- Email: [email protected]
Management of giant duodenal ulcer
Management of Giant Duodenal Ulcer Michael S. Nussbaum, MD, Cincinnati, Ohio Mark A. Sch.sterman, MD, Cincinnati, Ohio
Giant duodenal ulcer, a variant of peptic ulcer disease, is generally defined as a full-thickness ulceration at least 2 cm in diameter, usually involving a large portion of the duodenal bulb. These ulcers are unique in that they are easily missed on upper gastrointestinal series and are associated with high morbidity and mortality rates. In recent years the diagnosis is being made much more frequently because the disease is becoming more familiar to the surgeon and radiologist. Medical management of giant duodenal ulcer is fraught with complications and is only rarely successful. Since Brdiczka [1] first called attention to this entity in 1931, 196 cases have subsequently been reported. Most investigators agree that early diagnosis along with early surgical management are the mainstays of reversing the dismal prognosis of this disease. Giant duodenal ulcer is often associated with a long history of neglected duodenal ulcer disease, with massive hemorrhage, obstruction, and walledoff perforation being common presenting features. These ulcers are penetrating, usually involving more than 80 percent of the duodenal bulb and may be adherent to the pancreas, liver, colon, and other adjacent structures, thus making surgical management difficult. This study reviews our experience with 32 patients diagnosed with giant duodenal ulcer at the University of Cincinnati Medical Center between 1963 and 1982. Medical and surgical therapies are compared and symptoms, clinicalfeatures, management, morbidity, and mortality are discussed. Material and Methods
All patients at the University of Cincinnati Medical Center and the Cincinnati Veterans Administration Medical Center with the diagnosis of giant duodenal ulcer from 1963 through 1982 were identified and their case records and follow-up reports reviewed. A giant duodenal ulcer was so defined if it measured 2cm or larger in greatest diameter by radiologic examination, endoscopy, necropsy, or at surgery. If the diagnosis was made by radiologic methods or no histologic examination was made, a 3 year clinical follow-upwas necessary to avoid including cancers. From the Department of Surgery, University of Cincinnati Medical Center. Cincinnati. Ohio. Requests for reprints should be ad~essed to Michael S. Nussba~n, ME), Department of Surgery, University of Cincinnati Medical Center, 231 Bethesda Avenue (ML/fS58), Cincinnati, Ohio 45267.
Vol.n~ 149,March1985
All patients were evaluated for symptomatic, diagnostic, therai~,.~,'ic,short-term, and long-term follow-up data. Thirty-two patients were identifiedwith documented giant duodenal ulcer. Twenty-four of the patients (75 percent) were men and 23 (72 percent) were white. The mean age of the patientswas 59 years (range30 to 81 years). Twenty-seven patients (84 percent) presented with epigastricpain. Gastroinestinalhemorrhage was a presenting symptom in 24 patients (75 percent).Twenty-four patients (75 percent ~ complained of nausea and vomiting, with 9 " patients (28 percent) having documented gastric outlet obstruction.Marked weight lossoccurred in 11 patients(34 percent), back pain was noted in 8 patients (25 percent), and 3 patients (9 percent) presented with a free perforation.In addition, 14 patients (44 percent) relateda history of peptic ulcer disease.Fourteen patients (44 percent) in our serieshad a history of ethanol abuse. Serum albumin was measured in 27 patientsand ranged from 1.4 to 4.8 g/dl (mean 3.3 g/dl).Acid secretory data were obtained in eight patients.Basal acid output ranged from 1.7 to 21.4 mEq[hour (mean 9 mEq/hour), and maximal acid output ranged from 24.1 to 140 mEq/hour (mean 52.9 mEq/hour). Serum gastrin was measured in five patients,and values ranged from 50 to ]31 pg{ml (mean 92 pg/ml; normal islessthan 160 pg/ml). None of the patients with acid hypersecretion demonstrated abnormal serum gastrin or calcium values as seen in the Zollinger-Ellison syndrome or subsequently was shown to have the Zollinger-Ellisonor multiple endocrine neoplasia syndrome. The diagnosisof giant duodenal ulcer was made by upper gastrointestinal series in 24 patients. Endoscopy documented or confirmed the diagnosi3 in 11 patients. Three patients were diagnosed at surgery and one at necropsy. The diameter of the ulcers ranged from 2 to 6 cm (mean 3.5 cm). Twenty-four patients were initially managed medically (mean length of treatment 41 months), primarily with antacids and diet. Cimetidine was utilized alone or in conjunction with antacids and diet in 10 patients. The mean length of treatment with cimetidine was 18 months. There were two deaths due to hemorrhage (8 percent), two unrelated late deaths (8 percent), and 20 recurrences (83 percent) in the medical group. Twenty-seven operations were required in 25 patients. In 17 of these patients, medical treatment failed, 7 of whom required emergency surgery. Eight patients were managed primarily by surgery, five emergently. There were three deaths in the surgical group (12 percent), all after emergency surgery. In two of the three, medical treatment had failed. There were no deaths among the elective surgery group. There was a totalof 12 surgical complications (41 percent) with a mean follow-up period of 35 months. There
357
Nussbaum and Schusterman
TABLE I
Surgical Rq~ults
Procedure Truncal vagotomy and antrectomy, BIIIroth II (n = 12) Truncal vagotomy and pyloroplasty (n = 7) Truncal vagotomy and antrectomy, Billroth I (n ffi 3) Truncal vagotomy,
Emergency
Mean Follow-Up (me)
Asymptomatlc
Recurrence
2
37
8
0
7
43
5
0
0
2
0
24
3
0
1 (short-term)
0
1
35
2
0
1 (lntraoporative)
0
2
1
0
2
0
0
gastroenterostomy (n = 3) Exploration, oversewing of ulcer (n = 2)
was one intraoperative complication: an iatrogenic splenic injury requiring splenectomy. Five patients had six short-term complications including postprandial nausea and vomiting, gastric outlet obstruction (two patients, one resolved and one required reoperation), wound infection, pulmonary embolus, and recurrent epigastric pain. There were five long-term complications including incisional hernia, esophageal stricture, intermittent nausea and vomiting, small bowel obstruction, and afferent loop syndrome. Twenty-five of the 27 operative procedures were definitive acid-reducing operations including vagotomy and antrectomy with Billroth II reconstruction (12 patients), vagotomy and pyloroplasty (7 patients), vagotomy and antrectomy with Billroth I reconstruction (3 patients), and vagotomy and gastrojejunostomy (3 patients). A duodenostomy tube was utilized in 5 of the 12 Billroth lI operations without any complications. Two patients underwent emergency exploration and oversewing of a perforated giant duodenal ulcer alone. Both of these patients subsequently required a more definitive operation because of recurrent symptoms (Table I). Comments
