Figure 103-56. The preferred operative treatment of type I choledochal cyst consists of primary total transmural cyst excision with

Roux-Y hepaticojejunostomy.

Other Types of Cysts. Type II cysts are excised completely and the choledochotomy closed primarily.

Type III choledochoceles require a transduodenal approach with either marsupialization or excision of

the cyst. Care must be taken to identify the ampulla, and a formal sphincteroplasty may be required to

ensure adequate drainage from the biliary tract and the pancreas. Type IV and V intrahepatic cystic

diseases must be approached on an individualized basis. In general, unilobar or focal cystic disease can

be either resected or drained with Roux-Y jejunostomy reconstruction. Bilobar intraparenchymal

disease, particularly in the setting of hepatic fibrosis, may be difficult, if not impossible, to obtain

complete, adequate drainage.

Complications and Outcome. The major complications associated with operative repair of choledochal

cyst include cholangitis, stricture formation, choledocholithiasis, and development of biliary tract

malignancy. A summary of the incidence of complications, mortality, and reoperation for the different

procedures in 955 cases reported in the literature demonstrated a clear advantage for primary cyst

excision in terms of morbidity and need for reoperation, and no significant increase in mortality

occurred using this approach.189 Subsequent to this analysis in 1975, internal drainage procedures

without cyst excision have been essentially replaced by primary cyst excision with hepaticojejunostomy

reconstruction. Currently, several series have reported excellent results with little or no mortality using

total transmural excision of choledochal cysts.190 Primary transmural excision with biliary tract

reconstruction continues to be a safe and desirable operative approach for cystic lesions of the biliary

tract.

PEDIATRIC PANCREAS

Disorders of the pancreas are uncommon in infants and children. Pancreatic disorders in children include

congenital anatomic disorders, inflammatory pancreatitis, rare neoplastic lesions, and pancreatic

endocrinopathies.191 Several significant issues are relative to the pancreatic problems in the pediatric

age group, and a brief overview is presented in the following discussion.

Embryology

The fetal pancreas arises from the paired dorsal and ventral foregut diverticular buds during the sixth

week of gestation. The dorsal pancreatic anlage gives rise to the body and tail of the pancreas as well as

the main pancreatic duct. The ventral pancreatic anlage migrates 180 degrees to fuse with the dorsal

gland, forming the uncinate process and the distal portion of the duct of Wirsung (Fig. 103-57). The

independent ductal systems of the developing pancreatic anlage fuse. The dorsal (Santorini) duct opens

directly into the duodenum, and this anatomy is persistent in 10% to 15% of normal subjects. The

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ventral (Wirsung) duct opens into the duodenum by fusion with the common bile duct. Normally, the

dorsal duct fuses with the ventral duct just to the right of the mesenteric vessels. Failure of the dorsal

and ventral ducts to fuse normally leads to two separate pancreatic ducts, a condition known as

pancreatic divisum. Occasionally, the ventral duct regresses and the entire pancreatic gland is drained by

the accessory duct of Santorini.

Figure 103-57. A: Normal pancreatic ductal anatomy. B: Pancreas divisum. There is no communication between the duct of

Wirsung and the duct of Santorini. The duct of Wirsung is short or absent. Most of the pancreas is drained by the duct of Santorini

through the accessory papilla. This anatomy is found in about 10% to 15% of normal individuals.

Primitive duct cells of the pancreas give rise to both the acinar cells and the islet cells of the normal

pancreas. Primary islet cells become apparent at week 8 of gestation within the interlobular tissue, with

acini becoming visible shortly thereafter. The primary islet cells regress after the fifth month of

gestation and are usually nonexistent by term. Secondary islet cells arise from centroacinar proliferation

during the third month of gestation. These intralobular cells migrate out of the acini in which they were

formed and develop into islets that remain connected to the duct by a thin cellular stalk (the tubule of

Bensley).

Exocrine pancreatic anomalies generally arise from defective development during embryogenesis. The

most common of these conditions is annular pancreas. The circumferential distribution of pancreatic

tissue around the duodenum is thought to result from interrupted migration of the ventral pancreatic

anlage. Less common defects include pancreatic ductal abnormalities, pancreatic duplications,

heterotopic pancreatic tissue, and congenital agenesis of portions of the pancreas.

