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A 52-Year-Old Woman With Recurrent Hemoptysis* FREE TO VIEW

Kim-Hatt Lim, MBBS; Yew-Cheng Lim, MBBS; Chong-Kin Liam, MBBS, FCCP; Catherine Mee-Ming Wong, MBBS
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*From the Department of Medicine (Drs. K.-H. Lim, Liam, and Wong) and Department of Cardiothoracic Surgery (Dr. Y.-C. Lim), University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Correspondence to: Kim-Hatt Lim, Department of Medicine, Faculty of Medicine, University of Malaya, 50603 Kuala Lumpur, Malaysia; e-mail: limkh@medicine.med.um.edu.my

Chest. 2001;119(3):955-957. doi:10.1378/chest.119.3.955
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A 52 -year-old woman was referred to the hospital for management of recurrent hemoptysis. She also reported an episode of hematemesis, which was initially thought to be unrelated to the main presenting complaint. Three days prior to this admission, she received a blood transfusion of 6 U in another hospital for treatment of anemia as a result of repeated bouts of hemoptysis. On further questioning, she admitted to having had intermittent recurrent episodes of hemoptysis for 3 years. However, the physicians she consulted had not made any definitive diagnosis. There was no history of coughing with ingestion of solid or liquid food. She did not have dysphagia, fever, breathlessness, or chest pain. There was no history of recurrent pulmonary infections. She had no history of tuberculosis, malignancy, or chest trauma. She was a nonsmoker.

The patient was not cyanotic and was well-nourished. Her respiratory rate was 24 breaths/min. Her BP was 150/90 mm Hg, pulse rate was 90 beats/min, and temperature was 37°C. There was dullness to percussion and reduced breath sounds over the left lung base. The results of her cardiac examination were normal. No organomegaly was present. There were no enlarged lymph nodes, and she had no clubbing.

Laboratory investigations performed on hospital admission included hemoglobin, WBC, and platelet counts, a coagulation profile, blood glucose level, renal function, liver function, and urine microcopy, the results of which were all within normal limits. A chest radiograph demonstrated a left lower lobe consolidation. A computed axial tomographic scan of the thorax revealed consolidation involving the apical and posterior segments of the left lower lobe without evidence of tumor or enlarged mediastinal lymph nodes. On fiberoptic bronchoscopy, the tracheobronchial tree was normal except for the left lower lobe bronchus, which was not well-visualized due to an adherent blood clot in the lumen. Sputum and bronchial washing specimens that were sent for cytology, bacterial culture, acid-fast stains, and mycobacterial culture all yielded negative results.

Esophagogastroduodenoscopy was performed due to the earlier reported episode of hematemesis. It revealed a fistulous opening in the anterior wall of the middle third of the esophagus, 27 cm from the incisors. Fresh blood was continuously oozing from the fistula. The stomach and duodenum were normal. An image from esophagography, which was subsequently performed with 30 mL iopromide 300, a low-osmolar nonionic contrast medium, and a mouthful of thinly diluted barium, is shown in Figure 1 .

Diagnosis: Congenital bronchoesophageal fistula

In adults, communications between the esophagus and the tracheobronchial trees are mostly acquired. Most commonly, these fistulas have malignant origins. Acquired benign causes of tracheoesophageal or bronchoesophageal fistulas include prolonged intubation, infections (ie, tuberculosis, syphilis, histoplasmosis, actinomycosis, and candidiasis), inflammatory conditions (ie, Crohn’s disease and Behçet’s disease), corrosive ingestion, and iatrogenic complications (ie, endoscopic variceal sclerotherapy).

Congenital respiratory-esophageal fistulas are rare developmental anomalies. The persistent communication between the tracheobronchial tree and esophagus is thought to be the result of failed tracheobronchoesophageal separation in the early stage of embryonic development. Congenital tracheoesophageal fistulas occur in about 1 in 4,000 births, while the incidence of congenital bronchoesophageal fistulas, which are less common, is unknown. It is unusual to encounter such anomalous communications in adults. A congenital fistula that remains asymptomatic until adulthood is rare.

Bronchoesophageal fistulas occur with equal frequency in male and female patients. In a reported series of nine adults with such a condition, the average age at diagnosis was 35 years (range, 18 to 63 years). In a literature review of 100 patients with congenital bronchoesophageal fistulas reported between 1916 and 1990, these fistulas were found almost three times more commonly on the right than on the left side. The most commonly reported location of the fistula, between the middle third of the esophagus and the right lower lobe, occurred in 41% of the patients. The reason for this anatomical predilection is not understood.

A bronchoesophageal fistula is often associated with bouts of coughing when eating or drinking and with recurrent lung suppuration. Coughing was reported in 96% of patients. Bouts of coughing when swallowing liquids (Ohno’s sign) were present in 65% of patients. These symptoms, however, were absent in the present patient. Recurrent hemoptysis, which is present in 17% of reported cases, may prove fatal. The massive hemoptysis in our patient was probably the result of lung suppuration.

A fistula may cause symptoms in childhood but may not be manifest until adulthood. In the case presented, the fistula remained silent for 48 years. The relative lack of symptoms for many years is a well-recognized feature of this condition. Several explanations have been suggested for the long, silent interval until adulthood. Postulations include the presence of a membrane that subsequently ruptures, an occlusive valve-like mucosal flap that may close during swallowing due to the oblique direction of the fistulous tract, and spasm of the muscular layer occluding the lumen during swallowing.