Since Brdiczka [1] first described giant duodenal ulcer 51 years ago, several salient features have been emphasized in the literature. Diagnosis is often elusive unless specific diagnostic and roentgenologic criteria are followed. Failure to recognize this condition results in a high mortality, usually from hemorrhage, as is evident from the early case reports. By 1957, only 7 of 21 reported patients had survived, giving the disease a mortality rate of 67 percent [2]. Successful noninvasive treatment is rare [3-6]. Although the morbidity and mortality rates have decreased since 1957, the condition is usually fatal unless an accurate diagnosis is made and surgical treatment implemented. Early- diagnosis is the cornerstone of therapy. Giant duodenal ulcer is defined as an ulcer located
358
Complications 8 (5 long-term, 3 short-term)
Mortality 1
in the first part of the duodenum, accurately measured to be greater than 2 cm in diameter, and histologically studied at surgical exploration, extirpation, or autopsy. If the diagnosis is made by radiologic methods or no histologic examination is made, a 3 year clinical follow-up must be carried out to avoid including cancers [2]. The differential diagnosis of giant duodenal ulcer includes lymphosarcoma, carcinoma, and gastric ulcer [3]. The large majority of patients reported in the literature are men (approximately 80 percent) with an average age of 55 to 60 years. The patients commonly present with a history of long-standing ulcer symptoms that respond poorly to medical management. Because of the anatomic location, perforation usually occurs as a slow penetration so that adjacent structures or organs wall off the area of destroyed duodenal wall. At onset, epigastric pain often is more intense than in the usual ulcer patient, and as the ulcer burrows into the pancreas and adjacent structures, the pain may be referred more to the right upper quadrant or posteriorly into the back. It is generally not relieved by food or antacids, which is understandable in view of the size and penetration of the ulcer [2,3]. Gastrointestinal hemorrhage is another common presenting feature. The gastroduodenal artery has often been found in the middle of the ulcer bed, actively hemorrhaging at operation. Either partial or complete obstruction of the duodenal segment distal to the ulcer crater, manifested by vomiting, weight loss, and malnutrition, is another presenting problem. The cachexia can be so prominent with the association of an inflammatory mass in the epigastrium, that carcinoma may be the initial diagnosis in these patients. Most of the patients present to the hospit~ with chronic illness, weight loss, and abdominal pain. If they present as an emergency, it is usually for massive hemorrhage from
The AmeriCan Journal of Surgery
Giant Duodenal Ulcer
the gastroduodenal artery. All of these symptoms are related to the natural progression of the disease and were seen often in this series. In addition, 14 patients in our series had a history of ethanol abuse, and two operative deaths resulted directly from postoperative hepatic failure. Various investigators have pointed out that the highest mortality occurs in giant duodenal ulcer patients who remain undiagnosed. Mortality is decreased in those patients diagnosed intraoperatively and is lowest in patients accurately diagnosed preoperatively [2-I 7]. The upper gastrointestinal series, in conjunction with endoscopic confirmation and biopsy, is the most important tool for making the diagnosis of giant duodenal ulcer. The findings on roentgenologic exan~ination is by far the most discussed subject in the literature concerning giant duodenal ulcer. Since Brdiczka [I] first described misdiagnosis by upper gastrointestinal examination in two of his three original cases, the literature has been full of case reports of similar occurrences [2,5,7-14,18-21). However, as awareness of this lesion increased, certain diagnostic criteria were established that not only led to earlier diagnosis, b u t also demonstrated that the disease was not as rare as initially believed. The radiographs of this lesion have been so deceptive to radiologists and other physicians that the number of unreported cases must be large [15]. The major problem with the diagnosis of these ulcers is that they are so large they may resemble a normal or slightly abnormal bulb that may be considered a deformity rather than a giant ulcer. The radiologic diagnosis is subtle, the salient points being fixation of the duodenal bulb and lack of normal peristaltic waves; constancy in size and shape of the ulcer; loss of the mucosal pattern about the crater; a tendency to stand out as the duodenum empties the barium (Figure 1); multiple filling defects resembling polypoid tumors (pathologically, these represent either necrotic pancreas, granulation tissue, or blood clots); air-fluid level in the ulcer crater; a patulous or partially destroyed pylorns; and obstruction or spasm of the duodenum distal to the ulcer due to inflammation and edema [1-3]. These criteria have been refined and developed over 30 years and are the mainstay of early diagnosis. During endoscopy, the pyloric sphincter is widened, edematous, and hyperemic. In addition, it is often less mobile than normal. The ulcers are so large that they can be clearly seen through the pyloric sphincter. On passage through the pylorus, soft necrotic slough is seen extending circumferentially around the whole mucosa, and virtually replaces the duodenal wall. The major differential diagnosis of giant duodenal ulcer is pseudodiverticulum of the bulb or a true diverticulum of the postbulbous area and a carcinoma or lymphosarcoma of the duodenal bulb. The diver-
¥OIIJm¢l 14g, Irll~Ch 1|B$
Figure I. Radlologlc ~fagnos~ The uk:~r haB a tendency to stand out a.r the duodenum mmp#eB lie# barium.
