Intraluminal Duodenal Diverticulum in the Adult N. G. ECONOMIDES, M.D.,* R. P. McBURNEY, M.D.,t F. H. HAMILTON,

Four patients with intraluminal diverticulum of the duodenum are presented and compared with those reported previously. This lesion is a rare congenital disorder that usually becomes symptomatic in adult life. It is located in the second portion of the duodenum within the lumen, extending distally. It has the appearance of a "thumb of a glove" and it is lined by mucosa on both surfaces. It develops between the fourth and eighth week of the embryo's life, but it increases in size during adult life. It usually presents with typical or atypical symptoms of peptic ulcer disease, but sometimes manifestations such as gastrointestinal bleeding, duodenal obstructions or pancreatitis may predominate and may be severe and life-threatening. The diagnosis is best made with hypotonic duodenography, which demonstrates the lesion as a barium coated pouch within the air filled duodenal lumen. The treatment of choice is duodenotomy and excision of the lesion. Proper identification of the papilla of the ampulla of Vater is important since this structure is often adjacent to the diverticulum. No recurrences have been noted in the three patients operated upon. C

ONGENITAL INTRALUMINAL DIVERTICULUM in the

adult is a rare disorder. Approximately 35 cases have been reported in the literature. We have had the chance to see, diagnose and treat 4 patients with this disease. They all presented with upper gastrointestinal tract symptoms of varying severity. A definite diagnosis was made with hypotonic duodenography. All but one had surgical treatment and were followed 6 months or longer after surgery. It was Silcock in 188518 who first recognized and described this anatomical abnormality from an autopsy specimen. His description was: "In the duodenum, 6 inches below the pylorus is a congenital septum Submitted for publication April 26, 1976. Resident, Surgery, Baptist Memorial Hospital, Memphis, Tennessee. t Asst. Prof. Surg. University of Tennessee Medical School. t Resident, Radiology Baptist Memorial Hospital, Memphis, Tennessee. Reprint requests: R. P. McBurney, M.D., 910 Madison Avenue, Memphis, Tennessee 38103. *

III,

M.D.t

From the Departments of Surgery and Radiology of the Baptist Memorial Hospital and the University of Tennessee, Memphis, Tennessee

which barely admitted the tip of the little finger. A pouch formed of mucous and submucous tissue projects downward into the lumen of the gut and roughly may be likened in size and shape to the thumb of a glove." In this paper we present four cases with lesions exactly similar to this description. Case Reports CASE 1. A 27-year-old woman was referred to the Baptist Memorial Hospital emergency room six days postpartum because of bilateral pneumonitis, pleural effusion and gastrointestinal bleeding. The history revealed that she had had a spontaneous delivery and had been free of any symptoms prior to that event. On physical examination, the patient had a moderately distended abdomen, diminished intestinal sounds, right upper quadrant tenderness and epigastric tenderness without rebound. No masses were present. Examination of the chest revealed bilateral moist rales and ronchi associated with decreased breath sounds in both bases. Her hemoglobin was 7.5 gm/100 ml, hematocrit 35%, white blood count of 13.100 mm3 with normal differential. The urinalysis was normal. During the hospital course the patient had two spells of gastrointestinal bleeding which dropped the hematocrit to 22%. She was transfused a total of 6 units of whole blood. The urine amylase was 10 times the normal value. The serum amylase went up to 854 units. Serum calcium dropped to 3.5 mg/l. Supportive treatment with nasogastric suction, intravenous fluid, electrolyte and blood replacement was effective. She was greatly improved 12 days after admission. At this point, two oral cholecystograms failed to visualize the gallbladder. An upper gastrointestinal series showed an intraluminal duodenal lesion. Hypotonic duodenography which was obtained two days later demonstrated clearly an intraluminal diverticulum located in the second portion of the duodenum (Fig. 1). She underwent laparotomy and a 9 cm long, 4 cm wide intraluminal diverticulum of the duodenum was found, arising 1.3 cm

