Serial Transverse Enteroplasty Research Articles

Reversed Intestinal Segment Revisited

BackgroundReversed segments (RS) designed to slow intestinal transit and improve absorption in patients with short bowel syndrome (SBS) are performed infrequently, and patient selection remains controversial. Our aim was to evaluate patient selection and outcome for RS in SBS patients. MethodsSixteen adult patients underwent RS among 520 SBS patients. All patients had remnant length >80 cm and rapid intestinal transit. Ten patients had a colon remnant and 12 had an ostomy. SBS was present for 8 to 150 months prior to RS. ResultsRS was performed either alone (n = 9) or concurrently with ostomy closure (n = 5) or creation (n = 2). There were 3 postoperative complications and no deaths. Three patients had bacterial overgrowth. One required repair of an ileocolonic stricture. Two reversed segments were taken down 12 months and 96 months later. Two patients subsequently underwent serial transverse enteroplasty (STEP) procedures, and 1 had isolated intestinal transplant. Fourteen (88%) required parenteral nutrition (PN) pre-operatively and 2 (12%) had intractable diarrhea. Nine (56%) patients improved and 7 (44%) remained on PN or had persistent intractable diarrhea. Patients with a successful outcome were similar to those without improvement with respect to ostomy takedown, duration of SBS, Crohn's disease, intestinal length, a colon remnant, anatomy, and transit time. ConclusionsReversed segments significantly benefit one half of selected SBS patients who have rapid transit but adequate remnant length. Outcome in individual patients remains difficult to predict. Subsequent operation is frequently required. This procedure is applicable to a small proportion of SBS patients.

Neonatal Serial Transverse Enteroplasty (STEP): Case Report

Case ReportGastroschisis is the most frequent congenital abdominal wall defect. When associated with intestinal atresia (complex gastroschisis), short bowel syndrome may occur. Complicated gastroschisis is the most frequent cause of short bowel syndrome in our series. The serial transverse enteroplasty procedure has been used to lengthen the bowel and achieve intestinal rehabilitation in patients with dilated gut. The use of this technique in the newborn period, for tailoring the bowel while preserving absorptive mucosa, has been recently described. We present a video showing the surgical treatment of an intestinal obstruction produced by a complex intestinal atresia in a newborn baby in whom a primary closure of a gastroschisis had been done at birth. During laparotomy at the 21st day of life, a type IVa intestinal atresia was found, associated with a colonic stenosis. Proximal dilated jejunum was tailored with a serial transverse enteroplasty procedure, as shown in the video. End-to-end jejunal-ileal anastomosis was performed. Postoperative entero-cutaneous fistula occurred and was treated with vacuum-assisted therapy. Enteral feedings were initiated at 15 days after surgery. Parenteral nutrition was withdrawn at 30 days. After 16 months follow-up, actual weight was 8.7 kg (percentile 10% to 25%). The patient remained on full enteral feedings. ConclusionsIn cases of intestinal atresia, short bowel syndrome, and proximal dilated bowel, we propose an aggressive surgical approach to preserve bowel mucosal surface while tailoring the jejunal loop to improve motility. The serial transverse enteroplasty procedure is an acceptable alternative to tailoring methods that resect a segment of the bowel wall.

