- Email: [email protected]
Bleeding downhill esophageal varices: a complication of upper extremity hemodialysis access
Bleeding downhill esophageal varices
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tected after endoscopic injection sclerotherapy. Prog Digest Endosc 1989;34:240-3. Kokudo N, Sanjo K, Umekita N, Harihara Y, Tada Y, Idezuki Y. Squamous cell carcinoma after endoscopic injection sclerotherapy for esophageal varices. Am J Gastroenterol 1990; 85:861-4. Sato T, Watanabe M, Natsui K, Miyakawa H, Koito K, Yaosaka T, et al. Two cases of esophageal carcinoma developing after endoscopic injection sclerotherapy for esophageal varices. Clin Gastroenterol 1990;2:801-6. Claudel-Bonvoisin S, Descos L, Chatelard P, Souquet JC, Pasquier J. Cancer de l’oesophage et traitement sclerosant endoscopique de varices oesophagiennes. Gastroenterol Clin Biol 1990;14:189. Tanaka S, Yamamoto S, Senoo T, Nakamura M, Hirasawa S, Araki M, et al. A case of multiple esophageal cancers detected during following endoscopic injection sclerotherapy for esophageal varices. Gastroenterol Endosc 1992;34:402-7. Terada M, Ogino H, Adachi K, Nakazawa H, Hirose S, Miwa A. A case of triple cancer of the stomach, lung and esophagus after endoscopic injection sclerotherapy for the esophageal varices. Gastroenterol Endosc 1992;34:2347-53. Rodriguez M, de la Cruz S, Arrabal I, Herrera M. Esophageal carcinoma following endoscopic injection sclerotherapy. Rev Esp Enf Digest 1992;82:43-6. Tada M, Shimada M, Murakami F, Mizumachi M, Arima K, Yanai H, et al. Development of the strip-off biopsy. Gastroenterol Endosc 1984;26:833-9. Salaman MH, Glendenning OM. Tumor promotion in mouse skin by sclerosing agents. Br J Cancer 1957;11:434-44. Moon MR, Schulte WJ, Haaslet GB, Condon RE. Transhiatal
Bleeding downhill esophageal varices: a complication of upper extremity hemodialysis access Anca Pop, MD Alan F. Cutler, MD
Esophageal varices are a known complication of portal hypertension and primarily involve the esophagogastric junction. They are a result of hepatofugal blood flow. Proximal esophageal varices, often termed “downhill” varices,1 have been described sporadically and attributed mainly to superior vena cava obstruction with reversal of the normal blood flow direction in the upper esophageal plexus. We describe here the first case of bleeding downhill esophageal varices occurring as an iatrogenic complication of venous access for hemodialysis. From the Section of Gastroenterology, Sinai Hospital, Detroit, Michigan. Reprint requests: Alan F. Cutler, MD, Director, Gastroenterology Research, Section of Gastroenterology, Sinai Hospital, 6767 West Outer Dr., Detroit, MI 48235. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 1 0 37/4/87351 VOLUME 47, NO. 3, 1998
29.
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and transthoracic esophagectomy for adenocarcinoma of the esophagus. Arch Surg 1992;127:951-5. Ogle SJ, Kirk CJC, Bailey RJ, Johnson AG, Williams R, Murray-Lyon IM. Oesophageal function in cirrhotic patients undergoing injection sclerotherapy for oesophageal varices. Digestion 1978;18:178-85. Krevsky B. Tumors of the esophagus. In: Berk JE, editor. Bockus’ gastroenterology. Vol. 1. 5th ed. Philadelphia: WB Saunders; 1995. p. 534-57. Bombi JA, Rivemla A, Bordas JM, Cardesa A. Adenosquamous carcinoma of the esophagus. A case report. Path Res Pract 1991;187:514-9. Akagi T, Sakata Y, Takemori H, Osanai S. A case of advanced esophageal cancer showing good partial response by combination therapy of low dose 5-FU and low dose CDDP. Gan To Kagaku Ryoho 1996;23:103-6. Vigneswaran WT, Trastek VF, Pairolero PC, DeSchamps C, Daly RC, Allen MS. Transhiatal esophagectomy for carcinoma of the esophagus. Ann Thorac Surg 1993;56:838-46. Orsatti G, Corvalan AH, Sakurai H, Choi HS. Polypoid adenosquamous carcinoma of the esophagus with prominent spindle cells. Report of a case with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med 1993;117: 544-7. Nezu K, Kato H, Tachimori Y, Watanabe H. A clinicopathologic study of 11 adenosquamous carcinoma of the esophagus. Nippon Shoukaki Geka Gakkai Zasshi 1991;24:1-8. Suzuki H, Nagayo T. Primary tumors of the esophagus other than squamous cell carcinoma; histologic classification and statistics in the surgical and autopsied materials in Japan. Int Adv Surg Oncol 1980;3:73-109.
CASE REPORT A 52-year-old woman presented with dyspnea, orthopnea, cough, and extreme weakness. She also complained of blurred vision and bilateral tinnitus. Medical history was significant for hypertension, diabetes mellitus, peripheral vascular disease, and dialysis-dependent chronic renal failure. She also had a history of recurrent arteriovenous shunt occlusion, and on several previous occasions had required temporary placement of a hemodialysis access catheter. Six months before the present admission, she was diagnosed with superior vena cava syndrome secondary to thrombotic occlusion of the superior vena cava. At that time a bilateral arm venogram was performed that demonstrated patency of arm veins on the right and occlusion of brachial veins on the left. There was patency of the cephalic vein and numerous collaterals in the upper arm. The subclavian vein was only partially patent. The distal superior vena cava was patent from the level of the left innominate vein to the right atrium. A short occlusion of the distal right innominate vein and proximal superior vena cava was noted, with numerous collaterals present and extending into the azygous and hemiazygous system (Fig. 1). Attempts were made to improve flow in the superior vena cava. Despite efforts with multiple different guidewire and catheter combinations, the superior vena cava obstruction could not be crossed, thereby preventing placement of a stent or performance of balloon angioplasty. It was believed that urokinase or other direct anticoagulation would not be of benefit. The patient subGASTROINTESTINAL ENDOSCOPY 299
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Figure 2. Endoscopic photograph taken 25 cm from incisors demonstrating mucosal defect (open arrowhead) overlying “downhill” varices.
