THE DISEASES OF THE

PANCREAS

ACUTE AND CHRONIC PANCREATITIS

THE DISEASES OF THE PANCREAS

THE DISEASES OF THE PANCREAS INCLUDE THE FOLLOWING:

1 Pancreatitis

2 Diabetes mellitus (endocrine pancreatic insufficiency)

3 Exocrine pancreatic insufficiency

4 Cystic fibrosis

5 Pseudocysts

6 Cysts

7 Congenital malformations

7.1 Pancreas divisum

7.2 Annular pancreas

8 Neoplasms

8.1 Benign

8.2 Tumor predisposition

8.2.1Zollinger-Ellison syndrome

9 Hemosuccus pancreaticus
Acute pancreatitis
Acute pancreatitis is an inflammatory condition of the pancreas that is painful and at times deadly.
Despite the great advances in critical care medicine over the past 20 years, the mortality rate of acute
pancreatitis has remained at about 10%. Diagnosis of pancreatic problems is often difficult and
treatments are therefore delayed because the organ is relatively inaccessible. There are no easy ways to
see the pancreas directly without surgery, and available imaging studies are often inadequate. In
addition to the acute form, there are hereditary and chronic forms of pancreatitis which can devastate a
person over many years. Sufferers often endure pain and malnutrition, and are most likely left with a
higher risk of pancreatic cancer.

Once a working diagnosis of acute pancreatitis is reached, laboratory tests are obtained to support the
clinical impression, to help define the etiology, and to look for complications. Diagnostic imaging is
unnecessary in most cases but may be obtained when the diagnosis is in doubt, when severe
pancreatitis is present, or when an imaging study might provide specific information needed to answer a
clinical question. Image-guided aspiration may be useful. Genetic testing may be considered.

Management depends largely on severity. Medical treatment of mild acute pancreatitis is relatively
straightforward. Treatment of severe acute pancreatitis involves intensive care; the goals of medical
management are to provide aggressive supportive care, to decrease inflammation, to limit infection or
superinfection, and to identify and treat complications as appropriate. Surgical intervention (open or
minimally invasive) is indicated in selected case

Pathogenesis of acute pancreatitis

Acute pancreatitis may occur when factors involved in maintaining cellular
homeostasis are out of balance. The initiating event may be anything that injures
the acinar cell and impairs the secretion of zymogen granules; examples include
alcohol use, gallstones, and certain drugs.
At present, it is unclear exactly what pathophysiologic event triggers the onset of
acute pancreatitis. It is believed, however, that both extracellular factors (eg, neural
and vascular response) and intracellular factors (eg, intracellular digestive enzyme
activation, increased calcium signaling, and heat shock protein activation) play a
role. In addition, acute pancreatitis can develop when ductal cell injury leads to
delayed or absent enzymatic secretion, as seen in patients with the CFTR gene
mutation.
Once a cellular injury pattern has been initiated, cellular membrane trafficking
becomes chaotic, with the following deleterious effects:
• Lysosomal and zymogen granule compartments fuse, enabling activation of
trypsinogen to trypsin
• Intracellular trypsin triggers the entire zymogen activation cascade
• Secretory vesicles are extruded across the basolateral membrane into the
interstitium, where molecular fragments act as chemoattractants for inflammatory
cells
Activated neutrophils then exacerbate the problem by releasing superoxide (the
respiratory burst) or proteolytic enzymes (cathepsins B, D, and G; collagenase; and
elastase). Finally, macrophages release cytokines that further mediate local (and, in
severe cases, systemic) inflammatory responses. The early mediators defined to
date are tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, and IL-8.
These mediators of inflammation cause an increased pancreatic vascular
permeability, leading to hemorrhage, edema, and eventually pancreatic necrosis.
As the mediators are excreted into the circulation, systemic complications can
arise, such as bacteremia due to gut flora translocation, acute respiratory distress
syndrome (ARDS), pleural effusions, gastrointestinal (GI) hemorrhage, and renal
failure.
The systemic inflammatory response syndrome (SIRS) can also develop, leading to
the development of systemic shock. Eventually, the mediators of inflammation can
become so overwhelming that hemodynamic instability and death ensue.
In acute pancreatitis, parenchymal edema and peripancreatic fat necrosis occur
first; this is known as acute edematous pancreatitis. When necrosis involves the
parenchyma, accompanied by hemorrhage and dysfunction of the gland, the
inflammation evolves into hemorrhagic or necrotizing pancreatitis. Pseudocysts
and pancreatic abscesses can result from necrotizing pancreatitis because enzymes
can be walled off by granulation tissue (pseudocyst formation) or via bacterial
seeding of pancreatic or peripancreatic tissue (pancreatic abscess formation

ETIOLOGY
1) GALL STONES AND BILIARY TRACT DISEASES: One of the most common
causes of acute pancreatitis is gallstones (~40%). Gallstones that escape from the gallbladder can
block the pancreatic duct. (The pancreatic duct delivers digestive enzymes from the pancreas to
the small intestine.) When the pancreatic duct becomes blocked, enzymes can't flow properly.
They can back up into the pancreas. This causes the pancreas to become inflamed.

2) ALCOHOL USE: The other leading cause of pancreatitis is heavy alcohol use
(~35%). Most people who drink alcohol never develop pancreatitis. But certain
people will develop pancreatitis after drinking large amounts of alcohol. Alcohol
use may be over a period of time or in a single binge. Alcohol combined with
smoking increases the risk of acute pancreatitis.
3) ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY
(ERCP): Another common cause of acute pancreatitis is a complication of a
medical procedure called ERCP (~1-5%). ERCP is performed through an
endoscope. This is a flexible tube with a small camera and a light on one end and
an eyepiece on the other. ERCP is used to identify stones and tumors and to view
ducts in the pancreas, liver and gallbladder.

Other factors that sometimes can cause pancreatitis include:

• Use of any of a wide variety of medications, such as

o Sulfa drugs

o Water pills (hydrochlorothiazide, others)

o Immunosuppressants (azathioprine)

o Drugs used to treat HIV

• Abdominal surgery
• Severe trauma

• Metabolic conditions, such as high blood levels of calcium or triglycerides

• Some infections, such as mumps or viral hepatitis

In many cases, no cause can be found (idiopathic/cryptogenic acute

pancreatitis).

