Francesca Rea

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.

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.

Dynamic esophageal stents.

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

Three-dimensional endoanal ultrasound and anorectal manometry in children with anorectal malformations: new discoveries

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.

Italian survey on non-steroidal anti-inflammatory drugs and gastrointestinal bleeding in children

AIM To investigate gastrointestinal complications associated with non-steroidal anti-inflammatory drug (NSAIDs) use in children. METHODS A retrospective, multicenter study was conducted between January 2005 and January 2013, with the participation of 8 Italian pediatric gastroenterology centers. We collected all the cases of patients who refer to emergency room for suspected gastrointestinal bleeding following NSAIDs consumption, and underwent endoscopic evaluation. Previous medical history, associated risk factors, symptoms and signs at presentation, diagnostic procedures, severity of bleeding and management of gastrointestinal bleeding were collected. In addition, data regarding type of drug used, indication, dose, duration of treatment and prescriber (physician or self-medication) were examined. RESULTS Fifty-one patients, including 34 males, were enrolled (median age: 7.8 years). Ibuprofen was the most used NSAID [35/51 patients (68.6%)]. Pain was the most frequent indication for NSAIDs use [29/51 patients (56.9%)]. Seven patients had positive family history of Helicobacter pylori (H. pylori) infection or peptic ulcer, and 12 had associated comorbidities. Twenty-four (47%) out of 51 patients used medication inappropriately. Hematemesis was the most frequent symptom (33.3%). Upper gastrointestinal endoscopy revealed gastric lesions in 32/51 (62%) patients, duodenal lesions in 17 (33%) and esophageal lesions in 8 (15%). In 10/51 (19.6%) patients, a diagnosis of H. pylori gastritis was made. Forty-eight (94%) patients underwent medical therapy, with spontaneous bleeding resolution, while in 3/51 (6%) patients, an endoscopic hemostasis was needed. CONCLUSION The data collected in this study confirms that adverse events with the involvement of the gastrointestinal tract secondary to NSAID use are also common in children.

Miniprobe EUS in management of pancreatic pseudocyst.

Pancreatic pseudocysts (PP) arise from trauma and pancreatitis; endoscopic gastro-cyst drainage (EGCD) under endoscopic ultrasonography (EUS) in symptomatic PP is the treatment of choice. Miniprobe EUS (MEUS) allows EGCD in children. We report our experience on MEUS-EGCD in PP, reviewing 13 patients (12 children; male:female = 9:3; mean age: 10 years, 4 mo; one 27 years, malnourished male Belardinelli-syndrome; PP: 10 post-pancreatitis, 3 post-traumatic). All patients underwent ultrasonography, computed tomography and magnetic resonance imaging. Conservative treatment was the first option. MEUS EGCD was indicated for retrogastric cysts larger than 5 cm, diameter increase, symptoms or infection. EGCD (stent and/or nasogastrocystic tube) was performed after MEUS (20-MHz-miniprobe) identification of place for diathermy puncture and wire insertion. In 8 cases (61.5%), there was PP disappearance; one, surgical duodenotomy and marsupialization of retro-duodenal PP. In 4 cases (31%), there was successful MEUS-EGCD; stent removal after 3 mo. No complications and no PP relapse in 4 years of mean follow-up. MEUS EGCD represents an option for PP, allowing a safe and effective procedure.

Cystic fibrosis: a surgical matter?

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.

Eosinophilic esophagitis: is it also a surgical disease?

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.