The diagnosis of bronchoesophageal fistula is often made by esophagogram, with water-soluble contrast medium or dilute barium. This investigation provides a definitive diagnosis in 78% of cases. The esophagogram in the patient demonstrated a fistulous communication between the middle third of the esophagus and the left lower lobe bronchus. Bronchoscopy and esophagoscopy will sometimes reveal the orifices of the fistula, which are usually small and only recognizable when the exact sites are known. It has been noted that bronchoscopy was negative in 67% of cases and esophagoscopy was negative in 40% of cases when one or both of these examinations were performed. In this patient, the fistulous opening was demonstrated with esophagoscopy but not with bronchoscopy. The presence of a blood clot in the left lower lobe bronchus could have obscured the orifice of the fistula during bronchoscopy. Despite the frequent negative findings, bronchoscopic and esophagoscopic examinations are necessary to exclude acquired causes of the fistula. It is not unusual for the condition to be diagnosed only during surgery, despite extensive investigations, as the diagnosis may not be suspected preoperatively.

The congenital nature of the fistula in the adult may be assumed in the presence of chronic respiratory complaints without a demonstrated acquired cause and with histologic confirmation of an epithelial-lined tract with muscularis mucosa.

The recommended treatment is surgery that consists of the division of the fistula and the excision of any permanently damaged lung segments. Simple stapling has been advocated for elderly, poor-risk patients. This technique has the advantage of shorter operation time and less operative trauma. However, it has only been used in a small number of patients, and a histologic diagnosis cannot be made. Endoscopic obliteration of both the bronchial and esophageal ends of a congenital bronchoesophageal fistula with local application of sodium hydroxide and acetic acid has been recommended for patients who decline to undergo or are unfit for thoracotomy. However, the experience with endoscopic treatment is limited and may cause one to overlook other causes, such as malignant and inflammatory fistulas.

Clinical Course

The patient had a left lateral thoracotomy that demonstrated a connection about 5 mm in diameter and 2 cm in length between the middle third of the esophagus and the left lower lobe bronchus. Macroscopically, there was no abnormality in the surrounding area. The fistula was resected, and the esophageal and bronchial ends were sutured. Histologic examination of the resected fistulous tract revealed fibrous tissue and smooth muscle in the fistulous wall, and the lumen was covered with squamous epithelium. There was no evidence of inflammation, granulomatous disease, or malignancy. The patient’s postoperative course was uneventful.

  1. Congenital respiratory-esophageal fistulas are rare developmental anomalies, which may persist and remain asymptomatic until adulthood before the condition is recognized.

  2. The clinical presentations of such anomalous communication include bouts of coughing when eating or drinking, recurrent lung suppuration, and recurrent, sometimes life-threatening, hemoptysis.

  3. The congenital nature of the respiratory-esophageal fistula in an adult may be assumed in the presence of chronic respiratory complaints, without a demonstrated acquired cause, and with histologic confirmation of an epithelial-lined tract with muscularis mucosa.

  4. Esophageal disease should be considered, especially when patients have unexplained respiratory complaints.

  5. The diagnosis of congenital respiratory-esophageal fistula should be considered in an adult patient with unexplained recurrent hemoptysis, as this condition can be surgically corrected, thus preventing life-threatening complications, such as pulmonary sepsis and massive hemoptysis.

1 Acosta JL, Battesby JS. Congenital tracheoesophageal fistula in the adult. Ann Thorac Surg 1974; 17:51–56

Azoulay D, Regnard JF, Magdeleinat P, et al. Congenital respiratory-esophageal fistula in the adult: report of nine cases and review of the literature. J Thorac Cardiovasc Surg 1992; 104:381–384

Enoksen A, Lovaas J, Haavik PE. Congenital tracheo-oesophageal fistula in the adult. Scand J Thorac Cardiovasc Surg 1979; 13:173–176

Kinley CE, Lang HB. Congenital bronchoesophageal fistula in an adult: a case presenting with massive hemoptysis. Can Med Assoc J 1969; 100:390–392

Moorhead JM, Reid RA, Vontz FK. Congenital tracheoesophageal fistula in the adult. South Med J 1982; 75:1030–1031

Parry W, Juma A. Congenital bronchoesophageal fistula[ letter]. Ann Thorac Surg 1991; 51:346–347

Rama OJ, Salo JA, Mattila SP. Congenital bronchoesophageal fistula in the adult. Ann Thorac Surg 1995; 59:887–890

Risher WH, Arensman RM, Ochsner JL. Congenital bronchoesophageal fistula. Ann Thorac Surg 1990; 49:500–505

Smith BD, Mikaelian DO, Cohn HE. Congenital bronchoesophageal fistula in the adult. Ann Otol Rhinol Laryngol 1987; 96:65–67

Weissberg D, Kaufman M. Bronchoesophageal fistula in adults: congenital or acquired [letter]? J Thorac Cardiovasc Surg 1990; 99:756–757

Figure Jump LinkFigure 1. Esophagogram demonstrating a fistulous communication (arrow) between the middle third of the esophagus and the left lower lobe bronchusGrahic Jump Location


Figure Jump LinkFigure 1. Esophagogram demonstrating a fistulous communication (arrow) between the middle third of the esophagus and the left lower lobe bronchusGrahic Jump Location



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