Figure 1. Bilateral arm venogram demonstrating occlusion of proximal superior vena cava (black arrow) with azygous and hemiazygous systems collaterals.
sequently underwent placement of a new arteriovenous shunt at the level of her right thigh. At the present admission, physical examination revealed extreme facial, neck, and upper extremity edema. Jugular venous distension was present. The patient was dyspneic, and on auscultation she had bilateral rhonchi and distant heart sounds. Examination of the right thigh showed the presence of a functioning arteriovenous shunt. A previous shunt was also identifi ed in the left forearm. The admission laboratory values included the following: hemoglobin 6.7 mg/dL, hematocrit 33%, prothrombin time 14.1 seconds, partial thromboplastin time 27 seconds, platelet count 159,000, blood urea nitrogen 76 mg/dL, and creatinine 15 mg/dL. On the day after admission the patient developed massive hematemesis. She became hypotensive, requiring institution of pressor support and transfer to the medical intensive care unit. After hemodynamic stabilization she was taken to the operating room. Tracheal intubation was undertaken, and gastric lavage was performed. Endoscopy was then accomplished under controlled conditions. At endoscopy the presence of grade two esophageal varices was noted, confi ned to the proximal to mid-esophagus and extending from 20 to 32 cm from the incisors. The presence of a mucosal defect was noted overlying one of the varices (Fig. 2). There was no active bleeding at the time of endoscopy. The distal esophagus (Fig. 3), stomach, duodenal bulb, and second portion of the duodenum were free of mucosal lesions (Fig. 3). The patient was transferred back to the intensive care unit for monitoring, 300 GASTROINTESTINAL ENDOSCOPY
Figure 3. Endoscopic photograph of distal esophagus without apparent varices. awaiting further management decisions. A total of seven units of packed red blood cells were transfused. No interventions were performed at endoscopy. The patient was managed conservatively for the next 3 months during which she complained of severe headaches and facial edema. There were intermittent deteriorations in the patient’s mental status believed to be secondary to cerebral edema. Surgical management was therefore offered. She underwent a vascular Gore-Tex graft bypass with an end-to-side anastomosis to the right internal jugular vein and an end-to-end anastomosis to the right atrial appendage. Clinical improvement was noted immediately postoperatively, with partial resolution of facial edema. The patient did not experience any further episodes of gastrointestinal blood loss in the 5 months after the surgery. VOLUME 47, NO. 3, 1998
Bleeding downhill esophageal varices
DISCUSSION By draining into the inferior thyroid, azygous, hemiazygous, and gastric veins, the veins of the esophagus form a connection between the portal and systemic venous systems. The normal venous drainage of the esophagus occurs predominantly via the azygous and hemiazygous systems. In portal hypertension, the portal blood will be delivered to the superior vena cava and “uphill” varices will result. With obstruction of the superior vena cava, blood will return to the right atrium by way of collateral pathways to the inferior vena cava. The direction of the flow being retrograde, “downhill” varices will develop. The downward extent of downhill varices depends on the duration of obstruction and therefore adequacy of collateralization,2-4 as well as on the level of superior vena cava obstruction. If the obstruction is proximal to the azygous vein, blood will return via collaterals including internal mammary, vertebral, and patent azygous veins and the downhill varices will be confined to the proximal esophagus.5,6 When the azygous vein is additionally obstructed, blood must be diverted to the portal vein, hence the varices will be seen throughout the entire esophagus.1,2,4,5,7-9 The etiology of superior vena cava obstruction in reported cases includes carcinoma of the lung or thyroid, chronic mediastinal fibrosis, surgical ligation of the superior vena cava, and metastatic carcinoma or mediastinal mass of unknown origin.10 Superior vena cava obstruction has also been described as a complication of temporary central hemodialysis catheters.11 Although most cases of upper esophageal varices are secondary to superior vena cava obstruction, a primary or idiopathic type has also been described and has been attributed to crycopharingeal muscle constriction of abnormal caudal extensions of the posterior hypopharyngeal venous plexus.12-14 Downhill varices have also been described in the absence of vena cava obstruction after resection of a retrosternal thyroid gland.15,16 The diagnosis of esophageal varices should be made at endoscopy. Downhill varices should be recognized as a separate entity from the varices seen in portal hypertension because of differences in etiology, clinical behavior, and management. Bleeding from downhill esophageal varices occurs rarely compared with the uphill esophageal varices caused by portal hypertension. Differences in complications have been postulated to be due to differences in location of the distended esophageal varices within the esophageal wall. Stelzner and Lierse17 suggested that downhill varices distend predominantly in the submucosa, whereas uphill varices distend in VOLUME 47, NO. 3, 1998
