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Pulmonary sequestrations comprise a spectrum of congenital lung malformations, with abnormal lung tissue lacking connection with the tracheobronchial tree, supplied by an aberrant systemic artery. Until a few years ago, lobectomy was considered the standard treatment for intralobar pulmonary sequestration. However, minimally invasive sublobar resection gained a place as an interesting alternative therapeutic approach, guided by indocyanine green and computed tomography-based 3-dimensional anatomical models. Like pulmonary sequestrations,  pulmonary pseudosequestrations are a congenital lung malformation, but characterized by a normal lung tissue fed by systemic arterial branches. To the best of our knowledge, there are no published cases of pulmonary pseudosequestration combined with sequestration. We present a case of an intralobar pulmonary sequestration coupled with an adjacent pseudosequestration, resected using thoracoscopic surgery with the aid of a 3-dimensional anatomical model and indocyanine green., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Bronchopulmonary sequestration is a rare condition characterised by the presence of nonfunctional, dysplastic lung tissue. This tissue receives blood supply from the systemic circulation. In this case report, we document an exceptional presentation of a woman in her 50s where bronchopulmonary sequestration in the right upper lung lobe led to haemoptysis. This sequestration received blood supply from a pulmonary artery branch, which is unusual and contrary to the usual systemic circulation. The patient underwent a right upper lobectomy, which confirmed the diagnosis of pulmonary sequestration on histopathology. The unique vascular supply and location of this case challenge conventional diagnostic and management approaches. It emphasises the necessity for meticulous evaluation and consideration of alternative vascular sources in bronchopulmonary sequestration cases., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Pulmonary sequestration (PS), a rare pulmonary disease, arises from congenital pulmonary vascular dysplasia. Meanwhile, pulmonary actinomycosis is a purulent infection of lung lesions triggered by the inhalation of actinomycetes, which is also uncommon. Even rarer is the occurrence of pulmonary actinomycete infection secondary to PS. Herein, we present a case report of such a rare occurrence., Patient Concerns: The 21-year-old female patient had been erroneously diagnosed with pneumonia and tuberculosis, presenting symptoms of cough, sputum, and hemoptysis; however, the implemented anti-infection and antituberculosis treatments proved to be ineffective., Diagnoses: The diagnosis of the sequestration in the right lower lung was confirmed through an enhanced chest CT scan and a 3-dimensional reconstruction of the pulmonary vessels., Interventions: During the surgical video-assisted thoracoscopic resection of the right lower lobe lesion, it was discovered that the isolated lung tissue's blood supply vessel originated from the thoracic aorta. Additionally, the pathological examination revealed that the lung tissue of the right lower lobe lesion was infected with pulmonary actinomycetes., Outcomes: Following thorough evaluation, the patient received a final diagnosis of pulmonary actinomycete infection that occurred secondary to right lower lung sequestration. Consequently, they underwent treatment consisting of high-dose penicillin administered for a period of 6 months post-operation. Over the course of the subsequent 23-month follow-up, no recurrence of the infection or abnormal CT scan findings were observed., Lessons: Pulmonary sequestration bears clinical resemblance to pulmonary actinomycetes. In cases where recurrent episodes of pneumonia occur at the same location, and chest imaging indicates persistent lesions in the basal segment of the lower lobe near the spine, the possibility of PS should be considered. Prompt chest-enhanced CT and 3-dimensional reconstruction of pulmonary vessels are crucial for a definitive diagnosis. Imaging findings such as mass-like consolidation, cystic lesions, liquefactive necrosis, and pneumonia-like changes, coupled with typical air suspension signs and sulfur-like particles visible under tracheoscopy, suggest a potential pulmonary actinomycete infection. Timely biopsy is essential to confirm the diagnosis and prevent missed or incorrect diagnoses., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Intra-lobar (ILS) and extra-lobar lung (ELS) sequestrations represent rare congenital lung malformations. Despite their benign nature, the lesions pose risks such as recurrent pulmonary infections, hemoptysis, congestive heart failure, and tumor development. Pulmonary sequestration (PS) typically manifests in two forms, ILS and ELS, with bilateral occurrence being exceptionally rare and mostly requiring bilateral thoracic surgery., Case Presentation: A 9-year-old child, who initially presented with bilateral lung lesions without respiratory symptoms, was diagnosed with PS following a chest computed tomography scan. The surgical approach was determined based on the absence of inflammation and the clear demarcation of the lesions from normal lung tissue, highlighted by a unique tissue connection between the ILS and ELS across the chest cavities. We used a novel method wherein the left ELS was successfully pulled into the right chest cavity and both sequestrations were concurrently resected. Postoperative recovery was smooth, with no complications or residual lesions., Conclusions: Our findings highlight the importance of thorough preoperative planning with enhanced computed tomography. Simultaneous unilateral thoracoscopic surgery can be a viable, less invasive option for treating bilateral PS, offering benefits such as reduced recovery time and better cosmetic outcomes., (© 2024. The Author(s).)

