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A 55-Year-Old Man with Idiopathic Recurrent Pancreatitis

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A 55-Year-Old Man with Idiopathic Recurrent Pancreatitis A SELF-TEST IM BOARD REVIEW DAVID L. LONGWORTH, MD, JAMES K. STOLLER, MD, EDITORS OF CLINICAL RECOGNITION J. BRAD MORROW, MD DARWIN L. CONWELL, MD Department of Gastroenterology, Cleveland Clinic Director, Pancreas Clinic, Department of Gastroenterology, Cleveland Clinic A 5 5-year-old man with idiopathic recurrent pancreatitis 55 -FIVE'YEAR-OLD male business execu- is soft and nontender with no palpable masses. 0tive presents to the gastroenterology The liver and spleen are not enlarged. The clinic for further evaluation of recurrent extremities are without clubbing or edema. episodes of acute pancreatitis. Neurologic examination is normal. In the previous 18 years, he has had seven separate bouts of severe abdominal pain diag- • LABORATORY DATA nosed both clinically and biochemically as acute pancreatitis. These included two The results of a complete blood count and episodes of acute pancreatitis in the prior 8 blood chemistry panel (including liver bio- months. Each time, he was treated with sup- chemistries) are within normal limits. portive therapy, including intravenous fluids, bowel rest, and parenteral narcotics, and his DIFFERENTIAL DIAGNOSIS symptoms resolved completely. None of the episodes were accompanied by jaundice. Which of the following is not a possible Several ultrasound scans of the abdomen 1 cause of this patient's recurrent pancreatitis? were done during the course of these attacks, Up to 20% of and each time they were negative for gallstones • Sphincter of Oddi dysfunction or biliary ductal dilatation. Computed tomo- • Biliary microlithiasis cases of acute • graphic (CT) scans of the abdomen during • Hypertriglyceridemia pancreatitis are these episodes revealed only pancreatic edema Diabetes mellitus idiopathic and mild pancreatic ductal dilatation in the J Hypercalcemia head of the pancreas. Endoscopic retrograde cholangiopancreatography (ERCP) performed Gallstones and alcohol abuse are the two most 15 years earlier revealed nothing remarkable. common causes of acute pancreatitis, Other than the recurrent bouts of pancre- accounting for approximately 70% of all cases. atitis, the past medical history is unremark- Another 10% of cases can be explained by able. The patient denies any recent weight miscellaneous causes such as sphincter of loss, and he is asymptomatic at the time of his Oddi dysfunction, hypertriglyceridemia, or evaluation. His only medication is ranitidine, hypercalcemia (TABLE 1). This leaves up to 20% which he takes occasionally for symptoms of of patients presenting with acute pancreatitis dyspepsia. There is no family history of pan- that is characterized as "idiopathic." creatitis or pancreatic carcinoma. He denies Recently, biliary sludge (or microlithiasis) any ethanol consumption. He does not smoke has been implicated as the most common tobacco or use illicit drugs. cause of idiopathic recurrent pancreatitis. Biliary sludge consists of a suspension of either • PHYSICAL EXAMINATION cholesterol monohydrate crystals or calcium bilirubinate granules in liquid bile (FIGURE 1). It On examination, the patient is a well-appear- often appears on ultrasonography as layered, ing man, without scleral icterus. Examinations low-amplitude echoes without the shadowing of the heart and lung are normal. The abdomen associated with true gallstones. Clinically, 84 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 66 • NUMBER 2 FEBRUARY 1999 Downloaded from www.ccjm.org on September 26, 2021. For personal use only. All other uses require permission. physicians should suspect microlithiasis in any TABLE 1 patient with unexplained acute pancreatitis, especially in those with advanced age or abnor- Causes of acute pancreatitis mal liver chemistries during an acute episode. Structural Diabetes mellitus is not known to cause Gallstones acute pancreatitis. Microlithiasis Pancreas divisum • EVALUATION FOR MICROLITHIASIS Ampullary tumor Periampullary diverticula Sphincter of Oddi dysfunction If you suspect microlithiasis, what is the Toxins 2 single best test to make this diagnosis? Ethanol Medications • Magnetic resonance imaging (MRI) Methanol of the abdomen Scorpion venom • Hepatobiliary scintigraphy Organophosphate poisoning • Serum lipid profile Metabolic • Bile collection for microscopic analysis Hypertriglyceridemia Hypercalcemia • Ultrasonography of the abdomen Traumatic Endoscopic retrograde Microscopic evaluation of the bile for choles- cholangiopancreatography terol crystals or calcium bilirubinate granules Blunt abdominal trauma is the gold standard for diagnosing microlithi- Miscellaneous asis or biliary sludge. Ultrasonography is a Infection (viruses, parasites, bacteria) noninvasive and important initial test; how- Crohn's disease ever, it may miss up to 50% of cases of clini- idiopathic cally important microlithiasis. MRI, scintigra- phy, and lipid tests have no role in the evalu- ation for microlithiasis. Bile can be collected endoscopically or via pancreatitis due to