Congenital lung malformations.

Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.

Spindle-cell thymoma colliding with a bronchogenic cyst in a Yorkshire terrier.

An 8-year-old male Yorkshire terrier was presented to the Tufts Veterinary Hospital for evaluation of increased respiratory effort. A mediastinal mass composed of a spindle-cell thymoma within a bronchogenic cyst was diagnosed with computed tomography thoracic imaging, ultrasound-guided fine-needle aspirate biopsy, and histopathologic evaluation after surgical removal. Histologic evaluation showed a multilocular cyst structure as well as a mass characterized by spindle to polygonal thymic epithelial cells. The cyst was characterized by a lining of ciliated pseudostratified respiratory epithelium. To the authors' knowledge, this is the first report of a spindle-cell thymoma being associated with a mediastinal bronchogenic cyst in a dog.

[Diagnosis and treatment of pediatric cervical bronchogenic cyst].

Objective:To investigate the clinical characteristics and surgical treatment outcomes of children with cervical bronchogenic cysts. Methods:A retrospective study of 6 pediatric patients with bronchogenic cysts in the neck region treated in our hospital during 2014 to 2020 was performed. All children underwent complete resection of cervical mass under general anesthesia. Results:There were 6 children, aged from 1 to 5 years, with a median of 2.25 years. There were 3 males and 3 females. The lesions were located on the left neck in 3 cases, the midline neck in 2 cases and the right neck in 1 case. The clinical manifestations were painless mass in 5 cases and recurrent neck infection in 1 case. The size of the mass ranged from 2.1 to 7.5 cm. There was no characteristic clinical or imaging features of bronchogenic cysts. Misdiagnosed as lymphangioma in 3 cases, thyroglossal cyst in 2 cases and piriform fistula in 1 case. The follow-up ranged from 1.50 to 7.75 years, with a median of 4.13 years. All 6 children had no recurrence or complications. Conclusion:Although rare, bronchogenic cysts should be considered in the differential diagnosis of cervical cystic masses in children. Surgery is the most effective way to treat cervical bronchogenic cyst, and histopathological examination is the gold standard for diagnosis.

[A case of mediastinal bronchial cyst misdiagnosed as schwannoma: a case report].

A case of mediastinal bronchial cyst misdiagnosed as schwannoma is reported. The patient had no chest pain, dyspnea, cough, sputum and hemoptysis in the past. Chest enhanced CT showed mediastinal mass with clear boundary. The larger cross-section was about 3.9 cm × 3.4 cm, and there was no obvious enhancement. The primary diagnosis was considered as schwannoma, tracheal cyst or esophagus cyst. The mediastinal tumor was resected via cervical approach under general anesthesia. During the operation, it was found that the mass was located in the middle and upper mediastinum, with diameter of about 4 cm and a smooth surface, containing gelatinous cystic fluid and adhered to the esophagus and trachea. Postoperative pathological diagnosis was bronchogenic cyst. The patient recovered well after the operation, and has not recurred since follow-up.

Mediastinal cysts : a 52-case retrospective study.

INTRODUCTION: mediastinal cysts are rare lesions developed from mediastinal structures. They may be acquired like thoracic duct cysts or lymphangiomas or congenital like the bronchogenic cysts, enteric cysts or celomic cysts. These cysts are rare and may cause diagnostic challenges. AIM: To assess the major characteristics of these cysts based on a single institution experience. METHODS: the authors performed a descriptive, retrospective study from January 2009 to March 2020 in a single institution. Cystic lesions taking birth from the mediastinum for which gross features, microscopic features were available were included. RESULTS: this study contained 52 mediastinal cysts that were completely resected and no patient presented complications after the surgical resection. The bronchogenic cysts were the most frequent and represented 57.69% of all lesions. Thymic cysts and pericardial cysts represented respectively 40.38% and 1.92% of the cases. The positive diagnosis was based on the microscopic exam. The final diagnosis was concordant with the radiologic findings in 15 cases reaching a rate of 28%. CONCLUSION: the diagnosis of mediastinal cysts is based on the microscopic analysis of the cystic wall. Pericardial cysts may be suspected based on their characteristic location in the cardiophrenic angle, thymic cyst may be evoked based on their location in the thymic region and bronchogenic cysts are mainly located in the middle mediastinum. Inspite of these most frequent locations, the cysts may be located in any part of the mediastinum and may be difficult to diagnose when the key diagnostic features are absent.

A 4-Month-Old Girl with Persistent Respiratory Distress and Multiple Admissions to the Pediatric Intensive Care Unit Due to a Congenital Bronchogenic Cyst.

