Congenital Pulmonary Cyst Research Articles

Defective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts.

Abnormal lung development can cause congenital pulmonary cysts, the mechanisms of which remain largely unknown. Although the cystic lesions are believed to result directly from disrupted airway epithelial cell growth, the extent to which developmental defects in lung mesenchymal cells contribute to abnormal airway epithelial cell growth and subsequent cystic lesions has not been thoroughly examined. In the present study using genetic mouse models, we dissected the roles of bone morphogenetic protein (BMP) receptor 1a (Bmpr1a)-mediated BMP signaling in lung mesenchyme during prenatal lung development and discovered that abrogation of mesenchymal Bmpr1a disrupted normal lung branching morphogenesis, leading to the formation of prenatal pulmonary cystic lesions. Severe deficiency of airway smooth muscle cells and subepithelial elastin fibers were found in the cystic airways of the mesenchymal Bmpr1a knockout lungs. In addition, ectopic mesenchymal expression of BMP ligands and airway epithelial perturbation of the Sox2-Sox9 proximal-distal axis were detected in the mesenchymal Bmpr1a knockout lungs. However, deletion of Smad1/5, two major BMP signaling downstream effectors, from the lung mesenchyme did not phenocopy the cystic abnormalities observed in the mesenchymal Bmpr1a knockout lungs, suggesting that a Smad-independent mechanism contributes to prenatal pulmonary cystic lesions. These findings reveal for the first time the role of mesenchymal BMP signaling in lung development and a potential pathogenic mechanism underlying congenital pulmonary cysts.

Defective mesenchymal Bmpr1a-mediated BMP signaling causes congenital pulmonary cysts

Abnormal lung development can cause congenital pulmonary cysts, the mechanisms of which remain largely unknown. Although the cystic lesions are believed to result directly from disrupted airway epithelial cell growth, the extent to which developmental defects in lung mesenchymal cells contribute to abnormal airway epithelial cell growth and subsequent cystic lesions has not been thoroughly examined. In the present study using genetic mouse models, we dissected the roles of bone morphogenetic protein (BMP) receptor 1a (Bmpr1a)-mediated BMP signaling in lung mesenchyme during prenatal lung development and discovered that abrogation of mesenchymal Bmpr1a disrupted normal lung branching morphogenesis, leading to the formation of prenatal pulmonary cystic lesions. Severe deficiency of airway smooth muscle cells and subepithelial elastin fibers were found in the cystic airways of the mesenchymal Bmpr1a knockout lungs. In addition, ectopic mesenchymal expression of BMP ligands and airway epithelial perturbation of the Sox2-Sox9 proximal-distal axis were detected in the mesenchymal Bmpr1a knockout lungs. However, deletion of Smad1/5, two major BMP signaling downstream effectors, from the lung mesenchyme did not phenocopy the cystic abnormalities observed in the mesenchymal Bmpr1a knockout lungs, suggesting that a Smad-independent mechanism contributes to prenatal pulmonary cystic lesions. These findings reveal for the first time the role of mesenchymal BMP signaling in lung development and a potential pathogenic mechanism underlying congenital pulmonary cysts.

Neutrophil extracellular traps are not produced in pediatric patients with one-lung ventilation: a prospective, single-center, observational study.