Acute Pancreatitis

Acute pancreatitis is often overlooked as a cause of abdominal pain in children; however, it is the most

common disorder of the pancreas in both infants and children. Adult pancreatitis is often caused by

either alcohol ingestion or gallstones. In contrast, 50% to 80% of cases in children, particularly

adolescents, are posttraumatic or idiopathic in origin. An important clinical condition seen with

moderate frequency is acute pancreatitis in the setting of immunosuppression, in particular, high-dose

corticosteroids. Steroid-induced acute pancreatitis typically accounts for the third most common cause of

pancreatitis in most children’s hospitals. Other causes of acute pancreatitis include gallstones, CF,

hyperlipidemia, juvenile diabetes mellitus, mumps, coxsackievirus infection, infectious mononucleosis,

collagen vascular diseases, and anatomic lesions such as choledochal cyst. Medications significantly

associated with acute pancreatitis include oral contraceptives, chlorothiazide, tetracyclines.

azathioprine, and L-asparaginase. Valproic acid, an anticonvulsant useful in some children with seizure

disorders, is associated with a particularly virulent form of acute necrotizing pancreatitis that may be

life-threatening.

A child with acute pancreatitis presents with a history of midepigastric abdominal pain, anorexia, and

emesis. On examination, the child usually has a tender abdomen with distention. Fever and leukocytosis

are common. Serum amylase and lipase levels are elevated, although significant pancreatitis can occur

despite a normal amylase level. Conversely, the lipase may be mildly elevated in the absence of clinical

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symptoms or signs of acute pancreatitis. Ultrasonography and CT imaging of the abdomen are probably

the most commonly used adjunctive diagnostic tests. CT imaging is particularly useful in demonstrating

edema of the gland as well as evaluating for peripancreatic fluid collections. While usually deferred

during the acute phase of pancreatitis, ERCP or MRI cholangiography may be useful to evaluate the

hepatobiliary tract anatomy once the inflammatory process has resolved.

Treatment principles for childhood acute pancreatitis are not different from strategies used in adults.

The vast majority of children have simple edematous pancreatitis. Hemorrhagic pancreatitis and

necrotizing pancreatitis are distinctly unusual in this age group but can be seen in immunosuppressed

patients. Therefore, initial therapy is designed to be supportive and nonoperative in approach.

Endotracheal intubation and mechanical ventilation may be required. Intravenous fluid resuscitation,

pancreatic rest by nasogastric tube decompression, analgesia, and TPN are provided. No clinical efficacy

data are available to support the widespread use of anticholinergics or somatostatin analogues for acute

pancreatitis in children. Operative intervention is reserved for complications of pancreatitis such as

hemorrhage, infected pancreatic necrosis, or pancreatic pseudocyst.

Recurrent pancreatitis in a child usually is associated with an anatomic abnormality or specific

physiologic problem. Following recovery from the acute inflammatory event, an aggressive diagnostic

workup, including consideration of ERCP or MRCP to define ductal anatomy, should be performed. In

particular, the presence of pancreas divisum with stenosis of the accessory papilla should be considered.

Children with pancreatitis in the setting of cholelithiasis should undergo laparoscopic cholecystectomy

after resolution of the pancreatitis.

Pancreas Divisum

Pancreas divisum occurs with failure of normal fusion between the dorsal and ventral pancreatic ducts.

About 10% to 15% of normal persons have two separate pancreatic ducts, and up to 10% of

asymptomatic persons undergoing ERCP have two complete ductal systems. The anatomic variant of

pancreas divisum is not necessarily pathologic, but if the orifice of the accessory papilla is stenotic or

obstructed, pancreatitis can occur.192 Approximately 25% of affected individuals are thought to be at

risk for developing pancreatitis. Because pancreas divisum is characterized by a dominant dorsal duct

system dependent on secretion through the accessory papilla, stenosis of the accessory (lesser) papilla is

probably necessary to produce pancreatic symptoms. Most of the experience with surgical treatment of

pancreas divisum is reported in adults. Successful surgical management of children presenting with

recurrent pancreatitis secondary to accessory papilla stenosis with pancreas divisum also has been

reported.193

To diagnose pancreas divisum, ERCP is required. Endoscopic visualization of the major and accessory

papillae is essential. Radiographic findings with pancreas divisum demonstrate a short or absent duct of

Wirsung that does not communicate with the duct of Santorini (see Fig. 103-57). Definitive diagnosis of

pancreas divisum is made by demonstrating two separate, parallel ductal systems.