Ten-Year Experience With Bowel Transplantation at Seoul St. Mary's Hospital

A retrospective review of intestinal transplantation (ITx) at Seoul St. Mary's Hospital was made by collecting clinical data over the past 10 years. Fifteen consecutive cases from 2004 were analyzed. Five children and 10 adults (6 months to 69 years of age) were included. Primary diseases in adults included 4 mesenteric vessel thromboses, 2 strangulations, and 1 each of visceral myopathy, malignant gastrointestinal stromal tumor (GIST), mesenteric lymphangiectasis, and injury. Pediatric cases involved 2 Hirschsprung disease, 2 visceral myopathy, and 1 necrotizing enterocolitis. Three of 7 stomas were closed using a serial transverse enteroplasty procedure before transplantation. The ITx were performed using 3 living-donor Itx, 12 deceased-donor ITx, 14 isolated Itx, and 1 modified multivisceral transplantation. Daclizumab, basiliximab, alemtusumab, or basiliximab with rabbit antithymocyte globulin (rATG) was used for the induction; tacrolimus monotherapy was used as the basic maintenance immunosuppressant; and m-TOR inhibitor was used for renal dysfunction patients. Seven cases of acute cellular rejection were treated with rATG. Three cases of antibody-mediated rejection were treated with rituximab alone or with rituximab and bortezomib combination. There were 4 cases of early mortality within 6 months after Itx. Causes of death were declamping shock, cardiac tamponade with acute cellular rejection, dysmotility, and sepsis. Surgical complications consisted of 1 feeding jejunostomy displacement, and a minor leakage at a colo-colostomy site. One-year survival of the patient and graft was 73.33% (Kaplan–Meier survival curve). Although the total number of ITx is small, its social impact has been remarkable in changing the related laws and reimbursement policy in Korea.

Tiger Food for Short Bowel: Two Cases.

Dear Sir, Short Bowel Syndrome (SBS) refers to a critical reduction in the length of the intestine, leading to chronic diarrhoea, dehydration, malnutrition, weight loss and growth failure [1]. SBS is clinically evident when the total available length of small bowel is <200 cm and commonly caused by resection [2]. The nonsurgical options for management of SBS are: (a) total parenteral nutrition (TPN), which is costly, invasive and un-physiological; (b) para-sympathomimetics drugs, which cause dilatation of the bowel; (c) proton pump inhibitors and others. TPN in addition can lead to thrombosis of canulated vessel, bowel atrophy and sepsis. The surgical options are described to slow intestinal transit, taper dilated bowel segments in children in proximal to atretic segment and intestinal lengthening procedures such as serial transverse entero-plasty (STEP) and Bianchi’s procedure [3]. The third option is small bowel transplant. The standard dietary management of SBS with colon is 50-60% of caloric intake with complex carbohydrate, 20-30% with fat and 20-30% with protein. Complex carbohydrate in small bowel will cause diarrhoea as these will take long time and has to cover more distance to break down and undergo absorption. These reach colon and work as osmotic agent causing diarrhoea and hence simple sugars (mono/diasacharides) are used as these get absorbed in stomach and in available proximal small bowel. Diet rich in fat and protein cause delay in gastric emptying, higher gastric acid and bile and pancreatic juice secretion. Whereas, protein part get broken down and get absorbed in proximal jejunum and fat in the terminal ileum. The presence of Ileo Caecal valve (ICV) delays the discharge of fat rich fluid and bile acid. Living beings are divided into carnivorous (Flesh eating), herbivorous (plant eating) and omnivorous (eat flesh and plant). The herbivorous has got the longest small bowel, the carnivorous the shortest and omnivorous remain in the middle. Loss of a part of small bowel in human makes the bowel equivalent to carnivorous, where carnivorous diet (tiger food) is appropriate is the novel thinking.

Surgical Rehabilitation Techniques in Children with Poor Prognosis Short Bowel Syndrome.

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.

Success of Serial Transverse Enteroplasty in an Adult With Extreme Short Bowel Syndrome: A Case Report

Since its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty has been increasingly used as the surgical treatment of choice for children with refractory short bowel syndrome, but there have been few reports about the adult patients. This report describes the case of a 71-year-old man with a short bowel after distal gastrectomy with Billroth II reconstruction for gastric cancer, followed by extensive intestinal resection. The serial transverse enteroplasty operation was performed and lengthened the small intestine from 49 to 67 cm. The patient tolerated the procedure well and weaned off total parenteral nutrition. Liver function also improved. This case shows that the serial transverse enteroplasty procedure increases intestinal length. This procedure should be considered a surgical option for adult patients with extreme short bowel syndrome.

