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Ann Thorac Surg 1996;61:1636-1640
© 1996 The Society of Thoracic Surgeons

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Original Article: General Thoracic

Bronchogenic Cysts of the Lung

Service de Chirurgie and Laboratoire d'Anatomie Pathologique, Hôpital Calmette, Centre Hospitalier Régional Universitaire, Lille, France

Accepted for publication February 22, 1996.

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Background. The clinical presentation of lung bronchogenic cysts (BC) is variable, from respiratory distress at birth to late appearance of symptoms.

Methods. This study of BC was based on a retrospective review of 41 cases: 21 infants and children and 20 adults, aged 1 day to 68 years. The diagnosis was antenatal in 4 cases. Three infants required mechanical ventilation, and 2 had their cyst drained before resection. Twenty infants and children and 17 adults underwent operations.

Results. Compression was the most important complication in infants and children. Cough, infection, and hemoptysis occurred later in life; 80% of the total population was symptomatic. Seven cysts were infected. There were no deaths after resection, and there was no recurrence of symptoms during the follow-up period (13 months to 21 years).

Conclusions. Bronchogenic cysts originate from the foregut. Differentiation from other acquired or congenital lesions can be difficult. It is uncertain what proportion of BC remain asymptomatic. More than half of patients are diagnosed after the age of 15 years, and complications may appear late. Clinical findings and plain chest radiograms are often sufficient for diagnosis. Lobectomy is the standard treatment, whereas drainage is a temporary, palliative, and risky procedure in cases of life-threatening compression. We conclude that a symptomatic BC is an indication for resection and that the long-term prognosis of an asymptomatic BC is unpredictable. Thus, there is a role for preventive operations.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Congenital bronchogenic cysts (BC) of the lung are observed not only in infants and children, but also late in adults. They can threaten life by producing compression, infection, hemorrhage, and rupture. Some probably remain unknown. The objectives of this retrospective study were to review the clinical presentations of BC and to discuss their embryology, pathology, evolution, and treatment, which are controversial.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Over the 25-year period from 1967 to 1993, 21 infants and children (ages 1 day to 15 years) and 20 adults (ages 17 to 68 years) were admitted at one institution (Fig 1). Clinical records were available in all cases and histopathologic reports in 37 cases. The mean postoperative follow-up period was 2.6 years (range, 13 months to 21 years). Seven patients were lost to follow-up.

View larger version (12K): [in this window] [in a new window]   Fig 1. . Histogram of ages of patients with bronchogenic cyst. (D = day; M = month; Y = year.)

Drainage of the cyst was performed first in 3 patients because of an important intrathoracic compression. The operative approach for 20 patients consisted of posterolateral (7), lateral axillary (10), or anterolateral submammary (3) thoracotomy. Fifteen lobectomies were performed. One of these was associated with a lingulectomy, as the lingula did not aerate after a left lower lobectomy for a compressive cyst; one accompanied a partial pleurectomy for dense and hemorrhagic adhesions by an infected cyst; and one was done with a section suture of a large symptomatic patent ductus. The other procedures included one trisegmentectomy of the left pulmonary apex, one right upper dorsal segmentectomy, two total excisions of the cyst with sutures of bronchiolar orifices, and one subtotal excision with silver nitrate cauterization of the deepest part of the cystic wall. The three excisions were performed for subpleural locations of the cysts.

Adults
There were 5 men and 15 women. Standard posteroanterior and lateral radiograms were done in all cases, laminograms were done in 3 early cases, and computed tomograms were available for 8 patients. Fiberoptic bronchoscopy was done in 6 patients and transthoracic needle biopsy in 1 patient.

The operative approach for 17 patients consisted of posterolateral (10), lateral axillary (3), or anterolateral submammary (4) thoracotomy. Eleven lobectomies were performed, three of them associated with partial pleurectomies for pleural adhesions. The other procedures included three segmentectomies, one of them associated with a partial pleurectomy; and three excisions of the cyst, two of them associated with sutures of bronchiolar orifices and one done for a mucus-filled cyst with no bronchiolar suture.

