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Single incision laparoscopic surgery as a surgical approach in treatment of pancreatic disease has recently been reported in adults. However, its application in persistent hyperinsulinemic hypoglycemia of infancy (PHHI) in children is limited. In this article, we report single incision laparoscopic 90 % pancreatectomy for the treatment of persistent hyperinsulinemic hypoglycemia of infancy. Between July 2011 and February 2015, the single incision laparoscopic 90 % pancreatectomy was performed in three children with PHHI. All patients underwent (18)F-FDOPA PET/CT before the surgeries. The scans showed diffuse physiologic (18)F-FDOPA activity in entire pancreas. All patients were followed up. The levels of blood sugar and insulin were recorded postoperatively. The time required for surgery was 120-230 min, and blood loss was minimal. The hospital stay was 6 days. The duration of postoperative abdominal drainage was 4-5 days. The levels of fasting blood glucose after surgery were higher than those before surgery (4.38-8.9 vs. 0.54-1.8 mmol/L). The levels of fasting insulin after surgery were lower than those before surgery (2.4-5.5 vs. 14-33.3 uU/ml). The duration of follow-up was 4-46 months. During follow-up, the levels of blood glucose and insulin were normal in three patients. There was no recurrence of hypoglycemia after operation in all patients. Single incision laparoscopic 90 % pancreatectomy for children with PHHI is feasible and safe in well-selected cases in the experienced centers.

Solid-cystic papillary tumor (SCPT) of the pancreas is a rare neoplasm in children. The current study attempted to estimate the incidence and possible pathological origin of the tumor. Clinicopathologic features of the children under the age of 16 years with pancreatic tumors managed in a single institution between January 1993 and November 1999 were reviewed. Representative blocks of SCPT specimens were immunostained for neuron-specific enolase (NSE) chromogrannin, synaptophysin, insulin, glucagon, somatostatin, and gastrin. There were three SCPTs, two pancreatic endocrine tumors, and one acinar cell carcinoma during the study period. The estimated yearly incidence in the referral area of 2 million population is about 0.01 pediatric SCPT per year per 100,000 population. The children underwent surgical removal of the tumors. Postoperatively, they were followed up for 6 months to 4 years and were well. Immunostaining for NSE, chromogranin, synaptophysin, insulin, somatostatin, and gastrin was negative in all cases. SCPT may thus be the most common pancreatic tumor in the Asian pediatric population. The pathological origin of the tumor remains unclear and requires further investigations.

Diagnosis of duodenal and pancreatic injuries is frequently delayed, and optimal treatment is often controversial. Fourteen children with duodenal and/or pancreatic injuries secondary to blunt trauma were treated between 1980 and 1997. The pancreas was injured in all but 1 child. An associated duodenal injury was present in 4. The preoperative diagnosis was suspected in only 6 patients based on clinical signs and ultrasonography. One patient was treated successfully conservatively; all the others required surgical management. At operation, three procedures were used: peripancreatic drainage, suture of the gland or duodenum with drainage, and primary distal pancreatic resection without splenectomy. A duodenal resection with reconstruction by duodeno-duodenostomy was performed in 1 case. The overall complication rate was 14%: 1 fistula and 1 pseudocyst. Pancreatic ductal transection was recognized 3 days after the initial laparotomy by endoscopic retrograde cholangiopancreatography (ERCP). The mortality was 7%; 1 patient died from septic and neurologic complications. When the diagnosis of pancreatic ductal injuries is a major problem, ERCP may be a useful diagnostic procedure. Pancreatic injuries without a transected duct may often be treated conservatively. The surgical or conservative management of duodenal hematomas is still controversial; other duodenal injuries often need surgical treatment.

The authors present the case of a 12-year-old boy with a history of chronic upper abdominal pain. Based on ultrasonography, endoscopic retrograde cholangiopancreatography, and computerized tomography, a diagnosis of chronic calcific pancreatitis with dilation of the main pancreatic duct and a pseudocyst of the proximal pancreas was made. At surgery, in addition to confirmation of the above findings, a pseudoaneurysm arising from the splenic artery was found in the head and body of the pancreas. Pancreaticoduodenal resection including the pseudoaneurysm was performed. Postoperatively the child developed diabetes, which is easily managed with small doses of insulin.

