Impact of Focused Abdominal Ultrasound Utilization on Outcomes for Children With Midgut Volvulus.

BACKGROUND: Ultrasound (US) is gaining acceptance for the evaluation of midgut volvulus in children. However, its impact on clinical outcomes is unknown. We aim to determine whether using US as a first-line modality changes imaging mobilization, time to surgery and re-feeding, length of stay, and frequency of bowel necrosis, short bowel syndrome, and death. METHODS: An IRB-approved retrospective cohort study was performed at a tertiary pediatric institution. Eighty children with surgically confirmed midgut volvulus from 2014 to 2021 were compared before and after implementation of US as first-line imaging and based on the modality used to diagnose midgut volvulus. RESULTS: Outcomes were not statistically different pre- versus post-implementation. Compared with patients who had UGI only, those who had US only or both had significantly quicker imaging mobilization (median: -33 min; 95% CI: -61.2, -4.8; p = 0.023 and median: -31 min; 95% CI: -58.5, -3.6; p = 0.028 respectively). Patients with US only were less likely to have bowel necrosis compared with those who had UGI only (9.1% versus 43.8%, p = 0.042). Patients who had US only or both were less likely to develop short bowel syndrome compared to UGI only (4.8% US only, 0% both, 40% UGI only; p = 0.027 for US only, p = 0.005 for both). CONCLUSIONS: No statistically significant change in outcomes was found after implementation of US as first-line imaging for midgut volvulus. However, patients diagnosed with US only or US in combination with UGI had quicker imaging mobilization and decreased frequency of bowel necrosis and short bowel syndrome. Findings suggest that US has potential to improve patient outcomes. LEVEL OF EVIDENCE: III.

Intestinal failure-associated liver disease in patients with short bowel syndrome: Evaluation by transient elastography.

OBJECTIVE: Patients with short bowel syndrome (SBS) receiving long-term parenteral nutrition (PN) are at risk for intestinal failure-associated liver disease (IFALD). The aim of the present study was to evaluate dynamic changes of liver fibrosis and steatosis within 12 mo by transient elastography (TE), including controlled attenuation parameter (CAP) in a cohort of patients with SBS receiving long-term PN. METHODS: Twenty-five adult patients with SBS and PN requirement for ≥3 mo consecutively were included and prospectively followed. Liver stiffness by FibroScan (Echosens, Paris, France) and CAP measurement were done at study entry and after 12 mo. Clinical parameters, as well as data on underlying bowel disease and nutrition composition, were collected. Bioelectrical impedance analysis was performed in all patients. RESULTS: FibroScan and CAP did not show any significant differences after 12 mo (5.2 kPa [2.8-16.2 kPa]; 223dB/m [101-366 dB/m]) compared with study entry (5.3 kPa [2.7-12.3 kPa]; 237dB/m [100-344 dB/m]). There was no significant correlation between FibroScan and CAP and elevated transaminase levels. CAP significantly correlated with triacylglyceride levels (r = 0.411; P = 0.042) and body mass index (r = 0.468; P = 0.016). Patients with a remnant small bowel <100cm showed a significantly higher stiffness value by FibroScan than those having a remnant length ≥100cm (6.1 versus 4.7 kPa; P = 0.028). CONCLUSION: In the present study cohort, prevalence of advanced fibrosis or cirrhosis was low (<10%) without significant dynamic within the 12-mo follow-up. Short intestinal remnant length <100cm appeared to be a risk factor for development of fibrosis.

Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results.

PURPOSE: The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS). METHODS: A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis. RESULTS: Thirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (ß=0.16 m/s increase per stage, p=<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC=0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC=0.84, 95% CI 0.71-0.96). CONCLUSION: ARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS. TYPE OF STUDY: Retrospective Cohort Study LEVEL OF EVIDENCE: II.

Three-dimensional CT enterography versus barium follow-through examination in measurement of remnant small intestinal length in short bowel syndrome patients.

