Congenital Duodenal Anomalies in the Adult

PAPER Congenital Duodenal Anomalies in the Adult

Alan P. Ladd, MD; James A. Madura, MD

Background: Duodenal anomalies are defects in em- Main Outcome Measures: Surgical outcomes includ- bryologic development and usually present as gastric out- ing postoperative complications, deaths, and resolution let obstruction in infancy or early childhood. Occasion- of preoperative symptoms. ally, they remain asymptomatic until adulthood and, because they are unusual, may not be diagnosed. Results: The treatment for patients with duodenal webs was transduodenal web excision and duodenoplasty in 19 Hypothesis: Based on current experience and review of 22. Patients with annular pancreata were treated by tran- of the literature, recognition of diagnosis and the pre- section of the annulus and duodenoplasty (n=4) and proxi- ferred methods of treatment of duodenal anomalies can mal duodenal bypass (n=3). There were no operative deaths, be recommended. but 44% of patients had some complications. No pancreatic fistulas occurred in patients who had division of an an- Design: Retrospective study of congenital duodenal nular pancreas. Outcome was considered excellent or good anomalies in adults. in 17 of 20 patients with duodenal webs, 4 of 7 with annular pancreata, and 2 of 2 with the combined anomaly. Setting: Tertiary care university medical center. Conclusions: Duodenal anomalies are rare in adults. Duo- Patients: Twenty-nine patients were observed and denal webs are best managed by transduodenal excision treated between 1983 and 1999 (19 women and 10 men; and duodenoplasty. Annular pancreas is generally best mean±SD age, 52±16 years). Twenty patients had duo- treated by duodenal bypass to the distal duodenum or the denal webs, 7 had annular pancreata, and 2 had both. Nau- jejunum. Annulus division can be carried out if the annu- sea, vomiting, abdominal pain, and weight loss were pre- lus is extramural, without duodenal stenosis, and if access dominant symptoms in all groups. Peptic ulceration to the pancreaticobiliary sphincters is necessary. occurred in 13 of 20 patients with webs but in none of those with annular pancreata or combined anomaly. Arch Surg. 2001;136:576-584

ONGENITAL duodenal ing duodenum. Most studies of these lesions anomalies are rare lesions are single case reports or small series, and originate in the early which do not allow a single surgeon to ac- embryologic development cumulate extensive experience; therefore, of the foregut. Whereas the reliance on the combined experience of primitiveC foregut undergoes lengthening and others in recognition and appropriate man- rotation, the hepatobiliary and pancreatic agement has been the norm. This article pre- anlagen begin as buds or diverticula at the sents a recent series of adult patients with middle of the duodenum and similarly grow duodenal webs, annular pancreata, or a and rotate. During this period, duodenal combination of these 2 anomalies. Recog- atresias, intraluminal webs, annular and ec- nition and a rational approach to correc- topic pancreata, and malrotations of vari- tion of these problems is based on the ap- ous types develop. The delayed presenta- propriate treatment for each patient and a tion of these anomalies in the adult is knowledgeable expectation of the out- difficult to explain, but the presence of a di- come based on the chosen therapy. lated stomach and a proximal duodenal From the Department of bulb with a patulous pylorus suggests a RESULTS Surgery, Indiana University progressive loss of compensatory peri- School of Medicine, staltic action to overcome a small duode- Congenital duodenal anomalies were iden- Indianapolis. nal aperture or narrowing of the descend- tified in 29 patients. Seven patients with

