Pulmonary Sequestration Research Articles

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.

Pediatric Thoracoscopy: Ten Years Experience in Tertiary Hospital

Purpose: A minimally invasive Thoracoscopic Surgery (TS) offers several options in diagnosis and surgical treatment in pediatric surgery. This study evaluates experience and outcome of thoracoscopic surgery in pediatrics during 10 years period in tertiary hospital. Patients and methods: This retrospective study conducted at King Faisal Hospital and Research Center, Jeddah, Saudi Arabia from 2012 to 2022. Data of 43 patients who underwent TS were enrolled. Data include patient’s demographic characteristics, co-morbidity, diagnosis, type of thoracoscopic procedures, time of intervention, length of hospital stay, post-operative complications. Results: Mean age of patients was 4.99 years. Females were more than males (55.8 vs. 44.2%). Sixteen (37.2%) patients had comorbidities. Thoracoscopic proce- dures were feasible in 43 children and adolescents with chest diseases including lung empyema (20.9%), congenital lobar emphysema (14.0%), interstitial lung disease (14.0%), congenital pulmonary airway malformation (11.6%), mediastinal mass (9.3%), lung metastasis (11.6%), lymphoma (4.7%), chronic inflamma- tions (7.0%), hemothorax (2.3%), bronchogenic cyst (2.3%), pleural effusion (2.3%) and pulmonary sequestration and right diaphragmatic hernia (2.3%). Proce- dures were made mostly for lung biopsy (25.0%), lobectomy (22.7%), decortication (20.9%), mediastinum mass resection (6.8%), lung metastasectomy (6.8%), lymph node biopsy (4.5%), diagnostic (4.6%), cyst excision (4.6%), diaphragmatic hernia repair (2.3%) and thymectomy (2.3%). Duration of hospital stay ranged from 2-180 days. All of the cases had chest drain (100.0%) and some of them required post-operative intubations (30.2%). Conversion rate to open was (4.7%). Complications were pneumothorax (7.0%), bleeding (2.3%) and air leak (2.3%). Conclusions: Thoracoscopic approach is a challenging method for diagnosis and treatment in pediatrics. However, complications were reported as pneumo- thorax, bleeding and air leak. In order to improve results, conversion rates, and the capacity to handle thoracoscopic complications, proper training is crucial.

Sublobar resection utilizing near-infrared thoracoscopy with intravenous indocyanine green for intralobar pulmonary sequestration: a case report and literature review

BackgroundPulmonary sequestration is a rare pulmonary malformation, with intralobar pulmonary sequestration being the most common subtype. Lobectomy has generally been performed for its treatment, owing to unclear boundaries of the lesion. However, recent reports have introduced lung resection using intravenous indocyanine green (ICG) as a treatment for pulmonary sequestrations.Case descriptionA 34-year-old woman presented with chest pain, and enhanced chest computed tomography (CT) displayed a solid mass of 4.5 × 3.1 cm in the right S10 area. An aberrant artery was found running from the celiac artery through the diaphragm to the thoracic cavity. The patient was diagnosed as having pulmonary sequestration Pryce type III, and surgical resection was performed. Intrathoracic findings demonstrated that the precise area of the pulmonary sequestration could not be clearly identified, and a 5-mm aberrant artery was present in the pulmonary ligament. Following the separation of the aberrant artery, intravenous injection of ICG clearly delineated the border between the normal lung tissue and the pulmonary sequestration. Wedge resection was then performed without any postoperative events, and the pathological diagnosis was also pulmonary sequestration.ConclusionsWe herein reported a case of a patient who underwent sublobar resection for intrapulmonary sequestration using intravenous ICG injection, together with a literature review. Our case suggests that a comprehensive understanding of abnormal vessels and pulmonary vasculature in pulmonary resection for intrapulmonary sequestrations, complemented with the use of ICG, might potentially avoid unnecessary pulmonary resection and enable sublobar surgical resection.

