The role of 3-dimensional reconstruction imaging in bronchopulmonary sequestration.

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.

Recognition of an extralobar pulmonary sequestration during lung resection.

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.

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

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

Congenital lung malformations.

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

Unravelling the mystery of a rare infection: a challenging case of pulmonary sequestration with Mycobacterium avium complex and the importance of a thorough microbiological investigation.

Pulmonary sequestration is a rare congenital condition. It is a dysplastic lung tissue with a separate systemic blood supply and without a bronchial tree connection. The emergence of a superimposed infection can lead to its diagnosis, such as Staphylococcus aureus, Pseudomonas aeruginosa, Nocardia asteroids and Aspergillus sp pneumonia. Mycobacterium avium complex (MAC) superimposed disease is exceedingly rare. We report a case of a man in his third decade without known medical disorders presenting with a persistent cough. After an extensive microbiological workup, an MAC infection was diagnosed. An elevated carbohydrate antigen 19-9 (CA 19-9) was also noted. He was treated with antimycobacterial therapy and lobectomy resulting in clinical improvement and CA19-9 normalisation. This case illustrates the value of comprehensive microbiological investigations in patients with chronic respiratory symptoms and imaging findings that are not typical of bacterial pneumonia. Clinical studies remain needed to investigate the utility of CA 19-9 in a scoring system to guide MAC therapy.

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020.

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.

INTRODUCCIÓN: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. MÉTODOS: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. RESULTADOS: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. CONCLUSIONES: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.

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

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

Ten-year experience in the clinical management of intralobar pulmonary sequestration in children.

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.

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

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

Intrapulmonary Lung Sequestration.

A 16-year-old female, without relevant previous medical history, presented with 1 year episodic and progressive hemoptysis. CT scan revealed an abnormal blood supply from the descending thoracic aorta to the left lower lobe, the later presenting with significant signs of hyperperfusion. Therapy consisted of ligation of the anomalous vessel followed by left lower lobe lobectomy. Lung sequestration is a rare congenital pulmonary malformation, mostly diagnosed in childhood. The typical symptoms are recurrent pulmonary infections or productive cough and only in rare situations severe hemoptysis occurs. Preoperative planning with CT or MR angiography is important to identify the aberrant blood supply. The treatment of choice is surgical ligation of the feeding vessel in combination with lung resection.

Clinical analysis of extralobar pulmonary sequestration with torsion in children: report of 6 cases.

BACKGROUND: Extralobar pulmonary sequestration is an uncommon congenital pulmonary malformation. Clinically, pedicle torsion of extralobar pulmonary sequestration is extremely rare. Due to inadequate awareness of its atypical presentation and imaging characteristics, clinical diagnosis is very difficult, and it is extremely easy to misdiagnose. CASE PRESENTATION: There were 6 children (3 males and 3 females), aged 3-12 years old. The main clinical symptoms of the children were abdominal and chest pain (3 cases), abdominal pain (1 case), chest pain (1 case), and vomiting and abdominal distension (1 case). Two cases were accompanied by fever. Preoperative ultrasound revealed a well-bordered mass with soft-tissue density, accompanied by pleural effusion. On contrast-enhanced computed tomography scans, the mass showed no obvious enhancement. A blood supply was only present in 1 case, and there was no feeding artery shown in the other 5 cases. Extralobar pulmonary sequestration with haemorrhagic infarction was pathologically confirmed. On postoperative days 2-6, the children were discharged uneventfully. There were no complications during the median follow-up of 4 months. CONCLUSIONS: Torsed extralobar pulmonary sequestration usually occurs in childhood or adolescence, with abdominal and/or chest pain as the primary symptoms. Imaging examination shows a well-defined soft-tissue mass without enhancement. The feeding vessel is not clearly displayed in the mass, and extralobar pulmonary sequestration is accompanied by varying amounts of pleural effusion. Video-assisted thoracoscopic surgical resection is associated with excellent prognosis.

VATS surgical anatomical resection of bronchopulmonary sequestration presenting as chest sepsis.