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 tients and intestinal malrotation, insulinoma, and para- esophageal hiatal hernia in 1 patient each, as well as the PATIENTS AND METHODS concomitant annular pancreas in 2 patients. Complica- tions included delayed gastric emptying in 2 patients, a Hospital records from January 1, 1983, to Decem- wound infection in 1, abdominal fluid collection in 1, and ber 31, 1999, were searched for patients at the Indi- a duodenal leak in 1. The latter 2 were successfully treated ana University Medical Center Hospitals, Indianapo- without surgery. lis, who were older than 18 years and had International Seven patients had annular pancreata (5 women and Classification of Diseases, Ninth Revision,1 codes for 2 men). Both of the combined anomaly patients were congenital small-bowel atresia (code 751.1), duode- women. Their symptoms also included epigastric pain nal web (code 751.5), and annular pancreas (code (n=5), nausea and vomiting (n=2), and weight loss (n=4), 751.7). Medical charts were reviewed for patient de- but there were no patients with peptic ulcer disease (Table mographics, past medical and surgical histories, signs 2). Medical and surgical histories showed 6 patients un- and symptoms at presentation, location and type of derwent cholecystectomy previously, without mention duodenal anomaly, diagnostic studies, operation performed, associated anomalies, complications, mor- of the annular pancreas. tality, and patient outcome. Upper GI barium studies demonstrated duodenal stenosis in 3 patients (Figure 3). Abdominal computed tomographic scans confirmed the diagnosis in only 1 patient. Five patients with annular pancreata duodenal webs have been reported previously.2 There were only were diagnosed before surgery by either upper GI 19 women and 10 men (mean±SD age, 52±16 years). endoscopy or endoscopic retrograde cholangiopancrea- Of these patients, 20 were diagnosed as having duode- tography (ERCP). Two patients were diagnosed only at nal webs, 7 as having annular pancreata, and 2 as hav- the time of surgery. Of the 2 patients with combined ing both (Table 1). webs and annular pancreata, 1 was diagnosed by upper The duodenal web group consisted of 14 women and GI barium study and 1 by percutaneous transhepatic 8 men, including 2 patients with both anomalies. Four cholangiogram. patients had previously undergone cholecystectomy, 2 Surgical interventions for 7 patients with annular had undergone antrectomy, and 3 had undergone gas- pancreata included division of the annulus with trans- trojejunostomy or duodenojejunostomy for gastric out- verse duodenoplasty in 3, duodenoplasty only in 1, duo- let obstruction. Most patients with duodenal webs pre- denoduodenostomy in 1, and duodenojejunostomy in 2. sented with nausea, vomiting, epigastric pain, and early The latter 3 had a complete annulus with a fibrotic satiety. The duration of symptoms ranged from 1 month narrowed descending duodenum that prohibited annu- to 50 years, with a mean duration of 7 years before di- lus division or duodenoplasty. agnosis. Nine patients with duodenal webs manifested Overall results in patients with annular pancreata weight loss of an average of 6.4 kg. In 6 patients, epi- were excellent in 4 and fair in 3. The outcomes in the 2 sodic upper gastrointestinal (GI) tract bleeding oc- patients with both anomalies were classified as good or curred that was attributed to peptic ulcer disease. Sub- excellent. There was 1 wound infection and 1 episode sequently, ulcers were noted in 12 of 20 patients with of pancreatitis in a patient who had duodenojejunos- webs (Table 2). tomy without division of the annulus. One patient had Preoperative evaluation consisted of numerous ra- symptomatic delayed gastric emptying but has since re- diologic and endoscopic studies, including barium con- covered. There were no deaths in these patients, and no trast upper GI tract studies (Figure 1 and Figure 2), pancreatic fistulae occurred in the 5 patients having di- computed tomography, and percutaneous transhepatic vision of their annulus. cholangiography (1 patient with jaundice), which nicely demonstrated a saccular wind sock web. Upper GI endoscopy was performed in 20 patients, with the correct COMMENT diagnosis being made in 9. Fourteen patients were diagnosed only at the time of operation. Congenital duodenal anomalies are rare in adults. In the The duodenal web was found to be preampullary in pediatric population, the incidence is estimated to be 1 20 of 22 patients and postampullary in 2. All but 1 web in 20000 to 40000 births, with incomplete obstructive had a single aperture, and that patient had an imperfo- lesions including duodenal webs accounting for only 2% rate wind sock web with a second eccentrically located of these defects. Ravitch3 estimated the adult incidence aperture. There were 17 central apertures and 5 eccen- of duodenal stenosis from annular pancreata to be 3 in tric openings, with sizes ranging from 0.5 to 20.0 mm. 20000 autopsies, and Naylor and Juler4 described this Surgical treatment consisted of transduodenal web anomaly in 2 of 20000 laparotomies at their hospital. A excision with transverse duodenoplasty in 16 patients, review of the available published literature discovered 160 excision of the web in conjunction with an extended py- cases of annular pancreata and 76 cases of duodenal webs loroplasty in 2, and resection of a previously undiag- in adults. Most are single case reports or small series. Thus, nosed web in 3 during antrectomy with gastrojejunos- the diagnosis has often been overlooked in the differen- tomy or revision of a Billroth II anastomosis to a Billroth tial diagnosis of gastric outlet obstruction in the adult, I gastroduodenostomy for bile reflux (Table 3). Asso- and most frequently webs, at least, have been mistaken ciated anomalies included pancreas divisum in 2 pa- as scarring from duodenal ulcer disease.

