Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow‐up after pancreatic resection in children are scant.

Background: In children, EHPVO is the commonest cause of upper gastrointestinal bleeding. Although umbilical vein catheterization or omphalitis are frequently reported causes, most cases remain unexplained. Inherited thrombophilia has not been extensively evaluated. We investigated thrombophilia in EHPVO children, as compared with EHPVO in adults (without solid tumors or cirrhosis) and in adult controls. The two groups were compared with controls in terms of odds ratios and 95% CI.

Purpose: Diagnosis of nature of pancreatic masses in children, as well as their treatment, are often challenging because of their rarity. We reviewed our experience with these rare entities.

Background and aim: Esophageal high resolution manometry (HRM) is a well standardized diagnostic tool in adult patients with dysphagia and gastroesophageal reflux diesase (GERD). Its role in pediatric population is limited, but someone suggests a utility in the assessment in patients undergoing antireflux surgery. Scanty data are available in neurologically impaired (NI) children. We want to characterize manometrically the esophageal function in NI patients with GERD and dysphagia symptoms.

Background: Gastroesophageal reflux disease (GERD) unresponsive to medical treatment affects many neurologically impaired (NI) children.

Iatrogenic colonic perforation is the most severe complication of lower gastrointestinal endoscopy. A prompt diagnosis is necessary in order to identify the site of perforation and to reduce peritonitis due to bacterial seeding. We reported our experience with a 10-month old baby admitted for descending colonic perforation during an endoscopic procedure, in whom an immediate use of the laparoscopic approach allowed an early recognition of the perforation, that was successfully repaired by video-assisted colorrhapy, by the exteriorization of the colon from the umbilical wound.

Aim: To assess outcome in adulthood of patients survived with their native liver after Kasai Portoenterostomy (KPE) for biliary atresia (BA) with a follow-up ranging from 20 to 40 years.

A 13-year-old girl was referred to our pediatric surgery department with a 1-year history of recurrent episodes of acute cholangitis. She complained of recurrent upper acute abdominal pain, nausea, anorexia, vomiting, jaundice, and weight loss during the course of the previous 2 months. Her family history was negative for any malignant disease. The patient's past medical history was unremarkable until October 2009 when she was admitted to a regional hospital because of abdominal pain, vomiting, and jaundice. Laboratory examination showed high levels of conjugated bilirubin (6.02 mg/dL), alkaline phosphatase (448 U/L), aspartate transaminase (193 U/L), alanine aminotransferase (430 U/L) and gamma-glutamyl transpeptidase (105 U/L). Abdominal ultrasound (USS) showed dilatation of the intrahepatic and extrahepatic biliary tree. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the biliary stenosis, and an endoscopic papillotomy was performed. The girl was discharged in good condition. Subsequently, the patient had 6 episodes of cholangitis, associated with mild pancreatitis, treated with urgent ERCP and insertion of stent, which provided only temporary benefit. Between each cholestatic episode, she was in good health and resumed normal activity. In October 2010, she was referred to our hospital. On admission, abdominal USS showed the presence of a mass in the distal portion of the choledochal stent. A magnetic resonance cholangio-pancreatography showed the presence of a mass, 25 mm in diameter, with bilobate morphology around the choledochal stent which was imprinting the duodenal periampullary wall (Fig. 1). Cancer markers were negative: carbohydrate antigen 19-9 was 2.3 I.U./mL (n.v. <27 I.U./mL), cancer antigen 125 was 1.4 U/mL (n.v. <35 U/mL), carcinoembrionic antigen was 0.3 ng/mL (n.v.< 3.4 ng/mL), human chorionic gonadotropin was 0.2 mUI/mL (n.v. <5 mUI/mL in absence of pregnancy), and alpha fetoprotein was 0.8 ng/mL (n.v. 0.50–66 ng/mL).FIGURE 1: An MRCP documenting tumor size and morphology at diagnosis. The mass is localized in the pancreatic head (see black arrow) and compresses the choledochal stent (see red arrow). MRCP = magnetic resonance cholangio-pancreatography.USS-guided biopsy of the mass was performed. The histological examination revealed a mesenchymal neoplasm of pancreatic origin. Computerized tomography of the thorax and abdomen was negative for metastases. The patient underwent a pylorus-preserving pancreaticoduodenectomy. Histological examination showed an inflammatory myofibroblastic tumor (IMT) with compact fascicular proliferation of spindle and plump myofibroblasts and fibroblast. The biopsy contained myxoid, edematous, and collagenized regions as well as a distinctive inflammatory infiltrate of lymphocytes, plasma cells, and granulocytes (Fig. 2). Immunohistochemical analysis showed the anaplastic lymphoma kinase (ALK) expression, a marker considered highly suggestive for IMT (Fig. 3). No further treatment was required. She was discharged under oral pancreatic enzyme replacement therapy. The follow-up was uneventful: she reported feeling well, normal stool frequency, and normoglycemia. The patient has been monitored for 4 years without clinical or radiological evidence of recurrence.FIGURE 2: Histological examination showing marked inflammatory mixed infiltrate represented mainly by lymphocytes, granulocytes, plasma cells, intermixed with fibroblasts.FIGURE 3: Immunohistochemistry for ALK using antibody ALK 1 shows intense and diffuse labeling of tumor cells (magnification x40). ALK = anaplastic lymphoma kinase.Pancreatic tumors are rare in childhood, accounting for only 0.2% of childhood malignancies (1). IMTs are benign solid lesion of unclear etiology, commonly found in the lungs. Histological features include the presence of myofibroblastic proliferation and a varying degree of inflammatory infiltrates, mainly consisting of lymphocytes, histiocytes, and plasma cells. Constitutional symptoms, such as fever, malaise, and weight loss, due to the inflammatory nature of these lesions are reported in around 20% of cases. These symptoms resolve with treatment and their recurrence often signifies recurrence of the disease (2). Because complete surgical excision is the treatment of choice for localized IMTs, early diagnosis is important for pancreatic tumors to prevent recurrent acute cholangitis and pancreatitis and consequently avoid risky and ineffectual operative ERCP. Enucleation alone has an increased risk of local recurrence, in particular for extrapulmonary lesions (25% vs <2% for lung lesions), with the highest risk of recurrence within 6 months. Distant metastasis of IMTs is rare, occurring in <5% of cases (3). There are no specific chemotherapeutic regimens, although the various agents used include cyclosporin, cyclophosphamide, methotrexate, and 5 fluorouracil. Radiotherapy has been used for residual tumors; however, its use in children is limited. The ALK inhibitor Crizotinib could be used for IMTs which are shown to carry chromosomal rearrangements involving the ALK gene (approximately 50%–70% of total IMTs) (2,4)(2,4).

