina, and Abbot, outside the submitted work. M.M.T. reports grants from Baxter Healthcare Company, outside the submitted work. S.H. reports grants and other from Shire/Takeda, outside the submitted work., (Thieme. All rights reserved.)
- Published
- 2022
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5. European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome.
- Author
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Dariel A, Faure A, Martinez L, Morini F, Pini Prato A, Friedmacher F, and Coste ME
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- Adaptation, Physiological, Child, Europe, Female, Humans, Intestine, Small physiopathology, Male, Surveys and Questionnaires, Pediatrics methods, Short Bowel Syndrome surgery
- Abstract
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., Competing Interests: None declared., (Thieme. All rights reserved.)
- Published
- 2021
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6. Institutional Experience with Spiral Intestinal Lengthening and Tailoring.
- Author
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Coletta R, Aldeiri B, and Morabito A
- Subjects
- Female, Humans, Infant, Intestine, Small blood supply, Intestine, Small surgery, Male, Parenteral Nutrition methods, Retrospective Studies, Treatment Outcome, Intestine, Small pathology, Plastic Surgery Procedures methods, Short Bowel Syndrome surgery
- Abstract
Aim: The aim of this study was to report our initial experience using spiral intestinal lengthening and tailoring (SILT) technique in selected cases of short bowel syndrome (SBS)., Materials and Methods: We analyzed all cases of SBS underwent SILT in our unit since the introduction of the procedure in 2012. We retrospectively analyzed patients' demographics, pre- and postprocedure bowel length, surgical complications, and postoperative parenteral nutrition (PN) requirements. Data were compared using independent samples, Mann-Whitney's U -test., Results: Five children with SBS underwent SILT between 2012 and 2017. Median age at procedure was 8.3 months (4.5-16). Preoperative small bowel length measured a median of 22 cm (17.5-50) with a median diameter of 4 cm (3.5-4.6). SILT allowed a median increase in length of 56% (10-15 cm; p = 0.03) and tailoring of the dilated segment providing a reduction in diameter of 50% (4.3-2.1 cm; p = 0.01). No major complications related to SILT were encountered and none of the children required further surgical intervention following a median follow-up of 26 months (14.5-41). Interestingly, we observed a significant reduction of PN requirement at 6 months ( p = 0.008) associated with liver function preservation during the follow-up period., Conclusion: In our experience, SILT is a promising adjunct in the surgical management of SBS. It can be used to tailor and lengthen mildly dilated segments of the bowel where other procedures are technically challenging, with a view to reduce the risk of intestinal failure associated liver disease and thereby improving chances for quality survival. Further studies are needed to investigate long-term outcomes of SILT in pediatric SBS., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2019
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7. Pediatric Intestinal Rehabilitation and Transplantation Registry: Initial Report from a European Collaborative Registry.
- Author
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Totonelli G, Tambucci R, Boscarelli A, Hermans D, Dall'Oglio L, Diamanti A, d'Aische ADB, Pakarinen M, Reding R, Morini F, Bagolan P, and Fusaro F
- Subjects
- Child, Preschool, Europe, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Kaplan-Meier Estimate, Male, Parenteral Nutrition, Total, Registries, Retrospective Studies, Short Bowel Syndrome therapy, Treatment Outcome, Intestines transplantation, Short Bowel Syndrome surgery
- Abstract
Introduction: Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database., Materials and Methods: Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994-2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off)., Results: In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0-38.0); median birth weight was 2,440 g (IQR: 1,700-2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0-92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients., Conclusion: We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2018
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8. Ultrashort Bowel Syndrome Outcome in Children Treated in a Multidisciplinary Intestinal Rehabilitation Unit.
