Approach Considerations
Cases of uncomplicated acute pancreatitis usually resolve within 2-4 days. The management of acute pancreatitis is predominately supportive medical therapy, with intravenous hydration, pain control, and bowel rest. Parenteral nutrition may be required for prolonged episodes.
Nutrition has an important role in the management of patients with mild acute pancreatitis. A database analysis of all children admitted with mild acute pancreatitis from a major institution failed to show any relationship between pain severity among those patients who received either a low fat or regular diet compared to those patients who were restricted from oral feeding. [10] Moreover, most patients tolerated their nutrition by mouth when compared with those children who received nutritional therapy either from a nasogastric or jejunal feeding tube.
Surgical management is used to address complications of pancreatitis, including hemorrhage, necrosis, ductal fistulae, and pseudocysts. Surgical management of pancreatic pseudocysts is highly successful. Recurrence rates and mortality rates are low. Internal drainage is associated with lower recurrence rates compared to percutaneous or endoscopic drainage. Transendoscopic and percutaneous drainage of pancreatic pseudocysts have been predominately performed in the adult population, and further investigation and comparison of these techniques in children is warranted to determine the optimal management of this disease.
Sphincteroplasty is contraindicated in patients with evidence of intrapancreatic ductal obstruction. Pancreatic pseudocysts with associated major ductal disruption or a transected pancreas require operative therapy with cyst enterostomy. Definitive management with subtotal or total pancreatectomy is associated with considerable morbidity and mortality due to loss of both endocrine and exocrine functions of the pancreas. Generally, surgery is not indicated in children; however, it may be considered in cases of intractable pain and diffuse parenchymal damage without ductal dilation.
The use of total pancreatectomy with islet cell transplantation is undergoing evaluation for the treatment of chronic abdominal pain in children with chronic pancreatitis. Islet cell transplantation allows a patient to be treated for the pain of pancreatitis without the very serious adverse effects of a total pancreatectomy, including "brittle diabetes," which occurs when a person's blood glucose level often swings quickly from high to low and from low to high. [11]
Consultations
Treatment of pediatric patients with chronic pancreatitis requires the collaborative efforts of multispecialty teams that include gastroenterologists, surgeons, pharmacologists, nutritionists, child life specialists, psychologists, and psychiatrists.
Medical Care
The goal of medical management of acute pancreatitis is to achieve adequate rehydration, analgesia, and pancreatic rest and to restore normal metabolic homeostasis. In patients with severe pancreatitis, oral intake is restricted and parenteral nutrition is initiated within 3 days to prevent catabolism.
In cases of intractable vomiting or ileus, nasogastric suction is indicated to help intestinal-pancreatic rest by eliminating gastric secretions in the duodenum, the most potent activator of pancreatic secretion. Fluid electrolyte and mineral imbalances should be corrected urgently. Antibiotic therapy is indicated for systemic infections or sepsis.
Acute pancreatitis should resolve in 2-7 days with adequate resuscitation. In the setting of chronic relapsing pancreatitis, pancreatic enzyme supplementation, insulin, and elemental or low-fat diets are useful adjuncts to maximize nutritional status. [12]
For alleviation of pain, meperidine is preferred over morphine because of its decreased risk of ampullary spasm.
Preoperative studies with ultrasonography and computed tomography (CT) scanning, the preferred imaging modalities used to diagnosis and follow the course of pancreatitis and pancreatic pseudocysts, are important to assess the character and size of the pseudocyst. Endoscopic retrograde cholangiopancreatography (ERCP) is essential to assess various ductal abnormalities or pseudocyst communication with the pancreatic duct to determine definitive operative therapy.
Surgical Care
Surgical management of acute pancreatitis is rarely required. Surgical intervention is only needed if the symptoms are severe and prolonged or complicated by necrosis or abscess formation that requires debridement. Peritoneal lavage has been used in adults in an effort to reduce the incidence of secondary infection; however, this has not been through trials with children to test its efficacy. If underlying pancreaticobiliary disease is present, surgical intervention is required for cure.
Most surgical interventions are used in patients with chronic or relapsing pancreatitis. The goal of surgery in this instance is to alleviate pain and preserve the exocrine and endocrine functions of the pancreas. Surgical therapies include longitudinal pancreaticojejunostomy, distal pancreatectomy with Roux-en-Y pancreaticojejunostomy, decompression of pancreatic ducts, repair of pancreatic divisum, and sphincteroplasty.
However, operative management of chronic pancreatitis in children is controversial. Indications for operative intervention include unsuccessful conservative medical therapy, intractable pain, impaired nutrition, and narcotic addiction. Surgical options include distal pancreatectomy with Roux-en-Y pancreaticojejunostomy (ie, Duval procedure), lateral pancreaticojejunostomy (ie, Puestow procedure), or ERCP sphincteroplasty. A few pediatric patients with chronic pancreatitis and chronic abdominal pain were successfully treated with total pancreatectomy and islet cell transplantation. [11, 13, 14]
Surgical intervention is also indicated for the management of congenital anatomic defects (eg, pancreatic divisum) and other complications associated with acute pancreatitis (eg, pancreatic ascites, intra-abdominal abscess collections, pancreatic pseudocyst).
