A transition clinic model for inflammatory bowel disease between two tertiary care centers: outcomes and predictive factors.

OBJECTIVE: Few models of transition have been proposed for inflammatory bowel disease (IBD). The aim of the present study is to evaluate the feasibility of a transition model and the predictive factors for success/failure. PATIENTS AND METHODS: Patients with low activity or remission IBD were enrolled. Proposed model: three meetings every four-six weeks: the first one in the pediatric center (Bambino Gesù Children's Hospital); the second one, in the adult center (Foundation Polyclinic University A. Gemelli), with pediatric gastroenterologists; the last one, in the adult center, with adult gastroenterologists only. Questionnaires included anxiety and depression clinical scale, self-efficacy, quality of life, visual-analogic scale (VAS). Transition was considered successful if the three steps were completed. RESULTS: Twenty patients were enrolled (range 18-25 years; M/F: 12/8; Ulcerative Colitis/Crohn's Disease 10/10); eight accepted the transition program, four delayed the process and eight refused. Patients who completed transition generated higher scores on the resilience scale, better scores on well-being perception, and had lower anxiety scores. Patients who failed transition were mostly women. The perceived utility of the transition program was scored 7.3 on a VAS scale. CONCLUSIONS: The proposed transition program seems to be feasible. Psychological scores may help in selecting patients and predicting outcomes.

Primary Prophylaxis for Gastrointestinal Bleeding in Children With Biliary Atresia and Portal Hypertension Candidates for Liver Transplantation: A Single-Center Experience.

BACKGROUND: Cirrhosis for biliary atresia (BA) is associated with risk of gastrointestinal bleeding (GB) from gastroesophageal varices due to portal hypertension. Primary prophylaxis of GB is controversial in children who are candidates for liver transplantation (LT). The aim of the study was to define the management of gastroesophageal varices and to identify the benefit of primary prophylaxis for GB in BA children waiting for LT. METHODS: A retrospective single-center study including all BA children listed for LT in 2008-2016. Clinical, endoscopical, and biochemical data were analyzed. RESULTS: Of 82 children, 50 (61%) did not receive primary prophylaxis and did not present any episode of bleeding, 16 (19.5%) underwent primary prophylaxis, and 16 (19.5%) presented spontaneous GB and received secondary prophylaxis. Children without primary prophylaxis and GB were younger than patients with primary prophylaxis and those with GB (7.7 years [range, 4.1-37.9 years] vs 11.2 years [range, 5.1-43 years]; P = .03 vs 10.7 years [range, 6.9-39.9 years], respectively; P = .004). Seventy-five percent of GB occurred in children older than 8 months. Fifteen (93.8%) children with GB presented esophageal varices (grade III = 10 [62.5%]) and 10 (62.5%) required endoscopic treatments, consisting mainly of sclerotherapy. Median time to LT was similar for children with or without bleeding (2 months [range, 0-17.7 months] vs 2.2 months [0-17.9 months], respectively; P = .89). After 45.5 months (range, 13.7-105.5 months) of follow-up, the overall patient survival was 97.6%. At the intention-to-treat analysis, the survival rate was 100% for patients without bleeding episode and 87.5% for children with GB (P = .16). CONCLUSIONS: Despite the risk of GB being not clinically predictable in children with BA waiting for LT, our experience suggests that primary prophylaxis of GB might be unnecessary in children younger than 6 months, while it should be considered in older children. Thus, the occurrence of GB does not delay the timing of transplantation.