SIGNS AND SYMPTOMS:

Symptoms of acute pancreatitis include the following:

• Abdominal pain (cardinal symptom): Characteristically dull, boring, and
steady; usually sudden in onset and gradually becoming more severe until reaching
a constant ache; most often located in the upper abdomen and may radiate directly
through to the back
• Nausea and vomiting, sometimes with anorexia
• Diarrhea
Patients may have a history of the following:
• Recent operative or other invasive procedures
• Family history of hypertriglyceridemia
• Previous biliary colic and binge alcohol consumption (major causes of acute
pancreatitis)
The following physical findings may be noted, varying with the severity of the
disease:
• Fever (76%) and tachycardia (65%); hypotension
• Abdominal tenderness, muscular guarding (68%), and distention (65%);
diminished or absent bowel sounds
• Jaundice (28%)
• Dyspnea (10%); tachypnea; basilar rales, especially in the left lung
• In severe cases, hemodynamic instability (10%) and hematemesis or melena
(5%); pale, diaphoretic, and listless appearance
• Occasionally, extremity muscular spasms secondary to hypocalcemia
The following uncommon physical findings are associated with severe
necrotizing pancreatitis:
• Cullen sign (bluish discoloration around the umbilicus resulting from
hemoperitoneum)
• Grey-Turner sign (reddish-brown discoloration along the flanks resulting from
retroperitoneal blood dissecting along tissue planes); more commonly, patients
may have a ruddy erythema in the flanks secondary to extravasated pancreatic
exudate
• Erythematous skin nodules, usually no larger than 1 cm and typically located
on extensor skin surfaces; polyarthritis
DIAGNOSIS
Once a working diagnosis of acute pancreatitis is reached, laboratory tests are
obtained to support the clinical impression, such as the following:
• Serum amylase and lipase
• Liver-associated enzymes
• Blood urea nitrogen (BUN), creatinine, and electrolytes
• Blood glucose
• Serum cholesterol and triglyceride
• Complete blood count (CBC) and hematocrit; NLR
• C-reactive protein (CRP)
• Arterial blood gas values
• Serum lactic dehydrogenase (LDH) and bicarbonate
• Immunoglobulin G4 (IgG4)
Diagnostic imaging is unnecessary in most cases but may be obtained when the
diagnosis is in doubt, when pancreatitis is severe, or when a given study might
provide specific information required. Modalities employed include the following:
• Abdominal radiography (limited role): Kidneys-ureters-bladder (KUB)
radiography with the patient upright is primarily performed to detect free air in the
abdomen
• Abdominal ultrasonography (most useful initial test in determining the
etiology, and is the technique of choice for detecting gallstones)
• Endoscopic ultrasonography (EUS) (used mainly for detection of microlithiasis
and periampullary lesions not easily revealed by other methods)
• Abdominal computed tomography (CT) scanning (generally not indicated for
patients with mild pancreatitis but always indicated for those with severe acute
pancreatitis)
• Endoscopic retrograde cholangiopancreatography (ERCP); to be used with
extreme caution in this disease and never as a first-line diagnostic tool [1]
• Magnetic resonance cholangiopancreatography (MRCP) (not as sensitive as
ERCP but safer and noninvasive)

Other diagnostic modalities include the following:

• CT-guided or EUS-guided aspiration and drainage
• Genetic testing
Acute pancreatitis is broadly classified as either mild or severe. According to the
Atlanta classification, severe acute pancreatitis is signaled by the following :
• Evidence of organ failure (eg, systolic blood pressure below 90 mm Hg, arterial
partial pressure of oxygen [Pa O2] 60 mm Hg or lower, serum creatinine level 2
mg/dL or higher, GI bleeding amounting to 500 mL or more in 24 hours)
• Local complications (eg, necrosis, abscess, pseudocyst)
• Ranson score of 3 or higher or APACHE score of 8 or higher

Multivariate scores
APACHE II score
Not displayed in detail
Ranson score
Three or more points indicate increased mortality
At admission
Age >55 years
White cell count >16 GPT/L
Blood glucose >200 mg/dL
LDH >350 mg/dL
GOT/AST >250 U/L
After 48 h
Reduction in hematocrit >10%
Increase in BUN >5 mg/dL
Serum calcium <8 mg/dL
pO2 <60 mm Hg
SBE >4 mEq/L
Estimated fluid requirement >6,000 mL
Glasgow Criteria (Glasgow-Imrie score)
Three or more points indicate increased mortality, estimation 48 h from
admission
Age >55 years
White cell count >15 GPT/L
pO2 <60 mm Hg
Blood glucose >180 mg/dL (>10 mmol/L)
BUN under fluid resuscitation >96 mg/dL (>6 mmol/L)
Serum calcium <8 mg/dL (<2mmol/L)
Albumin <32 g/L
LDH >600 mg/dL
Transaminases >100 U/L

BISAP
Score >0 at admission indicates increased risk of mortality
BUN >25 mg/dL (>8.92 mmol/L)
Impaired mental status
>2 SIRS criteria
Age >60 years
Presence of pleural effusions
Single parameters
Parameter
Interpretation
Age
Age 55 years or older is associated with higher mortality
Body mass index
A body mass index >25 is associated with higher mortality
Hematocrit
Normal HCT (35–44%) on admission and after 48 h is associated with low rate of
complications and death
CRP
Levels >150 mg/L within 72 h from admission indicate risk of sever AP
BUN
Elevated BUN at admission or increase within 48 h is associated with mortality
 TREATMENT/MANAGEMENT:
Medical management of mild acute pancreatitis is relatively straightforward;
however, patients with severe acute pancreatitis require intensive care.
Initial supportive care includes the following:
• Fluid resuscitation : Fluid resuscitation is a crucial element of the early
conservative treatment of AP. However, the evidence basis for fluid therapy in AP
is relatively weak. Patients with mild AP usually have lower levels of fluid
sequestration and thus lower fluid resuscitation requirements than patients with
moderate or severe AP. This is mainly due to the higher occurrence of serious
complications of severe AP such as formation of fluid collections, necrosis, or
multiorgan failure. Vice versa it has been demonstrated in retrospective studies that
patients receiving inadequate fluid replacement therapy are at higher risk of
developing complications such as pancreatic necrosis.There is no universal
consensus definitively favoring one type of fluid over another type; both
crystalloids and colloids are used. Resuscitation should be sufficient to maintain
hemodynamic stability. This usually involves administration of several liters of
fluid as a bolus, followed by continuous infusion at a rate of 250-500 mL/h.
Central venous pressure, pulmonary artery wedge pressure, and urine output (>0.5
mL/kg/h) can be followed up as markers of adequate hydration. Careful attention
should be paid to signs of overhydration, such as pulmonary edema causing
hypoxia.
• Nutritional support: Oral feeding plays a pivotal role in the early conservative
treatment of AP. Data coming from randomized controlled trials demonstrated that
early feeding significantly reduces the risk of necrosis in the course of AP and
diminishes the rates of infected peripancreatic necrosis
and multiple organ failure. Most probably, early enteral nutrition protects the
mucosal intestinal barrier and reduces the risk of bacterial translocation. Early
feeding was demonstrated to be successful using various types of diet (i.e., low-fat,
normal-fat, soft, and solid). Some patients are intolerant towards early oral feeding
(mainly due to pain and vomiting) and may require placement of an enteral tube
for nutritional support. However, in patients at risk of severe AP, a randomized
controlled trial did not show a clinical benefit of tube feeding within
24 h versus on-demand tube feeding if oral diet was not tolerated after 72 h. There
is strong evidence showing that enteral nutrition is beneficial in comparison to total
parenteral nutrition. Enteral nutrition reduces the rate of
infected peripancreatic necrosis as well as single and multiple organ failure
 Antibiotic therapy is employed as follows:
• Antibiotics (usually of the imipenem class) should be used in any case of
pancreatitis complicated by infected pancreatic necrosis but should not be given
routinely for fever, especially early in the presentation
• Antibiotic prophylaxis in severe pancreatitis is controversial; routine use of
antibiotics as prophylaxis against infection in severe acute pancreatitis is not
currently recommended