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the rest of the gland had minimal changes. Intraoperative cholangiorevealed the right and left bile ducts running individually and merging with a long cystic duct one cm before entrance into the duodenum. Choledochotomy was done for transcholedochal identification of the papilla and protection of it during resection. No obstruction was found. The diverticulum was excised and the duodenum closed transversely. The postoperative course was uneventful. The patient was discharged on the twenty-first postoperative day. She was seen 6 months later and was asymptomatic with all laboratory values normal. An upper gastrointestinal series showed a patent duodenum with minimal deformity. CASE 2. A 52-year-old man was seen with a chief complaint of upper abdominal discomfort, poor appetite and some recent weight loss. The symptoms were suggestive of duodenal ulcer in that there was some burning pain in the epigastrium and some slight relief with antacids. The symptoms had been present for several months. He had numerous studies done which were negative except that the upper gastrointestinal x-ray showed what appeared to be an intraluminal diverticulum in the second and third portions of the duodenum. Hypotonic duodenography demonstrated the lesion clearly (Fig. 2). Surgery was advised and on March 29, 1975, he had resection of an intraluminal duodenal diverticulum and a plastic procedure to a circumferential stricture which was just proximal to the neck of the diverticulum. He did not have a duodenal ulcer and the diverticulum resection and Heinecke-Mikulicz duodenoplasty was all that was necessary. The ampulla was identified and was at least an inch proximal to the mouth of the diverticulum and did not appear to be related to it. Following the surgical procedure he did well and an upper gastrointestinal x-ray taken the day prior to dismissal showed a well healed operative site which had no evidence of any further stricture or diverticulum being present. He was asymptomatic 6 months later. CASE 3. A 51-year-old, Caucasian woman presented to the emergency room of Baptist Memorial Hospital with a 5 day history of acute epigastric burning pain, nausea and vomiting as well as two spells of melena. The past history revealed that she had been admitted to another hospital for the same symptoms 7 months prior to this episode. At that admission an upper gastrointestinal study revealed a duodenal ulcer. She had been on antacids and bland diet since that time. The physical findings were epigastric tenderness without rebound or guarding. On admission, she had positive stools for occult blood and a hematocrit of 31% with a hemoglobin of 8.0 gm/100 ml; the rest of the values were within normal limits. On the second hospital day, she underwent hypotonic duodenography which showed an intraluminal diverticulum of the duodenum (Fig. 3). She underwent surgery; duodenotomy and resection of the lesion were performed. The diverticulum measured 12 cm in length and 6 cm in breadth and contained undigested material. Interestingly gram

FIG. 1. Case 1. Hypotonic duodenography demonstrating intraluminal diverticulum in second portion of duodenum.

proximal to Vater's papilla and extending towards the third portion of the duodenum. The distal tip was found to be necrotic in its full width and it contained a piece of carrot which the patient recalled having eaten several days previously. An annular pancreas was found with changes compatible with subsiding pancreatitis;

FIG. 2. Case 2. Intraluminal

extending to portion of the

diverticulum the

third

duodenum.

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enough, there was an incomplete rotation of the colon with the cecum

I.. 11 i1 * |

S

Ow.

_.

