An Overview of Short-Bowel Syndrome in Pediatric Patients: Focus on Clinical Management and Prevention of Complications.

Short-bowel syndrome (SBS) in pediatric age is defined as a malabsorptive state, resulting from congenital malformations, significant small intestine surgical resection or disease-associated loss of absorption. SBS is the leading cause of intestinal failure in children and the underlying cause in 50% of patients on home parental nutrition. It is a life-altering and life-threatening disease due to the inability of the residual intestinal function to maintain nutritional homeostasis of protein, fluid, electrolyte or micronutrient without parenteral or enteral supplementation. The use of parenteral nutrition (PN) has improved medical care in SBS, decreasing mortality and improving the overall prognosis. However, the long-term use of PN is associated with the incidence of many complications, including liver disease and catheter-associated malfunction and bloodstream infections (CRBSIs). This manuscript is a narrative review of the current available evidence on the management of SBS in the pediatric population, focusing on prognostic factors and outcome. The literature review showed that in recent years, the standardization of management has demonstrated to improve the quality of life in these complex patients. Moreover, the development of knowledge in clinical practice has led to a reduction in mortality and morbidity. Diagnostic and therapeutic decisions should be made by a multidisciplinary team that includes neonatologists, pediatric surgeons, gastroenterologists, pediatricians, nutritionists and nurses. A significant improvement in prognosis can occur through the careful monitoring of nutritional status, avoiding dependence on PN and favoring an early introduction of enteral nutrition, and through the prevention, diagnosis and aggressive treatment of CRSBIs and SIBO. Multicenter initiatives, such as research consortium or data registries, are mandatory in order to personalize the management of these patients, improve their quality of life and reduce the cost of care.

Optimizing Nutritional Strategies to Prevent Necrotizing Enterocolitis and Growth Failure after Bowel Resection.

Necrotizing enterocolitis (NEC), the first cause of short bowel syndrome (SBS) in the neonate, is a serious neonatal gastrointestinal disease with an incidence of up to 11% in preterm newborns less than 1500 g of birth weight. The rate of severe NEC requiring surgery remains high, and it is estimated between 20-50%. Newborns who develop SBS need prolonged parenteral nutrition (PN), experience nutrient deficiency, failure to thrive and are at risk of neurodevelopmental impairment. Prevention of NEC is therefore mandatory to avoid SBS and its associated morbidities. In this regard, nutritional practices seem to play a key role in early life. Individualized medical and surgical therapies, as well as intestinal rehabilitation programs, are fundamental in the achievement of enteral autonomy in infants with acquired SBS. In this descriptive review, we describe the most recent evidence on nutritional practices to prevent NEC, the available tools to early detect it, the surgical management to limit bowel resection and the best nutrition to sustain growth and intestinal function.

What is the optimal lipid emulsion for preventing intestinal failure-associated liver disease following parenteral feeding in a rat model of short-bowel syndrome?

PURPOSE: Composite lipid emulsion (CLE) has been used for intestinal failure-associated liver disease (IFALD) to compensate for the disadvantages of soybean oil lipid emulsion (SOLE) or fish oil lipid emulsion (FOLE). However, the influence of its administration is unclear. We evaluated the effects of these emulsions on IFALD using a rat model of the short-bowel syndrome. METHODS: We performed jugular vein catheterization and 90% small bowel resection in Sprague-Dawley rats and divided them into four groups: control (C group), regular chow with intravenous administration of saline; and total parenteral nutrition co-infused with SOLE (SOLE group), CLE (CLE group) or FOLE (FOLE group). RESULTS: Histologically, obvious hepatic steatosis was observed in the SOLE and CLE groups but not the FOLE group. The liver injury grade of the steatosis and ballooning in the FOLE group was significantly better than in the SOLE group (p < 0.05). The TNF-α levels in the liver in the FOLE group were significantly lower than in the SOLE group (p < 0.05). Essential fatty acid deficiency (EFAD) was not observed in any group. CONCLUSION: Fish oil lipid emulsion attenuated hepatic steatosis without EFAD, while CLE induced moderate hepatic steatosis. The administration of CLE requires careful observation to prevent PN-induced hepatic steatosis.