Pain Management

Severe pain occurs frequently in AP. Since several studies proved that opioid
analgesics (particularly morphine) do not cause dysfunction of the sphincter of
Oddi, the WHO analgesia ladder is a pragmatic approach to the treatment of pain
in AP. It is based on stepwise escalation of treatment from low-potent to highly
potent nonsteroidal anti-inflammatory drugs alone or in combination with opioids.
There is lack of agreement on which analgesics should be preferred in the
treatment of patients with AP. In a recent study, Gülen et al. compared the
analgesic effectiveness of tramadol (a synthetic opioid), paracetamol, and
dexketoprofen in AP, showing no significant differences between the treated
groups. There were some suggestions about pethidine as a potent opioid analgesic
of choice in severe pain related to AP. Blamey et al. proved that buprenorphine has
a similar, strong analgesic activity, but a lower potential to cause opioid
dependency. Intestinal paralysis and ileus are a common problem in early AP,
which can get aggravated when using high doses of opioids. In some patients, use
of an epidural catheter for pain relief might be considered.

Surgical Interventions

Surgical intervention, whether by minimally invasive or conventional open

techniques, is indicated when an anatomic complication amenable to a mechanical
solution is present (eg, acute necrotizing pancreatitis in which the necrotic
phlegmon is excised to limit a potential site of sepsis, or hemorrhagic pancreatitis
in which surgical control of bleeding is warranted). Depending on the situation and
local expertise, this may require the talents of an interventional radiologist, an
interventional endoscopist, or surgeon (individually or in combination).
Soon after a person is admitted to the hospital with suspected narrowing of the
pancreatic duct or bile ducts, a physician with specialized training performs ERCP.

After lightly sedating the patient and giving medication to numb the throat, the
doctor inserts an endoscope-a long, flexible, lighted tube with a camera-through
the mouth, throat, and stomach into the small intestine. The endoscope is
connected to a computer and screen. The doctor guides the endoscope and injects a
special dye into the pancreatic or bile ducts that helps the pancreas, gallbladder,
and bile ducts appear on the screen while x rays are taken.

The following procedures can be performed using ERCP:

Sphincterotomy. Using a small wire on the endoscope, the doctor finds the muscle
that surrounds the pancreatic duct or bile ducts and makes a tiny cut to enlarge the
duct opening. When a pseudocyst is present, the duct is drained.

Gallstone removal. The endoscope is used to remove pancreatic or bile duct

stones with a tiny basket. Gallstone removal is sometimes performed along with a
sphincterotomy.

Stent placement. Using the endoscope, the doctor places a tiny piece of plastic or
metal that looks like a straw in a narrowed pancreatic or bile duct to keep it open.

Balloon dilatation. Some endoscopes have a small balloon that the doctor uses to
dilate, or stretch, a narrowed pancreatic or bile duct. A temporary stent may be
placed for a few months to keep the duct open.

People who undergo therapeutic ERCP are at slight risk for complications,
including severe pancreatitis, infection, bowel perforation, or bleeding.
Complications of ERCP are more common in people with acute or recurrent
pancreatitis. A patient who experiences fever, trouble swallowing, or increased
throat, chest, or abdominal pain after the procedure should notify a doctor
immediately.

Debridement and Drainage

If acute pancreatitis has caused severe complications, such as an infection that
doesn’t respond to antibiotics, surgeons may perform a debridement and drainage
procedure to remove infected pancreatic tissue or necrosis. This procedure also
allows doctors to drain any fluid from the pancreas that has accumulated as a result
of an infection. They may create a new drainage pathway in the pancreas to restore
normal function

Pancreatic Cyst Gastronomy

Pancreatic cyst gastronomy is a drainage procedure that an advanced endoscopist

or surgeon may use if a pancreatic pseudocyst—a fluid-filled sac—develops in the
abdomen and causes symptoms such as pain, the sensation of a full stomach, or
vomiting. This may occur as a complication of acute pancreatitis if inflammation
and swelling cause the ducts to become damaged.

Complications
Pancreatitis can lead to potentially fatal complications.

These include:

• obstruction of a bile or pancreatic duct

• leakage from the pancreatic duct

• pseudocysts, with a risk of rupture, hemorrhage, or infection

• damage to the pancreas

• pleural effusion and acute respiratory distress syndrome

• splenic vein thrombosis

Heart, lung, and kidney failure may occur. In severe cases, organ failure can
happen around 48 hours after symptoms appear. Without treatment, these can lead
to death.

CHRONIC PANCREATITIS
Chronic pancreatitis is a syndrome involving inflammation, fibrosis, and loss of
acinar and islet cells which can manifest in unrelenting abdominal pain,
malnutrition, and exocrine and endocrine insufficiency. The Toxic-Metabolic,
Idiopathic, Genetic, Autoimmune, Recurrent and Severe Acute Pancreatitis,
Obstructive (TIGAR-O) classification system categorizes known causes and
factors that contribute to chronic pancreatitis. Although determining disease
etiology provides a framework for focused and specific treatments, chronic
pancreatitis remains a challenging condition to treat owing to the often refractory,
centrally mediated pain and the lack of consensus regarding when endoscopic
therapy and surgery are indicated. Further complications incurred include both
exocrine and endocrine pancreatic insufficiency, pseudocyst formation, bile duct
obstruction, and pancreatic cancer. Medical treatment of chronic pancreatitis
involves controlling pain, addressing malnutrition via the treatment of vitamin and
mineral deficiencies and recognizing the risk of osteoporosis, and administering
appropriate pancreatic enzyme supplementation and diabetic agents. Cornerstones
in treatment include the recognition of pancreatic exocrine insufficiency and
administration of pancreatic enzyme replacement therapy, support to cease
smoking and alcohol consumption, consultation with a dietitian, and a systematic
follow-up to assure optimal treatment effect.

Etiology

Because of its varied presentation and clinical similarity to acute pancreatitis,

many cases of chronic pancreatitis are not diagnosed; therefore, the true prevalence
is unknown. A recent Japanese study estimated a prevalence of 12 cases per
100,000 women and 45 cases per 100,000 men. The average age at diagnosis is 35
to 55 years. Chronic alcohol use accounts for 70 percent of the cases of chronic
pancreatitis in adults, and most patients have consumed more than 150 g of alcohol
per day over six to 12 years. Genetic diseases (e.g., cystic fibrosis) and anatomic
defects predominate in children. The TIGAR-O (Toxic-metabolic, Idiopathic,
Genetic, Autoimmune, Recurrent and severe acute pancreatitis, Obstructive)
classification system is based on risk factors for chronic pancreatitis.