ticulum can be diagnosed by the observation of the changing size and shape of the crater. The neoplasms are extremely rare, but the distinction can be made at the operating table or by endoscopic biopsy. Successful medical treatment has been the exception in the past. There is only one long-term asymptomatic surviving patient in the literature, reported by Lumsden et al [6] and followed for 6 years. Other reports of successful medical management by Calonje and Ochsner [23], Dawson [5], Bruni et al [22], and Eisenberg [24] were followed only for 2 weeks to 6 months. There are many reports of patients initially treated medically who died from sudden massive hemorrhage or required emergency operations for bleeding or free perforation. Since the ulcer is literally a walled-off through-and-through perforation and involves more than 80 percent of the duodenal bulb, the long-term ability of the duodenum to function adequately after healing must be questioned, if the patient does not die from the complications. Medical therapy does play an adjunctive role as a prelude to surgery, its aim being to improve the patient's general condition and allow time for any in-
359
Nussbaum and Sc~sterman
flammatory swelling around the ulcer to subaide [6]. Most of these patients are malnourished and total parenteral nutrition may be indicated. Cimetidine and antibiotics are also helpful in reducing the inflammation and edema so that safe resection can be carried out~ Surgical treatment of this condition is not without complications. In the 1963 series by Mistilis et al [3] from our institution, 10 of 14 patients were operated on for giant duodenal ulcer, 3 of whom died due to problems with the duodenal stump. In our present series, there were three deaths and the complication rate was 41 percent, which correlates with the decreasing mortality rate demonstrated in the literature. The death rate in the 1940s and 1950s was extremely high, but by 1966 the overall mortality rate was 41 percent (15 of 37 patients), and by 1969 44 more patients had presented with only 8 deaths (18 percent). The most recent literature describes 57 more patients with 4 deaths, for a mortality rate of 7 percent [6,15,21,23,25-27]. The progressive decline in the mortality rate can be attributed to numerous factors. Improvements in radiologic equipment (such as image intensification), the advent of fiberoptic endoscopy, better preoperative and postoperative nutritional support, and improved surgical and anesthetic techniques have led to the increasing number of early diagnoses, earlier surgical intervention, and improved management of these patients. Most investigators agree that vagotomy and gastric resection with removal of the involved duodenum is the procedure of choice when possible. Frequently, however, the duodenal mass is so inflamed and indurated that duodenal dissection would be hazardous. Management of the duodenal stump has been controversial. If the inflammation and edema are markedly decreased, a Billroth I reconstruction can occasionally be performed safely. If there is still a large inflammatory mass in the duodenum at the time of exploration, truncal vagotomy and gastrojejunostomy may be the best alternative. Because of the size of the ulcer bed in some of these patients, many surgeons recommend that the ulcer bed be left in situ, resecting the duodenum around the ulcer bed and oversewing and decompressing it with a duodenostomy tube. Klamer and Mahr [4] have advocated rite Bennett. modification of the Bancroft procedure in managing the difficult duodenal stump and have reported success. This entails antrectomy, but the distal margin of resection leaves 3 to 4 cm of antrum and the pylorus intact. The antral mucosa is then carefully stripped from the muscle of the duodenum. A pursestring suture is then used to close the pylorus from inside, and the wall of the antrum is closed in layers. The most important principle is preservation of the distal antral blood supply because necrosis is a risk during this procedure [28,£9]. We believe that the Bancroft procedure, although a viable option, is
300
time consuming and generally not warranted in such high-risk patients. Our data support the use of duodenostomy as a safe and effective means of managing the difficult duodenal stump so often encountered in giant duodenal ulcer disease. A duodenostomy tube was utilized in 5 of the 12 Billroth II operations in our series..Infour of these patients complications developed; however, none was directly due to the duodenostomy and there were no deaths. The higher incidence of complications in patients in w h o m duodenostomy was utilized can be explained by the fact that, by definition, these patients usually had more extensive, longer-standir,g ulcer disease and were, therefore, more debilitated and generally poorer surgical risks. In conclusion, our results concur with those in the literature and indicate that giant duodelml ulcer should be primarily managed surgically. These patients do very poorly with medical therapy, and the mortality rate is increased if emergency surgery is required for acute hemorrhage. Medical treatment alone is ~az~sociatedwith a bigh morbidity but is useful as an adjunct to surgery. The success of the treatment of this dangerous variant of duodenal ulcer depends on accurate prepperative diagnosis and preparation and appropriate operative therapy. Should operation be required, a definitive acid-reduction operation is the procedure of choice. Duodenostomy is advocated as a safe alternative to management of the difficult duodenal stump.
