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Congenital cysts ( True cysts ):

Congenital cysts ( True cysts ) are defined as anomalous development of the pancreatic ducts.

Although their mode of origin is not understood, it is postulated that cysts are caused by the failure of embryonic ducts to regress as they are replaced by more permanent ones.

Remnants of persistent ducts are presumed to become obstructed and form cysts that fill with fluid.

Congenital cysts can be single or multiple and range in size from a few millimeters to large cysts that may fill the upper abdomen.

Such cysts are lined by cuboidal-to-flat epithelium and contain fluid with both amylase and proteolytic enzyme activity.

Cysts frequently coexist with kidney and liver cysts in congenital polycystic disease.

In von Hippel-Lindau disease, pancreatic cysts and angiomas of the central nervous system occur.

Pseudocysts:   Image Link

Pseudocyts are localized collections of fluid representing sequestered pancreatic secretions, almost always arising after bouts of acute or chronic pancreatitis.    Image Link

They do not possess an epithelial lining but instead are lined by fibrosed inflammatory tissue.

Pseudocyts are usually unilocular.

Symptoms include abdominal pain. They may become infected or hemorrhagic.

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Congenital pancreatic cyst arising from occlusion of the pancreatic duct.
Pediatr Surg Int. 2007 Jun 7;

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.

Pancreatic pseudocyst development due to organophosphate poisoning.
Turk J Gastroenterol. 2007 Jun;18(2):122-125.

Background/aims: Acute pancreatitis is a serious complication of organophosphate poisoning. There is no report in the literature dealing with the development of a pancreatic pseudocyst after complication of organophosphate-induced acute pancreatitis. Therefore, we present a case who developed pancreatic pseudocyst after complication of organophosphate-induced acute pancreatitis. Methods: A 17-year-old female patient with a history of ingestion of complication of organophosphate insecticide (DDVP EC 550, dichlorvos) was admitted with cholinergic symptoms. On admission, serum amylase and lipase levels were high and abdominal ultrasonography showed an edematous pancreas. No etiological factor for acute pancreatitis was evident. Results: We diagnosed complication of organophosphateinduced acute pancreatitis. After four weeks, abdominal abdominal ultrasonography and computerized tomography revealed a pancreatic pseudocyst of 6 cm diameter. During follow-up, the pancreatic pseudocyst size regressed to 4 cm. Conclusion: Complication of organophosphate poisoning can cause acute pancreatitis and its complications. Early diagnosis and appropriate treatment may reduce morbidity and mortality.

Congenital true pancreatic cyst: a rare case.Diagn Interv Radiol. 2006;12(1):31-3.

Congenital true pancreatic cysts are very rarely seen in children. We report magnetic resonance imaging features of a case of congenital true pancreatic cyst with a high level of enzymatic activity which, to the best of our knowledge, have not previously been reported. A 4-month-old boy was admitted to our clinic with a history of abdominal swelling for one month. A mobile, smooth, non-tender mass was palpated on the left side of the abdomen during physical examination. Ultrasonography and MR imaging revealed a bilocular cystic mass sized 9.5 x 8 x 6 cm. The spleen was displaced superiorly, whereas the left kidney was displaced posteriorly. Obliteration of the peripancreatic fat planes between the cystic mass and tail of the pancreas was observed. During abdominal surgical exploration, the pancreatic tail was larger than normal, and a pancreatic cyst arising from the tail of the pancreas was observed. Total cystectomy was performed with distal pancreatectomy. Although it is extremely rare in children, congenital true pancreatic cysts should be diagnostically considered in cases involving a cystic mass neighboring the pancreas. MR imaging is not helpful in differential diagnosis of other cysts originating from neighboring organs. Total excision with distal pancreatectomy and splenic preservation are advised for distal pancreatic cysts.

Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms.ANZ J Surg. 2006 May;76(5):325-31.

BACKGROUND: Cystic lesions of the pancreas consist of a broad range of pathological entities. With the exception of the pancreatic pseudocyst, these are usually caused by pancreatic cystic neoplasms. Non-neoplastic pancreatic cystic and cystic-like lesions are extremely rare. In the present article, the surgical experience with these unusual entities over a 14-year period is reported. METHODS: Between 1991 and 2004, all patients who underwent surgical exploration for a cystic lesion of the pancreas were retrospectively reviewed. Patients with a pancreatic pseudocyst were excluded. There were 106 patients of whom 8 (7.5%) had a final pathological diagnosis consistent with a non-neoplastic pancreatic cystic or cystic-like lesion, including 3 patients with a benign epithelial cyst, 2 with a pancreatic abscess (one tuberculous and one foreign body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic cyst. These eight patients are the focus of this study. RESULTS: There were six female and two male patients with a median age of 61.5 years (range, 41-71 years). All the patients were of Asian origin including seven Chinese and one Indian. Four of the patients were asymptomatic and their pancreatic cysts were discovered incidentally on radiological imaging for other indications. All the patients underwent preoperative radiological investigations, including ultrasonography, computed tomography or magnetic resonance imaging, which showed a cystic lesion of the pancreas. Three patients, all of whom were symptomatic, were diagnosed preoperatively with a malignant cystic neoplasm on the basis of radiological imaging. Two patients were eventually found to have a pancreatic abscess, one tuberculous and the other, secondary to foreign body perforation. The third patient was found on final histology to have chronic pancreatitis with retention cysts. The remaining five patients had a preoperative diagnosis of an indeterminate cyst; on pathological examination, they were found to have a benign epithelial (congenital) cyst (n = 3), retention cyst (n = 1) and mucinous non-neoplastic cyst (n = 1). At a median follow up of 20 months (range, 3-34 months), none of the patients had any evidence of recurrent disease. CONCLUSION: Non-neoplastic cystic and cystic-like lesions of the pancreas are rare causes of pancreatic cystic lesions that are generally benign and do not require surgery when asymptomatic. However, despite advances in diagnostic investigations such as endoscopic ultrasound with fluid aspirate and magnetic resonance imaging, the preoperative diagnosis remains unreliable. Hence, the challenge for all clinicians is to recognize these lesions preoperatively and to avoid 'unnecessary' surgery.

Three children with pancreatic (pseudo)cysts as the cause of undefined abdominal pain.Ned Tijdschr Geneeskd. 2006 Mar 4;150(9):500-4.

Three children, two boys aged 5 years and one 2-year-old girl, who were referred because of abdominal pain of variable duration, were found to have cystic malformations that arose from the pancreas. In the first boy, a traumatic pseudocyst was found that eventually turned out to have been caused by child abuse. The second boy had pseudocysts complicating chronic pancreatitis of presumably hereditary origin. In the girl, a congenital pancreatic cyst was found. Pancreatic disease, although rare, should be considered in the differential diagnosis of abdominal pain in children.


August 2007

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