Pathophysiology
Most studies of the pathophysiology of chronic pancreatitis are performed with
patients who drink alcohol. Disease characteristics include inflammation, glandular
atrophy, ductal changes, and fibrosis. It is presumed that when a person at risk is
exposed to toxins and oxidative stress, acute pancreatitis occurs. If the exposure
continues, early- and late-phase inflammatory responses result in production of
profibrotic cells, including the stellate cells. This can lead to collagen deposition,
periacinar fibrosis, and chronic pancreatitis. In addition, several genetic mutations
have been associated with idiopathic chronic pancreatitis.2,5,9 Autoimmune
pancreatitis accounts for 5 to 6 percent of chronic pancreatitis and is characterized
by autoimmune

The proposed pathologic mechanisms of chronic pancreatitis are as follows:

• Intraductal plugging and obstruction - Eg, ethanol (ETOH) abuse, stones, tumors
• Direct toxins and toxic metabolites - These act on the pancreatic acinar cells to
stimulate the release of cytokines, which stimulate the stellate cells to produce
collagen and to lay down fibrous tissue; cytokines also act to stimulate
inflammation by neutrophils, macrophages, and lymphocytes (eg, ETOH, tropical
sprue)
• Oxidative stress - Eg, idiopathic pancreatitis
• Necrosis-fibrosis - Recurrent acute pancreatitis that heals with fibrosis
• Ischemia - From obstruction and fibrosis; important in exacerbating or
perpetuating disease rather than in initiating disease
• Autoimmune disorders - Chronic pancreatitis has been found in association with
other autoimmune diseases, such as Sjögren syndrome, primary biliary cirrhosis,
and renal tubular acidosis.
• Secondary forms of autoimmune chronic pancreatitis are associated with primary
biliary cirrhosis, primary sclerosing cholangitis, and Sjögren syndrome.
• Although alcohol greatly influences the understanding of its pathophysiology
because it is the most common etiology (60-70%), approximately 20-30% of cases
are idiopathic and 10% of cases are due to rare diseases.

Epidemiology

Based on the estimates from hospital discharge data in the United States,
approximately 87,000 cases of pancreatitis occur annually.
Comparing the hospital admissions data from several cities around the globe, the
overall frequency is similar. Expressed as number of cases per 1000 hospital
admissions, the value for Marseille is 3.1; for Cape Town, 4.4; for Sao Paulo, 4.9;
and for Mexico City, 4.4. When the data from several centers were compared over
time, the incidence of chronic pancreatitis from 1945-1985 appeared to be
increasing.
Race-, sex-, and age-related demographics

Hospitalization rates for blacks are 3 times higher than for whites in the United
States. In population studies, males are affected more commonly than females (6.7
vs 3.2 per 100,000 population).
Differences in the hospitalization rates of patients with chronic pancreatitis exist
with respect to sex. Rates in males peak between ages 45 and 54 years and then
decline; female rates reach a plateau, which remains stable after age 35 years.
Sex differences with respect to etiology also exist. Alcohol-induced illness is more
prevalent in males, idiopathic and hyperlipidemic-induced pancreatitis is more
prevalent in females, and equal sex ratios are observed in chronic pancreatitis
associated with hereditary pancreatitis.
In aggregate, the mean age at diagnosis is 46 years, plus or minus 13 years.

Clinical Presentation
History

For most patients with chronic pancreatitis, abdominal pain is the presenting
symptom. Either the patient's age or the etiology of the disease has some influence
on the frequency of this presentation. Ninety-six percent of those with early onset
idiopathic pancreatitis present with abdominal pain, compared with 77% with
alcohol-induced disease and 54% with late-onset idiopathic chronic pancreatitis.
Clinically, the patient experiences intermittent attacks of severe pain, often in
the midabdomen or left upper abdomen and occasionally radiating in a
bandlike fashion or localized to the midback. The pain may occur either after
meals or independently of meals, but it is not fleeting or transient and tends to
last at least several hours. Unfortunately, patients often are symptomatic for years
before the diagnosis is established; the average time from the onset of symptoms
until a diagnosis of chronic pancreatitis is 62 months. The delay in diagnosis is
even longer in people without alcoholism, in whom the average time is 81 months
from the onset of symptoms to diagnosis.
The natural history of pain in chronic pancreatitis is highly variable. Most patients
experience intermittent attacks of pain at unpredictable intervals, while a minority
of patients experience chronic pain. In most patients, pain severity either decreases
or resolves over 5-25 years. Nevertheless, ignoring pain relief with the expectation
that the disease eventually will resolve itself is inappropriate. In alcohol-induced
disease, eventual cessation of alcohol intake may reduce the severity of pain.
Variability in the pain pattern contributes to the delay in the diagnosis and makes
determining the effect of any therapeutic intervention difficult.
Other symptoms associated with chronic pancreatitis include diarrhea and
weight loss. This may be due either to fear of eating (eg, postprandial
exacerbation of pain) or due to pancreatic exocrine insufficiency and
steatorrhea.
Physical Examination

In most instances, the standard physical examination does not help to establish a
diagnosis of chronic pancreatitis; however, a few points are noteworthy.
During an attack, patients may assume a characteristic position in an attempt
to relieve their abdominal pain (eg, lying on the left side, flexing the spine and
drawing the knees up toward the chest).
Occasionally, a tender fullness or mass may be palpated in the epigastrium,
suggesting the presence of a pseudocyst or an inflammatory mass in the
abdomen. Patients with advanced disease (ie, patients with steatorrhea) exhibit
decreased subcutaneous fat, temporal wasting, sunken supraclavicular fossa,
and other physical signs of malnutrition. Patients may also present with
steatorrhea, malabsorption, vitamin deficiency (A, D, E, K, B12), diabetes, or
weight loss. Approximately 10 to 20 percent
of patients may have exocrine insufficiency without abdominal pain

Differential diagnosis
More common
Acute cholecystitis
Acute pancreatitis
Intestinal ischemia or infarction
Obstruction of common bile duct
Pancreatic tumors
Peptic ulcer disease
Renal insufficiency
Less common
Acute appendicitis
Acute salpingitis
Crohn’s disease
Ectopic pregnancy
Gastroparesis
Intestinal obstruction
Irritable bowel syndrome
Malabsorption
Ovarian cysts
Papillary cystadenocarcinoma of ovary
Thoracic radiculopathy

Diagnosis
Blood tests

Serum amylase and lipase levels may be slightly elevated in chronic

pancreatitis; high levels are found only during acute attacks of pancreatitis. In
the later stages of chronic pancreatitis, atrophy of the pancreatic parenchyma
can result in normal serum enzyme levels because of significant fibrosis of the
pancreas, resulting in decreased concentrations of these enzymes within the
pancreas.
Although low concentrations of serum trypsin are relatively specific for advanced
chronic pancreatitis, they are not sensitive enough to be helpful in most patients
with mild to moderate disease.
Fecal tests

Because maldigestion and malabsorption do not occur until more than 90% of
the pancreas has been destroyed, steatorrhea is a manifestation of advanced
chronic pancreatitis. Neither qualitative nor quantitative fecal fat analysis can
detect early disease.
Assays of fecal chymotrypsin and human pancreatic elastase 1 have the same
limitations but are useful in confirming advanced chronic pancreatitis with
exocrine insufficiency.
Pancreatic Function Tests

