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Purpose: Short bowel syndrome is associated with intestinal mucosal inflammation and microbial dysbiosis, leading to intractable complications. Partially hydrolyzed guar gum (PHGG) has trophic and anti-inflammatory effects on the intestine. We investigated whether PHGG ameliorates small intestinal mucosal damage and alters the intestinal microbiota using a rat small bowel resection (SBR) model., Methods: Sprague Dawley rats were divided into sham operation (Sham), Sham/PHGG, SBR, and SBR/PHGG groups. On day 21, all rats were euthanized. To assess small intestinal mucosal damage, the degeneration rate was morphometrically evaluated and immunohistochemically examined using anti-CD45 antibodies. Analyses of fecal microbiota using 16S rRNA and short-chain fatty acid production were also performed., Results: The mucosal degeneration rate was significantly higher in the SBR group than in the Sham or SBR/PHGG groups. The number of CD45-positive cells was significantly higher in the SBR group than in the Sham, Sham/PHGG, or SBR/PHGG groups. The relative abundance of family Lachnospiraceae was significantly higher in the SBR/PHGG group than in the SBR group., Conclusions: PHGG administration alleviated small intestinal mucosal damage which could be associated with modulation of the intestinal microbiota., (Copyright © 2019. Published by Elsevier Inc.)

