The clinical presentation of in-testinal duplication in adultsranges from chronic nonspe-cific gastrointestinal discomfort to oneof acute abdominal pain. Althoughseveral series of these lesions have beendescribed in children,1,2 there havebeen few descriptions in adults. Wepresent the cases of 3 adults who pre-sented recently with duplication of theforegut, midgut and hindgut. Theselesions are important because theseduplications share patterns of presen-tation common to many nonspecific

intra-abdominal diseases, yet are read-ily managed by a combined radiologicand surgical approach. The cases pro-vide an opportunity to review the spec-trum of presentations associated withenteric duplication in adults and thecurrent literature on this topic.

CASE REPORTS

Case 1: foregut duplication

A 22-year-old woman was admit-ted with epigastric pain, squeezing in

nature, that had been present inter-mittently for 2 years. Previously,chronic gastritis had been diagnosed,and she had been treated conserva-tively. The pain was aggravated byfood and relieved by the applicationof pressure to the upper abdomen.The woman appeared thin, but physi-cal examination was otherwise unre-markable. Hematologic assessment re-vealed a mild normocytic anemia, andultrasonography demonstrated a cys-tic structure, 7 cm in dimension, inthe gastrohepatic region. Contrast en-

Case ReportÉtude de cas

ENTERIC DUPLICATION IN THE ADULT, DERIVEDFROM THE FOREGUT, MIDGUT AND HINDGUT:PRESENTATION, PATTERNS AND LITERATURE REVIEW

David J. Hackam, MD;*† Arthur Zalev, MD;‡ Marcus Burnstein, MD;†§ Ori D. Rotstein, MD;*† Jarley Koo, MD†§

From the *Division of General Surgery, The Toronto Hospital, the †Department of Surgery, University of Toronto, the ‡Department of Radiology, St. Michael’s Hospital, andthe §Division of General Surgery, St. Michael’s Hospital, Toronto, Ont.

Accepted for publication July 7, 1996

Correspondence and reprint requests to: Dr. Jarley Koo, Division of General Surgery, St. Michael’s Hospital, 30 Bond St., Toronto ON M5B 1W8

© 1997 Canadian Medical Association (text and abstract/résumé)

Duplication of the alimentary tract may affect patients of all ages. Although they are relatively rare, the im-portance of these congenital lesions lies in the fact that they readily mimic other surgical disease processesand may result in significant morbidity if left untreated. Prompt recognition and treatment using combinedradiologic and surgical management are generally associated with an excellent outcome. Three patientswho presented with intestinal duplication arising from each of the major embryologic origins are reported.Their clinical histories reveal the spectrum of presentation associated with these lesions and provide aframework for a discussion of current management strategies.

La duplication du tractus alimentaire peut se manifester chez des patients de n’importe quel âge. Même sices lésions congénitales sont relativement rares, leur importance réside dans le fait qu’elles imitent facile-ment d’autres processus morbides chirurgicaux et peuvent entraîner une morbidité importante si elles nesont pas traitées. L’identification et le traitement rapides par chirurgie et radiologie combinées donnent engénéral d’excellents résultats. On signale le cas de trois patients qui se sont présentés avec une duplicationintestinale découlant de chacune des principales origines embryologiques. Leurs antécédents cliniquesrévèlent l’éventail complet des phénomènes liés à ces lésions et établissent un cadre de discussion des straté-gies actuelles de prise en charge.

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hanced CT revealed a midline, fluid-filled mass between the liver and thestomach, causing indentation of thelesser curvature (Fig. 1). The patientunderwent laparotomy, at which timea pear-shaped cystic mass was identi-

fied in the gastrohepatic ligament,which was intimately associated withthe gastroesophageal junction. It wasresected en bloc, and the point of at-tachment to the gastroesophagealjunction was closed primarily. Patho-

logical evaluation revealed a gastricduplication cyst. The patient’s post-operative course was uncomplicated,and at follow-up 13 months later shewas asymptomatic.