Remnant Intestinal Length Defines Intestinal Adaptation and Hepatic Steatosis: Two Zebrafish Models.

BACKGROUND: Short bowel syndrome (SBS) is a condition that results from inadequate intestinal absorptive capacity, usually after the loss of functional intestine. We have previously developed a severe model of SBS in zebrafish that demonstrated increased intestinal adaptation (IA) and epithelial proliferation in SBS zebrafish. However, many children with SBS do not have this extreme intestinal loss. Therefore, in this study, we developed a variation of this model to evaluate the effects of increasing intestinal length on IA and the complications of SBS. MATERIALS AND METHODS: After Institutional Animal Care and Use Committee approval, adult male zebrafish were assigned to three groups: sham (n = 30), S1-SBS (n = 30), and S3-SBS (n = 30). Sham surgery included ventral laparotomy alone. S1-SBS surgery consisted of laparotomy with creation of a proximal stoma at S1 (jejunostomy equivalent) and ligation at S4. S3-SBS surgery had stoma creation at S3 (ileostomy equivalent) and the same ligation. Fish were harvested at 14 d. Markers of IA were measured from proximal intestinal segments, and the liver was analyzed for development of hepatic steatosis. RESULTS: At 14 d, S3-SBS fish lost less weight than S1-SBS and had increased markers of IA compared with sham fish, which were decreased compared with S1-SBS fish. S3-SBS fish had decreased proximal intestinal inflammation compared with S1-SBS fish. S1-SBS fish developed extensive hepatic steatosis. Although S3-SBS fish have increased hepatic steatosis compared with sham fish, it is decreased compared with S1-SBS. CONCLUSIONS: Longer remnant intestine decreases the extent of IA, inflammation, and hepatic steatosis in a zebrafish model of SBS.

The surgical management of acute bowel ischemia in octogenarian patients to avoid Short Bowel Syndrome: a multicenter study.

BACKGROUND: This is a multicenter study performed in two Italian tertiary care centers: General Emergency Surgery Unit at St. Orsola University Teaching Hospital - Bologna and Department of Surgical Sciences at Umberto I University Teaching Hospital - Rome. The aim was to compare the results of different approaches among elderly patients with acute bowel ischemia. METHODS: Sixty-three patients were divided in two groups: 1) DSgroup- 28 patients treated in Vascular Unit and 2) GEgroup- 35 patients treated in Emergency Surgery Unit. RESULTS: Mean age was 80 years, significantly higher for the GEgroup (p<0.001). Gender was predominantly female in both groups, without statistical difference. Pre-operatively, laboratory tests didn't show any difference in white blood cell count, serum lactate levels or serum creatinine among patients, while increase of c-reactive protein was observed in DSgroup with significant difference (p<0.001). The Romamain cause of acute bowel ischemia was embolism in DSgroup (p=0.03) and vascular spasm in GEgroup (p<0.001). On CT scan, bowel loop dilation was present in 58.7% of patients without statistical difference in both groups. The time lapse from diagnosis to operation didn't show significant differences between two groups (mean 349.4 min). Pre-operative heparin therapy was administered in DSgroup more frequently (p< 0.001). Among DS patients, thrombectomy was the most frequent procedure (19 patients) associated with bowel resection in 9 cases. In GEgroup, 22 patients had an explorative laparotomy (p<0.001), 8 had a bowel resection with anastomosis and 5 a bowel resection plus stoma. A second look was required more significantly in DSgroup (p<0.002). Post-operative morbidity affected significantly GEgroup (p=0.02). The 3-day survival was significantly higher in the DSgroup (p< 0.001). At discharge 32 patients (50.8%) were alive, 21 in DSgroup (p< 0.001). Only one patient among both groups (1.6%) developed a short bowel syndrome. CONCLUSIONS: In octogenarian patients with acute bowel ischemia, surgery should be always pursued whenever the interventional radiology is not assessed as a viable option. Both groups of patients showed an excellent outcome in terms of avoiding a short bowel syndrome. A multidisciplinary management by a dedicated team could offer the best results to prevent large intestinal resections.