Direct tests
These tests are the most sensitive and can be used to detect chronic pancreatitis at
its earliest stage; however, they are somewhat invasive, labor intensive, and
expensive.
Determination in duodenal aspirates
Intubation of the duodenum usually is performed with a Dreiling tube, which
allows for separate aspiration of the gastric and duodenal contents. The
methodology varies depending on the specific laboratory; however, exogenous
secretin with cholecystokinin (CCK) is used to achieve maximal stimulation of
the pancreas. The output of pancreatic bicarbonate, protease, amylase, and
lipase then is measured in the duodenal aspirates.
This test currently is available only in specialized centers. While the greatest
sensitivity can be obtained in prolonged infusions of a secretagogue to uncover a
decreased pancreatic secretory reserve, it is impractical for general clinical use.
Determination in pancreatic juice
This test generally is performed in conjunction with ERCP. The pancreatic duct is
freely cannulated, an exogenous secretagogue is administered as above, and the
pancreatic juice then is aspirated out of the duct as it is produced. The output of
pancreatic bicarbonate, protease, amylase, and lipase are measured.
Indirect tests

Noninvasive tests of pancreatic function have been developed for detecting chronic
pancreatitis. In principle, these tests work via oral administration of a complex
substance that is hydrolyzed by a specific pancreatic enzyme to release a
marker substance. The intestine absorbs the marker, which then is measured
in the serum or urine. These tests are capable of detecting moderate to severe
chronic pancreatitis. The presence of renal, intestinal, and liver disease may
interfere with the accuracy of these tests.

2018 Working Group for the International Consensus Guidelines for Chronic
Pancreatitis recommendations
The clinical practice guidelines for the diagnostic cross-sectional imaging and
severity scoring of chronic pancreatitis were released in October 2018 by the
Working Group for the International Consensus Guidelines for Chronic
Pancreatitis.
Computed tomography (CT) is often the most appropriate initial imaging
modality to evaluate suspected chronic pancreatitis (CP); it depicts most of the
changes in pancreatic morphology.

CT is also indicated to exclude other potential intra-abdominal pathologies

that present with symptoms similar to those of chronic pancreatitis, but CT
cannot exclude a diagnosis of CP and cannot exclusively diagnose early or mild
CP.

Magnetic resonance imaging (MRI) and MR cholangiopancreatography

(MRCP) are superior and are indicated especially in patients in whom no
specific pathologic changes are seen on CT.

Secretin-stimulated MRCP is more accurate than standard MRCP to identify

subtle ductal changes. Secretin-stimulated MRCP should be performed after a
negative MRCP if there is still clinical suspicion of CP.
Secretin-stimulated MRCP can provide assessment of exocrine function and ductal
compliance.
Endoscopic ultrasound (EUS) can also be used to diagnose parenchymal and
ductal changes mainly during the early stage of CP.
There are no known validated radiologic severity scoring systems for CP, but a
modified Cambridge Classification has been used for MRCP.
A new and validated radiologic CP severity scoring system is needed that is
based on imaging criteria (CT and/or MRI), including glandular volume loss,
ductal changes, parenchymal calcifications, and parenchymal fibrosis.
Imaging:

Abdominal radiography

Pancreatic calcifications, often considered pathognomonic of chronic pancreatitis,

are observed in approximately 30% of cases. Paired anteroposterior (AP) and
oblique views are preferred because the vertebral column otherwise could obscure
small flecks of calcium. The calcifications form within the ductal system—initially
in the head, and later in the body and tail, of the gland. Calcium deposition is most
common with alcoholic pancreatitis, hereditary pancreatitis, and tropical
pancreatitis; however, it is rare in idiopathic pancreatitis.
CT scanning

The advantage of CT scanning is that interpretation of pancreatic CT images is

relatively intuitive. However, although CT scanning excels at depicting the
morphologic changes of advanced chronic pancreatitis described above, the subtle
abnormalities of early to moderate chronic pancreatitis are beyond its resolution,
and a normal finding on this study does not rule out chronic pancreatitis.
CT scan studies are indicated to look for complications of the disease and are
useful in planning surgical or endoscopic intervention. The sensitivity and
specificity of CT scanning are 80% and 85%, respectively.

Chronic pancreatitis. CT scans of the abdomen following an endoscopic

transgastric pseudocystogastrostomy. Note that 2 stents are placed through
the stomach and into the pseudocyst. Before undertaking this type of
endoscopic intervention, the endoscopist must be confident that a
cystadenoma has not been mistaken for a pseudocyst
Endoscopic Retrograde Cholangiopancreatography

ERCP, demonstrated in the image below, provides the most accurate

visualization of the pancreatic ductal system and has been regarded as the
criterion standard for diagnosing chronic pancreatitis. One limitation of ERCP,
however, is that it cannot be used to evaluate the pancreatic parenchyma, and
histologically proven chronic pancreatitis has been documented in the setting of
normal findings on pancreatogram. Pancreatograms can be interpreted and
classified according to several schemes, such as the Cambridge criteria. A
comparison of ERCP scoring with direct pancreatic function tests demonstrated
good correlation. However, pancreatography tended to show significantly more
severe changes.
The problems with ERCP are that it is invasive and expensive, requires complete
opacification of the pancreatic duct to visualize side branches, and carries a
risk (operator-dependent) of pancreatitis.

This endoscopic retrograde cholangiopancreatography (ERCP) shows

advanced chronic pancreatitis. The pancreatogram has blunting of the lateral
branches, dilation of the main pancreatic duct, and filling defects consistent
with pancreatolithiasis. The cholangiogram also shows a stenosis of the distal
bile duct and a dilated biliary tree.

Magnetic Resonance Cholangiopancreatography

MRCP, demonstrated in the image below, provides information on the pancreatic

parenchyma and adjacent abdominal viscera, and MRCP uses heavily T2-weighted
images to visualize the biliary and pancreatic ductal system. The use of secretin
during the study enhances the quality of the pancreatogram. Accuracy is
improving, and MRCP is relatively safe, reasonably accurate, noninvasive, fast,
and very useful in planning surgical or endoscopic intervention.

Chronic pancreatitis. This magnetic resonance cholangiopancreatography

(MRCP) shows a healthy biliary system. The pancreatic ductal system is not
well visualized. A subsequent endoscopic retrograde
cholangiopancreatography (ERCP [not pictured]) showed pancreas divisum,
with no evidence of a communication with the pseudocyst. The endoscopic
features were ideal for an endoscopic transgastric pseudocystogastrostomy.

Endoscopic Ultrasonography
Although studies suggest that endoscopic ultrasonography (EUS) may be the best
test for imaging the pancreas, it requires a highly skilled gastroenterologist. Eleven
sonographic criteria have been developed that identify characteristic findings
of chronic pancreatitis. The most predictive endosonographic feature is the
presence of stones. Other suggestive features include the following:
• Visible side branches
• Cysts
• Lobularity
• An irregular main pancreatic duct
• Hyperechoic foci and strands
• Dilation of the main pancreatic duct
• Hyperechoic margins of the main pancreatic duct
Before 2001, three or more of these criteria on EUS were used to diagnose chronic
pancreatitis. However, subsequent data has suggested the use of five or more
criteria to have higher specificity, rather than sensitivity, to diagnose chronic
pancreatitis. In general, the presence of five or more of these features is
considered highly suggestive of chronic pancreatitis. EUS may be as sensitive and
specific as tube tests for mild and advanced disease, especially when combined
with fine needle aspiration or Tru-Cut biopsy.