Bronchopulmonary sequestration is a congenital abnormality characterized by non-functioning lung tissue, abnormal connection with the tracheobronchial tree and anomalous systemic arterial supply. Although considered a rare phenomenon presenting early in life, sequestration may also present with recurrent chest infections in late adulthood. Additionally, bronchopulmonary sequestration may rarely be incidentally encountered during thoracic surgery. Several subtypes exist including intralobar, extralobar and hybrid bronchopulmonary sequestration (congenital pulmonary artery malformation). Surgical resection is curative and serves as the treatment of choice for symptomatic patients. Radiological imaging prior to surgery is essential in these patients because the arterial supply may be anatomically complex, and aberrant artery branches are common. Utilization of virtual 3-dimensional remodelling and computed tomography reconstruction imaging can not only establish a diagnosis of bronchopulmonary sequestration but can also optimize preoperative planning. This approach will ultimately prove useful in anticipating surgically challenging steps and avoiding unnecessary intraoperative complications. We present a video tutorial on the role of 3-dimensional reconstruction imaging in bronchopulmonary sequestration and a step-by-step guide for performing a right robotic-assisted surgical resection of an S2 hybrid bronchopulmonary sequestrated segment. This case is followed by a second case of intralobar bronchopulmonary sequestration encountered incidentally during thoracic surgery., (© The Author 2024. Published by MMCTS on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: Extralobar pulmonary sequestration is located outside the lung parenchyma and is covered by a separated pleural sac, which comprises approximately 25% of all pulmonary sequestration., Case Presentation: This article reported one case of an extralobar pulmonary sequestration originated from the mesoesophagus, which was recognized and excised during a lung resection. Histologic examination revealed an ectopic lung tissue with hyperplasia of bronchioles, which was accord with an extralobar pulmonary sequestration., Conclusions: CT angiogram, ultrasound and MRI can be used to clarify the diagnosis and detect the abnormal feeding arteries of extralobar pulmonary sequestration. Carefulness should be taken while dissecting and ligating the potential feeding arteries. Endovascular occlusion might be an alternative option to surgery., (© 2024. The Author(s).)

Introduction: Congenital thoracic disorders represent a spectrum of fetal lung bud development abnormalities, which may affect breathing capacity and quality of life. We aim to evaluate the impact of surgery in the treatment of 4 major congenital conditions., Materials and Methods: We performed a retrospective cohort analysis of patients who underwent surgical treatment in our tertiary center, from 2007 to 2022., Results: Over the 15-year period, we treated 33 patients, with a male predominance of 55%. 22 patients (67%) were asymptomatic. When symptomatic, the recurrence of respiratory infections was the most common clinical presentation (18%). In 13 patients (39%), diagnosis was achieved through fetal ultrasonography. This study encompassed 13 patients with pulmonary sequestration (39%), 11 patients with bronchogenic cysts (33%), 7 patients with congenital pulmonary airway malformation (21%) and 2 patients with congenital lobar emphysema (6%). Considering solely lung malformation conditions, we accounted 22 patients with a median age of 3 [1-67] years-old. Surgery comprised bilobectomy (9%), lobectomy (77%), lobectomy with wedge resection (5%), segmentectomy (5%) and wedge resection (5%). Concerning bronchogenic cysts, we treated 11 patients with a median age of 19 [14-66] years-old. We identified 1 hilar, 1 intrapulmonary and 9 mediastinal lesions, of which 4 were paraesophageal, 4 were subcarinal and 1 was miscellaneous. Overall, surgery was conducted by thoracotomy in 61% of patients, VATS in 33% and RATS in 6%. The median drainage time was 3 [1-40] days and median hospital stay was 4 [1-41] days. There were no cases of mortality. Ensuing, 94% of patients experienced clinical improvement after surgery., Conclusion: Early diagnosis of congenital thoracic malformations increased considerably with the improvement in imaging technology and prenatal screening. Treatment may include expectant conservative treatment. However, in selected cases, surgery may play an important role in symptomatic control and prevention of disease progression.