Summary Giant duodenal ulcer is a variant of peptic ulcer that is 2 ~m in diameter or greater and essentially replaces the duodenal bulb. Diagnosis by upper gastrointestinal series is often missed, due to the large size of the ulcer, which causes it to look like a scarred duodenal bulb or duodenal diverticulum. This study reviews our experience with 32 patients who presented with giant duodenal ulcer between 1963 and 1982. Seventy-five percent of the patients were men between 30 and 81 years of age (mean age 59 years). Gastrointestinal hemorrhage was a presenting symptom in 75 percent of the patients and free perforation in 9 percent. Diagnosis was made by upper gastrointestinal series (24 patients), and endoscopy (11 patients), alone or in combination. Three patients were diagnosed at surgery and one at necropsy. Mean size of the ulcer was 3.5 cm in diameter, range 2 to 6 cm. Twenty-four patients were initially managed medically (me~m length of treatment 41 months), with 2 deaths (hemorrhage) and 20 recurrences (83 percent). Twenty-seven operations were required in 25 patients. In 17 of the 25, medical treatment had failed. Seven of these patients required emergency surgery. Eight patients were managed primarily by surgery, of whom five presented emergently. There were three deaths in the surgical group after emergency surgery. In two of these patients, medical
I~ ~
Joum~ of $ ~ z g ~ ,
Giant Duodonai Ulcer
treatment had failed. There were no deaths among the elective surgery group. Twenty-five of the 27 operative procedures were definitive, acid-reducing operations (15 vagotomy and antrectomy and 10 vagotomy and drainage). Two patients underwent emergency exploration and oversewing of a giant perforated ulcer alone, and both patients required subsequent surgery because of symptoms. The results indicate that giant duodenal ulcer should be primarily surgically managed and that an acid-reducing procedure should be performed during primary surgery. These patients do very poorly with medical therapy, and the mortality rate is increased if emergency surgery is required for hemorrhage. Medical treatment alone is associated with a high morbidity (92 percent). Should operation be required, a definitive acid reduction operation is the procedure of choice. Acknowledgment: We thank Josef E. Fischer, MD for his encouragement and counsel.
References 1. Brdiczka JG. Oas grosse ulcus duodenl in Rontgenblld. Fortschr Geb Rontgenstr 1931;44:177-81. 2. Stainton RM, Growdon Jl~. Benign giant duodenal ulcer. Am J Surg 1957;23:1081-96. 3, Mistilis SP, Wiot JF, Nedelman SH. Giant duodenal ulcer. Ann Intern Med 1963;59:155-64. 4. Kia/ner TW, Mahr MM. Giant duodenal ulcer: a dangerous variant of a common illness. Am J Surg 1978; 135:760-2. 5. Dawson J. Giant duodenal ulcer. Br J Radiol 1958;31:666-8. 6. Lumsden K, MacLamon JC, Dawson J. Giant duodenal ulcer. Gut 1970; 11:592-9. 7. Freedman E, Goehring HD. Diagnostic errors in ulcerative lesions of the stomach and duodenum. Am J Roentgenol Radium Thor 1940;44:48-58. 8. Elkln WP. Diagnostic problems in cases of large or giant duodenal ulcer. Radiology 1941;37:748-50. 9. Evashwick G. Giant benign duodenal ulcer: report of a c a s e ,
Volume 149, M ~ c h 1985
Ann Surg 1951;133:417-20. 10. Bullock WK, Snyd~" EN Jr. Benign giant duoderml ulcer. Gastroentoroiogy 1952;20:330-6. 11. Hamel JF. Giant benign ulcerations of the duodenum° Can Meal Assoc J 1952;67:665-6. 12. Ruby RH. Giant benign ulcer of the duodenum. Mo Meal 1953; 50:357-8. 13. Herrma~n SF. Giant duodena| ulcer. West J Sur9 1957;65: 239-40, 14. Plnck RL, Held BT. Giant ulcers or walled-off perforations of the duodenum. N Engl J Meal 1961;264:541-3. 15. Klrsh IE, Brendel T. The Importance of giant duodenal ulcer. Radiology 1968;91:14-9. 16. McCracken JD, Davenport DL. Carclnoid of the stomach with giant duodenal ulcer. Am J Surg 1965;110:776-9. 17. Marshal( RH, Maklansky D. Giant benign duodenal ulcers. Am J Dig Dis 1963;8:305-10. 18. Knutsson F. Die Nischendiagnostlk be~ sehr grossen ctuodenalgeschwuren. Acta Radiol 1932;13:35-40. 19, Vallejo EA. Las ulCeras glgantes del estoruago y del duodeno. Cllnica de la perforaclon cronica. Rev Esp Enferm Apar Dig 1955;14:21-36. 20. Crick A. Giant duodenal ulcer. J Coil Radiol Australas 1961; 5:75-6. 21. Lemalre A, Blanchon P, Ementl I, et al. UIcere giant di bulbe duodenal: a propos de deux observations. Presse Med 1966;74:1233-6. 22. Bruni H, DeMouy EH, Baiart LA, McHardy G. Response of giant duodenal ulcer to medical therapy: report of a case. Am J Dig Dis 19~39;14:914-21, 23. Calonje MA, Ochsner SF. Unusually large duodenal ulcers. South Med J 1969;62:763-6. 24. Elsenberg RL. Giant duodenal ulcers. Gastrolntast Rad|ol 1978;2:347-53. 25. Rosenquist CJ. Clinical and radiographic features of giant duodenal ulcers. Clin Radlol 1969;20:324-8. 26. Morrow CE, Mutholland MW, Dunn DH, et at. Giant duodenal ulcer. Am J Surg 1982;144:330-1. 27. Segal I, Solomon A, Ou Tim L, Lawson HH, Sottomayer MCCG. The giant duodenal ulcer syndrome. S Aft Med J 1977;52: 724-7. 28. Bennett JM. Modified Bancroft procedure for the duodenal stump. Arch Surg 1972;104:219-22. 29. Bancroft FW. A modificatior~ of the Devine operation of pyloric exclusion for duodenal ulcer. Am J Surg 1932;16:223-30.
