Extralobar Pulmonary Sequestration Research Articles

Recognition of an extralobar pulmonary sequestration during lung resection

BackgroundExtralobar 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 presentationThis 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.ConclusionsCT 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.

Bilateral extralobar pulmonary sequestration.

Bilateral extralobar pulmonary sequestration.

Extralobar pulmonary sequestration in an adult male with a heart defect. Case report

Pulmonary sequestration (PS) is a rare congenital malformation of the lungs referring to an abnormal (non-functioning) segment of the lung that has no connection with the tracheobronchial tree, which receives arterial blood from systemic circulation and has various types of venous outflow. PS is divided into intralobar (intrapulmonary) and extralobar (extrapulmonary) types. The distinctive feature of extralobar pulmonary sequestration is the presence of its own visceral pleura. PS is most common in pediatric practice; in some cases, this disease may remain oligosymptomatic or asymptomatic in children and adolescents. Adults with PS have a higher risk of recurrent pneumonia, chest pain, and episodes of hemoptysis. Cases of the late onset of PS are reported in a few foreign and Russian publications. The special feature was the elderly patients with a long asymptomatic course of PS, "pseudopneumonic" clinical onset, episodes of thoracalgia and hemoptysis. We present a case of an adult male soldier with PS clinically simulated a respiratory infection in the lower lobe of the left lung with severe pain and hemoptysis. In addition, the patient has a concomitant congenital heart defect.

Extralobar pulmonary sequestration: A case report and literature review

Key Clinical MessagePulmonary sequestration is a congenital malformation of lung development in which part of the lung tissue is separated from the normal lung during the embryonic phase and develops separately and receives blood supply from an aberrant systemic artery forming a nonrespiratory mass. In brief, early in embryonic development, certain tissues that should have atrophied and been gradually absorbed are left behind due to impairment of the atrophy process and form anomalous branches of the aorta, which pull parts of the lung tissue, isolating them from normal lung tissue and bronchi, and thus forming separate lung tissue. According to the relationship of the mass to the pleural covering, pulmonary sequestration can be divided into two types, intralobar pulmonary sequestration (ILS) and extralobar pulmonary sequestration (ELS), of which approximately 75% of cases are ILS, but ELS is less common. Symptoms are not obvious in either type, making diagnosis and differential diagnosis more difficult. Here we report a 33‐year‐old patient with only insignificant abdominal distension who was eventually diagnosed with retroperitoneal ELS.

The complexities in diagnosing intradiaphragmatic extrapulmonary sequestration: a case report

BackgroundExtralobar pulmonary sequestration–a congenital lung malformation characterized by nonfunctional lung tissue with its own visceral pleura and without a connection to the normal tracheobronchial tree–is often surgically resected given the potential for infectious complications. We report a case of a child with extralobar pulmonary sequestration in a rare and challenging intradiaphragmatic location, which made preoperative identification and planning difficult.Case presentationA 2-year-old boy presented for follow-up of a large left extralobar pulmonary sequestration initially diagnosed on an antenatal ultrasound. Follow-up imaging, including prenatal MRI and postnatal CT angiography, was inconclusive on the precise location of the extralobar pulmonary sequestration in relation to the diaphragm but did reveal a subdiaphragmatic arterial supply from a branch off the celiac trunk. Planned resection with diagnostic thoracoscopy revealed the mass to seemingly be below the diaphragm; however, subsequent abdominal laparoscopy identified it to be within the diaphragm. Once the supplying vessel off the celiac trunk was controlled and divided, the mass was circumferentially excised from the edges of the diaphragmatic muscle. The remaining diaphragmatic defect was then closed, and the patient did well postoperatively.ConclusionsThis case demonstrates the difficulty of making a definitive diagnosis of intradiaphragmatic extralobar pulmonary sequestration without operative intervention. CT angiography and identification of the sequestration’s arterial supply may not be conclusive in determining the precise location if in close proximity to the diaphragm.

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

Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. 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. 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. 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.

Uniportal video-assisted thoracoscopic surgery for pediatric cases.