Duodenal lengthening in short bowel with dilated duodenum

Although duodenal dilatation occurs in children with short bowel syndrome (SBS) facilitating dismotility and bacterial overgrowth, the duodenum has been an untouchable intestinal segment for lengthening procedures owing to its close relationship with bilio-pancreatic structures and blood supply shared with the pancreas. Three children (age range, 0.5–7 years) with SBS and dilated duodenum underwent a novel surgical procedure of duodenal lengthening combined with a technical modification of serial transverse enteroplasty (STEP). Pre-STEP, jejunum length was 5, 35 and 45cm, respectively. Duodenal lengthening was performed with sequential transverse applications of an endoscopic stapler on the anterior and posterior wall of the duodenum to avoid bilio-pancreatic structure injury. Two patients underwent 3 duodenal firings (stapler of 35mm) and the third 5 firings (stapler of 45mm). Duodenal firings were 17%, 21% and 83% of the total firings. ResultsNo surgical complications occurred. One patient developed transient episodes of D-lactic acidosis. Two patients (5 and 45cm) were weaned off parenteral nutrition at 12months post-surgery and the remaining patient´s (35cm) parenteral calorie requirements have decreased by 60%. ConclusionDuodenal lengthening is effective since it tailors and increases the absorptive surface of the duodenum, even in cases of extreme SBS.

New technique for intestinal lengthening -- from the idea to the clinical application

In severe short bowel syndrome, as a result of the natural adaptation, the bowel becomes overdilated, this interferes with the persitalsis and may lead to stasis, bacterial translocation and sepsis. At present two techniques are used to improve peristalsis. The Bianchi procedure is technically challenging, the Serial Transverse Enteroplasty (STEP) is easy however it results in an aphysiological ultrastructure altering the orientation of the muscle fibres. Our aim was to develop an easy technique, which does not alter intestinal muscular ultrastructure dramatically. The idea, Spiral Intestinal Lengthening and Tailoring (SILT), is based on a spiral shape incision of the intestine and retubularisation in a longer but narrower fashion. The feasibility and the effect on the muscular ultrastructure were tested on bowelsimulator and porcine intestine. The intramural microcirculation was checked with intravital microscopy. The outcome was assessed on minipigs (n = 6) than clinical application was commenced. SILT was feasible, did not change the orientation of muscle fibres significantly, did not compromised microcirculation, no surgical complication was noted when tailoring did not exceed 75%. The first clinical application was successful. SILT is a safe and easy technique and not altering the intestinal musculature significantly.

Improved tolerance for enteral nutrition after serial transverse enteroplasty (STEP) in infants and children with short bowel syndrome—A seven-year single-center experience

BackgroundSerial transverse enteroplasty (STEP) was designed to lengthen and taper the small intestine in patients with short bowel syndrome (SBS) and dilated small bowel. We hypothesized that tolerance for enteral nutrition (EN) improves after STEP. MethodsPatients who underwent STEP between March 2004 and January 2011 were identified. Candidates for STEP had radiographic evidence of dilated small bowel and either failed to advance EN or demonstrated deterioration in tolerance for EN. Clinical and nutritional data were analyzed pre- and post-STEP. EN was defined as the percentage of calories administered enterally. Statistical analysis employed the signed rank test with significance assumed when p<0.05. ResultsTwenty STEPs were performed at a median age of 13.7months. Median pre-STEP bowel length was 30cm with a median increase in bowel length of 42%. Five patients achieved enteral autonomy at a median of 6.5months post-STEP. EN increased in 75%, while 25% exhibited unchanged or decreased EN post-STEP. In aggregate, median EN tolerance increased from 22% at one month pre-STEP to 61% at six months post-STEP (p=0.003). ConclusionsThe STEP is an effective adjunct in the treatment of patients with intestinal failure. While enteral autonomy is eventually possible in some patients, improved enteral tolerance can be achieved in a majority of cases.

PO-0912 Short Bowel State: Does Autologous Gastrointestinal Reconstruction Reduce Catheter-related Blood Stream Infections?

