Idiopathic fibrosing pancreatitis in children--a single-center experience

Purpose

The experience of a single institution on idiopathic fibrosing pancreatitis (IFP) is presented.

Methodology

This is a retrospective review of medical records of affected patients.

Results

There were 7 cases with a mean age of 7 years. Upper abdominal pain followed by jaundice was the most common presentation. One child had varicella and 1 developed Crohn's disease 3 years later. In 5 cases, diagnosis was established intraoperatively, whereas 2 cases were diagnosed preoperatively. Ultrasonography suggested the diagnosis in 2 of the 7 cases, contrast computed tomography scan in 1 of the 3 cases, and magnetic resonance cholangiopancreatography in 1 of the 4 cases. Six patients were treated by biliary enteric bypass surgery. Treatment by endoscopic biliary stenting was successful in one. There were no postoperative complications. Pancreatic biopsies showed fibrosis of exocrine elements with preservation of islets. Three patients have pancreatic atrophy, and none has diabetes at follow-up (mean, 62 months).

Discussion

Idiopathic fibrosing pancreatitis presents as biliary obstruction in children. Precise preoperative diagnosis of IFP is difficult. Noninvasive imaging has limited sensitivity. Surgery offers satisfactory long-term relief of biliary obstruction. Treatment using temporary endoscopic biliary drainage appears promising in treatment of IFP. Patients should be followed up for pancreatic insufficiency, long-term biliary obstruction, and inflammatory bowel disease.     

Primary hepatic sarcomas in children--a single-center experience over 19 years

Background/ Purpose

Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience.

Methods

A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted.

Results

Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years.

Conclusion

This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.     

Long-term outcome after partial external biliary diversion for progressive familial intrahepatic cholestasis

Background

Though patients with progressive familial intrahepatic cholestasis (PFIC) typically require liver transplantation, initial surgical treatment includes partial biliary diversion (PBD) to relieve jaundice-associated pruritus. This study was undertaken to describe long-term PFIC outcome data, which are currently sparsely reported.

Methods

Retrospective review of 7 patients diagnosed with PFIC who underwent PBD between 2004 and 2008 was directed toward long-term postoperative outcome including resolution of jaundice/pruritus, stoma complications, interval to transplantation, and death.

Results

Six patients who underwent PBD experienced short-term resolution of jaundice and pruritus. Four patients experienced persistent stoma-related complications requiring a total of 14 revisions. Three symptom-free patients have not yet required liver transplantation post-PBD (average, 70 months; range, 59-78 months). Two patients underwent orthotopic liver transplantation (average, 44 ± 18 months post-PBD). Two patients died at home because of gastroenteritis-associated dehydration before transplantation.

Conclusion

Partial biliary diversion for PFIC is effective as a bridge to liver transplantation in improving jaundice and pruritus but may be associated with a high incidence of stoma-related complications. Persistent or recurrent pruritus after PFIC is associated with an increased risk of stoma prolapse or reflux. Insufficiently replaced stomal losses over time may increase the risk of dehydration-related complications in association with gastroenteritis.     

Percutaneous cholecysto-cholangiography in the diagnosis of obstructive jaundice in infants

Background

Once it is established that a jaundiced infant has an elevated direct bilirubin level, the principal diagnostic concern is the differentiation of hepatocellular from obstructive cholestasis, of disorders of physiology from disorders of anatomy, and of disease that is managed medically from disease that is managed surgically. Traditional tests such as ultrasonography, liver biopsy, and technotium 99m HIDA scan are often not sufficiently discriminating. General anesthesia is required for invasive imaging with endoscopic retrograde cholangio pancreatography (ERCP) or operative cholangiogram. The authors describe a facile alternative using percutaneous cholecystocholangiography (PCC) with intravenous sedation.

Methods

Nine cholestatic infants underwent PCC (age, 27 to 73 days; mean, 44 days) after ultrasoundscan, liver biopsy, and ^99mTcHIDA scan failed to provide a definitive diagnosis.

Results

In the 4 infants without complete biliary filling, we found biliary atresia (3) and biliary hypoplasia (1). The biliary tree was completely opacified in 5 infants with the following diagnosis: neonatal hepatitis (2), duplication of the gallbladder (1), choledochocele (1), total parenteral nutrition (TPN) cholestasis (1). There were no complications.