The potential advantages of video-assisted thoracoscopic surgery (VATS) for children include better cosmetic outcomes and reduced risk of postoperative musculoskeletal deformities. The uniportal approach is expected to promote minimally invasive surgery and help reduce the incidence of postoperative musculoskeletal deformities. Uniportal VATS was performed safely in three children (mean age of 23.3months) with congenital pulmonary airway malformation or extralobar pulmonary sequestration. Our findings suggest that minimally invasive lobectomy may be achieved through uniportal VATS in children.

Imaging and pathological characteristics, treatment, and prognosis of pulmonary sequestration-A retrospective study of 13 cases.

This study aimed to summarize and analyze the characteristics of pulmonary sequestration to improve our understanding of this disease. 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. 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. 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.

Extralobar pulmonary sequestration of the azygos lobe: report of a case of unusual location

Extralobar pulmonary sequestration of the azygos lobe: report of a case of unusual location

A case of adult extralobar pulmonary sequestration showing continuity with pulmonary artery and vein

症例は50歳女性．当科受診の11年前に偶発的に左肺腫瘤を指摘されるも，陰影が葉間にあり，局在が変化していたため胸水と診断されていた．今回，健診胸部単純X線で異常陰影を指摘され，胸部CTで左肺舌区に石灰化を伴う腫瘤影を認め，左肺腫瘍疑いとして当科紹介となった．病変の局在が変化することから孤立性線維性腫瘍を疑い，手術を施行した．腫瘤は肺門部を根部とする有茎性病変で，正常肺との連続性はなく，肺静脈および肺動脈と交通する血管を1本ずつ認めた．これらの異常血管を一括して切離し，病変を摘出した．摘出病変は一部に肺実質を認め，手術所見と合わせて肺葉外肺分画症と診断した．流入動脈が肺動脈，還流静脈が肺静脈の肺葉外肺分画症は極めて稀であり，報告する．

Infra-diaphragmatic extralobar pulmonary sequestration: Radiologic features

Infra-diaphragmatic extralobar pulmonary sequestration: Radiologic features

Extralobar Pulmonary Sequestration.

Male, 71 year-old, asymptomatic, former smoker and previous history of lung tuberculosis. Referred to outpatient clinic due to left lower lobe consolidation diagnosed on non-contrasted CT scan, with increased uptake on PET-CT. Then, a contrast-enhanced CT scan revealed extralobar pulmonary sequestration with venous drainage to the left azygos vein (Blue arrow) and a double branch arterial supply from the thoracic aorta (Red arrow).

Comparison of clinical outcomes of pulmonary sequestration in adults between surgery and non-surgery groups.

Pulmonary sequestration (PS) is a rare congenital lung malformation that can be incidentally diagnosed in adulthood. The natural course of PS in adults is scarcely known. In this retrospective cohort study, medical records and imaging results of adult patients diagnosed with PS between 1994 and 2019 were reviewed. Diagnoses of PS were confirmed by histopathological findings in resected cases, while non-resected cases were diagnosed based on the presence of anomalous systemic arterial supply and abnormal lung parenchyma on enhanced chest computed tomography (CT). Among 104 patients with PS, the median age at diagnosis was 40.5 years, and 69 (66.3%) patients were asymptomatic. Patients in the surgery group were significantly younger (38.6 vs. 45.3 years, respectively, P=0.016), were more likely to be symptomatic initially (51.6% vs. 28.6%, respectively, P=0.015), and had larger PS (90.0 vs. 66.3 mm, respectively, P<0.001) than the non-surgery group. Of the patients in the surgery group, 29.0% (18/62) experienced postoperative complications. In the surgically resected cases, infections were only detected in intralobar PS, not in extralobar PS. Among 25 subjects without initial symptoms in the non-surgery group, 24 (96.0%) remained asymptomatic at the last follow-up. Adults with PS tended to undergo resection if they were young, symptomatic, and had large PS (a median diameter of 90.0 mm). Almost all subjects who were initially asymptomatic and did not undergo surgery remained asymptomatic at the last follow-up. Therefore, considering the indolent course of PS, initially asymptomatic adults with PS could be followed up without surgery.

Intradiaphragmatic extralobar pulmonary sequestration: A rare anomaly

We present a case of intradiaphragmatic extralobar pulmonary sequestration (IDEPS) prenatally diagnosed via ultrasound and MRI. Originally seen in the second trimester, the lesion was followed with serial imaging pre and postnatally until being resected thorascopically at 2 years of age.