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Infants and Children
One of the cysts, which was diagnosed in utero at a gestational age of 27 weeks, caused fetal edema and anasarca. After the results of fetal karyotyping by amniocentesis had proved normal, thoracoamniotic shunting with a double pigtail (Rocket-Kings catheter, London, UK) silicone catheter was performed under ultrasound scanning. The cyst size decreased from 30 to 10 mm on echograms, and signs of fetal vitality improved. However, the membranes ruptured 2 days later; the premature newborn had anotia, was not brought to operation, and died within a few hours. No autopsy was performed.

The three other cysts diagnosed before birth were symptomatic immediately after delivery. Two neonates in respiratory distress were given mechanical ventilation before operation on their first day of life, 1 of them after 2 hours of decompressive drainage (Fig 2). A third neonate improved under oxygen therapy and then required ventilation on his seventh day of life, before operation on the same day.

View larger version (127K): [in this window] [in a new window]   Fig 2. . The diagnosis of this cyst was antenatal. The neonate was in respiratory distress and was immediately ventilated. A drain was put into the cavity to alleviate the pressure, which decreased gradually over 2 hours. A left lower lobectomy was performed on the first day of life.

Four cysts had always been asymptomatic and remained so at the time of operation. These had been diagnosed for 3 years in a 14-year-old girl, for 9 years in a 10-year-old boy, and for an undetermined period in a 12-year-old girl and a 13-year-old boy.

Associated malformations were thoracic in 3 cases: funnel chest, patent ductus (symptomatic at 4 months), and partial stenosis of the right pulmonary artery (diagnosed on the first day of life and asymptomatic at 18 months, perhaps because of mediastinal shift). Four benign anomalies were discovered at thoracotomy: a tracheal bronchus, an azygos lobe, an extralobar lung segment, and a pericardial defect. Malformations were extrathoracic in 3 cases: anotia, abdominal wall hypoplasia, and megacolon (same patient as the one with the azygos lobe). The clinical presentation of the pediatric population is summarized in Table 1.

View larger version (15K): [in this window] [in a new window]   Fig 3. . Locations of bronchogenic cysts.

View larger version (106K): [in this window] [in a new window]   Fig 4. . This cyst compressed the middle lobe bronchus and caused repeated bronchopulmonary infections for 3 months in a 7-year-old boy. It was situated in the lower lobe. A lobectomy was performed.

There were no deaths in the 20 patients after operation. Morbidity consisted of pulmonary infection (3) including two infected cysts, scoliosis (2), persisting pain (1), and retarded growth (1). The incompletely resected cyst did not recur during the period of follow-up (4 years). Symptoms did not reappear in 18 children during a mean period of 3 years and 6 months (range, 14 months to 21 years). Two were lost to follow-up.

Adults
For 15 symptomatic patients, the mean delay between appearance of symptoms, discovery of the cyst, and operation was 2 years and 3 months (range, 3 weeks to 12 years). In 2 cases, the delay could not be determined. Suppurative bronchitis, pneumonia, hemoptysis, and pain characterized the clinical presentation in adults (see Table 1). Three patients had always been asymptomatic, and remained so at operation. The cysts had been diagnosed for 5 years in a 42-year-old man, for 11 months in a 46-year-old woman, and for an undetermined period in a 31-year-old man. There was only one associated malformation: an extralobar lung segment discovered at thoracotomy.