- Author
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Dore M, Junco PT, Moreno AA, Cerezo VN, Muñoz MR, Galán AS, Sánchez AV, Prieto G, Ramos E, Hernandez F, Martínez LM, and Santamaria ML
- Subjects
- Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Intestine, Small transplantation, Male, Parenteral Nutrition, Total, Retrospective Studies, Short Bowel Syndrome mortality, Short Bowel Syndrome surgery, Spain, Treatment Outcome, Hospital Units, Patient Care Team, Short Bowel Syndrome rehabilitation
- Abstract
Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed. Results Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1-217) months and had undergone 3 (1-6) previous laparotomies that left 5 (0-9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4-175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14-715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil). Conclusion A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality., (Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2017
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9. Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome.
- Author
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Dore M, Junco PT, Andres AM, Sánchez-Galán A, Amesty MV, Ramos E, Prieto G, Hernandez F, and Lopez Santamaria M
- Subjects
- Child, Child, Preschool, Combined Modality Therapy, Female, Follow-Up Studies, Humans, Infant, Intestines transplantation, Male, Parenteral Nutrition, Total, Reoperation, Retrospective Studies, Short Bowel Syndrome mortality, Short Bowel Syndrome surgery, Treatment Outcome, Digestive System Surgical Procedures methods, Short Bowel Syndrome rehabilitation
- Abstract
Intestinal failure (IF) requires a multidisciplinary management based on nutritional support, surgical and medical rehabilitation, and transplantation. The aim of this study is to review our experience with surgical rehabilitation techniques (SRTs: enteroplasty, Bianchi, Serial Transverse Enteroplasty Procedure [STEP]) in patients with short bowel syndrome (SBS) and poor prognosis due to complex abdominal pathology. We performed a single-center retrospective study of patients with IF evaluated for intestinal transplantation in the Intestinal Rehabilitation Unit who underwent an SRT. Nonparametric tests were used for statistical analysis.A total of 205 patients (107 males/98 females) with mean age of 25 ± 7 months were assessed for IF. A total of 433 laparotomies were performed on 130 patients including intestinal resection, enteroplasties, adhesiolysis, and transit reconstruction. SRT were performed in 22 patients: 12 enteroplasties, 8 STEPs, and 4 Bianchi procedures. All patients were parenteral nutrition (PN) dependent with different stages of liver disease: mild (13), moderate (5), and severe (4). The adaptation rate for patients who underwent enteroplasty, STEP, and Bianchi were 70, 63, and 25%, respectively, although the techniques are not comparable. Overall, intestinal adaptation was achieved in nine (41%) patients, and four (18%) patients showed significant reduction of PN needs. One child did not respond to SRT and did not meet transplantation criteria. The remaining eight (36%) patients were included on the waiting list for transplant: four were transplanted, two are still on the waiting list, and two died. Better outcomes were observed in milder cases of liver disease (mild 77%, moderate 40%, severe 25%) (p < 0.05). Conversely, a trend toward a poorer outcome was observed in cases with ultrashort bowel (p > 0.05). One patient required reoperation after a Bianchi procedure due to intestinal ischemia and six needed further re-STEP or adhesiolysis procedure several months later. The median follow-up was 62 (3-135) months. Overall mortality was 19%, and was due to end-stage liver disease and/or central venous catheter-related sepsis. SRT led to intestinal adaptation in a significant number of patients with poor prognosis SBS referred for intestinal transplantation. However, SRT requires a multidisciplinary evaluation and should be attempted only in suitable cases. Careful assessment and optimal surgical timing is crucial to obtain a favorable outcome., (Georg Thieme Verlag KG Stuttgart · New York.)
- Published
- 2016
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10. The impact of longitudinal intestinal lengthening and tailoring on liver function in short bowel syndrome.