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the subepithelium, thereby increasing the risk of rupture at the lower esophageal level. This hypothesis has not been confirmed either by histopathology or by endoscopic ultrasound. Other factors that may play a role in the increased risk of bleeding from the lower esophagus include erosions caused by esophagogastric reflux and differences in coagulation capacity,18 factors that increase the bleeding propensity for lower varices associated with portal hypertension. A variety of nonsurgical methods have been described for treatment of esophageal varices, all of them applying to portal hypertension-induced varices. There are no studies in which endoscopic methods were used to treat downhill esophageal varices, including those caused by superior vena cava syndrome. Treatment is directed to the underlying etiology of the superior vena cava syndrome in an attempt to resolve the vascular obstruction. Radioactive iodine has been used in the treatment of superior cava syndrome as a result of substernal goiter.18 Chemotherapy and radiotherapy have been proven effective for malignant etiologies.19-22 There are also studies of thrombolytic therapy with either urokinase or streptokinase23 or with tissue plasminogen activator.24 Balloon angioplasty alone has not been shown to provide long-term efficacy and has a high risk of embolization.25 In recent years, expandable metal stents have been shown to be effective in managing superior vena cava obstruction. Patients with underlying malignancies have been studied and stents, including the Gianturco Z (Cook, Bloomington, Ind.),26-30 balloon expandable Palmaz (Johnson and Johnson, Warren, N.J.),25,31,32 and self expandable Wallstent (Schneider, Minneapolis, Minn.),33,36 have been used for both malignant and benign stenoses. The long-term effectiveness and side effects are unknown and at this time stent placement is not recommended as first line therapy for benign stenoses.37 There is an option for surgical bypass of superior vena cava obstruction using spiral vein grafts, synthetic materials,38 or autologous pericardium.39 For patients who are not surgical candidates, those patients who failed previous therapy or patients without superior vena cava obstruction but who nevertheless have downhill esophageal varices, palliative measures can be attempted. There is a report of effective control of bleeding using the Sengstaken-Blakemore tube, with inflation of the esophageal balloon in the proximal esophagus.18 In considering endoscopic therapy, either variceal ligation or injection sclerotherapy, the potential for any of the numerous complications described for these procedures when performed in the distal GASTROINTESTINAL ENDOSCOPY 301
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esophagus has to be extrapolated to downhill esophageal varices. The possibility of bleeding and perforation as a result of endoscopic banding may be greater for the proximal esophagus, considering the weaker esophageal posterior wall at the upper esophageal level40 and the overall lack of a serosal covering of the esophagus.41 Injection sclerotherapy is associated with local inflammatory and thrombotic responses and has caused disseminated infectious complications. In general, distal esophageal varices are not treated with injection sclerotherapy beyond 5 cm proximal to the esophagogastric junction because of potential complications. The possibility of retrograde flow of sclerosant via the azygous vein to the venous spinal vasculature, with possible spinal cord and vertebral body infarction,42 would also severely limit enthusiasm for sclerotherapy of downhill varices. Another important consideration is a possible reduction in the efficacy of endoscopic sclerotherapy of downhill varices. An endoscopic ultrasound study performed in cirrhotic portal hypertensive patients demonstrated the presence of multiple collateral channels, with perforating veins playing a major role in maintaining their patency.43 It was noted that the success of sclerotherapy may depend on early obliteration of the perforating veins; other major factors may be the direction of blood flow in the esophageal varices and perforating veins, as well as by the actual size of paraesophageal collaterals.43 All of these factors may be different in downhill varices, but endoscopic ultrasound studies of this particular entity are not available. Pressure and blood flow may differ within downhill esophageal varices compared with portal hypertensionrelated esophageal varices. At this time, the effectiveness of available endoscopic modalities cannot be predicted, and, given the lack of published data, endoscopic treatment of downhill varices cannot be advocated. Future studies could concentrate on noninvasive variceal pressure measurement44 in an attempt to predict the effect and potential benefit of endoscopic treatment of downhill esophageal varices. To our knowledge, this is the first reported case of bleeding downhill varices as a complication of an upper extremity hemodialysis access procedure. Downhill varices must be included in the differential diagnosis of upper gastrointestinal hemorrhage in any patient who has undergone such a procedure.
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esophageal varices. A rare cause of upper gastrointestinal bleeding. Arch Surg 1978;113:1463-4. Sheiner NM, Palayew MJ. “Downhill” esophageal varices in superior vena caval obstruction. Can Meal Assoc J 1969;100: 961-4. Otto DL, Kurtzman RS. Esophageal varices in superior vena caval obstruction. Am J Roentgenol 1964;92:1000-12. Martorell F. Varices del esophago por hipertension caval superior. Angiologia 1955;7:49-53. Sorokin JJ, Levine SM, Moss EG, Miller Biddle C. Downhill varices: report of a case 29 years after resection of a substernal thyroid gland. Gastroenterology 1977;73:345-8. Snodgrass RW, Mellinkoff SM. Bleeding varices in the upper esophagus due to obstruction of the superior vena cava. Gastroenterology 1961;41:505-8. Pugliese FM. A surgical approach to bleeding downhill varices. Angiology 1973;24:606-11. Glanz S, Koser MW, Dallemand S, Gordon DH. Upper esophageal varices: report of three cases and review of the