Histologic Findings

In the early stages of chronic pancreatitis, the parenchyma exhibits an increase

in connective tissue around the ducts and between the lobules. The degree of
inflammation is minimal to moderate, consisting mostly of T lymphocytes, and a
patchy, focal process unevenly affects the pancreas. With increasing severity, the
connective tissue progresses between the acini, which gradually become
distorted and tend to disappear. In advanced disease, fibrous tissue replaces
the acinar tissue, and the pancreas becomes contracted, small, and hard. The
islets of Langerhans are relatively spared until very late in the disease process.
Patients can have severe histopathologic changes of chronic pancreatitis despite
normal findings on imaging studies. In patients undergoing resection of the
pancreas for chronic pancreatitis, focal necrosis is found in 11.9% of cases and
segmental fibrosis is observed in approximately 40% of cases.
In chronic calcific pancreatitis, plugs of precipitated protein develop within
the ductal system. While they may be observed in all types of chronic pancreatitis,
in alcoholic and tropical forms these plugs tend to evolve into calculi by
deposition of calcium within them. The calcified pancreatic calculi are
distributed irregularly, affecting ducts of various sizes, and may be associated
with ulcerations of the ductal epithelium. Periductal connective tissue may
encroach into the lumen and cause ductal stenoses, creating the "chain of
lakes" pancreatogram appearance observed in advanced chronic calcific
pancreatitis.
Genetic testing
]

Indications: family history of chronic pancreatitis

young patients with idiopathic pancreatitis

• PRSS1

gene mutations: diagnostic of hereditary pancreatitis

• CFTR gene mutations: seen in ∼ 30% of patients with Idiopathic Chronic

pancreatitis

• SPINK1 gene mutations: seen in ∼ 20% of patients with chronic

pancreatitis

Management/Treatment
The goals of medical treatment are as follows:
• Modify behaviors that may exacerbate the natural history of the disease
• Enable the pancreas to heal itself
• Determine the cause of abdominal pain and alleviate it
• Detect pancreatic exocrine insufficiency and restore digestion and absorption to
normal
• Diagnose and treat endocrine insufficiency
Overview
Chronic pancreatitis patients require supportive measures. The initial stage in
management of patients with chronic pancreatitis should include assessment of the
etiology and severity of the disease, because both of these factors affect the mode
of treatment. Treatment is generally directed toward control of pain,
correction of
problems related to pancreatic exocrine and endocrine insufficiency, and the
correction of associated biliary tract and gastrointestinal tract pathology .
Abdominal pain is a difficult symptom to treat in chronic pancreatitis. Because
pain is a subjective sensation, there is no objective parameter for measurement or
means to monitor its occurrence.
Medical and non-pharmacological Therapy
Alcohol and Cigarette Smoking
Avoidance of alcohol ingestion decreases the frequency and the severity of
abdominal pain. Cigarette smoking has been correlated with intraductal
calcifications in chronic pancreatitis patients. Also, both alcohol and cigarette
smoking correlated significantly with number of pain relapses. Patients with
chronic pancreatitis shouldbe advised to avoid cigarettes and alcohol.
Analgesics
Non-narcotic analgesics (salicylates, acetaminophen, ibuprofen and
nonsteroidal analgesics) should be used initially for pain control. These drugs
should be used before meals to prevent postprandial exacerbation of pain.
Dosage should be individualized, beginning with the lowest effective dose. With
increased severity of pain,
dosing frequency and strength should be increased. Episodes of severe
abdominal pain may require limited use of narcotic analgesics such as
acetaminophen with codeine. Opiate analgesics are required in severe cases of
chronic pancreatitis. The pain of chronic pancreatitis is usually intermittent and
postprandial, but when pain becomes persistent, affecting the patient's lifestyle,
effective pain management becomes the most crucial part of treatment.
Enzyme Therapy
The therapeutic goal of pancreatic enzyme therapy is to control diarrhea and
help the patient to gain body weight. Many physicians advocate the use of
pancreatic enzymes with acid suppression to inhibit pancreatic secretion and
possibly decrease pancreatic intraductal pressure, and lessening pain. Enzyme
therapy in chronic
pancreatitis is critical for management of malabsorption problems. Diarrhea
symptoms significantly improve with oral pancreatic enzyme therapy (with at
least 24,000–32,000 units of lipase), but complete correction of steatorrhea is
sometimes difficult to achieve, even with large amounts of enzyme
supplementation. The clinical usefulness of pancreatic enzymes may be assessed
by the patient's weight, ideally gaining two pounds each week and stabilizing
at 10% below ideal body weight.
Treatment of Malnutrition
A result of maldigestion and malabsorption of fats, carbohydrates and proteins,
protein energy malnutrition is a frequent abnormality in patients with chronic
pancreatitis. Therapy for protein energy malnutrition requires correction of
malabsorption, and administration of high-protein, high-calorie diets. In
severely malnourished chronic pancreatitis patients, total parenteral nutrition
may be the preferred treatment. The pancreas is nutrition-sensitive;
consequently, malnutrition
may lead to atrophy or fibrosis. Medium-chain triglyceride preparations are a
good source of lipid calories for this group of patients. However, nausea and
unpleasant taste frequently limit its use.

Surgical Therapy
The progression of chronic pancreatitis is not always predictable, but typically the
disease can be characterized by intractable abdominal pain, a state of exhaustion
resulting from lack of food and water, chronic depression, and often chemical
dependency. Although the malabsorption and diabetes mellitus associated with
chronic pancreatitis can be treated medically, intractable pain ultimately becomes a
major surgical indication in approximately one-third of patients. There is
controversy over
the role and timing of surgery in management of the patient with chronic
pancreatitis. Early intervention is recommended to prevent irreversible functional
impairment of the pancreas. Because the surgery is not uniformly successful and
there is a significant recurrence of symptoms, others advocate expectant therapy.
There is no single surgical procedure uniformly recommended for all patients with
chronic pancreatitis.
 The surgical procedure is selected according to the severity of pain,
ductal morphology, the extent of parenchymal disease, and the overall
condition of the patient. The goal of surgery in chronic pancreatitis patients is
to relieve intractable pain while preserving endocrine and exocrine functions
of the pancreas. The results of surgical procedures are inconsistent in their ability
to control pain.

The Puestow Procedure

A Puestow procedure is indicated for the treatment of symptomatic chronic

pancreatitis patients with pancreatic ductal obstruction and a dilated main
pancreatic duct. The main pancreatic duct needs to be 6mm in diameter in the body
of the pancreas for this procedure to be possible.
The longitudinal pancreaticojejunostomy, or Puestow's procedure, is the
prototypic drainage procedure for patients with marked dilation of the main
pancreatic duct (greater than 7–8 mm). An 8–10-cm segment of the pancreatic
duct is unroofed and intraductal concretions removed. The jejunum is divided and
the opened pancreatic duct is anastomosed to the jejunum. This allows adequate
drainage to enter the jejunum. A jejunojejunostomy reconnects the jejunum to
restore continuity of the gastrointestinal tract. This procedure is successful in
relieving pain in 70–80% of patients in the short term. Pancreatic function
remains unchanged because there has not been resection of the gland. It is a
safe and effective surgery with low morbidity and mortality
One of the problems that can lead to failure of the Puestow procedure is that pain
can persist due to failure to drain the pancreatic duct on the head of the pancreas.
A Frey's procedure is an alternative surgical procedure to the Puestow that allows
for better drainage of the head, but pancreatic tissue is removed.