BACKGROUND Bronchogenic cysts are foregut duplication cysts that result in congenital cysts of the tracheobronchial tree. They can be fatal especially, if they present in early infancy. Persistent respiratory distress is the most frequent reported presentation. Histologically, bronchogenic cysts are unilocular fluid-filled lesions that are composed of respiratory epithelium. This report presents the case of a 4-month-old girl with persistent respiratory distress and multiple admissions to the Pediatric Intensive Care Unit (PICU) due to a congenital bronchogenic cyst. CASE REPORT We describe a 4-month-old girl with persistent respiratory distress, intermittent chocking, and recurrent PICU admissions. The patient was managed as a case of bronchiolitis, which led to ineffective treatment numerous times. Radiological work-up revealed unusual findings of asymmetrical hyperinflation. Bronchoscopy, which was performed to clear the airway and retrieve a possible foreign body, showed a non-pulsatile mass compressing the entry of the main bronchi with more pressure on the left main bronchus, raising the possibility of an external compression. A bronchogenic cyst was suspected and confirmed with high-resolution computed tomography (HRCT). Surgical intervention was performed, with no reported complications. CONCLUSIONS This report has shown that in neonates presenting with respiratory distress and no signs of infection, a broad differential diagnosis including congenital cysts should be considered. As in this case, lung and thoracic imaging are required to confirm the diagnosis. We also highlight the need to involve subspecialities to avoid potential delay in diagnosis or exposing patients to unexpected complications.

[Congenital Malformations of the Lung - an Overview]. / Angeborene Fehlbildungen der Lunge ­ eine Übersicht.

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.

Congenital Mediastinal Neuroenteric Cyst as a cause of respiratory distress since birth.

Mediastinal Neuroenteric Cyst is a rare congenital presentation in infants and is associated with a high mortality rate. It is a very uncommon benign lesion and usually develops from abnormal embryological development of the foregut. Till now, only 106 cases have been reported worldwide. In Pakistan only three cases have been published, with varying presentations. The clinical presentation and age at presentation vary from asymptomatic and coincidental finding on chest x-ray, to limb numbness or early presentation with severe symptoms like those in our case. In fact, it poses an important challenge for paediatricians. We present a rare case with emphasis on clinical presentation and diagnostic criteria.

Transesophageal endoscopic resection of mediastinal cysts (with video).

BACKGROUND AND AIMS: Primary mediastinal cysts are infrequent lesions derived from a variety of mediastinal organs or structures. Complete surgical resection is the treatment of choice even in asymptomatic patients to prevent severe adverse events (AEs) and to establish the diagnosis. Transesophageal endoscopic resection of benign mediastinal tumors has been proven feasible. The aim of this study was to evaluate the feasibility, safety, and efficacy of transesophageal endoscopic surgery for mediastinal cysts. METHODS: From January 2016 to May 2021, patients with mediastinal cysts who underwent transesophageal endoscopic resection were retrospectively included. Clinicopathologic characteristics, procedure-related parameters, AEs, and follow-up outcomes were analyzed. RESULTS: Ten patients with mediastinal cysts were included in this study. The mean cyst size was 3.3 ± 1.3 cm. Histopathology revealed 3 bronchogenic cysts (30.0%), 4 esophageal duplication cysts (40.0%), 2 gastroenteric cysts (20.0%), and 1 lymphatic cyst (10.0%). All procedures were performed uneventfully without conversion to traditional surgery. En-bloc resection was achieved in 6 patients (60.0%). Aggressive resection was avoided to prevent damage to the surrounding vital organs. Mean resection time and suture time were 58.0 ± 36.4 minutes and 5.4 ± 1.0 minutes, respectively. No major pneumothorax, bleeding, mucosal injury, or fistula occurred. One patient had a transient febrile episode (>38.5°C). Mean postoperative hospital stay was 2.7 ± .9 days. No residual or recurrent lesions were observed in any patient during a mean follow-up period of 29.8 ± 19.5 months. CONCLUSIONS: Transesophageal endoscopic surgery appears to be a feasible, safe, effective, and much less invasive approach for mediastinal cyst resection. Larger prospective studies are required to fully assess the efficacy and safety of this novel technique.

Atypical chest pain in a young woman with an interatrial bronchogenic cyst.

A 31-year-old woman presented to the emergency department with atypical retrosternal chest pain and dyspnoea. Investigations initially revealed atrial flutter on her electrocardiogram and an interatrial septal mass on CT angiography of the chest. Additional workup with cardiac MRI and transoesophageal echocardiogram were able to delineate the cardiac mass. Electrophysiology and cardiothoracic surgery were consulted. The mass was excised in the same hospitalisation and the pathology report demonstrated a bronchogenic cyst. After mass excision, the patient chest pain has decreased, and she reverted back to normal sinus rhythm. On further follow-up, her flecainide and metoprolol were stopped.