literature. Am J Gastroenterology 1982;77:194-8. Criado E, Marston WA, Jaques PF, Mauro MA, Keagy BA. Proximal venous outflow obstruction in patients with upper extremity arteriovenous dialysis access. Ann Vasc Surg 1994; 8:530-5. Rack FJ, Mincks JR, Simeone FA. Observations on the etiology of esophageal varices. AMA Arch Surg 1952;65: 422-9. Garrett N Jr, Gall EA. Esophageal varices without hepatic cirrhosis. AMA Arch Pathol 1953;55:196-202. Palmer ED. Primary varices of the cervical esophagus as a source of massive upper gastrointestinal hemorrhage. Am J Digest Dis 1952;19:375-7. Saudermann A, Kammerer J. Retrosternale Struma und Oesophagusvarizen. Munch Med Wochenschr 1960;102: 2133-7. Keiminger K, Kolb R. Symptomatische Oesophagusvarizen bei Struma. Chirurg 1972;43:277-9. Stelzner F, Lierse W. Der angiomuskulaere Verschluss tier Speiseroehre. Arch Klin Chir 1968;321:35-64. Fleig WE, Stange EF, Ditschuneit H. Upper gastrointestinal hemorrhage from downhill esophageal varices. Dig Dis Sciences 1982;27:23-7. Armstrong BA, Perez CA, Simpson JR, Hederman MA. Role of irradiation in the management of superior vena cava syndrome. Int J Radiat Oncol Biol Phys 1987;13:531-9. Sculier JP, Evans WK, Feld R, De Boer G, Payne DG, Shepherd FA, et al. Superior vena caval obstruction syndrome in small cell lung cancer. Cancer 1986;57:847-51. Perez-Soler R, McLaughlin P, Velasquez WS, Hagemeister FB, Zornoza J, Manning JT, et al. Clinical features and results of management of superior vena cava syndrome secondary to lymphoma. J Clin Oncol 1984;2:260-6. Davenport D, Ferree C, Blake D, Rabeb M. Radiation therapy in the treatment of superior vena caval obstruction. Cancer 1978;42:2600-3. Gray BH, Olin JW, Graor RA, Young JR, Bartholomew JR, Ruschhaupt WF. Safety and efficacy of thrombolytic therapy for superior vena cava syndrome. Chest 1991;99:54-9. Greenberg S, Kosinski R, Daniels J. Treatment of superior vena cava thrombosis with recombinant tissue type plasminogen activator. Chest 1991;99:1298-301. Elson JD, Becker GJ, Wholey MH, Ehrman KO. Vena caval and central venous stenoses: management with Palmaz balloon-expandable intraluminal stents. JVIR 1991;2:215-23. Putnam JS, Uchida BT, Antonovich R, Rosch J. Superior vena cava syndrome associated with massive thrombosis: treatment with expandable wire stents. Radiology 1988;167: 727-8. Charnsangavej C, Carrasco CH, Wallace S, Wright KC, Ogawa K, Richli W, et al. Stenosis of the vena cava: preliminary assessment of treatment with expandable metallic stents. Radiology 1986;161:295-8. Rosch J, Bedell JR, Putnam J, Antonovich R, Uchida B. VOLUME 47, NO. 3, 1998
Duplication cyst of the pancreatic duct presenting as pancreatitis
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Gianturco expandable wire stents in the treatment of superior vena cava syndrome recurring after maximum tolerance radiation. Cancer 1987;60:1243-6. Castaneda-Zuniga WR. Clinical experience placing Gianturco vascular stents in the venous system. Presented at the 2nd International Congress of Interventional Radiology in Cardiovascular Diseases; 1990 Feb 28-Mar 2; Toulouse, France. Radiology 1995;196:353-61. Kishi K, Sonomura T, Mitsuzane K, Nishida N, Yang RJ, Sato M, et al. Self-expandable metallic stent therapy for superior vena cava syndrome: clinical observations. Radiology 1993;189:531-5. Solomon N, Holey MH, Jarmolowski CR. Intravascular stents in the management of superior vena cava syndrome. Cathet Cardiovasc Diagn 1991;23:245-52. Dodds CA, Harrison JK, O’Laughlin MP, Wilson JS, Kisslo KB, Bashore TM. Relief of superior vena cava syndrome due to fibrosing mediastinitis using the Palmaz stent. Chest 1994;106:315-7. Zollikofer CL, Antonucci F, Stuckmann G, Mattias P, Brullman WF, Salomonowitz EK. Use of the Wallstent in the venous system including hemodialysis-related stenoses. Cardiovasc Intervent Radiol 1992;15:334-41. Dondelinger RF, Goffette P, Kurdziel JC, Roch A. Expandable metal stents for stenoses of the venae cavae and large veins. Semin Intervent Radiol 1991;8:252-63. Antonucci F, Salomonowitz E, Stuckmann G, Stiefel M, Largiader J, Zollikofer CL. Placement of venous stents: clinical experience with a self-expanding prosthesis. Radiology 1992;183:493-7. Watkinson AF, Hansell DM. Expandable Wallstent for the
treatment of obstruction of the superior vena cava. Thorax 1993;48:915-20. Hennequir LM, Fade O, Fays JG, Bic JF, Jaafar S, Bertal A, et al. Superior vena cava stent placement: results with the Wallstent endoprosthesis. Radiology 1995;196:353-61. Gloviczki P, Pairolero PC, Cherry KJ, Hallett JW Jr. Reconstruction of the vena cava and of its primary tributaries: a preliminary report. J Vasc Surg 1990;11:373-81. Piccione WJ, Faber LP, Warren WH. Superior vena caval reconstruction using autologous pericardium. Ann Thorac Surg 1990;50:417-9. Conklin JL, Christensen J. Motor functions of the pharynx and esophagus. In: Johnson LR, Alpers DH, Christensen J, Jacobson ED, Walsh JH, editors. Physiology of the gastrointestinal tract. New York: Raven Press; 1994. Boyce GA, Boyce HW. Esophagus: anatomy and structural anomalies. In: Yamaria T, Alpers DH, Owyang C, Powell DW, Silverstein FE, editors. Textbook of gastroenterology. Philadelphia: Lippincott; 1995. p. 1160. Heller SL, Meyer JR, Russell EJ. Spinal cord venous infarction following endoscopic sclerotherapy for esophageal varices. Neurology 1996;47:1081-5. Dhiman RK, Choudhuri G, Saraswat VA, Agarwal DK, Naik SR. Role of paraoesophageal collaterals and perforating veins on outcome of endoscopic sclerotherapy for oesophageal varices: an endosonographic study. Gut 1996;38:759-64. Ueno K, Hashizume M, Ohta M, Tomikawa M, Kitano S, Sugimachi K. Noninvasive variceal pressure measurement may be useful for predicting effect of sclerotherapy for esophageal varices. Dig Dis Sci 1996;41:191-6.
