Luigi Dall'Oglio

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.

Eosinophilic oesophagitis and coeliac disease: is it just a casual association?

Eosinophilic oesophagitis (EO) is the leading cause of dysphagia in children.1 Long underestimated in the past, it is today being reported with increasing frequency. Acute and chronic dysphagia and food impaction are the typical symptoms in both children and young adults, whereas vomiting and refusal of food may be more frequent in infants.1,2 Diagnosis of EE is mainly based on the eosinophilic infiltration of the oesophageal mucosa (>20 eosinophils/high power field (HPF)) in association with some classic endoscopic features (adherent whitish plaques, oesophageal concentric rings, linear furrowing). The aetiology of EE is far from clear, but in about half of the cases reported it is associated with an allergy either to food or to aeroallergens.1,3 We describe three cases of EE, that we diagnosed by chance when performing endoscopy for the duodenal biopsy to confirm coeliac disease (CD). One case was diagnosed at the Institute of Child Health, IRCSS Burlo Garofolo, Trieste Italy, and, two at the Surgical and Endoscopic Digestive Unit of Ospedale Pediatrico Bambino Gesu, Rome, Italy. A 7-year-old boy was referred to the Gastroenterology Department, IRCSS Burlo Garofolo, Trieste, Italy with persistent sideropaenic anaemia since the age of 4-years. Given the unresponsiveness of his anaemia to iron supplementation, CD was suspected and confirmed by the positivity of antiendomysial serum antibodies (EMAs) and antitransglutaminase antibodies (tTG immunoglobulin (Ig) A 213 IU). The boy was allergic to dust mite, cat epithelium and betullaceae. His most common allergic manifestations included rhinitis and asthma. He was referred to our institute to confirm the diagnosis of CD. …

Stenting for caustic strictures: Esophageal replacement replaced

METHODS From 1983 to 1996, 31 children with caustic esophageal strictures were seen at Bambino Gesù Childrens Hospital; they were all treated conservatively except for two cases complicated by tracheoesophageal fistula. The remaining 29 patients were divided into three groups depending on the treatment, which was modified over the years. Group A (1983 to 1987) consisted of seven patients treated by periodic dilatations; group B (1988 to 1992) consisted of 10 children treated by 40 days of esophageal stenting plus dexamethasone, 0.5 mg/kg/d plus ranitidine plus no oral feeding for 7 to 10 days; group C (1993 to 1996) consisted of 12 cases treated by 40 days of esophageal stenting plus dexamethasone, 1 mg/kg/d plus omeprazole plus early oral feeding resumption. RESULTS No differences were observed between the three groups of patients with regard to the mean age and to the ingested substance, whereas a significant difference (P = .007) was observed in the mean length of the stricture between group A and C (3.4+/-1.3 and 5.6+/-1.6 cm, respectively). In all but one of the patients (96.5%) complete healing of the stenosis was achieved by conservative treatment, with definitive relief of dysphagia. One patient in group C did not improve after a repeated stenting procedure and was surgically treated. However, in group A, resolution of the stricture was obtained after an average of 19.9+/-14.8 dilatations in a mean period of 25.3+/-17.2 months. In group B, a mean of 12+/-11.3 dilatations were required in a mean period of treatment of 14.1+/-10.6 months. In patients in group C, a mean of 3.5+/-3.2 dilatations were necessary in a mean of 5.8+/-4.8 months. A statistically significant difference was observed both with regard to the number of dilatations and to the duration of treatment, between group A and group C (P = .002) and group B and C (P = .03). CONCLUSION Esophageal replacement should be considered only in cases complicated by tracheoesophageal fistula or in the rare patients who do not respond to repeated esophageal stenting.

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.

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.

Gastroesophageal reflux in neurologically impaired children: partial or total fundoplication?

Background: It is difficult to give guidelines when approaching gastroesophageal disease in neurologically impaired children. Indication for surgery has been increasing over recent years, but there is no consensus on the surgical technique of choice. Nothing has been written specifically comparing the results of different procedures in these patients, so far. Study design: We retrospectively compare the short- and long-term results of two different types of fundoplication in a series of children operated on for documented gastroesophageal reflux disease at our institution. Results: One group (group A) of 27 patients, operated on between 1977 and 1993, underwent Nissen fundoplication, the other (group B), formed of 20 patients all of whom were operated on between 1993 and 1995, underwent Thal fundoplication. We compared the results in terms of positive outcome (recovery) and negative outcome (minor and major complication), computing the relative odds of group A versus group B in terms of risk of complication, and we compared the mean operative time and the length of hospital stay by means of a students t-test analysis. Conclusions: Our results show that there is no statistical difference between the two procedures in terms of relative risk of complication and success rate. The duration of surgery and hospital stay were significantly shorter in group B. The Thal procedure can, therefore, be proposed as first choice in the management of these patients.

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.