PURPOSE: To evaluate the feasibility and accuracy of remnant small intestinal length measured by barium follow-through (BaFT) examination and three-dimensional CT enterography (CTe). MATERIALS AND METHODS: Twenty-nine consecutive short bowel syndrome patients (SBS) who underwent BaFT, CTe, and prior surgical measurements of small intestine were included. Measurements of total remnant small intestinal length on BaFT and CTe were compared to surgical measurements using Spearman's rank correlation coefficients, Bland-Altman plots, and paired t test. RESULTS: The average remnant intestinal length was 73.1 ± 37.2 cm according to surgical measurement. There was a significant positive correlation between CTe and surgical measurement (r2 = 0.99; p < 0.0001), and a relatively weaker correlation between BaFT and surgical measurement (r2 = 0.71; p < 0.001). Compared with surgical measurement, the percentage differences of CTe and BaFT were 5.71 ± 6.71% and 27.14 ± 18.41% (mean ± SD), respectively. Furthermore, Bland-Altman plots showed good agreement between CTe and surgical measurement, whereas relatively poor agreement between BaFT and surgical measurement. However, significant difference was found among the three measurement methods by paired t test (p < 0.0001). CONCLUSIONS: Assessment of remnant small intestinal length by CTe is accurate and acceptable for clinical application, whereas BaFT is less accurate although BaFT is more convenient and cheaper for clinical application. And CTe can provide a cost-effective and noninvasive determination of remnant small intestinal length in planning surgical and nutritional intervention in SBS patients.

Duodenal lengthening in an adult with ultra-short bowel syndrome. A case report.

BACKGROUND: We have recently demonstrated the feasibility of lengthening the duodenum in children with short bowel syndrome and a dilated duodenum. This procedure gains additional intestinal length in a challenging area of autologous gut reconstruction. Herein, we report the successful application of this technique in an adult with ultra-short bowel syndrome. CASE REPORT: A 25-year-old man with a history of mid-gut volvulus was referred to our center for intestinal transplant evaluation. Only a megaduodenum stump that reached as far as the third portion (30 cm of length) and the colon up to the hepatic flexure in the form of a mucous fistula was retained. A gastrostomy tube drained gastric and bilio-pancreatic secretions (output range: 2.5-4 liters/day). The time spent on parenteral nutrition (3 liters/day; 1500 calories/day) and I.V. fluid (1.5-2 liters/day) administration was 24 hours per day. The patient underwent duodenal lengthening and tapering with 7 sequential transverse applications (5 of 45 mm and 2 of 60 mm) of an endoscopic stapler on the anterior and posterior walls of the duodenum, respecting the pancreatic parenchyma and end-to-side duodeno-colonic anastomosis. The final duodenal length was 83 cm. The pre-lengthening citrulline level increased from 13.6 micromol/L to 21.6 micromol/L one year post-lengthening. After 24 month of follow-up, the time on a parenteral pump was shortened to 9 hours during the night. The volume and calorie requirements were also reduced by half. DISCUSSION: Duodenal lengthening may be effective as part of the autologous intestinal reconstruction armamentarium in adults with short bowel syndrome.

Radiographic measurement of intestinal length among children with short bowel syndrome: Retrospective determination remains problematic.

PURPOSE: Small bowel length is the most reliable predictor of enteral independence in pediatric short bowel syndrome. Retrospectively measured bowel lengths on upper GI with small bowel follow-through (UGI/SBFT) were compared to operative measurements. METHODS: A pediatric radiologist and surgical trainees blinded to operative measurements retrospectively analyzed UGI/SBFT studies using the digital radiography curved measurement tool. Children with SBS and severe intestinal failure (parenteral nutrition >90days) at a multidisciplinary intestinal failure program 2002-2015 were included. Data were expressed as median (Q1, Q3). RESULTS: Thirty-six children aged 0.8 (0.4, 3.7) years were analyzed. Fifty-six percent had intestinal malrotation, and 58% had prior serial transverse enteroplasty. Studies were conducted within 10 (7, 20) days of surgery. Intraoperative bowel length was 90cm (45, 142), while UGI/SBFT measurement by radiologist was 45cm (28, 63), with a mean difference of 47cm (SD 58cm, p<0.001) and a mean percent error of 50%. Radiographic assessment underestimated intestinal length in 83% of patients. CONCLUSION: Bowel length measured retrospectively from upper GI with small bowel follow-through studies usually underestimated intraoperative bowel length. The limits of agreement were too wide for this technique to be clinically useful. Operative measurement remains necessary to assess intestinal length and rehabilitation potential. TYPE OF STUDY: Study of Diagnostic Test. LEVEL OF EVIDENCE: Level III.