Few pediatric patients with pancreatitis and pancreas divisum requiring operation have been

reported. Most of these children were female patients who had a history of recurrent pancreatitis. The

primary operative goal is to provide adequate drainage of the duct of Santorini by performing a

sphincterotomy of the accessory duct. An open dorsal duct sphincterotomy appears to be more durable

than endoscopic sphincterotomy. Some surgeons advocate sphincterotomy of the main papilla as well,

but dorsal duct sphincterotomy alone appears effective in preventing acute pancreatitis associated with

pancreas divisum. The reported surgical outcome in the limited number of children treated for

pancreatitis associated with pancreas divisum is favorable.

Pancreatic Cysts

Pancreatic pseudocysts are uncommon in the pediatric age group and are usually the result of blunt

traumatic abdominal injury or acute pancreatitis. Children typically present with abdominal pain,

nausea, emesis, and weight loss. There may be a palpable midepigastric mass on examination. Diagnosis

usually is made with ultrasound or CT imaging of the abdomen. The serum amylase and lipase are

usually elevated.

In children, many small asymptomatic pseudocysts regress spontaneously with resolution of the

pancreatic inflammation. Larger or symptomatic pseudocysts may require drainage. Symptoms and

potential complications of untreated pseudocysts include hemorrhage, infection, perforation,

gastrointestinal or biliary tract obstruction, or, rarely, development of pancreaticoenteric fistula. The

decision to use either external or internal drainage procedures for a pseudocyst depends on the status of

3034

the pancreatic duct. In many centers, definitive external drainage of a large or symptomatic pseudocyst

is performed with acceptable morbidity and mortality rates by an ultrasound- or CT-guided

percutaneous approach.194 Pseudocyst recurrence or persistent external drainage without resolution

suggests significant pancreatic duct injury or complete ductal transection. Determination of whether a

major pancreatic ductal injury is associated with a pseudocyst may be made by either percutaneous

contrast injection of the pseudocyst or ERCP.195 Pseudocysts associated with pancreatic duct injury may

not resolve with percutaneous external drainage or endoscopic pancreatic duct stenting, so internal

drainage procedures such as cystgastrostomy or cystojejunostomy with or without distal

pancreatectomy may be required.196

Epithelial cysts of the pancreas are rare in children. Congenital cysts are lined by epithelium and

acinar tissue and are seen most commonly in the body and tail of the pancreas. They may be associated

with other syndromes such as von Hippel–Lindau disease, which is characterized by hereditary

cerebellar cysts, retinal hemangioma, and pancreatic cysts. Congenital pancreatic cysts are typically

asymptomatic unless they are large enough to cause compression or obstruction of the stomach or

colon. In the absence of trauma, spontaneous rupture of a pancreatic epithelial cyst is rare. Treatment is

cyst excision or internal drainage into the stomach or the jejunum.

Other rare cystic lesions of the pancreas in children include retention cysts. These cysts result from

chronic ductal obstruction. They are characteristically lined by epithelium unless inflammatory

obliteration has occurred. Enteric duplication cysts of the stomach or the duodenum can also be

associated intimately with the pancreas and communicate with the pancreatic duct. Treatment of both

retention cysts and enteric duplication cysts either within or associated with the pancreas includes

excision, occasionally internal drainage, and, if necessary, distal pancreatectomy. Not all pancreatic

cysts in children can be uniformly considered benign. Suspicious lesions should be investigated

thoroughly and biopsy performed. Cystadenoma, cystadenocarcinoma, and rhabdomyosarcoma

associated with a pancreatic cyst have been reported. These neoplasms are treated by anatomic

pancreatic resection following histologic confirmation on biopsy.