Case 2: midgut duplication

A 23-year-old woman was admit-ted with acute, severe periumbilicalpain of 1 week’s duration. Similar butless intense pain had been present for1 year, and she had remained systemi-cally well. Irritable bowel syndromehad been diagnosed and treated con-servatively. The pain was crampy,episodic and associated with abdomi-nal distension and nausea. Examina-tion revealed a tender, 10-cm mobilemass in the right lower quadrant ofthe abdomen. Ultrasonography re-vealed a fluid-filled, septate mass in

that location, measuring 9 cm in max-imum dimension (Fig. 2). Contrastenhanced CT demonstrated abilobed, fluid-filled structure, strad-dling the patient’s midline (Fig. 3). Atlaparotomy, a 7-cm enteric duplica-tion of the mid-ileum was diagnosedand was resected along with adjacentsmall bowel. Pathological examinationof the resected specimen confirmedthe diagnosis of duplication of thesmall bowel. The patient’s postopera-tive course was uncomplicated, and atfollow-up 18 months later she wasasymptomatic.

Case 3: hindgut duplication

A 44-year-old woman was admittedwith a 12-month history of the sensa-tion of incomplete rectal emptying.Initially, constipation had been diag-nosed and managed conservatively.The symptoms had become intolera-ble in the month before admission andwere associated with lower back pain.There were no other symptoms. Digi-tal rectal examination revealed asmooth, well-circumscribed cysticmass situated at the tip of the examin-ing finger on the posterior rectal wall.Transrectal ultrasonography demon-strated a heterogeneous mass betweenthe rectum and sacrum (Fig. 4). CTsuggested that the mass had an inti-mate association with the rectum(Fig. 5). At operation, through aparasacral approach, the lesion wasidentified attached to the posteriorwall of the rectum. It was resected enbloc, and the rectal mucosal defect wasclosed primarily. Pathological exami-nation revealed a duplication cyst con-taining rectal mucosa. The patient’spostoperative course was smooth, andat follow-up 9 months later she wasasymptomatic.

DISCUSSION

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FIG. 1. Case 1. Contrast enhanced CT scan showing a midline fluid-filled mass (C) between the liverand the stomach. The lesser curve of the stomach is indented by the mass.

FIG. 2. Case 2. Ultrasonogram in the sagittal plane showing a fluid-filled mass with a septum andsolid debris layering posteriorly.

The above cases provide an oppor-tunity to review the current literatureregarding the pathologic and embry-ologic features and management ofenteric duplications in the adult.Enteric duplications by definition

comprise a group of lesions that con-tain a smooth-muscle wall and entericmucosa and are found only on themesenteric border of the intestine.1

These features differentiate intestinalduplications from the more commonMeckel’s diverticulum, which is foundon the antimesenteric border, andwith other intra-abdominal cysticprocesses, which are described inTable I. Duplications may be foundon intestine of foregut, midgut andhindgut derivation, although themidgut variety is the most common.2,3

In addition to an abdominal location,duplications are also seen in the chest,where they generally arise from theesophagus. In the majority of cases,some degree of communication existsbetween the duplication and the adja-cent lumen, varying between a largeopening and a thin fibrous strand.4

Twenty percent to 30% of lesions con-tain heterotopic mucosa, which is usu-ally gastric.3,5 There were no associatedanomalies in the current series, al-though vertebral and genitourinarydefects may be encountered.An accurate distinction can usually

be made between enteric duplicationsand other intra-abdominal cysticprocesses with the combined use of ul-trasonography and CT (Table I).4 Anemerging diagnostic technique thatmay provide additional diagnostic in-formation is endoscopic ultrasonogra-phy, which has been used in the inves-tigation of foregut lesions.6 It is of notethat before the current era of accuratecross-sectional radiologic evaluation, acorrect diagnosis of enteric duplicationwas seldom established preoperatively.This is because neither plain radiogra-phy nor intraluminal contrast radiog-

raphy accurately identifies either thecommunication between the duplica-tion and the adjacent bowel or me-chanical displacement of the bowel bythe enteric cyst.4,7

An understanding of the embry-ologic development of enteric duplica-tion may provide further insight intoits clinical presentation. The “entericbud” theory of Lewis and Thyng states

that buds of intestinal epithelium pro-

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FIG. 3. Case 2. Contrast enhanced CT scan showing 2 fluid-filled locules (C) of the mass straddlingthe patient’s midline.

