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Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS.Clinical, surgical and psychological data were collected for all patients with diagnosis of CRS, treated at our Pediatric Hospital from January 1995 to December 2020. Patients with a severe form of CRS according to Pang's classification were included in the study. All patients have been followed by a multidisciplinary team composed by urologists, neurosurgeons, neonatal and digestive surgeons, orthopedic surgeon, physiatrists and psychologists. On admission, patients were screened for CRS with sacrum, spine and legs X-ray, spine MRI, renal ultrasound, voiding cystography and urodynamic, and renography. Clinical data about bowel function were evaluated.During the study period, 55 patients with CRS were treated at our Institution. Six out of 55 (10.9%), presented with severe form of CRS (5 pts with type 1; 1 pt, with type 2) and represent our study group. Diagnosis of severe CRS was made at birth because of the typical deformities of the pelvis (fusion of the iliac wings), and of the lower extremities (undeveloped legs with flexion of the knees, clubfoot). All patients presented with neurogenic bladder, 4/6 (66.6%) with vesicoureteral reflux (VUR) and 2/6 (33.3%) with renal agenesia and contralateral ectopic hypoplastic kidney. Bowel symptoms occurred in 5/6 pts (83.3%). All patients were started with an earlier clean intermittent catheterization (CIC) regimen. In 3 patients oxybutynin has been effective or well tolerated, while in other 3 onabotulinumtoxin A has been used. Vesicoureteral reimplantation has been performed in 1 patient, 2 required endoscopic treatment of VUR. On long-term, 2 patients required bladder augmentation with ileum and appendicostomy (Mitrofanoff). Low adherences in CIC have been observed in three patients, mainly at puberty. Two patients presented with chronic renal failure. One patient reported suicide proposal. Regarding social life, only one is regularly performing sport activity.CRS is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. Management of CRS includes a wide variety of interventions to address the full spectrum of possible anatomical abnormalities. Hence, a multidisciplinary team is also mandatory for a correct bladder and bowel management, in order to maintain continence and preserve renal function, improve quality of life and increase self-esteem.

Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported.To share our experience and to discuss the management of ARM-UD-OSD association.We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation.All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy).UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Sigmoid volvulus (SV) is a rare cause of intestinal obstruction in children. Its varied presentation and rapid progression engender a high risk of morbidity and mortality. We report two cases of SV in teenage boys. Patient 1 is 16 years old and patient 2 is 17. Both presented to our institution with recent-onset abdominal pain, constipation, and nausea and vomiting, and both had previous episodes of SV. Patient 1 had been surgically treated with manual derotation, and patient 2, who had redundant colon, had two past episodes of endoscopically-treated SV. Both patients were in poor condition and had distended but treatable abdomens, with no peritoneal signs. After computed tomography (CT) confirmation of the clinical impression of no ischemia or perforation, we decided to attempt detorsion of the SV and decompression of proximal dilated colon by flexible endoscopy, and planned for elective surgery shortly after the endoscopic procedure. Because sigmoid volvulus is so rare in children, operative and technical details of endoscopic management are gleaned from the larger adult experience. In patients without signs of complication, initial endoscopic reduction is the gold standard, and elective sigmoid resection with primary anastomosis is often required to prevent recurrence. KEY WORDS: Case report, Children, Endoscopy, Endoscopic treatment, Sigmoid volvulus, Volvulus.Il Volvolo del Sigma (VS) è una rara causa di occlusione intestinale nel paziente pediatrico. La sua modalità di presentazione e la rapida progressione del quadro clinico comportano un elevato rischio di morbidità e mortalità. Riportiamo due casi di volvolo del sigma in pazienti adolescenti. Il paziente 1 ha 16 anni mentre il paziente 2 ha 17 anni. Entrambi sono giunti alla nostra osservazione con un quadro di dolore addominale acuto, nausea, vomito e occlusione intestinale. Entrambi i pazienti avevano già presentato episodi di VS in precedenza. Il paziente 1 è stato sottoposto in precedenza ad intervento chirurgico per derotazione manuale, mentre il paziente 2 presenta un sigma ridondante e ha presentato 2 episodi di volvolo trattati endoscopicamente. Entrambi i pazienti si presentavano in condizioni generali scadenti con un addome disteso ma trattabile senza segni di reazione peritoneale. E’ stata eseguita una Tomografia Computerizzata a conferma del sospetto diagnostico e al fine di escludere segni di ischemia e perforazione. Abbiamo quindi tentato un approccio non chirurgico in assenza di segni ischemia e perforazione mediante l’utilizzo di endoscopio flessibile e abbiamo programmato l’intervento chirurgico in regime di elezione a breve distanza dall’evento. Il VS in età pediatrica è raro e i dettagli tecnici e operativi del trattamento endoscopico sono ereditati dall’esperienza nel paziente adulto. In assenza di segni di complicanze, l’approccio endoscopico al paziente pediatrico con VS è ritenuto il gold standard, e la resezione endoscopica con anastomosi primaria è spesso richiesta per evitare la ricorrenza di recidive.

