Pulmonary Sequestration Research Articles

Intralobar pulmonary sequestration in an adult: a case report

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

A Case of Intralobar Pulmonary Sequestration Diagnosed Incidentally in a Geriatric Male

Pulmonary sequestration is a rare congenital malformation of lung in which a part of the lung is supplied by an anomalous systemic blood supply from thoracic or abdominal aorta. This is a nonfunctioning lung tissue and lacks normal communication with tracheobronchial tree. It develops from accessory lung bud. If developed before pleura formation, it results in intralobar sequestration, and extralobar, if it develops after pleura formation. Usually Intralobar type of pulmonary sequestration presents in late childhood or adolescence with recurrent pulmonary infections. It is less commonly associated with foregut communication or associated anomalies, unlike the latter. Extra lobar type usually presents with respiratory distress while recurrent infections are less common. In the present case report a 76-year-old male patient was diagnosed with intralobar pulmonary sequestration. This patient was presented with recurrent respiratory infections with complaints of fever and cough with expectoration for one week. Previously the patient was misdiagnosed with recurrent pneumonia. Despite the age of patient, consideration of pulmonary sequestration as a differential diagnosis lead to investigations in that direction. This helped in prompt diagnosis and further plan of management. Misdiagnosis of this condition may have lead to development of complications like recurrent infections and abscess. These complications can be avoided by prompt surgical excisions which are curative. This case report was unique because it diagnosed pulmonary sequestration in a 76 years old patient who presented with recurrent pneumonia.

The prognosis of type 3 congenital pulmonary airway malformations may not be poor: Antenatal sonographic evaluation and postnatal outcomes of non-cardiac thoracic malformations in a large cohort

Background: To analyze the prenatal sonographic findings, prevalence, and outcomes of non-cardiac thoracic malformations (NCTMs).Method: This study included a retrospective analysis of electronic health records from February 2014 to March 2021. The follow-up sonographic examinations of the fetuses with diagnoses of NCTM and postnatal clinical follow-up were retrieved from electronically stored files and reports. Results: Out of 19,640 fetuses, 25 fetuses were found to have NCTM with a prevalence of 0.12%. The most common NCTM was congenital pulmonary airway malformation (CPAM) (n=8, 32%), followed by pleural effusion (PE), pulmonary hypoplasia (PH), and congenital diaphragmatic hernia (CDH) (4 cases each, 16%), bronchopulmonary sequestration (BPS) (n=3, 12%), congenital lymphangioma (CL), and pectus excavatum (PEx) (1 case each, 4%). All type 3 CPAM cases and BPS cases resolved spontaneously in utero, whereas all type 2 CPAM (n=2) cases required surgery in the neonatal period and one of the type 1 cases resulted in fetal hydrops. All PE, PEx, and PH cases, and 25% of the CDH cases (n=1) were associated with underlying abnormalities. All cases with PH (n=4), all PE cases associated with hydrops (n=2, 50%) resulted in exitus in utero.Conclusion: NCTMs are rare congenital anomalies with a prevalence of 0.12%. The most common type of NCTM was found as CPAM. All cases of type 3 CPAM and BPS showed spontaneous resolution in utero. The prognosis of type 3 CPAM may be favorable.

Adult congenital horseshoe lung with bilateral pulmonary sequestration: A case report

Horseshoe lung (HL) is an infrequent congenital lung anomaly. Its main feature is that the lower lungs on both sides extend behind the pericardium and fuse across the midline, usually accompanied by pulmonary dysplasia. It is reported that 80% of HL is relevant to the abnormal return of some pulmonary veins from the right lung to the inferior vena cava or right atrium (scimitar syndrome). Most patients are within 5years old, most commonly within 1 year old, but HL may also have no apparent clinical symptoms or mild symptoms. This case is a 36-years-old adult female who developed left chest pain more than a month ago and continued to worsen for 10days. The patient also had repeated pulmonary infection with cough and expectoration.

Fatal pulmonary sequestration supplied by the pulmonary artery in a neonate..

Fatal pulmonary sequestration supplied by the pulmonary artery in a neonate..

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

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. Patients with ILS who received treatment between December 2010 and2020 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. 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. 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.

Can we predict the need for postnatal surgery in patients with prenatal fetal lung masses detected by CVR value?