FIG. 4. Case 3. Transrectal ultrasonogram, transverse view of the mass, shows 2 cystic spaces (C). Inthis plane the mass is mainly solid.

trude into the lamina propria and en-large to establish a connection with thebowel lumen.8 The “solid lumen the-ory” of Bremer postulates that therapidly enlarging embryonic gastroin-testinal tract outgrows the celomic cav-ity and causes an accumulation of in-testinal epithelial cells that thenvacuolize, coalesce and form the lu-men of the intestinal duplication.9 Rav-itch theorized that hindgut duplicationrepresents partial twinning,10 whereasothers have postulated that an adhe-sion in the presomite embryo betweenthe ectodermal and entodermal germlayers results in splitting or deviationof the notochord, which leads to ali-mentary duplications in associationwith vertebral anomalies.11 Although

superficially attractive, each of thesetheories falls short in explaining boththe presence of heterotopic mucosaand the consistent mesenteric location.The clinical presentation of enteric

duplication in adults is variable and hasundergone a transition with time. Inthe past, patients were operated onwith complications of these lesions,such as bleeding and perforation.7 To-day, as described in the current study,the diagnosis is made after abdominalCT or ultrasonography for persistent,often mild, gastrointestinal symptoms.This symptom complex is dictatedchiefly by the location and size of thelesion. For example, foregut duplica-tions are most commonly associatedwith epigastric pain and dysphagia, the

latter due to impingement of the cys-

tic mass on the esophageal lumen (case1).12 In foregut duplications, therehave been isolated reports of hetero-topic pancreatic tissue, which increasesthe morbidity,13 and rare instances inwhich these lesions communicatedwith the pancreatic duct or biliarytree.14,15 Duplications of the midgut aregenerally associated with abdominalpain; obstruction and intussusceptionhave been reported (case 2).16 Thepresence of heterotopic mucosa, whichis most commonly gastric, predisposesto bleeding or perforation.7,17 Duplica-tions of the hindgut may be seen in as-sociation with intestinal obstruction ortenesmus (case 3) and may be con-fused with a colorectal neoplasm.Once the diagnosis of an enteric

duplication is made, surgical correc-tion is warranted for 3 reasons. First,the majority of patients require surgi-cal intervention for the relief of symp-toms.12 Second, surgical treatment re-moves the otherwise persistent risk ofperforation and bleeding caused byheterotopic mucosa in the unresectedcyst.5 Third, there have been reportsof the development of carcinoma inthe lining of cyst.18,19 Orr and Ed-wards18 reviewed 10 cases of neoplasiadeveloping in duplication cysts. In thisseries, the most common location wasthe large intestine, and the most fre-quent cell type was adenocarcinoma.The goals of surgery are to com-

pletely remove the lesion and associ-ated heterotopic mucosa withoutjeopardizing the function of the re-maining bowel. The complexity of thesurgical procedure required is ex-tremely variable and depends on boththe size and location of the duplica-tion. For instance, our foregut andmidgut cases (cases 1 and 2) wereamenable to complete resection andrepresent the most common scenario.The hindgut duplication in case 3 wasmanaged via the parasacral route,which we felt would be less morbid

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FIG. 5. CT scan at another transverse plane showing the mass (arrowheads) posterior to a dilatedrectum. The mass contains several hypodense areas and is mainly cystic at this level.

Table I

Differential Diagnosis of Intra-abdominal Cysts Based on Histologic and Radiologic Features

Cyst type

Enteric duplication

Lymphangioma

Mesothelial cyst

Pseudocyst

Adapted from Dachman AH, Hjemstad BH, Sobin LH: Mesenteric and omental cysts: histologic classification with imaging correlation. Radiology 1987;164:327-32.4