Surgical and medical approach to patients requiring total small bowel resection: Managing the "no gut syndrome".

BACKGROUND: Total resection of the jejunum and ileum, a rarely performed procedure, is indicated after mesenteric vascular events, trauma, or resection of abdominal neoplasms. We describe our recent experience with the operative and medical management of patients with "no gut syndrome." METHODS: We retrospectively reviewed 341 adult patients who were referred to our center between January 2013 and December 2016. RESULTS: Thirteen patients with a mean age of 42.5 years (range 17 to 66 years) underwent near total enterectomy. Indications for small bowel resection were vascular event (n = 5), intraabdominal fibroid/desmoid (n = 4), and trauma (n = 4). Foregut secretions were managed with duodenocolostomy (n = 5), tube decompression (n = 5), and end duodenostomy (n = 2). Duodenal stump was stapled off in 4 cases. One patient underwent a spleen-preserving duodenopancreatectomy combined with total enterectomy. Biliary secretions were managed with choledochocolostomy. All patients were discharged on full total parenteral nutrition infused over a 10- to 16-hour period. Average total parenteral nutrition volume and caloric requirement were 2,800 mL/day (range 2,000 to 4,000) and 1,774 Kcal/day (range 1,443 to 2,290), respectively. Patients who underwent duodenocolonic anastomosis received smaller TPN volume (33.8 vs 49.8 mL/kg). Ten patients (77%) required supplemental intravenous fluid. There were no intraoperative or perioperative deaths. One patient was lost to follow-up 2 months after operation. After a 20-month median follow-up (range 4 to 48 months), 9 patients are still alive (75%). All patients with duodenocolostomy remain alive (median follow-up 36.4 months). Three patients underwent uneventful isolated small bowel transplantation, and another 4 are being evaluated or are already listed for visceral transplantation. CONCLUSION: In summary, resection of the entire small bowel is feasible and can be a lifesaving procedure for a select group of patients. Long-term survival can be achieved in specialized centers. In addition, reestablishment of gastrointestinal tract continuity after total enterectomy appears to be the best option for postoperative fluid and electrolyte management.

Factors affecting poor nutritional status after small bowel resection in patients with Crohn disease.

In Crohn disease, bowel-preserving surgery is necessary to prevent short bowel syndrome due to repeated operations. This study aimed to determine the remnant small bowel length cut-off and to evaluate the clinical factors related to nutritional status after small bowel resection in Crohn disease.We included 394 patients (69.3% male) who underwent small bowel resection for Crohn disease between 1991 and 2012. Patients who were classified as underweight (body mass index < 17.5) or at high risk of nutrition-related problems (modified nutritional risk index < 83.5) were regarded as having a poor nutritional status. Preliminary remnant small bowel length cut-offs were determined using receiver operating characteristic curves. Variables associated with poor nutritional status were assessed retrospectively using Student t tests, chi-squared tests, Fisher exact tests, and logistic regression analyses.The mean follow-up period was 52.9 months and the mean patient ages at the time of the last bowel surgery and last follow-up were 31.2 and 35.7 years, respectively. The mean remnant small bowel length was 331.8 cm. Forty-three patients (10.9%) underwent ileostomy, 309 (78.4%) underwent combined small bowel and colon resection, 111 (28.2%) had currently active disease, and 105 (26.6%) underwent at least 2 operations for recurrent disease. The mean body mass index and modified nutritional risk index were 20.6 and 100.8, respectively. The independent factors affecting underweight status were remnant small bowel length ≤240 cm (odds ratio: 4.84, P < 0.001), ileostomy (odds ratio: 4.70, P < 0.001), and currently active disease (odds ratio: 4.16, P < 0.001). The independent factors affecting high nutritional risk were remnant small bowel length ≤230 cm (odds ratio: 2.84, P = 0.012), presence of ileostomy (odds ratio: 3.36, P = 0.025), and currently active disease (odds ratio: 4.90, P < 0.001).Currently active disease, ileostomy, and remnant small bowel length ≤230 cm are risk factors affecting the poor nutritional status of patients with Crohn disease after small bowel resection.