A 53-year-old asymptomatic woman was admitted to our hospital for evaluation of an area of abnormal intensity in the right lower lobe on cardiovascular magnetic resonance imaging. She denied a history of pneumonia but occasionally expectorated bloody sputum. Contrast-enhanced chest computed tomography (CT) revealed areas of consolidations with multiple cysts within the right lower lobe and an anomalous artery that originated from the descending aorta and entered the right lower lobe. Based on contrast-enhanced CT findings, she was diagnosed with intralobar pulmonary sequestration, and we performed video-assisted thoracoscopic right basal segmentectomy. The anomalous artery was identified in the pulmonary ligament and was ligated using a silk suture at its proximal end, after which the peripheral segment was separated using an automatic suture device. The patient had an uneventful postoperative course, and plain CT at the 6-month postoperative follow-up indicated no evidence of edema of the anomalous artery stump. We recommend early surgical resection even in asymptomatic patients with pulmonary sequestration.

Background: Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the effects steroids have on lung lesion growth in utero., Methods: A 10-year retrospective review of all prenatally diagnosed lung lesions cared for at our fetal care center was performed. We evaluated the effects of prenatal steroids on congenital pulmonary airway malformation (CPAM)-volume-ratio (CVR), distinguishing change in CVR among CPAMs, bronchopulmonary sequestrations (BPS), and bronchial atresias. We also correlated fetal ultrasound and MRI findings with pathology to determine the accuracy of prenatal diagnosis., Results: We evaluated 199 fetuses with a prenatal lung lesion. Fifty-four (27 %) were treated with prenatal steroids with a subsequent 21 % mean reduction in the CVR (2.1 ± 1.4 to 1.1 ± 0.4, p = 0.003). Fetuses with hydrops and mediastinal shift who were treated with steroids rarely had resolution of these radiographic findings. Postnatal pathology was available for 91/199 patients (45.7 %). The most common diagnosis was CPAM (42/91, 46 %), followed by BPS (30/91, 33 %), and bronchial atresia (14/91, 15 %). Fetuses who received steroids and had pathology consistent with CPAM were more likely to have a reduction in their CVR (p = 0.02). Fetal ultrasound correctly diagnosed the type of lung lesion in 75 % of cases and fetal MRI in 81 % of cases., Conclusions: Prenatally diagnosed CPAMs are more likely to respond to maternal steroids than BPS or bronchial atresias. Knowing the diagnosis in utero could aid to steward steroid usage, however, fetal imagining modalities are not perfect in distinguishing subtype., Level of Evidence: III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Foetal thoracic lesions are uncommon, with the incidence of 1 in 15,000 live births. Antenatal monitoring of these lesions is required to prognosticate the parents about the postnatal outcome of the lesions and about the well-being of the baby. Foetal ultrasound and magnetic resonance imaging (MRI) are the modalities to detect these lesions and follow-up during pregnancy and postnatally. Congenital pulmonary adenomatoid malformations (CPAM), congenital diaphragmatic hernia (CDH) and bronchopulmonary sequestrations (BPS) are the commonly detected foetal thoracic lesions. Parameters such as congenital cystic adenomatoid malformation (CCAM) volume ratio (CVR) and lung-to-head ratio (LHR) determine the prognosis of these lesions. With this background, we planned to study the prognosis and outcome of antenatally detected thoracic lesions., Materials and Methods: This was a prospective study carried out for 2 years (January 2017 to December 2018). Pregnant females with foetuses diagnosed to have thoracic lesions on the second- and third-trimester ultrasound and foetal MRI were enrolled for the study. CVR and LHR were noted. Outcomes were analysed in terms of the need of termination of pregnancy, foetal or neonatal demise and need of surgery or conservative management., Results: Of a total of 521 pregnancies, thoracic lesions were detected in 22 (4.22%) cases. Individually, the incidence of each lesion was: CPAM-10 (45.45%), BPS-5 (22.73%), CDH-5 (22.73%) and congenital higher airway obstruction-2 (9.1%). Chromosomal screening was normal in 100% of cases. Follow-up was done for 2 years. Termination of pregnancy was required in 3 (13.64%) cases, postnatal death occurred in 4 (18.2%) cases, surgery was required in three cases of CDH soon after birth and two cases of extralobar BPS at 2 years of age. 10 (45.45%) cases were managed conservatively, who are doing well at 2-year follow-up., Conclusion: CPAMs, BPSs and CDH are the commonly detected antenatal thoracic lesions. Ultrasonography and MRI help detect and prognosticate the parents about the outcomes of these lesions. CVR and LHR with associated congenital malformations are important prognostic markers for these lesions. There is a need of long-term prospective studies to delineate the natural history of these lesions., (Copyright © 2023 Copyright: © 2023 African Journal of Paediatric Surgery.)