BackgroundOne-lung ventilation (OLV) may cause lung injury and induce pulmonary pro-inflammation; this ventilator-induced lung injury is associated with neutrophil infiltration. The infiltrated neutrophils release neutrophil extracellular traps (NETs), which are associated with tissue damage. It is not known whether NETs are involved in the pathogenesis of one-lung injury and if they could be a potential therapeutic target. In the present study, we quantified NETs in bronchoalveolar lavage fluid from pediatric patients who underwent OLV and assessed their relationship with prognosis.MethodsEighteen patients with congenital pulmonary cysts or pulmonary sequestration were enrolled in this prospective monocentric study. Myeloperoxidase (MPO) levels, NET markers [i.e., citrullinated histone-3 (CH-3) and free double-stranded DNA (dsDNA)], and inflammatory cytokine levels in bronchoalveolar lavage fluid were assessed. Continuous variables were compared using the paired t-test. The association of NET concentration in bronchoalveolar lavage fluid and clinical parameters was assessed using linear regression analyses.ResultsdsDNA concentration in bronchoalveolar lavage fluid was higher after OLV than before OLV in both the affected lung (0.23±0.30 vs. 0.97±1.05, P<0.05) and the healthy lung (0.28±0.19 vs. 2.45±2.23, P<0.05). However, there were no significant differences in concentrations of MPO, CH-3, and inflammatory cytokines before and after OLV. Serum interleukin (IL)-6 concentration was higher after OLV than before (t=–3.222, P=0.007). Moreover, no associations between dsDNA concentration in bronchoalveolar lavage fluid and the duration of postoperative mechanical ventilation, postoperative hospital stay, and chest high-resolution computed tomography score were observed. The durations of OLV, anesthesia, and operation, as well as the amount of blood loss, had no significant influence on postoperative dsDNA concentration in bronchoalveolar lavage fluid.ConclusionsNETs in bronchoalveolar lavage fluid are not involved in patients who undergo OLV.

Quiste pulmonar congénito como causa de neumonías recurrentes. A propósito de un caso

Introducción: Un quiste pulmonar se define como un espacio de parénquima pulmonar que contiene aire o líquido; se presentan asintomáticos o con infecciones respiratorias recurrentes. Se desarrolla por la separación de un segmento de uno de los bronquios principales, como resultado de una gemación anómala del intestino primitivo, con predilección por los lóbulos pulmonares inferiores. Tiene una incidencia mundial baja y pocos casos reportados por la literatura. &#x0D; Objetivo: Describir un caso clínico de Quiste pulmonar congénito como causa de neumonías recurrentes Material y métodos: Estudio descriptivo retrospectivo, presentación de caso clínico. &#x0D; Resultados: Se presenta el caso de una preescolar de 4 años con antecedente de infecciones respiratorias a repetición que es diagnosticada de quiste pulmonar congénito mediante hallazgos imagenológicos, durante el estudio de la patología respiratoria recurrente, tratada quirúrgicamente con lobectomía, con excelentes resultados.&#x0D; Conclusiones: Las infecciones respiratorias recurrentes es decir al menos 2 episodios en un año son la principal manifestación de los quistes pulmonares congénito en niños preescolares y escolares que requieren una investigación exhaustiva del aparato respiratorio del niño, ya que una de las principales causas son las malformaciones pulmonares, como es el quiste pulmonar

Congenital lung cysts in a newborn. About a case

Congenital pulmonary malformations, especially congenital pulmonary cysts, are a heterogeneous group of alterations in lung development that can occur at different stages of embryogenesis, affecting the parenchyma, the arterial supply, the venous drainage or being a combination of them. A clinical case is presented where it analyzes the congenital pulmonary malformations in a newborn patient at the term of adequate weight for the gestational age, in the same as when entering the Neonatology service. To the radiological control in which an image of bubbles of air trapped in the left base is visualized, initial suspicion of diaphragmatic hernia, the same that is discarded at 24 hours, changing to a diagnosis of congenital pulmonary malformations. Congenital lung cysts.

Imaging of congenital lung malformations

Congenital lung malformations are a heterogeneous group of anomalies that involve the lungs and tracheobronchial tree (congenital airway pulmonary malformation, bronchial atresia, bronchogenic cyst, congenital lobar overinflation, pulmonary cyst, hamartoma, pulmonary isomerism and azygous lobe), vascular abnormalities (arteriovenous malformations, anomalous pulmonary venous return, pulmonary artery sling, interrupted pulmonary artery, pulmonary varix, pulmonary vein stenosis and pulmonary lymphangiectasia), or frequently both entities (pulmonary sequestration, pulmonary maldevelopment and scimitar syndrome). Advances in diagnostic imaging (including sonography, multi-detector computer tomography, magnetic resonance imaging and angiography) have increased their detection during both antenatal and postnatal periods, and radiological characterisation, which in turn influence patient counselling and management stratification. An educational illustration of the clinical application in characterisation of these malformations is presented.