- Author
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Reinshagen K, Zahn K, Buch Cv, Zoeller M, Hagl CI, Ali M, and Waag KL
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- Adult, Alanine Transaminase blood, Female, Humans, Liver Cirrhosis enzymology, Liver Cirrhosis pathology, Liver Cirrhosis physiopathology, Male, Parenteral Nutrition, Retrospective Studies, Short Bowel Syndrome complications, Short Bowel Syndrome mortality, Short Bowel Syndrome physiopathology, Short Bowel Syndrome therapy, Intestines surgery, Liver Cirrhosis etiology, Short Bowel Syndrome surgery
- Abstract
Introduction: Short bowel syndrome is a functional or anatomic loss of major parts of the small bowel leading to severe malnutrition. The limiting factor for the survival of these patients remains parenteral nutrition-related liver damage leading to end-stage liver failure. Longitudinal intestinal lengthening and tailoring (LILT) has been proven to enhance peristalsis, to decrease bacterial overgrowth and to extend the mucosal contact time for the absorption of nutrients. The aim of this study was to show the impact of LILT on the development of parenteral nutrition-related liver damage., Patients and Methods: A cohort of 55 patients with short bowel syndrome managed with LILT in our institution between 1987 and 2007 was retrospectively reviewed. LILT was performed at a mean age of 24 months (range 4 - 150 months). Mean follow-up time was 83.76 months (range 5 - 240 months). We obtained reliable data from 31 patients with regard to liver enzymes and function parameters in blood samples before LILT and at the present time. Liver biopsy was performed in 14 patients prior to LILT., Results: Liver enzymes ALAT (mean 121 U/l), ASAT (mean 166 U/l) and bilirubin (mean 2.49 mg/dl) were elevated preoperatively in 27/31 children. After the lengthening procedure, ALAT (mean 50 U/l), ASAT (mean 63 U/l) and bilirubin (mean 1.059 mg/dl) normalized except in 5 of 8 patients who could not be weaned from parenteral nutrition after LILT. Liver function parameters such as the international normal ratio (INR) were slightly elevated in 5/31 patients. Albumin was generally low, probably due to parenteral nutrition. Liver biopsy was performed in 14 patients preoperatively, showing 4 patients with low-grade, 6 patients with intermediate and 4 patients with high-grade fibrosis. End-stage liver disease with cirrhosis was an exclusion criterion for LILT. All patients with liver fibrosis showed a normalization of liver enzymes when they were weaned from parenteral nutrition. But patients with higher grade liver fibrosis tend to develop more complications perioperatively., Conclusion: After LILT, all patients with liver fibrosis who could be weaned from parenteral nutrition showed a normalization of liver enzymes. Preoperative liver biopsy is mandatory in order to differentiate reversible liver fibrosis from end-stage liver disease. A higher grade of liver fibrosis and elevated INR has been shown to be a sensitive parameter for peri- and postoperative complications.
- Published
- 2008
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11. Long-term cognitive functions in neonatal short bowel syndrome patients.
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Huang J, Cai W, Tang Q, Feng Y, Tao Y, Wang Y, and Wu J
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- Adolescent, Child, Child, Preschool, Cognition Disorders diagnosis, Digestive System Abnormalities surgery, Digestive System Surgical Procedures adverse effects, Female, Follow-Up Studies, Humans, Intestine, Small surgery, Male, Nutritional Status, Parenteral Nutrition, Severity of Illness Index, Short Bowel Syndrome surgery, Treatment Outcome, Wechsler Scales, Cognition Disorders etiology, Digestive System Abnormalities complications, Short Bowel Syndrome complications
- Abstract
Objective: The aim of the study was to evaluate the long-term effects of neonatal short bowel syndrome on cognitive functions during development., Subjects and Methods: Nine patients diagnosed with short bowel syndrome during the neonatal period were enrolled in this study. Their medical records were reviewed; anthropometric measurements and blood tests were assayed; IQ tests (the Chinese versions of WAIS-R, WPPSI-R and WISC-R) were performed depending on their age, and a BSID assessment was carried out in those patients less than 4 years old., Results: Eight of 9 patients were followed up except for one patient who died in a car accident at the age of three. All patients had been weaned off parenteral nutrition for more than 2 years. The average residual small bowel length was 58.1 cm (range 35-70 cm), and the mean parenteral nutrition (PN) duration was 73.1 days (43-147 days). The mean duration of the period without PN was 7.4 years (range 2.1-17.1 years). Weight, height and BMI for age were normal in 7 children except for 1 child, who was overweight. Hemoglobin and albumin concentrations were normal in all 8 patients. Evaluation of cognitive development showed normal results for all 8 patients while a verbal/performance discrepancy was found in 2., Conclusion: Patients with neonatal SBS who were weaned off PN for more than 2 years were found to have normal growth and cognitive development during this long-term follow-up. There was no evidence for a strong correlation between SBS and nutritional/cognition disorder. Longer term and controlled studies with a larger sample size are warranted.