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.

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.

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 Vyšné 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.

Background: Congenital Diaphragmatic Hernia (CDH) is a severe congenital anomaly with significant morbidity and mortality. It can be isolated or can be associated with other congenital anomalies, including broncho-pulmonary sequestration (BPS). The association of CDH with BPS (CDH+BPS) is uncommon but has been previously reported, and it can complicate the course of the disease in patients with CDH. We report two cases of CDH+BPS that were recently treated at our CDH-Qatar (CDH-Q) program., Methods: We reviewed CDH-Q program registry to search for CDH+BPS and extracted the data for the identified cases. We also reviewed the previously published literature available on PubMed for similar cases., Results: Out of 53 cases of CDH referred to CDH-Q from January 2018 to December 2022, two cases of CDH+BPS were identified, with an estimated prevalence of 3.8% of this association in our CDH population. Both cases were born at term. Case 1 was diagnosed with CDH+BPS postnatally, while case 2 was diagnosed with CDH antenatally but BPS was diagnosed after birth. Both cases underwent a surgical repair of the CDH with resection of the associated BPS, and the histopathology of the resected lung tissue confirmed the presence of BPS in both. Both cases survived to discharge., Conclusion: The association of CDH+BPS is uncommon; however, it can have significant consequences on the management and the prognosis of patients with CDH. Reporting these cases is important to provide a better understanding of this association and its impact on CDH patients.

Background: Pulmonary sequestration (PS) is a congenital pulmonary malformation. Adenocarcinoma arising in PS is extremely rare., Methods and Results: We present the first reported case of synchronous intralobar PS and lung adenocarcinoma in the right lower lobe, which was successfully treated using robotic-assisted thoracic surgery (RATS). The robotic system allowed for easy identification, clipping, and dissection of the abnormal artery, highlighting its benefits over traditional surgical approaches., Conclusions: This case underscores the importance of considering the possibility of coexisting lung cancer in patients with a clinical diagnosis of PS and demonstrates the safety and efficacy of RATS in managing this rare condition., (© 2023 John Wiley & Sons Ltd.)

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.

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., (© 2023. Springer Nature Limited.)

Objective: This study aimed to summarize and analyze the characteristics of pulmonary sequestration to improve our understanding of this disease., Methods: Between January 2019 and April 2023, the clinical data of 13 patients with pulmonary sequestration underwent surgical treatment at the First Affiliated Hospital of Gannan Medical University., Results: The male-to-female ratio was 4:9, the age was 0.5 to 60 years, and the average age was 38 ± 19 years. There were 10 and 3 cases of intralobar and extralobar pulmonary sequestration, respectively. Chest enhanced computed tomography (CT) and three-dimensional vascular reconstruction showed that the abnormal blood vessels were derived from the descending thoracic aorta in nine cases and from other blood vessels in four cases. Three patients underwent thoracoscopic lobectomy, two underwent thoracoscopic segmentectomy, and eight underwent thoracoscopic wedge resection. All the patients successfully completed the surgery and were discharged postoperatively., Conclusions: Some patients with pulmonary sequestration exhibit no obvious symptoms. Patients with clinical symptoms are easily confused for pneumonia, bronchial cysts, lung abscesses, and lung tumors; therefore, patients with pulmonary sequestration are prone to missed diagnosis and misdiagnosis. Currently, enhanced chest CT combined with three-dimensional vascular reconstruction can accurately show the course, branches, and relationship with the mass of the feeding artery. Routine pathological examination is helpful to further clarify the diagnosis of pulmonary sequestration. Minimally invasive thoracoscopic surgery is the preferred treatment for patients with pulmonary sequestration. Surgical resection is safe and feasible, and satisfactory results are typically obtained., (© 2023 The Authors. The Clinical Respiratory Journal published by John Wiley & Sons Ltd.)