[Surgery in Crohn's disease]. / Chirurgie beim M. Crohn.

Surgical treatment is primarily used to treat complications of Crohn's disease but also to improve the quality of life. An adequate preoperative preparation including improvement of the nutritional status, weaning off or stopping immunosuppressive medication and preoperative drainage of abscesses can decrease the complication rate. With the exception of when neoplasia is present, bowel-sparing techniques (e. g. strictureplasty and limited resection) are now standard, which has resulted in a low risk of short bowel syndrome. The laparoscopic approach is possible for most indications even in the case of recurrent disease, in primary ileocecal resection the laparoscopic approach has been shown to be superior to the open approach. None of the available techniques for anastomotic reconstruction of the bowels has been shown to be superior. A drainage seton is a good option to retain the quality of life in complex fistulas and reconstructive repair should only be considered when the rectum is free from inflammation.

Challenges in the Medical and Surgical Management of Chronic Inflammatory Bowel Disease.

Inflammatory bowel disease patients will likely come to the surgeon's attention at some point in their course of disease, and they present several unique anatomic, metabolic, and physiologic challenges. Specific and well-recognized complications of chronic Crohn disease and ulcerative colitis are presented as well as an organized and evidence-based approach to the medical and surgical management of such disease sequelae. Topics addressed in this article include intestinal fistula and short bowel syndrome, pouch complications, and deep venous thrombosis with emphasis placed on optimization of the patient's physiologic state for best outcomes.

Surgical management of Crohn's disease.

INTRODUCTION: Crohn's disease is an inflammatory bowel disease that can affect the entire gastrointestinal tract. It is chronic and incurable, and the mainstay of therapy is medical management with surgical intervention as complications arise. Surgery is required in approximately 70% of patients with Crohn's disease. Because repeat interventions are often needed, these patients may benefit from bowel-sparing techniques and minimally invasive approaches. Various bowel-sparing techniques, including strictureplasty, can be applied to reduce the risk of short-bowel syndrome. METHODS: A review of the available literature using the PubMed search engine was undertaken to compile data on the surgical treatment of Crohn's disease. RESULTS AND CONCLUSION: Data support the use of laparoscopy in treating Crohn's disease, although the potential technical challenges in these settings mandate appropriate prerequisite surgical expertise.

How I do it: Side-to-side isoperistaltic strictureplasty for extensive Crohn's disease.

INTRODUCTION: Bowel-sparing surgical techniques, such as the Heineke-Mikulicz and the Finney strictureplasty, have been proposed as an alternative to lengthy intestinal resection in the treatment of small bowel strictures in Crohn's disease. However, these conventional strictureplasty techniques lend themselves poorly to cases of multiple short strictures closely clustered over a lengthy small bowel segment. DISCUSSION: In this article, we present the surgical technique of the side-to-side isoperistaltic strictureplasty, which is optimal in addressing these specific situations.

Side-to-side isoperistaltic strictureplasty for chronic ischemic enteritis: report of a case.

Chronic ischemic enteritis can cause intestinal strictures, but extensive resection of the small intestine may leave patients with short bowel syndrome. Thus, the importance of preserving diseased small bowel is now recognized. We report a case of successful side-to-side isoperistaltic strictureplasty (SSIS), performed to prevent short bowel syndrome, in a patient with ischemic enteritis caused by strangulated intestinal obstruction. SSIS is useful for preserving the intestinal absorptive function in patients with a long narrowed bowel loop caused by ischemic change. To our knowledge, this is the first report of the successful treatment of a long stricture resulting from ischemic enteritis, achieved by performing SSIS.