Conclusions

When the etiology of cholestasis remains elusive after traditional firstline tests, PCC has proven to be an accurate simple alternative in differentiating obstructive from hepatocellular causes of infantile cholestatic jaundice.     

Connective tissue growth factor expression is increased in biliary epithelial cells in biliary atresia

Background/Purpose

Connective tissue growth factor (CTGF) has been implicated in the pathogenesis of hepatic fibrosis and is elevated in the serum of children with biliary atresia (BA). The objective of this study was to evaluate hepatic CTGF messenger RNA (mRNA) expression and its relationship to hepatic histology in children with BA.

Methods

Connective tissue growth factor mRNA expression was evaluated by in situ hybridization in 26 liver biopsies from 11 patients with BA, 11 with other diseases, and 4 autopsy controls. Serial sections were immunostained with cell-specific markers to characterize the cells expressing CTGF. Biopsies were scored for CTGF expression (0-4) and inflammation and fibrosis (1-4).

Results

High levels of CTGF expression were observed in 9 of 11 BA with localization to biliary epithelial cells and vascular endothelial cells. Connective tissue growth factor mRNA expression was correlated with fibrosis in BA and all livers. In the 11 patients with other liver diseases, 7 had CTGF expression limited to hepatic stellate cells and vascular endothelial cells. None of the 4 livers in children without liver disease had significant levels of CTGF.

Conclusions

In BA livers, novel biliary epithelia CTGF mRNA expression is high and correlates with severity of fibrosis. These data support a role for biliary epithelial cell signaling in fibrogenesis.     

Intrabiliary rupture of hydatid cysts of the liver

Background

Intrabiliary rupture is a common and serious complication of hepatic hydatidosis, and its treatment remains controversial.

Methods

Sixty-seven patients who underwent surgery for rupture of a hydatid cyst in the biliary tree were studied retrospectively. The following data were analyzed: age, sex, clinical presentation, and so on.

Results

In 55 patients, intrabiliary rupture was diagnosed preoperatively and in 12 patients intraoperatively. In 51 patients, partial pericystectomy and closed-tube drainage followed. Pericystorraphy was performed in 9 patients. Omentoplasty was performed in 5 cases. After the meticulous cleansing of the common bile duct, T-tube drainage (60 patients), choledochoduodenostomy (4 patients), or sphincteroplasty (1 patient) was added. In 4 patients, there was a persisting external biliary fistula. The mean length of hospital stay was 18 days. Follow up (1-35 years) elicited 4 recurrences and 1 postoperative death.

Conclusions

Our results in intrabiliary rupture of echinococcal cysts are considered to be satisfactory. Surgical treatment can be improved with the modern methods of investigation, wider use of newer chemotherapeutics, and appropriate modification of surgical procedures.     

Significance of ductal plate malformation in the postoperative clinical course of biliary atresia

Purpose

Ductal plate malformation (DPM) is one of the etiologic theories for the development of biliary atresia (BA). In this study, we investigated the significance of DPM in the postoperative clinical course of BA, especially as a predictive factor of jaundice clearance.

Methods

Between 1988 and 2005, 31 patients with uncorrectable BA underwent hepatoportoenterostomy and steroid therapy. Immunohistochemistry was used to characterize biliary structures using cytokeratin 19. Specimens were defined as DPM-positive if a concentric cellular arrangement was detected. This retrospective study included comparisons of preoperative characteristics, the postoperative jaundice period, and cumulative steroid doses between patients with and without DPM.

Results

Of the 31 patients with uncorrectable BA, 25 (80.6%) became jaundice-free. Ductal plate malformation was detected in 11 (35.5%) of the 31. Between the 2 groups, there were no differences in preoperative characteristics or in the postoperative jaundice-free rate. Among those who became anicteric, the postoperative jaundice period was 145.3 ± 69.9 days in the DPM-positive group (n = 9) and 81.8 ± 44.7 days in the DPM-negative group (n = 16) (P < .05). The total administered steroids were 149.7 ± 78.2 and 95.0 ± 60.2 mg/kg, respectively (P = .09).