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- 2008
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12. Experience of 49 longitudinal intestinal lengthening procedures for short bowel syndrome.
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Hosie S, Loff S, Wirth H, Rapp HJ, von Buch C, and Waag KL
- Subjects
- Child, Child, Preschool, Digestive System Surgical Procedures, Humans, Infant, Short Bowel Syndrome surgery
- Abstract
Patients, Methods and Results: Forty-nine patients with a mean age of 25 months underwent a longitudinal intestinal lengthening procedure for short bowel syndrome (SBS) in our institution. Indications for the operation were dependence on parenteral nutrition in spite of adequate conservative management. The small bowel was lengthened from a mean of 27 cm to a mean of 51 cm. There was no intraoperative mortality. The following early complications occurred in our early series: ischemia of a short bowel segment of 2 cm, requiring resection in two patients, insufficiency of the longitudinal anastomosis in two patients and an intra-abdominal abscess in one. Four of 9 non-survivors died of liver failure and 3 of sepsis. Follow-up showed that 19 patients were weaned from parenteral nutrition after a mean of 9.1 months. Long-term complications encountered were dismotility with malabsorption due to bacterial overgrowth caused by progressive dilatation of the bowel, d-lactic acidosis, cholelithiasis and urolithiasis., Conclusions: A longitudinal intestinal lengthening procedure is an effective and safe surgical approach for SBS, provided it is performed in time, the patient's preoperative condition is optimized and technical surgical details are taken into account.
- Published
- 2006
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13. Poor effect of glutamin and human-EGF on autologic-allotopic transplanted ileum mucosa.
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Beiler HA, Steinorth J, and Zachariou Z
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- Animals, Colon surgery, Dogs, Humans, Ileum drug effects, Intestinal Mucosa transplantation, Short Bowel Syndrome surgery, Sodium Chloride pharmacology, Stents, Therapeutic Irrigation, Epidermal Growth Factor pharmacology, Glutamine pharmacology, Ileum transplantation, Intestinal Mucosa drug effects
- Abstract
Introduction: After establishing a new method of autologic-allotopic ileum mucosa transplantation as a therapy for short-bowel syndrome, the effects of glutamine and human epithelial growth factor (human EGF) on the transplanted ileum mucosa were evaluated., Methods: Ileum mucosa was transplanted in 28 young beagle dogs in a demucosed vascularised transverse colon segment. The ileum mucosa was kept in place with silicone stents in all animals. Eight animals of the control group were irrigated with saline solution. In the second group with 10 animals, 100 mg/kg glutamine were administered daily in the lumen. The 10 animals of the third group were treated with 25 microg/kg human EGF per day subcutaneously and irrigated with saline solution. 4 weeks later, histological specimens were harvested from the colon coat-ileum mucosa complex, the normal ileum and normal colon. Lumen diameter, percentage ileum mucosa uptake as well as mucosa and colon muscle coat thickness were evaluated., Results: In all groups, the diameter of the lumen was larger than 10 mm after fixation, due to the silicone stent. The group with glutamine irrigation showed the largest lumen diameter. A complete mucosa lining of the inner surface of the colon muscle coat was achieved in none of the animals. The highest percentage of ileum mucosa uptake was found in the group with glutamine irrigation. In most animals, the transplanted ileum mucosa was markedly thinner than normal ileum mucosa. Only in the group with glutamine irrigation did we find two animals with nearly normal mucosa thickness. The longitudinal muscle of the transplanted colon coat was thicker in all three groups compared to normal colon. There were no differences in thickness of the circular muscle in all animals compared to normal colon., Conclusions: Silicone stents maintain a lumen after autologic-allotopic ileum mucosa transplantation. However, additional irrigation with glutamine, as well as treatment with human EGF subcutaneously could not provide a complete lining of the colon muscle coat with transplanted ileum mucosa. A modification of the operative procedure is necessary to achieve a colon muscle coat that is completely lined with ileum mucosa before the absorptive capacity of the transplanted colon coat-ileum mucosa complex can be evaluated.