Frey's procedure is a surgical technique used in the treatment of

chronic pancreatitis in which the diseased portions of the pancreas head are cored
out. A lateral pancreaticojejunostomy (LRLPJ) is then performed in which a loop
of the jejunum is then mobilized and attached over the exposed pancreatic duct to
allow better drainage of the pancreas, including its head.[

Indication
Frey's operation is indicated on patients with chronic pancreatitis who have "head
dominant" disease.

Comparison to Puestow procedure

Compared with a Puestow procedure, a Frey's procedure allows for better drainage
of the pancreatic head.
Complications
Postoperative complications after LRLPJ are usually septic in nature and are likely
to occur more often in patients in whom endoscopic pancreatic stenting has been
performed before surgical intervention.
Pancreatic endocrine insufficiency occurs in 60% of patients.

Severe chronic pain sometimes does not respond to painkilling medications. The
ducts in the pancreas may have become blocked, causing an accumulation of
digestive juices which puts pressure on them, causing intense pain. Another cause
of chronic and intense pain could be inflammation of the head of the pancreas.

Several forms of surgery may be recommended to treat more severe cases.

Endoscopic surgery

A narrow, hollow, flexible tube called an endoscope is inserted into the digestive
system, guided by ultrasound. A device with a tiny, deflated balloon at the end is
threaded through the endoscope. When it reaches the duct, the balloon is inflated,
thus widening the duct. A stent is placed to stop the duct from narrowing back.

Pancreas resection

The head of the pancreas is surgically removed. This not only relieves the pain
caused by inflammation irritating the nerve endings, but it also reduces pressure on
the ducts. Three main techniques are used for pancreas resection:

 The Beger procedure: This involves resection of the inflamed pancreatic

head with careful sparing of the duodenum, the rest of the pancreas is
reconnected to the intestines.
 The Frey procedure: This is used when the doctor believes pain is being
caused by both inflammation of the head of the pancreas as well as the
blocked ducts. The Frey procedure adds a longitudinal duct decompression
 to the pancreatic head resection – the head of the pancreas is surgically
removed, and the ducts are decompressed by connecting them directly to the
intestines.
 Pylorus-sparing pancreaticoduodenectomy (PPPD): The gallbladder,
ducts, and the head of the pancreas are all surgically removed. This is only
done in very severe cases of intense chronic pain where the head of the
pancreas is inflamed, and the ducts are also blocked. This is the most
effective procedure for reducing pain and conserving pancreas function.
However, it has the highest risk of infection and internal bleeding.

Total pancreatectomy

This involves the surgical removal of the whole pancreas. It is very effective in
dealing with the pain. However, a person who has had a total pancreatectomy will
be dependent on treatment for some of the vital functions of the pancreas, such as
the release of insulin.

Whipple Procedure
Pancreatoduodenectomy, or Whipple resection, has been recommended for
treatment of chronic pancreatitis primarily involving the head of the pancreas. The
procedure has a mortality rate of less than 5% and 25–30% morbidity. The
procedure is indicated for patients who have failed previous duct drainage
procedures, those with multiple, small pseudocysts located in the head of the
pancreas and/or uncinate portions of the gland, those with symptomatic
gastric or biliary obstruction associated with extensive fibrosis or multiple
pseudocysts, and those with hemorrhage from inflammatory aneurysms
involving major peripancreatic vessels. Standard
pancreaticoduodenectomy involves resection of the head of the pancreas,
duodenum, gallbladder, distal common bile duct and antrum. In chronic
pancreatitis, preservation of the antrum and proximal 1–2 cm of duodenum is
a necessary modification in preserving the pylorus and minimizing severe
endocrine insufficiency.
Pain relief is achieved in 60–80% of patients in the first several years after surgery
Anatomy of a Whipple procedure

Distal Pancreatectomy
The term distal pancreatectomy describes resection of variable amounts of the
body and tail of the pancreas. Partial pancreatic resection is recommended for
patients with diffuse (moderate to severe) parenchyma disease without ductal
dilation, especially in the tail and body. Local resection of major pancreatic sites of
involvement
may be sufficient for those patients with regional disease, whereas a 95% distal
resection is recommended for patients with diffuse disease. Ninety-five percent
distal pancreatectomy entails removal of the spleen and almost all of the
pancreas, except for a thin rim of tissue within the "C" loop of the duodenum.
Splenic preservation
is attempted, but often fails because dissection of splenic vessels from the
chronically inflamed and scarred pancreas is extremely difficult. This
procedure provides pain relief for 75–80% of patients and has a mortality rate less
than 5%
Distal pancreatectomy