Pancreatic pseudocysts
Acute pancreatic pseudocysts smaller than 5 cm in diameter are managed with observation for 4-6 weeks because most resolve spontaneously. Pancreatic pseudocysts larger than 5 cm in diameter may require surgical intervention; however, conservative therapy is required for approximately 4-6 weeks to allow the cyst wall to mature. Results from a study by Ford et al indicate that pancreatic pseudocysts larger than 10 cm in diameter in children are associated with increased risk for spontaneous rupture and, thus, require aggressive monitoring. [15]
Chronic pancreatic pseudocysts (>3 mo) are best treated by surgical interventions. Ultrasonography-guided or CT-guided percutaneous drainage, [16] endoscopic drainage, and internal drainage via cyst gastrostomy or enterostomy have been used with success. Surgical approaches for internal drainage are largely determined by the anatomic location of the pseudocyst. If the pseudocyst is adherent to the posterior wall of the stomach, cystogastrostomy is performed. If the cyst is present in the head of the pancreas, cystoduodenostomy is considered. For other cysts not adherent to the stomach or duodenum, cystojejunostomy is preferred. Distal pancreatectomy is considered when the pseudocyst is in the tail of the gland.
Managing pancreatic pseudocysts with endoscopic treatment has been an increasing trend. It has been demonstrated that, in skilled hands, endoscopic treatment is safe and effective (for short-term and long-term treatment). Some authors suggest that endoscopic treatment should be the first interventional choice. The success rate is as high as 85%. Surgical treatment can be reserved for those cases that fail endoscopic treatment.
Pancreatic ductal disruption or compromise
Surgery for pancreatic ductal disruption or compromise (ie, acute traumatic pancreatitis with ductal injury) is indicated after medical failure. Endoscopic retrograde cholangiopancreatography (ERCP) or intraoperative pancreatic ductography is invaluable in identifying and determining the site of ductal disruption and directs surgical decision-making to the most appropriate operative procedure. [17, 18, 19]
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Pediatric Pancreatitis. This computed tomography (CT) scan of the abdomen in the region of the pancreas demonstrates a large well-marginated cystic structure that represents a pancreatic pseudocyst.
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Pediatric Pancreatitis. This real-time ultrasonogram of the abdomen, with attention to the right upper quadrant, reveals a loculated fluid collection in the hilum of the liver. This is compatible with a pancreatic pseudocyst. The differential diagnosis includes a large choledochal cyst.
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Pediatric Pancreatitis. Flow diagram for suspected acute pancreatitis . Etiologic factors and forms of acute pancreatitis and Ranson criteria are reviewed. ABG = arterial blood gas; Alk phos = alkaline phosphatase; ALT = alanine transaminase; AST = aspartate aminotransferase; BUN = blood urea nitrogen; CBC = complete blood cell count; Cr, creat = creatinine; ERCP = Endoscopic retrograde cholangiopancreatography; Gluc = glucose; lab = laboratory; LDH = lactate dehydrogenase; PO2 = partial pressure of oxygen; SBP = systolic blood pressure; and T bili = total bilirubin.
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Pediatric Pancreatitis. Flow diagram for mild pancreatitis. Favorable prognostic signs and medical management for acute pancreatitis, as well as studies used for acute pancreatitis. NPO = nothing by mouth; RUQ = right upper quadrant.
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Pediatric Pancreatitis. Prognostic indicators for severe pancreatitis and flow diagram for intensive care unit (ICU) management. BUN = blood urea nitrogen; CT = computed tomography; NG = nasogastric; and O2 = oxygen; paO2 = partial pressure of oxygen in arterial blood.
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Pediatric Pancreatitis. Flow diagram for diagnosis and treatment of necrotizing pancreatitis. CT = computed tomography; Tx = treatment.
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Pediatric Pancreatitis. Flow diagram for treatment of and studies used for pancreatic pseudocysts. ERCP = Endoscopic retrograde cholangiopancreatography
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Pediatric Pancreatitis. Flow diagram for management of idiopathic recurrent pancreatitis. Etiologies for acute pancreatitis. CBD = common bile duct; ERCP = Endoscopic retrograde cholangiopancreatography
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Pediatric Pancreatitis. Flow diagram for management of pancreatic abscess. Definition of an abscess.
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Pediatric Pancreatitis. A patient with acute gallstone pancreatitis underwent endoscopic retrograde cholangiopancreatography (ERCP). The cholangiogram showed no stones in the common bile duct and multiple small stones in the gallbladder. In this image, the pancreatogram shows narrowing of the pancreatic duct in the area of the genu, the result of extrinsic compression of the ductal system by inflammatory changes in the pancreas.
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Pediatric Pancreatitis. Abdominal computed tomography (CT) scan from the same patient as in the previous image showing pancreatic enlargement and peripancreatic fat stranding. The gallstones are not visible.