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- 2004
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14. Congenital short-bowel and malrotation: clinical presentation and outcome of six affected offspring in three related families.
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Erez I, Reish O, Kovalivker M, Lazar L, Raz A, and Katz S
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- Adolescent, Child, Colon abnormalities, Consanguinity, Female, Humans, Infant, Infant, Newborn, Intestine, Small abnormalities, Male, Pedigree, Prognosis, Radiography, Rotation, Short Bowel Syndrome diagnostic imaging, Short Bowel Syndrome surgery, Short Bowel Syndrome congenital
- Abstract
Newborns with congenital short small bowel associated with malrotation and intestinal dysmotility have a uniformly bad prognosis. However, few long-term survivors have been reported, suggesting that the disorder is not invariably fatal. The majority of cases previously reported were familial. We report on six affected siblings in three related families. The aim of this report is to assess the mode of inheritance, the expression of this disorder, and to point to the correlation between the onset of gastrointestinal symptoms and the outcome.
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- 2001
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15. Surgical approach to the short-bowel syndrome: procedures to slow intestinal transit.
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Thompson JS
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- Humans, Short Bowel Syndrome physiopathology, Anastomosis, Surgical methods, Gastrointestinal Motility, Intestine, Small surgery, Short Bowel Syndrome surgery
- Abstract
Procedures designed to slow intestinal transit should be applied cautiously in patients with near adequate remnant length and demonstrated rapid transit. They should be considered after maximum adaptation has occurred. A reversed intestinal segment appears to be efficacious in the short term. Colon interposition and intestinal valves might also have merit in selected patients but clinical experience is limited. Unfortunately, these procedures are applicable to only a small proportion of patients with SBS.
- Published
- 1999
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16. What do children look like after longitudinal intestinal lengthening.
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Waag KL, Hosie S, and Wessel L
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- Child, Child, Preschool, Follow-Up Studies, Humans, Infant, Treatment Outcome, Anastomosis, Surgical methods, Intestine, Small surgery, Short Bowel Syndrome surgery
- Abstract
The longitudinal intestinal lengthening, described by Bianchi in 1980, has been shown to be effective in improving intestinal function, absorption and transit time in patients with short-bowel syndrome. We report the long-term results of 18 survivors of a series of 25 intestinal lengthening procedures performed since 1984. Mean age of the patients was 18 months (range of 5 to 52 months), mean follow-up 6 years (0.9 to 12 years). Parenteral nutrition was progressively reduced in all patients and discontinued after 1 to 10 months (mean 5.1 months). Frequently encountered problems during long-term follow-up are hyperphagia, hyponatremia and hypochloremia, metabolic acidosis, including D-lactic acidosis, cholelithiasis and urolithiasis, gastro-esophageal reflux, dystrophy and symptoms caused by secondary dilatation of the lengthened bowel loops: a protruding abdomen, enteral stasis, leading to constipation or diarrhea with bacterial overgrowth. Overall performance has been acceptable in 13 out of 18 patients. Longitudinal intestinal lengthening is effective enabling patients with short-bowel syndrome to be weaned from parenteral nutrition, allowing for long-term survival. However, it is only one step on a long and difficult way. Multiple problems have to be searched for and adequately dealt with to achieve an acceptable and future worth living.
