Congenital Duodenal Anomalies in the Adult
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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 pancre- atic 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 an- nular 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 Cprimitive 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 (REPRINTED) ARCH SURG/ VOL 136, MAY 2001 WWW.ARCHSURG.COM 576 ©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 per- formed, 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 com- puted 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 en- doscopy was performed in 20 patients, with the correct COMMENT diagnosis being made in 9. Fourteen patients were diag- nosed 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