Epidermolysis bullosa (EB) comprises a group of genetic disorders with the hallmark of fragility of the skin and mucosal surfaces. The severity of different types of EB varies markedly as does the occurrence of extra-cutaneous involvement and complications. A number of emergency situations may occur in the context of EB including obstruction to oral intake from oral or esophageal blisters or scarring, acute airway obstruction, acute urinary retention, sepsis and corneal erosions. Whilst general management principles apply in each of these settings, specific considerations are essential in managing EB to avoid undue trauma or damage to delicate tissues. These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.

Video Video 1 Direct cholangioscopy for pancreatobiliary maljunction.

Venous malformation (VM) is the most common type among vascular malformations classified by the International Society for the Study of Vascular Anomalies. Most VMs are sporadic (94%), caused in 40% of cases by somatic mutation of TEK gene. VMs can be cutaneous, visceral, or combined. Visceral involvement is rare, and gastrointestinal (GI) tract is the most common localization. Visceral VMs, usually asymptomatic, may manifest with bleeding, anemia, and consumptive coagulopathy, which sometimes require an emergency treatment. Our aim is to study the possible GI involvement in patients with only one cutaneous VM. We analyzed a series of six patients who presented with a single cutaneous VM and have subsequently manifested intestinal involvement at our reference center for vascular anomalies since 2010. In our patients, cutaneous VMs were located on lower or upper limbs, and GI involvement manifested from 3 to 10 years after skin diagnosis. Our experience urges to early diagnose a GI involvement also in patients with only one skin VM and to prevent severe complications. A multidisciplinary approach is mandatory for the diagnosis and treatment of these patients.

Objective To analyze the findings of both multichannel intraluminal impedance with pH (MII-pH) and endoscopy/histopathology in children with esophageal atresia at age 1 year, according to current recommendations for the evaluation of gastroesophageal reflux disease (GERD) in esophageal atresia. Study design We retrospectively reviewed both MII-pH and endoscopy/histopathology performed in 1-year-old children with esophageal atresia who were followed up in accordance with international recommendations. Demographic data and clinical characteristics were also reviewed to investigate factors associated with abnormal GERD investigations. Results In our study cohort of 48 children with esophageal atresia, microscopic esophagitis was found in 33 (69%) and pathological esophageal acid exposure on MII-pH was detected in 12 (25%). Among baseline variables, only the presence of long-gap esophageal atresia was associated with abnormal MII-pH. Distal baseline impedance was significantly lower in patients with microscopic esophagitis, and it showed a very good diagnostic performance in predicting histological changes. Conclusions Histological esophagitis is highly prevalent at 1 year after esophageal atresia repair, but our results do not support a definitive causative role of acid-induced GERD. Instead, they support the hypothesis that chronic stasis in the dysmotile esophagus might lead to histological changes. MII-pH may be a helpful tool in selecting patients who need closer endoscopic surveillance and/or benefit from acid suppression.