Computed Tomography Imaging Features of Pulmonary Sequestration

Background: Pulmonary sequestration (PS), generally diagnosed using computed tomography pulmonary angiography (CTPA), is a rare congenital developmental malformation of the lung that is characterized by nonfunctional lung tissue, independent of the normal lung tissue. This paper summarizes the imaging features of the supplying arteries and draining vessels in patients with PS with an aim to assist in timely clinical diagnosis and operation guidance. Materials and Methods: A total of 55 patients with PS diagnosed using CTPA from multiple clinical centers were retrospectively analyzed. Data included demographic characteristics, imaging features, disease location, isolation type, and the features of supplying and draining vessels, as shown on CTPA images. Results: Of the 55 patients reviewed, 3 (5.45%) were children, 3 (5.45%) were adolescents, and 49 (89.09%) were adults; the mean age was 44 years. Fifty-four (98.18%) patients had intralobar sequestration and one (1.82%) had extralobar sequestration. PS was noted 3.5 times more frequently in the left lower lobe than in the right lower lobe. For the supplying arteries, 47 (85.45%) were derived from the descending thoracic aorta, 1 (1.82%) from the abdominal aorta, 7 (12.73%) from the celiac axis, and 1 (1.82%) from the bronchial artery. The draining vessels were the pulmonary veins in 49 patients (89.09%), the umbilical vein in 1 (1.82%), the venae intercostal in 1 (1.82%), the pulmonary arteries in 11 (20.00%), and the vessels were not shown on the images in 2 patients (3.64%). Conclusion: Clinical presentations of PS are non-specific and can be easily missed or misdiagnosed. However, CTPA can help to improve the diagnostic accuracy and identify the supplying arteries and draining vessels, which significantly contribute to surgical planning.

Complete visualization using indocyanine green in thoracic surgery for pulmonary sequestration.

Preoperative three-dimensional computed tomography (CT) facilitates accurate identification of aberrant systemic arteries in thoracic surgery for pulmonary sequestration (PS). Furthermore, the boundary between normal and sequestrated lungs can be visualized using the spread of fluorescent indocyanine green (ICG) when performing surgery for PS. This study aimed to determine how to completely visualize anatomical variations, safely treat aberrant arteries, remove only sequestrated lungs, and perform minimally invasive surgery for PS. Seventeen patients underwent lung resection for intralobar PS at our institution between 2009 and 2022. We retrospectively reviewed the surgical outcomes and intraoperative images using ICG to assess the efficacy and feasibility of near-infrared fluorescence imaging. Since 2019, intraoperative near-infrared fluorescence imaging with ICG has been used in six patients, including four females and two males (median age, 56 years), to visualize the boundary between normal and sequestrated lungs. Aberrant arteries were identified using preoperative three-dimensional CT, and the boundary between sequestrated and normal lungs could be clearly delineated intraoperatively using ICG in all cases. The median operative time was 145 min (range, 88-167 min), and the median blood loss was 5 mL (range, 1-191 mL). The overlay mode using near-infrared thoracoscopy, which merges visible light images with fluorescent images, was safer and more useful than conventional thoracoscopy for delineating boundaries with electrocautery. No intraoperative or postoperative complications occurred. The median postoperative hospital stay was 5 days (range, 3-7 days). Intraoperative identification of the boundary between normal and sequestrated lungs using ICG was simple and feasible. We suggested that this technique was effective for lesion resection and normal lung preservation during surgery for intralobar PS.

EP03.04: Prenatal sonographic characteristics of fetal intra‐abdominal extralobal pulmonary sequestration correlated with perinatal outcomes

EP03.04: Prenatal sonographic characteristics of fetal intra‐abdominal extralobal pulmonary sequestration correlated with perinatal outcomes

An actual concept of pulmonary sequestration

Annotation. Pulmonary sequestration is one of the most common malformations of this organ in clinical practice. But there may be difficulties in interpreting of radiology studies, which leads to a violation of the methodology of the study in such patients, and consequently – the lack of success of the therapy and the correct routing.Purpose of the study. To present a literature review of current data related to lung sequestration, to discuss important clinical aspects of this abnormality and features of tactics. The review also describes the radiology patterns of sequestration with an emphasis on CT signs; typical images and methodological specificity of scanning in these patients are present and based on own practice experience.Materials and methods. Both “classical” and modern local and foreign scientific papers about lung sequestration in adults and children are considered with a description of the morphology of the lesion, the clinical symptoms and the actual CXR and CT imaging; modern features of surgical treatment of such patients are presented.Results. Often, lung sequestration manifests itself already in adulthood, without creating the impression of a congenital anomaly, hiding under the “masks” of abscessing pneumonia, neoplasm. The clinical course of lung sequestration is not specific and is characteristic of many respiratory complaints, but at the same time – the anomaly requires a different management tactics than ordinary inflammatory processes and is associated with the need to consult a thoracic surgeon. Due to the limited differentiation of the sequestration structure in CXR, in current time, if sequestration is suspected, CT with angiography is indicated. An aberrant vessel can trace not only from the thoracic, but also from the abdominal aorta, its branches.Conclusion. The correct interpretation of the revealed CT-signs allowed routing patients to a hospital with thoracic surgery department because in the majority of cases, to prevent repeated episodes of sequestr inflammation, it is necessary to remove it. The expansion of the scanning area below the diaphragm can be useful because some of the sequesters are supplied with blood from the abdominal aorta and its visceral branches – this will prevent repeated CT-angiography studies because information about the source of blood supply is extremely important for surgeons.

