Surg Today (2001) 31:446–449

Congenital Bronchoesophageal Fistula with Crohn’s Disease inan Adult: Report of a Case

Hiroyuki Kato, Minako Yoshikawa, Tetsuya Saito, Minoru Fukuchi, Ryouji Kato, and Hiroyuki Kuwano

Department of Surgery I, Gunma University School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan

classified adult congenital bronchoesophageal fistulainto four types: Type I is a wide-necked diverticulumwith an inflammatory fistula at its tip, Type II is a simplefistula, Type III is a fistula with a cyst, and Type IV isa fistula with a sequestrated segment. Risher et al.6

reported that the prevalence of Type IV according toBraimbridge’s classification was only three out of 100cases of congenital bronchoesophageal fistula in theworld literature. In addition, congenital bronchoeso-phageal fistula concomitant with Crohn’s disease hasnever been reported.

In the present report, we document a rare case ofa congenital Type IV bronchoesophageal fistula ac-cording to Braimbridge’s classification that developedwhile the patient was undergoing treatment for Crohn’sdisease. The patient underwent a fistelectomy as-sisted by thoracoscopy, and a favorable outcome wasobtained.

Case Report

The patient was a 65-year-old man who had been re-ceiving steroids for the treatment of Crohn’s disease atanother hospital since 1989. From about 1991 he hadfrequently developed bronchitis or pneumonia. A leak-age of contrast medium from the middle region of theesophagus was found on esophagography, and a fistulawas detected by esophagoscopy in the middle of theesophagus. From about 1994, coughing occurred whendrinking liquid, and chest pain developed in 1996. InAugust 1996, endoscopic treatment with fibrin glue wasattempted, but the fistula could not be closed. FromMarch 1997, the injection of Histoacryl (synthetic glue)into the fistula and clipping of the fistula were per-formed, but no favorable effect was obtained and thefistula thereafter expanded. In January 1998, the patientwas admitted for surgical treatment at the First Depart-ment of Surgery, Gunma University Hospital.

Abstract A 65-year-old man was diagnosed to haveCrohn’s disease in 1989. In 1991, frequent bronchitisdeveloped, and bronchoesophageal fistula was diag-nosed by esophagography. On esophagography andaortography, the disease was diagnosed to be Type IVbased on Braimbridge’s classification accompaniedby pulmonary sequestration. A thoracoscope-assistedfistelectomy was performed. This paper reports thefindings of a fistelectomy assisted by thoracoscopy forthe treatment of a Type IV bronchoesophageal fistulaaccording to Braimbridge’s classification accompaniedby Crohn’s disease.

Key words Congenital bronchoesophageal fistula ·Crohn’s disease

Introduction

Most cases of bronchoesophageal fistula in adulthooddevelop as a sequela of malignant diseases such asesophageal cancer and pulmonary cancer,1,2 and con-genital cases are rare. When this disease develops inadulthood, a clear distinction between congenital andacquired cases is often difficult.3 As a diagnostic stan-dard for congenital bronchoesophageal fistula in adults,the following three points were proposed by Brunner4

in 1961: (1) the absence of inflammation around thefistula and esophagus at the time of operation, (2) noadhesion of the lymph nodes to the fistula, and (3) his-tologically, the fistula has a normal esophageal mucosaand also muscularis mucosa. Braimbridge and Keith5

Reprint requests to: H. KatoReceived: June 5 2000 / Accepted: November 20, 2000

447H. Kato et al.: Congenital Bronchoesophageal Fistula in an Adult

On a barium esophagram, a fistula from the middle ofthe esophagus in the left lower lobe was found. No otherabnormalities were observed (Fig. 1). On esophago-scopy, three fistulae, a cicatrization of the mucosa, andsmall polypoids were found on the left at an area some32 cm from the incisors, but no aphthoid changes, malig-nant features, esophagitis, or diverticula were evident(Fig. 2). The lesions were biopsied, but neither malig-nant features nor noncaseous granuloma were found.On chest CT, in addition to the bronchoesophagealfistula, a chronic inflammation of the left lower lobe wasfound, but no malignancy or mediastinal abnormalitywas detected. Pulmonary sequestration was not distinct.

On colonoscopy, a cicatrized ulcer in the end of thecolon and a deformation of the cecum were found, butthere was no evidence of any active Crohn’s disease. Onbronchoscopy, the fistula could not be observed clearly.Aortography demonstrated a directly nourishing bloodvessel from the descending aorta to the left lower lobe(Fig. 3).

