Intrapulmonary Bronchogenic Cysts Research Articles

Morphologic Features in Congenital Pulmonary Airway Malformations and Pulmonary Sequestrations Correlate With Mutation Status: A Mechanistic Approach to Classification.

Congenital pulmonary airway malformations (CPAMs) have a range of morphologies with varying cyst sizes and histologic features (types 1 to 3). Evidence suggested they arise secondary to bronchial atresia, however, we recently showed that cases with type 1 and 3 morphology are driven by mosaic KRAS mutations. We hypothesized that 2 distinct mechanisms account for most CPAMs: one subset is secondary to KRAS mosaicism and another is due to bronchial atresia. Cases with type 2 histology, similar to sequestrations, would be related to obstruction and therefore negative for KRAS mutations regardless of cyst size. We sequenced KRAS exon 2 in type 2 CPAMs, cystic intralobar and extralobar sequestrations, and intrapulmonary bronchogenic cysts. All were negative. Most sequestrations had a large airway in the subpleural parenchyma adjacent to the systemic vessel, anatomically confirming bronchial obstruction. We compared morphology to type 1 and 3 CPAMs. On average, type 1 CPAMs had significantly larger cysts, but there remained substantial size overlap between KRAS mutant and wild-type lesions. Features of mucostasis were frequent in sequestrations and type 2 CPAMs, while their cysts were generally simple and round with flat epithelium. Features of cyst architectural and epithelial complexity were more common in type 1 and 3 CPAMs, which rarely showed mucostasis. Similarity in histologic features among cases that are negative for KRAS mutation support the hypothesis that, like sequestrations, the malformation of type 2 CPAMs is related to obstruction during development. A mechanistic approach to classification may improve existing subjective morphologic methods.

Clinical Presentation and Surgical Management of Five Pediatric Cases with Bronchogenic Cysts: Retrospective Case Series.

Bronchogenic cysts (BCs) refer to congenital lesions that result from primitive or abnormal foregut budding, and can be pulmonary or mediastinal. Their occurrence can take place at any point on the tracheobronchial tree, but they are usually localized in the lung parenchyma and mediastinum, and may be symptomatic or asymptomatic. Bronchogenic cyst symptoms can vary, depending on the size and location of the cyst. A retrospective review of the charts of five patients with a histopathological diagnosis of bronchogenic cysts was performed between 2014 and 2020. The patients reported in this study were diagnosed and managed at Abha Maternity and Children Hospital, Abha, southwest Saudi Arabia. In addition, demographic information, as well as diagnostic and therapeutic information, was provided for each patient, both at discharge and after discharge. All patients had confirmed congenital bronchogenic cysts with different clinical phenotypes and radiological findings. All patients had histopathologically confirmed bronchogenic cysts with different clinical and radiological presentations. Two patients had mediastinal-located cysts; one had a laryngeal cyst; and the last two patients had infected intrapulmonary bronchogenic cysts. All patients underwent complete excision and did not experience recurrence or other postoperative complications during the follow-up period. The latter two patients required lobectomies of the right middle and upper lobes. Although bronchogenic cysts are considered a rare congenital pulmonary malformation, they should be considered in the differential diagnosis of pediatric patients with unusual airway and parenchymal lung manifestations, particularly, persistent stridor, feeding difficulty, and complicated pneumonia. Surgical excision of the cyst is the gold-standard therapy for symptomatic bronchogenic cysts and is highly recommended for asymptomatic ones. Long-term follow-up studies will be required to explore any long-term complications of BCs, particularly regarding the malignancy transformation.

The video-assisted thoracic surgery is the procedure of choice for the surgery of mediastinal bronchogenic cysts

Mediastinal bronchogenic cyst (MBC) is congenital, and a result of an abnormal budding of the primitive foregut. MBC is the most common mediastinal cyst, usually located along the tracheobronchial tree, most often found behind the carina (1). MBC can occur among all age group, from infants to adults. Bronchogenic cysts can occur intrapulmonary or extrapulmonary. If an abnormal budding occurs during early gestation, the bronchogenic cyst will usually be located in the mediastinum. MBCs rarely communicate with the tracheobronchial tree when compared to intrapulmonary bronchogenic cysts. MBCs are lined with respiratory ciliated columnar epithelium and filled with a whitish-gray mucinous material, which can change into pus with infection.

