The ten-year evaluation of clinical characteristics in congenital lung anomaly in pediatrics; a retrospective study in North of Iran.

INTRODUCTION: Congenital lung anomalies (CLA) are a group of anomalies, including congenital cystic adenomatoid malformation (CCAM), bronchopulmonary sequestrations (BPS), congenital lobar emphysema (CLE), and bronchogenic cysts (BC). The prevalence of these rare anomalies has risen in recent years, according to various population-based studies due to advances in fetal ultrasound technology. METHOD: This retrospective study examines the diagnosis of CLA, and was conducted on 72 patients between March 2014 and March 2024 at Taleghani Pediatric Hospital in Gorgan, Iran. RESULT: The average age was 18.8 ± 30.3 months, with the majority being boys (62.5%). Most participants had CCAM (41.7%), followed by CLE (18.1%), BPS (16.7%), pulmonary hypoplasia (9.7%), BC (8.3%), and hybrid lesion (5.6%). The majority of patients were Fars (62.5%), and the average hospitalization days was 9.4 ± 4.5 days. Cardiac anomalies were observed in 19.4% of the patients. 62 patients (86.1%) exhibited respiratory symptoms, and prenatal screening during pregnancy led to the diagnosis in 51 patients (70.8%). Most patients had left lung anomalies (43; 59.7%), and the majority (90.3%) survived. There is a statistically significant relation between needed for surgical treatment and patients' type of pulmonary lesions (p-value: 0.02). In addition, there was a significant relation between the Fars ethnicity and the presence of cardiac anomalies (p-value: 0.04). CONCLUSION: Some CLAs remain undiagnosed or untreated due to the rare nature of congenital lung anomalies. Nevertheless, improvements in ultrasound and other imaging methods will make diagnosing and managing these anomalies during the prenatal period more prevalent, resulting in enhanced understanding.

Pulmonary sequestration in adulthood: clinical-morphological study.

OBJECTIVES: The aim of the study was to evaluate pulmonary sequestration (PS). We report on location, blood supply, histology, clinical manifestation, and surgical treatment of PS, as well as on postoperative course in patients with PS. BACKGROUND: PS is a rare congenital defect of the lower respiratory tract, it represents locus minoris resistentiae of the body. Occasionally, PS is diagnosed for the first time in adulthood. METHODS: We evaluated 7 cases of PS treated at the Centre of Thoracic Surgery in Vysné Hágy, Slovakia, between years 2013 and 2020. RESULTS: Four of our seven patients were asymptomatic; the PS was found incidentally upon chest imaging. Three patients had recurrent bronchopneumonia related specifically to the intralobar type of sequestration. The most significant complication, observed in a singular patient, was a life-threatening episode of haemoptysis, requiring urgent surgical intervention. In the other 6 cases, the sequestra were surgically resected during the period when they were asymptomatic. and their sputum was confirmed negative upon microbiological examination. Anatomical resection of the affected pulmonary lobe by thoracotomy was the most common type of operation performed (4 cases, n = 7). There was no surgical mortality. CONCLUSION: To prevent complications, it is crucial to perform surgical treatment for pulmonary sequestration in patients who have sufficient functional capacity (Tab. 2, Fig. 4, Ref. 30). Text in PDF www.elis.sk Keywords: pulmonary sequestration, anatomic lobectomy, haemoptysis.

[The Successful Reduction of Resected Lung Volume for Intralobar Pulmonary Sequestration with Infrared Thoracoscope].

A 59-year-old male patient was referred to our hospital for further examinations and treatment due to an abnormal shadow detected in his left lower lung lobe on computed tomography. The patient was diagnosed with intralobar pulmonary sequestration and scheduled for an operation. During the surgery, after resection of the aberrant artery, indocyanine green was intravenously injected, and the border between normal lung and sequestrated lung was clearly identified by an infrared thoracoscope. Subsequently, wedge resection was performed, and the patient was discharged on postoperative day 5. Spirometry performed 6 months after the surgery indicated that the patient's lung function was well-preserved compared to the preoperative status.

