Paola De Angelis

The etiology of acute recurrent pancreatitis in children: A challenge for pediatricians

Objectives: To assess specific etiologies of acute recurrent pancreatitis at a single Italian pediatric cystic fibrosis (CF) center. Methods: We studied, retrospectively, 78 young patients (39 female subjects; mean age at diagnosis, 8.8 ± 5.1 years) affected by acute recurrent episodes of pancreatitis, remained etiologically undiagnosed at first-level assessment. All patients were submitted to endoscopic retrograde cholangiopancreatography to exclude biliopancreatic malformations and tested for CF by a sweat chloride test. Most patients also were studied for the research of CFTR, PRSS1, and SPINK1 gene mutations. Results: A high percentage of family history for chronic pancreatitis was observed (20.5%). The sweat test identified 8 subjects (10.3%) with classic CF (2 patients) or at risk for CF (6 patients). Genetic analysis showed mutations in CFTR, SPINK1, and PRSS1 genes in 39.6%, 7.1%, and 4.5% of patients, respectively. A biliopancreatic malformation was diagnosed in 15 patients (19.2%). We also observed biliary lithiasis (5 patients [6.5%]), congenital pancreatic polycystosis (2 patients), a case of dyslipidemia, and 1 patient with a posttransplantation, drug-induced pancreatitis. Conclusions: Recurrent pancreatitis in children has several etiologies. Genetic testing confirms the high frequency of CFTR mutations. This suggests that it is of some value to identify patients with late-onset CF and CFTR-related disorders.

Endoscopic management of congenital esophageal stenosis

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.

Eosinophilic Esophagitis and Barrett’s Esophagus: An Occasional Association or an Overlap Disease?

Background: Esophageal diseases are common in infants and children, and may present with several clinical and pathological aspects. Eosinophilic esophagitis (EE) is characterized by inflammatory, predominantly eosinophilic infiltrate (≧15 eosinophils per high-power field (HPF)) that is not responsive to acid suppression therapy. An immunoallergic pathogenesis has been hypothesized, likely related to food allergy. Barrett’s esophagus (BE) is due to chronic gastroesophageal reflux. The pathological consequence is the replacement of normal stratified squamous epithelium by columnar mucosa with goblet cells. Methods: We present 2 children with a history of food allergy. Endoscopy revealed linear furrows and yellow plaques in the mid-distal esophagus. Results: In both patients histology showed a high number of eosinophils (>30 at HPF) in the mid-distal esophagus and intestinal metaplasia with goblet cells in distal esophagus. Diagnosis of EE associated to BE was made. Restriction diet was administered to treat EE whereas, in 1 case, laparoscopic fundoplication was performed to treat BE. Follow-up showed a remission of endoscopic and histological aspects. Conclusions: The unusual, possibly fortuitous association of EE and BE, two conditions differing in etiopathogenesis, clinical and pathological features, calls for a correct diagnosis to offer suitable treatment and prognosis.

Treatment of esophageal achalasia in children: Today and tomorrow

BACKGROUND Esophageal achalasia (EA) is a rare esophageal motility disorder in children. Laparoscopic Heller myotomy (LHM) represents the treatment of choice in young patients. Peroral endoscopic myotomy (POEM) is becoming an alternative to LHM. The aim of this study is to evaluate the effectiveness, safety, and outcomes of POEM vs LHM in treatment of children with EA. METHODS Data of pediatric patients with EA, who underwent LHM and POEM from February 2009 to December 2013 in two centers, were collected. RESULTS Eighteen patients (9 male, mean age: 11.6 years; range: 2-17 years) were included. Nine patients (6 male, mean age: 10.7 years; range: 2-16 years) underwent LHM, and the other 9 (3 males, mean age: 12.2 years; range: 6-17 years) underwent POEM procedure. Mean operation time was shorter in POEM group compared with LHM group (62/149 minutes). Myotomy was longer in POEM group than in LHM group (11/7 cm). One major complication occurred after LHM (esophageal perforation). No clinical and manometric differences were observed between LHM and POEM in follow-up. The incidence of iatrogenic gastroesophageal reflux disease was low (1 patient in both groups). CONCLUSIONS Results of a midterm follow-up show that LHM and POEM are safe and effective treatments also in children. Besides, POEM is a mini-invasive technique with an inferior execution timing compared to LHM. A skilled endoscopic team is mandatory to perform this procedure.

Custom dynamic stent for esophageal strictures in children

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.

Role of endoscopic retrograde cholangiopancreatography in diagnosis and management of congenital choledochal cysts: 28 pediatric cases

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.

Delayed gastric emptying and typical scintigraphic gastric curves in children with gastroesophageal reflux disease: Could pyloromyotomy improve this condition?

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.

Update on management of caustic and foreign body ingestion in children

The following recommendations for management of caustic and foreign body ingestion in children have been developed following a multicentre study performed by the Italian Society for Paediatric Gastroenterology, Hepatology and Nutrition (SIGENP). They are principally addressed to medical professionals involved in casualty. Because there is paucity of good quality clinical trials in children on this topic, many of the recommendations are currently extrapolated from adult experiences or based on experts opinions. The document represents a level 2 to 5 degree of evidence (according to the Oxford Centre for Evidence-based Medicine Levels of Evidence), gathered from clinical experience, recent studies, and expert reports discussed during a consensus conference of the Endoscopic Section of the Italian Society of Paediatric Gastroenterology Hepatology and Nutrition. This working group comprises paediatricians, endoscopists, paediatric surgeons, toxicologists, and ENT surgeons, who are all actively involved in the management of these children. Recommendations are intended to serve as an aid to clinical judgement, not to replace it and therefore do not provide answers to every clinical question; nor does adherence to them ensure a successful outcome in every case. The ultimate decision on the clinical management of an individual patient will always depend on the specific clinical circumstances of the patient, and on the clinical judgement of the health care team.

Endoscopic management of esophageal stenosis in children: New and traditional treatments

Post-esophageal atresia anastomotic strictures and post-corrosive esophagitis are the most frequent types of cicatricial esophageal stricture. Congenital esophageal stenosis has been reported to be a rare but typical disease in children; other pediatric conditions are peptic, eosinophilic esophagitis and dystrophic recessive epidermolysis bullosa strictures. The conservative treatment of esophageal stenosis and strictures (ES) rather than surgery is a well-known strategy for children. Before planning esophageal dilation, the esophageal morphology should be assessed in detail for its length, aspect, number and level, and different conservative strategies should be chosen accordingly. Endoscopic dilators and techniques that involve different adjuvant treatment strategies have been reported and depend on the strictures etiology, the availability of different tools and the operators experience and preferences. Balloon and semirigid dilators are the most frequently used tools. No high-quality studies have reported on the differences in the efficacies and rates of complications associated with these two types of dilators. There is no consensus in the literature regarding the frequency of dilations or the diameter that should be achieved. The use of adjuvant treatments has been reported in cases of recalcitrant stenosis or strictures with evidence of dysphagic symptoms. Corticosteroids (either systemically or locally injected), the local application of mitomycin C, diathermy and laser ES sectioning have been reported. Some authors have suggested that stenting can reduce both the number of dilations and the treatment length. In many cases, this strategy is effective when either metallic or plastic stents are utilized. Treatment complications, such esophageal perforations, can be conservatively managed, considering surgery only in cases with severe pleural cavity involvement. In cases of stricture relapse, even if such relapses occur following the execution of well-conducted conservative strategies, surgical stricture resection and anastomosis or esophageal substitution are the only remaining options.

Strictureplasty and intestinal resection: different options in complicated pediatric-onset Crohn disease

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.