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Duplication cyst of the pancreatic duct presenting as pancreatitis J. Fernando del Rosario, William B. Silverman, Henri Ford, John Adkins, Eugene Wiener,
MD MD MD MD MD
The majority of cysts involving the pancreas are inflammatory pseudocysts. Enteric duplication cysts involving the pancreas are exceedingly rare1 and uncommonly give rise to symptoms. However, they can be easily mistaken for pseudocysts or choledochal cysts. We report a case of an enteric duplication cyst involving the pancreatic duct that presented clinically as acute pancreatitis. CASE REPORT An 11-year old boy was referred for abdominal pain after the pediatric surgical service ruled out a surgical From the Departments of Pediatrics (Division of Gastroenterology) and Pediatric Surgery, Children’s Hospital of Pittsburgh; Department of Internal Medicine, Division of Gastroenterology, Presbyterian Hospital, University of Pittsburgh School of Medicine. Reprint requests: J. Fernando del Rosario, MD, Dept. of Pediatrics, Div. of Gastroenterology, Children’s Hospital of Pittsburgh, 3705 Fifth Ave., Pittsburgh, PA 15213. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 1 0 37/4/87352 VOLUME 47, NO. 3, 1998
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abdomen. He had vague abdominal pain for 3 days. The pain was constant and diffuse, but without radiation to the back or flanks. There was no associated nausea, vomiting diarrhea, fever, or chills. There was no history of abdominal trauma. He had not been receiving any medications, had not consumed any alcohol, and had no known allergies. Medical history was remarkable only for a right thoracotomy during the first 2 weeks of life to remove a right intrathoracic, posterior mediastinal, cystic mass. Intraoperatively, the mass occupied nearly all of the right chest cavity and continued on through the midportion of the diaphragm as a long, tubular, muscular structure not contiguous with the gastrointestinal tract. The mass was thought to be an esophageal duplication cyst. However, biopsies of the mass disclosed a mature teratoma with elements of gastric mucosa, ganglion cells, and brain. Physical examination revealed a well-developed, wellnourished boy in no apparent distress, afebrile, and with normal vital signs. Abdominal examination revealed a soft, nontender abdomen without organomegaly, rebound tenderness, or guarding. The rest of the examination was normal. The patient was maintained on parenteral nutrition from the point of admission and took nothing by mouth. Laboratory tests at the referring hospital revealed a normal complete blood cell count without an elevated white blood cell count or left shift, normal electrolytes, normal urinalysis, but elevated amylase (638 U/L; normal: , 90 U/L) and lipase (515 U/L; normal: , 200 U/L) levels. Peak amylase and lipase levels reached 1243 U/L and GASTROINTESTINAL ENDOSCOPY 303
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tected after endoscopic injection sclerotherapy. Prog Digest Endosc 1989;34:240-3. Kokudo N, Sanjo K, Umekita N, Harihara Y, Tada Y, Idezuki Y. Squamous cell carcinoma after endoscopic injection sclerotherapy for esophageal varices. Am J Gastroenterol 1990; 85:861-4. Sato T, Watanabe M, Natsui K, Miyakawa H, Koito K, Yaosaka T, et al. Two cases of esophageal carcinoma developing after endoscopic injection sclerotherapy for esophageal varices. Clin Gastroenterol 1990;2:801-6. Claudel-Bonvoisin S, Descos L, Chatelard P, Souquet JC, Pasquier J. Cancer de l’oesophage et traitement sclerosant endoscopique de varices oesophagiennes. Gastroenterol Clin Biol 1990;14:189. Tanaka S, Yamamoto S, Senoo T, Nakamura M, Hirasawa S, Araki M, et al. A case of multiple esophageal cancers detected during following endoscopic injection sclerotherapy for esophageal varices. Gastroenterol Endosc 1992;34:402-7. Terada M, Ogino H, Adachi K, Nakazawa H, Hirose S, Miwa A. A case of triple cancer of the stomach, lung and esophagus after endoscopic injection sclerotherapy for the esophageal varices. Gastroenterol Endosc 1992;34:2347-53. Rodriguez M, de la Cruz S, Arrabal I, Herrera M. Esophageal carcinoma following endoscopic injection sclerotherapy. Rev Esp Enf Digest 1992;82:43-6. Tada M, Shimada M, Murakami F, Mizumachi M, Arima K, Yanai H, et al. Development of the strip-off biopsy. Gastroenterol Endosc 1984;26:833-9. Salaman MH, Glendenning OM. Tumor promotion in mouse skin by sclerosing agents. Br J Cancer 1957;11:434-44. Moon MR, Schulte WJ, Haaslet GB, Condon RE. Transhiatal
Bleeding downhill esophageal varices: a complication of upper extremity hemodialysis access Anca Pop, MD Alan F. Cutler, MD
Esophageal varices are a known complication of portal hypertension and primarily involve the esophagogastric junction. They are a result of hepatofugal blood flow. Proximal esophageal varices, often termed “downhill” varices,1 have been described sporadically and attributed mainly to superior vena cava obstruction with reversal of the normal blood flow direction in the upper esophageal plexus. We describe here the first case of bleeding downhill esophageal varices occurring as an iatrogenic complication of venous access for hemodialysis. From the Section of Gastroenterology, Sinai Hospital, Detroit, Michigan. Reprint requests: Alan F. Cutler, MD, Director, Gastroenterology Research, Section of Gastroenterology, Sinai Hospital, 6767 West Outer Dr., Detroit, MI 48235. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 1 0 37/4/87351 VOLUME 47, NO. 3, 1998
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35. 36.