Ex Utero intrapartum treatment of extralobar pulmonary sequestration: case report and literature review

Sofija Jackevičiūtė1, Jelena Volochovič1,2 1 Vilnius University, Faculty of Medicine, M. K. Čiurlionio street 21, Vilnius, Lithuania 2 Vilnius University hospital, Santaros clinic, Obstetrics, and Gynecology Center, Santariškių street 2, Vilnius, Lithuania Abstract Background: Extralobar pulmonary sequestration is an uncommon congenital lung lesion, very rarely causing severe symptoms prenatally or requiring prompt surgical actions taken. The purpose of this report is to present a unique symptomatic case of extralobar pulmonary sequestration which led to an Ex Utero intrapartum treatment being performed for the first time in Lithuania and followed with successful further treatment of pulmonary pathology. Case presentation: A nulliparous caucasian patient at 22 years of age was sent to a tertiary level clinic after alarming results were found during routine ultrasound examination at 29+4 weeks of gestation. After a detailed examination, extralobar pulmonary sequestration was diagnosed. Due to severely worsening fetal condition on week 32 of gestation the pregnancy was no longer continued and an Ex Utero intrapartum treatment procedure was performed. The pulmonary pathology later was surgically resected at 6 months of age. Conclusion: This rare case of extralobar pulmonary sequestration allows us to explore key moments in the diagnostic and treatment pathways of this pathology. Magnetic resonance imaging, which allows evaluating parous patients more precisely than ultrasound imaging, with fetal pulmonary vessels being seen more clearly, is inspected as a necessary step in fetal lung sequestration diagnosis establishment. The Ex Utero intrapartum treatment procedure and how anticipated respiratory challenges of the newborn can be tackled with its implementation leading to timely execution of crucial interventions is researched. Keywords: intrapartum, treatment, procedures.

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.

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

BackgroundExtralobar 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 presentationThere 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.ConclusionsTorsed 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.

Acute haemothorax and pleuropulmonary blastoma: Two extremely rare complications of extralobar pulmonary sequestration

Extralobar bronchopulmonary sequestration (eBPS) is a congenital lung malformation characterized by non-functional lung tissue with no connection with the bronchial tree, receiving systemic blood supply and covered by its own visceral pleura. It is mostly asymptomatic, however it can rarely get complicated with infections and neoplastic degeneration. Its management is still debated: many authors suggest observation for asymptomatic cases and intervention only in case of symptoms, others advocate surgery even in asymptomatic patients. Pleuropulmonary blastoma (PPB) is a rare malignant sarcomatous neoplasm, representing the most common primary lung malignancy in children. Typically, PPB is associated with congenital pulmonary airway malformation (CPAM). eBPS is rarely associated with PPB. We report a case of a previously healthy three-year-old boy presented to our emergency room with massive hemothorax after a low-energy chest trauma. Imaging revealed active thoracic bleeding by an anomalous artery arising from the celiac tripod, and an irregular mass in the lower right hemithorax. After performing biopsy, pathologic examination revealed a PPB. The patient underwent four cycles of chemotherapy before surgery. The operative findings revealed a right supradiaphragmatic extralobar mass, with no communication with the bronchial tree, strictly adherent to the right middle lobe. After mass excision, histological analysis confirmed the diagnosis of PPB type 2. The patient underwent postoperative chemotherapy. No recurrence nor metastasis were noted during the current follow-up. DICER-1 research revealed biallelic mutation on the tumor and on the surrounding normal tissue. We reviewed the literature concerning PPB and BPS. Based on these data, we would recommend intervention even in case of asymptomatic eBPS.

Ten cases of intradiaphragmatic extralobar pulmonary sequestration: a single-center experience

BackgroundIntradiaphragmatic extralobar pulmonary sequestration (IDEPS) is a rare type of pulmonary sequestration (PS). The purpose of this study is to assess diagnosis and operative treatment of IDEPS.MethodsPatients with PS who...

Videothoracoscopic segmentectomy in extralobar pulmonary sequestration: safe and effective procedure

Videothoracoscopic segmentectomy in extralobar pulmonary sequestration: safe and effective procedure