Jiri Snajdauf

What is the role of endoscopic retrograde cholangiopancreatography in assessing traumatic rupture of the pancreatic in children

Abstract Background and study aims: Trauma is one of the most common causes of morbidity and mortality in the pediatric population. The diagnosis of pancreatic injury is based on clinical presentation, laboratory and imaging findings, and endoscopic methods. CT scanning is considered the gold standard for diagnosing pancreatic trauma in children. Patients and methods: This retrospective study evaluates data from 25 pediatric patients admitted to the University Hospital Motol, Prague, with blunt pancreatic trauma between January 1999 and June 2013. Results: The exact grade of injury was determined by CT scans in 11 patients (47.8%). All 25 children underwent endoscopic retrograde cholangiopancreatography (ERCP). Distal pancreatic duct injury (grade III) was found in 13 patients (52%). Proximal pancreatic duct injury (grade IV) was found in four patients (16 %). Major contusion without duct injury (grade IIB) was found in six patients (24%). One patient experienced duodeno-gastric abruption not diagnosed on the CT scan. The diagnosis was made endoscopically during ERCP. Grade IIB pancreatic injury was found in this patient. One patient (4%) with pancreatic pseudocyst had a major contusion of pancreas without duct injury (grade IIA). Four patients (16%) with grade IIB, III and IV pancreatic injury were treated exclusively and nonoperatively with a pancreatic stent insertion and somatostatine. Two patients (8%) with a grade IIB injury were treated conservatively only with somatostatine without drainage. Eighteen (72 %) children underwent surgical intervention within 24 h after ERCP. Conclusion: ERCP is helpful when there is suspicion of pancreatic duct injury in order to exclude ductal leakage and the possibility of therapeutic intervention. ERCP can speed up diagnosis of higher grade of pancreatic injuries.

Alagille Syndrome Mimicking Biliary Atresia in Early Infancy.

Alagille syndrome may mimic biliary atresia in early infancy. Since mutations in JAG1 typical for Alagille syndrome type 1 have also been found in biliary atresia, we aimed to identify JAG1 mutations in newborns with proven biliary atresia (n = 72). Five biliary atresia patients with cholestasis, one additional characteristic feature of Alagille syndrome and ambiguous liver histology were single heterozygotes for nonsense or frameshift mutations in JAG1. No mutations were found in the remaining 67 patients. All “biliary atresia” carriers of JAG1 null mutations developed typical Alagille syndrome at the age of three years. Our data do not support association of biliary atresia with JAG1 mutations, at least in Czech patients. Rapid testing for JAG1 mutations could prevent misdiagnosis of Alagille syndrome in early infancy and improve their outcome.

Urinary Intestinal Fatty Acid-Binding Protein Can Distinguish Necrotizing Enterocolitis from Sepsis in Early Stage of the Disease

Necrotizing enterocolitis (NEC) is severe disease of gastrointestinal tract, yet its early symptoms are nonspecific, easily interchangeable with sepsis. Therefore, reliable biomarkers for early diagnostics are needed in clinical practice. Here, we analyzed if markers of gut mucosa damage, caspase cleaved cytokeratin 18 (ccCK18) and intestinal fatty acid-binding protein (I-FABP), could be used for differential diagnostics of NEC at early stage of disease. We collected paired serum (at enrollment and week later) and urine (collected for two days in 6 h intervals) samples from 42 patients with suspected NEC. These patients were later divided into NEC (n = 24), including 13 after gastrointestinal surgery, and sepsis (n = 18) groups using standard criteria. Healthy infants (n = 12), without any previous gut surgery, served as controls. Both biomarkers were measured by a commercial ELISA assay. There were no statistically significant differences in serum ccCK18 between NEC and sepsis but NEC patients had significantly higher levels of serum and urinary I-FABP than either sepsis patients or healthy infants. Urinary I-FABP has high sensitivity (81%) and specificity (100%) and can even distinguish NEC from sepsis in patients after surgery. Urinary I-FABP can be used to distinguish NEC from neonatal sepsis, including postoperative one, better than abdominal X-ray.