_ i X

stopping in the right upper quadrant. The appendix was retrocecal and attached to the duodenum and had inflammatory changes. A pyloric channel chronic ulcer was found but left untouched. The duodenum was closed transversly. Six months later she was asymptomatic and an upper gastrointestinal series showed a patent duodenum and no active ulcer. CASE 4. A 31-year-old Caucasian man was initially seen in the outpatient clinic of the Kennedy Veteran's Hospital, complaining of nausea and vomiting after every meal. Duration of symptoms was one week. According to the history, he would vomit approximately 30 minutes after eating a small meal, but if he ate a full meal, he would vomit immediately. Between the food intake and the vomiting he would have epigastric pain and burning appeared after the vomiting occurred. He claimed good relief of the pain and the indigestion with antacids. An upper gastrointestinal study was done and showed deformity of the second portion of the duodenum "secondary to chronic duodenal ulcer." The patient was discharged on antacids and an ulcer diet. Ten months later, he presented again in the emergency room complaining of constant "fullness : i,_pressure" after every meal, relieved by vomiting. He had had two spells of tarry stools. His hematocrit was 33% and the hemoglobin was 8.1 gm/100 ml. His stools were markedly positive for occult blood. The rest of the laboratory studies were normal. He was admitted and gastroscopy did not reveal any lesion. His acid studies were normal. Upper gastrointestinal series did not show any change since the previous ones. Barium enema was negative. Hypotonic duodenography revealed an intraluminal diverticulum (Fig. 4). He was instructed to undergo excision of the lesion, but he refused surgical intervention.

~~~~~~~~~~~~~~~~~Pathogenesis

; s

The origin of an intraluminal diverticulum of the duodenum is not clear. Duodenal defects in general develop between the 30th and 60th day after the mother's

FIG. 3. Case 3. Large intraluminal diverticulum almost obstructing

last menstruation. At that time and when the embryo

the duodenum. qt

s:.

FIG. 4. Case 4. Intraluminal diverticulum filling a large portion of second and third parts of the duodenum.

-

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ECONOMIDES, McBURNEY AND HAMILTON

Orifice of Diverticulum

FIG. 5. Illustration shows the attachment and position of an intraluminal diverticulum. (Reproduced by permission of Mayo Clinic Proceedings)

between 8.5 mm and 25 mm in length, the primitive duodenum which contains a lumen is invested by mesenchyme which obliterates it completely. Thereafter, vacuoles begin to coalesce, reestablishing the lumen. Impaired and incomplete vacuolization of the obliterated lumen results in intraluminal anomalies.2'19 The diverticulum represents one of those anomalies. The fact that it manifests usually during the adult life, the severity of the symptoms that it can produce, as well as its relationship to the biliary and pancreatic systems, necessitate the differentiation and individualization of this lesion from the other congenital lesions of the duodenum. Some authors have given different names to this entity, such as diaphragm-windsock-web and incomplete diaphragms.16 This lesion may be a combination of a longitudinal diaphragm and an incomplete atresia. The distal ballooning develops later in life and is the cause of most of the symptoms. Most authorities, though, feel that it represents the ultimate of pulsion forces on a duodenal

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Ann. Surg. X February 1977

papilla was between 2 mm and 3 cm away so that special care had to be taken to avoid it. In all three of our operated patients the diverticula were attached approximately to the one fifth of the duodenal circumference although it has been reported that the attachment can involve the entire circumference.7 Many other anatomical anomalies have been described to co-exist with intraluminal duodenal diverticula such as choledochocele, annular pancreas, double diverticula, malrotation of intestines and mongolism. There is a 40%o incidence of coexistant anatomical abnormalities16; this figure suggests strongly the congenital transfer of the disease.15 Our first patient had also an annular pancreas, as well as a left bile and right bile duct running individually to merge with a long cystic duct just one cm before the entrance to the duodenum. Our third patient had an incomplete rotation of the colon, with the cecum stopping where the hepatic flexure normally is and the appendix came off retrocecally and was adherent to the duodenum. In some of the reported cases foreign bodies have been found within the blind end of the diverticulum. These findings include gallstones, a penny'0 and residual food. Our first patient had a small piece of undigested carrot. The size of the lesion varies. It has been reported in the literature as long as 7 cm.6 In our cases the length varied from 4 to 12 cm and the width between 4 and 6 cm. The entrance of the pouch was large, in all cases measuring from 2 to 3 cm. The size of the blind end was found constantly smaller than that at the neck. The duodenum was not enlarged in any of our cases, although it can be not only massively enlarged, but also elongated with incompetence of the pylorus, leading to gastric dilatation.'2