microlithiasis? Ultrasound duodenal intubation with a Dreiling tube. In will detect only either case, the patient must fast overnight • Cholecystectomy and then receive an intravenous dose of • Endoscopic biliary sphincterotomy 50% of cases of cholecystokinin to stimulate gallbladder emp- • Medical dissolution therapy with ursodiol microlithiasis tying before bile is collected. The collected • Gemfibrozil bile is then sent to the laboratory for micro- scopic inspection for crystals. Cholecystectomy, endoscopic biliary sphinc- As part of our standard approach, our terotomy, and ursodiol therapy are all reason- patient underwent ERCP to evaluate his pan- able options once microlithiasis has been creatic ductal anatomy and rule out a structur- detected, as they have all been shown to signif- al etiology of his recurrent episodes of acutc icantly reduce the recurrence rate of acute pan- pancreatitis. During the procedure, biliary creatitis in patients with biliary microlithiasis. fluid was collected as described above. Cholecystectomy is considered the treat- Microscopic analysis of the bile revealed ment of choice for patients who are good sur- numerous cholesterol monohydrate crystals gical candidates. It is the best studied option (FIGURE 1). Based on this analysis, the etiology and can usually be performed laparoscopically of this patient's recurrent pancreatitis was felt with minimal morbidity. to be microlithiasis. Lee et al1 described 21 patients with recurrent pancreatitis and microlithiasis, of • THERAPY whom 6 underwent cholecystectomy and 4 underwent biliary sphincterotomy. The other Which of the following is not a therapeu- 11 patients had no intervention. None of the 3 tic option to prevent recurrent episodes of 6 cholecystectomy patients and only 1 of the 4 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 66 • NUMBER 2 FEBRUARY 1 999 1 17 Downloaded from www.ccjm.org on September 26, 2021. For personal use only. All other uses require permission. IM BOARD REVIEW £2 FIGURE 1. Microscopic features of biliary sludge (x 200). Left, biliary sludge consisting predominantly of cholesterol crystals. Note the typical rhomboidal plates. Right, biliary sludge containing predominantly calcium bilirubinate granules. Some cholesterol crystals are present, but they are outnumbered by the many reddish-brown granules composed of calcium bilirubinate. SOURCE: LEE SP, NICHOLLS JF, PARK HZ. BILIARY SLUDGE AS A CAUSE OF ACUTE PANCREATITIS. N ENGL J MED 1992; 326:589-593. COPYRIGHT 1992 MASSACHUSETTS MEDICAL SOCIETY. ALL RIGHTS RESERVED. sphincterotomy patients had recurrent episodes of acute pancreatitis at 12 months episodes of pancreatitis on follow-up (up to 7 follow-up. years). In contrast, 8 (73%) of the 11 patients who had no therapy suffered recurrent bouts • SUMMARY POINTS of acute pancreatitis. In a similar series of patients, Ros et al2 • Gallstones and alcohol abuse account Cholecystectomy reported that 17 (94%) of 18 patients who for 70% of cases of acute pancreatitis. prevents further underwent cholecystectomy did not have any • Microlithiasis (or biliary sludge) is a recurrent bouts of acute pancreatitis at a mean common, yet often unrecognized cause of attacks of follow-up of 36 months.2 "idiopathic" pancreatitis. pancreatitis due Ursodiol is a naturally occurring bile acid • The diagnosis of microlithiasis can be used clinically to dissolve radiolucent gall- made by either ultrasonography or microscop- to microlithiasis stones. It has been used successfully to prevent ic examination of collected bile, but ultra- further episodes of acute pancreatitis in sonography is less sensitive. patients whose collected bile contained cho- • Once microlithiasis has been detected, lesterol crystals.2 However, ursodiol therapy cholecystectomy should be performed to pre- requires several months to dissolve crystals vent further attacks of acute pancreatitis. and stones, and stones recur within 5 years in • Endoscopic biliary sphincterotomy or up to 50% of patients. We therefore recom- oral dissolution therapy with ursodiol are mend its use only in patients not felt to be options in patients not felt to be surgical can- candidates for cholecystectomy. didates. • Gemfibrozil is a lipid-modulating agent • REFERENCES used in patients with pancreatitis from hyper- 1. Lee SP, Nicholls JF, Park HZ. Biliary sludge as a cause of triglyceridemia, but not in cases of pancreati- acute pancreatitis. N Engl J Med 1992; 326:589-593. tis due to biliary crystals. 2. Ros E, Bru C, Garcia-Puges A, Valderrama R. Occult microlithiasis in idiopathic acute pancreatitis: prevention of relapses by cholecystectomy or ursodeoxycholic acid • FOLLOW-UP therapy. Gastroenterol 1991; 101:1701-1709. ADDRESS: Darwin L. Conwell, MD, Department of Our patient successfully underwent laparo- Gastroenterology, S40, The Cleveland Clinic Foundation, 9500 scopic cholecystectomy and had no further Euclid Avenue, Cleveland, OH 44195. CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 66 • NUMBER 2 FEBRUARY 1 999 1 17 Downloaded from www.ccjm.org on September 26, 2021. For personal use only. All other uses require permission..
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