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

Congenital Lung Malformations: Clinical and Functional Respiratory Outcomes after Surgery.

Congenital lung malformations (CLMs) involve anomalies of the lungs and respiratory tree such as congenital pulmonary airway malformation (CPAM), pulmonary sequestration (PS), bronchogenic cysts, congenital lobar emphysema, and bronchial atresia (BA). Although symptomatic lesions require surgical resection, the appropriateness of surgery for patients with asymptomatic malformations is a matter of ongoing debate. Limited data are available concerning the long-term follow-up of affected subjects. In this study, we sought to evaluate the long-term clinical and functional respiratory outcomes in children with CLMs who underwent surgical resection. We carried out a retrospective analysis of 77 children with CLMs who underwent pulmonary resection with at least one year of follow-up. The most common diagnoses were CPAM (50.65%), hybrid lesions (25.97%), lobar emphysema (11.69%), and PS (5.19%). The most common surgical approaches were lobectomy (61.3%), segmentectomy (10.7%), and pneumonectomy (5.3%). Acute post-surgery complications occurred in 31.2% of children. In addition, 73.7% experienced long-term complications, and we found no correlation between the presence of these complications and the sex of the patients, their age at time of surgery, the type of surgery undergone, the presence of symptoms prior to intervention, or acute complications after surgery. Pulmonary function tests revealed FEV1 Z-scores of &lt;-2 SDs in 16 patients, and we found a significant correlation between pneumonectomy and the development of lung function deficit (p = 0.031). In conclusion, clinical and functional respiratory complications may occur in children with CLMs who undergo surgical resection. Long-term monitoring is needed to improve the management of asymptomatic patients.

Congenital Diaphragmatic Hernia, Pulmonary Adenomatoid Malformation, Sequestration, and Lobar Emphysema in Pediatric Emergency Care.

Hon, Kam Lun Ellis MBBS, MD, FAAP, FCCM; Tan, Yok Weng BSc (MedGen), MUDr, MRCPCH; Leung, Karen K. Y. MBBS, MSc, MRCPCH; Hui, Wun Fung MBChB, MRCPCH; Cheung, Wing Lum MBBS, MRCPCH; Chung, Fung Shan MBChB, MRCPCH; Ku, Shu Wing MBBS, MRCP, FANZCA, FCICM; Fung, Genevieve P. G. MBBChir, MSc, MRCPCH Author Information

An unusual case of extrapulmonary sequestration presenting as vomiting in a neonate

Pulmonary sequestration is a rare, mostly sporadic disorder, with an incidence of 0.1–6% of all congenital lung malformations. It is also called as bronchopulmonary foregut malformation (BPFM) or accessory lung. A 16-day-old female child presented with vomiting and inability to take feeds. She was investigated further and differentials included foregut duplication cyst and BPFM. Surgical exploration confirmed the diagnosis of a BPFM. Delay in diagnosis may lead to complications such as torsion and sepsis.

Intralobar Pulmonary Sequestration Presenting as Chronic Nonproductive Cough

We report a case of a 60-year-old female who was known to have intralobar pulmonary sequestration and her only symptom was chronic cough. She had no history of infections and surgical resection led to complete resolution of her chronic cough.

Congenital lung malformations: Dysregulated lung developmental processes and altered signaling pathways

Congenital lung malformations comprise a diverse group of anomalies including congenital pulmonary airway malformation (CPAM, previously known as congenital cystic adenomatoid malformation or CCAM), bronchopulmonary sequestration (BPS), congenital lobar emphysema (CLE), bronchogenic cysts, and hybrid lesions. Little is known about the signaling pathways that underlie the pathophysiology of these lesions and the processes that may promote their malignant transformation. In the last decade, the use of transgenic/knockout animal models and the implementation of next generation sequencing on surgical lung specimens have increased our knowledge on the pathophysiology of these lesions. Herein, we provide an overview of normal lung development in humans and rodents, and we discuss the current state of knowledge on the pathophysiology and molecular pathways that are altered in each congenital lung malformation.