Background and aims Catheter-related blood stream infections (CRBSI) occur frequently in Short Bowel Syndrome (SBS) children on parenteral nutrition (PN). Central venous catheter (CVC) complication and complete loss of central venous access are indication for intestinal transplantation. Autologous gastrointestinal reconstruction surgery (AGIR) is mandatory in any chronically PN-dependent patient when there is substantial bowel dilation to reduce bacterial translocation. We reviewed patients who underwent lengthening surgery and calculated the rate of CRBSI pre and post surgery. Methods PN dependent children with SBS were identified. Inclusion criteria were CVC for PN administration pre and post-operatively, CVC removed after weaned off PN and having gained enteral autonomy. CRBSI episodes were defined as temperature above 38.0 °C, along with positive blood culture microbiological infection from the CVC. Results Nineteen patients were identified (male n = 13). Median gestation was 35 (33.5–36.5) completed weeks and birth weight 2080 g (1725–2374). Ten patients underwent tapering enteroplasty, eight Longitudinal Intestinal Lengthening and Tailoring (LILT) procedure, and one Serial Transverse Enteroplasty (STEP) procedure. Median duration of PN was 5.3 months (2.9–6.6) pre and 9.0 months (4.2–10.9) post surgery. A total of 115 septic episodes were confirmed (70 prior to surgery and 45 post surgery). The total rate of catheter related sepsis was significantly lower after AGIR compared to before it (p = 0.016). Conclusions CRBSI frequency in PN dependent patients with dilated bowel reduces after AGIR. AGIR appears associated with significantly reduced frequency of CRBSI in PN dependent children with bowel dilatation. These findings warrant further exploration in larger, preferably controlled studies.

Favorable Outcome With Teduglutide Treatment of a Young Woman With Long-Standing Intestinal Failure/Short Bowel Syndrome (SBS) With No Remaining Central Venous Access

Introduction: Describe treatment with teduglutide (rDNA origin) in a 23-year-old patient with longstanding intestinal failure/SBS due to congenital jejunal atresia Case Report: Our patient is a 23-year-old female born with jejunal atresia, who experienced intestinal failure due to SBS as a neonate. She has absence of the corpus callosum, seizure disorder, and neurodevelopmental delay. Her gastrointestinal history is significant for eosinophilic colitis, enteritis, gastro-esophageal reflux disease, and malabsorption due to SBS. She had a history of multiple prior central venous catheters. One year prior to this report, due to feeding intolerance and partial bowel obstruction, she underwent surgery with extensive lysis of adhesions, Ladd’s procedure, and serial transverse enteroplasty procedure (STEP). After surgery, we attempted to wean her off parenteral nutrition (PN). Following a catheter-associated central venous thrombosis, she was placed on treatment with fondaparinux to prevent progression of thrombosis. However, the catheter would no longer infuse, and she had no further central venous access for PN. Enteral feedings were increased with elemental formula to meet her estimated energy needs. Her daily fluid needs were partially met with ORS and additional peripheral intravenous fluids (IVF) to compensate for high fecal losses and electrolyte and mineral deficiencies. She developed pneumatosis intestinalis (PI) on this regimen. Once the PI resolved, she was placed on a low-carbohydrate enteral formula and oral rehydration solution (ORS) via G-tube with supplemental fluid, electrolyte, magnesium (Mg), and phosphorus (Phos.) administered via peripheral vein, based on stool output, serum electrolyte, and mineral levels. Peripheral IVs were changed frequently and with great difficulty by the hospital IV team. Due to lack of central venous access and inability to transition the patient from IVF, electrolytes, and minerals, she was started on teduglutide 0.05 mg/kg daily subcutaneously. She was monitored in the hospital for adverse effects, daily weights, fluid intake and output, serum electrolytes, and minerals. She experienced recurrence of PI and elevated lipase levels that resolved within 4-5 days with conservative management. Six weeks after starting teduglutide, the patient no longer required additional IVF, IV electrolyte, or mineral supplementation. After 9 weeks of teduglutide, she continues to tolerate G-tube feeds and ORS with normal serum electrolytes, Mg, and Phos. without IV supplementation at home.Table 1: Results

Infectious complications following serial transverse enteroplasty in infants and children with short bowel syndrome