Open abdominal surgery with mesh and zipper for superior mesenteric artery occlusion.

Intestinal infarction caused by superior mesenteric arterial occlusion (SMAO) often requires massive resection of the necrotic bowel. However, this procedure frequently causes the short bowel syndrome. To avoid the development of this syndrome, it is important to conserve as much of the remnant bowel as possible. However, SMAO frequently occurs in patients with atrial fibrillation; even if the operation saves the patient's life, the risk of disease recurrence remains. We developed a novel open abdominal surgical technique involving the use of a mesh with a zipper to monitor the blood flow around the primary anastomosis created during the initial operation. Here, we described this technique and the postoperative management procedures and evaluate the efficiency of the technique.

Preserving bowel length with a transluminal stent in neonates with multiple intestinal anastomoses: a case series and review of the literature.

BACKGROUND/PURPOSE: In neonatal surgery, preserving small bowel length is important to avoid short bowel syndrome. Our aim was to assess the outcomes of intraluminal stenting of neonatal multiple intestinal anastomoses. METHODS: We conducted a retrospective review of 9 patients (5, single institution; 4, published literature) who received multiple anastomoses stented by a silicon tube. Demographics, surgical anatomy and complications, nutritional outcomes, and follow-up were reviewed. RESULTS: Diagnosis was multiple intestinal atresias in 8 patients and necrotizing enterocolitis in 1. A silicon catheter entered either the mucous fistula (5 patients received a jejunostomy/mucous fistula) or a proximal opening on the dilated bowel and was threaded through viable segments of the bowel. The bowel ends were approximated. Stent was externalized in 7 patients. Final mean small bowel length was 63.9 cm. All complications (3 patients, leak/stricture) required surgery. Mean time to stent removal, feeds initiation, and parenteral nutrition (PN) discontinuation was 31.2 days, 17.3 days, and 159 days, respectively. Only 1 patient remains on PN (mean follow-up, 25.4 months). CONCLUSIONS: Multiple intestinal anastomoses stenting is an excellent technique to avoid short bowel syndrome in the setting of multiple viable segments of gut, such as type IV intestinal atresia or necrotizing enterocolitis. Both our experience and the published literature show no mortality and PN-free survival.

No free ride? The hidden costs of delayed operative management using a spring-loaded silo for gastroschisis.

PURPOSE: The ideal management of gastroschisis (primary vs staged closure) has not yet been established. Despite the ease of silo placement, anecdotal experience shows that silos do not always offer benefit. The aim of this study was to highlight concerns regarding use of spring loaded silos and compare outcomes to primary closure. METHODS: Thirty-seven neonates with gastroschisis treated with either primary (n = 10) or staged closure with a spring-loaded silo (n = 27) were reviewed (1998-2007). Variables included ventilator days, daily intravenous fluid, hospital days, and complication rates. SPSS (SPSS Inc, Chicago, Ill) was used to perform t test and chi(2) analyses (significance P < .05). RESULTS: Survival for primary closure was 100% (10/10) compared to 89% (24/27) for staged closure (P = .548). Patients managed with silos required prolonged ventilation (16.1 +/- 4 days vs 3.6 +/- 1 days; P < or = .05) and greater intravenous fluids on days 3, 4, and 5 of life (132 +/- 25 mL/kg per day vs 104 +/- 18 mL/kg per day; P < or = .01). Although there was no difference in the complication rates between the groups, several problems were evident in the silo group: 15% (4/27) required silo replacement, 44% (12/27) required fascial defect enlargement for silo placement, and 19% (5/27) required mesh at closure. No significant differences in recovery of intestinal function were observed. Three silo patients developed ischemic complications because of vascular insufficiency at the level of the abdominal wall, leading to significant intestinal loss, ventilator and total parenteral nutrition dependence, and increased hospital stay. CONCLUSIONS: Patients managed with a silo had longer ventilator requirements and greater fluid needs. This Specific technical complications leading to bowel ischemia were notable in the silo group. The silo should be carefully placed to avoid bowel twisting and the funnel effect. Larger prospective studies should be performed to provide decision-making criteria for the use of a silo vs primary closure.