Experience in minimally invasive Nuss operation for 406 children with pectus excavatum

This study was to investigate the advantages of thoracoscopy-assisted minimally invasive Nuss operation for the treatment of pectus excavatum (PE) in children. A total of 406 patients with PE (female: 93; male: 313) with an average age of 6.8 years (range: 3.5-17.5 years) were included in this study. Associated diseases included congenital heart disease in 9 patients and congenital pulmonary cyst in 2. The Haller index of the patients ranged from 3.35 to 7.23, with an average of 5.17±1.64. Minimally invasive Nuss operation was performed for all the patients. The operations were performed successfully and no operative mortality occurred. The average blood loss during the operation was less than 10 mL and the operating time ranged from 30 to 85 minutes with an average of 45 minutes. The length of hospital stay ranged from 5 to 9 days with an average of 7 days. Struts were implanted in 12 (3.0%) of the 406 patients. Injury of the pericardium occurred in 1 patient during the operation. Early post-operative complications occurred in 9 patients with pneumothorax and 6 patients with pleural effusion, which were cured by puncture or drainage. Poor wound healing occurred in 4 patients (1.0%) and was cured by nutritional support. During a 3-month to 6-year follow-up, 2 patients had scoliosis and 3 patients had displacement of the strut, which was cured by a second Nuss operation. Allergy occurred in 2 patients: the symptoms were improved in 1 patient after conservative treatment, but the strut was removed in advance due to allergy in the other patient. Totally 154 patients (40.0%) underwent operation for strut removal. Excellent repair results were achieved in 387 (95.3%) patients, good repair results in 12 (3.0%), and fair results in 7 (1.7%). Thoracoscopy-assisted Nuss operation has many advantages including small and masked incision, short operative time, minimal blood loss, fast recovery, less trauma, and satisfactory outcomes of repair. Nuss is a safe and reliable technique for repair of PE.

Beware of ?degenerating? congenital pulmonary cysts

Beware of ?degenerating? congenital pulmonary cysts

Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective?

Pleuropulmonary blastomas (PPB) are rare intrathoracic tumors that can develop in children with preexisting pulmonary cystic lesions, inferring that the prophylactic resection of such cysts might prevent the onset of these malignant tumors. We report a patient who went on to develop PPB in the right lung despite having had resections for bilateral congenital pulmonary cysts 23 months earlier. We therefore question the effectiveness of prophylactic resection of congenital lung cysts on this basis.

Respiratory Compromise and Dramatic Chest X-ray Changes during General Anesthesia in a Patient with a Bronchogenic Cyst

Respiratory Compromise and Dramatic Chest X-ray Changes during General Anesthesia in a Patient with a Bronchogenic Cyst

Primary intrathoracic rhabdomyosarcoma: a rare childhood malignancy

Primary intrathoracic rhabdomyosarcoma is a rare tumour in childhood. Three cases are presented and the radiological findings and clinical course are reviewed. The radiological manifestations are varied but a rapidly growing soft tissue mass with compression of adjacent structures is the most common. A rare association with an underlying congenital pulmonary cyst is described. The prognosis is worse than for rhabdomyosarcoma at other sites, with a predisposition for cerebral metastases.