- Published
- 1999
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17. Ultra-short-bowel syndrome is not an absolute indication to small-bowel transplantation in childhood.
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Gambarara M, Ferretti F, Bagolan P, Papadatou B, Rivosecchi M, Lucchetti MC, Nahom A, and Castro M
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- Child, Child, Preschool, Female, Humans, Ileocecal Valve, Infant, Infant, Newborn, Intestine, Small transplantation, Liver Diseases etiology, Male, Prognosis, Reoperation, Sepsis etiology, Short Bowel Syndrome complications, Treatment Outcome, Intestine, Small surgery, Short Bowel Syndrome surgery
- Abstract
Short-bowel syndrome (SBS) either in adults or in children is considered as an indication to small-bowel transplantation (SBTx), particularly in its most severe form with a residual bowel length below 20 cm. Among factors likely to worsen the prognosis, more recent reports also indicate the number of surgical interventions, early onset sepsis and early development of liver disease. We report six cases of ultra-short-bowel syndrome followed from birth to verify the importance of various prognostic factors. In our case series, the male sex is predominating (5:1). Intestinal resection was indicated in 3 patients for multiple intestinal atresias, in 2 for volvulus and in 1 for necrotizing enterocolitis. The length of intestine remaining was invariably less than 20 cm and 2 patients had a preserved ileocecal valve. In most cases, more than 50% of the colon remained. The number of abdominal operations ranged from 1 to 4. In almost all cases (5 of 6), sepsis and hepatopathy developed early. Our experience suggests that rather than depending on the length of intestine remaining or the presence of the ileocecal valve, the prognosis of patients with the extreme-short-bowel syndrome depends on recurrent neonatal onset sepsis and early onset liver impairment. In addition, our case review shows that the extreme-short-bowel syndrome is not necessarily an indication for bowel transplantation.
- Published
- 1999
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18. Autogenic allotopic small-bowel mucosa transplantation in beagles. A new perspective for treatment of small-bowel syndrome?
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Zachariou Z, Daum R, Beiler HA, and Gorgas K
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- Animals, Colon surgery, Dogs, Feasibility Studies, Ileum pathology, Ileum transplantation, Intestinal Mucosa pathology, Transplantation, Autologous, Intestinal Mucosa transplantation, Short Bowel Syndrome surgery
- Abstract
There is no standard treatment for the short-bowel syndrome. The aims of surgical therapy are based on: slowing the intestinal transit, increasing the absorbing intestinal area and small-bowel transplantation. Searching for a new surgical treatment we developed an alternative for increasing the absorbing small-bowel area by means of autogenic allotopic small-bowel mucosa transplantation in beagle dogs. In young animals we isolated the transverse colon leaving the blood supply intact. Colonic continuity was reestablished and two abdominal stomata were performed at the ends of the isolated transverse colon. A week thereafter the colonic mucosa of the isolated transverse colon was surgically removed and autologous small-bowel mucosa was transplanted in the demucosed colon. The animals were then sacrificed 2, 4 and 6 weeks after transplantation and the colon-coat-ileal-mucosa complex (CIC) was histologically examined. The ileal mucosa could be transplanted in the demucosed colon showing histological characteristics of ileal mucosa. The circular muscle of the colon coat developed a hypertrophy which was present even 6 weeks after transplantation. In this study we could show that autogenic allotopic small-bowel mucosa transplantation is feasible in beagle dogs and may prove a novel method of small bowel expansion in cases of small-bowel syndrome.
- Published
- 1998
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19. The Bianchi procedure in a patient with jejunal atresia.
