Bowel Lengthening Procedures Research Articles

Current practice and future perspectives in the treatment of short bowel syndrome in children—a systematic review

Short bowel syndrome (SBS) is a malabsorption disorder of the intestine, which leads to an inadequate alimentary supply. A number of therapeutic approaches are already in use, but research advances may provide new options in the future. The purpose of this paper was to provide an overview of the established therapeutic approaches together with a discussion of the future perspectives in the treatment of patients with SBS. We review those studies dealing with the treatment of SBS patients and discuss both surgical and non-surgical approaches together with tissue engineering. A systemic review of Medline-cited studies dealing with current practice and future perspectives in the treatment of short bowel in children was performed. Surgical approaches, non-surgical approaches, and tissue engineering which was used in the treatment of SBS were analyzed. Among the surgical approaches, the bowel lengthening procedures and small bowel transplantation are prevalent. Stimulants are most important concerning non-surgical approaches. Tissue engineering seems to be more experimental and was also evaluated. The treatment of SBS patients remains very complex. It is eminent to find the best therapeutic option for each patient and to individualize and modify the different possible types of applied techniques frequently.

Pediatric Intestinal Failure in the Intestinal Transplant Era: A Retrospective Review of 272 Infants

Background. Pediatric intestinal failure (IF) is a rare but devastating condition defined as the inability of the intestine to support growth and development without supplemental parenteral nutrition (PN). A retrospective study was performed to provide the foundation for the first prospective study of IF in North America. Methods. PIFCon includes 14 sites with multi-disciplinary IF management teams; 9 are also intestinal transplant (ITx) centers. Infants 60 continuous days for IF were included. Demographic, clinical, biochemical, nutritional and outcome data were abstracted from medical charts. Values are presented as median (25th, 75th percentiles) or as (n, %). Enteral autonomy (EA) was defined as the discontinuation of PN for > 3 consecutive mo. Results. 272 infants with a gestational age of 34 wks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 mo (11.2, 40.9). The majority were male (156, 57%) and Caucasian (204, 75%). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), intestinal atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), intestinal aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). The cumulative proportions achieving EA at 1 and 3 yr were 0.31 and 0.43, respectively. Commonly used medications were oral antibiotics (207, 76%), H2 blockers (187, 69%), and PPIs (156, 57%). Breast milk was given to 52 (19%); the cumulative probabilities of oral solid feeding were .67 at 1 yr and .85 at 3 yr. 25 different infant formulas were used as initial diet; 40 different formulas were used overall. 3.3 new catheter related blood stream infections occurred per person year (PPY) on PN and 0.2 intestinal bleeding episodes PPY of observation. 28 bowel lengthening procedures were performed. Cumulative probability of survival (CPS) was 0.84 at 1 yr and 0.74 at 3 yr. 58 died before and 10 died after ITx. Following study entry, time to death without ITx was 8.2 mo (4.8, 12.6). 137 were alive without ITx when the last data were abstracted from the medical record. 60 received ITx, the cumulative incidence of ITx at 1 and 3 years was .087 and .229, respectively. The CPS 1 year after ITx was 0.84. Conclusions. Children with IF suffer a high rate of mortality and morbidity. EA may require years to achieve. Substantial practice variability is noted among sites. Improved medical, nutritional, and surgical management of IF may reduce time on PN, mortality and need for ITx. Grant support: NIH/NIDDK R21 DK081059-01

The experience of a regional pediatric intestinal failure program: Successful outcomes from intestinal rehabilitation

Background The aim of this study was to evaluate the clinical experience of a regional multidisciplinary intestinal failure program for children established in 2005. Methods Data were collected from a prospective internal database. Univariate analyses were performed to compare pre- and post-treatment outcomes. Median values are reported. Results Forty-nine children were referred at an age of 7 months. Remnant small bowel length was 29 cm. With follow-up of 14 months, overall patient survival was 88%. Thirteen bowel-lengthening procedures were performed, thereby increasing small bowel length from 83 to 132 cm ( P < .05). Enteral autonomy was achieved in 22 patients (45%), and the caloric requirement for parenteral nutrition was decreased from 100% to 41% ( P < .01). Conjugated bilirubin was reduced from 4.1 to 0 mg/dL ( P < .05). Conclusion A multidisciplinary approach to pediatric intestinal failure that prioritizes intestinal rehabilitation can achieve successful enteral feeding advancement, improved liver function, and excellent survival in intermediate-range follow-up.