Background/purpose: Intestinal complications of acute graft-versus-host disease (aGVHD) include hemorrhage and perforation in the short-term, and stricture with bowel obstruction in the long-term. As medical management of severe aGVHD has improved, more patients are surviving even advanced stages of intestinal aGVHD. This review summarizes the available pediatric literature on surgical treatment of complications of intestinal GVHD., Methods: A systematic review was performed using PubMed, Cochrane, Embase, and Scopus databases. Any publication that addressed surgical treatment of acute and chronic intestinal GVHD in the pediatric population was reviewed in detail. Furthermore, we included information on 5 additional patients from the institutions of this review's authors, which had not been previously published., Results: We identified 8 studies, comprising 13 patients. Surgical interventions were undertaken for a variety of intestinal GVHD complications, including small bowel obstruction owing to stricture (n=8), enterocutaneous fistulae (n=2), gastrointestinal hemorrhage/perforation (n=1 each), and esophageal stricture (n=1). Among eight patients with bowel obstruction as an indication, pathology revealed ulceration with fibrosis in all but one; 3 had signs of persistent GVHD. Surgical mortality was reported in 4 patients (31%) at an average of 6weeks postoperatively. The median overall follow-up time was 20months (IQR, 2-21)., Conclusions: Although intestinal aGVHD management is almost exclusively medical, a small subset of patients develops complications of intestinal GVHD that require surgical intervention. With expanding indications for stem cell transplantation as well as improved survival after previously fatal bouts of intestinal aGVHD, it is likely that surgical intervention will become more common in these complicated patients., Systematic Review: Level of Evidence: Level IV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Intraamniotic meconium has been responsible for intestinal damage in gastroschisis and meconium-dependent intestinal ischemia has been proposed to induce additional intestinal damage in gastroschisis. This study is aimed to determine the effects of lipid and water-soluble meconium subfractions on the contractility of the superior mesenteric artery (SMA)., Materials and Methods: The study was conducted on 18-day fertilized chick embryos (Gallus Domesticus). Meconium is fractioned into water and lipid-soluble components. Only one SMA tissue was prepared from each embryo and suspended in the organ bath. Isometric contraction responses (ICR) were created in SMA tissues by one hour of incubation in Krebs-Henseleit solution for each group. Groups consisted of control, meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction. ICR of the SMA specimens were evaluated with a transducer-amplifier system on a computer. The data were expressed (mean±1SD) as milliNewton (mN)., Results: The ICR of the meconium, water-soluble meconium subfraction and lipid-soluble meconium subfraction groups were significantly high when compared to the control group (p<0.01). The meconium and water-soluble meconium subfraction created more contraction response than the lipid-soluble meconium subfraction (p<0.01). The ICR of the meconium group was not different from the ICR of the water-soluble meconium subfraction group (p>0.05)., Conclusion: Water-soluble meconium subfraction has a profound vasoconstrictor effect on the SMA compared to the lipid-soluble meconium subfraction., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: It is unclear which substances in meconium are responsible for intestinal damage in gastroschisis. An experimental study was designed to investigate the effects of the lipid or water soluble subfractions of meconium on the intestines of gastroschisis in a chick model., Methods: Meconium was pooled, homogenated, rota-evaporated dry and diluted. Meconium subfractions were obtained from water soluble and lipid soluble extracts of the meconium. Five days old fertilized chick embryos were used and divided into 5 groups: control, sham, water soluble meconium subfraction, lipid soluble meconium subfraction and whole meconium. All embryos were extirpated on the 18days and the intestines were harvested for histopathological examination. Serosal thickness was measured under light microscopy., Results: Serosal thickness of the meconium (36.36±2.8μm), the water soluble meconium (14.15±0.93μm) and the lipid soluble meconium (23.88±1.69μm) subfractions groups were significantly increased compared with the control (7.47±0.68μm) and the sham (7.48±0.71μm) groups (p<0.001). Serosal thickness of the lipid soluble meconium subfraction group was significantly increased compared with the water soluble meconium subfraction group (p<0.001). Serosal thickness of the meconium group was significantly increased compared to both the water and the lipid soluble meconium subfraction groups (p<0.001)., Conclusion: Lipid soluble meconium subfraction induces more intestinal damage compared to water soluble meconium subfraction., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The development of colorectal cancer screening programs in many countries has led to increasingly large numbers of patients undergoing optical colonoscopy. Although acute complications from screening optical colonoscopy are uncommon, they may occur in up to 5% or more of patients where biopsies or therapeutic procedures are performed. Abdominal radiographs are of value only for the detection of intraperitoneal perforation. There is a wide spectrum of other important associated complications. Such complications are most reliably identified using abdominal and pelvic CT, which also can guide appropriate conservative, interventional, or surgical management., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Purpose: Longitudinal follow-up of changes in bowel function in children with anorectal malformations (ARMs) with or without spinal cord pathology and neurogenic bladder dysfunction (NBD) as they grow. Another purpose was to identify predictors influencing bowel functional outcome., Material and Methods: The study included 41 patients with ARM, excluding perineal fistula (21 boys and 20 girls). Bowel function was evaluated at ages 5, 10 and 15 years using a structured questionnaire and a three-week registration of number and time of bowel movements, episodes of fecal leakage and soiling. Additional bowel treatment with enemas and stool softeners and use of diapers were recorded. A group of 52 healthy boys and girls was used as control., Results: A successive improvement in functional outcome with age in children with ARM and normal spinal cord was seen with respect to continence, soiling and constipation. Continence was achieved earlier in girls than in boys (at 10 years: girls 80%, boys 36%). Soiling and constipation decreased with age both in grade and frequency (at 10 years low grade soiling: girls 53%, boys 64%). Boys with spinal cord malformation with NBD in combination with prostatic/bladder neck fistula (PRF/BNF) and sacral agenesis had the worst functional outcome with minimal possibility of improvement over time. Functional outcome in girls with NBD and tethered cord did not differ significantly from those without NBD and with a normal spinal cord. Psychosocial co-morbidity, neuropsychiatric disorders, developmental delay and megarectosigmoid were also risk factors impeding the functional outcome., Conclusion: In the present study there was a successive improvement in bowel function during childhood and adolescence in ARM children, but they did not achieve the level of healthy children. NBD, spinal cord malformation, sacral malformation and PRF all were negative predictive factors for bowel score at 5 years., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Systemic childhood polyarteritis nodosa (C-PAN) is a rare primary vasculitis involving medium or small sized arteries. Abdominal angina is an important and serious complication of PAN, occurring usually 15 to 30 min after food intake, and particularly in adult patients. However, to our knowledge, this involvement as the first manifestation of C-PAN was not described. Therefore, we reported herein two C-PAN cases that fulfilled the new criteria for this vasculitis. These patients were young boys that had malignant arterial hypertension and recurrent post-prandial cramping with acute abdomen. Both of them were submitted to laparotomy that revealed multiple and diffuse intestinal necrosis. One of our cases had a severe post-prandial cramping, even after drinking water, and the laparotomy evidenced multiple intestinal perforations. In spite of use of antihypertensive therapies, immunosuppressive agents (corticosteroids, cyclophosphamide and/or methotrexate) and intravenous immunoglobulin, they died possibly due to severe and disseminated activity disease. In conclusion, we described herein the first two fatal cases of C-PAN that presented severe abdominal pain as initial manifestation. We suggest that the diagnosis of PAN should be considered in patients under acute abdominal angina with no apparent etiology., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: Our aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN)., Methods: We retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)--persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)--additional elevated total bilirubin (≥ 50 μmol/L); and type 3 (late)--additional clinical signs of end-stage liver disease., Results: Eighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex., Conclusion: Intestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: Although a physiologic relationship between intestinal mucosal integrity and hepatic function has been previously described, the effect of primary liver disease on intestinal mucosal homeostasis has not been previously well documented. In the current study, we studied the effects of chronic liver injury as a primary injury on enterocyte turnover (proliferation and apoptosis) in a mouse model., Methods: The liver toxin 3,5-diethoxycarbonyl-1,4-dihydrocollidine (DDC)-enriched diet was used to induce chronic cholestatic liver injury in mice. Livers and intestine were harvested after 3 weeks of dietary treatment of histologic analysis and a determination of cell proliferation (immunohistochemistry for Ki67), or apoptosis (immunohistochemistry for caspase-3), as well as a determination of Wnt/β-catenin signaling activity., Results: All DDC-fed animals exhibited histologic evidence of liver damage that was associated with the expansion of atypical ductal proliferation near the periportal areas and increased oxidative stress. In the intestine, DDC-induced liver damage was associated with decreased villus height, decreased enterocyte proliferation, and increased cell apoptosis compared with control animals. There was also evidence for decreased β-catenin expression by immunostaining in crypt and villus cells of DDC-fed mice compared with control animals., Conclusion: Primary liver injury and cholestasis is associated with intestinal mucosal hypoplasia. Decreased cell proliferation and increased cell apoptosis may be responsible for decreased intestinal epithelial cell mass. The observed decrease in cell turnover is accompanied by an alteration in Wnt/β-catenin signaling., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Intestinal failure (IF) is the dependence upon parenteral nutrition to maintain minimal energy requirements for growth and development. It may occur secondary to a loss of bowel length, disorders of motility, or both. Short bowel syndrome (SBS) is a malabsorptive state resulting from surgical resection, congenital defect, or diseases associated with loss of absorptive surface area. A particularly vexing problem is associated with whole bowel and/or segmental intestinal dysmotility. Motility disorders within the context of SBS and IF may relate to rapid intestinal transit secondary to loss of intestinal length, dysmotility associated with loss or poor antegrade peristalsis, or gastroparesis. Therapy may be classified into medical (prokinetic and antidiarrheal agents) and surgical to deal with the overdistended poorly motile bowel., Methods: We performed a systematic review of the literature pertaining to IF, SBS, and dysmotility in the pediatric population with gastroschisis, necrotizing enterocolitis, and intestinal atresia. In addition to the available treatment options, we have provided a review of the literature and a summary of the available evidence., Conclusion: Despite relatively poor level of evidence regarding the application of promotility and antidiarrheal medications in patients with SBS and IF, these agents continue to be used. Herein, we provide a review of the physiology and pathophysiology of intestinal motility/dysmotility and available strategies for the use of promotility and antidiarrheal agents in patients with IF/SBS., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Despite a good understanding of short-term outcomes of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures, limited data exist on long-term complications., Methods: This is a 15-year single-institution retrospective chart review of patients who underwent an intestinal lengthening procedure (ILP). Long-term ILP-related complications, their interval to development, patients' ability to wean off parenteral nutrition (PN), and the need for further procedures were analyzed., Results: Of 119 patients with short bowel syndrome, 14 had undergone an ILP. Seven patients had an LILT, and 9 patients had a STEP, including repeat ILPs on the same patient. Overall, 93% of patients had complications. Four patients in the LILT group and 3 patients in the STEP group weaned off PN. Eight patients (57%) experienced bowel redilation after their ILP. The 2 deaths in the study came from this group. Seven required another abdominal operation and only one weaned off PN. There were no significant differences in mean bowel length between the redilated group and the non-re-dilated group., Conclusions: Complications are common after ILPs, and patients who redilated their bowel after ILP did clinically worse than those who did not., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: We sought to identify causes of preventable complications related to operations for Hirschsprung disease., Methods: We reviewed the cases of 51 patients with Hirschsprung disease who underwent a primary procedure elsewhere, had a complication, and were referred for reoperation., Results: Thirty-five patients had 1 failed operation, 10 had 2, and 6 had 3. Initial operations were Soave (20), Duhamel (15), Swenson (5), transanal endorectal (4), myectomy (3), unknown (3), and laparoscopic Swenson (1). Thirty-one patients presented with a stoma. Patients without a stoma (20) had fecal impaction (8), recurrent enterocolitis (6), and fecal incontinence (6). None had both enterocolitis and incontinence. Reoperation was performed posterior sagittally (40) or transanally (5). Indications included stricture (21), megarectal Duhamel pouches (12), fistulae (11 [8 rectocutaneous, 2 rectourethral, and 1 rectovaginal]), pouchitis (2), and retained aganglionic bowel (8). After reoperation, 14 were continent, 11 had a stoma (8 permanent), 6 had voluntary bowel movements but soiled occasionally, 6 received rectal irrigations to avoid enterocolitis, 6 were incontinent but clean with bowel management, and 2 were lost to follow-up., Conclusion: Stricture, megarectal pouch, fistula, and retained aganglionic bowel are preventable complications. Enterocolitis is partially preventable but can occur after a technically correct procedure. Fecal incontinence is a preventable complication likely because of anal canal damage.

