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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.)

Objective: To study the clinical and morphological manifestations of lung sequestration., Material and Methods: Surgical material (lung tissue) of 18 patients (2017-2021) and 8 archival observations (1972-1994) of confirmed lung sequestration were examined. Histological staining (hematoxylin and eosin, Schiff reagent, Ziehl-Neelsen, picrofuchsin according to Van Gieson) and immunohistochemical reactions with antibodies to SMA, TTF1, CK5/6 (DAKO) were performed., Results: The ratio of men to women was 5:4; the average age of the patients was 35 years. Lung sequestration is localized mainly in the lower lobe segments, the left lung is involved in the pathological process more often. The cystic variant of lung sequestration with a chronic inflammatory process prevailed. Histological examination revealed the similarity of lung sequestration with congenital adenomatous lung malformation type 2 and rhabdomyomatous dysplasia. The respiratory and bronchial epithelium lost differentiation in the sequestered area., Conclusion: The morphological picture of lung sequestration is nonspecific and can manifest itself in various types of inflammatory reactions and remodeling of lung tissue.

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.

Congenital pulmonary airway malformations (CPAMs) have a range of morphologies with varying cyst sizes and histologic features (types 1 to 3). Evidence suggested they arise secondary to bronchial atresia, however, we recently showed that cases with type 1 and 3 morphology are driven by mosaic KRAS mutations. We hypothesized that 2 distinct mechanisms account for most CPAMs: one subset is secondary to KRAS mosaicism and another is due to bronchial atresia. Cases with type 2 histology, similar to sequestrations, would be related to obstruction and therefore negative for KRAS mutations regardless of cyst size. We sequenced KRAS exon 2 in type 2 CPAMs, cystic intralobar and extralobar sequestrations, and intrapulmonary bronchogenic cysts. All were negative. Most sequestrations had a large airway in the subpleural parenchyma adjacent to the systemic vessel, anatomically confirming bronchial obstruction. We compared morphology to type 1 and 3 CPAMs. On average, type 1 CPAMs had significantly larger cysts, but there remained substantial size overlap between KRAS mutant and wild-type lesions. Features of mucostasis were frequent in sequestrations and type 2 CPAMs, while their cysts were generally simple and round with flat epithelium. Features of cyst architectural and epithelial complexity were more common in type 1 and 3 CPAMs, which rarely showed mucostasis. Similarity in histologic features among cases that are negative for KRAS mutation support the hypothesis that, like sequestrations, the malformation of type 2 CPAMs is related to obstruction during development. A mechanistic approach to classification may improve existing subjective morphologic methods., Competing Interests: Conflicts of Interest and Source of Funding: Supported by the Society for Pediatric Pathology Young Investigator Grant (J.P.). The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

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.

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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.)

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.

Introduction: We describe the rare case of endobronchial tuberculosis (EBTB) and chronic pulmonary atelectasis with mediastinal distortion. Finding of the concomitant venous anomaly of inferior vena cava revealed the diagnosis of bronchopulmonary sequestration., Case Report: A 22-year-old Caucasian woman presented with a history of chronic cough, initially treated as bronchial asthma for a year. Chest X-ray showed fibrocaseous cavernous tuberculosis on the right lung. Acid Fast Bacilli (AFB) were found in sputum samples. Patient was treated for 6 months with usual antituberculous regiment. Control chest X-ray showed subatelectasis of the upper right lobe. Six months later the first thorax computed tomography (CT) showed complete atelectasis of the right lung. Patient was admitted to the hospital again after 6 years due to the persistent fever and cough. Endoscopic finding and histopathological analysis confirmed EBTB. Thoracic CT scan revealed duplication of inferior vena cava which led to profound vascular analysis and aberrant arterial vascularization of aortic origin that contributed to the diagnosis of bronchopulmonary sequestrations. Antituberculous treatment was initiated (streptomycin, isoniazid, rifampicin, ethambutol and pyrazinamide) and lasted for 8 months. After 8 months a follow-up fiberoptic bronchoscopy showed the progression of endoscopic finding with 60-70% tracheal stenosis. Histopathological finding of the mid-trachea showed non-specific granulations. During 7 years of follow-up repeated bronchoscopy and thoracic CT scans were unchanged and patient was well-shaped., Conclusions: The clinician should consider bronchopulmonary sequestration in the cases of recurrent EBTB., Competing Interests: No Conflict of Interest is declared, (Copyright (c) 2021 Tatjana Adzic-Vukicevic, Ana Petkovic, Nemanja Menkovic, Maja Stosic, Milos Bracanovic, Stefan Korica, Aleksandra Barac.)