Conclusion

Ductal plate malformation may disturb bile flow and require more steroid to improve bile drainage. Therefore, the presence of DPM in the liver predicts poor bile flow after hepatoportoenterostomy in infants with BA.     

Duct-to-Duct Biliary Reconstructions and Complications in 100 Living Donor Liver Transplantations

Objective

We evaluated the risk factors for biliary complications and surgical procedures for duct-to-duct reconstructions in adult living donor liver transplantation (LDLT).

Patients and Methods

From February 2005 to March 2008, we performed 100 cases of adult LDLT with duct-to-duct biliary reconstruction, using 64 right lobe grafts, 33 left lobe grafts, and 3 right lateral grafts. We employed 4 types of duct-to-duct procedures: all interrupted 6-0 Prolene suture (group 1, n = 9); continuous posterior and interrupted anterior wall 6-0 Prolene suture (group 2, n = 49); all continuous 7-0 Prolene suture (group 3, n = 26); and all continuous 7-0 Prolene suture with external stent (group 4, n = 16). Biliary complications were defined as an anastomosis stricture or a leakage.

Results

Thirty-four patients experienced biliary complications during the follow-up period (median, 27 months). The incidence of stricture was 27% and that of leakage, 8%. There were no perioperative, intraoperative, or anatomic risk factors for biliary complications, except the type of duct-to-duct procedure. Group 1 and 2 patients showed higher incidences of biliary strictures than groups 3 and 4 (43.1% vs 4.7%; P = .00). Group 3 patients experienced a higher incidence of bile leakage than the other groups (23.1% vs 2.7%; P = .004).

Conclusions

The type of biliary reconstruction is a factor affecting biliary complications following duct-to-duct anastomosis in LDLT. Duct-to-duct biliary anastomosis with 7-0 monofilament suture and a small external stent is a feasible procedure in LDLT that significantly reduces the incidence of biliary complications.     

The spectrum of surgical jaundice in infancy

Background

Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life.

Methods

A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors’ institution from January 1992 to December 1999.

Results

There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P < .01) and aspartate aminotransferase levels (P < .001) and came to surgery earlier (P < .01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention.

Conclusions

Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is currently expected.     

Gallbladder wall inflammatory cells in pediatric patients with biliary dyskinesia and cholelithiasis: a pilot study

Background/Purpose

Inflammation has been implicated in functional gastrointestinal disorders, including functional dyspepsia and irritable bowel syndrome. This study was undertaken to evaluate gallbladder wall inflammatory cells in children with abdominal pain related to gallstones and biliary dyskinesia to determine the candidate cell types that may be contributing to the pathophysiology of these entities.

Methods

Gallbladder specimens from 20 patients with cholelithiasis, 20 biliary patients with dyskinesia, and 12 autopsy controls were evaluated in a blinded fashion. Eosinophil, tryptase-positive, and CD3+ cell densities were determined for the lamina propria and muscularis mucosa layers and compared between groups.

Results

Patients with biliary dyskinesia and cholelithiasis had a 9- to 12-fold increase in mean and peak mast cell densities, respectively, in both layers as compared with controls. Peak (13.7 vs 8.4) and mean (9.2 vs 5.2) CD3+ cell densities were increased in the muscularis mucosae of cholelithiasis specimens as compared with biliary dyskinesia specimens.

Conclusion

Gallbladder wall inflammatory cell densities, particularly mast cells, differ between children with cholelithiasis, children with biliary dyskinesia, and controls. Future studies are warranted to define the roles for specific inflammatory cell types.     

Laparoscopic excision of choledochal cyst and Roux-en-Y hepaticojejunostomy in symptomatic neonates

Purpose

Choledochal cysts require surgical excision, preferably before the onset of cholangitis. Recently, it has become feasible to accomplish the excision laparoscopically in adults and older children. Yet, whether laparoscopic excision of choledochal cyst can be performed safely in symptomatic neonates with choledochal cyst is unclear. We herewith reviewed our experience of laparoscopic excision of choledochal cysts in neonates.