Aim: Hypoganglionosis has been associated with fewer intestinal ganglion cells. However, current reports questioned the validity of this clinical entity. The aim of this study is to demonstrate the existence of hypoganglionosis., Materials and Methods: We have experienced 24 cases of functional intestinal obstruction with abnormalities of the intestinal ganglia. A precise histological examination was performed using quantitative morphometric studies. These results were compared with age-matched controls (n = 13)., Results: Based on histological examination, disorders in the ganglion cells could be classified into 3 categories: immaturity of ganglia (n = 13), congenital hypoganglionosis (n = 7), and acquired hypoganglionosis (n = 4). In congenital hypoganglionosis, the number as well as the size of ganglion cells are small at birth. The size of ganglion cells tends to increase over time, but their numbers do not increase; as a result, the symptoms of dysmotility do not improve. On the other hand, acquired hypoganglionosis is late onset and characterized as a degeneration of ganglion cells and gliosis histologically. After performing a resection of the affected bowel, the prognosis is usually good., Conclusion: Congenital and acquired hypoganglionosis are 2 distinct entities. The histological findings as well as the clinical characteristics of these 2 types of hypoganglionosis are different.

Background: The aim of the study was to assess the utility of alpha glutathione S-transferase (alphaGST) as a potential marker of intestinal ischemia-reperfusion injury in children after cardiac surgery., Methods: Twenty-six patients undergoing cardiac surgery were enrolled in this longitudinal experimental study. Blood samples were drawn for analysis at specified time points during surgery and analyzed for alphaGST levels. Clinical indices of splanchnic morbidity were assessed up to discharge from hospital. Results were analyzed using Mann-Whitney tests and linear mixed effects models., Results: Two groups were identified. Group 1 (n = 16) showed no intestinal morbidity and group 2 (n = 10) had signs of intestinal morbidity. Statistical differences were shown between the 2 groups with respect to time with aortic cross-clamp (ACC) in situ, time on cardiac bypass, duration of operation, time to enteral feeding and full feeding, time on mechanical ventilation, and time in the intensive care unit postoperatively. The serum concentration of alphaGST was significantly higher for group 2 and this rise was greatest after removal of the ACC., Conclusions: AlphaGST showed significant elevation in patients with prolonged bypass times and ACC times. These patients also displayed signs of intestinal morbidity, suggesting that this marker may be useful in screening patients at risk for intestinal pathology. This rise in alphaGST was associated with a prolonged ischemia time, and was greatest after the cross-clamp was released, suggesting that it is a postischemic reperfusion phenomenon leading to its elevation. A low alphaGST level appears to exclude significant intestinal ischemia.