Kystes bronchogéniques intrapulmonaires

Bronchogenic cysts are congenital malformations that are usually located in the mediastinum. Intrapulmonary location is rare. Four cases of intrapulmonary bronchogenic cysts are reported in order to discuss their clinical and radiological presentation and their treatment. Intrapulmonary bronchogenic cysts diagnostic is often missed. This condition must however be known so as to foresee a resection in order to prevent a potential complication.

Cysts of the primary gut in children

Cysts of the primary gut are rare in children. In the clinic of pediatric surgery named after professor N.L.Kusch 43 children with primary gut cysts in age from one month to fifteen years during the last 30 years were on the treatment. Bronchogenic cysts had been diagnosed in 32 patients, enteric – 11. Females were 23, boys – 20. Intrapulmonary bronchogenic cysts location had 23 children, mediastinal - had 9. The indications to surgical treatment and their efficiency have been analyzed. Timely diagnostics and adequate operative treatment considerably reduce frequency of failures. /span>

Clinical Characteristics and Management of Intrathoracic Bronchogenic Cysts: A Single Center Experience

BackgroundThe aim of this study is to investigate the clinical characteristics and management of intrathoracic bronchogenic cysts.Materials and MethodsTwenty-four (n=24) patients with intrathoracic bronchogenic cysts were treated surgically between August 1990 and December 2009 at our institution. Patients were divided into two groups by bronchogenic cyst location: mediastinal or intrapulmonary. Symptoms at diagnosis, radiologic findings, locations, surgical methods, pathological findings, and surgical outcomes were investigated retrospectively from consecutive patient medical records.ResultsThere were 12 females (50.0%). The mean age was 26.8 (range, 5 to 64) years. The mean follow-up period was 27.3 (range, 1 to 121) months. There were 15 (62.5%) mediastinal and 9 (37.5%) intrapulmonary bronchogenic cysts. Symptoms occurred in 8 patients with mediastinal bronchogenic cysts (53.3%) and 5 patients with intrapulmonary bronchogenic cysts (55.6%) (p=1.000). On computed tomography (CT), 7 patients (46.7%) showed homogenous solid masses in mediastinal bronchogenic cysts and five (55.6%) patients exhibited heterogeneous cystic masses with air-fluid levels in intrapulmonary bronchogenic cystic masses. Open thoracotomy was performed in 17 (70.8%) patients, and video-assisted thoracic surgery was performed in 7 (29.2%) patients. On pathological findings, there were 16 (66.7%) complicated cysts, and in 13 symptomatic patients, 11 (84.6%) patients had complicated cysts. There was no operative death in this study. During the follow-up period, no recurrence was detected.ConclusionIntrathoracic bronchogenic cysts have a wide variety of clinical characteristics and radiologic findings. Even though some patients do not experience symptoms and signs caused by bronchogenic cysts, serious symptoms and complications may develop with the passage of time.

Bronchogenic Cysts of the Lung: Report of 29 Cases

Intrapulmonary bronchogenic cysts are congenital anomalies of the tracheobronchial tree and foregut. The aim of this retrospective study was to review the diagnosis, clinical and histological features, operative techniques, outcomes and follow-up of intrapulmonary bronchogenic cysts treated in a single institute. Twenty-nine patients with intrapulmonary bronchogenic cysts were treated surgically between 1990 and 2005. There were 17 female and 12 male patients and their ages ranged from 7 to 68 years. Patients were divided into two groups according to surgical procedure. Resection (lobectomy or wedge resection) was performed on Group I (n=18), and partial excision with de-epithelisation was performed on Group II (n=11). Twenty-five patients (86.2%) were symptomatic. Cough and sputum were the most common symptoms. Twenty-four of the 29 BCs were simple cysts (82.7%) whereas 5 (17.3%) were complicated cysts. Postoperative hospital stay was 4.55+/-0.86 days in group I and 6.54+/-3.34 days in group II (P=0.172). Complications in Group I were pneumonia in one case and wound infection in two cases; prolonged air leakage were observed in two cases of Group II. No statistical difference was determined between the complication rates of the two groups (P=0.91). However a significant difference was determined between the complication rates of simple and complicated cysts (P=0.026). Two cases in Group II showed recurrence, whereas no recurrence occurred in Group I. (P=0.065) No postoperative mortality was observed in any of the groups. All bronchogenic cysts should be treated surgically. We believe that partial excision with de-epithelisation may be an alternative to resection in symptomatic patients with limited respiratory capacity.