European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis - endorsed by the European Academy of Neurology

BACKGROUND AND PURPOSE: Current guidelines on cerebral venous thrombosis (CVT) diagnosis and management were issued by the European Federation of Neurological Societies in 2010. We aimed to update the previous European Federation of Neurological Societies guidelines using a clearer and evidence-based methodology. METHOD: We followed the Grading of Recommendations, Assessment, Development and Evaluation system, formulating relevant diagnostic and treatment questions, performing systematic reviews and writing recommendations based on the quality of available scientific evidence. RESULTS: We suggest using magnetic resonance or computed tomographic angiography for confirming the diagnosis of CVT and not routinely screening patients with CVT for thrombophilia or cancer. We recommend parenteral anticoagulation in acute CVT and decompressive surgery to prevent death due to brain herniation. We suggest preferentially using low-molecular-weight heparin in the acute phase and not direct oral anticoagulants. We suggest not using steroids and acetazolamide to reduce death or dependency. We suggest using antiepileptics in patients with an early seizure and supratentorial lesions to prevent further early seizures. We could not make recommendations concerning duration of anticoagulation after the acute phase, thrombolysis and/or thrombectomy, therapeutic lumbar puncture, and prevention of remote seizures with antiepileptic drugs. We suggest that, in women who have suffered a previous CVT, contraceptives containing oestrogens should be avoided. We suggest that subsequent pregnancies are safe, but use of prophylactic low-molecular-weight heparin should be considered throughout pregnancy and puerperium. CONCLUSIONS: Multicentre observational and experimental studies are needed to increase the level of evidence supporting recommendations on the diagnosis and management of CVT.

Paediatric ulcerative colitis surgery: Italian survey.

BACKGROUND AND AIMS: Recent epidemiological studies showed an increase in ulcerative colitis among children, especially in its aggressive form, requiring surgical treatment. Although medical therapeutic strategies are standardized, there is still no consensus regarding indications, timing and kind of surgery. This study aimed to define the surgical management of paediatric ulcerative colitis and describe attitudes to it among paediatric surgeons. METHODS: This was a retrospective cohort study. All national gastroenterology units were invited to participate. From January 2009 to December 2013, data on paediatric patients diagnosed with ulcerative colitis that required surgery were collected. RESULTS: Seven units participated in the study. Seventy-one colectomies were performed (77.3% laparoscopically). Main surgical indications were a severe ulcerative colitis attack (33.8%) and no response to medical therapies (56.3%). A three-stage strategy was chosen in 71% of cases. Straight anastomosis was performed in 14% and J-pouch anastomosis in 86% of cases. A reconstructive laparoscopic approach was used in 58% of patients. Ileo-anal anastomosis was performed by the Knight-Griffen technique in 85.4% and by the pull-through technique in 9.1% of patients. Complications after colectomy, after reconstruction and after stoma closure were reported in 12.7, 19.3 and 35% of cases, respectively. CONCLUSIONS: This study shows that there is general consensus regarding indications for surgery. The ideal surgical technique remains under debate. Laparoscopy is a procedure widely adopted for colectomy but its use in reconstructive surgery remains limited. Longer follow-up must be planned to define the quality of life of these patients.

Dynamic esophageal stents.

Esophageal stenting represents a new strategy in the treatment of resistant or recurrent stenosis that obviates the need for multiple dilations. Our custom dynamic stent (DS) improves esophageal motility unlike the widespread self-expandable plastic or metallic esophageal stents. The DS allows food and secretions to pass in the space between the esophageal wall and the stent wall. This contrasts with the other types of stent, in which food passes into the stent that presses into the esophageal wall. Until the stent patent is complete, we use slices of silicon drains overlapped with each other to fashion the stent to the desired length and diameter (7-, 9-, or 12.7-mm external diameter). It is built coaxially on a nasogastric tube that guarantees the correct position. The two ends are tailored to allow an easy introduction and food passage between stent and esophageal wall. The stent is inserted after stricture dilations (Savary-Gilliard dilators) under fluoroscopic guidance. All patients who underwent stenting were treated with dexamethasone (2 mg/kg/day) for 3 days and proton pump inhibitors (omeprazole or lansoprazole, 1-2 mg/kg/day). From 1992 to 2012, 387 patients (mean age 38.6 months; range 3-125 months) with post-surgical esophageal stricture because of esophageal atresia correction were enrolled in this study. Twenty-six of 387 patients (6.7%) underwent custom DS placement for recurrent stricture instead of a program of serial dilations. The stent was left in place for at least 40 days and was effective in 21 (80.7%) of 26 patients. There were two stent-related major complications (subclavian-esophageal fistula). Our custom stent represents an effective and safe option in the treatment of severe and recurrent post-surgical esophageal strictures. Surgery with stricture resection, and reanastomosis or jeunoplasty represents the rescue strategy.