Rationale: Pulmonary sequestration (PS) presenting with elevated serum tumor markers is rare, and it might be misdiagnosed as malignancy., Patient Concerns: A 26-year-old asymptomatic male patient was admitted because the x-ray showed an intrathoracic lesion. Meanwhile, the serum neuron-specific enolase (NSE) was elevated. Three-dimensional computed tomography angiography revealed an isolated feeding vessel arising from the aorta., Diagnoses: Extralobular PS was confirmed by computed tomography angiography and postoperative pathological staining., Interventions: Two-port thoracoscopic resection of the sequestrated lobe was performed., Outcomes: The serum NSE decreased to within the normal range and persisted during the follow up of 10 months., Lessons: A thorough work-up should be considered for the PS patients presenting with abnormal serum NSE. Detailed knowledge regarding the relationship between NSE and PS necessitates further studies.

In intralobar pulmonary sequestrations, vascular changes similar to those in pulmonary hypertension (PH) are generally observed, such as intimal proliferation and plexiform lesions. However, to our knowledge, a sequestrated lung manifesting vascular changes with both arteritis and a plexiform lesion has never been reported. A 25-year-old man was diagnosed with intralobar pulmonary sequestration. Pathologically, both arteritis and a plexiform lesion were observed in the sequestrated lung. Systemic vasculitis syndrome was clinically excluded, and the pathological findings appeared to be associated with local PH. Arteritis is an extremely rare finding; only one case of arteritis associated with local PH has been reported in intralobar sequestration. In this case, the artery near the plexiform lesion had milder inflammation and fibrosis, suggesting that the arteritis formed prior to the plexiform lesion. This is the first case of arteritis and a plexiform lesion co-occurring in intralobar pulmonary sequestration associated with local PH. This case may shed light on the formation of plexiform lesions and their association with arteritis.

This study aimed to evaluate the prognosis of pulmonary sequestration (PS) by measuring congenital cystic adenomatoid malformation volume ratio (CVR) value in fetal congenital PS. The fetal CVR in 49 cases of fetal PS diagnosed by prenatal ultrasound in Xiangyang No. 1 People's Hospital from March 2010 to June 2017 were measured, and the clinical outcomes were observed. According to the prenatal ultrasound CVR value, 49 fetuses diagnosed with PS were divided into 2 groups: group 1 with CVR≥1.26, and group 2 with CVR<1.26. The incidence rate of fetal edema, respiratory distress symptoms and survival rate were compared between the two groups. The risk factors of the fetal PS were evaluated by single and multiple Logistic regression analysis. The correlation between CVR and fetal prognosis was analyzed. Of the 49 fetuses, there were 34 cases of PS (ILS) type (69.39%, 34/49), 10 cases of PS (ELS) type I (20.41%, 10/49) and 5 cases of PS (ELS) type II (10.20%, 5/49). Forty-six cases (93.88%, 46/49) were born alive, there was 1 case (CVR ≥1.26) (2.04%, 1/49) of induced abortion, and 2 cases (CVR ≥1.26) (4.08%, 2/49) of stillbirths. In group 1 (n=24), 21 cases were born alive, and the incidence rate of newborn respiratory distress and fetal edema was 100% (21/21) and 79.17% (19/24) respectively. In group 2 (n=25), there were 3 cases (12%,3/25) of newborn respiratory distress, 3 cases (12%, 3/25) of fetal edema, and the rate of live birth was 100%. There were statistically significant differences between the two groups in the incidence of fetal edema, postpartum respiratory symptoms and survival rate. CVR was a risk factor for PS and was associated with fetal prognosis. CVR in the midtrimester of pregnancy is an effective index to evaluate the prognosis of fetal PS. CVR ≥1.26 is associated with an increased risk of fetal edema, infant respiratory distress and intrauterine or postnatal death.