Background:Since the esophagus has no redundancy, congenital and acquired esophageal diseases often require esophageal substitution, with complicated surgery and intestinal or gastric transposition. Peri-and-post-operative complications are frequent, with major problems related to the food transit and reflux. During the last years tissue engineering products became an interesting therapeutic alternative for esophageal replacement, since they could mimic the organ structure and potentially help to restore the native functions and physiology. The use of acellular matrices pre-seeded with cells showed promising results for esophageal replacement approaches, but cell homing and adhesion to the scaffold remain an important issue and were investigated.Methods:A porcine esophageal substitute constituted of a decellularized scaffold seeded with autologous bone marrow-derived mesenchymal stromal cells (BM-MSCs) was developed. In order to improve cell seeding and distribution throughout the scaffolds, they were micro-perforated by Quantum Molecular Resonance (QMR) technology (Telea Electronic Engineering).Results:The treatment created a microporous network and cells were able to colonize both outer and inner layers of the scaffolds. Non seeded (NSS) and BM-MSCs seeded scaffolds (SS) were implanted on the thoracic esophagus of 4 and 8 pigs respectively, substituting only the muscle layer in a mucosal sparing technique. After 3 months from surgery, we observed an esophageal substenosis in 2/4 NSS pigs and in 6/8 SS pigs and a non-practicable stricture in 1/4 NSS pigs and 2/8 SS pigs. All the animals exhibited a normal weight increase, except one case in the SS group. Actin and desmin staining of the post-implant scaffolds evidenced the regeneration of a muscular layer from one anastomosis to another in the SS group but not in the NSS one.Conclusions:A muscle esophageal substitute starting from a porcine scaffold was developed and it was fully repopulated by BM-MSCs after seeding. The substitute was able to recapitulate in shape and function the original esophageal muscle layer.

Objectives The aim of this study was to ascertain predictors of survival, liver disease (LD), and enteral autonomy 48 months after resection in neonatal short bowel syndrome (SBS) patients with residual small bowel length (SBL) ≤40 cm. Patients and Methods Medical records of all SBS patients followed up between 1996 and 2016 were retrospectively reviewed. Survival rate, prevalence of LD, and of enteral autonomy were evaluated. Results Forty-seven patients were included, and 43 were still alive at the end of the study period, with cumulative 48-month survival of 91.5%. Twenty-one (45%) patients developed LD, all within the first 6 months. On the final follow-up visit, three (6%) patients were still jaundiced and progressed toward end-stage LD. LD prevalence was higher in patients with recurrent bloodstream infections (odds ratio [OR] 5.4, 95% confidence interval [CI] 1.5–19.3). Of the 43 surviving patients, 22 (51%) had enteral autonomy 48 months after resection. The probability of weaning off parenteral nutrition (PN) was strongly correlated with the remaining SBL. Conclusion Survival of patients who have undergone neonatal massive small bowel resection has improved in recent years. Multidisciplinary strategies can improve the course of LD, but not the probability of weaning off PN, which seems to be strongly dependent on the anatomical profile of residual bowel. Therefore, the primary surgical approach should be as conservative as possible to gain even small amounts of intestinal length, which may be crucial in promoting intestinal adaptation.

Background Esophageal atresia (EA) is the most common congenital anomaly of the esophagus. Anastomotic strictures (AS) frequently occur in patients surgically treated for esophageal atresia (EA). The primary aim of this study is to determine the role of esophageal dilations in the management of AS in childhood after reconstruction of EA. Methods A retrospective chart review of patients treated with esophageal dilation for EA was conducted at our tertiary referral center from 2013 to 2017. We included patients treated at our Institution since diagnosis and patients referred from other Institutions. All dilations were performed with Savary-Gilliard polyvinyl bougies. Dilation was performed on an ‘as needed’ basis. Results Eighty-nine patients (68.5% males, 31.5% female) underwent 433 dilations overall in the study period (median: 3, range: 1–36). Type I of EA was 26.1%, Type II was 9.1%, Type III was 64.8%. Associated malformations in 52.8% cases. Six patients received 33 local applications of mitomycin C (MMC) and two of triamcinolone acetate for recurrent stenosis. Four patients developed a preanastomotic diverticulum-treated endoscopically. Nine patients (10.1%) underwent dynamic esophageal stent placement after a median of six dilations. Antireflux surgery was performed in 28 patients (31.5%), Nissen fundoplication in 20 patients, Toupet in 6, and both in 2. Patients treated with antireflux surgery received a mean of 7.1 dilations before surgery, versus 3.9 in patients without antireflux surgery (P = 0.0285, unpaired t test). Seven patients (7.9%), 5 referral, needed major esophageal surgery (4 esophageal reanastomosis and 3 esophagocolonplasty). Patients underwent major surgery received a mean of 19.3 dilations versus 3.6 dilations in no-surgery patients (P = 0.0002, Mann-Whitney U test). Six of 9 stented patients did not require surgery. Perforation was present in 0.4% of 433 dilatations. Conclusions Esophageal dilatation remains the mainstay of treatment for AS after EA repair. Stricture resection with end-to-end anastomosis is the reconstructive option of choice; esophageal substitution is reserved only in cases of intractable stricture. The use of esophageal dynamic stent, MMC, or steroids could help in avoiding or delaying the need of operative stricture resection. Antireflux surgery is mandatory in case of severe pathological gastroesophageal reflux to prevent intractable strictures from developing.