Background/purpose: Contact with amniotic fluid causes intestinal damage in gastroschisis, and intraamniotic meconium has been shown to be responsible. Meconium has been shown to contain a significant amount of IL-8, which may be the responsible cytokine for harmful effects of meconium. Neonatal urine contains high amount of urinary trypsin inhibitor (UTI) compared with adult human urine. Urinary trypsin inhibitor has been shown to exert inhibitory effects on IL-8. Therefore, far from being destructive, presence of fetal urine in the amniotic fluid might be beneficial because human urine contains UTI. An experimental study has been performed to investigate whether presence of intraamniotic human urine (consequently UTI) besides meconium is beneficial on intestines of chick embryo with gastroschisis., Methods: Five-day-old fertilized chick eggs were used. Gastroschisis was created through amniotic cavity without opening the allantoic cavity. Sterile urine and meconium were obtained from newborn humans. Study was conducted in 2 stages. In the first stage, gastroschisis was created, and meconium suspensions at minimal harmful meconium concentration were prepared using natural and denatured human neonatal urine and instilled into the amniotic cavity. In the second stage of study, various concentrations of UTI plus meconium suspension at minimal harmful meconium concentration was instilled into the amniotic cavity., Results: Serosal thickening, inflammation, and focal fibrin deposits were observed in intestines of the groups with meconium and meconium in denatured urine. Histopathologic features of intestines of the group with meconium in natural urine did not differ from the intestines of the control group. Histopathologic examination of intestines of groups with meconium and meconium plus 50 U/mL UTI showed serosal thickening, inflammation, focal fibrin, and collagen deposits. Histopathologic features of intestines of the groups with 1:400 intraamniotic meconium plus 100 and 200 U/mL UTI did not differ from the intestines of control group., Conclusion: Urinary trypsin inhibitor 100 U/mL prevented the intestinal damage via inhibiting IL-8, which is contained by 1:400 concentration of meconium. Therefore, besides the existence of threshold level of meconium, the existence of UTI, which is capable of inhibiting IL-8 contained by threshold level of meconium, may be a factor in the occurrence of intestinal damage in gastroschisis.

We document the sequelae of the inadvertent introduction of glutaraldehyde into the peritoneal cavity. It describes the clinical course, progressive histological changes to the bowel at different periods over the course of 1 year, and what long-term morbidity remains. The chemical structure, effects, and pathogenesis of glutaraldehyde are described as well as suggestions for avoiding similar problems in the future.

Background and Purpose: In this report, we present the first case of an immunologically impaired child surviving a lytic varicella-zoster virus infection affecting the enteric nervous system. In histological findings, myenteric and submucous enteric ganglia were nearly completely absent owing to virus infection., Methods: A 3-year-old girl with acute lymphoblastic leukemia and generalized varicella-zoster infection developed an ileus. She underwent multiple laparotomies in which histological sections of the entire small intestine could be obtained., Results: The histological evaluation of these samples showed a generalized aganglionosis with inflammatory residuals. A more detailed immunohistochemical analysis using neuronal (PGP, enolase), glial (S100), and lymphocytic (LCA) antibodies demonstrated a nearly complete neuronal loss., Conclusion: To our knowledge, this is the first case of a secondary intestinal aganglionosis after varicella-zoster virus infection.

Background/purpose: The aim of this study was to investigate the possible etiologic factors of small bowel atresia and to detect the prognostic role of adequate resection and tapering in postoperative morbidity and mortality., Methods: Intestinal resection specimens were obtained from 10 patients with jejunoileal atresia and 3 control subjects without any gastrointestinal disease. Intestinal specimens taken from 2-cm and 4-cm proximal sides of atresia, atretic segment, and 1-cm and 2-cm distal sides of atresia were stained with Masson trichrome and H&E. Immunohistochemical staining of the biopsy specimens with synaptophysin was also performed to ascertain the number, the intensity, and the morphology of ganglia., Results: At the blind proximal end, there was segmental absence of muscular layers, presence of neural defects, and replacement of the muscular layers with fibrous tissue beside the relatively intact mucosa., Conclusions: Segmental defects in muscular and neural structures of the intestinal wall observed in both the antimesenteric and mesenteric sides of the atretic small bowel were considered to support the vascular insult theory as an etiologic factor. Adequate resection rather than tapering the dilated proximal atretic intestinal segment should be included in the surgical treatment of this pathology to avoid the intestinal dysmotility, which may result in gut-related sepsis and death in the postoperative period.

Umbilical hernia is a common pediatric diagnosis for which the initial management is usually nonoperative. The high incidence of spontaneous closure and the infrequency of complications in the first few years of life justify this conservative management strategy. The authors present a case of a 5-month-old girl with a history of prematurity and prolonged ventilatory support, who subsequently experienced bowel evisceration via a previously uncomplicated umbilical hernia.

Background: This study evaluated the complications of colostomy and its closure in infants and children., Methods: One hundred forty-six colostomies were performed in 86 neonates, 23 infants, and 37 children older than 1 year. These children underwent colostomies for anorectal malformation (84), Hirschsprung's disease (47), and other miscellaneous (15) conditions like colonic atresia, volvulus, rectal tuberculosis, traumatic rectal perforation, and intestinal obstruction caused by ascariasis., Results: Of these, 17 (11.6%) had early complications, and 80 (69.8%) had stomal complications. Three patients died, but only 1 death was directly related to colostomy. Colostomy prolapse, peristomal excoriation, and malnutrition were the major complications. The complications were not dependant on the children's age or primary indication. Sigmoid colostomy had a lower malnutrition rate than transverse colostomy (34.9% v 16.9% P =.009). Among the 56 children who underwent colostomy closure, major complications include death (1.8%), anastomotic leak (7.1%), and wound infection (12.6%)., Conclusions: A divided sigmoid colostomy should be performed whenever possible. Proper stomal care, regular nutritional assessment, and early closure of the colostomy would minimize morbidity and mortality of colostomy and its closure.