ERCP with intracholedocal biopsy for the diagnosis of biliary tract rhabdomyosarcoma in children.

Rhabdomyosarcoma is the most common tumor of the biliary tract in children. Although some features at preoperative radiographic studies (ultrasound, CT, MRI) may be suggestive of BT-RMS, until few years ago the final diagnosis was obtained by either operative or transcutaneous biopsy, thus exposing to a risk of regional dissemination. More recent and still anecdotal, is the histological diagnosis on tissue obtained by transluminal biopsy either during transhepatic cholangiography or endoscopic retrograde cholangio-pancreatography (ERCP), the latter having the major advantage of a much lower risk of loco-regional dissemination. We present two cases of BT-RMS that were histologically diagnosed by intracholedocal biopsy performed during ERCP, after being suspected at conventional imaging.

Utility of a stool antigen test to detect the incidence of helicobacter pylori infection and familial and community enviromental risk factors for this infection in pediatric age.

BACKGROUND: Helicobacter pylori (Hp) infection is mainly acquired during childhood; it is recognised as a cause of gastritis and peptic ulcer and it has been classified as a group A carcinogen by World Health Organization. The exact mode of transmission is as yet, not known. Aim of our study has been to identify risk factors associated with Helicobacter pylori infection in a preschool and school population and to confirm if Hp antigen in faeces is useful as screening in epidemiological studies. METHODS: We interviewed, with questionnaire, 400 children (203 male; age range 3-10 years; mean age 6 years) of 3 different schools and stool samples were collected of all children too. 35 of 400 (8%) children underwent to upper gastrointestinal endoscopy because of a suspect of upper gastrointestinal disease. RESULTS: stool were collected from 400 school children and 35 of them shown positivity of Hp antigen test. A questionnaire about presence of nausea, vomit, recurrent abdominal pain, family size, parent's occupations and education, use of antibiotics, country of birth of child and parents, personal hygiene, breast feeding, presence of the animals was completed. 35 children with positive Hp stool antigen test and a suspicious of upper gastrointestinal disease (recurrent abdominal pain, diurnal or nocturnal abdominal pain, nausea, vomiting, iron deficiency) underwent to esophagogastroduodenoscopy (EGDS) that demonstrated antral gastritis and positive histology and urease rapid test. CONCLUSIONS: the results of this study suggest that risk factors for Hp infection are low socioeconomics factors, hygiene and living conditions and that Hp antigen in faeces is useful as screening test.

Indications to upper gastrointestinal endoscopy in children with dyspepsia.

OBJECTIVES: The objective of the study was to ascertain the appropriateness of indications for upper gastrointestinal (UGI) endoscopy in children with dyspepsia. METHODS: We used the RAND/University of California at Los Angeles method to investigate the appropriateness of the opinions of a panel of experts. The panel judged 2304 theoretical patient scenarios defined by a combination of demographic and clinical variables. Descriptive and multivariate logistic regression analyses were performed. RESULTS: The panel rated UGI endoscopy as appropriate in 27.2% of cases, inappropriate in 14.3%, and dubious in 58.5%. Disagreement emerged for 21% of cases. UGI endoscopy was considered increasingly appropriate in cases with a positive family history of peptic ulcer and/or Helicobacter pylori infection (odds ratio [OR] 8.518, P < 0.0001), when dyspepsia interfered with activities of daily living ("sleep" OR 7.540, P < 0.0001; "normal activities" OR 5.725, P < 0.0001), and when patients were older than 10 years ("

Eosinophilic esophagitis: management and pharmacotherapy.