The role of endoscopic retrograde cholangiopancreatography in choledochal cysts and/or abnormal pancreatobiliary junction in children

Introduction Biliary cysts with an abnormal pancreatobiliary junction are one of the most common pancreatobiliary malformations. The main symptom is cholestasis; endoscopic retrograde cholangiopancreatography (ERCP) plays a key role in diagnosis. Aim Retrospective evaluation of ERCP performed to diagnose abnormalities of the pancreatobiliary junction. Material and methods We retrospectively evaluated ERCP performed to diagnose abnormalities of the pancreatobiliary junction, mainly choledochal cysts, in 112 children between 1990 and 2011. Results We performed 112 examinations of 50 children with abnormal pancreatobiliary junction and choledochal cysts (15 males and 35 females, average age: 5 years, range: 1 month – 15 years). Cysts were associated with a common channel in 37 (74%) cases, were not associated with a common channel in 9 (18%) cases, and in 3 (6%) cases the common channel lacked cysts. We performed endoscopic papilla sphincterotomy on 33 (66%) patients; endoscopic drainage was performed 62 times, including 17 patients without papilla sphincterotomy. In 15 (30%) cases, we only performed sphincterotomy. Extraction of lithiasis was done in 2 (4%) cases. Both ERCP and magnetic resonance cholangiopancreatography (MRCP) were performed on 13 patients. There was concordance of the choledochal cyst and of the type of cyst in both methods; however, common channels could not be observed by MRCP. There was 1 serious complication (perforation after sphincterotomy) and 11 (9%) mild adverse events. Conclusions When performed at an expert centre, ECRP is a safe and reliable procedure for children with choledochal cysts and/or an abnormal pancreatobiliary junction. Endoscopic retrograde cholangiopancreatography remains a reasonable alternative because MRCP has a limited ability to diagnose the precise anomalies of the pancreatobiliary junction and has no therapeutic capabilities.

Endoscopic treatment of bile duct post-traumatic and post-operative lesions in children

Abstract Background and study aims: The aim of this study was to assess the significance and safety of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing and treating bile duct injuries in children. Patients and methods: Fourteen pediatric patients, with traumatic or postoperative bile duct injury, in which ERCP was performed, were retrospectively evaluated. Results: We performed 46 ERCP and 12 endoscopic papillotomies in children with suspected bile duct injuries. A bile stent was primarily inserted in 13 patients and there were 20 replacements. Endoscopic treatment of bile leakage without need for bile duct sutures or reconstruction was successful in 85.7%. Post ERCP complications included cholangitis and recurrent bleeding, which occurred only in two patients each. Conclusions: ERCP and endoscopic bile stent insertion is a highly effective, minimally-invasive treatment for bile duct injury and should be included as part of the therapeutic procedures in pediatric patients with suspected bile duct injury.

Pancreas Divisum in Children and Duodenum-Preserving Resection of the Pancreatic Head

Introduction A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives in children with pancreas divisum (PD). Materials and Methods Patients who underwent treatment for PD between 1999 and 2014 at our department were evaluated for sex, age, presenting symptoms, physical examination findings, biochemical markers, diagnostic methods, treatment modalities, and results of treatment during follow‐up. Results Seven patients who underwent treatment of symptomatic PD were included in the study. The median for follow‐up period was 8 years (from 26 months to 16 years). Male‐to‐female ratio was 4:3 and the median age at presentation was 11 years (2‐14 years). Presenting symptoms were recurrent episodic epigastric pain. Pancreatitis was documented by elevated amylase or lipase levels. Endoscopic retrograde cholangiopancreatography (ERCP) was the method of diagnosis of PD in all patients. Five patients had complete PD and two had incomplete variants. Three patients improved after ERCP papillotomy. In three patients, papillotomy was unsuccessful but they have only mild episodes of pancreatitis. One patient presented at the age of 4 years with recurrent pancreatitis. She was treated surgically by duodenum‐preserving resection of the pancreatic head (DPRPH) because of severe recurrent pancreatitis occurring even after ERCP papillotomy. The patient is 26 months after operation without any reported problems. Conclusion Patients with symptomatic PD are indicated for ERCP papillotomy attempt. If there is not improvement after ERCP, then recurrent bouts of severe pancreatitis are considered as an indication for surgical procedure. DPRPH is a safe and feasible surgical alternative.