[Right upper lobe pulmonary sequestration as a rare cause of recurrent spontaneous pneumothorax]. / Sekvestratsiya verkhnei doli pravogo legkogo ­ redkaya prichina pravostoronnego retsidiviruyushchego spontannogo pnevmotoraksa.

A 19-year-old patient after previous wedge resection of the right upper pulmonary lobe a year ago urgently admitted with recurrent right-sided spontaneous pneumothorax. According to standard management of spontaneous pneumothorax, we performed diagnostic thoracoscopy and drainage of the right pleural cavity with regular X-ray examinations. However, these measures were ineffective. The patient was scheduled for surgery, and we intraoperatively observed an unusual cause of pneumothorax. Thus, we present spontaneous pneumothorax following right upper lobe pulmonary sequestration. The uniqueness of this case is associated with unusual manifestation and non-standard localization of rare lesion. A few cases of pneumothorax in similar patients are described in the world literature. The key limiting factor in diagnosis of such defects (identification of aberrant vessel supplying abnormal lung parenchyma) is the lack of routine CT angiography in patients diagnosed with pneumothorax. That is why CT changes were interpreted as postoperative ones, and the true cause was established only during redo surgery. A thorough inspection of the pleural cavity and alertness regarding unusual appearance of the right upper pulmonary lobe made it possible to suggest a non-standard diagnosis, avoid complications (bleeding from afferent vessel) and perform adequate lung resection.

Pulmonary sequestration in adult patients: a single-center retrospective study.

BACKGROUND: Pulmonary sequestration (PS) is a rare congenital lower airway malformation. This study presents the clinical and imaging features and surgical outcomes of PS in adults, and compare the safety and feasibility of minimally invasive surgery versus open thoracotomy for PS. METHODS: Adult patients with PS treated at our center from July 2011 to September 2021 were included. Information regarding the patient demographics, clinical and CT features, arterial supply and venous drainage, and surgical outcomes were collected. RESULTS: Ninety seven patients were included. The most common CT findings were mass lesions (50.5%) and cystic lesions (20.6%). The vast majority of the lesions (96 out of 97) were located close to the spine in the lower lobes (left vs. right: 3.6 vs. 1). Arterial supply was mainly provided by the thoracic aorta (87.4%) and abdominal aorta (10.5%). Intralobar and extralobar PS accounted for 90.7% and 9.3% of the patients, respectively. Three (4.5%) patients who underwent minimally invasive surgery were converted to open thoracotomy due to dense adhesions. Though no significant differences regarding operative time (P = 0.133), the minimally invasive surgery group was significantly better than the open thoracotomy group regarding intraoperative blood loss (P = 0.001), drainage volume (P = 0.004), postoperative hospital days (P = 0.017) and duration of chest drainage (P = 0.001). There were no cases of perioperative mortality. Only four (4.1%) patients developed postoperative complications, and no significant difference existed between the two groups. CONCLUSION: Our study revealed PS can present with a variety of different clinical and radiologic manifestations. Clinicians should consider the possibility of PS when diagnosing a lesion in the lower lobes close to the spine. Moreover, minimally invasive surgery is a safe and effective treatment modality for the treatment of PS in an experienced center.

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.

Congenital cystic pulmonary lesions (CCPLs) are represented by the following entities: congenital pulmonary airway malformation (CPAM), formerly congenital cystic adenomatoid malformation, extra- and intralobar sequestration (EIS), congenital lobar emphysema (overexpansion), and bronchogenic cyst. The developmental model of CPAM histogenesis by Stocker proposed perturbations designated as CPAM type 0 to type 4 without known or specific pathogenetic mechanisms along the airway from the bronchus to the alveolus. This review highlights mutational events either at the somatic level in KRAS (CPAM types 1 and possibly 3) or germline variants in congenital acinar dysplasia, formerly CPAM type 0, and pleuropulmonary blastoma (PPB), type I, formerly CPAM type 4. The potential for overt malignant progression exists in the case of PPB type I and CPAM type 1 in some cases to well-differentiated mucinous adenocarcinoma. On the other hand, CPAM type 2 is an acquired lesion resulting from interruption in lung development secondary to bronchial atresia. The latter is also regarded as the etiology of EIS whose pathologic features are similar, if not identical, to CPAM type 2. These observations have provided important insights into the pathogenetic mechanisms in the development of the CPAMs since the Stocker classification.

