Quistes pancreáticos congénitos: revisión a propósito de un caso / Congenital pancreatic cysts: review on a case

Los quistes pancreáticos congénitos son una entidad muy poco frecuente en Pediatría. Se desconoce su causa. Generalmente, son asintomáticos y suponen un hallazgo radiológico casual. Se suelen localizar en la cola o en el cuello del páncreas. Lo más frecuente es que sean lesiones aisladas. De forma puntual se han descrito asociadas a otras enfermedades, como el síndrome de De Jeune, el síndrome de Beckwith-Wiedeman o la enfermedad de von Hippel-Lindau. Su tratamiento clásico ha sido la cirugía. No obstante, dada la benignidad de estas lesiones en la infancia, así como la complejidad que supone el tratamiento quirúrgico, se aconseja un manejo conservador de los mismos

Congenital pancreatic cysts are very rare in childhood. Its cause is unknown. Generally, they are asymptomatic and represent a casual radiological finding. They are usually located int the tail or in the neck of the pancreas. Most often, they are isolated lesions. They have been associated with other diseases, such as De Jeune syndrome, Beckwith-Wiedeman syndrome or von Hippel-Lindau disease. His classic treatment has been surgery. However, given the benign nature of these lesions in childhood, as well as the complexity of surgical treatment, a conservative management of them is advised

Traumatic perforation of a juxtapancreatic enteric duplication cyst: a paediatric emergency.

This report outlines a rare case of juxtapancreatic duplication cyst in a 5-month-old infant who presented with peritonitis, secondary to traumatic perforation of the cyst. We aim to highlight the role of selective mucosectomy in these cases.

Possible congenital dilatation of the pancreatic duct.

The main pancreatic duct can become dilated in a number of conditions. We describe a patient with gross dilatation of the main pancreatic duct without evidence of causative underlying pathology suggesting congenital dilatation of the pancreatic duct. A 36-year-old man presented with signs of intestinal obstruction and a history of surgery for congenital pyloric stenosis. Incidental findings on CT showed a massively dilated main pancreatic duct. On MRI there was no duct irregularity or solid mural nodule, making a main duct intraductal papillary mucinous neoplasm unlikely. Endoscopic ultrasound findings were in keeping with those on MRI. Fine needle aspiration revealed a non-viscous fluid with a low carcinoembryonic antigen and high amylase concentration, consistent with normal pancreatic fluid levels rather than a mucinous collection. After 1 year, the cyst remains unchanged. This patient will be kept under surveillance with yearly MRI.

Congenital Cyst of the Pancreas: A Case Report and Review of Literature.

This is a case of a newborn female with congenital pancreatic cysts discovered incidentally. The 5-week-old infant had multiple abdominal cysts originating from the pancreas. When the radiologist catheter placement failed to alleviate the symptoms, the infant underwent laparoscopic excision. The lesion, however, recurred 11 months after the first excision, leading to a second surgical procedure including excision and marsupialization. A review of the literature revealed that this is a rare condition. Herein, we discuss the characteristics of the case, including medical imaging, drainage catheter placement, surgical treatment, pathological findings, and follow-up. Differential diagnoses, clinical presentations, treatment options, and patient outcomes are also discussed. Although rare, congenital pancreatic cyst should be considered in the differential diagnosis of an infant with cystic lesion of the pancreas.

Congenital pancreatic cyst with Ivemark II syndrome: a rare case.

An infant with congenital pancreatic cyst with Ivemark II syndrome is reported because it is a rare association. The infant had associated situs inversus, asplenia, and complex congenital heart disease. The pancreatic cyst was successfully managed by cystoduodenostomy because of connection to the biliary tract. The infant succumbed as a result of heart failure at age 2 months. Prognosis depends on the presence of life-threatening malformations.

Pyloric duplication with communication to the pancreas in a neonate simulating hypertrophic pyloric stenosis.

An 11-day-old neonate presents with worsening nonbilious emesis and radiographic findings suggestive of pyloric stenosis. Using a laparoscopic approach, a slightly asymmetric and hypertrophied pylorus was visualized. The unusual age of presentation and appearance of the pylorus led to the decision to perform an open exploration for suspicion of a pyloric duplication. An incision of the pyloric muscle exposed and confirmed a duplication cyst. Further exposure revealed accessory pancreatic tissue communicating from the cyst to the main body of the pancreas. The duplication cyst and accessory pancreas were resected, and the patient had an uneventful recovery. Pyloric duplication is a rare cause of congenital extrinsic obstruction in the newborn. Even rarer is a communication of the duplication cyst with the pancreas. Resection of the duplication cyst and the aberrant pancreatic tissue and duct is recommended.

Pancreatitis because of foregut duplication cyst of the pancreas treated by laparoscopic resection.

We report a rare case of a foregut duplication cyst of the pancreas containing respiratory epithelium in a healthy adolescent male who presented with recurrent abdominal pain secondary to pancreatitis. The abnormality was revealed on computed tomography performed to evaluate severe abdominal pain radiating to the back. Further imaging (ultrasound and magnetic resonance imaging) confirmed that the lesion was a cyst with no apparent pancreatic ductal communication. Elective laparoscopic resection of the cyst was performed with complete resolution of the patient's symptoms.

Neonatal congenital pancreatic cyst: diagnosis and management.

Congenital pancreatic cysts are extremely rare in newborns. The case of a neonatal congenital pancreatic cyst with pancreatitis is reported. The rapid increase in cyst size concomitantly with clinical symptoms prompted surgical management. Intraoperatively, the pancreatic head showed signs of chronic pancreatitis, and in attempts to preserve most of the functional pancreatic tissue, a Roux-en-Y cystojejunostomy was performed. Histology demonstrated a true pancreatic cyst with degenerated epithelial lining.

Abdominal neurenteric cyst.

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a loculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy (CT) scan revealed a 16 cm multiply 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive single-layered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.

Subdiaphragmatic foregut cyst: case report, differential diagnosis, and review of the literature.

Foregut-derived cysts are uncommon lesions, particularly when located below the diaphragm. When so encountered, they enter into the differential diagnosis of upper retroperitoneal masses. We report a case of such a lesion presenting as a pancreatic mass and review the current literature on this topic.

Congenital true pancreatic cyst detected prenatally in neonate: a case report.

True congenital epithelium-lined cysts of the pancreas are rare, and the prenatal diagnosis of a true pancreatic cyst has been reported only rarely. In this article, the authors report a 3-day-old girl with histologically proven true pancreatic cyst that was detected by prenatal ultrasonography. The cystic mass was located in the left upper side of the abdomen, and total cystectomy was performed without complication.

Congenital pancreatic cyst arising from occlusion of the pancreatic duct.

Congenital pancreatic cysts, particularly solitary cysts, are rare causes of abdominal masses in newborns. The etiology of these cysts is unknown and they can vary in location within the pancreas. We present a novel case of an infant with a solitary congenital cyst of the pancreatic duct, itself, as a result of occlusion of the pancreatic duct. Solitary congenital pancreatic cysts are a rare cause of abdominal masses in infants. We recently evaluated a 7-week-old infant with progressive abdominal distension since birth. This child was found to have a large solitary pancreatic cyst. This cyst resulted from occlusion of the main pancreatic duct at the level of the ampulla resulting in cystic degeneration of the pancreatic duct. Based on our review of the literature we believe this is the first documented case of such a cyst.