Background: The potential complications of pulmonary sequestration are serious and may include recurrent pulmonary infection, hemoptysis, and tumorigenesis. Therefore, the gold standard of treatment has been surgery. Although an adequate boundary between the nonfunctional lung and normal lung is required for the resection of pulmonary sequestration, the boundaries have been conventionally identified intraoperatively with inflation/deflation of the target segment by clamping and unclamping the relevant bronchus. The technique of visualizing the demarcation line based on near-infrared fluorescence imaging with indocyanine green was recently developed., Case Presentation: A 42-year-old Japanese woman with right Pryce III intralobar sequestration was admitted to our hospital. We planned video-assisted thoracoscopic wedge resection of the right sequestration using near-infrared fluorescence imaging with indocyanine green because of the small volume of the nonfunctional region. The aberrant artery was recognized in the pulmonary ligament; the artery was cut off after ligation. Indocyanine green at 5 mg/body was rapidly injected into the peripheral vein, and the boundary of the sequestration was clearly identified under near-infrared fluorescence imaging., Conclusion: Near-infrared fluorescence imaging with indocyanine green is safe and useful for the identification of the boundary of a pulmonary sequestration.

Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia., (Copyright © 2019. Published by Elsevier Inc.)

Introduction:  Rhabdomyomatous dysplasia (RD) is a pathologic finding in CPAMs that was incorrectly attributed to their malignant potential. The increasing recognition of extrathoracic (intradiaphragmatic and intraabdominal) congenital pulmonary airway malformations (CPAMs) offers a clue to the origin of RD. We hypothesize that the presence of RD is related to the CPAM's anatomic location., Materials and Methods:  Retrospective review was performed of all children who underwent resection of a CPAM during a 10-year period. The age at the time of operation, location of the CPAM, and pathologic findings were collected. Peridiaphragmatic location was defined as within the inferior pulmonary ligament, deep to the diaphragmatic portion of the parietal pleura ("intradiaphragmatic") or adjacent to the abdominal side of the diaphragm. Statistical analysis was performed using Fisher's exact test for 2 × 2 tables., Results:  Twenty-six patients with CPAM were identified. Preoperative imaging was performed by computed tomography (CT) scan (16/26), ultrasound (5/26), magnetic resonance imaging (MRI) (1/26), and chest radiograph (4/26). The median age at resection was 15 months. Of these, 16 were pure cystic adenomatoid malformations, 4 were extralobar sequestrations, 4 were intralobar sequestrations, and 2 were bronchogenic cysts. Nine lesions were peridiaphragmatic with four being intradiaphragmatic (44%). Eight of the nine resected peridiaphragmatic lesions contained histologic evidence of rhabdomyomatous changes (89%, confidence interval [CI] 52-99%). None of the other lesions contained RD (CI 0-19%, p  < 0.001)., Conclusion:  RD was seen exclusively, and in virtually all peridiaphragmatic CPAMs. While the exact significance of RD remains unclear, it may represent incorporation of striated muscle tissue associated with the developing diaphragm., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: Pulmonary sequestration (PS) is a form of congenital pulmonary malformation that is generally diagnosed in childhood or adolescence and usually resected when diagnosed. We aim to identify the clinical presentation and course of patients diagnosed to have PS during adulthood., Methods: Using a computer-assisted search of Mayo clinic medical records, we identified adult patients with PS diagnosed between 1997 and 2016. Clinical and radiological data were collected including postoperative course for those who underwent surgical resection., Results: We identified 32 adult patients with PS; median age at diagnosis was 42 years (IQR 28-53); 17 patients (53%) were men. The median sequestration size was 6.6 cm (IQR 4.4-9.3). The type of sequestration was intralobar in 81% and extralobar in 19%. The most common location was left lower lobe posteromedially (56%). Forty-seven percent of the patients presented with no relevant symptoms. The most common radiographic finding was mass/consolidation in 61% and the most common feeding artery origin was the thoracic aorta (54%). Surgical resection was performed in 18 patients (56%) and postoperative complication was reported in 5 patients (28%). There was no surgical mortality. Median duration of follow-up after diagnosis for unresected cases, most of whom were asymptomatic, was 19 months (IQR 4-26) with no complications related to the PS reported., Conclusions: Nearly one-half of adult patients with PS present with no relevant symptoms. The decision regarding surgical resection needs to weigh various factors including clinical manifestations related to PS, risk of surgical complications, comorbidities, and individual patient preferences.