Celiac Plexus block/ neurolytics

Patients with advanced stages of chronic pancreatitis may fail to have adequate
control of pain with oral drug therapy. In these patients, more aggressive
intervention is required. In cases of intractable pain, injection of a local anesthetic
around the nerve temporarily inhibits nerve fibers from transmitting pain
messages. Celiac plexus blocks are a sufficiently safe and effective treatment
for the management of abdominal pain. These treatments, however, provide
only short-term relief and repeat procedures may be necessary.
Pain management in chronic pancreatitis may employ a neurolytic substance like
ethanol. Neurolytic blocks should be used in a multimodal approach to control
pain and not as a "cure." Ethanol has been used extensively in neurolytic
procedures to destroy nerve tissues by extraction of cholesterol and other lipids and
by protein
precipitation.
The celiac plexus is located on the anterolateral surface of the aorta at the
T12–L2 spinal level. Celiac plexus block reduces pain from abdominal organs.
This form of pain treatment has gained acceptance for the treatment of chronic
pancreatitis. An 84% incidence of pain relief has been reported with celiac plexus
block, although
occasionally repeat blocks are necessary.
Celiac plexus block can be performed by three different approaches.
 One approach, performed by an anesthesiologist, involves the passage
of a needle through the skin (percutaneous) into the celiac plexus. This
procedure is low risk and performed on an outpatient basis in 30–60
minutes.
 A second approach involves injection of the celiac plexus during
surgery while the abdomen is open .
 The final approach employs endoscopic ultrasound guidance to
insert the needle into the celiac plexus
Endoscopic Therapy
Several studies have shown that endoscopic sphincterotomy, stent placement,
stricture dilation, and stone extraction are effective in short-term pain relief. The
mechanism of this improvement is based on the theory that in a significant number
of patients, pain is predicated on increased intraductal pressure. The goal of
endoscopic therapy, like surgery, is decompression of the main pancreatic duct.
There is a 75% success rate for endoscopic therapy. The advantage of endoscopic
therapy is that it is a relatively noninvasive procedure. The endoscopic therapeutic
approach should be regarded as an alternative to surgery in an integrated
management plan for the chronic pancreatitis patient and may predict candidates
who will benefit from surgical intervention.
Endoscopy is a well-established alternative for the management of a variety of
biliary tract diseases, and has proven to be useful in the treatment of strictures and
other obstructions (stones and protein plugs) that affect patients with chronic
pancreatitis. Matching the appropriate endoscopic treatment modality to the
appropriate candidate is critical for optimal therapeutic results. Chronic
pancreatitis is a new and exciting challenge for the potential of endoscopic therapy.
Careful patient selection is crucial for optimal therapeutic results.
Endoscopic management of the chronic pancreatitis patient should be considered
one option along with medical, surgical and percutaneous treatment. Specific
recommendations are difficult to make because of the scarcity of literature
regarding controlled studies, long-term follow-up, cost-efficacy studies, and results
ofsurgical versus endoscopic treatment.
Endoscopic Pancreatic Sphincterotomy
Endoscopic pancreatic sphincterotomy has been used to reduce pancreatic duct
pressure and to facilitate other procedures such as pancreatic stent placement,
tissue sampling, dilation of strictures, or stone removal. The procedure is
performed at the major papilla in most chronic pancreatitis patients, but at the
minor papilla in those patients with pancreas divisum. The papilla is divided
maximally for stone extraction, whereas more modest splits suffice for drainage of
pancreatic
secretions.
Two devices may be utilized to perform pancreatic sphincterotomy: a pull-type
sphincterotome (with or without a guide wire) or a needle-knife
sphincterotome. A pull-type sphincterotome is inserted into the pancreatic duct
and a 5–10-mm incision is made in the 1–2 o'clock orientation along the pancreatic
duct axis.
Treatment of Strictures
Endoscopic treatment strategies are less invasive alternatives to surgical duct
decompression procedures, and are used to treat strictures resulting from chronic
pancreatitis. Sphincterotomy , catheter or balloon dilation, and stent placement
are included in these techniques. These techniques are often challenging because
of the tortuosity and fibrotic nature of the ductal system. Endoscopic treatment
may require multiple sessions to achieve or maintain a positive therapeutic result.
Careful
selection of patients is essential for these procedures. Relief of symptomatic pain
associated with chronic pancreatitis is the primary rationale for treatment of
pancreatic duct strictures. The mechanism of pain in chronic pancreatitis may be
multifocal, including elevated parenchymal ischemia pressure resulting from
outflow obstruction, ischemia, and inflammation causing nerve entrapment, and
pancreatic ischemia. Current studies on chronic pancreatitis report that
decompression of the pancreatic duct may have a beneficial effect on
preserving organ function.
Ductal dilation alone is rarely successful in resolving strictures that arise from
chronic pancreatitis and is, therefore, often accompanied by stenting. A guide
wire is passed to the tail end of the pancreas, and dilating bougie or balloon
catheters are advanced over the wire to dilate the stricture (some patients may
require sphincterotomy to facilitate endoprosthesis insertion). Passage of the
deflated balloon through the stenosis may be difficult and require preliminary
bougienage. After insertion, the balloon is filled with contrast medium to a
specified pressure utilizing an inflation device for a variable
duration, enlarging lumen diameter. Usually a stent is inserted extending beyond
the previous stricture site to maintain patency).
Standard pancreatic stents are plastic tubes with holes along the sides at 1-cm
intervals for better side branch pancreatic juice flow. The stent is anchored in place
by pigtails or flaps. The diameter and length of the stent are subjective, depending
upon the location and severity of the stricture as well as the duct size, but generally
stent diameter should not be greater than the size of the downstream duct. The stent
is inserted coaxially over the guide wire after the dilation is completed and the
dilating catheter removed.
Frequently, shorter stents are used to decompress the duct in the head of the
pancreas. The stent is usually left in place for a short time. Prolonged stent usage
may induce changes of chronic pancreatitis
Stone Extraction
Duct stones, which may obstruct the flow of pancreatic juices, are a common
finding in severe chronic pancreatitis. Endoscopic extraction, alone or combined
with extracorpeal shock wave lithotripsy, has been useful in the treatment of
ductal obstructions.
Extracorporeal shock wave lithotripsy (ESWL) has been used to fragment
calcified pancreatic stones before extraction. ESWL is not necessary with protein
plugs (non-calcified stones), which are usually soft and pliable. During ESWL, 100
shock waves per minute are delivered at an electric power of approximately 20 kV
during a 30-minute session. A mean of 1,500 shock waves per stone is usually
required. Fluoroscopic monitoring evaluates quality of stone fragmentation. A
small Dormia
basket is used to remove stone fragments. The closed Dormia basket is introduced
and extended beyond the calculi. It is opened and traps the stone (Figure 36). The
basket is tightened, securing the stone, and withdrawn. A sphincterotomy may be
required to facilitate passage through the papilla
Pancreatic pseudocysts

Advances in interventional endoscopy now enable endoscopic treatment of many

pseudocysts. In the appropriate clinical setting, obtain a pancreatogram to
determine whether the pancreatic duct communicates with the pseudocyst. Ideally,
communicating pseudocysts found in the head or body can be treated with
transpapillary stents, with a success rate of 83% and a complication rate of 12%.
Noncommunicating pseudocysts that bulge into the foregut and have a mature wall
less than 1 cm thick are treatable by endoscopic transduodenal or transgastric
pseudocystostomy. The success rate is 85%, with a 17% complication rate. The
transduodenal approach has fewer complications and recurrences than the
transgastric approach. The long-term success rate of the initial procedure is
reported at 62%.

Total pancreatectomy and islet autotransplantation

In 46 patients undergoing near-total pancreatectomy, pain relief occurred in 82%

(resolved in 39% and improved in 43%). Although 51% were insulin independent
initially, this decreased to 34% (one third) from 2-10 years after transplantation.
In selected patients, the long-term morbidity caused by diabetes following total
pancreatectomy can be avoided. Doing so involves harvesting the islets from the
resected pancreas and injecting them into the portal system, which then lodges
them in the liver. Increasing severity of pancreatic fibrosis correlates positively
with poor recovery of islets (< 300,000) and insulin dependence.
Diet

A diet low in fat and high in protein and carbohydrates is recommended, especially
in patients with steatorrhea. The degree of fat restriction depends on the severity of
fat malabsorption; generally, an intake of 20 g/day or less is sufficient. Patients
who continue to suffer from steatorrhea following fat restriction require medical
therapy.
Clinically significant protein and fat deficiencies do not occur until over 90% of
pancreatic function is lost. Steatorrhea usually occurs prior to protein deficiencies,
since lipolytic activity decreases faster than proteolysis.
Specific recommendations include a daily diet of 2000-3000 calories, consisting of
1.5-2 g/kg of protein, 5-6 g/kg of carbohydrates, and 20-25% of total calories
consumed as fat (about 50-75g) per day.
Malabsorption of the fat soluble vitamins (A, D, E, and K) and vitamin B-12 may
also occur. Oral supplementation of these enzymes is recommended.
Complications
Chronic pancreatitis is associated with a variety of complications, including
intractable pain, pseudocyst formation, fistulas, obstructive
jaundice, intestinal obstruction, and hemorrhage, some of which
may necessitate surgery