Methods

We managed 9 neonates with choledochal cysts between April 2003 and February 2007. The choledochal cysts were excised laparoscopically. The Roux-en-Y hepaticojejunostomy was fashioned extracorporeally by exteriorizing the jejunum through the extended umbilical port site. End-to-side anastomosis between the common hepatic duct stump and Roux loop was carried out intracorporeally. The patients were followed up for an average of 26 months.

Results

The patients presented with jaundice, pale stool, and deranged liver function tests. The diagnosis was confirmed with ultrasonography postnatally. The median operation time was 3.6 hours. There was no operative complication and no conversion. The blood loss was minimal. The recovery was uneventful, and the median hospital stay was 6 days. The liver function tests normalized 3 to 16 weeks postoperatively. No complication was detected at the follow-up visits.

Conclusions

Our preliminary results show that laparoscopic excision of choledochal cyst and Roux-en-Y hepaticojejunostomy in neonates is both feasible and safe. It curtails further complication of the cysts and reverses the derangement of liver function. In addition, the laparoscopic approach minimizes surgical trauma.     

Comparison of drainage techniques for biliary atresia

Purpose

Traditional Kasai portoenterostomy and porto-appendiceal duodenostomy have been utilized for biliary atresia. Differences in outcome between patients who underwent either Kasai portoenterostomy or porto-appendiceal duodenostomy were compared.

Methods

A review of all children who underwent a drainage procedure for biliary atresia from 1986 to 2000 (n = 30) was performed. Age at drainage procedure, subsequent liver transplantation, and outcomes were evaluated. Outcome variables included success rates (total bilirubin < 2.0 mg/dL) and survival rate. Statistical analysis was done with χ² and Student’s t test.

Results

Long-term follow-up was available on 28 of 30 patients. Age at biliary drainage was insignificant. Success rates between porto-appendiceal duodenostomy (31%) and Kasai portoenterostomy (82%) were statistically significant. Survival rate for patients who underwent a Kasai portoenterostomy was 10 of 11 patients. Survival rate for patients who underwent porto-appendiceal duodenostomy was 14 of 16 patients. Overall survival rate was comparable between porto-appendiceal duodenostomy (88%) and Kasai portoenterostomy (91%).

Conclusions

Although overall survival rate was comparable, patients who underwent porto-appendiceal duodenostomy were less successful in alleviating hyperbilirubinemia compared with Kasai portoenterostomy. This is shown further by the greater incidence of subsequent liver transplantation in infants with prior porto-appendiceal duodenostomy. Although the appendix may serve as an alternative biliary conduit, traditional Kasai portoenterostomy appears to achieve better biliary drainage.     

Las hepatectomías mayores en pacientes con colangiocarcinoma e ictericia son seguras

Background

Surgical resection is the only possibility of long term survival in patients with Klatskin tumours. However, surgical resection is a challenging problem and hepatic resection is often necessary.

Objective

The aim of our study was to assess the need for biliary drainage, resection rate and outcome of hilar cholangiocarcinoma in a single tertiary referral centre.

Patients and methods

From 2005 to 2008, 26 patients with Klatskin tumours were identified and assessed prospectively with multidetector CT and MR cholangiography in special cases. Seven patients (27%) were deemed to be unresectable in pre-operative staging. A total of 19 surgical procedures were performed, 8 left hepatectomies, 5 right hepatectomies and 6 resections exclusively of the biliary tree.

Results

Resection rate was 73%, transfusion rate 53% and preoperative biliary drainage was performed only in 7 cases (37%). Major complications occurred in 11 (58%), including two post-operative deaths (10%).There were no differences in the epidemiological data, when we separately analysed the outcomes of the 9 patients with bilirubin <15 mg/dL and the 10 patients with bilirubin >15 mg/dL. Biliary drainage was required in 6 (67%) patients in the group with low bilirubin levels vs. 1(10%) in the other group (P=0.02). The mean bilirubin level in the jaundiced group was 22.1±3.9 vs. 4.7±4.3 (P<0.001) in the other group. There were no differences in the postoperative outcome between both groups.

Conclusion

Resection and survival rates have increased recently but still carries the risk of significant morbidity and mortality. Major hepatectomies in selected patients without percutaneous biliary drainage are safe.     