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.

Objectives: Intralobar pulmonary sequestration (ILS) is rare and its optimal clinical management remains ambiguous. This study aimed to introduce our 10-year experience in clinical management of ILS. And the application of our novel surgical method, thoracoscopic anatomical lesion resection (TALR) on ILS was introduced., Materials and Methods: Patients with ILS who received treatment between December 2010 and 2020 were included in this study, retrospectively. A binary logistic regression model was used to assess risk factors for preoperative symptoms. Intraoperative and postoperative outcomes were compared between the thoracoscopic lobectomy and lung-sparing surgery groups., Results: A total of 112 patients were included in this study. Age and maximum cyst diameter were risk factors for preoperative symptoms. Lung-sparing surgery proved to be safe and feasible with no residual lesions., Conclusions: The overall prognosis of early thoracoscopic surgery for ILS was good. Lung-sparing surgery, especially TALR could be used as a first-line surgery for ILS. It may resolve the long-standing controversy over whether surgery for asymptomatic patients with ILS., (© 2022 Wiley Periodicals LLC.)

Aim: The purpose of this study is to evaluate whether CPAM-volume ratio (CVR) can predict postnatal management (follow up for resolution and surgical treatment) in fetuses with fetal lung masses in the prenatal period., Materials and Methods: 44 patients who presented at our center with prenatally diagnosed CPAM (Congenital Pulmonary Airway Malformation) and BPS (Bronchopulmonary Sequestration) were analyzed. Obstetric history and outcomes, karyotype results, CVR, additional sonographic findings, characteristics of masses were recorded. CVR was calculated for all cases. İn the study we sought to identify a CVR threshold and did not use the thresholds classically used in the literature., Results: 20 fetal BPS and 24 CPAM cases were analyzed. After excluding 5 patients, 46% of the patients were diagnosed with BPS and 54% with CPAM. İn this study the cut off < 0,53 for CVR is taken, it predicts the no need for postnatal surgery with a sensitivity of 85% and a specificity of 88%. When we take the > 0,76 cut-off value for patients who will require emergency surgery within the first 10 days, it predicts the need for surgery with 90% sensitivity and 89% specificity. In addition, it was determined that all patients with mediastinal shift were operated., Conclusion: We believe that the CVR value and the presence of mediastinal shift should be evaluated in all cases of CPAM and BPS for prediction of the surgery. Proper counseling about the prognosis could be given to the family in cases with mediastinal shift and CVR value above 0,76., Competing Interests: Declaration of competing interest The authors did not declare any conflict of interest., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

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.

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., (© 2023. The Author(s).)

Background: Pulmonary sequestration is a rare congenital lung anomaly, presenting mostly in childhood and adolescence., Case Presentation: We report the case of a 26-year-old male patient presenting with pleuritic left sided chest pain and haemoptysis. Computed tomography of the chest showed features of intralobar pulmonary sequestration involving the left lower lobe, with arterial supply arising from the descending thoracic aorta above the diaphragm and normal venous drainage. Video assisted thoracic surgery was planned to perform a left lower lobectomy. Considering the risk of bleeding from the large artery supplying the sequestered segment, a posterolateral thoracotomy incision was made and left lower lobectomy was completed, with successful division of the arterial feeder. The patient was discharged home without complications. Pathologic examination of the specimen grossly revealed partial division of the lobe by two fissures with extensive adhesions into an upper and lower portion with no clear demarcation and a large vessel which enters the lower portion at the posterior inferior aspect, separate from the hilum with a diameter 10 mm. Microscopically, both portions of the lobe showed normally alveolated lung tissue with patchy recent intra-alveolar haemorrhage and evidence of chronic inflammation in the sequestered segment. There was no evidence of malignancy., Conclusion: This case highlights the rare presentation of pulmonary sequestration in adulthood and the importance of imaging to identify anomalous arterial supply to the sequestered segment in the left lower lobe of the lung. The use of safe surgical techniques to control the anomalous systemic arterial feeding vessel cannot be overemphasized., (© 2023. Crown.)