OBJECTIVE:To evaluate ultrasonographic features of the liver and biliary tree, including the presence of the triangular cord, in infants with biliary atresia and to analyze the correspondence between hepatic echostructure and histological aspects of the liver.MATERIALS AND METHODS:35 consecutive infants (19 males) with documented diagnosis of biliary atresia were included. Ultrasonography evaluation, performed at a mean age of 63.1 ± 34.9 days, was focused on the extrahepatic bile ducts, characteristics of the gallbladder and liver, and the presence of the triangular cord. Liver biopsies were examined with particular regard to the presence and severity of fibrosis.RESULTS:On ultrasound, the gallbladder was not seen in 11 (31 %) cases, while in the remaining 24 patients the gallbladder was regular in 6 patients and irregular in 18 cases. The triangular cord was identified in 9 (26 %) of 35 patients. In 21 patients the liver echostructure appeared normal, while in 14 infants the liver parenchyma was more echogenic and coarse than normal. Liver biopsy showed signs of cirrhosis or fibrosis in all cases, including patients with a normal hepatic echostructure.CONCLUSION:Although the triangular cord was visualized in one-fourth of the infants with biliary atresia, abnormalities of the gallbladder on ultrasound (absence or abnormalities of length/shape) were detected in 83 % of the patients. Therefore, ultrasound evaluation of the liver and biliary tree plays an important role in suspecting biliary atresia. On the other hand, a low correspondence between liver echostructure aspects and the presence and severity of fibrosis at liver biopsy was identified. Therefore, severe liver disease in infants with biliary atresia cannot be excluded only on the basis of ultrasound findings.

Objectives: Previous studies have described the high sensitivity and specificity of antibodies against synthetic deamidated gliadin peptides (AdGA) in celiac disease (CD) patients (pts). The aims of this study are to confirm the sensibility, specificity, negative and positive predictive value (NPV, PPV) of AdGA and to verify the correlation of AdGA with anti tissue transglutaminase antibodies (tTG-Ab) in pts investigated for CD. Materials and methods: IgA and IgG AdGA were tested by an enzymelinked immunoabsorbent assay (Kit Inova Diagnostic). IgA and IgG tTG-Ab were measured by radio-binding assay. The histological study was done according to the Marsh’s classification modified by Oberhuber (M/O). Patients: 102 pts (62 F, 40 M, age M±SD: 7±4 ys) were studied by upper endoscopic biopsy for gastrointestinal symptoms or for suspected CD: n=78 → CD (typical lesions) n=11 → potential CD (positive tTG-Ab and DQ2/DQ8, normal histology) n=13 → gastrointestinal control (normal histology, negative tTG-Ab) Results: (1) AdGA-IgA were positive in 52/78 of CD pts, in 2/11 of potential