Objective: This study aimed to determine the significance of the venous drainage pattern of bronchopulmonary sequestration (BPS) in the prenatal course., Methods: The venous drainage pattern of fetuses with BPS was determined with high-definition flow and confirmed by postnatal three-dimensional computed tomography angiography scan or autopsy. The volume of BPS lesions during gestation was recorded by the three-dimensional ultrasonographic Virtual Organ Computer-Aided Analysis software. The relationship between venous drainage pattern and prenatal characteristics was determined., Results: Seventy-one fetuses were enrolled: 35 with systemic venous drainage (SVD) and 36 with pulmonary venous drainage (PVD). The volumes of BPS lesions significantly increased from the middle second trimester to the later second trimester in the SVD group. A marked decrease from the later second trimester to the third trimester was observed in the PVD group. The incidences of associated anomalies, hydrops, and polyhydramnios in the SVD group were 14.2, 23.3, and 33.3%, respectively, significantly higher than those in the PVD group (0, 0, and 5.6%, respectively)., Conclusions: Our data indicate that SVD is correlated with a higher risk of associated anomalies and an unfavorable prenatal course in fetal BPS. Identification of the venous drainage pattern is of clinical significance in predicting the prenatal behavior of fetal BPS., (© 2017 S. Karger AG, Basel.)

Two cases of extralobar pulmonary sequestrations from a walrus (Odobenus rosmarus) and a Steller sea lion (Eumetopias jubatus) are described in the present study. Grossly, an independent, soft unilocular cystic mass was found within the abdominal cavities of both animals, adherent to the diaphragm in O. rosmarus and attached to the cardia of the stomach in E. jubatus. Histopathologically, the cysts were lined by pseudostratified ciliated columnar epithelium with abundant goblet cells, while the wall comprised of glands, hyaline cartilage, bronchiole- and alveolus-like structures, smooth muscles, and large, well-developed elastic and muscular arteries. The pinniped cases presented are exceptionally rare and to the best of the authors' knowledge, marks the first descriptions of this congenital anomaly in wildlife.

Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings. An 8-year retrospective review of the perinatal and pathology database of a single tertiary care center identified 42 cases of congenital cystic lung lesions of which 36 had known prenatal ultrasound and prenatal course available. Final pathologic diagnoses were 15 CPAM (41%), 7 BPS (19%), and 9 hybrid BPS and CPAM lesions (25%). Five cases with bronchial atresia were also identified (either in isolation or associated with CPAM or BPS). The overall characteristics of these lesions by prenatal ultrasound, postnatal imaging, and ultimate histopathologic diagnosis are described.

Background: A paucity of evidence regarding the natural history of congenital pulmonary airway malformations (CPAMs) and pulmonary sequestration (PS) has resulted in a divergence in management strategy of asymptomatic cases., Methods: We describe the long-term clinical course of 119 children diagnosed with these lesions treated at Great Ormond Street Hospital (GOSH). Cases were identified via the GOSH patient database. Study entry required the identification of a cystic lung lesion on prenatal ultrasound and confirmation of CPAM/PS on postnatal CT imaging. Patients followed up for at least 5 years were included., Results: 51 (43%) patients were managed surgically; 8 (6.7%) as an emergency during the neonatal period, 6 (5.1%) electively due to concerning features on CT scan, 20 (17%) following medical advice, 1 (0.8%) following a severe respiratory infection and in 5 (4.2%) the indication was unclear. The indication in 11 (9.2%) was recurrent respiratory infection and median age at surgery was 1.6 years (range 0.4 to 4.6 years). No cases of malignancy were identified on histological examination of resected lesions. 68 (57%) patients were managed conservatively for a median period of 9.9 years (range 5.2 years to 18 years). Seven (10%) were discharged, one was followed-up elsewhere (1.5%) and eight (11%) were lost to follow-up. In four patients (5.9%), the lesion resolved spontaneously. 52 (76%) continue to be followed-up and remain asymptomatic., Conclusions: This is one of the largest case series documenting the natural history of CPAMs and PS following a prenatal diagnosis and demonstrates that conservative management is a reasonable option in selected cases., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Pulmonary sequestration is a rare congenital pulmonary anomaly. Here we report a case of extralobar sequestration with calcification. A 34-year-old man was admitted to our department because of a tumor shadow on the chest radiological examination. Computed tomography examination revealed a mass measuring 5 cm above the left diaphragm. The mass contained cysts and calcification. Aberrant vessels were not detected. Teratoma, hamartoma or solitary fibrous tumor were suspected and the surgical treatment was performed. The postoperative pathological diagnosis was extralobar pulmonary sequestration.