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Table 1. Demographic Characteristics of 29 Adults With Congenital Duodenal Anomalies, 1983-1999

Anomaly P Duodenal Web Annular Pancreas (AP) Both Characteristic (n = 20) (n=7) (n = 2) Web vs AP Web vs Both AP vs Both Sex, F/M 12/8 5/2 2/0 .49 .49 .49 Age, mean ± SD, y 53.1 ± 16.7 49.6 ± 11.6 39.0 ± 9.0 .62 .26 .28 Duration of symptoms, mean ± SD, mo 93.6 ± 164.1 11.7 ± 15.2 16.0 ± 2.0 .20 .52 .72

Table 2. Symptoms in 29 Adults With Congenital Duodenal Anomalies, 1983-1999

Patients, No. (%)

Duodenal Annular Both Web Pancreas Anomalies Symptom (n = 20) (n=7) (n=2) Upper abdominal pain 13 (65) 5 (71) 2 (100) Nausea and vomiting 17 (85) 2 (29) 2 (100) Satiety, fullness 12 (60) 0 1 (50) Gastroesophageal reflux 8 (40) 0 0 Weight loss 8 (40) 4 (57) 1 (50) Gastrointestinal bleeding 6 (30) 1 (14) 0 Ulcers 12 (60) 0 0 Jaundice 0 1 (14) 1 (50) Diarrhea 3 (15) 1 (14) 1 (50)

ANNULAR PANCREAS

The embryologic origin of the annular pancreas is pro- posed to be a defect in the normal rotation of the ventral pancreatic anlage to the right and dorsally to join the dorsal pancreas.5 It is thought that when this migration is imperfect, a ring of pancreatic tissue might be left around the second part of the duodenum (Figure 4). During this same process, failure of fusion of the dorsal and ventral pancreata leads to pancreas divisum. Baldwin,6 in 1910, reported that his studies suggested that a persis- tent left ventral bud was responsible for the encircling Figure 1. Upper gastrointestinal tract barium contrast study demonstrating of the descending duodenum by pancreatic tissue. It was a duodenal web in the preampullary region with an eccentrically placed postulated that in most individuals, the left ventral an- aperture (arrow). lage disappears completely and only the right ventral anlage rotates around the duodenum to join its dorsal coun- of the ductal orifice of the annulus is suggested to be simi- terpart. The annulus can completely or only partially lar to the cause of pancreatitis seen in pancreas divisum encircle the duodenum and might be loosely applied to alone.9 the serosal surface of the duodenum or, in other cases, intimately interdigitated with the muscularis mucosa of DUODENAL WEBS the duodenum. The pancreatic duct of the annulus has been observed to drain into either the intrapancreatic com- The accepted cause of intraluminal duodenal anomalies mon duct or the ventral duct of Wirsung, providing fur- such as atresias or webs arises from the seminal studies ther evidence that the right ventral pancreatic anlage alone of Tandler,10 a Viennese anatomist, in 1900. He studied is responsible for the annular pancreas. sections from eleven 8.5- to 20.0-mm human embryos An annular pancreas has been shown to be fre- and described a process of luminal obliteration by epi- quently associated with pancreas divisum, attesting to the thelial overgrowth. As the duodenum enlarges and timing of these defects during the same phase of early lengthens, vacuoles are formed that coalesce, and even- embryologic development. More recently, ERCP stud- tually a lumen is reestablished10 (Figure 5). This ies have demonstrated a much higher incidence of pan- theory has been supported by Lynn and Espinas,11 creas divisum in individuals with annular pancreata than Streeter,12 Boyden et al,13 and others in subsequent stud- in the general population.7,8 The role of relative stenosis ies. Boyden et al elegantly stratified this process and fur-