BACKGROUND: Bronchopulmonary sequestration (BPS) is a malformation of the lungs resulting in lung tissue lacking direct communication to the tracheobronchial tree. Most cases demonstrate systemic arterial blood supply from the descending thoracic aorta, the abdominal aorta, celiac axis or splenic artery and venous drainage via the pulmonary veins with occasional drainage into azygos vein. BPS is considered a childhood disease and accounts for 0.15-6.40% of congenital pulmonary malformations. BPS is divided into intralobar sequestrations (ILS) and extralobar sequestrations (ELS) with ILS accounting for 75% of all cases. METHODS: Here we present our 11-year experience of dealing with BPS; all cases presented with recurrent chest sepsis in young-late adulthood regardless of the type of pathological sequestration. The surgical technique employed was a minimally invasive video-assisted thoracoscopic anterior approach (VATS). RESULTS: Between May 2010 and September 2021, we have operated on nine adult patients with bronchopulmonary sequestration who presented late with symptoms of recurrent chest sepsis. Most patients in the cohort had lower lobe pathology, with a roughly even split between right and left sided pathology. Moreover, the majority were life-long never smokers and an equal preponderance in males and females. The majority were extralobar sequestrations (56%) with pathological features in keeping with extensive bronchopneumonia and bronchiectasis. There were no major intra-operative or indeed post-operative complications. Median length of stay was 3 days. CONCLUSIONS: Dissection and division of the systemic feeding vessel was readily achievable through a successful anterior VATS approach, regardless of the type of sequestration and without the use of pre-operative coiling of embolization techniques. This approach gave excellent access to the hilar structures yet in this pathology, judicious and perhaps a lower threshold for open approach should be considered.

A Horseshoe Intralobar Lung Sequestration Resection by Bilateral Robot-Assisted Surgery.

We present the case of a 20-year-old male patient presenting a right lower intralobar pulmonary "horseshoe" sequestration extending into the left cavity supplied by 4 aberrant arteries from the thoracic descending aorta. The surgical approach for this exceptional and challenging presentation was based on thorough analysis of the chest computed tomography scan aided by 3-dimensional reconstructions. The latter helped us better understand this complex malformation. Surgery was done by robot-assisted bilateral approach with en bloc extraction through the left side.

'Hybrid' Bronchopulmonary Malformation - Lobar Emphysema and Extra Lobar Sequestration.

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.

Endovascular treatment of a right pulmonary sequestration supplied by an aneurysmal aberrant artery originating from the abdominal aorta / Tratamento endovascular de sequestro pulmonar direito irrigado por artéria aneurismática aberrante originária da aorta abdominal

Abstract Endovascular embolization of arteries feeding pulmonary sequestrations is a growing therapeutic option. A 51-year-old woman with chest pain and hemoptysis was admitted. During hospitalization she presented 150 mL hemoptysis, hypotension, and hematocrit fell to 23.3%. Contrast-enhanced computed tomography confirmed a pulmonary sequestration irrigated by an aneurysmal artery from the abdominal aorta. The patient underwent endovascular coil embolization of the artery feeding the aneurysm and an Amplatzer device was deployed in the proximal third of the sequestration artery. Subsequent contrast-enhanced computed tomography confirmed complete thrombosis of the aberrant artery feeding the aneurysm and absence of irrigation of the pulmonary sequestration. At 56 months follow-up the patient remains asymptomatic, tomography showed involution of the sequestration and complete thrombosis of the aberrant artery. The challenges presented by the different treatment alternatives are discussed.

