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Background: Gastroesophageal reflux disease (GERD) is very common in patients with chronic lung diseases. We evaluated the incidence of GERD in young patients with cystic fibrosis (CF) and defined the characteristics of gastroesophageal reflux episodes analyzed by pH-multichannel intraluminal impedance (pH-MII) and esophagogastric scintigraphy., Patients and Methods: Since 2010, 31 patients with CF underwent pH-MII. Scintigraphy and upper endoscopy were performed in positive GERD patients. Forced expiratory volume in 1 second (FEV1%) predicted was detected., Results: pH-MII was positive in 17/31 (54.8%) patients (mean age: 12.4 years; range: 4-17 years). pH monitoring detected an average of 64.6 acid reflux events 4.4 episodes >5 minutes in duration. The DeMeester score was 38.5. Impedance identified a mean number of reflux episodes of 66 (65.2% acid; 32% weakly acidic; 2.8% nonacidic), 28% of which reached the proximal esophagus. Esophageal transit and gastric emptying were delayed in 6/13 (46.1%) and in 5/15 (33.3%) cases, respectively. No differences were found in lung function between positive and negative GERD patients (P=0.88)., Conclusions: Pediatric patients with CF have a high incidence of GERD with acidic events. These patients should be investigated with pH-MII and scintigraphy in order to make an early diagnosis and determine the most appropriate follow-up., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Strictures of the extra-hepatic biliary tree are rare in children and have a benign non-traumatic inflammatory origin or are related to idiopathic fibrosing pancreatitis. Primary sclerosing cholangitis (PSC) can manifest as multiple biliary strictures or as a single dominant stricture. We describe the presentation, treatment, and outcome of six cases of isolated benign choledochal stricture (IBCS)., Methods: All patients underwent magnetic resonance cholangiography (MRC). Five patients underwent diagnostic and therapeutic ERCP, and 4 patients underwent intra-choledochal mini-probe EUS and biopsy. Colonoscopy was performed in suspected ulcerative colitis (UC)., Results: We report 6 patients (mean age at diagnosis: four males, 12.1 years; two females, 14.2 years) with IBCS. Clinical onset included 3 cases of acute biliary pancreatitis and obstructive jaundice, one obstructive jaundice, one cholestasis, and one pancreatitis. At diagnosis, MRC confirmed IBCS in all patients. Biliary sphincterotomy, stricture dilation, and stenting were performed in 4 patients. One child underwent hepaticojejunostomy for a type I choledocal cyst. During follow-up (mean: 21 months; range: 1-3 years), all patients were asymptomatic. Four patients developed UC (three pancolitis, one descending colitis). One child developed PSC., Conclusion: IBCS can be successfully treated by therapeutic ERCP. The occurrence of UC could suggest that IBCS is a form of PSC., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Eosinophilic esophagitis (EoE) is a chronic immune/antigen-mediated disease with esophageal dysfunction and eosinophil-predominant inflammation. An association between EoE and gastro-esophageal reflux disease (GERD) has not been well established., Aims: The aim was to evaluate patients with EoE who underwent pH-Multichannel Intraluminal Impedance (pH-MII), investigating proton-pump-inhibitors (PPI) therapy/anti-reflux surgery requirement., Methods: Twenty-five patients [mean age 7.6 (range 1-17 years)] with EoE underwent pH-MII. The children were then divided into Group 1 (pathological pH-MII) and Group 2 (normal pH-MII). PPI was administered for two months in Group 1 and in those children in Group 2 unresponsive to standard EoE therapy (diet and corticosteroids). All patients underwent endoscopy and clinical follow-up. Data are described as mean (range)., Results: Group 1 (n=16, M:F=14:2) had mean reflux index (RI) 13.9% (0.8%-53.4%) with a mean number of total reflux episodes (RE) of 65.8 (14-341). Group 2 (n=9, M:F=6:3) had a mean RI 1.2% (0.2%-2.7%) with a mean number of total RE of 27.4 (14-39). There was a histological response to repeated cycles of PPI in 11/16 (69%) children in Group 1 and 4/9 (44%) children in Group 2. Fundoplication, because of dependence on PPI, was required in 4/11 PPI-responders in Group 1, allowing discontinuation without relapse of EoE., Conclusions: The use of PPI is suggested in EoE at time of diagnosis in addition to standard treatment and may even have benefit in children who do not appear to have significant GERD but are unresponsive to standard therapy., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background/purpose: Fecal incontinence and constipation are common problems in follow-up of anorectal malformations (ARMs). We evaluated the anal sphincters using the 3-dimensional endoanal ultrasonography (3D-EAUS) and the anorectal manometry after ARMs repair., Methods: Seventeen patients, divided into 3 groups according to Wingspread classification, underwent anorectal manometry and 3D-EAUS. Clinical, manometric, and endosonographic scoring systems were used., Results: The average anal resting pressure (aARP) was significantly higher in low ARMs than in intermediate and high ARMs. The anal squeeze pressure was not statistically different between the 3 groups. Three-dimensional EAUS visualized internal anal sphincter (IAS) disruptions in 7 of 17 patients and absence of IAS in 6 of 17 children with high ARMs. Scars of the external anal sphincter were localized in low ARMs and generalized in the other groups. In the case of IAS disruption with aARP greater than 20 mm Hg, fecal incontinence and constipation improved with biofeedback and/or laxatives, whereas daily enemas were necessary in absence of IAS with aARP less than 20 mm Hg. Statistical correlation was observed between endosonographic and manometric findings and clinical outcomes., Conclusions: Lesions of the anal sphincter are common in ARMs. Three-dimensional EAUS and anorectal manometry ensure a complete assessment of the anal sphincter and could provide useful information to define the most appropriate treatments to improve the quality of life., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Management of choledochal cysts consists of surgical excision and hepaticojejunal anastomosis. Endoscopic retrograde cholangiopancreatography (ERCP) can be used to resolve complications and to evaluate the biliary tract and pancreatobiliary duct junction. Our aim was to underline the importance of ERCP for optimal management., Methods: From 2005 to 2011, 28 patients were reviewed (21 female, 7 male; mean age, 5.71 years; range, 2-16 years). After imaging, all patients underwent elective ERCP and were referred for surgery., Results: Choledochal cyst was diagnosed at ultrasound and magnetic resonance cholangiopancreatography in all examined patients; common biliopancreatic duct was diagnosed in 3 (20%) of 15 patients at magnetic resonance cholangiopancreatography and in none at ultrasound. Endoscopic retrograde cholangiopancreatography showed choledochal cyst in all patients and common biliopancreatic duct in 19 (68%) of 28 patients. Twelve patients underwent sphincterotomy. All patients underwent surgical extrahepatic biliary tree resection and hepaticojejunal anastomosis. Mean period of hospitalization was 9.5 days (range, 6-13 days). No major complications related to ERCP were observed. Two patients needed postoperative ERCP for complications (pancreatitis during follow-up)., Conclusions: In our pediatric experience, ERCP is feasible and safe. It can rule out other possible biliary tract anomalies and help plan the timing and choice of the appropriate surgical procedure., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Esophageal stenosis is a severe complication in dystrophic epidermolysis bullosa (EB). Endoscopic dilations may cause mucosal injury with stricture recurrence. Our aim was to describe our referral EB-center experience on safety and long-term efficacy of fluoroscopically guided balloon dilation without endoscopy., Methods: Over 14 years, 34 patients with EB, previously evaluated with barium esophagogram for dysphagia, underwent balloon esophageal dilation. Under fluoroscopy, a guide wire was introduced via a nostril into the stomach. A 12-mm pneumatic balloon, which passed over the wire, was filled using radio-opaque contrast, dilating the stricture. Orotracheal intubation was avoided. Antibiotics, dexamethasone, and proton-pump inhibitors were administered. Study approval was obtained from our ethical board., Results: Ninety-three dilations were performed. Seventeen patients had a single stenosis. The mean age of onset was 18 years (range, 3-47 years). Thirteen patients underwent one dilation. In 6 cases, endoscopy was necessary to visualize the esophageal lumen. Complications included cervical esophageal perforation (2) and transitory dysphagia (10). Thirty patients were feeding within 24 hours. During the follow-up, 2 patients required a gastrostomy, and 2 patients underwent fundoplication for gastroesophageal reflux disease., Conclusions: Fluoroscopically guided balloon dilation in EB is a safe and well-tolerated procedure. An experienced endoscopy team is necessary in certain cases., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Esophageal stenting represents a new strategy to avoid multiple dilations owing to stenosis relapse. Our custom stent improves esophageal motility unlike the widespread self-expandable plastic esophageal stents. The aim of the study was to confirm the efficacy of treatment with silicone custom stents in esophageal stenosis (ES) in pediatric patients., Methods: A silicone stent of 7-, 9-, or 12.7-mm external diameter is built coaxially on a nasogastric tube that guarantees the correct position. The 2 ends