Pancreatic Neoplasms

Childhood malignant neoplasms of the pancreas are uncommon. Typically, pancreatic malignancy in

childhood is found on discovery of an asymptomatic abdominal mass. Infrequently, the lesion may be

found incidentally or during a diagnostic imaging workup for abdominal trauma. Jaundice may occur

with a lesion in the pancreatic head causing common bile duct obstruction. The most frequently

encountered lesions include islet cell carcinoma and adenocarcinoma. The treatment of choice for

localized malignant neoplasms of the pancreas is surgical resection.197 Pancreaticoduodenectomy in

children can successfully control some malignancies of the pancreatic head. To promote adequate

nutrient absorption and growth, children undergoing pancreaticoduodenectomy should be considered

for oral pancreatic enzyme replacement and fat-soluble vitamin supplementation.198

Two pancreatic neoplasms seen in childhood and adolescence deserve further discussion. The papillary

cystic neoplasm of the pancreas occurs predominantly in younger female children, is typically slow

growing with low malignant potential, and is highly curable with surgical resection.199 This tumor is

thought to be a neoplasm of the ductuloacinar primordial cells of the pancreas. The other lesion is the

pancreatoblastoma, also referred to as juvenile adenocarcinoma of the pancreas. Pancreatoblastoma is

somewhat more common in boys than in girls and typically has slow growth with low malignant

potential as well. Histologic examination demonstrates undifferentiated ductular and acinar areas with

nodules of squamous epithelium, suggesting that these tumors arise from primordial pancreatic cells.

Both papillary cystic neoplasm and pancreatoblastoma have more favorable prognoses than

adenocarcinoma following surgical resection.

Endocrine Lesions of the Pancreas

Zollinger–Ellison Syndrome

Childhood tumors of the endocrine pancreas are exceedingly rare. The diagnosis and management of

these tumors do not differ greatly from the adult population. The most common of these lesions include

gastrinoma and insulinoma. The functional endocrine tumors of the pancreas are characterized by

secreted peptide products such as glucagon, vasoactive intestinal peptide, somatostatin, and pancreatic

polypeptide.

Similar to adults, children with Zollinger–Ellison syndrome clinically present with symptoms related

to gastric hypersecretion. Peptic ulcer disease with or without gastrointestinal hemorrhage, abdominal

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pain, and diarrhea are common presenting symptoms. The diagnosis is confirmed by finding elevated

serum gastrin levels. The basal acid output typically is elevated as well. A paradoxical increase in serum

gastrin levels is found following intravenous administration of secretin (secretin stimulation test).

Contemporary management of Zollinger–Ellison syndrome in children relies on control of gastric acid

hypersecretion with the oral administration of a proton–pump inhibitor. Diagnostic imaging studies,

including helical CT scanning and MRI, are useful in localization of a primary gastrinoma and evaluating

for metastatic disease before exploration. Definitive treatment relies on complete excision of the

primary gastrinoma, which typically is found in the right of the superior mesenteric vessels in the head

of the pancreas or the duodenum. The growth and progression of gastrinoma appear to be less

aggressive in children than in adults; however, total gastrectomy occasionally is required in a child to

control intractable symptoms related to persistent hypergastrinemia or metastatic disease.200

About 25% of gastrinomas occur in the setting of MEN syndrome, reviewed elsewhere in this book. At

least 90% of patients with MEN type I have hyperparathyroidism secondary to hyperplasia. In addition,

30% to 80% of patients have pancreatic islet cell tumors and 15% to 50% have pituitary tumors.

Hypoglycemia

There are diverse metabolic causes for hypoglycemia in infancy and childhood. These include

endocrinopathies such as panhypopituitarism, hypothyroidism, adrenal insufficiency, and congenital

adrenal hyperplasia (adrenogenital syndrome). Several inborn errors of metabolism interrupt normal

glucose regulatory mechanisms. Systemic disease states, perinatal stress, and sepsis can predispose an

otherwise normal infant to low blood glucose levels. Infants who remain unresponsive to glucose

infusion typically have inappropriately high circulating insulin levels for a given blood glucose level.

Hyperinsulinemia should be suspected in any infant or child younger than 1 year with persistent

hypoglycemia. There are heterogeneous causes for hyperinsulinemic hypoglycemia, but the most

common historical cause in infancy is nesidioblastosis, which is characterized by uncontrolled

development of the pancreatic endocrine tissue that functions abnormally during infancy.201,202

Clinically, these cells secrete inappropriately high amounts of insulin, causing clinical hypoglycemia in

infants. Two distinct histologic lesions appear to be responsible for congenital hyperinsulinism.