Background/purpose: The aim of this study was to determine causes of late graft loss and long-term outcome after isolated intestinal transplantation in children at a single center., Methods: All children who underwent primary isolated intestinal transplantation at our center with a minimum follow-up of 1 year were the subject of this retrospective study., Results: Twenty-eight children underwent primary isolated intestinal transplantation. Median graft survival was 705 days (range, 0 to 2,630 days) and median patient survival was 1,006 days (range, 0 to 2,630 days). There were 6 deaths and 15 graft losses (including the 6 nonsurvivors). Seven of the losses occurred 6 or more months after transplant. Of these, 2 losses occurred because of death of the recipients of sepsis; both recipients had functioning grafts. The 5 remaining late graft losses occurred because of acute rejection in 2 patients, chronic rejection in 2 (1 with concomitant acute rejection) and a diffuse stricturing process without the histologic hallmarks of chronic rejection in the fifth. All late survivors with intact grafts are off total parenteral nutrition (TPN)., Conclusions: Late graft loss remains a concern in a small percentage of patients after isolated intestinal transplantation. Nutritional autonomy from TPN is possible in the majority of these children after transplantation.

Background/purpose: Increasing evidence of physiologic in utero defecation supports the hypothesis that bowel damage in gastroschisis may be meconium dependent. In this study, the author investigated the role of meconium on parameters of bowel damage in a fetal rat model of gastroschisis., Methods: Pregnant rats underwent laparotomy at 18 1/2 days gestational age (GA). There were 4 experimental groups of 11 fetuses each; the G(M) group consisted of fetuses with isolated gastroschisis and was considered to have moderate meconium contamination of the amniotic fluid (MCAF); the G(L) group consisted of fetuses with gastroschisis and anal ligation, performed to prevent MCAF; the G(H) group consisted of fetuses with gastroschisis and colon perforation, performed to increase MCAF; and the Sham group consisted of sham operated controls. All fetuses were harvested by cesarean section at 21 1/2 days GA, and the fetal intestine was assessed for peel, intestinal length, intestinal weight per unit length, and histologic appearance., Results: The authors achieved the following fetal survival rates: G(M) group, 91% (10 of 11); G(L) group, 78% (7 of 9, the ligation was not successful in 2 fetuses); G(H) group, 82% (9 of 11). Sham group, 100% (11 of 11). Intestinal length was decreased in fetuses with gastroschisis, and this reduction was related directly to the grade of MCAF (Sham, 18.4 +/- 0.6; G(L), 11.5 +/- 0.5; G(M), 10.2 +/- 0.6; G(H), 9.1 +/- 0.6 cm; P <.01). In contrast, intestinal weight per unit length increased in fetuses with gastroschisis, and this increase was related directly to the grade of MCAF (Sham, 7.8 +/- 0.5; G(L), 9.4 +/- 0.5; G(M), 11.3 +/- 0.5; G(H), 16.9 +/- 0.7 mg/cm; P <.01). In comparison with the G(M) group, the degree of peel coverage and bowel adherence were increased markedly in the G(H) group, whereas the fetuses of the G(L) group had neither peel nor bowel adherence., Conclusions: All bowel damage parameters were affected by MCAF supporting the hypothesis that bowel damage in gastroschisis is at least partially dependent on meconium exposure. Further research is required to clarify other factors that contribute to bowel damage and to identify risk factors that may allow prenatal identification of severely affected fetuses., (Copyright 2002 by W.B. Saunders Company.)

Four cases of antenatally diagnosed gastroschisis are described in whom there was significant closure of the abdominal wall defect around the prolapsed midgut. In 2 cases this resulted in near-total midgut infarction and short bowel syndrome. In a further case there was entry and exit jejunal and colonic atresia and significant midgut damage but some viability of extrabdominal bowel. In the final case, although there was complete closure of the ring around the base of the midgut, no intestinal loss had occurred. In the latter 2 cases, serial antenatal ultrasound imaging had shown development of intrabdominal bowel dilatation prompting early preterm delivery. Three of these infants currently are alive and well. The authors suggest the use of the term closed (or closing) gastroschisis to describe this phenomenon. J Pediatr Surg 36:1834-1837., (Copyright 2001 by W.B. Saunders Company.)

Background/purpose: Contact with amniotic fluid (AF) causes intestinal damage in gastroschisis, which has been shown to be caused by intraamniotic meconium. However, whether this intraamniotic meconium-induced intestinal damage is concentration dependent has not been investigated previously. The purpose of this study is to investigate the effects of intraamniotic human meconium at various concentrations on the intestines of chick embryo with gastroschisis., Methods: Five-day-old fertilized chick eggs were used. Gastroschisis was created through the amniotic cavity without opening the allantoic cavity. Sterile meconium was obtained from newborn humans. Meconium suspensions at various concentrations were prepared using saline and instilled into the amniotic cavity., Results: Intraamniotic 1:200 and 1:400 meconium was found to cause intestinal damage. Meconium concentrations lower than 1:400 did not cause intestinal damage. Histopathologic examination of the intestines of the 1:200 and 1:400 meconium groups showed serosal thickening, inflammation, focal fibrin, and collagen deposits. Histopathologic features of the intestines of the 1:600 and 1:800 meconium groups did not differ from the intestines of the control group., Conclusion: Intraamniotic meconium, which is responsible for intestinal damage in gastroschisis, must reach a threshold level to induce intestinal damage. J Pediatr Surg 36:1811-1815., (Copyright 2001 by W.B. Saunders Company.)