The upper airway parameters: the potential diagnostic clues for congenital intrathoracic lesions.

BACKGROUND: The diagnosis of congenital intrathoracic lesions still has limitations. The airway development was influenced by intrathoracic factors. Whether the diagnostic value of the upper airway parameters in congenital intrathoracic lesions has not been confirmed. OBJECTIVES: We aimed to compare fetal upper airway parameters between normal fetuses and fetuses with intrathoracic lesions, and we tried to verify its diagnostic value in intrathoracic lesions. METHODS: This was an observational case-control study. In the control group, 77 women were screened at 20-24 weeks' gestational age, 23 were screened at 24-28 weeks' gestational age, and 27 were screened at 28-34 weeks' gestational age. In the case group, 41 cases were enrolled (6 cases of intrathoracic bronchopulmonary sequestration, 22 of congenital pulmonary airway malformations, and 13 of congenital diaphragmatic hernia). Fetal upper airway parameters (tracheal width, the narrowest lumen width, and width of the subglottic cavity and laryngeal vestibule) were measured using ultrasound equipment. The correlations between fetal upper airway parameters and gestational age, and the differences in fetal upper airway parameters between cases and controls, were analyzed. The standardized airway paraments were acquired, and their potential diagnostic value for congenital intrathoracic lesions were analyzed. RESULTS: The fetal upper airway parameters of both groups were positively correlated with the gestational age: The control group, tracheal width (R2 = 0.569, p < 0.001), narrowest lumen width (R2 = 0.429, p < 0.001), subglottic cavity width (R2 = 0.551, p < 0.001), laryngeal vestibule width (R2 = 0.349, p < 0.001). The case group (tracheal width R2 = 0.474, p < 0.001) narrowest lumen width (R2 = 0.425, p < 0.001), subglottic cavity width (R2 = 0.623, p < 0.001), laryngeal vestibule width (R2 = 0.347, p < 0.001). Fetal upper airway parameters of the cases group were smaller than those of the controls group. The tracheal width in fetuses with congenital diaphragmatic hernia was the smallest among the other case groups studied. The standardized tracheal width has the best diagnostic value for congenital intrathoracic lesions in the standardized airway paraments (the area under the ROC curve was 0.894), and has a high diagnostic value for congenital pulmonary airway malformations and congenital diaphragmatic hernia (the area under the ROC curve was 0.911 and 0.992, respectively). CONCLUSION: Fetal upper airway parameters differ between normal fetuses and fetuses with intrathoracic lesions, and might offer potential diagnostic clues for congenital intrathoracic lesions.

Epidemiology and prevalence of pulmonary sequestration in Chinese population, 2010-2019.

BACKGROUND: Pulmonary sequestration (PS) is the second common congenital lung malformation and has been known for over 150 years. However, there is a scarcity of epidemiological studies on it. This study aimed to characterize the epidemiology of pulmonary sequestration in Chinese population in the recent decade by using a nationwide database. METHODS: Using data from the Chinese Birth Defects Monitoring Network during 2010-2019, the prevalence rates for PS were calculated by birth year, maternal age, residence area, geographical region, and infant sex. Variations in prevalence and changes over time were further examined. Other variables of interest for analysis included the pregnancy outcomes of affected infants, the prenatal diagnosis, and the co-occurring anomalies of PS cases. RESULTS: During the study period, we identified an average prevalence rate of 0.31, 0.11, and 0.42 per 10,000 live and still births for the isolated, non-isolated, and overall PS, respectively. An upward trend was observed for each category of PS. The prevalence rates varied significantly by maternal age (< 20 years, 0.34/10,000; 20-24 years, 0.33/10,000; 25-29 years, 0.45/10,000; 30-34 years, 0.46/10,000; ≥ 35 years, 0.36/10,000), residence area (urban vs. rural, 0.51/10,000 vs. 0.30/10,000), geographical region (western, 0.33/10,000; eastern, 0.49/10,000; central, 0.43/10,000), and by infant sex (male vs. female, 0.45/10,000 vs. 0.38/10,000). Non-isolated PS cases were more likely born prematurely than isolated cases (15.29% vs. 7.83%). 40.28% and 33.80% of non-isolated cases were accompanied by additional respiratory, and circulatory system malformations, respectively. CONCLUSIONS: The study presents for the first time the prevalence of pulmonary sequestration in Chinese population. The rising prevalence and relatively poor perinatal outcome of affected fetuses or newborns indicate the necessity to improve perinatal management of PS.