BackgroundSerial transverse enteroplasty (STEP) lengthens and tapers dilated small bowel in patients with short bowel syndrome (SBS). Previous reports document encouraging outcomes with regard to tolerance for enteral nutrition (EN) and complications appear related to the re-operative nature of many cases and to the presence of multiple staple lines. However, infectious complications following STEP have not been examined. Since infections, especially catheter-related blood stream infections (CRBSI), are considered detrimental in infants and children with SBS, we sought to define the frequency and outcomes of peri-operative infections associated with STEP. MethodsAll children with SBS who underwent a STEP between 2004 and 2012 were indentified and their medical records were reviewed. Patients were considered candidates for a STEP if they had dilated small bowel and failure to advance enteral nutrition. For the purpose of this study, infections occurring within a 14-day period after STEP were considered procedure-related and were the focus of the study. ResultsA total of 18 patients underwent 23 STEP procedures. Primary diagnoses included intestinal atresia, gastroschisis, necrotizing enterocolitis, and midgut volvulus. After the STEP, eight patients (35%) developed CRBSI, three developed wound infections, and two had urinary tract infections. Organisms isolated from either blood, wound or urine cultures included gram-positive cocci, gram-negative rods, and yeast. Perioperative antibiotics were administered in all cases with cefoxitin (43%) and piperacillin/tazobactam (30%) being most common. Neither antibiotic appeared superior in reducing the incidence of CRBSI. In three patients with persistent bacteremia despite adequate antibiotic therapy, a 74% ethanol lock resulted in negative blood cultures in all cases. Only one central venous catheter required replacement acutely for persistent fungemia. ConclusionSTEP can improve enteral tolerance. In this fragile patient population, however, STEP carries a documented infectious burden. The optimal antibiotic prophylaxis and the role of ethanol locking in patients undergoing STEP require further study.

Serial transverse enteroplasty to facilitate enteral autonomy in selected children with short bowel syndrome.

BackgroundSerial transverse enteroplasty (STEP) was first described in 2003 as a method for lengthening and tapering of the bowel in short bowel syndrome. The aim of this multicentre study was to review the outcome of a Swedish cohort of children who underwent STEP.MethodsAll children who had a STEP procedure at one of the four centres of paediatric surgery in Sweden between September 2005 and January 2013 were included in this observational cohort study. Demographic details, and data from the time of STEP and at follow-up were collected from the case records and analysed.ResultsTwelve patients had a total of 16 STEP procedures; four children underwent a second STEP. The first STEP was performed at a median age of 5·8 (range 0·9–19·0) months. There was no death at a median follow-up of 37·2 (range 3·0–87·5) months and no child had small bowel transplantation. Seven of the 12 children were weaned from parenteral nutrition at a median of 19·5 (range 2·3–42·9) months after STEP.ConclusionSTEP is a useful procedure for selected patients with short bowel syndrome and seems to facilitate weaning from parenteral nutrition. At mid-term follow-up a majority of the children had achieved enteral autonomy. The study is limited by the small sample size and lack of a control group. Good results in selected children

Surgical options to enhance intestinal function in patients with short bowel syndrome.

This review will provide the practitioner with an understanding of the spectrum of nontransplant surgical options for managing patients with short bowel syndrome (SBS). Intestinal lengthening procedures are a promising therapy to allow autonomy from parenteral nutrition. The recently described serial transverse enteroplasty is an effective procedure that is easier to perform and has similar outcomes to the more standard longitudinal lengthening procedure described by Bianchi. There are several surgical options for management of the SBS, including construction of intestinal valves or reversed intestinal segments, interposition of segments of colon, or intestinal lengthening procedures. The choice of technique is dictated by the patient's underlying pathophysiology and includes such factors as intestinal transit time, length of remnant bowel, presence of intact colon, and degree of small bowel dilation. Nontransplant surgical interventions are important adjuncts to the elimination of parenteral nutrition dependence and need for intestinal transplantation.

Update on surgical therapies for intestinal failure.