Symptomatic retention of the Agile® patency capsule.

The Agile® capsule has shown to be useful when evaluating the patency of the small bowel in patients prior to capsule endoscopy studies. It is a safe tool and a low rate of complications have been reported, highlighting symptomatic retention, although references in literature are scarce and it is only observed in 1.2% of the procedures. We present the case of a symptomatic retention of this device in a patient with previously known colonic Crohn's disease in who a small bowel study was indicated and was sent for prior patency test.

The use of bi-planar tissue expanders to augment abdominal domain in a pediatric intestinal transplant recipient.

Intestinal transplantation is a well-accepted treatment for SBS. However, patients with SBS often have decreased abdominal capacity, which makes size-matching of donor organs more difficult, thus decreasing organ availability. Reported approaches for addressing this problem include surgically reducing the graft size, leaving an open abdomen for a prolonged period, and cotransplanting rectus fascia as a non-vascularized allograft. Each approach has significant disadvantages. There has been one previous report of tissue expanders used intra-abdominally and two reports of subcutaneous use to increase intra-abdominal capacity prior to transplantation. We report the first use of bi-planar expander placement for this purpose. In our case, a two-yr-old male child with SBS due to malrotation was treated with tissue expanders 10 months prior to intestinal transplantation, thus allowing transplantation of a larger graft with the ability to close the abdomen safely. There were no complications, and the patient is now doing well and tolerating diet off PN. The use of tissue expanders prior to intestinal transplantation is a promising approach for such patients and avoids the morbidity associated with other approaches. This approach requires a multidisciplinary effort by gastroenterology, transplant surgery, and plastic surgery teams.

The first clinical application of the spiral intestinal lengthening and tailoring (silt) in extreme short bowel syndrome.

AIM: Spiral Intestinal Lengthening and Tailoring (SILT) invented by our team is a new technique that offers minimal mesenteric handling and a more physiological result compared to the STEP procedure. Its feasibility has been tested in animal models and now we report the first successful human application in extreme short bowel syndrome. MATERIALS AND METHODS: A 3-year-old girl suffered subtotal loss of her small bowel and ileocaecal junction as a result of midgut volvulus. Only 15 cm of jejunum remained intact. Parenteral nutrition (PN), gastrostomy feeding, controlled bowel expansion and SILT procedure were applied. RESULTS: The length of the jejunum increased from the initial 15 to 22 cm during 12 months of PN and bowel expansion. Eleven centimeter of distended bowel was further lengthened up to 20 cm by SILT giving a total small bowel length of 31 cm. Oral and gastrostomy feedings were commenced 5 days postoperatively. There were no surgical complications 6 months after the procedure. The patient's liver function was preserved, she was weaned off PN, discharged from hospital, but remained on gastrostomy top up feeding. The net weight gain of the patient was 1,800 g 6 months after the procedure. CONCLUSION: SILT procedure is a safe and feasible technique for human intestinal lengthening and tailoring.

Second serial transverse enteroplasty procedure in an infant with extreme short bowel syndrome.

The serial transverse enteroplasty (STEP) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome. The advantages of STEP include not only technical ease and simplicity, but also the ability to repeat the procedure. Herein, we report a case of extreme short bowel syndrome that was successfully treated by the second STEP procedure. A 3-day old newborn girl underwent STEP because of jejunal atresia with the small bowel length of 15 cm, but her bowel elongation was not enough to escape from short bowel syndrome. At the age of 6 months, she underwent a second STEP procedure. The bowel lengthening by the second STEP made her tolerable to enteral feeding with body weight gain and rescued her from short bowel syndrome. This case showed that second STEP is very helpful in treatment of extreme short bowel syndrome.