Physiology The diverticulum, due to the lack of innervation, does not produce peristaltic waves. It passively distends as much as three to four times the original size. This mechanism contributed to the explanation of the postprandial fullness that many of the patients with the disease complain of. The distended diverticulum diaphragm. 1416 acts also as a large foreign body in the lumen, apt to cause intermittent or permanent obstruction of the Anatomy entire lumen. Pancreatitis can be a result due either The diverticulum is a lesion located within the lumen to obstruction of the papilla or to a change of the hydroof the second portion of the duodenum, although in static balance, which promotes reflux of duodenal conone reported case it arose in the third portion and tent into the pancreatic duct." When the duodenum is extended to the ligament of Treitz" 6 (Fig. 5). empty, the diverticulum has a tendency to collapse; In three of our four cases (the last one was not treated this explains the asymptomatic periods. The movability of the lesion is proportioned to the surgically) the diverticulum was found deriving from the wall forming the outside of the C-loop. Vater's length of the distal portion, which is not attached to

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ulum20 or elsewhere in the gastrointesintal tract is not common. Only one of our cases had a mucosal ulcer with necrotic bed right at the tip of the distal portion Pathology which was ready to perforate. The pathogenesis of this of ulcer should be different than that of the peptic; type In all specimens mucosa lining was found in both this is a result of ischemic necrosis due to we believe surfaces as well as a muscular layer consisting of fine, of the area and the traumatic vascularity poor the smooth fibers underlying the epithelium. Innervation of food. This condition reresidual by caused pressure the diverticulum was not found. No individual blood in diverticulitis, thus occurring changes sembles the supply, such as a vascular pedicle to the lesion was could be used. diverticulitis" "intraluminal the term identified. As a matter of fact, the vascularity was very which was ulcer channel a pyloric third had patient Our poor; this finding sugests that the areas of the diverticnot acute. ulum that are exposed to high pressure are prone to Physical findings are usually atypical and consist of necrosis, as we found in our first case where the distal tenderness to direct palpation unless one Qf epigastric one cm was necrotic and almost perforated. Furthermore, of the disease predominates. manifestations other the based on this mechanism, there is a possibility that longitudinal diaphragms of the duodenum in adults Diagnosis were first diverticula which sometime in life perforated to convert spontaneously to a different type of anatomical The diagnosis is made based on certain radiologic situation. findings. Potential malignant changes have not been reported. Plain abdominal films might show obstruction at the level of the duodenum but then no differentation can Symptomatology be made as to the causing factor. An upper gastrointestinal series will -sometimes show decade until the third Symptoms usually do not appear the lesion within the C-loop. It appears as a barium have disease of life, though 20% of the patients with the filled sac surrounded by barium so that only the mucosa had symptoms since childhood. as a radiolucent band.'3 If the diverticulum is is seen Our youngest patient was a 27-year-old Caucasian woman and the oldest a 51-year-old Caucasian woman. not filled with barium it might give the appearance of In the literature the youngest patient was 11 years old a pedunculated polyp.14 In one of our cases there was deformity of the lateral while the oldest was 76 years old. It appears that there is a slight sex predominance in favor of males,14 although aspect of the C-loop, which raised the question of duodenal abnormality, but the definitive diagnosis was our patients were two females and two males. In general, symptoms are vague and consist of post- not obtained until hypotonic duodenography was perprandial epigastric pain and fullness which are present formed. This roentgenologic procedure demonstrated in 90% of the cases. Vomiting is present in about one- the lesion in all our cases. If the diverticulum is suspected from symptomatology third of the patients. It is postprandial and sometimes contains old food. The period of time from the onset or on upper gastrointestinal series, hypotonic duodenogof symptoms until the patients first consult a physician raphy should be obtained. This study is done by coating varies from a few months to 15 years. About 50% of the mucosa with thick barium and insufflation of the all patients reported had had symptoms for more than C-loop through a gastric tube inserted in the second two years. Our patients had a 3 to 9 month history before portion of the duodenum. Glucagon or probanthine is used intramuscularly to abolish peristaltic activity. they were seen by us. Gastrointestinal bleeding is present in 25% of the The appearance of the hypotonic study is a barium reported cases6 although in our experience it was the coated pouch within the air filled duodenal lumen. Endoscopy was unsuccessful in detecting the lesion dominant symptom that brought three of our patients in all of our cases, although there has been successful to the emergency room. Pancreatitis is present in about 20o of cases.14 Our diagnosis reported by our authors. We believe that first patient had pancreatitis which in addition to the failure of this method is often due to the fact that other complications proved to be very severe and life the scope is passed into the lumen of the large diverticulum, which, lined by mucosa, appears as normal threatening. Weight loss is attributed to the food intolerance that duodenal lumen. Some authors have studied these lesions with these patients have. It does not appear to be due to loss of fluids or proteins from the diverticulum. cineradiography.15 Kaltori and his colleagues were able Coexistence of ulcer located either within the divertic- to demonstrate a lesion with intravenous cholangiogram.8

the duodenal wall. In our series this distance was found to be between 3 to 6 cm.