Extralobar Pulmonary Sequestration in Adrenal Mimicking Neuroblastoma: A Case Report.

Background: Extralobar pulmonary sequestration (ELS) is a malformation composed of bronchopulmonary tissue outside the lungs that is discontinuous from the main tracheobronchial tree. ELS can present as a suprarenal space occupying lesion. Case Report: A 1-day old girl presented with a right supra-adrenal mass, first detected in-utero at 34 weeks. The differential included congenital neuroblastoma, but the urinary VMA was normal. At resection, there was lung tissue composed of dilated and tortuous bronchioles lined by columnar epithelium present in a back- to- back arrangement along with thick-walled vessels, features of an ELS with congenital pulmonary airway malformation type II. Conclusion: ELS enters the differential diagnosis of neonatal suprarenal masses with normal urinary catecholamines, and can have the morphology of a type II congenital pulmonary airway malformation.

[Intralobar Sequestration Associated with Aneurysm of the Aberrant Artery and Aspergillus Infection: Report of a Case].

A 32-year-old man was referred to our department for surgery because of recurrent pneumonia. Aspergillus fumigatus was detected in his sputum culture at the first visit. We started antifungal therapy one month before surgery. His chest radiograph showed an infiltrative shadow in the right lower lobe, and chest computed tomography (CT) showed an infiltrative shadow and large and small cystic changes in the right lower lobe. CT angiography (CTA) revealed two anomalous arteries supplied by the aorta that drained into the right lower lobe. An aneurysm with a diameter of 25 mm had formed in one anomalous artery. Based on these findings, intralobar pulmonary sequestration with Aspergillus infection and an anomalous artery forming an aneurysm was diagnosed. In addition, we embolized the aneurysm of the anomalous artery. After embolization, right lower lobectomy was safely performed. The patient was discharged on the 15th post-operative day with no complications.

[Scimitar syndrome with pulmonary sequestration containing carcinoma: a case report].

We reported the manifestations, auxiliary examination, and treatment courses of a case of scimitar syndrome with pulmonary sequestration containing carcinoma. The clinical characteristics of scimitar syndrome with pulmonary sequestration, pulmonary sequestration containing carcinoma were summarized based on the data of this case and the related literatures before January 2022. Scimitar syndrome can coexist with ipsilateral pulmonary sequestration. Because sequestered lung tissue has a risk of malignant transformation, a cancer screening test is useful for early diagnosis and timely treatment.

Intrapulmonary sequestration with destructive pneumonia and life-threatening hemoptysis in an adult patient: a case report.

The article reports the case of a patient with bronchopulmonary sequestration complicated by destructive actinomycotic inflammation leading to life-threatening hemoptysis. It was an adult patient with the history of repeated right-sided pneumonia the cause of which had not been investigated in detail in the past. Only hemoptysis, which appeared as a complication, led to a closer investigation of the background of repeated right-sided pneumonia. CT scan of the chest revealed a lesion of the middle lobe of the right lung with anomalous vascularization - compatible with intralobar sequestration. Initially, conservative antibiotic treatment of pneumonia was provided at a local clinic. Embolization of the afferent vessels of the sequestrum was indicated due to persistent hemoptysis; this led to a reduction of its blood supply, proven by a follow-up CT examination of the chest. Clinically, the hemoptysis subsided. Three weeks later, the hemoptysis reocurred. The patient was acutely hospitalized at a specialized thoracic surgery department where shortly after admission, hemoptysis progressed to life-threatening hemoptea. Urgent middle lobectomy of the right lung was approached via thoracotomy to treat the source of bleeding. The case describes unrecognized bronchopulmonary sequestration as a possible cause of recurrent ipsilateral pneumonia in adulthood; additionally, it emphasizes the possible risks associated with a pathologically altered tissue microenvironment of pulmonary sequestration, and the need for surgical removal in all indicated cases.

