- Email: [email protected]
Endoscopic therapy of a duodenal duplication cyst
REFERENCES
than $900, but their use in our patient enabled his discharge from the hospital, and probably lowered the total cost of his medical care as a result. Complications may occur when the Wallstent is used to palliate malignant biliary strictures, and luminal use of the device could be accompanied by similar complications. Stent obstruction can be caused by ingrowth of tumor through the interstices of the stainless steel mesh, by overgrowth of tumor around the ends of the stent, or by vegetable fiber clogging the lumen. Wallstent dislocation and hemobilia have occurred infrequently.14 The stents can only be removed surgically. The possibility of perforation is a concern when using stents in the gastrointestinal tract, although Wallstent perforations of the biliary tree or the duodenum opposite the papilla have not been reported. Further investigation into the stent's effect on the bowel is warranted, with design modifications if needed. The effect of the Wallstent on the jejunal wall in our patient is unknown. Because the Wallstent shortens during deployment, care must be taken to ensure that the shortened stent will adequately bridge the stricture. Our patient required the placement of two stents, one partially nested inside the other, to stent his entire occluded segment of jejunum. Although the inner diameter of the Wallstent is somewhat less than that of most commercial rigid esophageal endoprostheses (10 mm VS. 12 mm), palliation of our patient's symptoms was obtained for the short duration of his remaining life. Wallstent endoprosthesis placement in the intestinallumen may join the array of palliative techniques available to patients with incurable, inoperable malignant gastrointestinal obstruction.
1. Lope M, Natali M, DiLauro L, et al. 5-Fluorouracil, epirubicin, and BCND in advanced measurable gastric cancer. Am J Clin Oncol 1990;13:204-7. 2. Elliot TE, Moertel CG, Wieand HS, et al. A phase II study of the combination of etoposide and cisplatin in the therapy of advanced gastric cancer. Cancer 1990;65:1491-4. 3. Eckhauser ML. The neodynium-YAG laser and gastrointestinal malignancy. Arch Surg 1990;125:1152-4. 4. Suzuki H, Miho 0, Watanabe Y, Kohyama M, Nagao F. Endoscopic laser therapy in the curative and palliative treatment of upper gastrointestinal cancer. World J Surg 1989;13:158-64. 5. Schwegler D, Orth M, Kuntz HD, May B. Endoscopic palliative therapy of malignant esophageal and cardia stenoses. Results, complications, follow-up. Bildgebung 1987-1989;56:69-75. 6. Fugger R, Niederle B, Jantsch H, Schiessel R, Schulz F. Endoscopic tube implantation for the palliation of malignant esophageal stenosis. Endoscopy 1990;22:101-4. 7. Buset M, des Marez B, Baize M, et al. Palliative endoscopic management of obstructive esophagogastric cancer: laser or prosthesis? Gastrointest Endosc 1987;33:357-61. 8. Adam A, Chetty N, Roddie M, Yeung E, Benjamin IS. Selfexpandable stainless steel endoprostheses for treatment of malignant bile duct obstruction. AJR 1991;156:321-5. 9. Huibregtse K, Cheng J, Coene PPLO, Fockens P, Tytgat GNJ. Endoscopic placement of expandable metal stents for biliary strictures-a preliminary report with 33 patients. Endoscopy 1989;21:280-2. 10. Domschke W, Foerster E Ch, Matek W, Rodl W. Self-expanding mesh stent for esophageal cancer stenosis. Endoscopy 1990;22:134-6. 11. Serruys PW, Strauss BH, Beatt KJ, et al. Angiographic followup after placement of a self-expanding coronary artery stent. N Engl J Med 1991;324:13-7. 12. Raillat C, Rousseau H, Joffre F, Roux D. Treatment of iliac artery stenoses with the Wallstent endoprosthesis. AJR 1990;154:613-6. 13. Adam A, Jager R, McLoughlin J, et al. Wallstent endoprostheses for the relief of prostatic urethral obstruction in high risk patients. Clin Radiol 1990;42:228-32. 14. Lammer J. Biliary endoprostheses: plastic versus metal stents. Radiol Clin North Am 1990;28:1211-22.
Endoscopic therapy of a duodenal duplication cyst
this report describes both the endoscopic diagnosis and treatment of a large peri-ampullary duodenal cyst.
John F. Johanson, MD Joseph E. Geenen, MD Walter J. Hogan, MD Kees Huibregtse, MD
Submucosal duodenal cysts are distinctly uncommon and most often present in infancy or early childhood. 1 The clinical presentation is generally duodenal obstruction although abdominal pain, jaundice, hemorrhage, or pancreatitis may occur. 2 Duodenal cysts are almost exclusively treated by operation. However, From the Division of Gastroenterology, Medical College of Wisconsin, Milwaukee, Wisconsin, St. Luke's Hospital, Racine, Wisconsin, and Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands. Reprint requests: John F. Johanson, MD, 401 Roxbury Road, Rockford, Illinois 61107-5078.