Background: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology., Methods: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment., Results: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization., Conclusions: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations., (Copyright: © 2023 Permanyer.)

Pulmonary sequestration (PS) is a rare congenital malformation, which mainly contains two variants involving extralobar and intralobar sequestrations. Extralobar sequestrations (ELS) are isolated from the remaining lung tissue and have their visceral pleura. Herein, we report the first case of a torsed ELS associated with congenital lobar emphysema. We described a boy who mainly presented with abdominal and chest pain. A contrast-enhanced computed tomography (CT) scan revealed a mildly enhanced posterior mediastinal mass with left lower lobar emphysema. Thoracoscopic surgery identified a dark and hemorrhagic mass that was connected to the thoracic aorta by a twisted feeding vessel and had its visceral pleura. Pathological findings were consistent with an ELS that had undergone torsion and infarction. The postoperative recovery was uneventful. For the left lower lobar emphysema, he was required for a regular outpatient follow-up. In conclusion, although extremely rare, physicians should maintain a high index of suspicion for torsed ELS when a posterior mediastinal mass with abdominal or chest pain is presented 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, χ 2 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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Congenital lung malformations represent a spectrum of lesions, each with a distinct cause and tailored clinical approach. This article will focus on the following malformations: congenital pulmonary airway malformations, formally known as congenital cystic adenomatoid malformations, bronchopulmonary sequestration, congenital lobar emphysema, and bronchogenic cyst. Each of these malformations will be defined and examined from an embryologic, pathophysiologic, and clinical management perspective unique to that specific lesion. A review of current recommendations in both medical and surgical management of these lesions will be discussed as well as widely accepted treatment algorithms., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction:  Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review., Methods:  Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed., Results:  Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 ± 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 ± 2.3 weeks and 3,276 ± 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration., Conclusion:  Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery., Material and Methods: A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed., Results: Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery., Conclusion: Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms., Level of Evidence: III., (Copyright © 2022. Published by Elsevier Inc.)

Background: Congenital-pulmonary-airway-malformation (CPAM) and bronchopulmonary-sequestration (BPS) are rare conditions. The objective of this study was to describe the sonographic characteristics and prenatal course of fetal lung mass., Methods: A retrospective study on 20 pregnancies with CPAM and BPS diagnosed during 2018-2018 was performed. Data evaluated included gestational age (GA) at diagnosis, size of lesion, CPAM-volume-ratio (CVR), associated sonographic findings and pregnancy outcome., Results: Fourteen cases of CPAM and six cases of BPS were evaluated. The average GA at diagnosis was 22 weeks'-gestation (range 17-26). 66% were macrocystic-multicystic. Five cases of BPS (71.4%) had other structural anomalies. None of the fetuses with CPAM had additional anomalies. Four pregnancies (20%) underwent termination due to severe hydrops fetalis. Two cases of CPAM (15%) had spontaneous resolution by 30 week's-gestation; three cases (20%) continued to grow during pregnancy and all the rest reached their largest size at 25-28 week's-gestation. Most cases delivered at term (average 37.6 range 32.2-41.4). Two children had lobectomy at 11 and 12 months of life., Conclusions: Careful follow-up during pregnancy may result in term delivery of adequate-for-gestational-age newborns, with no need for admission to the NICU, with only 10% need for lobectomy during the first year of life.