Intrapulmonary Bronchogenic Cysts: Computed Tomography, Clinical and Histopathologic Correlations

Bronchogenic cysts (BCs) are usually located in the mediastinum and they occur less commonly in the lung parenchyma. This study investigated the findings from computed tomography (CT) images, clinical presentation and histopathologic findings of intrapulmonary BCs. From the last 7 years, the CT images of 20 patients (12 females, 8 males; mean age, 38.8 +/- 21.7 years; median age, 34 years) with intrapulmonary BC were available. Contrast-enhanced CT findings were characterized and correlated with clinical presentation and histopathologic findings (using Fisher's exact tests). The majority of intrapulmonary BCs were subpleural in location (55%), in the lower lobes (60%), symptomatic (80%), and in adults (90%). Three CT patterns were identified: cyst with content of fluid attenuation (9 patients), cyst with air and fluid content (9 patients), cyst with content of soft tissue attenuation (2 patients). Preoperative diagnosis of intrapulmonary BC was correct in only 20% using the CT criteria of cysts with fluid attenuation and without anomalous blood supply. Cysts with air component were significantly larger than those without air component (p = 0.0452), but cyst size and air component were not correlated with clinical presentation. Surrounding infiltration or thick wall on CT were significantly correlated with the presence of any clinical symptom (p = 0.014) or fever (p = 0.042). CT findings of surrounding consolidation, ground glass opacity or thick wall were significantly correlated with chronic inflammation or pneumonic change on histopathology (p = 0.0008). There is a wide spectrum of intrapulmonary BCs that have CT findings that are correlated with clinical presentations and histopathologic findings.

Asymptomatic congenital lung malformations

Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.

Intrapulmonary bronchogenic cyst: CT and pathologic findings in five adult patients.

The purpose of our study was to describe the CT and pathologic findings of intrapulmonary bronchogenic cysts in five adult patients. Intrapulmonary bronchogenic cysts in adults appear on CT as well-defined ovoid lesions with the attenuation of soft tissue or water, similar to their mediastinal counterparts. Associated with these cysts are areas of mosaic low attenuation and bandlike linear attenuation representing histopathologically confirmed emphysema and bronchiolization or fibrotic change or both in the surrounding lung.

Esophageal duplication cyst associated with pulmonary cystic malformations

Two cases of esophageal duplication cyst associated with pulmonary cystic malformations (cystic bronchiectasis with pneumonia in one, intrapulmonary bronchogenic cysts with bronchial atresia in the other) are reported. The coexistence of these complex anomalies supports the recognition that esophageal duplication cyst also is an entity of a broad spectrum of developmental abnormalities caused by abnormal budding of the primitive foregut. Nine cases of similar complex anomalies in the lung and esophagus have been reported. Although rare, this malformation complex should be borne in mind in the treatment of pediatric mediastinal and pulmonary malformations.

CT Findings of Bronchogenic Cyst

We studied to evaluate CT characteristics of bronchogenic cysts. We retrospectively evaluated CT of 11 patients with pathologically proved bronchogenic cyst. Precontrast and postcontrast CT scan was performed in all. We analyzed CT with viewpoints of location, size, attenuation on pre- and postcontrast scan, and calcification. Three of 11 bronchogenic cysts were intrapulmonary in location and eight were located in the mediastinum. Two of 3 intrapulmonary bronchogenic cysts were located in the right lower lobe, and the remaining one was left lower lobe. Intrapulmonary bronchogenic cysts ranged from 6cm to 12cm in diameter (average, 9.7 cm). On CT, intrapulmonary bronchogenic cysts appeared as thin-wall air cyst, homogenous water attenuation and soft tissue attenuation with air bubble respectively. Mediastinal bronchogenic cysts were located in posterior mediastinum(n=5), superior mediastinum(n=2), middle mediastinum(n=1) respectively. These cysts ranged in size from 3cm to 8cm in diameter (average, 5.0 cm). On CT, five showed homogenous water attenuation, two soft tissue attenuation similar to that of muscle, one air-fluid level. Calcification or contrast enhancement was not detected in any cases. On operative findings, all of intrapulmonary bronchogenic cysts contained dirty pus-like material and all of mediastinal bronchogenic cysts contained whitish or yellowish mucus material. Bronchogenic cysts showed homogenous water density in many cases, homogenous soft tissue density, air-fluid level and air-filled cyst. The constellation of CT findings may be helpful in the diagnosis and differentiation of bronchogenic cyst.