60
CASE REPORT
A 62-year-old man was referred to the Medical College of Wisconsin for evaluation of epigastric abdominal pain and a peri-ampullary mass. The patient's initial episode of pain occurred 3 years previously. The pain was sharp, epigastric in location without radiation, and lasted for approximately 10 min. It was described as "the most severe pain" he had ever experienced. Due to the severity of this pain, he was brought by ambulance to the emergency room and was subsequently hospitalized. Admission amylase and liver tests including total bilirubin, alkaline phosphatase, and AST were normal. A CT scan of the abdomen was unremarkable. After 4 days, he was discharged without a specific diagnosis. Three years later the patient experienced a similar attack of severe epigastric pain which again necessitated hospitalization. Admission amylase, lipase, and liver tests were normal. GASTROINTESTINAL ENDOSCOPY
An ultrasound of the gallbladder revealed no evidence of cholelithiasis or choledocholithiasis, although the common bile duct was slightly dilated at 7.5 mm. An upper gastrointestinal x-ray and repeat CT scan both demonstrated a mass located at the junction of the second and third portion of the duodenum (Figs. 1 and 2). Subsequent esophagogastroduodenoscopy identified a 4- X 5-cm peri-ampullary mass and ERCP was performed. ERCP confirmed the presence of a large peri-ampullary mass which was soft and compressible (Fig. 3). The papilla was easily identified and cannulated. Injection of contrast revealed a long common channel. The common bile duct appeared to be normal while the pancreatic duct was slightly dilated. Both ducts appeared to drain appropriately. The peri-ampullary mass was found to be cystic and communicated with the common channel via a small opening adjacent to the papilla (Fig. 4). Although an endoscopic sphincterotomy was considered, it was not performed because of the unclear relationship between the cyst and the patient's
Figure 3. Multiple endoscopic views of the duodenal cyst demonstrating the ectopic papillary orifice (arrow).
Figure 1. Upper gastrointestinal radiograph demonstrating a duodenal mass (arrow) located at the junction of the second and third portions.
symptoms. Four hours after ERCP, however, the patient developed severe epigastric abdominal pain identical to the pain he had experienced previously. An abdominal x-ray revealed marked distention of the cyst with contrast and minimal residual contrast in the pancreatic and biliary ducts (Fig. 5). Emergent duodenoscopy confirmed the x-ray findings of cyst enlargement. In light of the patient's unrelenting abdominal pain, a 2-cm incision was made using a needle knife papillotome at the dependant portion of the cyst. Prompt deluge of contrast was observed associated with immediate resolution of pain. The incision was complicated by a small amount of bleeding which was easily controlled with the submucosal injection of a 1:10,000 dilution of epinephrine. In order to better define its nature, mucosal biopsies were obtained from inside the cyst. The cyst was then flushed with normal saline and multiple stones were evacuated. A nasobiliary catheter was placed into the cyst to ensure continued drainage and to observe for recurrent' bleeding. The catheter remained in place for 48 hours draining small amounts of bile and was then removed. Subsequent histologic examination of the biopsy specimens revealed normal duodenal mucosa. Repeat ERCP 2 months later demonstrated continued patency of the incision with a marked diminution in the size of the cyst. The patient has subsequently been followed for nearly a year without symptoms. At the last endoscopy, 11 months after initial treatment, the incision was found to still be patent while a portion of the cyst remained. DISCUSSION
Figure 2. Contrast enhanced CT scan revealing a cystic duodenal mass (arrow). VOLUME 38, NO.1, 1992
The differential diagnosis of a peri-ampullary mass includes adenoma, lipoma, carcinoid, ampullary carcinoma, choledochocele, and duodenal duplication cyst. 3,4 However, the cystic nature of the mass in this patient restricts the potential etiology to either choledochocele or duodenal duplication cyst. 61
of the choledochocele differentiate these newly described variants, with drainage either through a fistula between cyst and duodenum or through a caudally positioned papilla contiguous with or separate from the pancreatic duct. The principal clinical presentations of a choledochocele are biliary colic, jaundice, or acute recurrent pancreatitis. 12 A duplication cyst is a spherical or tubular structure which possesses a smooth muscle layer, is lined by intestinal mucosa, and is attached to some portion of the alimentary tract. 13 Duodenal duplication cysts are likewise uncommon comprising only 5% of all intestinal duplications, exhibiting a prevalence rate of 1 per 100,000. 14 ,15 The majority of duodenal duplications occur in the second portion of the duodenum along the posterior medial wall. They tend to be paramesenteric, sharing a common muscular wall although the cyst has its own mucosal lining.16 Histologically, duodenal duplications are covered on both sides by duodenal mucosa. In between mucosal layers lies a well developed layer of smooth muscle with fibers running in the same or varying directions. 13,17 Anatomically, both pancreatic and common bile ducts enter into the cyst with a separate opening into the duodenum. 18
Figure 4. ERCP shows filling of the biliary and pancreatic ducts through a common channel. The peri-ampullary mass communicates with the common channel via a small opening adjacent to the papillary orifice.