Purpose: To describe the natural course of extralobar pulmonary sequestration (EPS) and identify factors associated with spontaneous regression of EPS., Materials and Methods: We retrospectively searched for patients diagnosed with EPS on initial contrast CT scan within 1 month after birth and had a follow-up CT scan without treatment. Spontaneous regression of EPS was assessed by percentage decrease in volume (PDV) and percentage decrease in sum of the diameter of systemic feeding arteries (PDD) by comparing initial and follow-up CT scans. Clinical and CT features were analysed to determine factors associated with PDV and PDD rates., Results: Fifty-one neonates were included. The cumulative proportions of patients reaching PDV > 50 % and PDD > 50 % were 93.0 % and 73.3 % at 4 years, respectively. Tissue attenuation was significantly associated with PDV rate (B = -21.78, P < .001). The tissue attenuation (B = -22.62, P = .001) and diameter of the largest systemic feeding arteries (B = -48.31, P = .011) were significant factors associated with PDD rate., Conclusion: The volume and diameter of systemic feeding arteries of EPS spontaneously decreased within 4 years without treatment. EPSs showing a low tissue attenuation and small diameter of the largest systemic feeding arteries on initial contrast-enhanced CT scans were likely to regress spontaneously., Key Points: • Extralobar pulmonary sequestration (EPS) could show spontaneous regression. • Initial CT features may predict spontaneous regression of EPS. • The tissue attenuation and diameter of systemic feeding artery are important factors.

Recurrent pneumonia is one of the most frequent reasons for referral to paediatric chest physicians. The diagnostic work-up is dependent on whether infection repeatedly occurs in the same lung lobe, or affects multiple lobes and/or different areas in different episodes. A 13-year-old girl was admitted with a second episode of right lower lobe pneumonia. The chest x-ray demonstrated an inhomogeneous opacity, without a clearly recognizable segmental distribution. A contrast-enhanced CT scan, was therefore performed that showed a polycyclic consolidation with blood supply from a systemic artery, originated from the thoracic aorta. A diagnosis of superinfection of an intralobar sequestration was made. The patient was treated with systemic antibiotics and, four weeks later, a segmental resection of the lesion was performed. The histological evaluation of the surgical specimen confirmed the diagnosis.

We reported a case of bronchial atresia requiring differentiation from the intralobar sequestration. A 42-year-old man was referred to our institution with suspicion of intralobar sequestration, based on a 3-dimensional computed tomography (CT) angiography that showed abnormal blood vessels from the right inferior phrenic artery flowing into the right lower lobe. CT revealed a lesion between S9 and S10 wherein there were refluxed blood vessels from A9 without an accompanying bronchus, with polycysts and emphysematous changes. Ventilation-perfusion scintigraphy revealed a reduction in uptake in the same sites. He was diagnosed as congenital bronchial atresia preoperatively, and we performed a right basal segmentectomy. Pathological examination confirmed the bronchiectasis and emphysematous changes in the lung parenchyma, but malignant findings were not confirmed.