Pancreatitis in children is uncommon, but can be the cause of significant morbidity. We undertook this study to review recent changes in the management of pediatric pancreatitis. Between 1974 and 1992, 57 cases of pancreatitis in children up to 19 years of age were treated at our institution. The etiologies were quite diverse and included idiopathic, traumatic, drug-related, biliary, congenital, and alcoholic causes. The diagnosis of pancreatitis was made from the clinical presentation and laboratory values in most cases. Serum amylase was elevated in the majority of children. Further diagnostic evaluations included abdominal ultrasound, computed tomography, and endoscopic retrograde cholangiopancreatography. Pseudocyst development was the most common complication, especially in those cases associated with trauma. In contrast to adults with pancreatitis, who usually respond to nonoperative therapy, only 32 of the 57 children responded to nonoperative treatment. The most common long-term morbidity was recurrent pancreatitis; however, the majority of patients suffered no long-term sequelae of their pancreatitis or its treatment.

We present two cases of nesidioblastosis, a common cause of persistent hypoglycemia in infancy that, if inadequately treated, can lead to mental retardation. Tissue insulin data obtained from both radioimmunoassay and immunohistochemistry are presented. In each case, the insulin content correlated well with the quantity of insulinpositive cells in each portion of the pancreas. However, the insulin content varied from case to case and from portion to portion of the same pancreas. Thus, discrepancies in clinical results in nesidioblastosis may be due to variability of insulin content in the resected pancreas.

A rare case of a gastric duplication in the tail of the pancreas in a child presenting with recurrent abdominal pain and evidence of pancreatic calcification suggesting pancreatitis was cured by excision of the cyst and adjacent pancreas. Congenital causes of pancreatitis are rare, but are curable with surgery. These lesions are often misdiagnosed, and patients may be subjected to inappropriate surgery. Imaging is sensitive in the detection of such lesions, but the lack of specific features necessitates an index of suspicion. Similar cases have been described previously, but the pancreatic tail is rarely involved. Pediatr Surg Int

The diagnosis of a non-surgical cause of delayed passage of meconium in a neonate may be challenging to the pediatric surgeon. The usefulness of determining trypsin activity (TA) in stool and duodenal aspirate for the diagnosis of cystic fibrosis (CF) and the existence of an entity called transient deficiency of trypsin (TTD) was assessed in 49 neonates over a 14-month period. TA was determined by a gelatin liquefaction technique. An absence of TA was considered if gelatin liquefaction was seen at a dilution of less than 1:100. Neonates with negative activity in both samples were started on supplementary pancreatic enzymes (pancreatin) for 1 month. TA was retested in the stool after stopping pancreatin for 1 week. Neonates with a return of TA were considered to have TTD; those who showed persistent TA deficiency at re-evaluation were investigated for CF by sweat iontophoresis. Twenty-one neonates had negative TA (in 1 only stool was tested); 13 could be re-evaluated (4 died, 4 were lost to follow-up). Nine were found to have TTD as a stool or duodenal sample showed the presence of TA; 3 of 4 patients with persistent negative TA were confirmed to have CF. One patient with normal sweat chloride is awaiting genetic studies. Determination of TA by gelatin liquefaction is a simple, rapid, inexpensive, and reliable (sensitivity 100%, negative predictive value 100%) means to differentiate non-surgical causes of intestinal obstruction such as neonatal and postoperative TTD and CF.

Persistent pseudocysts, which are rare in children, have traditionally been managed by open surgery. We describe two children who presented with large, established pseudocysts of the pancreas. They were successfully treated in the short term by minimally-invasive stent placement to create an internal cyst-gastric communication. This approach merits comparison with open surgical techniques.

Anomalous pancreaticobiliary union (APBU) has varied presentations. We report the case of a 12-year-old female who presented with biliary peritonitis due to a perforation of the common bile duct due to impaction of a pancreatic calculus at the duodenal papilla. She had a long common-biliary channel and pancreas divisum with chronic calcific pancreatitis involving the pancreatic head and neck. To our knowledge, this is the first such reported case in the literature.

Two children with incidentally-diagnosed ectopic pancreatic tissue in the jejunum at surgery for extrahepatic biliary atresia (EHBA) and choledochal cyst (CC) are reported. No case has been reported in the literature describing the association of a CC with ectopic pancreas, and only one case of EHBA associated with ectopic pancreas has been reported. We believe that incidentally-detected ectopic pancreatic tissue should be excised, even though the patient is symptom-free, in order to prevent the risk of serious complications due to either the mass effect or the potential for acute pancreatitis, cystic degeneration, or malignant transformation at a later date.