Repeat STEP procedure to establish enteral nutrition in an infant with short bowel syndrome

The serial transverse enteroplasty (STEP) procedure is a safe and successful way to lengthen the small bowel in patients with short bowel syndrome. However, postoperative dilatation of the intestine may occur, which induces bacterial overgrowth and malabsorption leading to liver failure. We describe the case of an infant boy with short bowel syndrome caused by jejunal atresia requiring the STEP procedure twice. The first STEP improved the liver function, and the second STEP allowed 80% of the total calorie intake to be tolerated enterally. One should not hesitate to perform a second STEP if after the initial bowel lengthening procedure the patient develops small bowel dilatation that interferes with enteral nutrition.

Bowel-lengthening procedures for patients with short bowel syndrome

Bowel-lengthening procedures for patients with short bowel syndrome

Interdisciplinary Management of Pediatric Intestinal Failure: A 10-Year Review of Rehabilitation and Transplantation

Management of children with intestinal failure is optimized by interdisciplinary coordination of parenteral and enteral nutrition support, medical management of associated complications, surgical lengthening procedures, and intestinal transplantation. Three hundred eighty-nine pediatric patients have been referred to our center for interdisciplinary assessment of intestinal failure since 1996 (median age = 1 year; range 1 day–28.8 years). Factors predictive of weaning from parenteral nutrition without transplantation included increased mean bowel length for patients with gastroschisis (44 vs. 23 cm, p < 0.05) and atresia (35 vs. 20 cm, p < 0.01) and lower mean total bilirubin for patients with NEC (6.1 vs. 12.7 mg/dL, p < 0.05). Others were also more likely to survive if referred with a lower mean total bilirubin (NEC, 7.9 vs. 12.7 mg/dL, p < 0.05; pseudo-obstruction, 2.3 vs. 16.3 mg/dL, p < 0.01). Patients weaned from parenteral nutrition by 2.5 years after referral achieved 95% survival at 5 years vs. 52% for those not weaned. Bowel lengthening procedures were performed on 25 patients. Eight subsequently weaned from parenteral nutrition without transplantation. Aggressive medical and nutritional intervention along with early referral, intestinal lengthening procedures, and intestinal transplantation in children with intestinal failure dependent on parenteral nutrition can result in the achievement of enteral autonomy and improved survival.

Intestinal Failure-Associated Liver Disease: Management and Treatment Strategies Past, Present, and Future

Liver disease is estimated to develop in 40% to 60% of infants on long-term parenteral nutrition (PN) secondary to intestinal failure. The etiology of intestinal failure-associated liver disease (IFALD) is multifactorial with primary contributors including prematurity, sepsis, deficiencies or hepatotoxicities of infusates, and lack of enterally stimulated bile flow. IFALD treatment strategies have historically emphasized the following: choleretics such as ursodeoxycholic acid, bowel decontamination for small bowel bacterial overgrowth, bowel tapering and lengthening procedures, and manipulations of PN prescriptions (e.g., cycling). This review highlights current and proposed novel treatment and management strategies for IFALD. These include a discussion of state-of-the art central line care practices, novel bowel-lengthening procedures such as serial transverse enteroplasty, isolated liver transplant for IFALD, probiotics and glutamine for bowel decontamination, hormonal therapies for achieving bowel adaptation, and a discussion of new PN formulations that may have emerging roles in IFALD.

Management of intestinal failure

Intestinal failure (IF) occurs when the body is unable to sustain its energy and fluid requirements without support, due to loss of functional small bowel. Prolonged IF is seen after large intestinal resection and described as short bowel syndrome (SBS). The hallmark of the management is parental nutrition (PN), which is costly and may be associated with the well-recognized problems of parental nutrition associated liver disease (PNALD) and line related sepsis. Cessation of PN at the earliest possible stage is desirable but for this enteral autonomy has to be achieved first. Intestinal adaptation occurs when the remaining gut goes through morphological changes increasing its absorptive capacity. Factors such as intraluminal nutrients, gastrointestinal secretions and hormones facilitate adaptation. Enteral feeds are a potent stimulant to adaptation and should be started as soon as the clinical situation permits. Some drugs are thought to increase intestinal adaptation. These include glutamine, growth hormone and glucagon like peptide- 2, but there is a paucity of pediatric data to guide their use. In some cases surgical bowel lengthening procedures can be performed to increase the absorptive surface area. An isolated liver transplantation may be required if the liver has sustained irreversible damage but intestinal autonomy seems achievable. When prolonged PN is either unsustainable or associated with unacceptable side effects, small bowel transplantation should be considered as a treatment option.