Background: Pulmonary sequestration (PS) is a rare lesion with independent blood supply from an anomalous systemic artery. A timely resection is considered as the best treatment for PS. Three-dimensional computed tomography angiography (3D-CTA) has been widely utilized for precise thoracic surgery. This study aimed to investigate the role of preoperative 3D-CTA and resection simulation in uniportal video-assisted thoracoscopic surgery (VATS) anatomical lung surgery for PS., Methods: The data of 20 consecutive PS patents undergoing anatomic lung resection between April 2011 and May 2021 in a single centre were retrospectively reviewed. These patients were divided into the 3D-CTA group (10 patients) and the control group (10 patients) according to the initial surgical planning with or without 3D-CTA. The perioperative parameters regarding safety and fluency such as the incidence of conversion to open thoracotomy, operation time, blood loss, complications and chest tube duration were analyzed., Results: This cohort included 12 female and 8 male patients, with a mean age of 45 years old (range 24-60 years). Nine cases demonstrated recurrent febrile, cough, or hemoptysis; whereas the other 11 patients were asymptomatic on admission. Eighteen (90.0%) intralobar and 2 extralobar PS were confirmed, whereas 18 (90.0%) lesions were located in the left thorax. The feeding vessels originated from the thoracic aorta in 16 patients (80.0%), the abdominal aorta in 3 (15.0%), and the inferior phrenic artery in 1 patient (5.0%). There was no major bleeding or 30 days mortality. The initial surgical planning included 9 uniportal and 1 two-port VATS in the 3D-CTA group, as compared with 10 two-port VATS in the control group. Thirteen lobectomies, 5 segmentectomies and 2 mass excisions were performed. However, no conversion was needed in the 3D-CTA group; whereas 6 (60.0%) conversions (4 to multiple-port and 2 to thoracotomy) occurred in the control group, indicating a significant difference (P = 0.003). In addition, the operation time in the 3D-CTA group was significantly shorter than those in the control group [(108.5 ± 24.9) min vs. (154.5 ± 39.4) min, P = 0.006]. The other surgery-related outcomes were similar between the two groups., Conclusion: Preoperative 3D-CTA facilitates the safe and fluent performance of uniportal VATS anatomical lung resection for PS with shortened operation time and lessened surgical conversions., (© 2022. The Author(s).)

We encountered a rare case of pulmonary sequestration supplied from the right renal artery, which was resected by video-assisted thoracic surgery with carbon dioxide insufflation and indocyanine green-guided technique. A 41-year-old woman with intralobar pulmonary sequestration supplied from the right renal artery was referred to our department. At the time of surgery, we used carbon dioxide insufflation to improve the manoeuvrable workspace for shutting off aberrant arteries and indocyanine green fluorescence guidance to differentiate the boundary of the sequestered lung from the normal lung. These procedures helped in the efficient resection of the lesion., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

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., (© 2022. The Author(s).)

Rationale: Pulmonary sequestration, which can be divided into 2 main types: intralobar pulmonary sequestration (IPS) and extralobar pulmonary sequestration, is an uncommon congenital condition for which surgical resection is usually indicated. Video-assisted thoracoscopic surgery, as compared with open thoracotomy, has increasingly become the preferred operative procedure in the treatment of PS, owing to less postoperative pain and faster recovery. This report describes a rare and challenging case with a giant IPS undergoing video-assisted thoracic lobectomy., Patient Concerns: A 39-year old woman suffered from recurrent pneumonia for nearly 3 years. An enhanced computed tomography scan performed in our hospital revealed a 12.0 cm × 10.0 cm-sized IPS in the left lower lobe, supplied by an 8-mm aberrant artery originating from the descending thoracic aorta., Diagnosis: Histology of the resected lobe confirmed the diagnosis of giant intralobar pulmonary sequestration associated with infection., Interventions: Thoracoscopic left lower lobectomy was performed., Outcomes: The patient has been discharged from the hospital on the ninth day after surgery with an uneventful recovery, she was in good health after a 1-year follow-up., Lessons: Although full of challenges, thoracoscopic lobectomy for giant IPS is a safe and feasible surgical procedure associated with reduced surgical trauma and postoperative pain as well as improved cosmetic results compared with traditional thoracotomy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

A pulmonary sequestration is a congenital malformation characterized by non-functional lung tissue with abnormal arterial systemic supply and abnormal connection to the bronchial tree. This may lead to recurrent infections rendering a surgical intervention more demanding. Because of multiple anatomic variations, it is important to obtain high-quality preoperative radiological clarification to determine the most suitable surgical approach. Although a non-surgical technique, consisting of embolization of the aberrant artery has been described, a surgical technique remains the treatment of choice in operable patients. Preoperative embolization of the aberrant artery may reduce the risk of haemorrhage but could cause technical challenges in a hybrid approach and therefore unforeseen peroperative stress to the surgical team. We report 2 adult patients with unusual intra-lobar sequestration with aberrant vascular rare anatomy. Both were treated by surgery. In the latter patient, we performed a hybrid approach. This was complicated by peroperative coils exposure making it a technical challenge to proceed., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a 'hybrid' malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.