361
Giant duodenal ulcer, a variant of peptic ulcer disease, is generally defined as a full-thickness ulceration at least 2 cm in diameter, usually involving a large portion of the duodenal bulb. These ulcers are unique in that they are easily missed on upper gastrointestinal series and are associated with high morbidity and mortality rates. In recent years the diagnosis is being made much more frequently because the disease is becoming more familiar to the surgeon and radiologist. Medical management of giant duodenal ulcer is fraught with complications and is only rarely successful. Since Brdiczka [1] first called attention to this entity in 1931, 196 cases have subsequently been reported. Most investigators agree that early diagnosis along with early surgical management are the mainstays of reversing the dismal prognosis of this disease. Giant duodenal ulcer is often associated with a long history of neglected duodenal ulcer disease, with massive hemorrhage, obstruction, and walledoff perforation being common presenting features. These ulcers are penetrating, usually involving more than 80 percent of the duodenal bulb and may be adherent to the pancreas, liver, colon, and other adjacent structures, thus making surgical management difficult. This study reviews our experience with 32 patients diagnosed with giant duodenal ulcer at the University of Cincinnati Medical Center between 1963 and 1982. Medical and surgical therapies are compared and symptoms, clinicalfeatures, management, morbidity, and mortality are discussed. Material and Methods
All patients at the University of Cincinnati Medical Center and the Cincinnati Veterans Administration Medical Center with the diagnosis of giant duodenal ulcer from 1963 through 1982 were identified and their case records and follow-up reports reviewed. A giant duodenal ulcer was so defined if it measured 2cm or larger in greatest diameter by radiologic examination, endoscopy, necropsy, or at surgery. If the diagnosis was made by radiologic methods or no histologic examination was made, a 3 year clinical follow-upwas necessary to avoid including cancers. From the Department of Surgery, University of Cincinnati Medical Center. Cincinnati. Ohio. Requests for reprints should be ad~essed to Michael S. Nussba~n, ME), Department of Surgery, University of Cincinnati Medical Center, 231 Bethesda Avenue (ML/fS58), Cincinnati, Ohio 45267.
Vol.n~ 149,March1985
All patients were evaluated for symptomatic, diagnostic, therai~,.~,'ic,short-term, and long-term follow-up data. Thirty-two patients were identifiedwith documented giant duodenal ulcer. Twenty-four of the patients (75 percent) were men and 23 (72 percent) were white. The mean age of the patientswas 59 years (range30 to 81 years). Twenty-seven patients (84 percent) presented with epigastricpain. Gastroinestinalhemorrhage was a presenting symptom in 24 patients (75 percent).Twenty-four patients (75 percent ~ complained of nausea and vomiting, with 9 " patients (28 percent) having documented gastric outlet obstruction.Marked weight lossoccurred in 11 patients(34 percent), back pain was noted in 8 patients (25 percent), and 3 patients (9 percent) presented with a free perforation.In addition, 14 patients (44 percent) relateda history of peptic ulcer disease.Fourteen patients (44 percent) in our serieshad a history of ethanol abuse. Serum albumin was measured in 27 patientsand ranged from 1.4 to 4.8 g/dl (mean 3.3 g/dl).Acid secretory data were obtained in eight patients.Basal acid output ranged from 1.7 to 21.4 mEq[hour (mean 9 mEq/hour), and maximal acid output ranged from 24.1 to 140 mEq/hour (mean 52.9 mEq/hour). Serum gastrin was measured in five patients,and values ranged from 50 to ]31 pg{ml (mean 92 pg/ml; normal islessthan 160 pg/ml). None of the patients with acid hypersecretion demonstrated abnormal serum gastrin or calcium values as seen in the Zollinger-Ellison syndrome or subsequently was shown to have the Zollinger-Ellisonor multiple endocrine neoplasia syndrome. The diagnosisof giant duodenal ulcer was made by upper gastrointestinal series in 24 patients. Endoscopy documented or confirmed the diagnosi3 in 11 patients. Three patients were diagnosed at surgery and one at necropsy. The diameter of the ulcers ranged from 2 to 6 cm (mean 3.5 cm). Twenty-four patients were initially managed medically (mean length of treatment 41 months), primarily with antacids and diet. Cimetidine was utilized alone or in conjunction with antacids and diet in 10 patients. The mean length of treatment with cimetidine was 18 months. There were two deaths due to hemorrhage (8 percent), two unrelated late deaths (8 percent), and 20 recurrences (83 percent) in the medical group. Twenty-seven operations were required in 25 patients. In 17 of these patients, medical treatment failed, 7 of whom required emergency surgery. Eight patients were managed primarily by surgery, five emergently. There were three deaths in the surgical group (12 percent), all after emergency surgery. In two of the three, medical treatment had failed. There were no deaths among the elective surgery group. There was a totalof 12 surgical complications (41 percent) with a mean follow-up period of 35 months. There
357
Nussbaum and Schusterman
TABLE I
Surgical Rq~ults
Procedure Truncal vagotomy and antrectomy, BIIIroth II (n = 12) Truncal vagotomy and pyloroplasty (n = 7) Truncal vagotomy and antrectomy, Billroth I (n ffi 3) Truncal vagotomy,
Emergency
Mean Follow-Up (me)
Asymptomatlc
Recurrence
2
37
8
0
7
43
5
0
0
2
0
24
3
0
1 (short-term)
0
1
35
2
0
1 (lntraoporative)
0
2
1
0
2
0
0
gastroenterostomy (n = 3) Exploration, oversewing of ulcer (n = 2)
was one intraoperative complication: an iatrogenic splenic injury requiring splenectomy. Five patients had six short-term complications including postprandial nausea and vomiting, gastric outlet obstruction (two patients, one resolved and one required reoperation), wound infection, pulmonary embolus, and recurrent epigastric pain. There were five long-term complications including incisional hernia, esophageal stricture, intermittent nausea and vomiting, small bowel obstruction, and afferent loop syndrome. Twenty-five of the 27 operative procedures were definitive acid-reducing operations including vagotomy and antrectomy with Billroth II reconstruction (12 patients), vagotomy and pyloroplasty (7 patients), vagotomy and antrectomy with Billroth I reconstruction (3 patients), and vagotomy and gastrojejunostomy (3 patients). A duodenostomy tube was utilized in 5 of the 12 Billroth lI operations without any complications. Two patients underwent emergency exploration and oversewing of a perforated giant duodenal ulcer alone. Both of these patients subsequently required a more definitive operation because of recurrent symptoms (Table I). Comments