Based on these results, the illness was diagnosed to bea congenital Type IV bronchoesophageal fistula accord-ing to Braimbridge’s classification, and a fistelectomywas thus performed under thoracoscopic assistance.

During the operation, tight adhesion between the lowerlobe of the left lung and the pleura of the chest wall wasfound. After separating them, a fistula between the pre-sumptive S6 lung segment and esophagus was observed(Fig. 4). No adhesion of the lymph nodes around thefistula or inflammatory changes were found, and thefistula was also easily exposed. The fistula was incised,

Fig. 1. Barium esophagram showing a fistula from the middleof the esophagus to the left lower lobe and leakage of contrastmedium from there to be bronchus

Fig. 2. Esophagoscopy shows three fistulae, cicatrization ofthe mucosa, and small polypoids on the left at an area some32 cm from the incisors

Fig. 3. Aortography demonstrates a directly nourishing bloodvessel from the descending aorta to the left lower lobe

448 H. Kato et al.: Congenital Bronchoesophageal Fistula in an Adult

Fig. 4. Operative photography showing the fistula extendingfrom the pulmonary tissue to the esophagus

and the esophageal and bronchial walls were closed bydouble-layer suturing, respectively. Since no pulmonarydestruction due to infection was observed, the opera-tion was finished without conducting a resection of thelung. A pathological examination showed the squamousepithelium accompanied by muscularis mucosae, butno noncaseous granuloma. In the transitional region, achange to a columnar epithelium was observed. Thepostoperative course of the patient was uneventful andhe was discharged on the 18th postoperative day with-out any symptoms.

Discussion

Fistula between the bronchus/trachea and the esopha-gus results from a failed tracheoesophageal separationin the early stage of embryonic development.7 The tra-

cheal band appears on the ventral aspect of the primi-tive foregut at the 3-mm stage. At the 5-mm stage thereis a rapid elongation of the trachea, which then sepa-rates from the esophagus. A persistent attachmentbetween the tracheobronchial tree and the esophagusresults from the discoordination of these two processes.The eventual site of the fistula in the bronchial treedepends on the degree of tracheoesophageal separationthat occurs before a caudal elongation of the trachea.Therefore, if rapid growth occurs in the early stage, thenthe fistula will be low in the bronchial tree.8

The main symptoms of congenital bronchoesoph-ageal fistula are coughing while drinking and eating,followed by sputum, fever, and bloody sputum. Theexpectoration of sputum differs depending on changesin the body position. Many cases are incidentally foundduring general examinations because the symptomsare mild. Both esophagography and esophagoscopy areuseful examination methods, and the fistula can be di-rectly observed by the latter modality in many cases.

Congenital bronchoesophageal fistula concomitantwith Crohn’s disease has never been reported. Bron-choesophageal fistula accompanied with Crohn’s dis-ease is rarely found, and only four cases have beenreported to date.9–12 These cases were diagnosed ashaving Crohn’s disease before the detection of bron-choesophageal fistula, and the manifestation of bron-choesophageal fistula coincided with the active phaseof Crohn’s disease. In the present case, no aphthoidchanges were seen on esophagoscopy and no non-caseous granuloma was apparent in the pathologicalexamination. In addition, bronchoesophageal fistulawas detected during the inactive period of Crohn’sdisease.

Regarding treatment, a fistelectomy is normally con-sidered to be adequate, but a concomitant pulmonarylobectomy is also frequently performed. In the presentcase, endoscopic treatment was initially attempted butthe fistula could not be closed, and surgery was thusperformed. To minimize the degree of operative inva-siveness, a thoracoscope-assisted fistelectomy was suc-cessfully performed. This method decreases morbidityand mortality, especially in elderly patients. Rämö etal.13 experienced nine patients with bronchoesophagealfistula, one of whom died before treatment as a result oflung complications, thus further emphasizing the needfor prompt surgical treatment.

In summary, it is recommended that the surgicaltreatment of a congenital bronchoesophageal fistulashould be promptly performed after making a definitivediagnosis to avoid any aggravation of pulmonary com-plications and a possible fatal outcome.14 Surgery usinga thoracoscope seems to be one of the most suitablemethods, since it reduces the degree of operativeinvasiveness.

449H. Kato et al.: Congenital Bronchoesophageal Fistula in an Adult

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