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 ther described a stage of fusiform dilation above and anomalies has resulted in great variability in the surgi- below the hepatobiliary and dorsal pancreatic outpouch- cal treatments carried out. During the past several de- ings. Subsequently, a wave of epithelial material clearing cades, however, new technology has produced the means passes from pylorus to jejunum, leaving a patent lumen to accurately diagnose the condition and to make accu- in the normal embryo by 12 weeks after gestation. Fail- rate plans for correction and treatment. ure to clear the epithelial plug completely can result in In the treatment of duodenal webs, the most accept- atresias or webs anywhere in the duodenum. This pro- able procedure currently is longitudinal duodenotomy cess is thought to be most faulty in areas of fusion or vis- followed by careful excision of the web—either com- ceral outgrowth, hence the propensity for the webs to pletely or partially—mucosal reapproximation, and sub- occur in the ampullary region. sequent transverse closure of the duodenum. In the early There are several varieties of webs: complete duodenal atresias or imperforate webs, intraluminal imperforate webs (wind sock webs), and perforated webs with either central or eccentric apertures. Although most webs have been observed in the preampullary or postampullary region, they have also been reported throughout the third and fourth portions of the duodenum. The juxta- ampullary position of the webs makes surgical or endoscopic extirpation a potentially dangerous procedure. Duodenal atresias or imperforate webs always present shortly after birth; long-term survival is not pos- sible with total duodenal obstruction, but, for the past 100 years, numerous case reports have been published describing duodenal webs and annular pancreata in the second, third, and later decades of life. The onset of symptoms in the adult seems to be the effect of a progressive decompensation of the peristaltic force of the stomach and proximal duodenum. This is attested to by marked dilation of the stomach and the first part of the duodenum as well as a patulous pylorus. Although the same mechanism might be responsible in the annular pancreas, several authors have documented acute pancreatitis in the annulus, causing high-grade duodenal obstruction, with subsequent resolution of the inflammatory process allowing relief of the obstruction.14 Surgical treatment of duodenal webs was not at- tempted until 1936 by Kreig,15 who performed a gastrojejunostomy, bypassing the duodenal obstruction. Vidal16 successfully treated a patient with an annular pancreas with gastrojejunostomy in the early 20th century. Many of the earliest reports are autopsy descriptions in patients with gastric outlet obstructive symptoms or inci- Figure 2. Hypotonic duodenography demonstrating a preampullary duodenal dental observations. The infrequent occurrence of these web with a central aperture.

Table 3. Treatment and Outcomes in 29 Adults With Congenital Duodenal Anomalies

Outcome

Surgical Treatment Patients, No. Complications, No. Excellent/Good Fair/Poor Duodenal Web (n = 20) Web excision with duodenoplasty 14 4 14 0 Web excision, vagotomy and pyloroplasty 2 2 1 1 Web excision, convert Billroth II to Billroth I 2 2 1 1 Web excision, vagotomy and antrectomy 1 0 1 0 Vagotomy and antrectomy, dilate web 1 0 0 1 Annular Pancreas (n = 7) Division annulus, duodenoplasty, and sphincteroplasty 3 0 1 2 Duodenoplasty 1 1 1 0 Duodenoduodenostomy 1 1 0 1 Duodenojejunostomy Roux-en-Y 2 1 2 0 Both Anomalies (n = 2) Web excision, division of annulus, duodenoplasty, and sphincteroplasty 2 0 2 0

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 might be required through the annulus. Preoperative endoscopy and ERCP will be revealing in this situation and assist in planning the operation. More recently, use of magnetic resonance cholangiopancreatography has been useful in making the diagnosis without the need for a more invasive procedure. In the early literature, transection or resection of the annulus frequently resulted in pancreatic fistula formation. Early surgeons did not have the advantage of radiologic studies to demonstrate the presence and location of the annular ductal system and did not recognize or control the transected duct. If the annulus is partial and not densely