In this article, disease of the small intestine will be discussed, with particular reference to those conditions leading to malabsorption and malnutrition. The work up of these entities will be emphasized, with focus on bacterial overgrowth, celiac sprue and nonsteroidal-induced enteropathy.

The primary care physician must make rapid diagnostic and therapeutic decisions pertaining to all body systems on a daily basis. Anorectal symptomatology generally is straightforward. Once the physician becomes familiar with the various disease processes that underlie hematochezia, anorectal pain and irritation, and changes in stooling habits, he or she can become more comfortable with managing anorectal disease in the office.

Purpose: A clinical trial of intestinal transplantation (Itx) under tacrolimus and prednisone immunosuppression was initiated in June 1990 in children with irreversible intestinal failure and who were dependent on total parenteral nutrition (TPN)., Methods: Fifty-five patients (28 girls, 27 boys) with a median age of 3.2 years (range, 0.5 to 18 years) received 58 intestinal transplants that included isolated small bowel (SB) (n = 17), liver SB (LSB) (n=33), and multivisceral (MV) (n=8) allografts. Nine patients also received bone marrow infusion, and there were 20 colonic allografts. Azathioprine, cyclophosphamide, or mycophenolate mofetil were used in different phases of the series. Indications for Itx included: gastroschisis (n=14), volvulus (n=13), necrotizing enterocolitis (n=6), intestinal atresia (n=8), chronic intestinal pseudoobstruction (n=5), Hirschsprung's disease (n=4), microvillus inclusion disease (n=3), multiple polyposis (n=1), and trauma [n=1)., Results: Currently, 30 patients are alive (patient survival, 55%; graft survival, 52%). Twenty-nine children with functioning grafts are living at home and off TPN, with a mean follow-up of 962 (range, 75 to 2,424) days. Immunologic complications have included liver allograft rejection (n=18), intestinal allograft rejection (n=52), posttransplant lymphoproliferative disease (n=16), cytomegalovirus (n=16) and graft-versus-host disease (n=4). A combination of associated complications included intestinal perforation (n=4), biliary leak (n=3), bile duct stenosis (n=1), intestinal leak (n=6), dehiscence with evisceration (n=4), hepatic artery thrombosis (n=3), bleeding (n=9), portal vein stenosis (n=1), intraabdominal abscess (n=11), and chylous ascites (n=4). Graft loss occurred as a result of rejection (n=8), infection (n=12), technical complications (n=8), and complications of TPN after graft removal (n=3). There were four retransplants (SB, n=1; LSB n=3)., Conclusions: Intestinal transplantation is a valid therapeutic option for patients with intestinal failure suffering complications of TPN. The complex clinical and immunologic course of these patients is reflected in a higher complication rate as well as patient and graft loss than seen after heart, liver, and kidney transplantation, although better than after lung transplantation.

The authors encountered two cases of gastrointestinal complication caused by ingested magnetic beads. Because of the magnetic property, the steel beads, components of bracelets or necklaces worn for naturalistic healing power, have caused perforations, fistula formation, and obstruction. The authors believe the public should be warned of the health hazard of such devices.

Malacoplakia is a specific chronic inflammatory disease. In the English-language literature, there are a few reports of gastrointestinal malacoplakia occurring in childhood. The authors present the first case of gastrointestinal malacoplakia involving a surgical wound and preventing healing, with persistent drainage; the patient was a 4-year-old boy. The clinicopathologic features of this rare entity are discussed, with emphasis on diagnosis and treatment.

Necrotizing enterocolitis (NEC) is a serious condition affecting predominantly the premature infant. The purpose of this study is to report a multicenter experience of complications in 252 infants requiring surgical therapy for NEC. Data from eight institutions for the years 1980 through 1990 were collected and analyzed for infants undergoing surgical therapy for NEC. Records were reviewed for gestational age, birth weight, age at operation, indications for operation, degree of intestinal involvement, operation(s) performed, complications, and 30-day mortality rates. A total of 264 infants underwent surgical intervention for NEC during the study period. Complete information was available for 252 patients. The mean gestational age was 31 +/- 5 weeks and the mean birth weight was 1,552 +/- 823 g. The mean age at operation was 18 +/- 35 days. Pneumoperitoneum was the most common indication for operation (42%). The 30-day survival rate was 72%. Eighty-one percent of patients underwent primary laparotomy, whereas peritoneal drainage was performed in 48 (19%) patients. Postoperative complications were identified in 119 (47%) patients. The most common postoperative complications were sepsis (9%), intestinal strictures (9%), and short gut (9%). Wound infections occurred in 6%, and the incidence of intraabdominal abscess formation was only 2.3%. Gestational age < 27 weeks (P < .005) and birth weight < 1,000 g (P < .005) were associated with significantly increased mortality but no increase in postoperative morbidity. The incidence of complications was similar in the very low birth weight (< 1,000 g) infants (51%) compared with infants > or = 1,000 g (46%).(ABSTRACT TRUNCATED AT 250 WORDS)

EPI in dogs represents a well-defined condition that can now be diagnosed simply by the analysis of a single serum sample for TLI. A low TLI concentration represents a highly sensitive and specific test for EPI and may also predict the development of disease before the onset of clinical signs. A lack of pancreatic enzymes results in interference with degradation of the major dietary constituents, and there are secondary changes in the small intestine including a decreased synthesis of enterocyte proteins; bacterial overgrowth in the proximal intestine (SIBO); and malabsorption of vitamins, including cobalamin. Management with uncoated pancreatic extract and a low-fat, high-quality protein diet fed in small, divided meals should be effective in most cases. In animals showing a poor response, additional treatment may be necessary with long-term oral antibiotic for SIBO and H2-receptor blockers before a meal to inhibit acid secretion and minimize degradation of pancreatic extract. Diagnosis of the relatively rare cases of EPI in cats is best achieved by analysis of fecal trypsin by the use of specific substrates until a TLI test becomes readily available, and management should follow similar principles to those established for dogs. The major question for the future is the underlying cause of pancreatic acinar atrophy in dogs, particularly the relative importance of genetic and environmental factors. This information may allow detection and elimination of a genetic abnormality by selective breeding or prophylactic treatment that would prevent the development of the disease.