A choledochocele is a form of choledochal cyst. 5,6 Since its original description in the mid-1700s, over 1500 cases of choledochal cyst have been reported. 7 Choledochoceles are distinctly uncommon comprising only 1.5% of all choledochal cystS. 8,9 A choledochocele is not a true cyst, but rather is a localized dilation of the distal bile duct. Typically this dilation is circumferential and is limited to the intramural segment of the common bile duct. lO The wall of a choledochocele generally measures 4 to 5 mm in thickness and is comprised of fibrous tissue. Its lining is variable and may consist of bile duct, gallbladder, or intestinal epithelia. 5 At least two distinct anatomical patterns have been observed. l l In one type, the common bile duct terminates into the cyst which then drains into the duodenum via a hole in its wall. In the other type, the cyst drains into the adjacent intramural portion ofthe common bile duct and out the papilla. Recently, three additional anatomical variants of choledochoceles were described based on findings from endoscopic retrograde cholangiography.8 In all three, the common bile duct terminates in the choledochocele. The location of the pancreatic duct and the opening 62
Figure 5. Abdominal radiograph demonstrating distention of the cyst with drainage of contrast from the biliary and pancreatic ducts.
GASTROINTESTINAL ENDOSCOPY
Duplication cysts are typically filled with clear fluid, although cysts which communicate with the papilla may contain bile, pancreatic juice, or even gallstones. 16 The symptoms of a duodenal duplication are usually those of obstruction; however, ulceration, bleeding, jaundice, or pancreatitis may occurY Comparison of the two entities, choledochocele and duodenal duplication, reveals more similarities than true differences. They are both distinctly uncommon, are congenital in origin, are located in the peri-ampullary region with direct communication between cyst and biliary tree, and when symptomatic may cause jaundice or pancreatitis. The principal distinguishing feature between these conditions is their histologic characteristics. The duodenal duplication is covered both inside and outside by duodenal mucosa with a distinct layer of smooth muscle between. By contrast, the choledochocele is lined by bile duct, or gallbladder mucosa and lacks the layer of smooth muscle. Although there have been reports of "choledochoceles" being lined by intestinal mucosa, it is believed that these choledochoceles in fact would be better classified as duodenal duplications. 18 ,19 Based on the identification of duodenal mucosa inside the cyst, this patient's peri-ampullary mass is most likely a duodenal duplication. Without a full-thickness biopsy demonstrating the presence of a smooth muscle layer, however, the diagnosis of duodenal duplication cannot be established unequivocally. The infrequent nature of the patient's symptoms is unusual, but still compatible with the diagnosis of duodenal duplication. Duodenal duplications may be silent for many years before causing symptoms. 20 When they do become symptomatic, they manifest a variety of clinical presentations: obstruction of the duodenum secondary to the presence of the cyst in the intestinal lumen, necrosis and bleeding of the adjacent intestine due to pressure upon the mesenteric blood vessels, hemorrhage from the duplication cyst itself (approximately 15% may be lined by gastric mucosa), acute pancreatitis caused by obstruction of the pancreatic duct, or pain related to distention of the cystic mass. 14 In the case presented here, the development of severe abdominal pain after initial ERCP and subsequent dilation of the cyst suggest that the patient's pain was the result of distention of the duplication. This is corroborated by the similarity of the pain to his previous attacks both in quality and intensity. Furthermore, upon incision and drainage of the cyst, abrupt relief of pain was achieved and the patient has remained asymptomatic for nearly 1 year. The sporadic nature of the patient's symptoms, however, makes it difficult to definitively establish the duplication cyst as the etiology of the pain. The primary mode of treatment of duodenal duplications has traditionally been operative intervention. 16,17 Treatment should be tailored to the anatomical relationships between the cyst, blood vessels, and VOLUME 38, NO.1, 1992
pancreatic and biliary ducts. 14 Previously, complete resection of the duplication was recommended with marsupialization reserved for cysts in which complete excision was not possible. Since duodenal duplications rarely contain gastric mucosa the risk of ulceration is low and complete excision is now thought to be unnecessary.14,16 Furthermore, complete excision may result in injury to the common bile or pancreatic ducts. 18 Consequently, simple drainage by removal of a portion of the cyst wall with formation of a window between the cyst and adjacent duodenum has become an accepted mode of treatment. This window should be located in the dependent portion of the duplication and be of ample size to allow adequate drainage particularly if the duplication contains stones or other debris. 13 ,14 Before the exact nature of the treatment can be determined, delineation of the pancreatic and biliary ducts is necessary. This is best accomplished via retrograde cholangiopancreatography. It would seem, therefore, that both eludication of anatomical relationships as well as definitive treatment of the cyst could be performed during the same procedure. Although complete excision would not be possible endoscopically' adequate cyst drainage could be accomplished safely. The potential role of endoscopic therapy in the treatment of duodenal duplication cysts is further supported by recent reports describing its successful use in the treatment of choledochocele. 8,21 Because of its relative ease and safety, endoscopic drainage of duodenal duplication cysts may provide a reasonable therapeutic alternative to surgical intervention. REFERENCES 1. Thompson NW, Labow SS. Duplication of the duodenum in the adult, Arch Surg 1967;94:301-6. 2. Soper RT, Selke AC. Duplication cyst of the duodenum: case report and discussion. Surgery 1970;68:562-6. 3. Jordan GL. Benign and malignant tumors of the pancreas and periampullary region. In Howard JM, Jordan GL, eds. Surgical diseases of the pancreas, Philadelphia: JB Lippincott, 1960:449532. 4. Classen M, Phillip J. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic therapy in pancreatic disease. Clin Gastroenterol 1984;13:819-42. 5, Serfas LS, Lyter CS, Choledochal cyst, Am J Surg 1957;93:97989, 6, Stephens Fa, Pauline GJ. Choledochocele: an unusual type of choledochal cyst which presented as acute pancreatitis. Aust N Z J Surg 1960:124-7, 7, Weidmeyer DA, Stewart ET, Dodds WJ, et at. Choledochal cyst: findings on cholangiopancreatography with emphasis on ectasia of the common channel. AJR 1989;153:969-72. 8. Kagiyama S, Okazaki K, Yamamoto Y, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J Gastroenterol 1987;82:641-9. 9. Crittenden SL, McKinley MJ. Choledochal cyst: clinical features and classification. Am J GastroenteroI1985;80:643-7. 10. Oldham KT, Hart MJ, White TT. Choledochal cysts presenting in late childhood and adulthood. Am J Surg 1981;141:568-71. 11. Scholz FJ, Carrera GF, Larsen CR. The choledochocele: correlation of radiological, clinical and pathologic findings. Radiology 1976;118:25-8. 12. Venu RP, Geenen JE, Hogan WJ, et al. Role of endoscopic
63
13. 14. 15. 16.