adherent to the duodenal serosa, and the duodenal segment beneath the annulus is not fibrotic and strictured, then dissection of the annulus, with or without complete removal, might be considered and transverse duodenoplasty carried out. A recent report19 of successful staple management of the transected annulus presents another surgical option in this condition. Otherwise, its seems safest to bypass the annular constriction by duodenoduodenostomy or duodenojejunostomy to the proximal duodenal bulb. Early articles in the literature frequently document gastric resection with posterior gastroenterostomy, but again this seems to be too much surgery and might predispose to postgastrectomy problems such as stomal ulceration, stricturing, dumping syndrome, bile reflux gastritis, and anemias. During the past 100 years, to our knowledge, 160 adults with annular pancreata and 76 with duodenal webs have been described. In the 76 patients with duodenal Figure 3. Upper gastrointestinal barium contrast study demonstrating the webs, there is no sex difference, and the mean±SD age smooth extrinsic narrowing of the second portion of the duodenum caused by an annular pancreas. at diagnosis is 51.5±17.1 years (range, 17-81 years). Pa- tients with annular pancreata had a slightly greater male incidence (ratio, 1.78:1). The mean age of patients with literature, bypass of the web by posterior gastroenteros- annular pancreata is 44.4±15.1 years (range, 17-79 years). tomy, duodenoduodenostomy, or duodenojejunostomy There seems to be a difference in duration of symptoms was carried out, but it has been largely abandoned. In before presentation between the 2 groups, with patients the face of concomitant ulcer disease, ulcer operations with webs averaging 137±208 months compared with such as vagotomy and pyloroplasty and antrectomy with 68±84 months for those with annular pancreata. Most gastrojejunostomy have also been performed, but these patients in both groups presented with signs of gastric procedures are more treatment than is needed. Relief of outlet obstruction, nausea and vomiting, upper abdomi- the duodenal obstruction should allow the stomach to nal discomfort, and early satiety. Patients in both groups regain its peristaltic function, and antral decompression were noted to have gastric or duodenal ulcers, and a few should diminish gastrin production, with return of gas- patients with annular pancreata presented with biliary tric acidity to normal levels and healing of ulcers. When obstruction and jaundice. web excision is performed, careful attention should be Gastric and proximal duodenal dilation are com- given to protection of the biliopancreatic sphincter mecha- mon to both groups on upper GI contrast studies, but in nism. By either leaving the periampullary portion of the patients with webs, a transverse diaphragm in the descend- web or intubating the bile duct and ampulla with a probe, ing duodenum with an eccentric or central aperture might injury to this structure can be prevented. Several re- be seen. Hypotonic duodenography is more likely to draw ports17,18 of endoscopic incision or Nd:YAG laser abla- the radiologist’s attention to such a finding than a usual tion of a web appear in the literature, but subsequent scar upper GI barium study. The annular pancreas, since it is formation has resulted in stenosis and the need for sub- an extrinsic lesion, will be seen as a smooth or tapered nar- sequent surgery. rowing of the second part of the duodenum. Endoscopic The embryologic development of the annular pan- examination will reveal only a circumferential narrow- creas also affects its clinical presentation, diagnosis, and ing, but an ERCP might delineate the ductal system of the eventual treatment. The “trail” of pancreas around the de- annulus and its junction with the ventral pancreatic and scending duodenum can be complete, partial, extramu- biliary ducts. Computed tomography and magnetic reso- ral, or intermingled with the duodenal muscular wall, caus- nance imaging have recently been shown to benefit the di- ing a fibrotic stricture. There is also an increasing awareness agnosis and overall management of these patients. of coexistent pancreas divisum in some of these patients, The presentation, location,and number of webs and a surgical approach to the ampulla and accessory duct is variable, with a preponderance of preampullary