Parenteral nutrition enables long-term survival in patients with intestinal failure; however, it is associated with life-threatening complications necessitating alternative techniques of management to enable weaning parenteral nutrition and minimizing parenteral nutrition-associated comorbidities. This review aims at discussing the indications and techniques most commonly utilized for surgical management in a short gut. Surgical therapy for short gut has evolved and undergone refinement in the past 30-40 years with the older procedures being largely abandoned and replaced by newer and refined techniques. Lengthening surgeries (longitudinal intestinal lengthening and tapering and serial transverse enteroplasty) are the most commonly applied procedures in a dilated bowel with intestinal transplantation option reserved as a last resort for recurrent life-threatening central venous line infections, progressive loss of central venous access, and development of progressive liver disease. Management of intestinal failure requires a multidisciplinary approach to optimize intestinal rehabilitation and overall patient outcome. Although intestinal transplantation remains an excellent option for patients with severe life-threatening complications, autologous intestinal reconstruction appears to remain the better overall option.

Su1749 Intestinal Anastomotic Ulcers in Children With Short Bowel Syndrome and Anemia Detected by Capsule Endoscopy

Anastomotic ulcers are a known cause of anemia in children with a history of intestinal resection. The etiology of these ulcers may be secondary to ischemia or small bowel bacterial overgrowth (SBBO). Surgical resection may be required in the setting of anemia requiring multiple blood transfusions. Colonoscopy can be used to diagnose these ulcers. However, following serial transverse enteroplasty procedures (STEP), the area of involvement may be difficult to visualize with standard endoscopic techniques.

Surgical Management of Short Bowel Syndrome

For patients with short bowel syndrome (SBS), surgery can play an important role in preventing, mitigating, and, in some cases, reversing intestinal failure (IF). During intestinal resection, bowel length should be conserved to the fullest extent possible to avoid dependence on parenteral nutrition (PN). Bowel salvage may be improved by initially preserving tissue of questionable viability and later reevaluating during "second-look" procedures. Once the patient is stabilized, ostomy reversal and recruitment of distal unused bowel should be prioritized whenever feasible. Following progression to IF, surgical management of SBS depends on the symptoms and anatomical characteristics of the individual patient. For carefully selected patients with rapid intestinal transit and dilated bowel, longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures may provide benefit. Outcomes following STEP and LILT are generally similar, and the choice between these procedures may rest on surgeon preference. For patients with rapid intestinal transit in the absence of bowel dilation, segmental reversal of the small bowel may reduce PN requirements. Intestinal transplantation is the standard of care for patients in whom intestinal rehabilitation attempts have failed and who are at risk of life-threatening complications of PN. Because patients awaiting isolated intestine transplant show increased survival compared with patients awaiting combined intestine-liver transplant, early referral of appropriate patients, before the development of advanced liver disease, is critical to enhancing patient outcomes.

Gastrointestinal bleeding as a complication of serial transverse enteroplasty

PurposeSerial transverse enteroplasty (STEP) lengthens and tapers bowel in patients with intestinal failure. Evaluation and treatment of serious late gastrointestinal bleeding (GIB) in three STEP patients are described. MethodsPatients participating in an interdisciplinary intestinal rehabilitation program were reviewed to identify those who underwent STEP and had GIB requiring transfusion. ResultsOf 296 patients, 23 underwent STEP, and 3 (13%) had subsequent GIB requiring transfusion. Diagnoses were vanishing gastroschisis/atresia, malrotation/atresia, and gastroschisis.. STEP was performed at ages 3–5months, using 5–15 stapler-firings with an increase in mean bowel length from 39 to 62cm. GIB was diagnosed 5–30months post-op and resulted in 1–7 transfusions per patient. Endoscopy demonstrated staple-line ulceration in two patients and eosinophilic enterocolitis in the third. All were treated with enteral antibiotics, sulfasalazine, and luminal steroids. Those with ulcers responded to bowel rest, and the patient with eosinophilic enterocolitis stabilized with luminal steroids. In all three, hemoglobin levels improved despite persistent occult bleeding. ConclusionsSignificant GIB is a potential late complication of STEP. Endoscopy identified the underlying source of GIB in all three patients. A combination of enteral antibiotics, anti-inflammatory medications, and bowel rest was effective in treating post-STEP GIB, without the need for additional bowel resection.