Introduction The usefulness of positive expiratory pressure (PEP) through a PEP valve to enhance expiratory flow and improve cough efficiency is well known in children with tracheoesophageal fistula (TEF) and residual tracheomalacia. Gastroesophageal reflux (GER) is one of the most frequent complications encountered in patients with repaired esophageal atresia (EA) ± TEF. The impact of airway clearance techniques with a PEP valve on GER is not well defined in this group of patients. The aim of this prospective study was to determine the effects of PEP chest physiotherapy on GER burden measured by multichannel intraluminal impedance and pH monitoring (MII-pH) in EA/TEF children. Since the study is still ongoing, preliminary results are hereby presented. Methods EA/TEF patients with tracheomalacia received three 20-min sessions of PEP chest therapy during the 24-hour MII-pH monitoring performed as a routine care. All children were off acid suppression therapy. The PEP session, performed after a 3-hour fasting period, consisted of 10 cycles lasting 1 minute of breathing with a PEP valve, followed by the forced expiratory technique and coughing. Esophageal acid exposure time (AET) and number of reflux episodes (RE) during each PEP session were measured as primary outcomes. Baseline impedance (BI) was also evaluated. MII-pH was defined abnormal if AET was >7% and/or the total RE was >70. Results From January and December 2018, 5 consecutive male EA/TEF children (mean age 8.5 years; range 3–14 years) were enrolled. All of them showed low distal BI (1040 ± 143.2) and normal MII-pH findings (AET: 1.8 ± 2.9%; number of RE: 20.2 ± 9.0). Over the period of the PEP sessions, no significant changes in AET (0.0 ± 0.0), RE (0.6 ± 0.5), and BI were detected compared with the background monitoring. Conclusions Although the limited number of participants prevents any definitive conclusions, our preliminary results indicate that PEP chest physiotherapy does not exacerbate GER in EA/TEF children. Continuation of the study across a larger sample of EA/TEF patients is needed to better define the effects of this simple chest physiotherapy technique on GER burden.

Aim of the Study Barrett's esophagus (BE) could be a rare late complication in children with repaired esophageal atresia, partly due to a long-term history of gastro-esophageal reflux disease (GERD). Radiofrequency ablation (RFA) is the best endoscopic technique in adults with BE and dysplasia. Guidelines do not support this approach for non-dysplastic BE. Aim of this study is to present our experience as Tertiary Gastroenterology referral Centers in the treatment of children and young adults with BE using RFA. Methods A multicenter retrospective study conducted on children with BE, subjected to RFA at the Digestive Surgery and Endoscopy Unit, Bambino Gesù Children's Hospital and the Department of General Surgery ‘Paride Stefanini’, Sapienza University of Rome, between June 2012 and December 2017.Demographic and clinical data were collected. BE characteristics according to Prague classification, presence and grade of dysplasia, number of RFA treatments, complications, clinic and endoscopic outcomes were described. All patients were treated with HALO RF BARRXtm (HALO 360 Covidien, Sunnyvale, California, USA). Endoscopic evaluation and treatment were performed every 3 months until complete eradication. Endoscopic follow-up was: 6 months after eradication, and then annually. Results Five patients were evaluated (M:5). Mean age at diagnosis was 14.4 years (range: 9–17); mean age at first treatment was 23 years (range: 13–36). Two patients presented with repaired esophageal atresia (long gap, type 3 atresia), 3 with peptic strictures. All patients exhibited a long-term history of symptomatic GERD and PPI dependency. Four patients underwent Nissen fundoplication before BE diagnosis. All patients presented with long segment BE (mean baseline length was 8,8 cm, range 7–10 cm). According to the Prague classification, patients were classified as follows: C0M7, C0M17, C8M10, C0M9 and C4M8. One patient presented with LGD. Patients received a mean of 3 HALO treatments (range 1–4 sessions). Eradication was 100% during 2- to 6-year follow-up (mean 4.5 year). No complications occurred. Conclusions Pediatric patients with EA present an high risk to develop early BE, therefore endoscopic surveillance is mandatory. RFA represents a safe and effective treatment option in patients with early onset BE. Treatment of BE with this technique could be a useful and minimal invasive strategy for dysplasia and cancer prevention.