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Figure 4. The origin of the annular pancreas patterned after Lecco.5 The primitive ventral pancreatic anlage rotates to the right and dorsally. A defect in rotation allows a “trail” of pancreas to envelop the duodenum ventrally.

Figure 5. Duodenal embryologic development according to Tandler10 showing the epithelial plugging of the lumen followed by vacuolization and eventual reestablishment of the duodenal lumen.

positioning in 70% of patients described. Of the remain- had multiple webs occurring in preampullary and post- ing webs, 25% were postampullary and 5% intra- ampullary positions. Several authors suggest passing a ampullary, ie, at the ampulla itself. Virtually all of these Foley catheter distally in the duodenum to ensure that webs had a single aperture, either central or eccentric. one or more distal webs are not overlooked. The evolu- Eighty-six percent of patients had single webs, and 14% tion of therapy for duodenal webs has been toward trans-

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 Table 4. Surgical Treatment and Outcomes in Adults Table 5. Surgical Treatment and Outcomes in Adults With Duodenal Webs, Literature Search, 1900-1999* With Annular Pancreata, Literature Search, 1900-1999*

Patients, Complications, Deaths, Patients, Complications, Deaths, Surgical Treatment No. No. No. Surgical Treatment No. No. No. None 8 3 7 None 19 1 5 Excision web + duodenoplasty 32 4 1 Divide or resect annulus 26 14 2 Excision web + vagotomy and 610 Divide annulus/ 100 pyloroplasty duodenojejunostomy Gastric resection Billroth II 6 1 0 Divide annulus/gastric 710 Excise web + 200 resection duodenojejunostomy Divide annulus/vagotomy 100 Gastric resection Billroth I 2 1 0 and pyloroplasty Duodenoduodenostomy or 831 Posterior gastrojejunostomy 19 1 2 jejunoduodenostomy Duodenojejunostomy 18 1 0 Vagotomy and pyloroplasty 310 Duodenoduodenostomy 4 0 0 gastrojejunostomy Vagotomy and 11 1 0 Incise web only 2 0 0 gastrojejunostomy Endoscopic/laser ablation 3 2 0 Gastric resection Billroth II 22 1 0 Gastric resection Billroth I 1 1 0 *Not all studies provided all the data included in this table. A complete Whipple 3 0 0 reference list is available from the author. *Not all studies provided all the data included in this table. A complete reference list is available from the author. duodenal web excision and duodenoplasty (Table 4). Fifteen percent of reported patients had proximal duodenal diversion, and 9% had partial gastrectomy with gas- visum, and combined anomalies will present challenges troenterostomy. The resections were done in the earlier to the surgeon, but knowledge of the anatomy and aware- years, and this might be because of lack of the ability to ness of potential complications should lead to more sat- make the diagnosis before surgery. In addition, many of isfactory outcomes. these patients had signs and symptoms of chronic pep- As a result of this extensive review of a single insti- tic ulcer disease and, therefore, the operation was in- tutional experience and an exhaustive literature search, tended to treat the ulcer diathesis. Complications oc- the following recommendations are made: curred in 16% of these patients, and most deaths were 1. In patients with duodenal webs, a transduode- in patients without surgical treatment. Complications of nal web excision should be carried out through a longi- web excision included pancreatitis, duodenal stenosis, tudinal incision that is then closed transversely in Heineke- and leaks. Symptomatic relief was observed in 61 pa- Mikulicz fashion. The ampullary structures should be tients (77%), and recurrent symptoms occurred in 4%, protected by either avoiding resection of the juxta- with stomal ulceration in 1 patient after gastroenteros- ampullary portion of the web or intubating the com- tomy without vagotomy. mon duct to protect the ducts. The duodenum should In patients with annular pancreata, surgical therapy be inspected for distal webs by passing a Foley catheter has been more varied, without arriving at a single best distally and withdrawing it with the balloon inflated. solution as seen with duodenal web excision. Early at- Duodenal bypass is usually not indicated, and neither is tempts at resection or division of the annulus resulted major gastric or duodenal resection. in more complications of duodenal leak, pancreatitis, and 2. In patients with annular pancreata, one must de- pancreatic fistula. Bypass of the annulus by duodeno- termine by inspection if the annulus is complete, par- duodenostomy, duodenojejunostomy, and antrectomy tial, intramural, or extramural. If the annulus is partial with gastrojejunostomy have evolved as the seemingly and the duodenum is not densely strictured, incision or preferred methods of therapy (Table 5). In patients who partial excision of the annulus and a Heineke-Mikulicz– had no surgical treatment, mortality was 26.3%. After gas- type duodenoplasty can be carried out, especially if ac- tric resection and gastroenterostomy, patients still de- cess to the biliopancreatic sphincter is deemed surgi- veloped pancreatitis, pancreatic and enteric fistulae, sto- cally necessary. Careful search for and control of the mal ulceration, and death. On the other hand, the transected pancreatic ducts of the annulus is mandatory outcomes were considered satisfactory in 73% of all in that procedure. Otherwise, duodenoduodenostomy and surgical patients. duodenojejunostomy are the safest procedures to by- In summary, awareness of the problem and preop- pass the obstructing annulus and prevent long-term post- erative diagnosis are extremely important. Not all pa- gastrectomy problems, anastomotic leaks, and pancre- tients need surgery, but those with significant gastric out- atic fistulae. Care must be taken, however, to use an let obstruction will be best served by surgery. Newer incision high enough on the proximal dilated duode- techniques such as computed tomographic scanning, mag- num to achieve a widely patent anastomosis that will en- netic resonance cholangiopancreatography, and endo- sure adequate duodenal drainage. scopic evaluation by experienced GI endoscopists will A useful clinical observation at the time of surgery make a difference in preoperative planning. Patients with for presumed duodenal stenosis from ulcer scarring is that associated anomalies such as malrotation, pancreas di- external transverse duodenal dimpling or obstruction to