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Pediatric Pancreatitis. Pancreas divisum associated with minor papilla stenosis causing recurrent pancreatitis. Because pancreas divisum is relatively common in the general population, it is best regarded as a variant of normal anatomy and not necessarily as a cause of pancreatitis. In this case, note the bulbous contour of the duct adjacent to the cannula. This appearance has been termed a santorinicele. A dorsal duct outflow obstruction is the probable cause of pancreatitis when a santorinicele is present and associated with a minor papilla that accommodates only a guidewire.
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Pediatric Pancreatitis. Normal-appearing ventral pancreas in a patient with recurrent acute pancreatitis. The dorsal pancreas (not pictured) showed evidence of chronic pancreatitis.
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Pediatric Pancreatitis. Computed tomography (CT) scan of the abdomen in a child with traumatic pancreatitis. The fluid collection adjacent to the pancreas will become a pseudocyst. Note that the pancreas is lacerated, nearly cut in half, by the force of the abdominal trauma. Also, note the typical location of this injury in relation to the vertebral column.
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Pediatric Pancreatitis. Computed tomography (CT) scan of a young man who was referred after 2 weeks into his second bout of severe acute pancreatitis. Gravely ill, he had fever and leukocytosis as well as hypotension requiring pressors and respiratory distress requiring mechanical ventilation. His abdominal CT scan shows severe acute pancreatitis. A percutaneous drain was placed in the dominant fluid collection to establish drainage while he was given imipenem/cilastatin, which stabilized his condition.
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Pediatric Pancreatitis. Endoscopic retrograde cholangiopancreatography (ERCP) in the same patient as in the previous image excluded suppurative cholangitis and established the presence of annular pancreas divisum. The dorsal pancreatogram showed extravasation into the retroperitoneum, and a sphincterotomy was performed on the minor papilla. As shown in this radiographic film, a pigtailed nasopancreatic tube was then inserted into the dorsal duct and out into the retroperitoneal fluid collection. The other end of the tube was attached to the bulb suction and monitored every shift.
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Pediatric Pancreatitis. While percutaneous drains removed loculated fluid collections elsewhere in the abdomen in the same patient as in the previous image, the nasopancreatic tube contained the retroperitoneal fluid collection. One week later, the retroperitoneal fluid collection was much smaller, as shown in this radiographic film (the image is reversed in the horizontal direction). By this time, the patient was off pressors and was ready to be extubated.
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Pediatric Pancreatitis. In the same patient as in the previous image, 4 months later, after the pseudocyst was converted into a pseudocystogastrostomy using minimally invasive techniques, the pancreatogram revealed the more proximal pancreatic duct.
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Pediatric Pancreatitis. In the same patient as in the previous image, a guidewire was placed into the dorsal duct, crossed the stenotic area, and advanced into the proximal duct. A dilating catheter was then advanced over the wire to enlarge the stenosis. The duct was subsequently stented.
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Pediatric Pancreatitis. Six months after severe acute pancreatitis, the same patient as in the previous image remained symptom free and was living independently. As shown in this follow-up abdominal computed tomography (CT) scan, minimally invasive techniques were successful in removing the pockets of infection, restoring the integrity of the pancreatic ductal system. These techniques also preserved the endocrine function of the pancreas, and at the time of this follow-up, the patient had no evidence of diabetes mellitus.
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Pediatric Pancreatitis. Familial adenomatous polyposis syndrome in a patient with persistent pancreatitis due to a partially obstructing ampullary adenoma. The pancreatogram shown here reveals a very prominent ductal system. Because the patient had undergone several previous abdominal operations, she opted to have an endoscopic ampullectomy.
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Pediatric Pancreatitis. In this radiograph from the same patient as in the previous image, it can be seen that stents were placed into the biliary and pancreatic ductal systems following ampullectomy. The smoldering pancreatitis resolved within a week, the stents were subsequently removed, and the patient participated in an endoscopic surveillance program, with no recurrence at the time of this article's publication.
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Pediatric Pancreatitis. Recurrent pancreatitis associated with pancreas divisum in an elderly man. This pancreatogram of the dorsal duct shows a distal stenosis with upstream chronic pancreatitis. After the stenosis was dilated and stented, the patient's pain resolved, and he improved clinically during 1 year of stent exchanges on a quarterly basis. Follow-up computed tomography (CT) scans showed resolution of an inflammatory mass. Although ductal biopsies and cytology were repeatedly negative, pain and pancreatitis returned when the stents were removed. The patient developed duodenal outflow obstruction and was sent to surgery; a Whipple procedure revealed a periampullary adenocarcinoma (of the minor papilla).
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Pediatric Pancreatitis. Computed tomography (CT) scan of a patient initially seen for recurrent abdominal pain. An esophagogastroduodenoscopy (EGD) showed a submucosal nodule in the antrum, which prompted a referral to another center, with a request for endoscopic ultrasonography and polypectomy. Because the endoscopic ultrasonogram was indeterminate, a polypectomy was attempted. That evening, the patient developed progressively severe epigastric abdominal pain radiating to the back and presented to an emergency department. She had a leukocytosis and a mild elevation of her lipase and was admitted with a diagnosis of pancreatitis. This CT scan of her abdomen shows circumferential hypodense thickening of her antrum, with a normal-appearing pancreas. A small portion of pancreatic tissue was later identified as pancreatic rectitis in the pathology specimen.