Mediastinal bronchogenic cyst: prenatal sonographic diagnosis

Bronchogenic cyst of the mediastinum in infancy is a welldefined entity characterized by recurrent cough, wheezing, pneumonia, atelectasis, and obstructive emphysema and causing various degrees of respiratory distress [1 ]. These cysts generally are located near the carina between the trachea and the esophagus and often obstruct the main bronchi, causing respiratory distress[1]. The recognition of thoracic malformations in utero is important because many of these anomalies present with severe respiratory distress in the immediate neonatal period [2]. Early recognition and identification of a pulmonary abnormality can expedite obstetric management and direct effective postparturn treatment. Several conditions, among them congenital cystic adenomatoid malformation, diaphragmatic hernia, bronchopulmonary sequestration, hydrothorax, and intrapulmonary bronchogenic cysts have been reported to have distinct prenatal sonographic characteristics [2, 3]. We report a case in which the prenatal diagnosis of a bronchogenic cyst was made by sonography.

Congenital cystic disease of the lung

In 19 cases of congenital cystic disease of the lung, the cysts were always multiple or multilocular and thin-walled. Their walls resembled those of bronchioles with surrounding patches of smooth muscle without cartilage. The cysts communicated with bronchi proximally and with alveoli distally suggesting bronchiolectasis. However, there was also proliferation of bronchiolar structures, shown by their increase in number and by papillary overgrowth of the lining epithelium. Bronchiolar proliferation is also a feature of adenomatoid malformation. The 2 terms therefore, may possibly apply to different degrees of the same anomaly, a severe one producing stillbirth or neonatal death, and a milder one allowing survival long enough for successful surgical resection. One third of the children were full-term neonates, one third were aged 1 to 12 mth, and one third 1 to 10 yr. Fifteen of the specimens were surgical and 4 were necropsies. Half also had emphysema in the affected lobe, and 4 had associated extra lobar sequestration. None was associated with cysts in other organs. Radiologically and macroscopically, the overdistended lobe sometimes mimicked congenital lobar emphysema. All cases with more than focal chronic inflammation were omitted, in order to exclude post inflammatory pneumatoceles. Intrapulmonary bronchogenic cysts and intrapulmonary sequestrations differed in being usually single, often having cartilage and inflammatory fibrosis in their walls, and not having alveoli budding off the cyst. In 19 cases of congenital cystic disease of the lung, the cysts were always multiple or multilocular and thin-walled. Their walls resembled those of bronchioles with surrounding patches of smooth muscle without cartilage. The cysts communicated with bronchi proximally and with alveoli distally suggesting bronchiolectasis. However, there was also proliferation of bronchiolar structures, shown by their increase in number and by papillary overgrowth of the lining epithelium. Bronchiolar proliferation is also a feature of adenomatoid malformation. The 2 terms therefore, may possibly apply to different degrees of the same anomaly, a severe one producing stillbirth or neonatal death, and a milder one allowing survival long enough for successful surgical resection. One third of the children were full-term neonates, one third were aged 1 to 12 mth, and one third 1 to 10 yr. Fifteen of the specimens were surgical and 4 were necropsies. Half also had emphysema in the affected lobe, and 4 had associated extra lobar sequestration. None was associated with cysts in other organs. Radiologically and macroscopically, the overdistended lobe sometimes mimicked congenital lobar emphysema. All cases with more than focal chronic inflammation were omitted, in order to exclude post inflammatory pneumatoceles. Intrapulmonary bronchogenic cysts and intrapulmonary sequestrations differed in being usually single, often having cartilage and inflammatory fibrosis in their walls, and not having alveoli budding off the cyst.