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 the finger in the descending duodenum is probably not doscopic diagnosis. The authors state that the upper GI stud- from an ulcer but might be an intraluminal web. An an- ies were done and 3 were diagnostic. nular pancreas, on the other hand, should be obvious. My first question is how many upper GI studies were done in total, as in my experience this is the most accurate means of securing the diagnosis. I continue to believe that in any upper Presented at the 108th Scientific Session of the Western Sur- GI dysmotility syndrome, both endoscopy and contrast stud- gical Association, Dana Point, Calif, November 15, 2000. ies are needed as they provide different information. And I sus- Corresponding author and reprints: James A. Madura, pect that the 11 endoscopy patients who were not diagnosed MD, Department of Surgery, Indiana University Medical by these means would have been identified by an upper GI. Re- Center, 545 Barnhill Dr, EM 244, Indianapolis, IN 46202- garding the treatment of webs, the authors’ wisely urge cau- 5125 (e-mail: [email protected]). tion as many of these are juxta-ampullary, and if you merely evert and oversew the wind sock, you can compromise the ampulla. Their advice to only perform a lateral web excision or to REFERENCES protect the ampulla by selectively cannulating it from above or below should be considered by anybody treating this con- 1. World Health Organization. International Classification of Diseases, Ninth Revi- dition. My only experience with endoscopic excision of a duo- sion (ICD-9). Geneva, Switzerland: World Health Organization; 1977. denal web came in the form of a patient transferred to me with 2. Madura JA, Goulet RJ Jr, Wahle DT. Duodenal webs in the adult. Am Surg. 1991; a near fatal pancreatitis secondary to this procedure, and so I 57:607-614. am not particularly sanguine about this form of treatment as a 3. Ravitch MM. The pancreas in infants and children. Surg Clin North Am. 1975; routine approach. 55:377-385. My last comment has to do with the treatment of the an- 4. Naylor RG, Juler GL. Congenital causes of duodenal ulcers in adults. Arch Surg. 1976;111:658-662. nular pancreas. Although the authors appear to advocate ex- 5. Lecco TM. Zu¨r morphologie des Pankreas annulare. Sitzungsb Akad Wissen- cision of the annulus with a transverse duodenoplasty, I would sch. 1910;119:391-406. prefer a duodenoduodenostomy after complete kocherization 6. Baldwin WM. A specimen of annular pancreas. Anat Rec. 1910;4:299-304. of the duodenum. Both procedures necessitate a duodenal su- 7. Lehman GA, O’Connor KW. Coexistence of annular pancreas and pancreas di- ture line, but my approach eliminates the possibility of post- visum: ERCP diagnosis. Gastrointest Endosc. 1985;31:25-28. operative pancreatitis or a pancreatic fistula. Perhaps the au- 8. Baggot BB, Long WB. Annular pancreas as a cause of extrahepatic biliary ob- thors could comment on my bias. struction. Am J Gastroenterol. 1991;86:224-226. In closing, these conditions are rare, and each of you will 9. Gilinsky NH, Lewis JW, Flueck JA, Fried AM. Annular pancreas associated with therefore see few of them in your careers. However, the poten- diffuse chronic pancreatitis. Am J Gastroenterol. 1987;82:681-684. tial for diagnostic and therapeutic mishaps are real. I feel this 10. Tandler J. Zu¨r die entwicklungsgeschichte des menschlichen duodenums in fru¨hen embryonalstadiem. Morphol Jahrbuch. 1900;29:187-216. paper belongs in the permanent reprint file of all practicing gen- 11. Lynn HB, Espinas EE. Intestinal atresia. Arch Surg. 1959;79:357. eral surgeons. 12. Streeter GL. Developmental Horizons in Human Embryos: Descriptions of Age Thomas A. Stellato, MD, Cleveland, Ohio: The authors Groups xv, xvi, xvii and xviii. Washington, DC: Carnegie Institute; 1948:162- suggest that excision can be performed for a loose annulus. Yet, 177. Contributions to Embryology publication 575. the only fair to poor result in the study seemed to be in those 13. Boyden EA, Cope JG, Bill AH Jr. Anatomy and embryology of congenital intrinsic patients who had an excision of the annular pancreas. All of obstruction of the duodenum. Am J Surg. 1967;114:190-202. the other results seem to be excellent, and I wonder once again 14. Sperazza JC, Flanagan RA Jr, Katlic MR. Annular pancreas and intermittent duo- whether the authors could reevaluate that recommendation. denal obstruction in an alcoholic adult. Cleve Clin J Med. 1992;59:208-210. Theodore X. O’Connell, MD, Los Angeles, Calif: I also 15. Kreig EG. Duodenal diaphragm. Ann Surg. 1937;106:33-39. have a question about the annular pancreas because maybe in 16. Vidal E. Quelques cas de chirurgie pancre´atique. Proces Verbaux Memores Dis- cussions l’Association Francaise de Chirurgie. 1905;18:739-747. my simple-minded approach they seem to be taken care of 17. Gertsch PH, Mosimann R. Endoscopic laser treatment of a congenital duodenal fairly easily with a duodenoduodenostomy or a duodenojeju- diaphragm. Gastrointest Endosc. 1984;30:253-254. nostomy where you would predict a good outcome. Yet, 40% 18. Jex RK, Hughes RW. Endoscopic management of duodenal diaphragm in the adult. of their 5 patients, 2 of 5 patients, had a fair to poor outcome. Gastrointest Endosc. 1986;32:416-419. I wish you would explain that a little bit more. Exactly what 19. McGuinness CL, Choy A, Gajraj H, Chilvers AS. Stapling technique for annular was the poor outcome due to and was it due to the operative pancreas. Br J Surg. 1993;80:758. intervention? Philipe E. Donahue, MD, Chicago, Ill: I, too, congratu- late the authors on an important study showing the impor- DISCUSSION tance of complete evaluation in foregut symptoms, especially Jack Pickleman, MD, Maywood, Ill: I commend the Western the upper GI series. My one question: “What was the time in- Surgical Association in seeking out experts in their field to dis- terval from first symptoms until final diagnosis?” These pa- cuss each of these excellent papers. It is important to define an tients often creep along the diagnostic algorithm of “diagnosis expert. For the purposes of the present discussion, it will be as of symptom,” “proton pump inhibitor,” “watchful waiting,” “en- follows. The expert should have heard of the condition, the ex- doscopy,” “repeat endoscopy,”... aprocess which can con- pert should have treated 3 patients with the condition, and the sume years. It is very expensive and often wasteful of time and expert should have a beating heart. As such, I stand before you resources. Tell us something about that if you can. as an expert. Dr Madura: Dr Ladd and I would again like to express The GI surgery group in Indiana brings to our attention our thanks to the Program Committee and to the Western Sur- the largest reported series of congenital duodenal abnormali- gical Association for allowing us to present this unusual re- ties in adults, all presenting with symptoms of chronic gastric port. Not only did we have a primary discussant with a beat- outlet obstruction. My first comments have to do with the di- ing heart, but it was a gentler, kinder beating heart, and we agnosis of these conditions. With the hairpin triggers pos- appreciate Dr Pickleman’s remarks. sessed by most gastroenterologists for inserting an endoscope Of these conditions, the first web I ever saw, I missed at into both northern and southern orifices at the onset of any GI surgery as well. We had a patient with recurrent duodenal ul- symptom, it is not surprising that 20 of 29 of these patients un- cer disease who did not get better, and we were actually doing derwent this procedure. However, only a few had a correct en- a Jaboulay pyloroplasty when we discovered the web. I remem-

