CHRONIC FIBROSING PANCREATITIS

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CHRONIC FIBROSING PANCREATITIS

Chronic pancreatitis is uncommon in the pediatric age group and has received

little attention in pediatric textbooks. Diagnosis is often difficult and is

usually missed for several months or even years. In adults, chronic pancreatitis

is usually secondary to alcohol abuse or biliary tract disease.

The two most common causes of chronic pancreatitis in childhoodarehereditary

pancreatitis and fibrosing pancreatitis. Hereditary pancreatitis is a cause of

chronic relapsing pancreatitis that is of autosomal dominant inheritance. The

cause of hereditary pancreatitis is not known. However the presence of

pancreatic ductular malformations such as ductular ectasia, even in the youngest

patient reported, or hypertrophy of the sphincter of Oddi suggests a basic

anatomic abnormality in the pancreatic ductular system. These patients often

demonstrate aminoaciduria or hyperlipidemia.

Other causes of recurrent pancreatitis in childhood include cystic fibrosis,

hereditary hyperparathyroidism, hyperlipoproteinemia, ascariasis, and idiopathic

pancreatitis which is termed chronic fibrosing or sclerosing pancreatitis.

Fibrosing pancreatitis is characterized by exacerbations of pain and upper

abdominal discomfort with intervals of seeming well- being. The outstanding

feature of fibrosing pancreatitis is usually severe pain. Most paitents have a

long history of chronic relapsing pain with vomiting, weight loss, and

steatorrhea. It should be noted however that Meneely et al reported a case of

fibrosing pancreatis in a patient who had no abdominal pain.

Laboratory examinations of these patients usually reveal elevated amylase values

with an increased amylase to creatinine clearance ratio, normal lipid panels,

normal to elevated direct bilirubin, and moderately elevated SGOT and SGPT with

a markedly elevated alkaline phosphatase. The diagnostic laboratory tests are

helpful only in determining the presence of obstructive jaundice and of

involvement of the pancreas. The exact cause of this disorder is not known but

some type of ampullary stenosis which may lead to reflux of bile into the

pancreatic ducts is at present the most appealing explanation. Studies done by

Wainwright in 1951 suggest that chronic fibrosing pancreatitis is due to

intermittent episodes of focal acinar rupture with subsequent scarring and

ductal narrowing. It is possible that bile reflux due to a common channel and

perhaps sphincteric stenosis might be responsible for this condition.

Patients with fibrosing pancreatitis who have obstructive jaundice may be

evaluated preoperatively with two modalities. ERCP can be performed in children

over the age of 5 years. Percutaneous transhepatic cholangiography can be

utilized in younger children, especially when dilated biliary ducts are

suspected from ultrasonography. In the series of patients reviewed by Ghishan et

al at Vanderbilt, ERCP has proved to be a safe procedure.

The ideal surgical approach is uncertain and only time will tell what is most

appropriate. Sphincteroplasty has been associated with long lasting relief in a

series of patients described by Holcomb et al. The choice of surgery depends on

the anatomy of the pancreatic and biliary trees. If bile duct obstruction is

complete, Roux- Y drainage is probably the safest form of management. In those

instances in which the biliary tree appears to be normal and the pancreas is the

site of severe fibrosing process, direct pancreatic drainage is in order. It

would appear from follow- up studies ina series of patients treated surgically

for recurrent pancreatitis that progressive disease can be arrested if

obstruction of biliary and pancreatic flow can be relieved. Early surgery

appears to offer two advantages: possible prevention of exocrine pancreatic

insufficiency and diabetes, and normal life without frequent episodes of pain

and hospitalization.

Mark E. Armstrong

www.top5plus5.com

Oregon State Chapter Rep

Pancreatitis Association, International