retrograde cholangiopancreatography in the diagnosis and treatment of choledochocele. Gastroenterology 1984;87:1144-9. Leffall LS, Jackson M, Press H, Syphax B. Duplication cyst of the duodenum. Arch Surg 1967;94:30-4. Soper RT, Selke AC. Duplication cyst of the duodenum: case report and discussion. Surgery 1970;68:562-6. Potter EL. Pathology of the fetus and the infant. Chicago: Year Book Medical Publishers, 1961. Holcomb GW, Gheissari A, O'Neil JA, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167-74.
17. Gordimer H, Bluestone L. Enterogenous cyst of the duodenum. Ann Surg 1950;132:1149-53. 18. Warren KW, Mountain JC, Erickson EE. Duodenal duplication cyst: a case report in which the pancreaticobiliary system drained directly into the cyst. Lahey Clin Found Bull 1972;20:114-21. 19. Reinus FZ, Weingarten G. Choledochocele of the common bile duct. Am J Surg 1976;132:646-8. 20. Browning RW. Duodenal duplications. Rev Surg 1963;226-9. 21. Siegel JH, Harding GT, Chateau F. Endoscopic incision of choledochal cysts (choledochocele). Endoscopy 1981;13:200-2.
Endoscopic drainage of a duodenal duplication cyst Ibrahim AI Traif MD M. H. Khan, MD
We endoscopically drained a duodenal duplication cyst in an ll-year-old male who had recurrent attacks of pancreatitis. CASE REPORT
An ll-year-old Saudi male was admitted in March 1986 to our hospital with severe sudden epigastric pain and vomiting after being hit in the mid-abdomen while playing. There was no previous history of abdominal pain, hematemesis, melena, or diarrhea. There was no history of drug intake or significant past medical or surgical history. Physical examination revealed a well-built child, in pain with a temperature of 38.6°C. Epigastric tenderness, fullness, and guarding were noted with normal bowel sounds. WBC count was 14,000 with normal differential. Admission amylase was 766 IV (normal, 23 to 85). Liver enzymes, calcium, triglycerides, cholesterol and plain abdominal x-ray were normal. The next day, he improved and his amylase fell to 345 IV. He was signed out against medical advice by his father. He was readmitted in May 1987 again with epigastric pain, guarding, epigastric tenderness, and abdominal fullness. Amylase was 727 IV and the next day decreased to 327 IV. Real time ultrasound showed a peristaltic wave passing through a cystic structure, making it highly likely to be a duplication cyst. CT of the abdomen and barium meal revealed that this structure was in the C loop of the duodenum, compressing the head of the pancreas, and stretching the second and third portion of the duodenum. The child improved and was discharged because of school examinations. He was given an appointment for readmission, but was lost to follow-up. In March 1988 he presented to the emergency room with epigastric pain for the third time, and was admitted with an amylase of 160 IV, which normalized quickly. Repeat CT of the abdomen showed no change in the cyst since the previous examination 10 months earlier. The cyst was easily accessible for endoscopic drainage, as From the Department of Medicine, Gw;troenterology Division of King Fahad National Guard Hospital, Riyadh, Saudi Arabia. Reprint requests: Ibrahim Al Trail, MD, 519 Newton Research Bldg., University of Alberta, Edmonton, Alberta, Canada T6G 2C2. Dr. Al Traif is currently working w; a Gw;trointestinal Fellow at the University of Alberta.