Pancreaticoduodenectomies in patients without periampullary neoplasms: lesions that masquerade as cancer

Background

Most pancreaticoduodenectomies (PDs) are performed to treat periampullary malignancies (PMs). Final pathologic analysis on these specimens does not always contain PMs. Our aim was to classify diseases that preoperatively mimic PMs.

Methods

A prospective database of PDs performed at a single institution was reviewed. Clinicopathologic data on patients without PM on pathologic review with preoperative suspicion of PM were studied.

Results

Of the 461 PDs performed at our institution, 45 (10%) had no PM; of these cases, 35 (78%) were performed for a clinical suspicion of malignancy. The final pathologic review showed chronic pancreatitis (CP) in 23 (66%) patients, biliary tract disease in 10 (28%) patients, duodenal ulcer in 1 (3%) patient, and distal common bile duct stricture with localized pancreatic fibrosis in 1 (3%) patient.

Conclusion

Most patients undergoing PD have evidence of a PM. A subset of patients may have lesions that mimic a PM. In these patients, when PM cannot be ruled out, if possible, they should be offered PD.     

Advanced Glycation End Products in the Development of Ischemic and Dilated Cardiomyopathy in Patients With Diabetes Mellitus Type 2

Introduction

Hyperglycemia intensifies nonenzymatic glucose coupling to tissues, resulting in myocardial stiffness and formation of advanced glycation end products (AGE). The aim of this study was to assess seeking AGE in the myocardium from patients with type 2 diabetes (DM2) subjected to orthotopic heart transplantation (OHT), seeking to establish whether AGE play a role in the development of cardiomyopathies leading to OHT.

Material

The 2 studied groups consisted of 11 hearts explanted from patients with ischemic cardiomyopathy+DM2 (ICM+DM2, 55 ± 6.5 years) and 8 from dilated cardiomyopathy+DM2 (DCM+DM2, 49.6 ± 4.5 years). Comparative subgroups were composed of nondiabetic explanted hearts, 41 with ICM (52.8 ± 5.8 years) and 41 with DCM (52.7 ± 4.2 years). All patients were males.

Methods

We examined immunohistochemical localization of AGE using a semiquantitative scale of reaction intensity in cardiomyocytes, fibroblasts, capillaries, arterioles, and arteries. Additionally, we calculated the scores for cardiocytes (AGE_Cardiocyte) and all left ventricular components (AGE_LV).

Results

The cytoplasmic AGE deposits in cardiomyocytes were predominantly diffuse-granular in DM2 groups, whereas nondiabetic groups showed a lack of a reaction or a diffuse pattern. There were no differences in the reaction intensity between the 2 studied groups, or 2 comparative groups. All myocardial constituents showed higher AGE intensity in DM2 than nondiabetic groups. Only in the ICM+DM2 group did the DM2 duration correlate with AGE staining in selected myocardial layers and with AGE_Cardiocyte and AGE_LV.

Conclusions

The presence of AGE in the hearts of patients requiring transplantation was related to the duration of DM2. The deposition of AGE in left ventricular myocardium was enhanced by DM2 particularly in patients with ICM.     

Outcome of central hepatectomy for hepatoblastomas

Background/Purpose

Central hepatoblastomas (CHBL) involving liver segments (IV + V) or (IV + V + VIII) are in contact with the portal bifurcation. Their resection may be achieved by central hepatectomy (CH) with thin resection margins on both sides of the liver pedicle, by extended right or left hepatectomy with thin resection margins on one side, or by liver transplantation with thick free margins. The aim of this study is to assess the operative and postoperative outcome of CH for hepatoblastoma.

Methods

This was a retrospective monocentric study of 9 patients who underwent CH for CHBL between 1996 and 2008.

Results

The operative time was 4 hours 50 minutes (2 hours 20 minutes to 7 hours), vascular clamping lasted 30 minutes (0-90 minutes), and the amount of blood cell transfusion was 250 mL (0-1800 mL). Two patients had biliary leakage requiring percutaneous drainage. Median follow-up time was 27 months (14-120 months). All of 8 nonmetastatic patients are alive and disease-free; 1 metastatic patient died of recurrent metastases at last follow-up. Although 3 of 9 patients had surgical margins less than 1 mm, none, including the patients who died from metastases, had local recurrence.