Growth and Nutritional Status in Infants With Short-Bowel Syndrome After the Serial Transverse Enteroplasty Procedure

The aim of this study was to describe the long-term nutritional outcomes of 4 patients with short bowel syndrome (SBS) who underwent a newly described bowel-lengthening procedure, the serial transverse enteroplasty (STEP) procedure. We performed a retrospective review of the medical records of 4 children who underwent STEP at 1 center. The primary outcome measure was the percentage of total dietary energy received by the enteral route before and after STEP. Other outcome measures were weight and height Z scores and body composition, as measured by arm anthropometric values. Four children with SBS were identified, all of whom had been dependent on parenteral nutrition since birth. The mean preoperative follow-up period was 234 days (range, 63-502 days), and the mean postoperative follow-up period was 362 days (range, 252-493 days). By using model-based mean estimates, the mean enteral nutrition intake was 48% preoperatively vs 62% postoperatively (P = .02). The model mean weight for age Z score increased by .7 SDs postoperatively (P = .01), and the model mean weight for height increased by .6 SDs (P < .0001). The percent standard mid-upper-arm circumference increased by 13.1% postoperatively (P = .03), and the percent standard triceps skinfold increased by 24.5% postoperatively (P < .0001). The STEP procedure was associated with improved growth and body composition among 4 children with SBS in the face of a decreasing need for parenteral nutrition. Among patients with refractory SBS and dilated small intestine, the STEP procedure is associated with improved clinical and nutritional outcomes in the first year after surgery.

Vanishing Bowel: A Therapeutic Challenge

"Vanishing bowel" has been rarely reported in the literature. This case and discussion are intended to describe the surgical, medical, and nutrition management of this unusual congenital abnormality. Of 3 reported cases surviving vanishing bowel, one had substantial residual small bowel, another had residual small bowel and bowel-lengthening procedure, and one received an intestinal liver transplant. The patient presented in this case had residual small bowel and lengthening procedure. The patient described is well as of October 2005 (age 3.5 years), still receiving nocturnal parenteral nutrition (PN), but is eating solid foods without significant malabsorption or abdominal distention. He has experienced modest weight gain over the past 2 months.

Serial transverse enteroplasty is associated with successful short-term outcomes in infants with short bowel syndrome

Background The serial transverse enteroplasty (STEP) has been shown to improve nutritional indices in an animal model of short bowel syndrome. The aim of this study was to review short-term surgical and nutritional outcomes in the first cohort of infants to undergo the STEP procedure at our institution. Methods All patients who underwent the STEP procedure during a 26-month period from February 2002 to March 2004 were reviewed. Paired t tests were used for comparisons between values pre-STEP and post-STEP ( P < .05 deemed significant). Data are expressed as mean and range. Results The STEP was performed on 5 patients, including 1 newborn. The STEP was used as a primary lengthening operation in 4 patients. Intestinal length was significantly increased in all patients with 18 (10-26) stapler applications. There were no perioperative complications and no evidence of intestinal leak or obstruction on routine postoperative contrast study. Nutritional follow-up was available on 3 subjects at 17 (11-26) months post-STEP. Percentage of enteral nutrition was significantly increased in these subjects ( P < .05). One subject was fully weaned from total parenteral nutrition 6 weeks after the STEP, and bilirubin in another patient with profound cholestasis who had been listed for liver–small bowel transplant normalized after the STEP. An additional patient, with established cirrhosis before operation, underwent successful liver–small bowel transplantation 8 months after intestinal lengthening. Conclusion The STEP procedure is a simple bowel-lengthening procedure with promising early surgical and nutritional outcomes. Further data from a multicenter registry are needed to demonstrate its long-term efficacy.

Serial transverse enteroplasty for short bowel syndrome: a case report

The patient is a 2-year-old boy born with gastroschisis and midgut volvulus that left him dependent on total parenteral nutrition (TPN). At 11 months of age, a Bianchi procedure was performed increasing the total length of bowel from 72 cm to 130 cm. Although he appeared to have sufficient bowel length, he continued to have malabsorption and could only tolerate 10% of his caloric requirement enterally. A barium study found significant dilatation of the lengthened small bowel. At 23 months, we performed a novel bowel lengthening procedure that we have reported previously in an animal model. The serial transverse enteroplasty (STEP) operation increased the 83 cm of dilated and previously lengthened bowel to 147 cm, making the total small bowel length 200 cm. The patient tolerated the procedure well and began to have semisolid bowel movements. Small intestinal absorptive capacity measured by D-xylose absorption showed a substantial increase from 5 to 12 mg/dL (normal range, >20), implying improved but not completely normal small bowel function. This case shows that the STEP procedure increases intestinal length, can be used after a prior Bianchi, and may result in improved intestinal absorptive capacity. The STEP procedure should be considered a surgical option for children with short bowel syndrome.