64
Figure 1. Endoscopic view showing bulging mass stretching the mucosa of the second part of the duodenum. Figure 2. Endoscopic view from the same angle as in Figure 1 showing partial collapse of the cyst after an opening has been made through it with a needle knife sphincterotome. Figure 3. Endoscopic view of the duodenum 2 years after, drainage showing redundant mucosal folds in place of the previously located cyst. Wide cystoduodenostomy opening is clearly seen. GASTROINTESTINAL ENDOSCOPY
than $900, but their use in our patient enabled his discharge from the hospital, and probably lowered the total cost of his medical care as a result. Complications may occur when the Wallstent is used to palliate malignant biliary strictures, and luminal use of the device could be accompanied by similar complications. Stent obstruction can be caused by ingrowth of tumor through the interstices of the stainless steel mesh, by overgrowth of tumor around the ends of the stent, or by vegetable fiber clogging the lumen. Wallstent dislocation and hemobilia have occurred infrequently.14 The stents can only be removed surgically. The possibility of perforation is a concern when using stents in the gastrointestinal tract, although Wallstent perforations of the biliary tree or the duodenum opposite the papilla have not been reported. Further investigation into the stent's effect on the bowel is warranted, with design modifications if needed. The effect of the Wallstent on the jejunal wall in our patient is unknown. Because the Wallstent shortens during deployment, care must be taken to ensure that the shortened stent will adequately bridge the stricture. Our patient required the placement of two stents, one partially nested inside the other, to stent his entire occluded segment of jejunum. Although the inner diameter of the Wallstent is somewhat less than that of most commercial rigid esophageal endoprostheses (10 mm VS. 12 mm), palliation of our patient's symptoms was obtained for the short duration of his remaining life. Wallstent endoprosthesis placement in the intestinallumen may join the array of palliative techniques available to patients with incurable, inoperable malignant gastrointestinal obstruction.
1. Lope M, Natali M, DiLauro L, et al. 5-Fluorouracil, epirubicin, and BCND in advanced measurable gastric cancer. Am J Clin Oncol 1990;13:204-7. 2. Elliot TE, Moertel CG, Wieand HS, et al. A phase II study of the combination of etoposide and cisplatin in the therapy of advanced gastric cancer. Cancer 1990;65:1491-4. 3. Eckhauser ML. The neodynium-YAG laser and gastrointestinal malignancy. Arch Surg 1990;125:1152-4. 4. Suzuki H, Miho 0, Watanabe Y, Kohyama M, Nagao F. Endoscopic laser therapy in the curative and palliative treatment of upper gastrointestinal cancer. World J Surg 1989;13:158-64. 5. Schwegler D, Orth M, Kuntz HD, May B. Endoscopic palliative therapy of malignant esophageal and cardia stenoses. Results, complications, follow-up. Bildgebung 1987-1989;56:69-75. 6. Fugger R, Niederle B, Jantsch H, Schiessel R, Schulz F. Endoscopic tube implantation for the palliation of malignant esophageal stenosis. Endoscopy 1990;22:101-4. 7. Buset M, des Marez B, Baize M, et al. Palliative endoscopic management of obstructive esophagogastric cancer: laser or prosthesis? Gastrointest Endosc 1987;33:357-61. 8. Adam A, Chetty N, Roddie M, Yeung E, Benjamin IS. Selfexpandable stainless steel endoprostheses for treatment of malignant bile duct obstruction. AJR 1991;156:321-5. 9. Huibregtse K, Cheng J, Coene PPLO, Fockens P, Tytgat GNJ. Endoscopic placement of expandable metal stents for biliary strictures-a preliminary report with 33 patients. Endoscopy 1989;21:280-2. 10. Domschke W, Foerster E Ch, Matek W, Rodl W. Self-expanding mesh stent for esophageal cancer stenosis. Endoscopy 1990;22:134-6. 11. Serruys PW, Strauss BH, Beatt KJ, et al. Angiographic followup after placement of a self-expanding coronary artery stent. N Engl J Med 1991;324:13-7. 12. Raillat C, Rousseau H, Joffre F, Roux D. Treatment of iliac artery stenoses with the Wallstent endoprosthesis. AJR 1990;154:613-6. 13. Adam A, Jager R, McLoughlin J, et al. Wallstent endoprostheses for the relief of prostatic urethral obstruction in high risk patients. Clin Radiol 1990;42:228-32. 14. Lammer J. Biliary endoprostheses: plastic versus metal stents. Radiol Clin North Am 1990;28:1211-22.
Endoscopic therapy of a duodenal duplication cyst
this report describes both the endoscopic diagnosis and treatment of a large peri-ampullary duodenal cyst.
John F. Johanson, MD Joseph E. Geenen, MD Walter J. Hogan, MD Kees Huibregtse, MD
Submucosal duodenal cysts are distinctly uncommon and most often present in infancy or early childhood. 1 The clinical presentation is generally duodenal obstruction although abdominal pain, jaundice, hemorrhage, or pancreatitis may occur. 2 Duodenal cysts are almost exclusively treated by operation. However, From the Division of Gastroenterology, Medical College of Wisconsin, Milwaukee, Wisconsin, St. Luke's Hospital, Racine, Wisconsin, and Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands. Reprint requests: John F. Johanson, MD, 401 Roxbury Road, Rockford, Illinois 61107-5078.