Second Serial Transverse Enteroplasty Procedure in an Infant with Extreme Short Bowel Syndrome

The serial transverse enteroplasty (STEP) procedure is a novel technique to lengthen and taper the bowel in patients with short bowel syndrome. The advantages of STEP include not only technical ease and simplicity, but also the ability to repeat the procedure. Herein, we report a case of extreme short bowel syndrome that was successfully treated by the second STEP procedure. A 3-day old newborn girl underwent STEP because of jejunal atresia with the small bowel length of 15 cm, but her bowel elongation was not enough to escape from short bowel syndrome. At the age of 6 months, she underwent a second STEP procedure. The bowel lengthening by the second STEP made her tolerable to enteral feeding with body weight gain and rescued her from short bowel syndrome. This case showed that second STEP is very helpful in treatment of extreme short bowel syndrome.

Successful management of short gut due to vanishing gastroschisis - case report and review of the literature.

Vanishing gastroschisis is a rare in utero complication of gastroschisis. It is associated with a high mortality. We present a case report of an infant with vanishing gastroschisis that was managed with a combination of reconstructive bowel surgery and hepatosparing parenteral nutrition. The technique is described and a review of the literature is provided.

Ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum in a newborn with pyloric atresia, annular pancreas and congenital short bowel syndrome.

We report a newborn with bilious vomiting and the rare combination of pyloric atresia, annular pancreas and ectopic drainage of the common bile duct into the lesser curvature of the gastric antrum. Radiologic, sonographic and percutaneous transhepatic transcholecystic cholangiographic (PTTC) findings, with surgical correlation, are presented.

Short bowel syndrome: amelioration of diarrhea after vagotomy and pyloroplasty for peptic hemorrhage.

Acute mesenteric ischemia is a rare symptomatic manifestation of arteriosclerosis. Prognosis crucially depends on rapid diagnosis and surgical management to prevent or at least minimize the bowel infarction. The length of the small bowel is considered to be between 3 and 8 m, and a normal bowel function can be maintained even after resection of its one third. But loss of a major part (> 60%) can lead to malnutrition and death. However, patients, who survived an extended intestinal resection due to improved postoperative care (intensive care unit and parenteral nutrition), develop short bowel syndrome. This phenomenon is a medical problem, and several surgical techniques have been used to slow down intestinal transit time or to increase the area of absorption. All these procedures have controversial outcomes and are still on different experimental levels; namely, they cannot be recommended for routine use. In our report of a patient suffering from short bowel syndrome, vagotomy and pyloroplasty were performed to repair a sudden peptic hemorrhage. This operation cured bleeding peptic ulcer and also palliated the diarrhea, a main clinical manifestation of short bowel syndrome. In this study, our aim is to emphasize the favorable clinical outcome of vagotomy concerning a principal manifestation of short bowel syndrome, such as diarrhea. To the best of our knowledge, the present study is the first report showing the vagotomy as a possible procedure for the treatment of diarrhea, although this occurrence has no clear explanation. We also discuss the management of short bowel syndrome.

Congenital intestinal anomalies, neonatal short bowel syndrome, and prenatal/neonatal counseling.

BACKGROUND: Short bowel syndrome (SBS) is a severe malabsorption caused by bowel loss. Congenital intestinal anomalies (CIA) detectable by prenatal ultrasound as jejunoileal atresia, meconium peritonitis, complicated meconium ileus, and fetal volvulus can be responsible for SBS. AIMS: This study aims to investigate either frequency of SBS or the morbidity in CIA population during the first admission. MATERIAL AND METHODS: Records of CIA treated from 1997 to 2003 were reviewed. The prenatal ultrasound findings were correlated with SBS. Student's t and chi(2) tests were performed to analyze epidemiological data, growth at discharge, sepsis, liver disease, catheter-related complications, motor developmental delay, and hospital stay in CIA with and without SBS. RESULTS: Forty-four CIA: SBS developed in 43%, ranging from 83% in volvulus to 0% in complicated meconium ileus. Thirty-six prenatal diagnoses: a strong correlation with SBS was observed in isolated dilated bowel (58%). In SBS neonates, birth weight, gestational age, and growth at discharge were statistically lower, whereas sepsis, motor delay, and hospital stay were statistically higher. CONCLUSIONS: Many neonates with CIA detectable by prenatal ultrasound develop SBS. Short bowel syndrome presents a significant higher morbidity. The counseling should stress the frequent association between CIA and SBS as well as the significant morbidity in SBS.