Pulmonary sequestrations

Congenital lung malformations account for 2.2 – 6.6% of all congenital abnormalities of the lungs. This is a pretty rare problem as compared to acquired lung diseases. Pulmonary sequestrations account for 0.15 – 1.8% in the incidence of all lung malformations, occupying the second place after the complex abnormalities called “lung agenesis-hypoplasia”. Pulmonary sequestrations are common in children and adolescents so one could encounter them in pediatric practice. But pulmonary sequestrations may be associated with insignificant symptoms or even be asymptomatic in selected pediatric cases. Thereby, adult general practitioners, pulmonologists, and thoracic surgeons may encounter such patients. The aim. Review had the purpose to inform the general practitioners, pulmonologists, and thoracic surgeons about pulmonary sequestrations and their diagnostic, clinics course, and treatment. Conclusion. There is a wide range of congenital abnormalities of human body. Pulmonary sequestrations are only a part of this huge problem. But modern medicine has a vast selection of methods for revealing and treating these conditions (including minimally invasive surgery).

Pre-natal diagnosis of pulmonary sequestration

Pulmonary sequestration (PS) consists of a rare lung malformation, characterized by a mass of non-functional lung tissue, which does not maintain normal communication with the tracheobronchial tree and whose blood supply is from an anomalous systemic artery. Its prenatal diagnosis is still rare, usually performed in centers of reference by trained professionals. The following is a case report of a prenatal diagnosis of pulmonary sequestration, correlating the pre and postnatal findings in different methods of diagnostic imaging.

Pulmonary Sequestration and Congenital Pulmonary Airway Malformation (CPAM) 1 Presented as A Left Suprarenal Mass:ACase Report

Introduction Pulmonary sequestration is a rare condition accounting for 0.1% to 6% of all congenital lung malformations. The correlation with Congenital Pulmonary Airway Malformation (CPAM) type 1 has not been adequately elucidated. Case presentation From routine fetal sonographic screening, a 20-day-old female baby's ultrasound revealed hydronephrosis with enlargement above the left kidney. Magnetic resonance imaging during a follow-up appointment uncovered a left suprarenal lesion of approximately 30 mm, which was not suspected to be adrenal neuroblastoma. After resection, histological analysis was consistent with pulmonary sequestration and confirmed the connection with CPAM type 1. Conclusion Surgery remains the only effective therapeutic choice for intra-abdominal pulmonary sequestration. The definitive diagnosis is confirmed through histological examination.

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.

A strange case of pleuritic pain in the third trimester of pregnancy.

Pulmonary sequestration is an uncommon congenital malformation of the lung, generally diagnosed in childhood or adolescence, corresponding to dysplastic lung tissue not communicating with the rest of vascular or bronchial lung system but receiving an arterial blood supply from systemic arteries. Currently, surgical resection is usually indicated in order to prevent or treat related symptoms or complications, although controversy exists regarding its use in asymptomatic patients and adults. We present the case of a 32-year-old pregnant woman with acute chest pain and vomiting diagnosed with intralobar sequestration at 32+2 weeks of gestation and treated with pulmonary lobectomy after giving birth by cesarean section at 33+0 weeks of gestation.

Intralobar bronchopulmonary sequestration: an example of the late onset of this rare congenital pathology

Bronchopulmonary sequestration (BPS) refers to a rare congenital lung malformation with a nonventilated dysplastic fragment of parenchyma separated from the main bronchial tree. This segment has a systemic blood supply through aberrant arteries and venous outflow into the systemic venous bed or pulmonary veins and has a common with the rest of the lung (intralobar BPS) or an independent (extralobar BPS) visceral pleura. Purpose of the study is to describe an unusual late onset of right-sided intralobar BPS characterized by a pseudopneumonic course in a patient with concomitant anomalies of the thoracic spine and diaphragm; to discuss the issues of formation, manifestation, diagnosis, differential diagnosis, complications, and treatment of intralobar BPS, and to consider its combination with other developmental anomalies. Conclusion. BLS should be considered in patients with an unusual course of pulmonary anomalies, especially localized in the basal segments of the left (more common) or right (less common) lung and characterized by abnormal blood supply and other malformations.