60
CASE REPORT
A 62-year-old man was referred to the Medical College of Wisconsin for evaluation of epigastric abdominal pain and a peri-ampullary mass. The patient's initial episode of pain occurred 3 years previously. The pain was sharp, epigastric in location without radiation, and lasted for approximately 10 min. It was described as "the most severe pain" he had ever experienced. Due to the severity of this pain, he was brought by ambulance to the emergency room and was subsequently hospitalized. Admission amylase and liver tests including total bilirubin, alkaline phosphatase, and AST were normal. A CT scan of the abdomen was unremarkable. After 4 days, he was discharged without a specific diagnosis. Three years later the patient experienced a similar attack of severe epigastric pain which again necessitated hospitalization. Admission amylase, lipase, and liver tests were normal. GASTROINTESTINAL ENDOSCOPY
An ultrasound of the gallbladder revealed no evidence of cholelithiasis or choledocholithiasis, although the common bile duct was slightly dilated at 7.5 mm. An upper gastrointestinal x-ray and repeat CT scan both demonstrated a mass located at the junction of the second and third portion of the duodenum (Figs. 1 and 2). Subsequent esophagogastroduodenoscopy identified a 4- X 5-cm peri-ampullary mass and ERCP was performed. ERCP confirmed the presence of a large peri-ampullary mass which was soft and compressible (Fig. 3). The papilla was easily identified and cannulated. Injection of contrast revealed a long common channel. The common bile duct appeared to be normal while the pancreatic duct was slightly dilated. Both ducts appeared to drain appropriately. The peri-ampullary mass was found to be cystic and communicated with the common channel via a small opening adjacent to the papilla (Fig. 4). Although an endoscopic sphincterotomy was considered, it was not performed because of the unclear relationship between the cyst and the patient's
Figure 3. Multiple endoscopic views of the duodenal cyst demonstrating the ectopic papillary orifice (arrow).
Figure 1. Upper gastrointestinal radiograph demonstrating a duodenal mass (arrow) located at the junction of the second and third portions.
symptoms. Four hours after ERCP, however, the patient developed severe epigastric abdominal pain identical to the pain he had experienced previously. An abdominal x-ray revealed marked distention of the cyst with contrast and minimal residual contrast in the pancreatic and biliary ducts (Fig. 5). Emergent duodenoscopy confirmed the x-ray findings of cyst enlargement. In light of the patient's unrelenting abdominal pain, a 2-cm incision was made using a needle knife papillotome at the dependant portion of the cyst. Prompt deluge of contrast was observed associated with immediate resolution of pain. The incision was complicated by a small amount of bleeding which was easily controlled with the submucosal injection of a 1:10,000 dilution of epinephrine. In order to better define its nature, mucosal biopsies were obtained from inside the cyst. The cyst was then flushed with normal saline and multiple stones were evacuated. A nasobiliary catheter was placed into the cyst to ensure continued drainage and to observe for recurrent' bleeding. The catheter remained in place for 48 hours draining small amounts of bile and was then removed. Subsequent histologic examination of the biopsy specimens revealed normal duodenal mucosa. Repeat ERCP 2 months later demonstrated continued patency of the incision with a marked diminution in the size of the cyst. The patient has subsequently been followed for nearly a year without symptoms. At the last endoscopy, 11 months after initial treatment, the incision was found to still be patent while a portion of the cyst remained. DISCUSSION
Figure 2. Contrast enhanced CT scan revealing a cystic duodenal mass (arrow). VOLUME 38, NO.1, 1992
The differential diagnosis of a peri-ampullary mass includes adenoma, lipoma, carcinoid, ampullary carcinoma, choledochocele, and duodenal duplication cyst. 3,4 However, the cystic nature of the mass in this patient restricts the potential etiology to either choledochocele or duodenal duplication cyst. 61
of the choledochocele differentiate these newly described variants, with drainage either through a fistula between cyst and duodenum or through a caudally positioned papilla contiguous with or separate from the pancreatic duct. The principal clinical presentations of a choledochocele are biliary colic, jaundice, or acute recurrent pancreatitis. 12 A duplication cyst is a spherical or tubular structure which possesses a smooth muscle layer, is lined by intestinal mucosa, and is attached to some portion of the alimentary tract. 13 Duodenal duplication cysts are likewise uncommon comprising only 5% of all intestinal duplications, exhibiting a prevalence rate of 1 per 100,000. 14 ,15 The majority of duodenal duplications occur in the second portion of the duodenum along the posterior medial wall. They tend to be paramesenteric, sharing a common muscular wall although the cyst has its own mucosal lining.16 Histologically, duodenal duplications are covered on both sides by duodenal mucosa. In between mucosal layers lies a well developed layer of smooth muscle with fibers running in the same or varying directions. 13,17 Anatomically, both pancreatic and common bile ducts enter into the cyst with a separate opening into the duodenum. 18
Figure 4. ERCP shows filling of the biliary and pancreatic ducts through a common channel. The peri-ampullary mass communicates with the common channel via a small opening adjacent to the papillary orifice.