Repeated or prolonged organic obstruction of the small intestine in the neonatal period can lead to severe refeeding problems, despite a transient ostomy. These problems are thought to result from a postobstructive enteropathy (POE) of the apparently normal small intestine segment above the obstruction. Ten infants with a POE, characterized by limited oral caloric and carbohydrate intakes and increased ostomy effluent, were compared with 8 controls with an enterostomy and a normal postoperative refeeding pattern. There was no statistical difference in the histomorphometric appearance of the mucosa or its digestive or absorptive capacity (brush-border hydrolases, glucose transport) between the two groups. The effluent and duodenal floras of the two groups were similar. However, all POE patients showed significant abnormal peristalsis characterized by barium and carmin transit times. This suggests that repeated or prolonged obstruction in the neonatal period could lead to a POE, caused by chronic motricity abnormalities of the small intestine above the obstruction. Although this POE is more frequent after small bowel atresia, it may also occur with other conditions causing prenatal and postnatal intestinal obstruction.

The finding of extraluminal calcifications is commonly reported and usually indicates intrauterine intestinal perforation with intraperitoneal extravasation of meconium, most often associated with intestinal obstruction and/or atresias. Intraluminal calcification of meconium is more rare and appears to result from the mixing of stagnant urine and meconium in utero. The presence of the intraluminal calcifications in a dilated loop of intestine, particularly with an associated urinary tract abnormality, should suggest a rectourinary fistula. Two cases of prenatally diagnosed imperforate anus with rectourinary fistulae are reported.

Previous work in the fetal lamb examined the relative effects of amniotic fluid and bowel constriction in the etiology of bowel damage in gastroschisis. The present study used the same model to assess the timing and reversibility of these changes during gestation. Gastroschisis was created at 80 days' gestation, and a tape was placed around the bowel to cause gradual constriction with growth. Lambs were killed at 100 days, 120 days, and term. Bowel damage was assessed using histology, mucosal enzyme activity, and in vitro motility. In an additional "repaired" group, the constrictor was removed at 120 days, a silastic pouch placed over the bowel, and bowel damage assessed at term. Normal fetuses at each gestational age were used as controls. A fibrous peel was observed at all gestational ages. Mucosal villous atrophy and mesenteric venous and lymphatic dilation were mild at 100 and 120 days, but severe at term. These changes were present but mild in repaired animals at term. Mucosal enzyme activity decreased gradually with gestational age; inhibition of maltase activity was maximal at term, and was significantly reversed by repair, whereas inhibition of aminooligopeptidase activity was maximal at 120 days, and was not affected by repair. Protein/DNA, DNA/weight, and protein/weight ratios showed that repaired mucosal cells were significantly more proliferative, smaller, and less mature than control or gastroschisis cells. In vitro motility studies demonstrated a mild decrease in contractility at 100 and 120 days, and a large decrease at term. This deleterious effect at the end of gestation was only partially reversed by repair in utero.(ABSTRACT TRUNCATED AT 250 WORDS)

Intestinal intussusception, perforation, necrosis, and massive gastrointestinal bleeding are the surgical conditions most often seen in Henoch-Schönlein purpura (HSP). We reviewed the charts of 183 patients who were diagnosed with HSP at two children's hospitals over a 17-year period. Ten patients underwent laparotomy. Intussusception was the most common surgical lesion. Sequential barium enema and upper gastrointestinal series established the diagnosis of intussusception preoperatively in five of six patients. Two patients developed jejunal perforation and segmental ileal ischemia. Although gastrointestinal bleeding occurred in 52% of patients, the bleeding was self-limiting and no blood transfusion was required. Laboratory data were not helpful in differentiating patients with and without surgical problems.

In a 3-yr period, eight infants among 43 survivors of acute NEC developed intestinal stricture. Four infants developed multiple stricture after proximal diversion procedures, and four had single strictures after medical therapy. Nineteen cases of intestinal stricture after NEC were collected from the literature. Radiographic examinations at the time of the acute disease were not predictive of the risk of subsequent stricture. Histologic examination showed various stages of wound healing, most prominently in the submucosa. Stricture should be considered as the cause of intestinal malfunction in any child who survives acute NEC.

The incidence of postoperative intestinal adhesions and microscopic intestinal injury resulting from the use of overhead infrared heating lamps was studied in 45 rabbits. Exposure of intestine to infrared light for 2 hr or longer at a distance of 1-1/2 ft produced adhesions in 87% of the rabbits, whereas exposure to heat under the same conditions but at a distance of 3 ft resulted in adhesions in 37% of rabbits. The incidence and severity of adhesions correlated directly with the period of intestinal exposure to infrared heat. No histologic evidence of intestinal injury was apparent in intestine exposed to infrared heat under the conditions of this study.