No lining. Fibrous wall

Mesothelial lining

Endothelial lining

Enteric lining. Double musclelayer

Histologic features

Thick wall. May containechogenic debris

Thin wall. Unilocular

Thin wall. Multiple septations

Thick wall. Unilocular ormultilocular fluid-filled mass

Radiologic features

than operating through the anteriorabdominal wall. Various strategieshave been used for lesions that are notamenable to complete resection. Theclassic treatment for complex duode-nal duplications is cystoduodenos-tomy,20 involving the formation of alarge communication between the du-plication and the adjacent bowel.Long ileal duplications, whose lengthprecludes complete resection, havebeen treated by segmental stripping ofthe mucosa through a series of trans-verse incisions into the seromuscularwall.21 In general, such strategies arerarely required, and the commonestscenario involves a straightforward re-section of the duplication along withthe attached bowel.In conclusion, through a series of

case reports we have described thespectrum of disease associated withduplication of the foregut, midgutand hindgut in adults. The case histo-ries emphasize the similarities betweenthese relatively rare lesions and other,more common surgical diseaseprocesses. This study illustrates theimportance of adequate investigationfor refractory abdominal pain, and theexcellent outcome that may be ob-tained in appropriately treated adultpatients with enteric duplication.

References

1. Ladd WE, Gross RE. Surgical treat-ment of duplications of the alimen-tary tract. Enterogenous cysts, entericcysts or ileum duplex. Surg GynecolObstet 1940;70:295-307.

2. Bower RJ, Sieber WK, KiesewetterWB. Alimentary tract duplications inchildren. Ann Surg 1978;188:669-74.

3. Nolan JJ, Lee JG. Duplication of thealimentary tract in adults with a re-port of 3 cases. Ann Surg 1953;137:342-8.

4. Dachman AH, Hjemstad BH, SobinLH. Mesenteric and omental cysts:histological classification with imag-ing correlation. Radiology 1987;164:327-32.

5. Ildstad ST, Tollerud DJ, Weiss RG,Ryan DP, McGowan MA, Martin LW.Duplications of the alimentary tract.Clinical characteristics, preferred treat-ment, and associated malformations.Ann Surg 1988;208:184-9.

6. Geller A, Wang KK, DiMagno EP.Diagnosis of foregut duplication cystsby endoscopic ultrasonography. Gas-troenterology 1995;109:838-42.

7. Anderson MC, Silberman WW,Shields TW. Duplications of the ali-mentary tract in the adult. Arch Surg(Chicago) 1962;85:110-24.

8. Lewis FT, Thyng FW. The regularoccurrence of intestinal diverticula inthe embryos of the pig, rabbit andman. Am J Anat 1907;7:505-19.

9. Bremer JL. Diverticula and duplica-tions of the intestinal tract. ArchPathol 1944;38:132-40.

10. Ravitch MM. Hind-gut duplication —doubling of colon and genital urinarytracts. Ann Surg 1953;137:588-601.

11. Faris JC, Crowe JE. The split noto-chord syndrome. J Pediatr Surg1975; 10: 467-72.

12. Holcomb GW 3d, Gheissari A,O’Neill JA Jr, Shorter NA, BishopHC. Surgical management of alimen-tary tract duplications. Ann Surg1989;209:167-74.

13. Luks FI, Shah MN, Bulauitan MC,LoPresti PA, Pizzi WF. Adultforegut duplication. Surgery 1990;108:101-4.

14. Johnstone DW, Forde KA,Markowitz D, Green PH, Farman J.Gastric duplication cyst communicat-ing with the pancreatic duct: a rarecause of recurrent abdominal pain.Surgery 1991;109:97-100.

15. Akers DR, Favara BE, Franciosi RA,Nelson JM. Duplications of the ali-

mentary tract: report of three unusualcases associated with bile and pancre-atic ducts. Surgery 1972;71:817-23.

16. Salameh RN, Hausner RJ, ThomasSJ, Johnson CD. Massive hemor-rhage in an ileal duplication cyst.South Med J 1984;77:1606-7.

17. Cecil BD, Larson GM, Sewell C,Redinger RN. Abdominal pain fromintestinal duplication. J Clin Gas-troenterol 1990;12:316-9.

18. Orr MM, Edwards AJ. Neoplasticchange in duplications of the alimen-tary tract. Br J Surg 1975;62:269-74.

19. Tamoney HJ, Testa RE. Carcinomaarising in a duplicated colon: case re-port. Cancer 1967;20:4-6.

20. Gardner CE Jr, Hart D. Enteroge-nous cysts of the duodenum. JAMA1935;104:1809-13.

21. Wrenn EL. Tubular duplication ofthe small intestine. Surgery 1962;52:494-7.

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