Hepatic Lesions in Children Related to Congenital Intrahepatic Portal Venous Shunts

We present two patients with hepatic lesions associated with congenital portosystemic shunts (CPSS), a rare vascular malformation, in order to emphasize the variable clinical presentation of this condition and different management strategies in each case. CPSS can give rise to several complications such as hepatic encephalopathy, portopulmonary hypertension and hepatic tumors. These hepatic lesions though most commonly benign in nature have an increased risk of malignant transformation. Therefore, we underline the necessity of considering CPSS in the differential diagnoses for all cases of space-occupying hepatic lesions in pediatric patients

Endoscopic transgastric drainage of pancreatic pseudocyst in hereditary pancreatitis. A case report

Hereditary pancreatitis (HP) is a rare genetic disorder characterized by acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) that runs in families. It’ s symptoms are usually typical ones pancreatitis but HP is more amenable to treatment, especially when complication presents. Recent single-center studies have identified several genetic risk factors, including cystc fibrosis transmembrane conductance regulator (CFTR), cationic trypsinogen (PRSS1), pancreatic secretory trypsin inhibitor (SPINK1), chymotrypsin (CFTP) and carboxypeptidase 1 (CPA1) genes. Other risk factors include obstructive, traumatic, infectious and systemic causes. Our case report presents a 9-years old boy, with a pancreatic pseudocyst (8cm in diameter) as a consequence of recurrent episodes of pancreatitis. The diagnostic investigation (MRCP, Cystic Fibrosis test) had proved no obvious aetiology. An enterocystic roux en Y anastomosis was performed, but the boy continued o develop episodes of pancreatitis and after 2 years, he presented with a new pancreatic pseudocyst (PPC) of the same dimensions, which was attributed to genetic factors. A more conservative approach was decided: endoscopic transgastric drainage of the pseudocyst. In gastroscopy, the cyst was protruded on the posterior wall of the stomach and it was drained into it via a pig tail catheter, which was removed 6 weeks later. After 3 months abdominal ultrasonography follow up confirms the successful drainage of the pseudocyst