The role of pre- and intraoperative procedures for the localization of insulinomas has been extensively debated. We report a case of successful treatment using preoperative selective intra-arterial calcium injection and intraoperative glucose monitoring. A 12-year-old boy with hypoglycemic attacks had a large insulinoma in the head of the pancreas on computed tomography. Preoperative selective angiography combined with arterial stimulation-venous sampling (ASVS) by intra-arterial injection of calcium revealed no other insulinomas in the body and tail of the pancreas. Elevation of serum glucose on intraoperative monitoring confirmed complete enucleation of the insulinoma. Preoperative ASVS can accurately localize an insulinoma, and may help to increase the success rate of surgery and avoid blind pancreatectomy.

Primary hydatid disease of the pancreas is rare. The authors report a 3-year-old girl who had a cystic mass in the head of the pancreas with no other viscera involved. At laparatomy the entire endocyst was totally extracted from the surrounding pancreatic parenchyma. The postoperative course was uneventful. She is currently disease-free with a follow-up of 5 years. Only four other pediatric cases have been reported in the English literature.

We describe a rare case of pancreatic inflammatory pseudotumour that clinically presented as a malignant tumour in an 11-year-old boy. We also review all six previously described cases, including three that occurred in children. Complete surgical removal is regarded as the best choice of therapy, and follow-up in our case showed no evidence of disease after 3 years. It is important to be aware of this lesion, since it can be confused with a malignant tumour both radiologically and clinically.

Heterotopic pancreatic tissue in the small-bowel mesentery is an extremely rare anomaly: no such case has been reported in the English literature. We report a 5-year-old boy in whom the aberrant tissue formed a retroperitoneal cyst. The condition was further complicated by a ventral pancreatic duct defect. The unique clinical manifestations and difficulty in making the diagnosis are discussed.

Pancreatic tumors rarely present in childhood. Diagnostic difficulty and inappropriate treatment may occur due to the variable clinical appearance of the illness. A 26-year audit (1968-1994) at this institution showed five patients ranging in age from 5 to 14 years who were operated upon for pancreatic tumors, four of which were malignant. In two cases primary resection of the tumor was possible and in the remaining three, after establishing the histopathologic diagnosis, chemo- and radiotherapy were used. Three of the five children are still alive; the mean follow-up was 9 years.

A 14-year-old patient with a mucinous cystadenoma of the pancreas (MCAP) is presented. She presented with a palpable left-sided abdominal mass and underwent a left hemipancreatectomy. MCAP occurs mostly in middle-aged women, and no post-pubertal cases have been reported to date in the English literature.

To determine the incidence of cystic fibrosis (CF) in neonates with intestinal obstruction (NIO) secondary to meconium ileus (MI), jejunoileal atresia (JA), meconium plug syndrome (MPS), volvulus (V), and meconium peritonitis (MP) and analyze the correlation of ultrasonographic (US) signs with CF in NIO with a prenatal diagnosis of intestinal anomaly, a prospective analysis of different types of NIO from 1990 to 1998 was undertaken. Immunoreactive trypsin measurement, genetic studies, and sweat tests were performed to confirm or rule out CF. Cases with prenatal diagnosis were analyzed for gestational age, dilated bowel, ascites, hyperechoic bowel, and calcifications. Of 80 neonates, 19 (24%) had CF: 2/33 (6%) JA, 6/14 (43%) MPS, 1/14 (7.1%) MP, 10/10 (100%) MI, and 0/9 V. Thirty (37.5%) had a prenatal diagnosis of an intestinal anomaly. The overall incidence of CF in NIO with a prenatal diagnosis of intestinal anomaly was 4/30 (13%), or 333 times the estimated risk of CF in the general population. A hyperechoic pattern with dilated bowel was associated with higher specificity for CF: 3/3 cases (100%), followed by hyperechoic bowel with ascites: 3/4 cases (75%). All babies with any type of NIO should thus be screened for CF. Prenatal screening for CF should be indicated in all pregnancies with US patterns of specific intestinal disorders.

We describe an extremely rare case of acute pancreatitis presenting as an acute abdomen that appeared as a complication of mumps vaccination in a young child. A laparotomy performed because of suspected perforated appendicitis proved unnecessary in retrospect. No similar case in infancy and early childhood has been reported to date.