Resumo A embolização endovascular das artérias que alimentam os sequestros pulmonares é uma opção terapêutica em crescimento. Uma mulher de 51 anos com dor torácica e hemoptise foi internada. Durante a internação, ela apresentou hemoptise de 150 mL, hipotensão e queda do hematócrito para 23,3%. A tomografia computadorizada com contraste confirmou um sequestro pulmonar irrigado por uma artéria aneurismática originária da aorta abdominal. A paciente foi submetida a embolização endovascular da artéria que alimentava o aneurisma com uso de coils e dispositivo Amplatzer no terço proximal da artéria sequestrante. A tomografia subsequente confirmou a trombose completa da artéria aberrante que alimentava o aneurisma e a ausência de irrigação dentro do sequestro pulmonar. No seguimento de 56 meses, a paciente permanecia assintomática, e a tomografia mostrou involução do sequestro e trombose completa da artéria aberrante. Os desafios apresentados pelas diferentes alternativas de tratamento são discutidos neste artigo.

Secuestro pulmonar intralobar / Intralobar Pulmonary Sequestration

Introducción: El secuestro pulmonar está representado por masas de tejido pulmonar displásico, no funcionante, sin comunicación con el árbol bronquial. Objetivo: Presentar tres casos de secuestro pulmonar intralobar tratados por el autor. Caso clínico: Se presentan tres pacientes tratados entre 2013 y 2018 con diagnóstico de secuestro pulmonar. Dos fueron del sexo masculino (44 y 60 años de edad) y una del femenino (20 años de edad). Los síntomas fueron dolor torácico (1) y cuadro de infección pulmonar grave (2). En dos enfermos el secuestro se localizó en el lóbulo inferior derecho y en uno en el inferior izquierdo. Las operaciones fueron lobectomías inferiores derecha (1) e izquierda (1) y bilobectomía inferior y media derechas por afectación del lóbulo medio. Dos tuvieron una evolución satisfactoria y uno presentó un empiema pleural que resolvió con tratamiento antibiótico. Conclusiones: Los secuestros pulmonares son raros y los síntomas suelen asociarse con infección pulmonar. En la mayoría de los casos es necesario realizar una lobectomía. La evolución postoperatoria suele ser buena(AU)

Introduction: Pulmonary sequestration is characterized by masses of dysplastic, nonfunctioning lung tissue, without any communication with the bronchial tree. Objective: To present three cases of intralobar pulmonary sequestration treated by the author. Clinical case: The respective cases are presented of three patients with a diagnosis of pulmonary sequestration treated between 2013 and 2018. Two were male (44 and 60 years old, respectively) and one was female (20 years old). Their symptoms were chest pain (1) and severe lung infection (2). In two patients, the sequestration was located in the lower right lobe, while, in one, it was in the lower left lobe. The operations were right (1) and left (1) lower lobectomies and right lower and middle bilobectomy due to middle lobe involvement. Two had a satisfactory evolution, while one presented a pleural empyema healed with antibiotic treatment. Conclusions: Pulmonary sequestration is rare and their symptoms are usually associated with pulmonary infection. In most cases, a lobectomy is necessary. The postoperative evolution is usually good(AU)

A Young Woman With Recurrent Episodes of Fever and Cough.

CASE PRESENTATION: A 19-year-old woman presented to pulmonary clinic with recurrent episodes of fevers and productive cough over the last 2 years. She was diagnosed with several episodes of respiratory infection that required antibiotic therapy. Her symptoms improved transiently after antibiotic therapy. However, symptoms continued to recur every 1 to 2 months. She denied any close TB contacts or travel outside the United States. She was a nonsmoker and had no history of immunodeficiency. There was no history of cystic fibrosis or any foreign body aspiration.

Secuestro pulmonar: caracterización y tratamiento en pacientes adultos y pediátricos / Pulmonary sequestration: characteristics and treatment in adult and pediatric patients