Central venous recanalization in patients with short gut syndrome: restoration of candidacy for intestinal and multivisceral transplantation.

PURPOSE: To assess feasibility and success of venous recanalization in patients with short gut syndrome who have lost their traditional central venous access and required intestinal or multivisceral transplantation. MATERIALS AND METHODS: Twelve patients between the ages of 7 and 55 years with short gut syndrome and long-standing total parenteral nutrition (TPN) dependency and/or hypercoagulability were treated. All had extensive chronic central venous occlusions and survival was dependent on restoration of access and planned transplantation. Central venous recanalizations were obtained via sharp needle recanalization techniques, venous reconstructions with stents, and/or extraanatomic access to the central venous system for placement of central venous tunneled catheters. RESULTS: Central venous access was restored in all patients without operative-related mortality. Three major hemodynamic perioperative technical complications were recorded and successfully treated. There were three self-limited early infectious complications. With a mean follow-up of 22 months, eight of the 12 patients were alive with successful small bowel or multivisceral transplantation; six of those became independent of TPN. The remaining four patients died of complications related to TPN (n = 3) or transplantation (n = 1). With a mean follow-up of 20 months, all but two of the recanalized venous accesses were maintained, for a success rate of 83%. CONCLUSIONS: Recanalizations of extensive chronic vein occlusions are feasible but associated with high risk. The technique is life-saving for TPN-dependent patients and can restore candidacy for intestinal and multivisceral transplantation. This approach is likely to be increasingly requested because of the current clinical availability of the transplant procedure.

Congenital short-gut syndrome.

A case of congenital short gut is reported in a 4-month-old boy presenting with failure to thrive. Upper gastrointestinal examination (UGI) with small bowel follow-through (SBFT) demonstrated dilation of the duodenum and jejunum, with rapid transit to rectum. On barium enema (BE), rapid transit of barium was noted to the dilated proximal small bowel seen on UGI/SBFT. Neither study delineated the ileocecal region, and the overall length of bowel appeared short. The diagnosis of congenital short gut was confirmed at surgery. Congenital short-gut syndrome is a rare entity. The diagnosis can be suggested by imaging, but is usually confirmed operatively.

Isolated small bowel transplantation from a living-related donor at the Catholic University of Korea--a case report of rejection -free course-.

The bowel transplantation team at the Catholic Medical Center, Korea, on April 9 2004, accomplished a case of isolated small bowel transplantation (SBT) in a 57 year-old female with short bowel syndrome. The primary surgery was a jejunocolostomy due to mesenteric vein thrombosis, while maintaining 30 cm of the jejunum and colon distal to the splenic flexure. Her renal function was partially unbalanced. During more than 2 years of home TPN, the superior vena cava (VC) and subclavian veins had become occluded, but the inferior VC line remained. SBT was planned due to the repeated life-threatening infections of the last central line. One hundred and fifty centimeter of the distal ileum of the 27 year-old living-related donor, the patient's daughter, was harvested. The graft mesenteric artery and vein were anastomosed to the recipient's inferior mesenteric vessels. A proximal end-to-end jejuno-ileostomy and a distal end-to-side ileo-colostomy of the graft were made, creating a Bishop-Koop enterostomy for graft surveillance. A tube jejunostomy, via a gastrostomy, was established for early feeding and simultaneous gastric drainage. Induction with Daclizumab and immunosuppression consisted of tacrolimus and methylprednisolone, given intravenously, and then mycophenolate mofetil (MMF), enterally from day 3. The patient was discharged on day 42. A CMV infection on day 83 was successfully treated with 3 weeks of gancyclovir therapy. She has been nutritionally independent, with complete oral feeding, and free of rejection until day 170 after the transplantation.