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©2001 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/26/2021 bered back to my training with Dr Zollinger, who, in the hey- patients who either had an incomplete annulus or in whom the day of gastric surgery, always told me that when doing a pylo- annulus was loosely applied to the duodenal serosa, and we could roplasty or gastric resection you should always put your finger dissect it off and carefully control the ductal system. Why didn’t down into the duodenum because they could have a second we just do a bypass? Well, these patients also had concomi- ulcer stricture, which I never understood very well and which tant stenosis of the sphincter of Oddi, which had been diag- I never saw until my first patient with the web. These ulcer stric- nosed by pancreatic duct manometry and, because of the an- tures that he described were probably not due to ulcers but prob- nulus, it was difficult for our ERCP service to get there and divide ably were webs. There are undoubtedly a lot more individuals the pancreatic sphincter. So they referred them to us. These were out there with webs than we would suspect. combined procedures in these patients with excision of the web, Now, why were they not preoperatively diagnosed? Well, duodenoplasty, and transduodenal sphincteroplasty. The rea- with a large, big, dilated pylorus and duodenal bulb, the en- son they did less well in some of these cases was they had con- doscopist many times mistakes that web aperture for the py- tinued problems with their pancreas and some had delayed gas- lorus. They really mistake the duodenal bulb dilation as an- tric emptying, and, therefore, we concluded that those patients trum, and unless they biopsy it (which they don’t), they will were not excellent outcomes. That answers Dr Stellato’s ques- miss the diagnosis completely. So they reported a strictured py- tion as well as Dr O’Connell’s question. The fair to poor out- lorus in many of our cases. Upper GI series is similar, and many come was due to pancreatitis and delayed gastric emptying. of these were not initially diagnosed by an upper GI series but, One other point that we want to make is that these pain retrospect, you can clearly see the web like you did in one tients, a third of them, will present with ulcer disease and they of our photos in a patient who came from another hospital. We, don’t need an ulcer operation. Once you eliminate the web and too, agree with you, Dr Pickleman, that endoscopic excision open up the duodenum, their gastric dilation and antral dis- should not be undertaken lightly. There are 3 reports in the tention goes away. Their resting gastrin levels decrease, and their literature, 2 laser ablations and 1 simple incision, with a pap- ulcers can be easily managed with either no medication or short- illotome through the endoscope, and although all of the en- term antacid medications. doscopists were cautious about how they did it, they got duo- The timing interval, Dr Donahue, from onset of symp- denal strictures, and several of them had to be reoperated on. toms to diagnosis was up to 10 to 15 years. One of our patients So there is not a huge experience in the endoscopy literature who was in her 70s said that she had had problems with di- for ablating these endoscopically. Now, the patients in whom gestion ever since she was a child but would eat more fre- we excised or divided an annulus were not patients who had quently and would not eat solid and high-residue foodstuffs the tight fibrotic strictures of the duodenum. These were the but ate more of a liquid diet.

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