Eosinophilic esophagitis represents the most debated disease of the last 10 years, too often speculated or overestimated and certainly well known and examined. The aim of this study was to summarize the recent therapeutic trends in order to show persistent doubts regarding several debated therapies. The study combined the most recent international literature and the authors' daily experience to define the scope of the review, with limits caused by a lack of available randomized studies between dietetic and pharmacological treatment. It was concluded that eosinophilic esophagitis is an immunoallergic disease that is generally caused by identifiable food and environmental allergens although, in a minority of cases, the etiological trigger remains undetermined. Therapy usually fights the responsible agents, but sometimes they are not resolved. A need for more pathogenetically driven treatments is invoked.

Reflux oesophagitis in children; the role of endoscopy. A multicentric Italian survey.

BACKGROUND: The decision whether to perform endoscopy in children with suspected reflux oesophagitis is not a straightforward one. Few symptoms are specific for oesophagitis and the diagnosis is not always correlated even to visual findings on endoscopy. AIM: The aim of this study was to define the role of endoscopy and especially of histology in the diagnosis of reflux oesophagitis and to examine the correlations between symptoms, endoscopic findings and histology in children with suspected gastroesophageal reflux disease. PATIENTS AND METHODS: One hundred and thirty-six patients with a clinical diagnosis of reflux oesophagitis, aged 1-18 years (mean 8.43; standard deviation +/-4.4), were enrolled from 12 Italian Paediatrics Gastroenterology Centres; symptom score, endoscopic and histologic oesophagitis scores were observed before and after therapy with proton pump inhibitors. RESULTS: Before therapy, a high correlation between the prevailing symptom score and endoscopic score was demonstrated, but not with histologic score: there was a significant tendency for histologic grade to exceed visual findings. After therapy, endoscopic score and histologic score were significantly improved. CONCLUSIONS: Oesophageal biopsies increase the diagnostic accuracy of upper endoscopy. Histologic grading is often much more important than the endoscopic appearance, so that endoscopic oesophageal biopsies are very important aids in the diagnosis of oesophagitis. Appropriate clinical evaluation of symptoms must occur before endoscopic examination.

Paediatric eosinophilic oesophagitis: towards early diagnosis and best treatment.

UNLABELLED: Eosinophilic oesophagitis is an emerging disease, well known also in paediatric age, probably caused by both IgE and non-IgE mediated food allergies, diagnosed by upper endoscopy with biopsy. The most severe complication is oesophageal stenosis. The identification of the offending allergens is often difficult; therapy is focused to eliminate the supposed antigenic stimulus, to control the acute symptoms and to induce long-term remission. AIM: We report the clinical outcome and the typical endoscopic findings of children and adolescents affected by eosinophilic oesophagitis, referring a proposal of diagnostic and treatment protocol. PATIENTS AND METHODS: Twelve patients, affected by eosinophilic oesophagitis with a histological diagnosis, underwent radiographic upper gastro-intestinal series, 24 h pH-probe and standardised allergic testing; they were treated with steroids (oral prednisone and swallowed aerosolised fluticasone) and elimination diet. Dilations were performed when eosinophilic oesophagitis was not yet diagnosed, or in patients resistant to conventional treatment. RESULTS: Two patients were lost to follow up (mean follow up: 1 year 11 months); seven patients have no symptoms and normal histology, five of them on restricted diet (without cow's milk protein) and two patients on elemental diet (amino acid formula). In two patients (no allergens identified), mild dysphagia and eosinophilic infiltration persist; one patients underwent Nissen fundoplication for Barrett's oesophagus: he has no symptoms and normal oesophagus, on restricted diet (without cow's milk/eggs protein and wheat). CONCLUSION: The recognition of typical endoscopic picture with careful biopsies extended to the whole oesophagus, even in emergency, could more quickly lead to the correct diagnosis and avoid severe complications of eosinophilic oesophagitis in children, as stricture and failure to growth. Elimination diet is the key of resolution when the allergens are identified. A great challenge remains the relation between gastro-oesophageal reflux disease and eosinophilic oesophagitis, which should however be explained.