A study to evaluate peritoneal fluid as an index of intestinal gangrene in infants with necrotizing entercolitis (NEC) was begun in 1974. Twenty samples of peritoneal fluid were obtained by paracentesis or lavage from 15 infants with nonperforated NEC. A brown color in the peritoneal fluid was noted in all 8 patients found to have intestinal gangrene at subsequent operation. Gram stain showed bacteria in 6 of these 8 patients and bacterial cultures were confimatory in all but one. In 12 samples of peritoneal fluid in patients without intestinal gangrene, the fluid was straw-colored or pink and Gram stain showed no bacteria. The decision to operate on an infant with intestinal gangrene and impending perforation may be aided by analysis of the peritoneal fluid.

An organized approach to the radiographic analysis of abnormal small bowel based on evaluation of contour and caliber is presented. Diseases affecting the small bowel can be categorized by performing a suggested sequence of observations, and a more specific diagnosis can then be made by integrating clinical information with the radiographic observations.

Gastroschisis is often complicated by damage to the herniated small bowel, resulting in motility and absorption disturbances and occasional intestinal necrosis and atresia. To study the pathophysiology of this process, a model of gastroschisis was developed in fetal lambs. At 80 days' gestation, the anterior abdominal wall was partially excised to create a small peritoneal cavity, and the small bowel was extruded through a Silastic ring to create a defect of uniform size. In one experimental group, a tie was placed around the herniated bowel at the level of the abdominal wall to provide gradual constriction as the fetus grew. In a second group, no tie was placed. Control animals had a simple laparotomy and no abdominal wall defect; some also had a constrictor placed around the base of the bowel. The animals were delivered near term, and the bowel was evaluated histologically and by an in-vitro bowel motility assay. Histologic examination showed normal ganglion cells in all groups and no evidence of ischemic injury. A fibrous peel was seen only in bowel exposed to amniotic fluid, with or without a constrictor. Lymphatic and venous dilation, smooth-muscle thickening, and focal mucosal blunting were seen in bowel subjected to chronic obstruction by a constrictor, regardless of whether it was exposed to amniotic fluid. Both constriction of the bowel and amniotic fluid exposure were associated with a decrease in motility; these two effects were independent and additive.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary cholelithiasis is rare in childhood. A particular etiology has appeared over the last few years in connection with unusual physiological situations. Three cases of cholelithiasis after ileal atresia or resection are presented in children of 1, 1, and 7 yr. Another case is presented after abdominal irradiation for Wilms' tumor and ileal resection in a child of 7. The pathogenesis is discussed, raising the question of the interruption of the enterohepatic circulation of bile salts.

Fortunately, the incidence of serious bowel and conduit problems in the immediate postoperative period and within the first year after diversion is low (5 to 10 per cent). The ileal or colon conduit still serves as the standard method of urinary diversion in adults with pelvic malignancy. Prevention of these complications should truly begin in the preoperative period, and careful judgement should be used postoperatively so that no therapeutic option is undertaken too early. The goal in managing these complications is the preservation of renal function, the maintenance of longest possible amount of functioning bowel, and the absence of indwelling stents and tubes. Patience is needed, along with the maintenance of drainage, adequate nutrition, observation for and treatment of sepsis, and a careful delineation of the anatomic defects. These patients, with their high reoperative mortality rate (approximately 50 per cent), present one of the most intriguing and complicated challenges to the urologist. Using the principles outlined here, we have had only one death in 22 consecutive patients referred to the Lahey Clinic for the management of complex bowel and urinary tract complications following urinary diversion.

During the 7-year period from 1980 to 1986, 56 neonates with gastrointestinal (GI) perforation were treated in the Hospital for Sick Children, London. The overall mortality rate was 30%. The highest mortality rate (60%) was associated with gastric perforation, which in this series occurred only in premature neonates. Colorectal perforation carried the lowest risk of mortality (17%). The mortality rate from small bowel perforation was 35%. Prematurity and low birth weight were significant factors increasing the mortality rate from neonatal intestinal perforation. The predominant cause of perforation in the small and large intestine was necrotizing enterocolitis and the most common site of perforation was the terminal ileum. Mechanical ventilation was thought to be the cause of the perforation in four of the five neonates with gastric perforation. Primary anastomosis is a definite option in neonates who are stable intraoperatively. The mortality rate and in-patient stay for neonates who had primary anastomosis were less than for those who had stomas.

A study to determine the value of contrast enemas in diagnosing and managing intestinal strictures following nonoperative treatment of necrotizing enterocolitis was performed from 1978 through 1983. From 1974 through 1977, 17 patients survived nonoperative treatment of NEC and three developed symptomatic strictures, an incidence of 18% (3/17). Since then a total of 31 infants were treated for NEC; three patients survived operation for perforation and there were seven deaths, leaving 21 in the study group. Sixteen patients had contrast enemas three to six weeks after resolution of NEC, which revealed strictures in five patients. Four of the five patients with strictures demonstrated on contrast enema were without obstructive symptoms. Three of the four remained asymptomatic without treatment, and one eventually required surgery for intestinal obstruction. The fifth patient developed intestinal obstruction while still in the nursery and a contrast study demonstrated an ileal stricture. A sixth patient had a normal contrast study and developed intestinal obstruction from an ileal stricture. The incidence of strictures was 38% (6/16). In five patients, appointments for contrast studies were not kept, although clinical follow-up was complete in all. The incidence of symptomatic strictures for the contrast study period was therefore 14% (3/21). Although some authors have recommended routine contrast enemas in patients surviving nonoperative treatment of NEC, contrast enemas had no advantage over clinical follow-up in the management of patients in this study. We have discontinued the use of routine contrast enemas in favor of close follow-up and careful instruction to parents as to the early signs of intestinal obstruction.