Esophageal intramural pseudodiver-ticulosis (EIP) is an uncommoncondition in which submucosal

glands within the esophageal wall becomedilated and appear as pseudodiverticuli.1

Since the disorder was first described in1960,2 fewer than 150 cases have been re-ported in adults.3 Only 12 cases have beendescribed in children.The diagnostic and pathological fea-

tures of EIP are well documented,4 but theetiology and pathogenesis are unknown.In children, this rare disorder has been as-sociated with gastroesophageal reflux(GER), corrosive acid ingestion5 and dys-motility.6 In adults, diabetes and candidia-sis are additional associations.4

Although EIP is usually a benign discov-ery, 3 cases of the perforation of the pseu-dodiverticuli have been reported in the adultliterature.7–9 We describe the first case of aruptured pseudodiverticulum in a teenager.

CASE REPORT

An 11-year-old boy of East Indian de-scent presented with dysphagia to solidfood and intermittent central chest pain,associated with occasional vomiting andpostprandial eructation. Endoscopy at thattime showed a proximal esophageal stric-ture. He was treated with omeprazole (20mg orally once a day) and cisapride (10 mgorally three times a day) with reasonablesymptom relief. He was lost to follow-upuntil the age of 14 years when he was seenbecause of recurrent symptoms while offmedication. Endoscopy showed concentric

rings (trachealization of the esophagus)and evidence of reflux esophagitis, con-firmed by biopsy. Dilatation was performedup to 11 mm with Savoury dilators.Two months later, he presented with a

sudden onset of retrosternal chest pain asso-ciated with a dry cough and a temperatureof 39.3 °C. He also complained of ongoingdysphagia as well as odynophagia but hadnot been retching or vomiting. No other ab-normalities were found on physical exami-nation. Laboratory studies and chest radiog-raphy gave normal results. Ampicillin (1 gevery 6 hours), gentamicin (50 mg every 8hours) and metronidazole (80 mg every 8 hours) were started intravenously. The following day, barium swallow examinationshowed a small EIP with the characte r is -tic appearance within a long proximalesophageal stricture (Fig. 1). There was lon-gitudinal tracking of barium intramurallywith no free leak. An extrinsic mass effectwas noted, suggesting local inflammation.Computed tomography of the chest showedmediastinal air from a localized esophagealperforation, which was thought to be sec-ondary to a ruptured EIP with associatedperidiverticulitis (Fig. 2). The possibility of asmall, contained perforation from the previ-ous dilatation was considered. However, thefact that he had been asymptomatic for 2months made this unlikely.The patient was started on parenteral

nutrition. He responded to medical man-agement with resolution of his fever andchest pain in 2 days. On a repeat bariumstudy 5 days later, the EIP and the extrin-sic mass effect were no longer evident. His

diet was advanced and he was dischargedafter 9 days of hospitalization to continueamoxicillin-clavulanate (500 mg orallythree times a day) for 1 week and omepra-zole (20 mg orally twice a day) in the longterm. A repeat barium swallow examina-tion a month later showed the stricturebut no EIP. Esophageal manometry and24-hour pH study (off omeprazole for 4days) gave normal results.When the boy was 15 years old, ulcera-

tive colitis was diagnosed. Upper andlower gastrointestinal endoscopy bothshowed persistent esophagitis and also col-itis involving the left colon and transversecolon. At the time of writing, at 17 yearsof age, his main symptoms are related tohis ulcerative colitis.

DISCUSSION

Thirteen cases of EIP (including thepresent case) have been reported in the pe-diatric literature (Table I5,6,10–15). The meanage at diagnosis was 11.4 years, with theyoungest child being 4 years old. Theslight male predominance observed inchildren is consistent with that in the adultliterature.4 The high preponderance of thisdisorder in East Indian children is skewedby the series of Kochhar and associates5

who reported 14 East Indian patients withcorrosive acid-related EIP, 4 of whomwere under the age of 18 years.Four children had feeding difficulty in

infancy6,10,14 and 2 had tracheoesophagealfistula repair.13,15 Underlying long-stand-ing GER probably contributed to the de-

Brief CommunicationCommunication abrégé

RUPTURED ESOPHAGEAL INTRAMURALPSEUDODIVERTICULUM: AN UNUSUAL CAUSEOF MEDIASTINITIS IN A CHILD

Jason Wong, MD;* Mark Walton, MD;* Robert Issenman, MD†

From the *Division of Pediatric Surgery and †Division of Gastroenterology, Children’s Hospital, Hamilton Health Sciences Corporation, Hamilton, Ont.

Accepted for publication July 27, 1998.