Long gap esophageal atresia and esophageal replacement: moving toward a separation?

BACKGROUND/PURPOSE: Treatment of long gap esophageal atresia (EA) is still a major challenge. Gastric transposition and colon interposition are the 2 most popular choices for esophageal replacement, but there is general agreement that the child's own esophagus is the best. The aim of the study was to critically evaluate the feasibility and outcome of primary repair of long gap EA with or without tracheoesophageal fistula (TEF) by direct esophago-esophageal anastomosis as the only technique. METHODS: Seventy-one neonates with EA+/-TEF were considered. Nineteen cases were classified as long gap (> or =3 cm). All infants underwent either primary or shortly delayed repair. In the latter group, a gastrostomy was performed along with an x-ray evaluation of the gap a few days before surgery (mean age, 46.4 days). To avoid disruptive anastomotic force, all infants were kept paralyzed and mechanically ventilated for an additional 6 days after esophageal anastomosis. Before starting feeding, postoperative esophagogram was done on day 7. Endoscopy was done routinely, starting 1 month after surgery; pH monitoring was conventionally performed at 1 year of age or even earlier, should gastroesophageal reflux disease (GERD) be suspected. Follow-up ranged from 11 months to 7 years. RESULTS: In all 19 long gap EA infants an esophago-esophageal anastomosis was performed. Six of them (31%) required an anterior esophageal flap to bridge residual gap. Complications included minor anastomotic leak in 2 cases and anastomotic stricture (<5mm) in 12 (80%) cases, which were treated with an average of 5 dilatations (1 of which with resection of the stricture). GERD occurred in 8 cases (53.3%), of which, 3 required fundoplication. None of the patients had esophageal swallowing difficulties or persistent dysphagia. Two children experienced food aversion. Mean hospital stay was 66.2 (22 to 230) days. There were 4 deaths (very low birth weight, 1; associated anomalies, 1; and late sepsis, 2). CONCLUSIONS: Considering heat gap determination remains imprecise, it seems possible to conclude that in a well-established tertiary care level referral center: (1) long gap EA could be treated successfully with primary repair and anastomosis; (2) strictures and GER represent the most frequent postoperative problem, but additional procedures required seem "acceptable" to maintain the patient's own esophagus and avoid replacement; (3) esophageal substitution in long gap EA should be reserved for cases in which a previous attempt of esophageal reconstruction failed.

Strictureplasty: An alternative approach in long segment bowel stenosis Crohn's disease.

BACKGROUND/PURPOSE: Intestinal resection is the most frequent surgical procedure for bowel stenoses in Crohn's disease (CD). Recurrence of strictures, particularly with ileocolonic disease, often requires resection of lengthy segments of bowel, potentially resulting in short bowel syndrome. Different techniques of strictureplasty, such as those described by Mikulicz, Finney and Michelassi, are used in adults. However, these procedures are uncommon in pediatric surgery. The authors report their experience with different techniques of strictureplasty and with their modified Michelassi technique for the surgical treatment of long intestinal strictures caused by CD. METHODS: Five adolescents (2 boys; 3 girls; mean age, 16 age; range, 14 to 20 years) with severe ileocolonic stenoses and intestinal obstruction, not responsive to medical and nutritional therapy, were treated with different strictureplasty techniques. In 3 of them the modified side-to-side Michelassi technique was used. RESULTS: No postoperative complications occurred. After a mean follow-up of 20.5 months (range, 6 to 28 months), patients are free of symptoms with good nutritional status and off steroid therapy. CONCLUSIONS: Strictureplasty is a good and effective surgical option for sparing bowel length in CD patients with extensive intestinal strictures.