Serial transverse enteroplasty (STEP): A novel bowel lengthening procedure

Background/Purpose: Bowel lengthening may be beneficial for children with short bowel syndrome. However, current techniques require at least one intestinal anastomosis and place the mesenteric blood supply at risk. This study seeks to establish the technical principles of a new, simple, and potentially safer bowel lengthening procedure. Methods: Young pigs (n = 6) underwent interposition of a reversed intestinal segment to produce proximal small bowel dilation. Five weeks later the reversed segment was resected. Lengthening of the dilated bowel then was performed by serial transverse applications of a GIA stapler, from opposite directions, to create a zig zag channel. A distal segment of equal length served as an in situ morphometric control. Contrast radiologic studies were performed 6 weeks later, and the animals were killed. Statistical comparisons were made by paired t test with P less than.05 considered significant. Results: After bowel lengthening, all animals gained weight (66.7 ± 3.0 [SD] kg v 42.5 ± 3.5 kg; P <.001) and showed no clinical or radiologic evidence of intestinal obstruction. Intraoperatively, immediately after serial transverse enteroplasty, the intestine was substantially elongated (82.8 ± 6.7 cm v 49.2 ± 2 cm; P <.01). Six weeks after surgery, the lengthened intestinal segment became practically straight and, compared with the in situ control, remained significantly longer (80.7 ± 13.1 cm v 57.2 ± 10.4 cm; P <.01). There was no difference in diameter between these segments (4.3 ± 0.7 cm v 3.8 ± 0.4 cm; P value, not significant). Conclusions: Serial transverse enteroplasty (STEP) significantly increases intestinal length without any evidence of obstruction. This procedure may be a safe and facile alternative for intestinal lengthening in children with short bowel syndrome. J Pediatr Surg 38:425-429. Copyright 2003, Elsevier Science (USA). All rights reserved.

Teaching Case: The Appearance of Pica in a 5 1/2‐Year‐Old Child With Short Bowel Syndrome

W.M., a 5 1/2‐year‐old male with short bowel syndrome (SBS) secondary to an in utero midgut volvulus, had been dependent on total parenteral nutrition (TPN) in addition to gastrostomy tube (GT) feeds to maintain his nutritional status since birth. Bowel lengthening procedures performed at 15 and 30 months of age left W.M. with 82 centimeters of small bowel. Despite these procedures and what was presumed to be adequate nutrition, W.M.'s weight/age and height/age remained below the 5th percentile. With an observed improvement in oral intake at 5 1/2 years of age (2000 kcal/d), his GT was removed. TPN, which was continued for several months thereafter, was subsequently discontinued after a septic episode that resulted in severe neutropenia. However, family members soon began to report significant alterations in his behavior. An evaluation performed by a behavioral psychologist demonstrated no familial or developmental contributions to this new behavior pattern, which is termed pica in the psychological literature. Although a nutritional deficiency was suspected, serum levels of trace metals were found to be normal. With a reported increase in listlessness and lethargy at school, TPN was promptly reinstituted (approximately 6 months after it had been discontinued). The observed pica disappeared 1 week following the reinitiation of TPN therapy.

An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures: P. Sacher and U.G. Stauffer. Eur J Pediatr Surg 7:207–211, (August), 1997

An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures: P. Sacher and U.G. Stauffer. Eur J Pediatr Surg 7:207–211, (August), 1997

An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures.

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.

Jejunum is preferable for construction of a bianchi bowel-lengthening procedure in swine short bowel

This study compared the efficacy of a Bianchi bowel-lengthening procedure performed in residual ileum and jejunum of a 75% short bowel model. Eighteen female piglets underwent a 75% mid small bowel resection. After a 6-week period, animal weights were similar and pigs were randomly assigned to one of three treatment groups: (1) a control group receiving no further therapy; (2) a group receiving a Bianchi procedure in the residual jejunal segment; and (3) a group receiving a Bianchi procedure in the residual ileal segment. All were followed for a further 12 weeks. Jejunal Bianchi-treated short bowel animals demonstrated a greater final weight gain (78.8 +/- 4.9 kg) compared with nontreated short bowel (63.0 +/- 6.6 kg) and ileal Bianchi-treated short bowel groups (69.3 +/- 6.9 kg) in addition to a larger jejunal diameter. The increased weight gain in the jejunal Bianchi-treated group was not a consequence of initial bowel length, food intake, changes in bowel length, digestibility of nitrogen or fat, or nutritional status. Furthermore, kinetic constants for D-glucose absorption following 18 weeks of short-bowel syndrome demonstrated a lowered glucose maximal transport rate (Vmax) in animals with nontreated short bowel compared with sham-operated controls. Additionally, jejunal and ileal glucose Vmax was further lowered in the presence of a Bianchi procedure. We conclude that: (1) during short-bowel syndrome, body weight gain was significantly higher in animals when the Bianchi procedure was performed in jejunum; (2) the short-bowel syndrome decreased intestinal glucose absorption; and (3) the Bianchi procedure itself further impaired glucose transport.

Ion transport in rabbit ileal mucosa. I. Na and Cl fluxes and short-circuit current.

Ion transport in rabbit ileal mucosa. I. Na and Cl fluxes and short-circuit current.