Locations of the cysts are summarized in Figure 3. Their presentations on standard roentgenograms are shown in Table 2. Three cysts had been observed as solid masses before cavitating and containing a fluid level (Figs 5, 6), one of them during 6 years in a 59-year-old man. There was no case of compression of the mediastinum and diaphragm. Computed tomograms confirmed the presence of a round mass inside an area of pneumonia in 1 case and under a pleurisy in 1 case. They also showed what proved to be blood clots in a cavity in 1 case (Fig 7) and a communication with the bronchial tree in 2 cases. Endoscopy ascertained the origin of blood in 5 patients with hemoptysis and eliminated carcinoma in 1 patient with heavy smoking habits, weight loss, and a solid mass. Transthoracic needle biopsy of a mass in the lingula produced inflammatory cells. Preoperative diagnosis was made in 11 cases; diagnosis was uncertain in 4 cases of solid masses and 2 cases of cavities.

View larger version (152K): [in this window] [in a new window]   Fig 5. . This cyst was discovered in a 28-year-old woman complaining of chronic thoracic pain and dyspnea.

View larger version (166K): [in this window] [in a new window]   Fig 6. . (Same patient as in Figure 5.) Twenty days after the diagnosis, acute pain occurred. The cyst was infected with Streptococcus, had fistulized into a bronchus, and had ruptured into the pleura. An excision with bronchiolar suture was done.

View larger version (126K): [in this window] [in a new window]   Fig 7. . This cyst was discovered after hemoptysis in a 30-year-old woman. It contained clots. A left lower lobectomy was performed.

The diameter of the 17 resected cysts varied between 3.5 and 10 cm (mean, 5.5 cm). One was contained in an extralobar segment by the left lower lobe. The contents were purulent in 5 cases, in which cultures found Streptococcus (2), E coli (2), and Enterococcus (1). Histopathologic examination showed a ciliated uni (8) or pluri (6) stratified ciliated columnar mucosal lining in 14 cases, with cuboid and/or malpighian metaplasia in 3 cases. The submucosal layer was inflammatory in 4 cases. Bronchial glands (1), smooth muscle (10), islets of cartilage (4), and nerve tissue (2) were also found in the cyst wall. In 3 cases, including two aerated cavities and one with a fluid level, no submucosal constituent of the bronchial wall was identified. However, there was no previous history of lung infection to suggest an acquired pneumatocele. Neighboring atelectasis was noted in 2 cases, chronic pneumonia in 3, bronchiolar ectasis in 3, and emphysema in 1. There were no other pericystic anomalies.

There was one postoperative bronchial fistula after a right lower lobectomy for an infected cyst. It was treated with pleural drainage and endoscopic cauterization with silver nitrate. There was no further morbidity. Symptoms did not recur in 15 adults during a mean period of 1 year and 6 months (range, 15 months to 2 years and 6 months). Two were lost to follow-up. The 3 patients who had refused operation did not answer our inquiries, and their family doctors had lost contact with them.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Bronchogenic cysts of the lung originate from the foregut, as do such cysts in the mediastinum [1–3]. The foregut cysts develop within the cleavage between the respiratory tract and the digestive tube. When they form early, they are located in the mediastinum, by the trachea and esophagus or close to the carina and main bronchi. When they occur later, during bronchial budding and branching, they grow within the lung [4, 5]. The later the anomaly develops, the more peripheral it is in the lung. The congenital or acquired nature of the lung cyst is sometimes difficult to ascertain. Although the late appearance of symptoms or late radiologic discovery does not preclude a congenital nature of the lesion, the prevalence of associated maformations is high in infants and children and low after the age of 15 years. A history of bronchopulmonary infection is important to consider; it may be difficult to differentiate a BC from a bronchial atresia that has become cystic or from a chronic healed lung abscess communicating with a bronchiole and lined with a spreading epithelium. On the other hand, infection can also alter the wall of a BC.

Other lung cysts of mesothelial or endothelial origin exist, and different lesions can appear as cysts on radiograms: a sequestration, a giant bulla, a hydatid cyst, lobar emphysema, and even interstitial pulmonary emphysema after successful treatment of newborn respiratory distress [4, 6]. In particular, the frontier between solitary BC and type I congenital cystic adenomatoid malformation is undecided [7]. Similarities include the mucosal lining, ages and modes of presentation [8, 9], absence or presence of cartilage [10], communication with the bronchial tree [11], location, and lung tissue modifications due to compression and infection [5]; these characteristics are not discriminating or they may blur the distinction [12]. Some authors have stated that true congenital cystic adenomatoid malformation includes only types II and III, consisting of a solid mass of bronchiolar microcysts with cuboid epithelium or of numerous microcysts less than 1 cm in size, lined with cuboid cells and showing a pseudobronchiolar structure [13].