Resumen Introducción: El secuestro pulmonar (SP) es una malformación congénita caracterizada por tejido pulmonar con vascularización de una arteria sistémica anómala. Objetivo: Analizar las características y tratamiento de pacientes adultos y pediátricos con secuestro pulmonar. Materiales y Método: Estudio descriptivo transversal. Periodo: enero de 1988 a diciembre de 2018. La información se obtuvo de fichas clínicas y registros de anatomía patológica. Se describen edad, sexo, características clínicas, diagnóstico, tratamiento quirúrgico y hallazgos anatomopatológicos. Se realizó análisis estadístico mediante SPSS25® y se usó la prueba Mann-Whitney y X2, considerándose significativo p < 0,05. Resultados: Total 33 pacientes, 25 (75,8%) mujeres. Edad promedio 30,2 años, rango: 0-68. Adultos 23 (69,7%) pacientes y pediátricos (< 15 años) 10 (30,3%) pacientes. La presentación clínica fue sintomatología pulmonar en 23 (69,7%) casos y 9 (27,3%) eran asintomáticos. Tres (9,1%) presentaron malformación congénita asociada. Diagnóstico preoperatorio en 15 (45,5%) pacientes. La ubicación más frecuente fue lóbulo inferior izquierdo. El tipo intralobar fue el más frecuente en 23 (69,7%) casos. La cirugía más frecuente fue la lobectomía con identificación y ligadura del vaso sistémico. El vaso aberrante se originó en aorta torácica en 27 (81,8%) casos e infradiafragmático (no precisado) en 3 (9,1%) casos. Vaso único en 26 (78,8%) y doble en 5 (15,2%) casos. No hubo mortalidad. Existen diferencias en las características entre los secuestros en pacientes adultos y pediátricos. Discusión y Conclusión: Los SP son infrecuentes, se presentan principalmente en adultos jóvenes como neumopatías a repetición, se distinguen diferencias en las características entre los pacientes adultos y pediátricos, y tienen excelente pronóstico posoperatorio.

Background: Pulmonary sequestration (PS) is a congenital malformation characterized by lung tissue with vascularization from anomalous systemic arteries. Aim: To analyze characteristics and treatment of adult and pediatric patients with pulmonary sequestration. Materials and Method: Transversal descriptive study. Period: January-1988 to December-2018. Information was obtained from clinical files and pathological anatomy records. Age, sex, clinical characteristics, diagnosis, surgical treatment and pathological findings are described. Statistical analysis was performed using SPSS25® and the Mann-Whitney and Chi square test were used, considering p < 0.05 to be significant. Results: Total 33 patients, 25 (75.8%) women. Average age 30.2 years, range: 0-68. Adults 23 (69.7%) patients and pediatric (< 15 years) 10 (30.3%) patients. The clinical presentation was pulmonary symptoms in 23 (69.7%) cases and 9 (27.3%) were asymptomatic. Three (9.1%) presented another congenital malformation. Preoperative diagnosis in 15 (48.4%) patients. The most frequent location was the left lower lobe. The intralobar type was the most frequent: 23 (69.7%) cases. The most frequent surgery was lobectomy with identification and ligation of the systemic vessel. The systemic vessel originated in the thoracic aorta in 27 (81.8%) cases and infradiaphragmatic (not specified) in 3 (9.1%) cases. Single vessel in 26 (78.8%) and double in 5 (15.2%) cases. There was no mortality. Differences were found in characteristics between adult and pediatric patients. Conclusion: SP are infrequent, they mostly appear in young adults as recurrent lung diseases, differences in characteristics are distinguished between adult and pediatric patients and they have an excellent postoperative prognosis.

Pulmonary sequestration with Aspergillus infection presenting as massive hemoptysis and hemothorax with highly elevated carcinoembryonic antigen in pleural effusion that mimics advanced lung malignancy.

BACKGROUND: Pulmonary sequestration (PS) associated with massive hemoptysis, hemothorax, and elevated tumor markers or even lung malignancy has been reported in several studies. These clinical features combined with lung lesions on chest imaging are sometimes hard to differentiate from lung malignancies and often complicate the diagnostic procedure. CASE PRESENTATION: A 45-year-old man with PS presented with massive hemoptysis, hemothorax, and extremely elevated carcinoembryonic antigen (CEA) in pleural effusion was initially misdiagnosed with advanced lung carcinoma, but was ultimately diagnosed with PS with Aspergillus infection. CONCLUSIONS: PS is rarely concurrent with lung cancer; most of the time, it is misdiagnosed as a malignancy, especially when presenting with a fungal infection, which could remarkably elevate CEA in pleural effusion.