Vascular changes observed in intralobar pulmonary sequestration (PS) have been reported to be similar to those observed in pulmonary hypertension (PH). However, atherosclerosis in the pulmonary artery, which is one of the characteristic arterial changes of PH, has scarcely been reported in PS. Here, we report this unique manifestation in a 66-year-old man, in whom an intralobar PS fed by an aberrant artery branching from the left gastric artery had been identified 10 years earlier, and who was diagnosed with pneumonia after having symptoms of fever and cough. He was treated by lobectomy of the lower lobe of his right lung. Subsequently, a culture test of the fluid from the sequestrated lung was performed, which identified Mycobacterium avium. Subsequent pathological examination of the sequestrated lung demonstrated multiple epithelioid cell granulomas. Furthermore, atherosclerosis of the aberrant artery was also found, which may be associated with his advanced age. This is an extremely rare case of right intralobar PS supplied by aberrant artery with atherosclerosis originating from left gastric artery passing over the diaphragm., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Pulmonary sequestration is a rare congenital anomaly and most intralobar sequestrations were located in lower lobes., Case Report: We reported an unusual 28-yearold female patient with intralobar pulmonary sequestration on the left lower lobe, successfully treated with lobectomy. Computed tomography (CT) of the chest with intravenous contrast revealed multiple clustered cystic lesions in the left lower lobe with aberrant artery from descedenting aorta. Additional aortography showed an aberrant artery (3 mm in diameter) arising from the abdominal aorta and flowing into the lesion., Conclusion: Standard therapy regimen for pulmonary sequestration includes surgery. CT scan of thorax with intravenous contrast and aortography represent the gold standard for its diagnosis. Tumor-like shadows seen on the chest radiography or CT scans should not be always suspected on malignant lesions.

Background: Pulmonary sequestration is a rare pulmonary malformation whose complex pathogenesis is not completely understood. Extrapulmonary sequestrations are always hereditary malformations and are usually diagnosed during childhood. Some intralobar sequestrations in adults, by contrast, seem to be acquired. The clinical presentation is non-specific and often misinterpreted, which results in delayed diagnosis. Surgical resection continues to be the gold standard. Despite the low incidence, new technologies developed in the past few decades, e.g. preoperative interventional angiology procedures and video-assisted lung resection, have changed the management of the disease., Methods: A prospective data collection was performed on adult patients who had undergone surgical resection of a pulmonary sequestration in four different centres during a period of 23 years. These data were retrospectively analysed., Results: A total of 14 patients with intralobar sequestrations (n = 11, 79 % left lower lobe) underwent surgical resection. The male/female ratio was 8/6 (median age 50 years). Non-specific pulmonary symptoms such as recurrent infections had a high prevalence (n = 6, 42 %). Two patients had haemoptysis. Three patients were asymptomatic. All patients had a chest CT, 7 patients had an additional abdominal CT, while 10 underwent angiography. Preoperative embolization was performed in 5 of these patients (branches of the thoracic aorta: in 2 cases, branches of abdominal aorta: in 2 cases, and branches of both: in 1 case). Resection was mostly performed with an open surgical approach (n = 12, 86 %). Most patients had a non-anatomic pulmonary resection (n = 8, 57 %). In 7 patients, a microbiologic examination revealed a superinfection with Streptococcus pneumoniae, while 1 patient had an invasive mycosis with Candida albicans., Conclusion: In patients with non-specific pulmonary symptoms and undetermined lesions, especially in the lower lobes, pulmonary sequestrations must be considered in the differential diagnostic evaluation even in adult and elderly patients. If there are no contraindications, surgical removal is basically recommended and may be minimally-invasive in selected cases. The planning of the resection can be facilitated by preoperative evaluation of the arterial supply (branches of the thoracic or abdominal aorta, or both). In cases with haemoptysis or blood supply over the abdominal aorta, preoperative embolization is indicated., (Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: This study aimed to evaluate the role of multidetector computed tomography angiography in diagnosing patients with pulmonary sequestration., Methods: We retrospectively analyzed the computed tomography studies and clinical materials of 43 patients who had undergone preoperative multidetector computed tomography angiography in our hospital and had pathologically proven pulmonary sequestration. Each examination of pulmonary sequestration was reviewed for type, location, parenchymal changes, arterial supply and venous drainage on two-dimensional and three-dimensional computed tomography images., Results: Multidetector computed tomography successfully detected all pulmonary sequestrations in the 43 patients (100%). This included 40 patients (93.0%) with intralobar sequestration and 3 patients (7.0%) with extralobar sequestration. The locations of pulmonary sequestration were left lower lobe (28 cases, 70% of intralobar sequestrations), right lower lobe (12 cases, 30% of intralobar sequestrations) and costodiaphragmatic sulcus (3 cases). Cases of sequestered lung presented as mass lesions (37.2%), cystic lesions (32.6%), pneumonic lesions (16.3%), cavitary lesions (9.3%) and bronchiectasis (4.6%). The angioarchitecture of pulmonary sequestration, including feeding arteries from the thoracic aorta (86.1%), celiac truck (9.3%), abdominal aorta (2.3%) and left gastric artery (2.3%) and venous drainage into inferior pulmonary veins (86.0%) and the azygos vein system (14.0%), was visualized on multidetector computed tomography. Finally, the multidetector computed tomography angiography results of the sequestered lungs and angioarchitectures were surgically confirmed in all the patients., Conclusions: As a noninvasive modality, multidetector computed tomography angiography is helpful for making diagnostic decisions regarding pulmonary sequestration with high confidence and for visualizing the related parenchymal characteristics, arterial supply, and venous drainage features to help plan surgical strategies.