Combined approach to functional constipation in children.

BACKGROUND/PURPOSE: The authors' 15-year experience with children shows a high percentage of recurrence of functional constipation (FC) with conventional treatment. These data, confirmed in the international literature, led them to develop a new therapeutic approach. The aim of this study was to achieve intestinal control and avoid recurrence of FC in children through use of medical-psychological treatment. METHODS: The authors studied 25 children (18 boys; mean age, 4.7 years; range, 2.10 to 7), 20% of whom had anal fissure, 30% retentive soiling, 52% pain on defecation, and 88% fecal retention owing to FC. Children and parents were questioned about eating and sleeping habits, school, toilet training, and daily routine. Treatment included increasing water and fiber intake, laxatives, and family therapy including making rules and working on autonomy and paternal role. RESULTS: Mean onset of FC was 3.5 years, after "stressful events" in 88%. The questionnaire shows that 68% lacked parental autonomy and authority; 84% of children decided on their own about eating habits and sleeping; 68% had a "peripheral" father with a mother-child symbiotic relationship. After one month of therapy, 92% of children showed a modification of at least 2 behavioral patterns; after 3 months, 88% had regular bowel movements. During follow-up (range, 6 to 28 months), 48% had 2 or 3 recurrent episodes. After one year, 68% had reinforced the new behavioral patterns with resolution of the pathologic aspects. CONCLUSIONS: A multidisciplinary approach in the treatment of childhood functional constipation showed consistent therapeutic results by making rules and by equalizing family roles.

[Ascariasis as a cause of acute abdomen: a case report]. / Ascaridiasi come possibile causa di addome acuto anche in Italia: presentazione di un caso clinico.

Ascaris Lumbricoides Infestation (ALI) is one of the most common helmintic disease of the gastrointestinal tract, and may cause severe surgical complications, especially in children. ALI is frequent in tropical and subtropical countries. We present our experience with a case of a 5-years old pakistan girl treated in Italy for acute abdomen in which ALI was detected during surgical exploration.

Esophagogastric dissociation versus fundoplication: Which is best for severely neurologically impaired children?

PURPOSE: Neurologically impaired children (NIC) often have swallowing difficulties, severe gastroesophageal reflux, recurrent respiratory infections, and malnutrition. Bianchi proposed esophagogastric dissociation (EGD) as an alternative to fundoplication and gastrostomy. The authors compared these 2 approaches. METHODS: Twenty-nine consecutive symptomatic NIC refractory to medical therapy were enrolled in a prospective study and divided into 2 groups: A (n = 12), NIC who underwent fundoplication and gastrostomy; B (n = 14), NIC who underwent EGD. Three were excluded because of previous fundoplication. Anthropometric (percentage of the 50th percentile/age of healthy children) and biochemical parameters, respiratory infections per year, hospitalization (days per year), feeding time (minutes), and "quality of life" (parental psychological questionnaire, range 0 to 60), were analyzed (t test and Mann-Whitney test) preoperatively and 1 year postoperatively. Complications were recorded. RESULTS: Compared with group A, group B presented a statistically significant increase of all anthropometric and nearly all biochemical parameters with a statistical difference in terms of respiratory infections, hospital stay, feeding time, and psychological questionnaire. In group A, 2 bowel obstructions, 1 tight fundoplication, 1 dumping syndrome, and 3 failures of fundoplication occurred. Group B presented 1 anastomotic stricture, 1 paraesophageal hernia, and 1 bowel obstruction. CONCLUSIONS: Compared with fundoplication and gastrostomy, EGD offered better nutritional rehabilitation, reduction in respiratory infections, and improved quality of life. EGD can be rightfully chosen as a primary procedure.