A choledochocele is a form of choledochal cyst. 5,6 Since its original description in the mid-1700s, over 1500 cases of choledochal cyst have been reported. 7 Choledochoceles are distinctly uncommon comprising only 1.5% of all choledochal cystS. 8,9 A choledochocele is not a true cyst, but rather is a localized dilation of the distal bile duct. Typically this dilation is circumferential and is limited to the intramural segment of the common bile duct. lO The wall of a choledochocele generally measures 4 to 5 mm in thickness and is comprised of fibrous tissue. Its lining is variable and may consist of bile duct, gallbladder, or intestinal epithelia. 5 At least two distinct anatomical patterns have been observed. l l In one type, the common bile duct terminates into the cyst which then drains into the duodenum via a hole in its wall. In the other type, the cyst drains into the adjacent intramural portion ofthe common bile duct and out the papilla. Recently, three additional anatomical variants of choledochoceles were described based on findings from endoscopic retrograde cholangiography.8 In all three, the common bile duct terminates in the choledochocele. The location of the pancreatic duct and the opening 62
Figure 5. Abdominal radiograph demonstrating distention of the cyst with drainage of contrast from the biliary and pancreatic ducts.
GASTROINTESTINAL ENDOSCOPY
Duplication cysts are typically filled with clear fluid, although cysts which communicate with the papilla may contain bile, pancreatic juice, or even gallstones. 16 The symptoms of a duodenal duplication are usually those of obstruction; however, ulceration, bleeding, jaundice, or pancreatitis may occurY Comparison of the two entities, choledochocele and duodenal duplication, reveals more similarities than true differences. They are both distinctly uncommon, are congenital in origin, are located in the peri-ampullary region with direct communication between cyst and biliary tree, and when symptomatic may cause jaundice or pancreatitis. The principal distinguishing feature between these conditions is their histologic characteristics. The duodenal duplication is covered both inside and outside by duodenal mucosa with a distinct layer of smooth muscle between. By contrast, the choledochocele is lined by bile duct, or gallbladder mucosa and lacks the layer of smooth muscle. Although there have been reports of "choledochoceles" being lined by intestinal mucosa, it is believed that these choledochoceles in fact would be better classified as duodenal duplications. 18 ,19 Based on the identification of duodenal mucosa inside the cyst, this patient's peri-ampullary mass is most likely a duodenal duplication. Without a full-thickness biopsy demonstrating the presence of a smooth muscle layer, however, the diagnosis of duodenal duplication cannot be established unequivocally. The infrequent nature of the patient's symptoms is unusual, but still compatible with the diagnosis of duodenal duplication. Duodenal duplications may be silent for many years before causing symptoms. 20 When they do become symptomatic, they manifest a variety of clinical presentations: obstruction of the duodenum secondary to the presence of the cyst in the intestinal lumen, necrosis and bleeding of the adjacent intestine due to pressure upon the mesenteric blood vessels, hemorrhage from the duplication cyst itself (approximately 15% may be lined by gastric mucosa), acute pancreatitis caused by obstruction of the pancreatic duct, or pain related to distention of the cystic mass. 14 In the case presented here, the development of severe abdominal pain after initial ERCP and subsequent dilation of the cyst suggest that the patient's pain was the result of distention of the duplication. This is corroborated by the similarity of the pain to his previous attacks both in quality and intensity. Furthermore, upon incision and drainage of the cyst, abrupt relief of pain was achieved and the patient has remained asymptomatic for nearly 1 year. The sporadic nature of the patient's symptoms, however, makes it difficult to definitively establish the duplication cyst as the etiology of the pain. The primary mode of treatment of duodenal duplications has traditionally been operative intervention. 16,17 Treatment should be tailored to the anatomical relationships between the cyst, blood vessels, and VOLUME 38, NO.1, 1992
pancreatic and biliary ducts. 14 Previously, complete resection of the duplication was recommended with marsupialization reserved for cysts in which complete excision was not possible. Since duodenal duplications rarely contain gastric mucosa the risk of ulceration is low and complete excision is now thought to be unnecessary.14,16 Furthermore, complete excision may result in injury to the common bile or pancreatic ducts. 18 Consequently, simple drainage by removal of a portion of the cyst wall with formation of a window between the cyst and adjacent duodenum has become an accepted mode of treatment. This window should be located in the dependent portion of the duplication and be of ample size to allow adequate drainage particularly if the duplication contains stones or other debris. 13 ,14 Before the exact nature of the treatment can be determined, delineation of the pancreatic and biliary ducts is necessary. This is best accomplished via retrograde cholangiopancreatography. It would seem, therefore, that both eludication of anatomical relationships as well as definitive treatment of the cyst could be performed during the same procedure. Although complete excision would not be possible endoscopically' adequate cyst drainage could be accomplished safely. The potential role of endoscopic therapy in the treatment of duodenal duplication cysts is further supported by recent reports describing its successful use in the treatment of choledochocele. 8,21 Because of its relative ease and safety, endoscopic drainage of duodenal duplication cysts may provide a reasonable therapeutic alternative to surgical intervention. REFERENCES 1. Thompson NW, Labow SS. Duplication of the duodenum in the adult, Arch Surg 1967;94:301-6. 2. Soper RT, Selke AC. Duplication cyst of the duodenum: case report and discussion. Surgery 1970;68:562-6. 3. Jordan GL. Benign and malignant tumors of the pancreas and periampullary region. In Howard JM, Jordan GL, eds. Surgical diseases of the pancreas, Philadelphia: JB Lippincott, 1960:449532. 4. Classen M, Phillip J. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic therapy in pancreatic disease. Clin Gastroenterol 1984;13:819-42. 5, Serfas LS, Lyter CS, Choledochal cyst, Am J Surg 1957;93:97989, 6, Stephens Fa, Pauline GJ. Choledochocele: an unusual type of choledochal cyst which presented as acute pancreatitis. Aust N Z J Surg 1960:124-7, 7, Weidmeyer DA, Stewart ET, Dodds WJ, et at. Choledochal cyst: findings on cholangiopancreatography with emphasis on ectasia of the common channel. AJR 1989;153:969-72. 8. Kagiyama S, Okazaki K, Yamamoto Y, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J Gastroenterol 1987;82:641-9. 9. Crittenden SL, McKinley MJ. Choledochal cyst: clinical features and classification. Am J GastroenteroI1985;80:643-7. 10. Oldham KT, Hart MJ, White TT. Choledochal cysts presenting in late childhood and adulthood. Am J Surg 1981;141:568-71. 11. Scholz FJ, Carrera GF, Larsen CR. The choledochocele: correlation of radiological, clinical and pathologic findings. Radiology 1976;118:25-8. 12. Venu RP, Geenen JE, Hogan WJ, et al. Role of endoscopic
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13. 14. 15. 16.