Necrotizing arteritis is a complex lesion of pulmonary hypertension, as are plexiform lesions, and is classically recognized as grade 6 in the Heath and Edwards grading scheme for hypertensive pulmonary vascular disease. The vascular changes observed in intralobar pulmonary sequestration have been reported to be similar to those observed in pulmonary hypertension, such as plexiform lesions. However, necrotizing arteritis occurring in an intralobar sequestration of a patient without systemic vasculitis syndrome has never been reported to our knowledge. Here, we report a case of a 38-year-old woman with pulmonary sequestration detected on a medical checkup. She was treated with surgery, and subsequent pathological analyses revealed necrotizing vasculitis in her sequestrated lung. We suspected systemic vasculitis syndromes, such as Takayasu arteritis, polyarteritis nodosa, and antineutrophil cytoplasmic antibody-associated vasculitis. However, physical and blood examination did not show any other abnormalities, and hence, she did not have systemic vasculitis syndrome. Immunohistochemical analyses of the resected specimen showed that inflammatory cells of the arteries were mainly composed of T lymphocytes. T-lymphocytic inflammation with little neutrophil and histiocyte infiltration may be a pathological feature of necrotizing arteritis observed in pulmonary sequestration. This is the first case to our knowledge of necrotizing arteritis in an intralobar pulmonary sequestration of a patient without systemic vasculitis syndrome., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Ectopic adrenocorticotrophic hormone (ACTH)-producing bronchopulmonary carcinoid arising in a bronchopulmonary sequestration is extremely rare. The case of a 67-year-old woman with a 1.7-cm nodule in the mediastinal side of the left lower lobe is presented. At 52 years of age, she was diagnosed as having ACTH-dependent Cushing's syndrome (CS). However, no ectopic source of ACTH-secretion was detected. Seven years later, she underwent a bilateral adrenalectomy because of aggravation of her health condition. This time, tumor excision was performed by thoracoscopic surgery. The tumor adhered sparsely to the mediastinal pleura and the left lower lobe and was bluntly separated from these tissues. Pathologically, the tumor was a typical carcinoid arising in an extralobar pulmonary sequestration. Immunohistochemical staining confirmed the secretion of ACTH by bronchopulmonary carcinoid tumor cells. After surgery, the serum ACTH level was almost normalized, and the dexamethasone (1 mg) suppression test showed significant suppression of ACTH., (© The Author(s) 2015.)