Conclusions

Our study demonstrates the feasibility of CH for CHBL without operative mortality or local recurrence. Central hepatectomy is an alternative to extensive liver resections in selected patients.     

Problems during and after pregnancy in female patients with biliary atresia

Background/Purpose

Advances in the management for biliary atresia (BA) have improved the prognosis and has greatly increased the number of long-term survivors. Even the long-term survivors, however, still face some problems. This retrospective review was performed to assess pregnancy-associated issues in long-term survivors after surgery for BA.

Materials and Methods

Of 55 patients with BA surviving for 16 years or more without liver transplantation, 9 patients have experienced pregnancy and delivery. Clinical courses, the outcome of pregnancy and delivery, and current statuses were retrospectively evaluated from their clinical records.

Results

The study revealed 14 pregnancies and 11 deliveries. Before pregnancy, no patient showed visible jaundice, but 6 patients had some complications such as episodes of cholangitis and portal hypertension. Two of the patients had both conditions and 2 others developed visible jaundice after pregnancy. One intrauterine fetal death occurred.

Conclusions

Our retrospective review suggests that the previously mentioned conditions can be risk factor for cholangitis and gastrointestinal bleeding during or after pregnancy but are not considered to be contraindications for pregnancy and delivery. Complications can occur with pregnancy even during the normal course. Thus, careful observation is recommended.     

Histologic oddities at the porta hepatis in biliary atresia

Purpose

Highly unusual histologic findings at the porta hepatis in 3 infants who underwent Kasai portoenterostomy for biliary atresia are reported.

Methods

Portoenterostomy was performed using a standard operative technique. Serial transverse sections of the excised portal plate were examined by light microscopy along with sections from the distal extrahepatic biliary remnants, gallbladder, and liver biopsy.

Results

Of 61 consecutive infants who underwent Kasai portoenterostomy for biliary atresia, 3 were found to have highly unusual histologic features at the porta hepatis. All had type 3 biliary atresia. Two had hilar biliary ductules lined in part by squamous epithelium, and the third had a focus of mature hyaline cartilage surrounded by perichondrium adjacent to biliary ductules. In each case, these unusual histologic features were localized to the porta hepatis in the region of the transected portal plate.

Conclusions

The presence of hyaline cartilage at the portal plate is likely to be an expression of defective morphogenesis, thus supporting the concept of disordered embryogenesis in the etiology of biliary atresia. Squamous epithelium within biliary ductules might also reflect a similar mechanism but could alternatively be an unusual metaplastic response to inflammation at this site.     

Endoscopic drainage of pancreatic pseudocysts in children

Background/Purpose

Symptomatic pancreatic pseudocysts have traditionally been managed with surgical, percutaneous, and, more recently, endoscopic drainage. Although the role of the latter is well defined in the adult population, its utility in children needs to be clarified. The authors reviewed their experience with endoscopic drainage of pancreatic pseudocyst (EDPP).

Methods

A retrospective chart review was conducted, and relevant demographic and clinical data were obtained for all patients with pancreatic pseudocysts managed with endoscopic drainage in the period from 1997 through 2001, inclusive.

Results

Three children had successful endoscopic drainage of pancreatic pseudocysts. They were 9, 13, and 14 years old, and were all boys. The etiology of the pancreatitis was idiopathic related to anomalous pancreatic divisum ducts in the first 2 and azathioprine induced in the latter. The first 2 patients had endoscopic transpapillary drainage, whereas the third had an endoscopic cystduodenostomy. All patients had complete resolution of the pseudocyst clinically and radiologically after follow-up periods of 3, 31, and 21 months, respectively. The first needed a subsequent pancreaticojejunostomy for persistent symptoms related to chronic pancreatitis. A successful endoscopic drainage of a posttraumatic pancreatic pseudocyst has previously been reported from our institution.

Conclusions

This experience would indicate that endoscopic drainage of pancreatic pseudocyst is an effective and relatively safe option of managing this problem in children.