are tailored to allow food passage between stent and esophageal wall. All patients received dexamethasone (2 mg/kg per day) for 3 days and ranitidine/proton-pump inhibitors. Study approval was obtained from our ethical board., Results: From 1988 to 2010, 79 patients with ES, mean age 35.4 months (3-125 months), underwent esophageal hydrostatic/Savary dilations and custom-stent placement, left in place for at least 40 days. Stenting was effective in 70 (88.6%) of 79 patients. Fifty percent of the patients with effective treatment received only one dilation for stent placement. Fourteen patients received more stents successfully. There was one stent-related major complication., Conclusion: Our custom stent improves treatment in ES. In caustic injuries, ES stenting represents the first option. In postsurgical ES, we stent after at least 5 dilations., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purposes: Delayed gastric emptying (DGE) is a cofactor in the etiopathogenesis of gastroesophageal reflux disease (GERD). Scintigraphy is the criterion standard to evaluate gastric emptying (GE). This study aims to define typical scintigraphic activity-time curves (ATCs) related to DGE and esophageal atresia (EA) and to demonstrate the effectiveness of pyloromyotomy (P) in improving GE., Methods: Since 2002, 83 children underwent Nissen fundoplication. Patients were divided into 2 groups: group I, GERD-only patients; group II, patients with GERD owing to EA. Depending on preoperative scintigraphy, each group was subdivided into 2 subgroups. Before surgery and 1 year after, endoscopy and scintigraphy were performed. In the presence of DGE, P was associated with Nissen fundoplication. Gastric emptying differences at baseline and at follow-up were estimated by the Student t test. Pre- and post-ATCs were evaluated by the χ(2) test., Results: During follow-up, GE completely normalized in subgroups with DGE. Scintigraphic ATC analysis documented an association between DGE and a typical rectilinear fitting, with a higher rate in EA patients. After P, the scintigraphic pattern changed in an exponential manner related to a faster GE., Conclusions: Delayed gastric emptying is frequent in EA, and the scintigraphic ATCs are typical. Pyloromyotomy is a safe and effective technique to fully normalize GE., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Gastrointestinal duplications (duodenal duplications [DDs]) are a rare congenital malformation generally located in or adjacent to the medial border of the duodenal wall. The goal of therapy is surgical excision. Conservative endoscopic management represents an alternative option., Aim: The aim of the study was to highlight the role of endoscopic ultrasound (EUS) in guiding the endoscopic or surgical treatment of DD., Methods: Between 2002 and 2010, 6 patients (2 male; mean age, 7.83 years; range, 2-18 years), all with recurrent acute pancreatitis, were diagnosed with DD by ultrasound and magnetic resonance imaging. Endoscopy was always performed together with EUS (Olympus UM-3R 20-MHz radial miniprobe, Tokyo, Japan). An endoscopic section of the common duodenal-DD wall, using a precut needle or sphincterotome, was chosen by EUS when the biliary tree was not involved in the DD. Otherwise, surgery with duodenotomy and complete opening of the common wall was used., Results: After EUS evaluation, endoscopic treatment was successfully performed in 4 patients, 2 of whom required surgical treatment. Bleeding occurred in 1 patient after endoscopic resection and in 1 patient after surgery. The mean follow-up time without pathologic signs was 3.3 years (range, 0.25-8)., Conclusions: Endoscopic ultrasound can effectively guide surgical or endoscopic therapies. Bleeding is a possible complication., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Congenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES., Patients and Methods: Patients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary)., Results: Forty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mean age at the diagnosis was 28.3 months (range, 1 day to 146 months). Symptoms were solid food refusal, regurgitation, vomiting, and dysphagia. Congenital esophageal stenosis was located in the distal esophagus. Endoscopic ultrasonography demonstrated TBR and FMH in 6 patients. One hundred forty-eight dilations in 47 patients were performed. The stenosis healed in 45 (95.7%). Complications were 5 (10.6%) esophageal perforations, hydrostatic (3/32, or 9.3%), and Savary (2/116, or 1.7%). At follow-up, 1 patient with FMH CES and 1 patient with TBR CES required operation for persistent dysphagia., Conclusions: The conservative treatment yielded positive outcomes in CES. Endoscopic ultrasonography allows for a correct diagnosis of TBR/FMH CES. A surgical approach should be reserved for CES not responsive to dilations., (Copyright © 2011 Elsevier Inc. All rights reserved.)