Correspondence to: Dr. Mark Walton, Rm. 4E3, Children’s Hospital at Hamilton Health Sciences Corporation, 1200 Main St. W, Hamilton ON L8N 3Z5; fax 905 521-9992,waltonj@fhs.csu.mcmaster.ca

© 1999 Canadian Medical Association

CJS, Vol. 42, No. 5, October 1999 389CJS, Vol. 42, No. 5, October 1999 389

velopment of EIP in these cases. The eti-ologic role of dysmotility in children,however, has yet to be examined becausemanometry has seldom been used in chil-

dren with EIP.6 In adults, up to 77% ofthose tested revealed abnormalities in-cluding aperistalsis, poor contractions,uncoordinated motor activity and an in-competent lower esophageal sphincter.4

Four patients had dysphagia after acidingestion (1 to 48 months before presen-tation) and had evidence of EIP. Acid-induced esophagitis followed by healingand fibrosis leads to stricture formation and

WONG ET AL

390 JCC, Vol. 42, No 5, octobre 1999

FIG. 1. Barium esophagogram shows a long, butmild stricture of the proximal esophagus. Anesophageal intramural pseudodiverticulum (ar-row) is seen.

FIG. 2. Oral and intravenous enhanced CT scan of the chest reveals mediastinal air adjacent to thebarium column (arrow).

Table I

Summary of Features of 13 Children Who Had Esophageal Intramural Pseudodiverticulosis (EIP)

Series

Kochhar et al, 19915

van Overbeek et al, 19786 13

10

10

16

16

Age atdiagnosis,

yr

Caucasian

East Indian

East Indian

East Indian

East Indian

Race

M

M

M

F

F

Sex

Weller, 197210 11 Caucasian M

Cramer, 197211 15 Caucasian M

Braun et al, 197812 8 Caucasian M Dysphagia, food impaction,gastroesophageal reflux, hiatushernia

Asthma, dysphagia

Dysphagia

Dysphagia

Acid ingestion, dysphagia

Acid ingestion, dysphagia

Acid ingestion, dysphagia

Acid ingestion, dysphagia

Presentation

Proximal

Proximal

Proximal

Proximal

Distal

Distal

Entire length

Middle

Stricturelocation inesophagus

Proximal,within, distal

Distal

Within

Within

Within

Within

Within

Within

EIP relativeto stricture*

Not reported

None

Dilatation

Dilatation

Dilatation

Dilatation

Dilatation

Dilatation

Treatment

Muhletaler et al, 198013 18 Notreported

F Tracheoesophageal fistula repair(at 5 d), hiatus hernia repair (at6 yr), gastroesophageal reflux,recurrent dysphagia

Distal Proximal Dilatation, colonicinterposition bypass

Peters et al, 198314 5 Notreported

F Developmental delay, dysphagia,hiatus hernia

Middle Within None (16-yr follow-upwith no complication)

Markle et al, 199215 4 Caucasian F Tracheoesophageal fistula,eosphageal atresia repair (at 2 d), gastroesophageal reflux

Proximal Within, distal None (12-yr follow-upwith no complication)

8 Caucasian M Dysphagia, gastroesophagealreflux

Distal Proximal,within

Dilatation andfundoplication

Present case 14 East Indian M Dysphagia, gastroesophagealreflux, chest pain, mediastinitis

Proximal Within Antibiotics,antireflux treatment

*EIP was found in various locations in 3 cases.12,15

possible shortening of the esophagus. Thismay result in the development of GER andchronic esophagitis, which predisposes tothe formation of EIP. Candidiasis and dia-betes have been associated with EIP inadults,4,5 but much less so in children.6,10

An esophageal stricture was found in allchildren regardless of the presence of GERor a history of acid ingestion. This findingsuggests that chronic esophagitis representsthe common pathway in the developmentof EIP. The stricture is only a part of thedisease process rather than the cause of EIP,as its anatomic relationship to EIP is notconstant (Table I). Paradoxically, EIP hasbeen seen in adults without a stricture.1,2

GER has been reported in 5 childrenwith 1 associated hiatus hernia,12,13,15 but thediagnostic modalities for GER were notclearly indicated in these cases. Our patienthad clear endoscopic evidence of GER(concentric rings) as well as biopsy-provenreflux esophagitis. There was no history ofcaustic ingestion. The normal 24-hour pHstudy done at only 4 days off omeprazolemight be due to partial acid suppression. Aproper study for a child of this age wouldrequire 10 days without omeprazole.Barium swallow examination offers the

most sensitive method of diagnosis. Theappearance of pin-head sized outpouch-ings projecting perpendicularly from themucosal surface is pathognomonic.4 An in-teresting observation was made in previ-ous reports that the appearance of the EIPwould become less obvious11 and the num-ber of cases of EIP would decrease10 withtime. In our patient, with proper treat-ment, the EIP disappeared in the repeatbarium study. CT may be of value in mak-ing the diagnosis, as it was in our case.Typical findings on CT consist of intra-mural gas collections with associated wallthickening and mucosal irregularity.16