An aberrant pancreatic lobe associated with an enteric duplication cyst is a rare cause of relapsing pancreatitis in childhood. We present an 8-year-old boy with relapsing pancreatitis caused by this rare congenital foregut anomaly. The computed tomography (CT) findings revealed an unusually long segment of aberrant pancreatic lobe arising from the pancreatic neck, projecting anteriorly at a distance to a cystic duodenal duplication and appearing as an inflammatory mass. There has been no previous report of this unusual appearance on CT. Appreciation of the relevant anatomy provided by CT led to the successful management of this surgically-treatable cause of relapsing pancreatitis.

Drainage from the umbilicus may be due to the presence of structures derived from the vitelline duct or urachus, granulomas, ectopic tissue, or poor hygienic practice. This paper discusses the case of a 6-month-old infant with a wet umbilicus caused by ectopic pancreatic tissue, in urachal remains. No similar cases have been found in the literature.

We examined the changes in pulmonary function values in 27 patients who underwent a lobectomy due to cystic lung disease and compared the results regarding such factors as disease type, age at operation, and preoperative infections. Percent vital capacity (%VC) decreased immediately after lobectomy, but recovered to normal values within 2 postoperative years and remained within or above the normal range. The ratio of residual volume to total lung capacity (RV/TLC) rose temporally with the increase in %VC, but then remained normal after 2 postoperative years. There was no difference in %VC and RV/TLC between diseases, while bronchial atresia showed a significantly lower correlation with percent of forced expiratory volume at 1 s. The older group operated upon at over 4␣years of age and the group that had infections before operation showed relatively low %VC and high RV/TLC. Some patients demonstrated extremely low %VC along with funnel chest deformities. Our study suggests that overinflation of the remaining lung compensates VC in the early period after lobectomy while subsequently alveolar multiplication occurs. Factors affecting compensatory lung growth were considered to be operation later than 4 years of age, preoperative infection, and a thoracic deformity.

The case of a large cyst arising from heterotopically-situated pancreatic tissue in an 11-month-old girl is reported. This is the first published report of childhood pancreatic cyst that developed in heterotopic pancreatic tissue. There is strong evidence to suggest that the cyst became symptomatic as a result of secondary infection, an additional unreported phenomenon. This case serves to underscore the fact that pancreatic cysts should be considered in the differential diagnosis of intestinal duplication cysts of childhood.

Most patients with cystic fibrosis require oral administration of pancreatic enzymes to treat pancreatic insufficiency. Recent use of higher-strength enzyme preparations in large doses has been found to be associated with fibrotic strictures of the colon. We report a case of pan-colonic fibrosis due to pancreatic enzyme use.

Two children aged 11 and 9 years were operated upon for acute appendicitis and were discovered to have cystic fibrosis: the histologic pattern of the removed appendix was typical for this disorder. Appendicitis may thus be the first presenting sign of occult cystic fibrosis.

The management of a high-output pancreatic fistula is often difficult, and can be even more challenging in the pediatric patient. Octreotide acetate (OA) (Sandostatin, Sandoz, East Hanover, NJ) has served to facilitate the treatment of this difficult problem, but experience has been limited to adults. Somatostatin is a hormone that decreases the production of pancreatic exocrine and endocrine secretions. The use of the long-acting somatostatin analogue, OA, has reduced pancreatic fistula output and facilitated resolution of pancreatic fistulae in adults. This report summarizes the IV use of OA and external drainage in the complete resolution of high-output traumatic pancreatic fistulae in three pediatric patients. The treatment was well tolerated without side effects, and resulted in a dramatic decrease in the amount of fistula drainage within the first 24 to 48 h. OA can be safely administered IV (5–10 μg/kg per day) and is valuable in the management of traumatic pancreatic fistula in children.

Painless cholestasis and hydrops of the gallbladder may occur as secondary symptoms in Kawasaki syndrome. Surgical treatment is rarely indicated. Our case report confirms the validity of this conservative approach. In addition, during the course of the disease we observed a pancreatitis without severe symptoms; this has not been described in connection with mucocutaneous lymph node syndrome to date.

We report a case of recurrent pancreatitis caused by a duodenal duplication cyst with aberrant pancreatic ductal communication in a \(2{\raise0.5ex\hbox{$\scriptstyle 1$}\kern-0.1em/\kern-0.15em\lower0.25ex\hbox{$\scriptstyle 2$}}\)-year-old female who had been previously treated for duodenal atresia. The evaluation of the pancreas included a computed tomography scan, endoscopic retrograde cholangiopancreatography, intraoperative ultrasound, and intraoperative cholangiogram. In the setting of duodenal atresia, this case underscores the complex embryology of pancreatic, duodenal, and biliary development. Any infant with recurrent pancreatitis should have a complete evaluation in order to identify a potentially correctable lesion. The surgical options remain defined by the anatomy and include resection, marsupialization, or internal drainage.