Initial management of extensive mesenteric venous thrombosis: retrospective study of nine cases.

BACKGROUND: The development of mesenteric venous thrombosis (MVT) does not necessarily require surgical intervention. The aim of this study was to assess the efficacy of avoiding early operative intervention, which can lead to significant sacrifice of the small bowel. METHODS: Patients with MVT were identified using the inpatient registry for the years between 2003 and 2007. Each patient's past medical history, history of prior deep venous thrombosis or hypercoagulable state, clinical and biologic presentation, and computed tomography (CT) results were analyzed. The proportion of ischemic bowel observed on the CT scans was compared with the length of the bowel resected. RESULTS: Nine patients were admitted for extensive MVT during the time period evaluated (six men, three women). All CT scans demonstrated signs of severe bowel ischemia, with a mean ischemic bowel proportion of 21% (range 5-45%). Four patients received medical management alone. Five patients underwent surgery. The mean admission time for these patients prior to the operation was 14.8 days (6-36 days). Surgery was required only in cases of intestinal perforation. The mean length of the bowel resections was 33 cm (20-45 cm). At 6 months after admission, none of the patients required parenteral nutrition. The mean follow-up evaluation period was 27 months (15-38 months). One patient died secondary to amyotrophic lateral sclerosis during the follow-up. CONCLUSIONS: Initial nonsurgical management comprised of inpatient observation on a surgical ward along with systemic anticoagulation must be considered an alternative treatment strategy for MVT. This strategy delays surgery and therefore avoids short bowel syndrome.

Antimesenteric tapering enteroplasty in an adult patient with clinical evidence of obstruction: a bowel-preserving alternative to resection.

BACKGROUND/AIMS: The approach to small bowel obstruction, when unrelieved with medical management, is open or laparoscopic exploration, often necessitating lysis of adhesions and the resection of any compromised bowel. In patients with prior bowel resections, the surgeon must tread a fine line, so as not to precipitate the clinical derangement known as short bowel syndrome (SBS). Techniques have been described that curtail the extent of intestinal resection, but these are primarily limited to the pediatric literature and are not commonly practiced in the general surgical population. METHODS: We report a case of a complicated small bowel obstruction in a patient with pre-existing short bowel length, in which a tapering enteroplasty was performed. RESULTS: Antimesenteric tapering successfully achieved a return of bowel function, avoiding the morbidity of an extended small bowel resection and the possibility of developing SBS. CONCLUSION: In such patients who are at high risk of developing SBS, in whom a segment of dilated small bowel has become defunctionalized, leading to significant and life-threatening symptoms, this procedure has the potential to help prevent SBS and its lifelong complications and associated mortality.

Small intestinal nonmeckelian diverticulosis.

Nonmeckelian jejunoileal diverticula (JID) are rare, but potentially clinically significant lesions. Despite recent advances in modern diagnostic modalities, diagnosis of JID may be problematic. Upper gastrointestinal contrast series with small bowel follow-through examination and mainly enteroclysis are the 2 main diagnostic methods. In selected cases (mainly complicated JID), the physician could use other diagnostic methods, such as ultrasound, computed tomography, endoscopy, intraoperative endoscopy, laparoscopy, radiotagged erythrocyte bleeding scans, and selective mesenteric arteriography. JID may be clinically silent or symptomatic causing chronic pain or malabsorption or other acute complications, such as hemorrhage, inflammation, perforation, etc. Laparotomy remains the gold standard for definite diagnosis of asymptomatic and complicated diverticula. Treatment should be individualized. Surgery could be indicated, mainly in symptomatic diverticula. The extent of resection may be a problem, especially in patients with extensive disease involving large parts of the bowel. In these cases, clinical judgment is required from the part of surgeon to avoid short bowel syndrome.