Hospital Volume and Postoperative Outcomes after Elective Lung Resection in Infants with Congenital Lung Malformations

INTRODUCTION: The relationship between hospital volume and patient outcomes after pediatric lung resection remains unknown. This study evaluated the association between hospital volume and postoperative outcomes in infants undergoing elective lung resection for congenital lung malformations (CLM). METHODS: After IRB approval, infants >30 days of age who underwent elective CLM resection were identified in the Pediatric Health Information System database (2016 to 2020). Those with previous symptomatic disease were excluded. Annual lung resection tertiles were used to classify hospitals into low (1 to 7 cases), medium (8 to 11 cases), or high (>11 cases) volume centers. Primary outcomes were hospital length of stay (LOS) and postoperative complications. Linear or Poisson multivariable regressions were performed. RESULTS: There were 1,420 infants from 48 hospitals. CLMs included congenital pulmonary airway malformations (n = 623, 43.9%) and bronchopulmonary sequestrations (n = 364, 25.6%). The mean annual cases at low-, medium-, and high-volume hospitals were 4.6 ± 1.6, 8.9 ± 1.2, and 36.4 ± 23.6, respectively. The median LOS was 2 days (interquartile range 2 to 3), and there were no differences based on center volume (p = 0.139). Overall postoperative complications were significantly increased at low-volume centers (31.1%) compared with medium-volume (16%) and high-volume (25%) centers (p < 0.001; Table 1). Respiratory complications were significantly increased in low-volume centers (28.8%) compared with medium-volume (14%) and high-volume (23%) centers (p < 0.001). Adjusted odd ratios showed a 51% reduction in overall complications (95% CI 0.37 to 0.65, p < 0.001) at medium/high-volume centers. Table 1. - Patient Outcomes by Center Volume Variable Low (n =89 patients) Medium (n = 534 patients) High (n = 497 patients) p Value Operative approach Thoracoscopic, n (%) 295 (75.8) 403 (75.5) 248 (49.9) <0.001 Open, n (%) 121 (31.1) 83 (15.5) 124 (24.9) <0.001 Respiratory complications, n (%) 112 (28.8) 73 (13.7) 112 (22.5) <0.001 Postoperative ventilation, n (%) 25 (6.4) 32 (6.0) 15 (3.0) 0.034 Length of stay (d), median (IQR) 2.00 (2–4) 2.00 (2–3) 2.00 (2–3) 0.139 Mortality, n (%) 1 (0.3) 1 (0.2) 0 (0) 0.7366 30-day readmission, n (%) 22 (5.7) 16 (3.0) 14 (2.8) 0.062 CONCLUSION: For elective infant lung resections, these data suggest that low-volume hospitals are associated with increased postoperative complications. Further study of CLM outcomes, including the role of surgeon-specific volume, is warranted.

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

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

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.

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

CYSTIC ADENOMATOID MALFORMATION OF THE LUNG IN AN ADULT: A CASE REPORT

“ROLE OF FETAL MRI IN EVALUATION OF CONGENITAL ANOMALIES”

When US evaluation of the second and third-trimester foetus is limited, MR imaging might be used as a supplemental test. Due to a lack of an acceptable acoustic window, US is often limited in cases of oligohydramnios or breech presentation. In both cases, however, MR imaging can clearly show the foetal anatomy in detail. Ossification of the calvaria[1], which inhibits view of the posterior fossa at US, can also impede third trimester foetal assessment. US frequently suggests intracranial pathologic abnormalities such as posterior fossa anomalies (Chiari II syndrome, cerebellar aplasia or hypoplasia). MR imaging can corroborate the US findings, and it can also provide a diagnosis in some circumstances, such as neuronal migrational anomalies. MR imaging certainly has the advantage of being able to analyse the foetus in many planes within a greater field of view. T1-weighted MR imaging can show acute and chronic germinal matrix bleeding, as well as ischemia alterations. Although ultrasound is helpful in detecting corpus callosum abnormalities, it can miss many of the central nervous system problems associated with corpus callosum agenesis. Because the prognosis of agenesis of the corpus callosum is so unpredictable, detecting concomitant central system defects on MR imaging can help with prenatal and postnatal medical care. Although the majority of prenatal MR imaging applications are related to the cranial nervous system, new developments in foetal surgical intervention have expanded the utility of foetal MR imaging in thoracoabdominal illness detection. Fetal MR imaging can help diagnose abnormalities such congenital cystic adenomatoid malformation, congenital diaphragmatic hernia, cystic hygroma, and bronchopulmonary sequestration. Accurate assessment of foetal lung and liver sizes is critical in foetal surgical planning and postnatal prognosis in the case of congenital diaphragmatic hernia. Some high-risk pregnancies may also benefit from obstetric MR imaging, according to preliminary research. With a combination of foetal weight estimates and liver volume measurements, birth weight may be correctly predicted and the diagnosis of intrauterine growth retardation or macrosomia can be made with confidence.