Introduction Since recently, after detection of eosinophilic predominant inflammation of esophagus a trial with proton-pump inhibitors (PPIs) was needed to individuate children with PPI-responsive esophageal eosinophilia (PPI-REE), only those non-responders received eosinophilic esophagitis (EoE) diagnosis. In 2018 updated international consensus suggested removing the PPI trial as a diagnostic criterion and consider PPIs as a treatment together with diets and topical steroids. The role of PPIs is evaluated in children with esophageal atresia (EA) and EoE versus EoE from general population. Method A retrospective chart review of both children with EA and EoE followed-up from at January 2005 has been performed. According to ESPGHAN guidelines published in 2014 patients showing eosinophilic inflammation received high-dose PPI trial to identified PPI-REE. Those non-responders were labeled as EoE and underwent to dietary and/or topical steroid treatment. Demographics and disease characteristics of EA patients with EoE were analyzed and compared with those with EoE from general population. Results Overall, 370 EA and 118 EoE patients were analyzed. Of them 15 EA-EoE patients were detected. Consequently, in our cohorts, 4.0% of EA patients developed EoE. Male-to-female prevalence ratio was of 2.55 with no difference in gender prevalence between groups. At diagnosis EoE-EA children were significantly younger compared to EoE group (mean: 5.1 vs 10.8 years; P Conclusion Similar gender distribution and high prevalence of allergy suggest that common genetic susceptibility factors for EoE exist. However, high prevalence of PPI-REE coupled early EoE onset might also suggest that other factors (e.g. esophageal motility disorders) might play a physiopathological role in EoE development in EA children. Our study suggests that a stepwise approach with PPIs as a first-line treatment for EoE management in EA children should still be considered.

OBJECTIVE The aim of the study was to evaluate long-term nutritional outcomes and clinical characteristics in a cohort of children with pediatric intestinal pseudo-obstruction (PIPO) at neonatal-onset (NO-PIPO) and at later-onset (LO-PIPO). METHODS All children fulfilling new PIPO criteria over a 30-year period were reviewed. Baseline demographic and clinical features as well as nutritional outcomes were collected. Nutritional outcomes included overall survival, prevalence of enteral autonomy and parenteral nutrition (PN) dependency, rate of major PN complications, and growth course. RESULTS Forty-four patients were still alive at the end of the follow-up. Twenty-five patients (57%) achieved enteral autonomy, whilst 18 remained on PN. Among the patients requiring PN at the beginning of the study period, we found that 55% (CI 34-70) has the probability of remaining on PN at the latest follow-up. Prevalence of gastrointestinal obstruction symptoms (P

Multichannel intraluminal impedance pH (MII-pH) monitoring currently represents the gold standard diagnostic technique for the detection of gastro-esophageal reflux (GER), since it allows to quantify and characterize all reflux events and their possible relation with symptoms. Over the last ten years, thanks to its strengths and along with the publication of several clinical studies, its worldwide use has gradually increased, particularly in infants and children. Nevertheless, factors such as the limited pediatric reference values and limited therapeutic options still weaken its current clinical impact. Through an up-to-date review of the available scientific evidence, our aim was to produce a position paper on behalf of the working group on neurogastroenterology and acid-related disorders of the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) on MII-pH monitoring technique, indications and interpretation in pediatric age, in order to standardise its use and to help clinicians in the diagnostic approach to children with GER symptoms.