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Flageole H, Adolph VR, Sigalet DL, Fecteau A, Drouin E, and Laberge JM
- Subjects
- Humans, Infant, Newborn, Jejunum surgery, Male, Parenteral Nutrition, Total, Short Bowel Syndrome etiology, Short Bowel Syndrome therapy, Intestinal Atresia surgery, Jejunum abnormalities, Short Bowel Syndrome surgery
- Abstract
While small-bowel transplantation remains an option for the management of short-bowel syndrome (SBS), every effort must be made to optimize the function of the native bowel. This report describes a patient with SBS who dramatically improved after a Bianchi procedure. The patient was born with type IIIb jejunal atresia, complicated by volvulus of the terminal ileum. He was left with 40 cm of small bowel and his ileocecal valve. During the first 20 months of his life, he was always hospitalized except for 3 months. He was on parenteral nutrition (TPN) and suffered multiple episodes of central line sepsis. Massive small-bowel dilatation caused a functional bowel obstruction with bacterial overgrowth, and villous atrophy of the jejunal mucosa was discovered on biopsies done by colonoscopy. In March 1994, we elected to proceed with a Bianchi procedure. Thirty cm of jejunum were divided longitudinally. During the following year, his enteral tolerance steadily improved to the point where TPN could be discontinued. We conclude that pediatric surgeons should not hesitate to use this procedure in the management of SBS.
- Published
- 1997
- Full Text
- View/download PDF
20. An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures.
- Author
-
Sacher P and Stauffer UG
- Subjects
- Animals, Blood Proteins metabolism, Body Weight physiology, Dilatation, Pathologic physiopathology, Dilatation, Pathologic surgery, Female, Humans, Infant, Intestinal Obstruction physiopathology, Intestinal Obstruction surgery, Potassium blood, Protein-Energy Malnutrition physiopathology, Protein-Energy Malnutrition surgery, Short Bowel Syndrome physiopathology, Swine, Swine, Miniature, Treatment Outcome, Disease Models, Animal, Short Bowel Syndrome surgery
- Abstract
Intestinal lengthening is considered to be one of the most effective surgical interventions to improve the deleterious sequelae of short-bowel syndrome. To assess the efficiency of bowel-lengthening procedures in short-bowel syndrome, an animal model was created in minipigs that show malnutrition, weight loss and small-bowel dilatation. Group 1 consisted of three animals with a 95% distal intestinal resection leaving 15 cm of ileum. Group 2 comprised six animals with a 95% distal intestinal resection leaving only 5 cm of ileum, and group 3 consisted of three animals with a 95% distal intestinal resection leaving 5 cm of ileum and construction of a distal small-bowel stenosis by temporary banding. In group 1 there was a significant increase in length of residual bowel at terminal laparotomy at 10 weeks and a significant difference for protein and potassium. No bowel dilatation occurred and no animal lost weight. Group 2 animals showed a continuous loss of weight after the intestinal resection and significantly different values in animal profile were found at 10 weeks but no significant difference in length or in diameter of the residual bowel. In group 3, all animals demonstrated a significant dilatation of the residual small bowel at 1 week when the stenosis was resected and bowel continuity restored. We conclude that in piglets a 95% distal intestinal resection leaving 5 cm of ileum leads to a short-bowel syndrome with malnutrition and weight loss, but no intestinal dilatation. To provoke an additional intestinal dilatation, creation of a distal stenosis is mandatory. Animals of group 3 represent a suitable model to assess the efficacy of short/bowel lengthening procedures.