Succinate Activation of SUCNR1 Predisposes Severely Injured Patients to Neutrophil-mediated ARDS.

OBJECTIVES: Identify the metabolites that are increased in the plasma of severely injured patients that developed ARDS versus severely injured patients that did not, and assay if these increased metabolites prime pulmonary sequestration of neutrophils (PMNs) and induce pulmonary sequestration in an animal model of ARDS. We hypothesize that metabolic derangement due to advanced shock in critically injured patients leads to the PMNs, which serves as the first event in the ARDS. Summary of Background Data: Intracellular metabolites accumulate in the plasma of severely injured patients. METHODS: Untargeted metabolomics profiling of 67 critically injured patients was completed to establish a metabolic signature associated with ARDS development. Metabolites that significantly increased were assayed for PMN priming activity in vitro. The metabolites that primed PMNs were tested in a 2-event animal model of ARDS to identify a molecular link between circulating metabolites and clinical risk for ARDS. RESULTS: After controlling for confounders, 4 metabolites significantly increased: creatine, dehydroascorbate, fumarate, and succinate in trauma patients who developed ARDS ( P < 0.05). Succinate alone primed the PMN oxidase in vitro at physiologically relevant levels. Intravenous succinate-induced PMN sequestration in the lung, a first event, and followed by intravenous lipopolysaccharide, a second event, resulted in ARDS in vivo requiring PMNs. SUCNR1 inhibition abrogated PMN priming, PMN sequestration, and ARDS. Conclusion: Significant increases in plasma succinate post-injury may serve as the first event in ARDS. Targeted inhibition of the SUCNR1 may decrease ARDS development from other disease states to prevent ARDS globally.

Intralobular pulmonary sequestration in the middle lobe supplied by a right internal mammary artery: a case report.

BACKGROUND: Pulmonary sequestration (PS) is a rare congenital malformation that is more common in the left lower lobe, and the thoracic aorta is the most common arterial supply. CASE PRESENTATION: We describe a case of a 67-year-old man with a chief complaint of intermittent cough and hemoptysis who had been diagnosed by multidetector computed tomography angiography with right middle lobe intralobular pulmonary sequestration supplied by a right internal mammary artery. Finally, he underwent middle pulmonary lobectomy with normal postoperative recovery. DISCUSSION: This is a rare intralobular pulmonary sequestration case for a feeding artery from the right internal mammary. Multidetector computed tomography angiography should be performed for diagnosis and preoperative evaluation once pulmonary sequestration is suspected.

Fetal imaging of congenital lung lesions with postnatal correlation.

Congenital lung lesions are a rare group of developmental pulmonary abnormalities that are often first identified prenatally on routine second-trimester US. Congenital pulmonary airway malformation (CPAM) is the most common anomaly while others include bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and bronchial atresia. Clinical presentation is highly variable, ranging from apparent in utero resolution to severe mass effect with resultant hydrops fetalis and fetal demise. Differentiation among these lesions can be challenging because overlapping imaging features are often present. The roles of the radiologist are to identify key imaging findings that help in diagnosing congenital lung lesions and to recognize any ominous features that might require prenatal or perinatal intervention. High-resolution US and complementary rapid-acquisition fetal MRI provide valuable information necessary for lesion characterization. Postnatal US and CT angiography are helpful for lesion evaluation and for possible surgical planning. This article reviews the embryology of the lungs, the normal prenatal imaging appearance of the thorax and its contents, and the prenatal and neonatal imaging characteristics, prognosis and management of various congenital lung lesions.

Debating the embolization of a large aberrant systemic artery for pulmonary sequestration using an Amplatzer duct occluder: a case report and literature review.