The embryologic and pathologic features of a cyst are perhaps less important to discuss than its clinical presentation and the decision to operate. The prevalence of cystic pulmonary diseases is difficult to determine because of their different forms and natures; Cooke and Blades [14] estimated it as 0.04% to 0.06%. An historic study by Sehenck [15] showed that 10% of cases were recognized at birth, 14% were discovered in the first year of life, and 57% were found in patients over the age of 15. Our figures are not different. A minority of 20% of BC is asymptomatic, and this figure is the same before and after 15 years of age in our experience. Complications are still observed during the sixth decade of life. We did not operate on any asymptomatic patients older than 42 years because we did not observe any. It is difficult to know what percentage of undiagnosed or diagnosed, untreated cysts remains asymptomatic during a lifetime.

Cysts often seem to have check valves, which lead to their enlargement, producing compression. This compression is common in neonates, infants, and children; it can be life-threatening [7] and thus requires immediate treatment. It can also be observed and treated during gestation. Nicolaides and Azar [16] treated three large unilocular lung cysts, surrounded by smooth muscle and bars of cartilage, with progressive enlargement causing mediastinal shift and compression of the contralateral lung. Thoracoamniotic shunting was performed in the 24th, 25th, and 32nd weeks of gestation, and lobectomies were done after deliveries at 38, 39, and 33 weeks of gestation, respectively.

Later on in life, cysts usually lose this compressive character and may remain asymptomatic until infection occurs, producing cough, dyspnea, and thoracic pain [8]. The incidence of infection differentiates lung cysts from mediastinal cysts [3]. Hemorrhage is not unusual, and pleural perforation is possible. The presence of a fluid level is a sign of airway communication. This complication may appear late after the discovery of the cyst, and for this reason, operation should not be limited to symptomatic young patients. In adults, the diagnosis may be delayed, and there is a threat of bacterial or fungal infection.

Clinical findings and plain chest radiograms are often sufficient for diagnosis. Computed tomograms are not uniformly necessary, particularly in infants and children, although they can be helpful in cases of atelectasis or pneumonia. They are more helpful in adults, as neighboring lung modifications become more frequent. Endoscopy and transthoracic needle biopsy are rarely contributive, and are used when carcinoma is suspected.

Lobectomy is the standard procedure, as the cyst is often surrounded by areas of atelectasis and pneumonia. Anatomic segmentectomy is reasonable for a small cyst. Localized excision can be done for a superficial cyst; hemostasis of the surrounding lung tissue must be complete and bronchiolar air leaks, sometimes difficult to detect, must be carefully sealed. Drainage of a compressive cyst in a child remains a useful but temporary palliative procedure, introducing the risk of tension pneumothorax, and thoracotomy should not be delayed. We have no experience with video-assisted thoracoscopic surgery for these cysts; its indication should consider the importance of duration of the operation in pediatric cases and the degree of infection in adult cases.

Deaths are unusual, and morbidity is acceptable. We conclude that the diagnosis of a symptomatic bronchogenic lung cyst is an indication for resection in all cases, that late complications of an asymptomatic lung cyst are frequent, and that its prognosis is unpredictable; thus, there is a role for preventive operations.

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 
Address reprint requests to Dr Copin, Laboratoire d'Anatomie Pathologique, Hôpital Calmette, F 59037 Lille Cedex, France.

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment References

 

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Michel E. Ribet Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ribet, M. E. Articles by Gosselin, B. H. Search for Related Content PubMed PubMed Citation Articles by Ribet, M. E. Articles by Gosselin, B. H.