Since Brdiczka [1] first described giant duodenal ulcer 51 years ago, several salient features have been emphasized in the literature. Diagnosis is often elusive unless specific diagnostic and roentgenologic criteria are followed. Failure to recognize this condition results in a high mortality, usually from hemorrhage, as is evident from the early case reports. By 1957, only 7 of 21 reported patients had survived, giving the disease a mortality rate of 67 percent [2]. Successful noninvasive treatment is rare [3-6]. Although the morbidity and mortality rates have decreased since 1957, the condition is usually fatal unless an accurate diagnosis is made and surgical treatment implemented. Early- diagnosis is the cornerstone of therapy. Giant duodenal ulcer is defined as an ulcer located
358
Complications 8 (5 long-term, 3 short-term)
Mortality 1
in the first part of the duodenum, accurately measured to be greater than 2 cm in diameter, and histologically studied at surgical exploration, extirpation, or autopsy. If the diagnosis is made by radiologic methods or no histologic examination is made, a 3 year clinical follow-up must be carried out to avoid including cancers [2]. The differential diagnosis of giant duodenal ulcer includes lymphosarcoma, carcinoma, and gastric ulcer [3]. The large majority of patients reported in the literature are men (approximately 80 percent) with an average age of 55 to 60 years. The patients commonly present with a history of long-standing ulcer symptoms that respond poorly to medical management. Because of the anatomic location, perforation usually occurs as a slow penetration so that adjacent structures or organs wall off the area of destroyed duodenal wall. At onset, epigastric pain often is more intense than in the usual ulcer patient, and as the ulcer burrows into the pancreas and adjacent structures, the pain may be referred more to the right upper quadrant or posteriorly into the back. It is generally not relieved by food or antacids, which is understandable in view of the size and penetration of the ulcer [2,3]. Gastrointestinal hemorrhage is another common presenting feature. The gastroduodenal artery has often been found in the middle of the ulcer bed, actively hemorrhaging at operation. Either partial or complete obstruction of the duodenal segment distal to the ulcer crater, manifested by vomiting, weight loss, and malnutrition, is another presenting problem. The cachexia can be so prominent with the association of an inflammatory mass in the epigastrium, that carcinoma may be the initial diagnosis in these patients. Most of the patients present to the hospit~ with chronic illness, weight loss, and abdominal pain. If they present as an emergency, it is usually for massive hemorrhage from
The AmeriCan Journal of Surgery
Giant Duodenal Ulcer
the gastroduodenal artery. All of these symptoms are related to the natural progression of the disease and were seen often in this series. In addition, 14 patients in our series had a history of ethanol abuse, and two operative deaths resulted directly from postoperative hepatic failure. Various investigators have pointed out that the highest mortality occurs in giant duodenal ulcer patients who remain undiagnosed. Mortality is decreased in those patients diagnosed intraoperatively and is lowest in patients accurately diagnosed preoperatively [2-I 7]. The upper gastrointestinal series, in conjunction with endoscopic confirmation and biopsy, is the most important tool for making the diagnosis of giant duodenal ulcer. The findings on roentgenologic exan~ination is by far the most discussed subject in the literature concerning giant duodenal ulcer. Since Brdiczka [I] first described misdiagnosis by upper gastrointestinal examination in two of his three original cases, the literature has been full of case reports of similar occurrences [2,5,7-14,18-21). However, as awareness of this lesion increased, certain diagnostic criteria were established that not only led to earlier diagnosis, b u t also demonstrated that the disease was not as rare as initially believed. The radiographs of this lesion have been so deceptive to radiologists and other physicians that the number of unreported cases must be large [15]. The major problem with the diagnosis of these ulcers is that they are so large they may resemble a normal or slightly abnormal bulb that may be considered a deformity rather than a giant ulcer. The radiologic diagnosis is subtle, the salient points being fixation of the duodenal bulb and lack of normal peristaltic waves; constancy in size and shape of the ulcer; loss of the mucosal pattern about the crater; a tendency to stand out as the duodenum empties the barium (Figure 1); multiple filling defects resembling polypoid tumors (pathologically, these represent either necrotic pancreas, granulation tissue, or blood clots); air-fluid level in the ulcer crater; a patulous or partially destroyed pylorns; and obstruction or spasm of the duodenum distal to the ulcer due to inflammation and edema [1-3]. These criteria have been refined and developed over 30 years and are the mainstay of early diagnosis. During endoscopy, the pyloric sphincter is widened, edematous, and hyperemic. In addition, it is often less mobile than normal. The ulcers are so large that they can be clearly seen through the pyloric sphincter. On passage through the pylorus, soft necrotic slough is seen extending circumferentially around the whole mucosa, and virtually replaces the duodenal wall. The major differential diagnosis of giant duodenal ulcer is pseudodiverticulum of the bulb or a true diverticulum of the postbulbous area and a carcinoma or lymphosarcoma of the duodenal bulb. The diver-
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Figure I. Radlologlc ~fagnos~ The uk:~r haB a tendency to stand out a.r the duodenum mmp#eB lie# barium.