Here, we report two rare cases of pulmonary sequestration that were fed by large systemic arteries and embolized with a large Amplatzer duct occluder and their 3-year follow-up, and we discuss the efficacy and safety of the embolization of a large aberrant systemic artery to pulmonary sequestration using an Amplatzer duct occluder. A 9-year-old boy complained of chest pain for 1 month, and a 6-year-old boy initially complained of recurrent cough for 3 months. A series of examinations was launched to evaluate any possible malformation or abnormalities in the patients. Chest CT and CTA identified a right lower pulmonary sequestration with infection. After admission, transcatheter device occlusion was planned after essential antibiotic treatment, and postoperative infection prevention and anti-inflammatory treatment were given. In the following 2 years of follow-up, neither of the children had recurrent chest pain, cough or other related symptoms. However, the CT follow-up demonstrated that a residual mass was visible in both patients. The same chest scan section revealed slight reductions in lung lesions from 38.344 cm2 to 37.119 cm2 (3% reduction) and 14.243 cm2 to 13.178 cm2 (7.5% reduction) for each patient. No follow-up data demonstrated the long-term clinical outcomes of the residual lesion. We do not recommend that embolization be performed for large pulmonary sequestration lesions with an aberrant artery larger than 6 mm that is planned to receive a device larger than 10 mm, as their outcomes showed a higher possibility of rebuilding the vascularization network feeding the pulmonary sequestration, indicating a higher risk for long-term complications.

Isolated partial anomalous origin of a branch pulmonary artery from the descending aorta.

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation using the Amplatzer Vascular Plug. We also set up a diagnostic algorithm to differentiate these diseases from anomalous systemic arterial supply to the pulmonary region. It is possible to make the correct diagnosis using the step-by-step diagnostic algorithm and careful interpretation of chest computed tomography angiography.

Comparing open and thoracoscopy approach for the treatment of pulmonary sequestration in children.

BACKGROUND: In this article, we are going to report our experience on thoracoscopy approach for pulmonary sequestration in children and compare it with traditional open approach. METHODS: This retrospective study included 25 patients with pulmonary sequestration (marked as Group 1), who were treated from January 2003 to December 2009 with open approach, and 32 patients treated between January 2010 and December 2019, who underwent thoracoscopy procedure (marked as Group 2). The preoperative data, operative details, and follow-up outcomes were studied and compared between two groups to discuss the advantages of thoracoscopy approach. Statistical comparison was made with unpaired t tests, χ2 tests and Fisher exact probabilities, with P value less than 0.05 considered significant. RESULTS: To bring less-related effect during the comparison, patients with similar preoperative data were selected. Group 2 whose operation time was longer than Group 1 was completed with thoracoscopy approach in all but one patient, who was converted to open surgery. However, the antibiotics consumption, drainage time, and hospital stay after surgery were shorter in Group 2 with less intraoperative blood loss, less postoperative complication and less volume of drainage. All patients were followed-up by CT scan for 3-24 months (mean, 13.8 months) and the recovery and improvement were better in Group 2. CONCLUSION: Compared to open surgery, thoracoscopy surgery has many benefits and was safe, feasible, and effective for the treatment of pulmonary sequestration in child patients.

Successful Treatment of Bronchopulmonary Sequestration by Radiofrequency Ablation of Feeding Artery.

INTRODUCTION: Most cases of bronchopulmonary sequestration (BPS) regress. Prenatal intervention is needed in cases of fetal hydrothorax or hydrops. Laser is commonly used to ablate the feeding artery. CASE PRESENTATION: In a fetus with BPS, radiofrequency ablation (RFA) was used to ablate the feeding artery arising from descending aorta at 29 weeks gestation. There was an extralobar BPS and significant pleural effusion causing mediastinal shift and collapse of lung. The effusion and tumor started decreasing from day 3 after procedure, and by the time patient delivered at 36 weeks gestation, the lesion had almost resolved. CONCLUSION: With proper technique, RFA can be safely used to ablate feeding artery in BPS.

[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.