Giant Intrapericardial bronchogenic cyst associated with congestive heart failure and atrial fibrillation: a case report.

BACKGROUND: Large intracardiac bronchogenic cysts are rare mediastinal masses. However, they must always be considered in the differential diagnosis of heart failure with abnormal chest X-ray. CASE PRESENTATION: We present a 60-year-old female patient with de novo atrial fibrillation, heart failure and a very large intrapericardial mass. The patient underwent successful surgical resection, with pathological findings confirming a bronchogenic cyst. CONCLUSIONS: Large bronchogenic cysts located intrapericardially are very rare. However, they should be included in the differential diagnosis of patients presenting with atrial fibrillation and heart failure with abnormal radiologic studies.

Bronchogenic cyst presenting with respiratory distress in a neonate.

Bronchogenic cysts are considered rare airway anomalies that can have a significant impact on the well-being of infants. In this case report, we present a rare presentation of bronchogenic cyst presenting with early neonatal respiratory distress due to airway and vascular compression. Surgical excision was curative with an excellent prognosis.

A Bronchogenic Cyst Masquerading as a Tongue Mass.

Bronchogenic cysts are foregut-derived developmental anomalies found along the developmental pathway of the foregut. The putative theory of pathogenesis is abnormal budding or branching of epithelial cells during the development of tracheobronchial tree. Over 99 % of cases occur in the mediastinum and lung while the head and neck area is affected in less than 1 % of cases with only rare cases reported in the oral cavity. This is a report of a case of a bronchogenic cyst arising in a 6-year-old male. The lesion presented as a painless swelling of the left underside of the tongue. Microscopically, the cyst was lined by pseudostratified columnar epithelium exhibiting many ciliated and mucous cells. A focus of cartilage and discontinuous bundles of smooth muscle were present adjacent to the lining. Where there was cyst rupture, there was granulation tissue associated with many foamy macrophages and acute and chronic inflammation. Four other cases, three in the tongue and one in the lower lip vestibule with cutaneous extension, all in the midline, have been reported in a 1 day-old male, 4 year-old male, 6 year-old female and 3 year-old male. There was no recurrence after excision and this is in keeping with the behavior in previous reports. Other developmental cysts including foregut cysts may be focally lined with respiratory epithelium but the presence of cartilage is the sine qua non for the diagnosis of a bronchogenic cyst.

Resection of Bronchogenic Cysts in Symptomatic Versus Asymptomatic Patients: An Outcome Analysis.

BACKGROUND: The natural evolution of bronchogenic cysts (BCs) is unpredictable. Although most surgeons agree that symptomatic BCs should be resected, questions remain regarding the optimal management of asymptomatic mediastinal cysts. We present a case series of BCs to compare patients who underwent preventive operation with those who underwent surgical procedure after symptom onset. METHODS: This 15-year multicenter retrospective study included 114 patients (32 children and 82 adults). Data on clinical history, pathology, mean hospital stay, intraoperative and postoperative complications, and associated intraoperative procedures were analyzed separately for symptomatic and asymptomatic patients. RESULTS: A total of 53 asymptomatic patients (46.5%) were compared with 61 symptomatic patients (53.5%). There were significantly more adults in the symptomatic group than in the asymptomatic group (48 vs 34 patients, P < .05). A thoracoscopic approach was used in 88 patients (77%), with 7 conversions to thoracotomy (9%), all in symptomatic patients. There were significantly more additional procedures (20% vs 4%, P = .01) and more intraoperative complications (20% vs 4%, P = .01) in symptomatic patients, but postoperative complications between symptomatic and asymptomatic patients were similar. The postoperative length of stay was significantly longer in symptomatic patients (5.71 days vs 4 days, P < .001). Pathologic examination found significantly more inflammatory reactions in symptomatic patients. CONCLUSION: Early surgical management of BCs may be recommended to prevent symptomatic complications, which are unpredictable and whose management is more complicated in advanced BCs. Surgery can be performed with a thoracoscopic approach, which is easier and safer when the cyst is small and uncomplicated.

Resection of a Giant Bronchogenic Cyst in the Left Atrium.

A bronchogenic cyst in the left atrium is rare. We report the case of a 17-year-old male patient who was admitted to the emergency department because of severe chest pain and dyspnea. He was diagnosed using echocardiography and computed tomography, which revealed a huge cardiac tumour in the dome of the left atrium. He was surgically treated with tumour enucleation. The resultant atrial dome defect was reconstructed with a bovine pericardial patch. Pathologic investigation revealed that the tumour was a bronchogenic benign cyst.