Gastric outlet obstruction caused by ectopic pancreas in infants is very uncommon; there are few reported cases in the literature and in these cases the diagnostic and operative procedures were notably dissimilar. In our patient the diagnosis was established by sonography, which appears not to have been previously reported. Total excision of the tumor was possible despite the extensive inflammatory response surrounding the ectopic pancreatic tissue.

An 8-year-old male presented with an acute abdomen. Exploration revealed bilious ascites, oedematous pancreatitis with areas of necrosis, and omentum showing patches of fat necrosis. The common bile duct and almost all of the common hepatic duct were gangrenous and had sloughed, with bile leaking from the junctional stump of the right and left hepatic ducts. The patient was managed successfully by Roux-en-Y hepaticojejunostomy. This is a rare case showing necrosis and sloughing of the extrahepatic bile ducts in acute pancreatitis.

Ectopic pancreatic rests are rare. We report two cases discovered in infants who underwent surgical exploration of the umbilicus for persistent umbilical discharge with peri-umbilical excoriation. A shallow sinus leading to a firm cyst was excised in both infants, and histopathological study confirmed the presence of exocrine and endocrine pancreatic tissue. There is only one other case of ectopic umbilical pancreatic tissue reported, and this was in a patient presenting with an umbilical mass.

A case of papillary and cystic tumour of the pancreas (Frantz's tumour) in 12-year-old male is described. A review of the pathology and the paediatric literature emphasises the low-grade malignancy of this tumour in children, with a 12% incidence of local recurrence. Conservative surgery with long-term follow-up is recommended.

Six cases of successful percutaneous catheter drainage (PCD) of pancreatic pseudocysts in children ages 3–13 years are presented. All cases involved blunt abdominal trauma and presented with abdominal pain, vomiting, epigastric tenderness without a palpable mass, and elevated serum amylase. Indications for PCD were an enlarging pseudocysts and marked abdominal pain. Catheter placement was accomplished using CT guidance 15.3±1.8 days (mean ± SEM) following injury. Two patients had transgastric PCD. None of the pseudocysts were infected. Catheters were left in place until the drainage ceased, the serum amylase approached normal, the pseudocyst resolved on ultrasound, and the asymptomatic patient tolerated enteral feedings with the catheter clamped. The mean duration of catheter drainage was 18.3±3.7 days. At the time of catheter insertion, the range of serum amylase values was 242–2810 IU/l, while discharge values were 50–147 IU/l (normal: 16–108 IU/l). The mean hospital stay was 36.2±3.8 days and the mean length of total parenteral nutrition was 27.5±4.5 days. All pseudocysts were treated successfully without complication. PCD of traumatic pancreatic pseudocysts in children was performed safely and effectively in our study. Use of this therapeutic modality allowed earlier treatment, decreased the duration of symptoms, and avoided abdominal operation. PCD may prove to be the treatment of choice for pancreatic pseudocysts in children.

Only six cases of aberrant pancreatic tissue causing pyloric obstruction have been reported [1–6] thus far. We report a 27-day-old female infant with aberrant pancreas at the pylorus who showed signs similar to those of hypertrophic pyloric stenosis. Initial pyloroplasty did not relieve the obstruction, which required antrectomy and reanstomosis 8 days later.

Cystic teratoma of the pancreas is an extremely rare entity: only 11 cases have been described so far in the world literature. We report the 12th case, in a 4-month-old girl.

This report comprises ten children with hyperinsulinaemic hypoglycaemia who did not respond to medical management and required surgical treatment. Eight were infants under the age of 1 year while two were children aged 3.5 and 5 years, respectively. In each case the diagnosis of nesidioblastosis was confirmed at operation by frozen section histopathological examination and a 95% pancreatectomy was performed. No patient developed recurrent hypoglycaemia, although transient hyperglycaemia, which temporarily required insulin therapy, appeared in five children. All ten patients are now normoglycaemic without treatment.

A case of prolonged conjugated hyperbilirubinaemia in infancy due to cystic fibrosis is reported. The patient was referred as a probable biliary atresia, but because of recurrent pulmonary infections the diagnosis of cystic fibrosis was entertained and confirmed on sweat test and liver histology.