Rhabdomyosarcoma arising within congenital pulmonary cysts: Report of three cases

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Traumatic Pulmonary Pseudocysts and Paramediastinal Air Cyst

Traumatic pulmonary pseudocysts and paramediastinal air cysts due to closed chest trauma are very uncommon. Minor clinical symptoms and major radiologic signs are characteristic of this form of injury to the lung parenchyma. Treatment of combined pseudocysts and pneumatocele in a 17-year-old girl is described. Chest X-ray and computed tomography provided the diagnosis. No surgical treatment was required. Intensive bronchial toilet and PEEP-assisted respiration by mask were followed by recovery. This condition must be differentiated from lung abscesses after infected hematomas, specific cavities, or congenital pulmonary cysts.

Mesenchymal neoplasia and congenital pulmonary cysts

A malignant mesenchymoma exhibiting a varied spectrum of differentiation developed within a congenital pulmonary cyst 6 1/2 years after the cyst was first recognized. Related tumors with a similar gross appearance have been previously described and have included rhabdomyosarcomas and so-called pulmonary blastomas. There is a low but distinct risk for the development of mesenchymal sarcomas within congenital peripheral pulmonary cysts.

Congenital pulmonary cysts versus a differential diagnosis in the newborn: Diaphragmatic hernia

Congenital pulmonary cysts versus a differential diagnosis in the newborn: Diaphragmatic hernia

Congenital Cystic Adenomatoid Malformation of the Lung

BARTHOLINUS first described congenital cystic disease of the lung in 1687,1 but it has become apparent in recent years that acquired cysts of the lung far outnumber the entire group of cyst of congenital origin.2,3 In their classification of congenital pulmonary anomalies, the American College of Chest Physicians4include eight types of cystic lesions, one of the rarest of which is congenital cystic adenomatoid malformation (CCAM). Cystic Lesions of the Lung4 A. Cystic Malformation 1. Solitary cysts 2. Multiple cysts 3. Cystic sequestration 4. Cystic adenomatoid malformation 5. Diffuse cystic lymphangiectasis B. Congenital Pulmonary Cyst 1. Solitary a. Asymptomatic b. Tension and communicating 2. Multiple 3. Cystic bronchiectasis In 1949, Ch'in and Tang5 reviewed ten cases from the German literature and added one case of their own in the first report in the English language. Although Fischer et all successfully resected this malformation as early as 1943, there has been

Congenital pulmonary cysts : F. Bergami, G. Fabbrini, and C. Romualdi. Riv. Chir. Ped. 10:333–340 (July–Sept.) 1968

Congenital pulmonary cysts : F. Bergami, G. Fabbrini, and C. Romualdi. Riv. Chir. Ped. 10:333–340 (July–Sept.) 1968

Bilateral congenital pulmonary cyst in an infant : M. Kabelka. Z. K. Chir. 6:15–26, 1968

Bilateral congenital pulmonary cyst in an infant : M. Kabelka. Z. K. Chir. 6:15–26, 1968

Congenital pulmonary cysts in the newborn. A report of a successful lobectomy in a 3-week-old child : M. Kabelka. Čs. Pediat. 22:995–998 (November) 1967

Congenital pulmonary cysts in the newborn. A report of a successful lobectomy in a 3-week-old child : M. Kabelka. Čs. Pediat. 22:995–998 (November) 1967

PULMONARY CYST

There have been various opinions in the classification and nomenclature of the lung cyst. but the recently approved by most of the clinicians and pathologists is as follows; Ⅰ. Congenital Pulmonary Cysts. A. Bronchogcnic cell type 1. Solitary 2. Multiple B. Alveolar cell type 1. Solitary(balloon cyst, pneumatocele) 2. Multiple C. Bronchogenic and alveolar types combinedⅡ. Acquired Pulmonary Cysts. A. Bullous emphysema B. Subpleural blebs.According to the statistical data by Weaver, etc, the incidence of the congenital lung cyst in young adults is 5 out of every 100,000. This writer has experienced 2 cases of congenital lung cyst, one without any clinical symptom and the other with the complaints of hemoptysis, which I am therefore reporting.