Aim: Postural measures are frequently recommended for gastroesophageal reflux (GER) symptoms, despite limited evidence. This was the first study to assess the impact of upright and recumbent body positions on GER episodes in children and adolescents, not just infants. Methods: We retrospectively assessed the pH-impedance parameters of paediatric patients referred for possible GER-related symptoms to two hospitals in Naples and Rome, Italy, from September 2016 to September 2018. Data were separately obtained for the time that the patients spent in upright and recumbent positions. Results: Data from 187 patients under the age of 18 were collected, at a mean age of just over seven years. We found that the acid exposure time was stable irrespective of changes in body position (P >.05). The mean number of reflux episodes per hour was 2.99 during the upright position and 1.21 during the recumbent position (P

Meckel diverticulum, a common congenital anomaly of the small intestine, can be responsible of several complications due to the presence of ectopic gastric mucosa and represents a challenge for diagnosis. We present the case of a 11-year boy suffering from intestinal pain and bleeding in which radiological examinations unexpectedly raised the suspicion of Meckel diverticulum. The diagnosis was confirmed using 99mTc-pertechnetate scintigraphy. At surgery, a fistulous tract between Meckel diverticulum and an inflamed appendix was found. The authors discuss the role of medical nuclear imaging which, notwithstanding its limitations, is of fundamental importance to achieve a correct and timely diagnosis. This is of particular relevance in unusual cases, as the one presented, in which Meckel diverticulum is found concurrently with other intestinal abnormalities.

Background: Colorectal carcinoma (CRC) is very rare in the pediatric and adolescent age range and clinical management is performed according to adult protocols. We report, for the first time in the literature, a case of a child with metastatic CRC successfully treated with panitumumab associated to chemotherapy. Methods: A twelve-year-old male was diagnosed with CRC with nodal metastasis and peritoneal neoplastic effusion. After performing a genetic evaluation, in light of the absence of mutations in RAS family genes, anti-Epidermal Growth Factor Receptor (EGFR) monoclonal antibody, panitumumab, was added to chemotherapy FOLFOXIRI. Results: The child successfully responded to therapy with normalization of the Carbohydrate Antigen (CA) 19.9 value after the third cycle of treatment. After the sixth cycle, he underwent surgery that consisted in sigmoid resection with complete D3 lymphadenectomy. At histological evaluation, no residual neoplastic cells were detectable in the surgical specimen. He completed 12 cycles of chemotherapy plus panitumomab and he is alive without disease 14 months from diagnosis. Conclusions: Our results suggest performing mutational screening for colorectal cancer also in the pediatric setting, in order to orient treatment that should include targeted therapies.

Improved surgical techniques, as well as preoperative and postoperative care have dramatically changed survival of children with esophageal atresia (EA) over the last decades. Nowadays, we are increasingly seeing EA patients experiencing significant short- and long-term gastrointestinal morbidities. Anastomotic stricture (AS) is the most common complication following operative repair. An esophageal stricture is defined as an intrinsic luminal narrowing in a clinically symptomatic patient, but no symptoms are sensitive or specific enough to diagnose an AS. This review aims to provide a comprehensive view of AS in EA children. Given the lack of evidence-based data, we critically analyzed significant studies on children as well as adults, including comments on benign strictures with other etiologies.Despite there is no consensus about the goal of the luminal diameter based on the patient’s age, esophageal contrast study and/or endoscopy are recommended to assess the degree of the narrowing. A high variability in incidence of ASs is reported in literature, depending on different definitions of AS and on a great number of pre-, intra- and postoperative risk factor influencing the anastomosis outcome. The presence of a long gap between the two esophageal ends, with consequent anastomotic tension, is determinant for stricture formation as well as its response to treatment. The cornerstone of treatment is endoscopic dilation, whose primary aims are to achieve symptom relief, allow age-appropriate capacity for oral feeding and reduce the risk of pulmonary aspiration.. No clear advantage of either balloon or bougie dilator has been demonstrated, therefore the choice is based on operator experience and comfort with the equipment. Retrospective evidences suggest that selective dilatations (performed only in symptomatic patients) results in significantly less number of dilatation sessions than routine dilations (performed to prevent symptoms) with equal long-term outcomes. The response to dilation treatment is variable, and some patients may experience recurrent and refractory ASs. Adjunctive treatments have been used, including local injection of steroids, topical application of Mitomycin-C and esophageal stenting, but long-term studies are needed to prove their efficacy and safety. Stricture resection or esophageal replacement with an interposition graft remain options for AS refractory to conservative treatments.