and transthoracic esophagectomy for adenocarcinoma of the esophagus. Arch Surg 1992;127:951-5. Ogle SJ, Kirk CJC, Bailey RJ, Johnson AG, Williams R, Murray-Lyon IM. Oesophageal function in cirrhotic patients undergoing injection sclerotherapy for oesophageal varices. Digestion 1978;18:178-85. Krevsky B. Tumors of the esophagus. In: Berk JE, editor. Bockus’ gastroenterology. Vol. 1. 5th ed. Philadelphia: WB Saunders; 1995. p. 534-57. Bombi JA, Rivemla A, Bordas JM, Cardesa A. Adenosquamous carcinoma of the esophagus. A case report. Path Res Pract 1991;187:514-9. Akagi T, Sakata Y, Takemori H, Osanai S. A case of advanced esophageal cancer showing good partial response by combination therapy of low dose 5-FU and low dose CDDP. Gan To Kagaku Ryoho 1996;23:103-6. Vigneswaran WT, Trastek VF, Pairolero PC, DeSchamps C, Daly RC, Allen MS. Transhiatal esophagectomy for carcinoma of the esophagus. Ann Thorac Surg 1993;56:838-46. Orsatti G, Corvalan AH, Sakurai H, Choi HS. Polypoid adenosquamous carcinoma of the esophagus with prominent spindle cells. Report of a case with immunohistochemical and ultrastructural studies. Arch Pathol Lab Med 1993;117: 544-7. Nezu K, Kato H, Tachimori Y, Watanabe H. A clinicopathologic study of 11 adenosquamous carcinoma of the esophagus. Nippon Shoukaki Geka Gakkai Zasshi 1991;24:1-8. Suzuki H, Nagayo T. Primary tumors of the esophagus other than squamous cell carcinoma; histologic classification and statistics in the surgical and autopsied materials in Japan. Int Adv Surg Oncol 1980;3:73-109.
CASE REPORT A 52-year-old woman presented with dyspnea, orthopnea, cough, and extreme weakness. She also complained of blurred vision and bilateral tinnitus. Medical history was significant for hypertension, diabetes mellitus, peripheral vascular disease, and dialysis-dependent chronic renal failure. She also had a history of recurrent arteriovenous shunt occlusion, and on several previous occasions had required temporary placement of a hemodialysis access catheter. Six months before the present admission, she was diagnosed with superior vena cava syndrome secondary to thrombotic occlusion of the superior vena cava. At that time a bilateral arm venogram was performed that demonstrated patency of arm veins on the right and occlusion of brachial veins on the left. There was patency of the cephalic vein and numerous collaterals in the upper arm. The subclavian vein was only partially patent. The distal superior vena cava was patent from the level of the left innominate vein to the right atrium. A short occlusion of the distal right innominate vein and proximal superior vena cava was noted, with numerous collaterals present and extending into the azygous and hemiazygous system (Fig. 1). Attempts were made to improve flow in the superior vena cava. Despite efforts with multiple different guidewire and catheter combinations, the superior vena cava obstruction could not be crossed, thereby preventing placement of a stent or performance of balloon angioplasty. It was believed that urokinase or other direct anticoagulation would not be of benefit. The patient subGASTROINTESTINAL ENDOSCOPY 299
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Figure 2. Endoscopic photograph taken 25 cm from incisors demonstrating mucosal defect (open arrowhead) overlying “downhill” varices.
Figure 1. Bilateral arm venogram demonstrating occlusion of proximal superior vena cava (black arrow) with azygous and hemiazygous systems collaterals.
sequently underwent placement of a new arteriovenous shunt at the level of her right thigh. At the present admission, physical examination revealed extreme facial, neck, and upper extremity edema. Jugular venous distension was present. The patient was dyspneic, and on auscultation she had bilateral rhonchi and distant heart sounds. Examination of the right thigh showed the presence of a functioning arteriovenous shunt. A previous shunt was also identifi ed in the left forearm. The admission laboratory values included the following: hemoglobin 6.7 mg/dL, hematocrit 33%, prothrombin time 14.1 seconds, partial thromboplastin time 27 seconds, platelet count 159,000, blood urea nitrogen 76 mg/dL, and creatinine 15 mg/dL. On the day after admission the patient developed massive hematemesis. She became hypotensive, requiring institution of pressor support and transfer to the medical intensive care unit. After hemodynamic stabilization she was taken to the operating room. Tracheal intubation was undertaken, and gastric lavage was performed. Endoscopy was then accomplished under controlled conditions. At endoscopy the presence of grade two esophageal varices was noted, confi ned to the proximal to mid-esophagus and extending from 20 to 32 cm from the incisors. The presence of a mucosal defect was noted overlying one of the varices (Fig. 2). There was no active bleeding at the time of endoscopy. The distal esophagus (Fig. 3), stomach, duodenal bulb, and second portion of the duodenum were free of mucosal lesions (Fig. 3). The patient was transferred back to the intensive care unit for monitoring, 300 GASTROINTESTINAL ENDOSCOPY
Figure 3. Endoscopic photograph of distal esophagus without apparent varices. awaiting further management decisions. A total of seven units of packed red blood cells were transfused. No interventions were performed at endoscopy. The patient was managed conservatively for the next 3 months during which she complained of severe headaches and facial edema. There were intermittent deteriorations in the patient’s mental status believed to be secondary to cerebral edema. Surgical management was therefore offered. She underwent a vascular Gore-Tex graft bypass with an end-to-side anastomosis to the right internal jugular vein and an end-to-end anastomosis to the right atrial appendage. Clinical improvement was noted immediately postoperatively, with partial resolution of facial edema. The patient did not experience any further episodes of gastrointestinal blood loss in the 5 months after the surgery. VOLUME 47, NO. 3, 1998