Mutations in the beta cell KATP channel genes may lead to either focal, adenomatous hyperinsulinism or

diffuse hyperinsulinism; both genotypes lead to phenotypic hypoglycemia if left untreated.

Infants with congenital hyperinsulinism usually become symptomatic from hypoglycemia within the

first few hours or days of life. These infants commonly present with neurologic symptoms such as

lethargy or generalized seizures and have corresponding fasting blood glucose levels below 40 mg/dL.

The diagnosis of hyperinsulinemia is supported by the clinical features of Whipple triad that includes (a)

neurologic changes with fasting or activity, (b) fasting blood glucose levels below 40 to 50 mg/dL, and

(c) neurologic symptoms reversed by the administration of glucose. The diagnosis is made by

demonstrating inappropriately high levels of circulating insulin for a given level of blood glucose. An

insulin (IU/mL) to glucose (mg/dL) ratio that is greater than 0.5 in a fasting patient is consistent with

hyperinsulinemic hypoglycemia. In infants and children, an absolute insulin level greater than 5 IU/mL

in the presence of a blood glucose less than 40 mg/dL is diagnostic. Ketone body production is impaired

in infants with congenital hyperinsulinism.

The initial management of a hypoglycemic infant with congenital hyperinsulinism is to provide

adequate glucose concentrations to prevent permanent neurologic injury. Dextrose-containing

intravenous solutions are titrated to maintain blood glucose levels greater than 40 mg/dL and may

require a central venous catheter. The short-term administration of somatostatin analogues to increase

blood glucose levels in hyperinsulinemic states has been demonstrated to be useful.203 Other

pharmacologic agents used to reduce insulin levels and raise blood glucose concentration include

diazoxide (15 mg/kg daily). The use of streptozocin to control hyperinsulinemia most often is reserved

for adults with metastatic islet cell carcinoma and is not widely used in infants because of the potential

side effects.

Following initial control of blood glucose and clinical confirmation of hyperinsulinemia, operative

intervention should be considered a means of providing definitive control of hypoglycemia. Diagnostic

imaging using CT scan imaging, MRI, or more recently,18 F-FDOPA PET scanning may be useful either

to identify or to exclude a focal lesion.204 Localization of focal lesions is important in that this approach

may lead to effective glucose control with selective partial pancreatectomy and allow for preservation

of normal pancreatic tissue and function.205,206

Operatively, the abdomen is explored thoroughly, and the entire pancreas must be visualized.

3036

Preoperative localization data guided by intraoperative ultrasound and frozen section analysis may

identify focal lesions amenable to partial resection. In the absence of a focal lesion, the infant is

presumed to have diffuse islet cell hyperplasia. In this setting, the general operative strategy is total or

near total pancreatectomy.207 Lesser procedures such as subtotal (80%) pancreatectomy do not

effectively treat diffuse congenital hyperinsulinism and recurrent hypoglycemia places the infant at risk

for hypoglycemic encephalopathy. Near-total pancreatectomy involves resection of the distal 95% of the

gland with preservation of the spleen. The entire distal pancreas, including the uncinate process, is

resected, leaving a small rim of pancreatic tissue adjacent to the duodenum (Fig. 103-58). Total

pancreatectomy is usually reserved for persistent or recurrent hypoglycemia following lesser

procedures. Near-total pancreatectomy controls hypoglycemia in 90% of infants with diffuse congenital

hyperinsulinism. The remaining infants with persistent hypoglycemia may require further pancreatic

resection. With extensive pancreatic resection, the typical postoperative course is a transient period of

hyperglycemia with subsequent stabilization of blood glucose levels. Pancreatic exocrine function is

ablated and oral replacement therapy is required. Despite clinical remission following resection,

however, diabetes mellitus may occur as a long-term consequence in children treated either medically

or surgically for diffuse congenital hyperinsulinism.208 Therefore, these infants need long-term

metabolic follow-up for both pancreatic endocrine and exocrine function.

Figure 103-58. Illustration of the various degrees of pancreatic resection.

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