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anterior gastroenterostomy at the age of 6 days for what was thought to be duodenal atresia. The patient conSince the manifestations and complications of this tinued to have problems and at the age of 24 years lesion are due to its anatomical existence, the only roentgenograms showed a typical diverticulum. He treatment is complete surgical removal. Of course, underwent excision, which relieved his symptoms conservative medical treatment has been tried and good results have been achieved in about 40o of those permanently. instances. However, because of the high recurrence Acknowledgment rate of symptoms, conservative treatment should be used only for the poor risk patient or as a temporary measure. The authors wish to acknowledge with appreciation the permission The procedure of choice is duodenotomy and excision of the Mayo Clinic Proceedings to use Figure 5 and also permission of the diverticulum.4'16 The duodenum is exposed first of Dr. James Alston, Memphis, Tennessee, to report Case 3. through an appropriate incision. A Kocher maneuver is necessary to obtain optimal mobilization of the References duodenum. An attempt is made to feel the lesion. From G.: Angebroene Membranstenosen in Duodenum Beim previous reports this can be done. In our cases we 1. Bargon, Erwachsenen mit Intraduodenaler (Intraluminarer) Pseudowere unable to feel the lesion from the outside. This diverticikelbildung. Fortschr. Geb. Roentgenstr. Nuklearmed., 100:319, 1964. is due to the mucosal consistency of the diverticulum 2. E. A., Cope, J. G. and Bill, A. H., Jr.: Anatomy and Boyden, as well as to its tendency to collapse when empty. Embryology- of Congenital Intrinsic Obstruction of the Anterior duodenotomy is done next. The first impression Duodenum. Am. J. Surg., 114:190, 1967. of the surgeon at this point might be that of duplication 3. Brunton, F. J. and Bamforth, J.: Intraluminal Diverticulum of the Duodenum and Choledochocele. Gut, 13:207, 1972. of the lumen. This, though, can easily be ruled out by 4. Cooperman, A. M., Adachi, M., Rankin, G. B. and Sivak, M.: finding the lumen with the blind end. Identification of Congenital Duodenal Diaphragm in Adults. Ann. Surg., 182:739, 1975. the papilla of Vater is the next important thing. After 5. Coors, G. A. and Mitchum, W. R.: Intraluminal Duodenal this is done, excision of the diverticulum from its Diverticulum. Am. J. Surg., 103:400, 1962. mucosal attachment is performed without difficulty. 6. Fleming, R. C., Newcomer, A. D., Stephens, D. H. and Carlson, H. C.: Intraluminal Duodenal Diverticulum. Mayo If the papilla cannot be recognized and protected, Clin. Proc., 50:244, 1975. choledochotomy should be done prior to the excision. 7. Heilbrun, N. and Boyden, E. A.: Intraluminal Duodenal A probe through the common duct will easily demonDiverticula. Radiology, 82:887, 1964. strate the ampulla. Excision then is safe with the ampulla 8. Kaftori, J. K., Munk, J., Schramek, A. and Barzilai, D.: Intraluminal Diverticulum of the Duodenum Demonstrated cannulated. The mucosal defect can be closed then by Intravenous Cholangiography. Br. J. Radiol., 39:388, 1966. with absorbable suture. 9. Levi, A. J. and Kreel, L.: Congenital Intraduodenal Diverticulum. Proc. R. Soc. Med., 56:168, 1963. If feasible the duodenum is then closed transversly A. G., and Edgren, D. C.: Duodenal Obstruction by in two layers. If choledochotomy is done a T-tube is 10. Meyer, an Intraluminal Diverticulum. Arch. Surg., 103:1, 1956. placed in the common duct and is left for two to three 11. Nance, F. C., Cocchiara, J. and Kinder, J. L.: Acute Pancreatitis Associated with an Intraluminal Duodenal Diverticulum. weeks. Gastroenterology, 52:544, 1967. No deaths have been reported from the procedure. 12. Nance, F. C.: Intraluminal Duodenal Diverticulae. Surg. Gynecol. The operation does not have a higher risk than other Obstet., 124:613, 1967. 13. Nelson, W. L.: Congenital Diaphragm of the Duodenum; Case abdominal procedures. Report with Preoperative Study. Minn. Med., 30:745, 1947. Potential complications include injury of the biliary 14. Nosher, J. L. and Seaman, W. B.: Association of Intraluminal tract or the papilla, stricture of the common duct, panDuodenal Diverticulum with Acute Pancreatitis. Radiology, 115:21-22. creatitis, fistulas and abdominal abscesses. In all three F., Willemin, A. and Levy, C.: Diverticule Interne of our cases we did not experience any introperative 15. Pengola, de Duodenum. Arch. Mal. Appar. Dig., 53:844, 1964. or postoperative complications. The first patient had 16. Richardson, W. R. and Martin, L. W.: Pitfalls in the Surgical Management of the Incomplete Duodenal Diaphragm. J. Pediat. choledochotomy and transcholedochal identification of Surg., 4:303, 1969. the ampulla. All three patients were free of symptoms 17. Rouffiat, J., Zangumier, J. and Langumier, J. F.: Les Diverticules when they were discharged. The first three patients Endoluminaux du Duodenum; A Propose de Deux Cas. Ann. Radiol., (Paris) 14:871, 1971. were seen again up to 6 months and they were asymp18. Silcock, A. Q.: Epithelioma of the Ascending Colon: Enterocolitis; tomatic. Congenital Duodenal Septum with Internal Diverticulum. Other procedures have been suggested; they are Tr. Path. Soc., Lond., 36:207, 1885. bypass procedures like gastroenterostomy, duodeno- 19. Tandler, Y.: Zur Entwicklungsgeschichte des schilichen Duodenums in Fruhen Embryonalstac: Morph. Jahrb., 29:187, 1902. enterostomy,16 therefore, the lesion is not removed. 20. Zatzkin, H. R., Macy, J. J., Kveton, F. W.: Intraluminal These operations have been attempted because of misDuodenal Diverticulum: Report of a Case: Am. J. Roentgenol. Radium Ther. Nucl. Med., 103:326, 1968. judgement of the lesion. Levi and Kreel9 reported an Treatment