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DISCUSSION By draining into the inferior thyroid, azygous, hemiazygous, and gastric veins, the veins of the esophagus form a connection between the portal and systemic venous systems. The normal venous drainage of the esophagus occurs predominantly via the azygous and hemiazygous systems. In portal hypertension, the portal blood will be delivered to the superior vena cava and “uphill” varices will result. With obstruction of the superior vena cava, blood will return to the right atrium by way of collateral pathways to the inferior vena cava. The direction of the flow being retrograde, “downhill” varices will develop. The downward extent of downhill varices depends on the duration of obstruction and therefore adequacy of collateralization,2-4 as well as on the level of superior vena cava obstruction. If the obstruction is proximal to the azygous vein, blood will return via collaterals including internal mammary, vertebral, and patent azygous veins and the downhill varices will be confined to the proximal esophagus.5,6 When the azygous vein is additionally obstructed, blood must be diverted to the portal vein, hence the varices will be seen throughout the entire esophagus.1,2,4,5,7-9 The etiology of superior vena cava obstruction in reported cases includes carcinoma of the lung or thyroid, chronic mediastinal fibrosis, surgical ligation of the superior vena cava, and metastatic carcinoma or mediastinal mass of unknown origin.10 Superior vena cava obstruction has also been described as a complication of temporary central hemodialysis catheters.11 Although most cases of upper esophageal varices are secondary to superior vena cava obstruction, a primary or idiopathic type has also been described and has been attributed to crycopharingeal muscle constriction of abnormal caudal extensions of the posterior hypopharyngeal venous plexus.12-14 Downhill varices have also been described in the absence of vena cava obstruction after resection of a retrosternal thyroid gland.15,16 The diagnosis of esophageal varices should be made at endoscopy. Downhill varices should be recognized as a separate entity from the varices seen in portal hypertension because of differences in etiology, clinical behavior, and management. Bleeding from downhill esophageal varices occurs rarely compared with the uphill esophageal varices caused by portal hypertension. Differences in complications have been postulated to be due to differences in location of the distended esophageal varices within the esophageal wall. Stelzner and Lierse17 suggested that downhill varices distend predominantly in the submucosa, whereas uphill varices distend in VOLUME 47, NO. 3, 1998
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the subepithelium, thereby increasing the risk of rupture at the lower esophageal level. This hypothesis has not been confirmed either by histopathology or by endoscopic ultrasound. Other factors that may play a role in the increased risk of bleeding from the lower esophagus include erosions caused by esophagogastric reflux and differences in coagulation capacity,18 factors that increase the bleeding propensity for lower varices associated with portal hypertension. A variety of nonsurgical methods have been described for treatment of esophageal varices, all of them applying to portal hypertension-induced varices. There are no studies in which endoscopic methods were used to treat downhill esophageal varices, including those caused by superior vena cava syndrome. Treatment is directed to the underlying etiology of the superior vena cava syndrome in an attempt to resolve the vascular obstruction. Radioactive iodine has been used in the treatment of superior cava syndrome as a result of substernal goiter.18 Chemotherapy and radiotherapy have been proven effective for malignant etiologies.19-22 There are also studies of thrombolytic therapy with either urokinase or streptokinase23 or with tissue plasminogen activator.24 Balloon angioplasty alone has not been shown to provide long-term efficacy and has a high risk of embolization.25 In recent years, expandable metal stents have been shown to be effective in managing superior vena cava obstruction. Patients with underlying malignancies have been studied and stents, including the Gianturco Z (Cook, Bloomington, Ind.),26-30 balloon expandable Palmaz (Johnson and Johnson, Warren, N.J.),25,31,32 and self expandable Wallstent (Schneider, Minneapolis, Minn.),33,36 have been used for both malignant and benign stenoses. The long-term effectiveness and side effects are unknown and at this time stent placement is not recommended as first line therapy for benign stenoses.37 There is an option for surgical bypass of superior vena cava obstruction using spiral vein grafts, synthetic materials,38 or autologous pericardium.39 For patients who are not surgical candidates, those patients who failed previous therapy or patients without superior vena cava obstruction but who nevertheless have downhill esophageal varices, palliative measures can be attempted. There is a report of effective control of bleeding using the Sengstaken-Blakemore tube, with inflation of the esophageal balloon in the proximal esophagus.18 In considering endoscopic therapy, either variceal ligation or injection sclerotherapy, the potential for any of the numerous complications described for these procedures when performed in the distal GASTROINTESTINAL ENDOSCOPY 301
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esophagus has to be extrapolated to downhill esophageal varices. The possibility of bleeding and perforation as a result of endoscopic banding may be greater for the proximal esophagus, considering the weaker esophageal posterior wall at the upper esophageal level40 and the overall lack of a serosal covering of the esophagus.41 Injection sclerotherapy is associated with local inflammatory and thrombotic responses and has caused disseminated infectious complications. In general, distal esophageal varices are not treated with injection sclerotherapy beyond 5 cm proximal to the esophagogastric junction because of potential complications. The possibility of retrograde flow of sclerosant via the azygous vein to the venous spinal vasculature, with possible spinal cord and vertebral body infarction,42 would also severely limit enthusiasm for sclerotherapy of downhill varices. Another important consideration is a possible reduction in the efficacy of endoscopic sclerotherapy of downhill varices. An endoscopic ultrasound study performed in cirrhotic portal hypertensive patients demonstrated the presence of multiple collateral channels, with perforating veins playing a major role in maintaining their patency.43 It was noted that the success of sclerotherapy may depend on early obliteration of the perforating veins; other major factors may be the direction of blood flow in the esophageal varices and perforating veins, as well as by the actual size of paraesophageal collaterals.43 All of these factors may be different in downhill varices, but endoscopic ultrasound studies of this particular entity are not available. Pressure and blood flow may differ within downhill esophageal varices compared with portal hypertensionrelated esophageal varices. At this time, the effectiveness of available endoscopic modalities cannot be predicted, and, given the lack of published data, endoscopic treatment of downhill varices cannot be advocated. Future studies could concentrate on noninvasive variceal pressure measurement44 in an attempt to predict the effect and potential benefit of endoscopic treatment of downhill esophageal varices. To our knowledge, this is the first reported case of bleeding downhill varices as a complication of an upper extremity hemodialysis access procedure. Downhill varices must be included in the differential diagnosis of upper gastrointestinal hemorrhage in any patient who has undergone such a procedure.