none 34 no Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries. open Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; D’antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dall’oglio, Luigi; Spada, Marco; Grandi, Francesca Diamanti, Antonella; Capriati, Teresa; Gandullia, Paolo; Dileo, Grazia; Lezo, Antonella; Lacitignola, Laura; Spagnuolo, Mariaimmacolata; Gatti, Simona; Dâ antiga, Lorenzo; Verlato, Giovanna; Roggero, Paola; Amarri, Sergio; Baldassarre, Mariaelisabetta; Cirillo, Francesco; Elia, Domenica; Boldrini, Renata; Campanozzi, Angelo; Catassi, Carlo; Aloi, Marina; Romano, Claudio; Candusso, Manila; Cecchi, Nicola; Bellini, Tommaso; Tyndall, Elaine; Fusaro, Fabio; Caldaro, Tamara; Alberti, Daniele; Gamba, Piergiorgio; Lima, Mario; Bagolan, Pietro; De Goyet, Jean De Ville; Dallâ oglio, Luigi; Spada, Marco; Grandi, Francesca

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 1second (FEV1%) predicted was detected. Results pH-MII was positive in 17/31 (54.8%) patients (mean age: 12.4years; range: 4–17years). pH monitoring detected an average of 64.6 acid reflux events 4.4 episodes >5minutes 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.

Summary 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.

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.

Gastric emptying is a radionuclide technique with a low effective radiation dose (maximum 0.3 mSv for 37 MBq) that is frequently used for measuring gastric emptying. In addition, other important clinical information achievable by this simple test are identifying repetitive reflux episodes, quantifying reflux level, duration and frequency, and evaluating possible lung aspiration.

Gastrointestinal (GI) bleeding in infants and children is a fairly common problem, and it could be related to several clinical pathology according to patient age; however, it is usually limited in volume allowing time for diagnosis and treatment. One of the most frequent causes of lower gastrointestinal bleeding in pediatrics is Meckel’s diverticulum.

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.

Background/Purpose Surgical resection or strictureplasty (SP) are different options for intestinal Crohn disease (CD) strictures. The aim of this article is evaluation of long-term outcome of SP and resection. Methods From 1996 to 2011, 39 patients (23 male, 16 female) with symptomatic ileal and ileocolonic CD strictures resistant to medical/nutritional therapy and treated with surgery in 2 different surgical units were reviewed. The mean age at diagnosis was 11.82 years (range, 4-17 years). Mean age at surgery was 15.94 years (range, 4-24 years). Mean follow-up was 6.88 years (range, 0.5-15 years). Patients underwent resection (group A) or different SP techniques (group B). Results Twenty patients underwent intestinal resection (ileal or ileocolonic resection), and 19 patients underwent SP (jejunal, ileal, or ileocolic). Early postsurgical complications were observed in 2 patients of group A. Follow-up of group A patients revealed that 1 patient needed emergency treatment after 8 months surgery because of adhesions and 1 patient developed recurrence treated with medical therapy. In the follow-up group B, 3 patients experienced disease recurrence, 2 of them at the site of previous surgery. Conclusions At long-term follow-up, no significant difference in relapsing rate was observed between the 2 groups. Strictureplasty and resection represent an effective treatment of pediatric CD strictures. Strictureplasty could represent the first option for intestinal preservation.

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.

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.

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.

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.

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, GORD-only patients; group II, patients with GORD owing to OA. 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 OA patients. After P, the scintigraphic pattern changed in an exponential manner related to a faster GE. Conclusions Delayed gastric emptying is frequent in OA, and the scintigraphic ATCs are typical. Pyloromyotomy is a safe and effective technique to fully normalize GE.