- Published
- 1997
- Full Text
- View/download PDF
21. Expansion of mucosal surface by intestinal patching in experimental cases of short bowel.
- Author
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Sigge W
- Subjects
- Animals, Dogs, Female, Intestinal Absorption physiology, Intestinal Mucosa pathology, Intestine, Small pathology, Intestine, Small surgery, Muscle, Smooth pathology, Short Bowel Syndrome pathology, Suture Techniques, Intestinal Mucosa transplantation, Muscle, Smooth transplantation, Short Bowel Syndrome surgery
- Abstract
Young female beagles underwent either 85% resection of the small intestine; simultaneous 85% resection and patching of incised ideal remnant with colon serosa or with muscularis propria from transposed colon; or muscularis propria patching of ileum without resection of the small intestine. Patch areas were created in antiperistaltic position of participating bowel loops. After 20 weeks 50% of serosal patch and 34% of muscularis propria patch were overgrown by neomucosa. After 40 weeks 75% of the serosal patch was covered by neomucosa. Serosal patch area at necropsy 20 weeks after 85% resection of small intestine was 60% of the initial size; muscularis propria patch showed 44% of its primary area, but was only 12% of the initial surface area without bowel resection. Increases in resorptive surface consisted of neomucosa and an impressive adaptive elongation of short bowel, that was intensified by the simultaneous growth of the neomucosa.
- Published
- 1995
- Full Text
- View/download PDF
22. Intraperitoneal fetal small bowel transplantation as therapy for the short bowel syndrome: an animal experimental study.
- Author
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Kellnar S and Rattanasouwan T
- Subjects
- Animals, Animals, Newborn, Body Weight physiology, Disease Models, Animal, Fetal Tissue Transplantation pathology, Gestational Age, Intestinal Absorption physiology, Intestine, Small embryology, Intestine, Small pathology, Rats, Rats, Inbred Lew, Short Bowel Syndrome pathology, Fetal Tissue Transplantation methods, Intestine, Small transplantation, Short Bowel Syndrome surgery
- Abstract
After demonstration of the morphologic integrity of fetal rat intestinal transplant and in vitro evidence of both digestive and resorptive function in the transplanted small bowel, in another experimental approach we tried to assess intestinal function in vivo. In this experiment, fetal intestinal grafts were placed in host animals and allowed to mature for 4 weeks. Then we resected the whole small bowel of the host from the ligament of Treitz to the ileo-cecal valve, afterwards interposing the matured fetal intestinal segment. A control group of animals (their small bowels were resected but they received no graft replacement) showed massive weight loss. The animals with fetal small bowel transplant replacement thrived. This shows that in the rat model fetal small bowel, previously transplanted into a host, can be an actual functioning substitute for normal small bowel.
- Published
- 1992
- Full Text
- View/download PDF
23. Is intestinal lengthening effective in treating extreme short bowel syndrome?
- Author
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Sacher P and Stauffer UG
- Subjects
- Colostomy, Enteral Nutrition, Humans, Infant, Newborn, Jejunostomy, Short Bowel Syndrome congenital, Intestine, Small surgery, Short Bowel Syndrome surgery
- Abstract
Our report concerns a child with short bowel syndrome who had 20 cm of small intestine distal to the pylorus (duodenum and jejunum) which remained after subtotal intestinal resection; the resection was necessitated by intrauterine volvulus with intestinal necrosis as a result of gastroschisis. In addition, only 25 cm of the colon remained. Despite continuous enteral nutrition with a semi-elementary diet and conservative therapy, it was not possible to provide within six months at least occasionally half of the caloric intake required on an enteral basis. In such cases treatment is usually discontinued. As final alternative to a small intestine transplantation, we conducted an operation to lengthen the intestine (method according to Bianchi) when the patient was 8 months old. The massively dilatated jejunum section of the intestine was lengthened from 20 cm to 37 cm. Postoperatively enteral caloric intake could be increased from 11 kJ/kg/m to 20 kJ/kg/m. Unfortunately, at 14 months of age, the child died from hepatobiliary complications arising from aggravation of the cholestasis and acidosis. This case shows, nevertheless, that intestinal lengthening had a positive influence on intestinal resorption.
- Published
- 1991
- Full Text
- View/download PDF
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