Pulmonary congenital abnormalities are rare disorders including congenital pulmonary airway malformations (CPAM) and pulmonary sequestration (PS). CPAM is a lesion characterized by the presence of anomalous bronchiolar or acinar structures, variable in size, either cystic or not cystic. PS is generally defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that derives its blood supply from systemic vessels. We describe a case of a baby girl with a very rare association between CPAM type 2 and intralobar pulmonary sequestration (IPS) focusing on the cystic lesions typical of CPAM and on the lymphatic and blood vessels. The cells lining the cysts often were positive for D2-40 (oncofetal protein M2A). Lymphatic endothelial cells, positive for D2-40, were widely present in the lung parenchyma and dilated lymphatic vessels were present also in the inter-alveolar septa. Moreover, we discuss the pathogenesis of CPAM and its classification criteria., (© Copyright Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Aim: The management of asymptomatic congenital cystic lung malformations is controversial. Arguments for excision of asymptomatic lesions are the potential for infection and malignancy. Following antenatal detection, our institute performs a CT at 1month, clinic follow-up by 3months to discuss the controversial management, and offers surgery by 6months of age. We investigated the histopathology of asymptomatic lesions to determine whether there was evidence of subclinical infection or malignancy., Methods: A retrospective review of prospectively collected antenatal congenital cystic lung malformations more than a 10year period (2005-2014) was conducted. Information was gathered from the antenatal registry and histopathology reports. Infection was defined by the presence of microabscesses or neutrophil/macrophage infiltration, as per histopathological criteria., Main Results: From the cohort of 99 patients, the study focused on 69 asymptomatic lesions. These cases comprised 34 congenital pulmonary airway malformations (CPAM), 15 pulmonary sequestrations (PS), and 20 hybrid lesions. Eighteen cases (26%) had microscopic disease - 16 cases of infection and 2 tumors. The infectious cases comprised 7 with microabscesses and 9 with neutrophil/macrophage infiltration. There were two cases of tumors, namely pleuropulmonary blastoma. These tumors were followed up by the oncology team with regular imaging until 3years of age and clinical review thereafter., Conclusion: Twenty-six percent of antenatally detected, asymptomatic cystic lung malformations demonstrated either subclinical infection or malignancy. This information can be used for counseling parents and determining the method of treatment., (Copyright © 2016 Elsevier Inc. All rights reserved.)

While bronchopulmonary sequestration typically has a benign course, this congenital lung malformation has a high mortality rate when associated with untreated in utero tension hydrothorax and hydrops. Hydrops related to bronchopulmonary sequestration is believed to result from torsion of the mass with compromise of the associated blood supply. The impaired venous return of the mass then leads to tension hydrothorax with compression of the heart and mediastinal vessels, impairing global venous return. To our knowledge, this scenario has only been described prenatally by ultrasound. We present the imaging findings of a dichorionic, diamniotic twin gestation with one fetus developing tension hydrothorax and hydrops from presumed intermittent torsion of a bronchopulmonary sequestration. This diagnosis was only able to be confirmed by MRI prior to the use of ultrasound-guided interstitial laser photocoagulation for the treatment of this anomaly., (© 2015 S. Karger AG, Basel.)

We report a case of extralobar pulmonary sequestration (ELS) in a young woman, presenting with right recurring massive pleural effusion. The patient initially underwent a diagnostic Video Assisted Thoracic Surgery (VATS) for a suspected diffuse malignancy. After the aspiration of the pleural effusion we observed a highly vascularised cystic mass, with its origin from the right lower lobe. As we tried to retract the right lower lobe, the mass broke with massive bleeding requiring emergency right lateral thoracotomy. The mass was succesfully excised, resembling an extra-lobar pulmonary sequestration. The patient was discharged on post-operative day 5., (Copyright © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: To investigate clinical and pathological features of lung lesions in children., Methods: Clinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children., Results: A total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%)., Conclusions: Infectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.

We report the case of a 47-year-old woman who presented with the unique asymptomatic malformation of an extralobar pulmonary sequestration communicating with a bronchogenic cyst. Despite computed tomography and magnetic resonance imaging of the chest, the diagnosis could not be established before a left thoracotomy was performed. The sequestrated lobe and bronchogenic cyst were then successfully resected., (© The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.)

Surgical resection of congenital lung lesions has evolved with minimally invasive and parenchyma-preserving techniques. Although these lesions are usually small and their limits can be suspected by direct vision or palpation, there are no clear anatomic landmarks to enable a precise resection. This report presents a new technique that helps to define the limits of intralobar sequestrations, leading to a safe and anatomic thoracoscopic segmentectomy., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)