Autologous Intestinal Reconstructive Surgery to Reduce Bowel Dilatation Improves Intestinal Adaptation in SBS

To the Editor: Almond et al (1) reported 8 of 26 patients referred with intestinal failure (IF) from short bowel syndrome with >40 cm of dilated residual bowel. The study aimed to establish whether surgery to reduce dilatation could improve outcome; timing of surgery varied from approximately 3 months to 3 years (median approximately 10 months). Strikingly, 7 of 8 patients developed severe IF-associated liver disease (IFALD) (2) (mean peak bilirubin 250 μmol/L) preoperatively, yet liver function tests normalised after surgery, and 7 of 8 patients were weaned from parenteral nutrition. These interesting observations should be interpreted with caution. This seems to be an extremely unusual group of patients because, as the authors stressed, all were totally dependent on parenteral nutrition for nutrient requirements. It appears that despite “hepatosparing” strategies, severe IFALD (bilirubin >100 μmol/L) (2) developed in all but 1. Underlying condition and interventions were heterogeneous, including serial transverse enteroplasty procedure, longitudinal intestinal lengthening, and tapering enteroplasty. Although the authors considered small intestinal bacterial overgrowth to be a key factor in failure of adaptation, no diagnostic tests were performed (3), and no information was given about empirical preoperative treatment with antibiotics or prokinetics. This article brings into focus a difficult group of patients with bowel dilatation, but raises questions about the optimal nature and timing for surgery, difficulty in reliably diagnosing small intestinal bacterial overgrowth, and whether aggressive medical management of early IFALD, including prokinetics and antibiotics, may have facilitated enteral feeding and gut adaptation, while preventing the progression of liver disease. Robust research will be needed to provide definitive answers to these questions.

First STEPs: Serial transverse enteroplasty as a primary procedure in neonates with congenital short bowel

BackgroundSince its introduction as an alternative intestinal lengthening technique, serial transverse enteroplasty (STEP) has been increasingly used as the surgical treatment of choice for patients with refractory short bowel syndrome (SBS). While primary STEP for the treatment of congenital conditions was proposed in the original description of the procedure, emphasis was placed on a delayed or staged approach to these patients. To date, a comprehensive review of the outcomes from this sub-population has not been reported by the International STEP Data Registry. MethodsA retrospective review of the International STEP Data Registry was performed to identify all patients who underwent STEP as a primary operative procedure for the treatment of congenital SBS. Changes in pre- and post-STEP values were assessed using paired t-tests with significance set at p<0.05. Data are presented as mean ± standard deviation. ResultsFifteen patients underwent primary STEP for congenital SBS between September 1, 2004, and April 10, 2012. Thirteen patients had follow-up information available. Causes of congenital SBS included closing gastroschisis, small bowel atresia, and midgut volvulus. Twelve patients had pre- and post-STEP bowel measurements taken. Average pre- and post-STEP bowel lengths were 32 ± 16 cm and 47 ± 22 cm, respectively. Intestinal length was increased by a mean of 15 ± 12 cm for a relative small bowel length increase of 50.4 ± 27.3% (p<0.001). Only one patient required an ostomy at the time of primary STEP. A second patient required a temporary ostomy at 3months of age that was later closed. There was one death from intestinal failure associated liver disease (IFALD). Another patient experienced IFALD progression and required liver and intestinal transplantation. The most commonly reported complication following primary STEP was obstruction or bowel re-dilatation requiring additional operative interventions. Nine patients underwent second STEP procedures under these circumstances. Eight patients remain dependent on parenteral nutrition, while three patients achieved enteral autonomy. ConclusionsPrimary STEP is a feasible and safe surgical option for the treatment of congenital conditions resulting in SBS. Primary STEP establishes early bowel continuity, creates intestinal length from congenitally dilated bowel, and appears to obviate the need for interval stomas and their associated loss of bowel length in neonates with congenital SBS. However, with recent changes in SBS management emphasizing intestinal rehabilitation, additional studies are needed to assess the long-term impact on intestinal adaptation of STEP performed in the neonatal period prior to adoption of this technique.