4. 5. 6. 7. 8. 9. 10. 11.
12. 13. 14. 15. 16. 17. 18. 19. 20. 21.
22. 23. 24. 25. 26.
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treatment of obstruction of the superior vena cava. Thorax 1993;48:915-20. Hennequir LM, Fade O, Fays JG, Bic JF, Jaafar S, Bertal A, et al. Superior vena cava stent placement: results with the Wallstent endoprosthesis. Radiology 1995;196:353-61. Gloviczki P, Pairolero PC, Cherry KJ, Hallett JW Jr. Reconstruction of the vena cava and of its primary tributaries: a preliminary report. J Vasc Surg 1990;11:373-81. Piccione WJ, Faber LP, Warren WH. Superior vena caval reconstruction using autologous pericardium. Ann Thorac Surg 1990;50:417-9. Conklin JL, Christensen J. Motor functions of the pharynx and esophagus. In: Johnson LR, Alpers DH, Christensen J, Jacobson ED, Walsh JH, editors. Physiology of the gastrointestinal tract. New York: Raven Press; 1994. Boyce GA, Boyce HW. Esophagus: anatomy and structural anomalies. In: Yamaria T, Alpers DH, Owyang C, Powell DW, Silverstein FE, editors. Textbook of gastroenterology. Philadelphia: Lippincott; 1995. p. 1160. Heller SL, Meyer JR, Russell EJ. Spinal cord venous infarction following endoscopic sclerotherapy for esophageal varices. Neurology 1996;47:1081-5. Dhiman RK, Choudhuri G, Saraswat VA, Agarwal DK, Naik SR. Role of paraoesophageal collaterals and perforating veins on outcome of endoscopic sclerotherapy for oesophageal varices: an endosonographic study. Gut 1996;38:759-64. Ueno K, Hashizume M, Ohta M, Tomikawa M, Kitano S, Sugimachi K. Noninvasive variceal pressure measurement may be useful for predicting effect of sclerotherapy for esophageal varices. Dig Dis Sci 1996;41:191-6.
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Duplication cyst of the pancreatic duct presenting as pancreatitis J. Fernando del Rosario, William B. Silverman, Henri Ford, John Adkins, Eugene Wiener,
MD MD MD MD MD
The majority of cysts involving the pancreas are inflammatory pseudocysts. Enteric duplication cysts involving the pancreas are exceedingly rare1 and uncommonly give rise to symptoms. However, they can be easily mistaken for pseudocysts or choledochal cysts. We report a case of an enteric duplication cyst involving the pancreatic duct that presented clinically as acute pancreatitis. CASE REPORT An 11-year old boy was referred for abdominal pain after the pediatric surgical service ruled out a surgical From the Departments of Pediatrics (Division of Gastroenterology) and Pediatric Surgery, Children’s Hospital of Pittsburgh; Department of Internal Medicine, Division of Gastroenterology, Presbyterian Hospital, University of Pittsburgh School of Medicine. Reprint requests: J. Fernando del Rosario, MD, Dept. of Pediatrics, Div. of Gastroenterology, Children’s Hospital of Pittsburgh, 3705 Fifth Ave., Pittsburgh, PA 15213. Copyright © 1998 by the American Society for Gastrointestinal Endoscopy 0016-5107/98/$5.00 1 0 37/4/87352 VOLUME 47, NO. 3, 1998
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abdomen. He had vague abdominal pain for 3 days. The pain was constant and diffuse, but without radiation to the back or flanks. There was no associated nausea, vomiting diarrhea, fever, or chills. There was no history of abdominal trauma. He had not been receiving any medications, had not consumed any alcohol, and had no known allergies. Medical history was remarkable only for a right thoracotomy during the first 2 weeks of life to remove a right intrathoracic, posterior mediastinal, cystic mass. Intraoperatively, the mass occupied nearly all of the right chest cavity and continued on through the midportion of the diaphragm as a long, tubular, muscular structure not contiguous with the gastrointestinal tract. The mass was thought to be an esophageal duplication cyst. However, biopsies of the mass disclosed a mature teratoma with elements of gastric mucosa, ganglion cells, and brain. Physical examination revealed a well-developed, wellnourished boy in no apparent distress, afebrile, and with normal vital signs. Abdominal examination revealed a soft, nontender abdomen without organomegaly, rebound tenderness, or guarding. The rest of the examination was normal. The patient was maintained on parenteral nutrition from the point of admission and took nothing by mouth. Laboratory tests at the referring hospital revealed a normal complete blood cell count without an elevated white blood cell count or left shift, normal electrolytes, normal urinalysis, but elevated amylase (638 U/L; normal: , 90 U/L) and lipase (515 U/L; normal: , 200 U/L) levels. Peak amylase and lipase levels reached 1243 U/L and GASTROINTESTINAL ENDOSCOPY 303