retrograde cholangiopancreatography in the diagnosis and treatment of choledochocele. Gastroenterology 1984;87:1144-9. Leffall LS, Jackson M, Press H, Syphax B. Duplication cyst of the duodenum. Arch Surg 1967;94:30-4. Soper RT, Selke AC. Duplication cyst of the duodenum: case report and discussion. Surgery 1970;68:562-6. Potter EL. Pathology of the fetus and the infant. Chicago: Year Book Medical Publishers, 1961. Holcomb GW, Gheissari A, O'Neil JA, Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167-74.
17. Gordimer H, Bluestone L. Enterogenous cyst of the duodenum. Ann Surg 1950;132:1149-53. 18. Warren KW, Mountain JC, Erickson EE. Duodenal duplication cyst: a case report in which the pancreaticobiliary system drained directly into the cyst. Lahey Clin Found Bull 1972;20:114-21. 19. Reinus FZ, Weingarten G. Choledochocele of the common bile duct. Am J Surg 1976;132:646-8. 20. Browning RW. Duodenal duplications. Rev Surg 1963;226-9. 21. Siegel JH, Harding GT, Chateau F. Endoscopic incision of choledochal cysts (choledochocele). Endoscopy 1981;13:200-2.
Endoscopic drainage of a duodenal duplication cyst Ibrahim AI Traif MD M. H. Khan, MD
We endoscopically drained a duodenal duplication cyst in an ll-year-old male who had recurrent attacks of pancreatitis. CASE REPORT
An ll-year-old Saudi male was admitted in March 1986 to our hospital with severe sudden epigastric pain and vomiting after being hit in the mid-abdomen while playing. There was no previous history of abdominal pain, hematemesis, melena, or diarrhea. There was no history of drug intake or significant past medical or surgical history. Physical examination revealed a well-built child, in pain with a temperature of 38.6°C. Epigastric tenderness, fullness, and guarding were noted with normal bowel sounds. WBC count was 14,000 with normal differential. Admission amylase was 766 IV (normal, 23 to 85). Liver enzymes, calcium, triglycerides, cholesterol and plain abdominal x-ray were normal. The next day, he improved and his amylase fell to 345 IV. He was signed out against medical advice by his father. He was readmitted in May 1987 again with epigastric pain, guarding, epigastric tenderness, and abdominal fullness. Amylase was 727 IV and the next day decreased to 327 IV. Real time ultrasound showed a peristaltic wave passing through a cystic structure, making it highly likely to be a duplication cyst. CT of the abdomen and barium meal revealed that this structure was in the C loop of the duodenum, compressing the head of the pancreas, and stretching the second and third portion of the duodenum. The child improved and was discharged because of school examinations. He was given an appointment for readmission, but was lost to follow-up. In March 1988 he presented to the emergency room with epigastric pain for the third time, and was admitted with an amylase of 160 IV, which normalized quickly. Repeat CT of the abdomen showed no change in the cyst since the previous examination 10 months earlier. The cyst was easily accessible for endoscopic drainage, as From the Department of Medicine, Gw;troenterology Division of King Fahad National Guard Hospital, Riyadh, Saudi Arabia. Reprint requests: Ibrahim Al Trail, MD, 519 Newton Research Bldg., University of Alberta, Edmonton, Alberta, Canada T6G 2C2. Dr. Al Traif is currently working w; a Gw;trointestinal Fellow at the University of Alberta.
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Figure 1. Endoscopic view showing bulging mass stretching the mucosa of the second part of the duodenum. Figure 2. Endoscopic view from the same angle as in Figure 1 showing partial collapse of the cyst after an opening has been made through it with a needle knife sphincterotome. Figure 3. Endoscopic view of the duodenum 2 years after, drainage showing redundant mucosal folds in place of the previously located cyst. Wide cystoduodenostomy opening is clearly seen. GASTROINTESTINAL ENDOSCOPY