Indocyanine Green to Visualize and Resect Pulmonary Sequestration in Adults

Pulmonary sequestration (PS) is a rare congenital pulmonary dysplasia which presents with recurrent chest infections and haemoptysis. Its diagnosis is difficult and suspected on contrast-enhanced chest CT. The definitive treatment of pulmonary sequestration is surgical resection or endoluminal occlusion of the abnormal feeding vessel. Here, we report an innovative technique in which per-operative indocyanine green guided excision of intra-lobar sequestrated lung parenchyma and ligation of abnormal feeding vessel. The first patient was a 44-year-old gentleman with recurrent bouts of cough for 2 years. Courses of antibiotics failed to resolve it. Fibreoptic bronchoscopy and routine blood tests did not show any abnormality. CECT chest showed multiloculated cystic solid and low density mass in the right lower lung. The area of the lesion received its blood supply from anomalous systemic artery originating from coeliac trunk. Intraoperative, indocyanine green was injected into the atypical vessel, and pulmonary sequestration turned green. A sequential atypical lung resection could be completely removed with parenchyma preservation. The second patient was a 53-year-old lady presenting with chest pain, cough and expectoration for 2 years not responding to general management and antibiotics. CECT showed a low-density mass in the right lower lung lobe, with a feeding arterial vessel arising from the abdominal aorta. This was diagnosed as PS and resected using per-operative indocyanine green with parenchymal preservation and ligation of feeding vessel. Both patients have been followed up for at least 1 year and are symptom free. The intraoperative use of indocyanine green could greatly facilitate the presentation of the extent of a lung sequestration and safe resection.

Short- and mid-term outcome of transcatheter embolization of pulmonary sequestration in children: A single-center 22-years experience

A pulmonary sequestration (PS) is a non-functioning bronchopulmonary tissue, not connected to the tracheobronchial tree and receiving arterial supply from the systemic circulation. Recently, transcatheter embolization of the feeding arteries has been reported as an effective and minimally invasive option but large series on outcomes in children are scarce. We aimed to evaluate the efficacy of endovascular embolization of PS and report short- and mid-term outcomes. Single-center retrospective clinical data review of all children referred to Cath lab at M3C-Necker-Enfants–Malades hospital for PS between Jan-1999 and May-2021. Demographic, clinical and procedural data were collected at baseline and at follow-up. Seventy-one patients were included. Median age and weight were 4.9 months (IQR: 2.1–26.6) and 4.2 kg (IQR: 3.9–12.1) respectively. Sixty-one (86%) patients had CHD, of which 47 had scimitar syndrome. Fifty-three (74.7%) patients had transcatheter embolization of PS. At baseline, 36 (68%) patients had PHT. One (1.4%) procedural death was reported. Two (2.8%) patients had PS surgical ligation that was too large for embolization. No patient needed PS surgical resection. Median follow-up was 36.4 months (IQR: 2.1–89.9) without residual shunt. Eight (11.3%) patients died with a median delay of 2.2 (IQR: 1.3–14.4) months after the procedure. Death was related to complex CHD. Thirty-one (86.1%) patients from those who had PHT became (PHT-Free) at last FU. Endovascular treatment of PS in children is safe and effective therapeutic option with very good short- and mid-term results leading to improvement in survival rates and to significant decrease in pulmonary hypertension.

Patent Ductus Arteriosus Can Coexist With Pulmonary Sequestration in Adults, Conditioning Pulmonary Hypertension

A woman in her early 40s attended the hospital for dyspnea. Her chest radiograph revealed cardiomegaly, prominence of the cone of the pulmonary artery, and hyperflow (Figure A). The electrocardiogram revealed growth of the atrium and right ventricle. The chest computed tomography angiogram depicted right intralobar pulmonary sequestration (PS) with a patent ductus arteriosus (Figure B); the blood supply of the sequestrum originated from the abdominal aorta with venous drainage toward the pulmonary vein and the left atrium (Figure C and D).