ticulum can be diagnosed by the observation of the changing size and shape of the crater. The neoplasms are extremely rare, but the distinction can be made at the operating table or by endoscopic biopsy. Successful medical treatment has been the exception in the past. There is only one long-term asymptomatic surviving patient in the literature, reported by Lumsden et al [6] and followed for 6 years. Other reports of successful medical management by Calonje and Ochsner [23], Dawson [5], Bruni et al [22], and Eisenberg [24] were followed only for 2 weeks to 6 months. There are many reports of patients initially treated medically who died from sudden massive hemorrhage or required emergency operations for bleeding or free perforation. Since the ulcer is literally a walled-off through-and-through perforation and involves more than 80 percent of the duodenal bulb, the long-term ability of the duodenum to function adequately after healing must be questioned, if the patient does not die from the complications. Medical therapy does play an adjunctive role as a prelude to surgery, its aim being to improve the patient's general condition and allow time for any in-
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flammatory swelling around the ulcer to subaide [6]. Most of these patients are malnourished and total parenteral nutrition may be indicated. Cimetidine and antibiotics are also helpful in reducing the inflammation and edema so that safe resection can be carried out~ Surgical treatment of this condition is not without complications. In the 1963 series by Mistilis et al [3] from our institution, 10 of 14 patients were operated on for giant duodenal ulcer, 3 of whom died due to problems with the duodenal stump. In our present series, there were three deaths and the complication rate was 41 percent, which correlates with the decreasing mortality rate demonstrated in the literature. The death rate in the 1940s and 1950s was extremely high, but by 1966 the overall mortality rate was 41 percent (15 of 37 patients), and by 1969 44 more patients had presented with only 8 deaths (18 percent). The most recent literature describes 57 more patients with 4 deaths, for a mortality rate of 7 percent [6,15,21,23,25-27]. The progressive decline in the mortality rate can be attributed to numerous factors. Improvements in radiologic equipment (such as image intensification), the advent of fiberoptic endoscopy, better preoperative and postoperative nutritional support, and improved surgical and anesthetic techniques have led to the increasing number of early diagnoses, earlier surgical intervention, and improved management of these patients. Most investigators agree that vagotomy and gastric resection with removal of the involved duodenum is the procedure of choice when possible. Frequently, however, the duodenal mass is so inflamed and indurated that duodenal dissection would be hazardous. Management of the duodenal stump has been controversial. If the inflammation and edema are markedly decreased, a Billroth I reconstruction can occasionally be performed safely. If there is still a large inflammatory mass in the duodenum at the time of exploration, truncal vagotomy and gastrojejunostomy may be the best alternative. Because of the size of the ulcer bed in some of these patients, many surgeons recommend that the ulcer bed be left in situ, resecting the duodenum around the ulcer bed and oversewing and decompressing it with a duodenostomy tube. Klamer and Mahr [4] have advocated rite Bennett. modification of the Bancroft procedure in managing the difficult duodenal stump and have reported success. This entails antrectomy, but the distal margin of resection leaves 3 to 4 cm of antrum and the pylorus intact. The antral mucosa is then carefully stripped from the muscle of the duodenum. A pursestring suture is then used to close the pylorus from inside, and the wall of the antrum is closed in layers. The most important principle is preservation of the distal antral blood supply because necrosis is a risk during this procedure [28,£9]. We believe that the Bancroft procedure, although a viable option, is
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time consuming and generally not warranted in such high-risk patients. Our data support the use of duodenostomy as a safe and effective means of managing the difficult duodenal stump so often encountered in giant duodenal ulcer disease. A duodenostomy tube was utilized in 5 of the 12 Billroth II operations in our series..Infour of these patients complications developed; however, none was directly due to the duodenostomy and there were no deaths. The higher incidence of complications in patients in w h o m duodenostomy was utilized can be explained by the fact that, by definition, these patients usually had more extensive, longer-standir,g ulcer disease and were, therefore, more debilitated and generally poorer surgical risks. In conclusion, our results concur with those in the literature and indicate that giant duodelml ulcer should be primarily managed surgically. These patients do very poorly with medical therapy, and the mortality rate is increased if emergency surgery is required for acute hemorrhage. Medical treatment alone is ~az~sociatedwith a bigh morbidity but is useful as an adjunct to surgery. The success of the treatment of this dangerous variant of duodenal ulcer depends on accurate prepperative diagnosis and preparation and appropriate operative therapy. Should operation be required, a definitive acid-reduction operation is the procedure of choice. Duodenostomy is advocated as a safe alternative to management of the difficult duodenal stump.