Endoscopy was performed in less than50% (6 of 13) of the children and showedesophagitis (4 children) and strictures (5children). Ostia of the dilated glands wereseen in only 2 patients.13,15 This direct endo-scopic evidence of EIP was observed infre-quently (17%) in adults.4 The ostia on themucosal surface correspond to the main ex-cretory ducts of the dilated esophageal sub-mucosal glands, which are lined by stratifiedsquamous epithelium. In most cases, acuteor chronic inflammation, or both, are noted,presumably causing ductal obstruction lead-ing to subsequent dilatation of the glands

and squamous metaplasia. In uncomplicatedcases, these dilated glands do not penetratethe muscularis propria. Endoscopic mucosalbiopsies were non-diagnostic because of thesubmucosal location of the EIP.6,10,12,15

Treatment of EIP should be directedtoward the underlying cause to preventfurther damage and should deal with com-plications. In the 12 children reported pre-viously (Table I), 6 underwent dilatationof the stricture, 1 had dilatation and fun-doplication, 1 had colonic interpositionbypass. Three children had no treatment;2 of them had no complications during along follow-up.14,15 Although not men-tioned in any of the cases, GER oresophagitis should be treated aggressively.Our patient underwent dilatation and re-ceived medical therapy for GER. Becauseof difficulty in obtaining medical controlof his ulcerative colitis, fundoplication hasnot been performed since his esophagitisis currently not his main problem.Only 3 cases of perforation of EIP have

been reported.7–9 All were in adults olderthan 50 years. The 2 patients with medias-tinitis were treated successfully with antibi-otics.7,9 Kim and associates8 reported a 67-year-old man who underwent a subtotalesophagectomy for a distal esophageal massthat was actually a periesophageal abscessfrom a perforated pseudodiverticulum.Our case appears to be the first one of

perforation of EIP with associated medias-tinitis in a child. Despite the potentialmorbidity and mortality, the patient recov-ered successfully with conservative ther-apy. Unlike other forms of esophageal per-foration, that from a ruptured EIP is likelymicroscopic and is sealed off relativelyrapidly by the surrounding tissue reaction.Contamination is therefore limited, thusallowing adequate control with antibioticsalone. It is apparent that pediatric EIP ismost likely a result of long-standingchronic esophagitis. The etiology of theesophagitis should be identified and ag-gressive treatment should be instituted toavoid possible progression to perforation.

References

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2. Mendl K, McKay JM, Tanner CH. Intra-mural diverticulosis of the esophagus and

Rokitansky–Aschoff sinuses in the gallblad-der. Br J Radiol 1960;33:496-501.

3. Papo M, Quer JC, Garcia G, Lorenzo A,Sauri A, Richart C. Esophageal intramuralpseudodiverticulosis. Rev Esp Enferm Dig1995;87(9):665-7.

4. Fromkes J, Thomas FB, Mekhjian H, Cald-well JH, Johnson JC. Esophageal intra-mural pseudodiverticulosis. Dig Dis 1977;22 (8):690-700.

5. Kochhar R, Mehta SK, Nagi B, Goenka M.Corrosive acid-induced esophageal intra-mural pseudodiverticulosis: a study of 14patients. J Clin Gastroenterol 1991;13 (4):371-5.

6. van Overbeek JJ, Edens ET, GokemeijerJD, Broker FH. Intramural diverticulosis ofthe esophagus. Laryngoscope 1978;88 (10):1671-9.

7. Rahlf G, Wilbert L, Lankisch PG, Hutte-mann U. Intramural esophageal diverticulo-sis. Acta Hepatogastroenterol 1977;24: 110-5.

8. Kim S, Choi CD, Groskin SA. Esophagealintramural pseudodiverticulitis. Radiology1989;173(2):418.

9. Abrams LJ, Levine MS, Laufer I.Esophageal peridiverticulitis: an unusualcomplication of esophageal intramuralpseudodiverticulosis. Eur J Radiol 1995;19:139-41.

10. Weller MH. Intramural diverticulosis of theesophagus: report of a case in a child. J Pe-diatr 1972; 80:281-5.

11. Cramer KR. Intramural diverticulosis of theesophagus. Br J Radiol 1972;45:857-9.

12. Braun P, Nussle D, Roy CC, Cuendet A.Intramural diverticulosis of the esophagusin an eight-year-old boy. Pediatr Radiol1978;6:235-7.

13. Muhletaler CA, Lams PM, Johnson AC.Occurrence of oesophageal intramuralpseudodiverticulosis in patients with pre-existing benign oesophageal stricture. Br JRadiol 1980;53:299-303.

14. Peters ME, Crummy AB, Wojtowycz MM,Toussaint JB. Intramural esophageal pseu-dodiverticulosis: a report in a child with a16-year follow-up. Pediatr Radiol 1983;13(4):229-30.

15. Markle BM, Hanson K. Esophageal pseu-dodiverticulosis: two new cases in children.Pediatr Radiol 1992;22:194-5.

16. Pearlberg JL, Sandler MA, Madrazo BL.Computed tomographic features ofesophageal intramural pseudodiverticulosis.Radiology 1983;147:189-90.

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