Summary Giant duodenal ulcer is a variant of peptic ulcer that is 2 ~m in diameter or greater and essentially replaces the duodenal bulb. Diagnosis by upper gastrointestinal series is often missed, due to the large size of the ulcer, which causes it to look like a scarred duodenal bulb or duodenal diverticulum. This study reviews our experience with 32 patients who presented with giant duodenal ulcer between 1963 and 1982. Seventy-five percent of the patients were men between 30 and 81 years of age (mean age 59 years). Gastrointestinal hemorrhage was a presenting symptom in 75 percent of the patients and free perforation in 9 percent. Diagnosis was made by upper gastrointestinal series (24 patients), and endoscopy (11 patients), alone or in combination. Three patients were diagnosed at surgery and one at necropsy. Mean size of the ulcer was 3.5 cm in diameter, range 2 to 6 cm. Twenty-four patients were initially managed medically (me~m length of treatment 41 months), with 2 deaths (hemorrhage) and 20 recurrences (83 percent). Twenty-seven operations were required in 25 patients. In 17 of the 25, medical treatment had failed. Seven of these patients required emergency surgery. Eight patients were managed primarily by surgery, of whom five presented emergently. There were three deaths in the surgical group after emergency surgery. In two of these patients, medical
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Joum~ of $ ~ z g ~ ,
Giant Duodonai Ulcer
treatment had failed. There were no deaths among the elective surgery group. Twenty-five of the 27 operative procedures were definitive, acid-reducing operations (15 vagotomy and antrectomy and 10 vagotomy and drainage). Two patients underwent emergency exploration and oversewing of a giant perforated ulcer alone, and both patients required subsequent surgery because of symptoms. The results indicate that giant duodenal ulcer should be primarily surgically managed and that an acid-reducing procedure should be performed during primary surgery. These patients do very poorly with medical therapy, and the mortality rate is increased if emergency surgery is required for hemorrhage. Medical treatment alone is associated with a high morbidity (92 percent). Should operation be required, a definitive acid reduction operation is the procedure of choice. Acknowledgment: We thank Josef E. Fischer, MD for his encouragement and counsel.
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Ann Surg 1951;133:417-20. 10. Bullock WK, Snyd~" EN Jr. Benign giant duoderml ulcer. Gastroentoroiogy 1952;20:330-6. 11. Hamel JF. Giant benign ulcerations of the duodenum° Can Meal Assoc J 1952;67:665-6. 12. Ruby RH. Giant benign ulcer of the duodenum. Mo Meal 1953; 50:357-8. 13. Herrma~n SF. Giant duodena| ulcer. West J Sur9 1957;65: 239-40, 14. Plnck RL, Held BT. Giant ulcers or walled-off perforations of the duodenum. N Engl J Meal 1961;264:541-3. 15. Klrsh IE, Brendel T. The Importance of giant duodenal ulcer. Radiology 1968;91:14-9. 16. McCracken JD, Davenport DL. Carclnoid of the stomach with giant duodenal ulcer. Am J Surg 1965;110:776-9. 17. Marshal( RH, Maklansky D. Giant benign duodenal ulcers. Am J Dig Dis 1963;8:305-10. 18. Knutsson F. Die Nischendiagnostlk be~ sehr grossen ctuodenalgeschwuren. Acta Radiol 1932;13:35-40. 19, Vallejo EA. Las ulCeras glgantes del estoruago y del duodeno. Cllnica de la perforaclon cronica. Rev Esp Enferm Apar Dig 1955;14:21-36. 20. Crick A. Giant duodenal ulcer. J Coil Radiol Australas 1961; 5:75-6. 21. Lemalre A, Blanchon P, Ementl I, et al. UIcere giant di bulbe duodenal: a propos de deux observations. Presse Med 1966;74:1233-6. 22. Bruni H, DeMouy EH, Baiart LA, McHardy G. Response of giant duodenal ulcer to medical therapy: report of a case. Am J Dig Dis 19~39;14:914-21, 23. Calonje MA, Ochsner SF. Unusually large duodenal ulcers. South Med J 1969;62:763-6. 24. Elsenberg RL. Giant duodenal ulcers. Gastrolntast Rad|ol 1978;2:347-53. 25. Rosenquist CJ. Clinical and radiographic features of giant duodenal ulcers. Clin Radlol 1969;20:324-8. 26. Morrow CE, Mutholland MW, Dunn DH, et at. Giant duodenal ulcer. Am J Surg 1982;144:330-1. 27. Segal I, Solomon A, Ou Tim L, Lawson HH, Sottomayer MCCG. The giant duodenal ulcer syndrome. S Aft Med J 1977;52: 724-7. 28. Bennett JM. Modified Bancroft procedure for the duodenal stump. Arch Surg 1972;104:219-22. 29. Bancroft FW. A modificatior~ of the Devine operation of pyloric exclusion for duodenal ulcer. Am J Surg 1932;16:223-30.
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