The diagnosis and surgical management of pulmonary sequestration in adults: a case series from a single centre in the UK

Pulmonary sequestration (PS) is a rare congenital malformation where extrapulmonary lung tissue receives systemic blood supply from an anomalous branch directly from the thoracic or abdominal aorta. Whilst non-malignant, it can often present with similar symptoms as lung cancer. We present a retrospective review of 8 consecutive adult patients undergoing surgical management for PS within a single centre in the UK. Of our cohort, 62.5% had never smoked. PS in the right lung was seen in 62.5% of cases. Anomalous branches of the pulmonary artery, pulmonary vein or coeliac axis supplied 37.5% of the PS seen in our cohort, and 12.5% did not have a radiologically identifiable blood supply. Techniques varied from thoracotomy (n = 4), video-assisted thoracoscopic surgery (VATS) (n = 3) to robotic resection (n = 1) with no intra-operative or post-operative complications reported within hospital. The mean length of stay was 2 days. The post-operative mortality rate was 12.5%; one patient had died following the robotic resection of the mass of pneumonia in the local district hospital 26 days post-operatively after being discharged. No other complications nor recurrence was recorded over the follow-up period. Where pulmonary masses receive blood supply from anomalous branches of the pulmonary vein and coeliac axis, diagnoses of PS should be considered. The clinical feasibility of discharge in 2 days with no symptom recurrence should undergo further investigation with a larger sample size.

CHIRURGIE DE LA SEQUESTRATION PULMONAIRE : ANALYSE ET RESULTATS DE 23 CAS

Pulmonarysequestrationisarare congenital non hereditarymalformation (0.15 to 6.4%). It can present in two forms intra andextralobare.Its clinical presentation is very variable, and its diagnosis is essentially based on CT scan with injection of product which shows the aberrant systemic artery. The treatment is essentially surgical with excellent results.Wereport in our studies 23 observations of pulmonary sequestration.

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.

Association of prenatal thoracic ultrasound abnormalities with copy number variants at a single Chinese tertiary center.

To systematically evaluate the association of prenatal thoracic ultrasound abnormalities with copy number variants (CNVs). Chromosomal microarray (CMA) data and clinical characteristics from fetuses with thoracic ultrasound abnormalities were retrieved and analyzed. Thoracic ultrasound findings were mainly isolated except for fetal pleural effusion (FPE) and pulmonary hypoplasia. The diagnostic yield of CMA for thoracic anomaly was 9.66%, and FPE (17/68, 25%), pulmonary hypoplasia (1/8, 12.5%), and congenital diaphragmatic hernia (CDH) (6/79, 7.59%) indicated relatively high pathogenic/likely pathogenic (P/LP) CNV findings. The detection rate for P/LP CNVs was obviously increased in non-isolated thoracic anomalies (27.91% vs. 1.96%, P < 0.0001), non-isolated FPE (37.78% vs. 0%, P = 0.0007) and non-isolated congenital pulmonary airway malformation (CPAM) (27.27% vs. 0%, P < 0.0001), and significantly different among thoracic anomalies. Additionally, the rate of termination of pregnancy in cases with non-isolated thoracic anomalies (58.49% vs. 12.34%, P < 0.0001) and P/LP CNVs (85.71% vs. 24.15%, P < 0.0001) was obviously increased. The present study expanded phenotype spectrums for particular recurrent CNVs. FPE, CDH, and pulmonary hypoplasia indicated relatively high P/LP CNV findings among common thoracic ultrasound abnormalities, CPAM associated with other ultrasound abnormalities increased the incidence of diagnostic CNVs, while bronchopulmonary sequestration might not be associated with positive CNVs. The present data recommended CMA application for cases with prenatal thoracic ultrasound abnormalities, especially non-isolated FPE, non-isolated CPAM, CDH, and pulmonary hypoplasia.

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.

Intralobular Pulmonary Sequestration of the Right Upper Lobe Supplied by Ascending Aorta.

Pulmonary sequestration (PS) is a rare congenital lung malformation that is more common in the left lower lobe. In 95% of cases, the artery supplying the sequestration usually originates from the thoracic and abdominal aorta. We report a rare intralobular PS case for a feeding artery from the ascending aorta. Angio-computed tomography should be performed for diagnosis once PS is suspected.

Long-term follow-up and novel variant in Suleiman-El-Hattab syndrome: Expanding the genotypic and clinical spectrum of a rare neurodevelopmental disorder

Suleiman-El-Hattab syndrome (SULEHS, OMIM #618950) is an autosomal recessive multisystem developmental disorder characterized by distinctive facial appearance, global developmental delay/intellectual disability, poor expressive speech and happy demeanor. SULEHS is an ultra-rare disorder associated with biallelic loss-of-function variants of the TASP1 gene, and up-to-date, seven patients from five families have been reported in the literature. Loss of TASP1 function has been reported to alter H3K4 histone modifications and expression of TFIIA and HOX transcription factors in the SULEHS phenotype. In this report, a new patient molecularly diagnosed with SULEHS by a novel homozygous c.404-2A > G variant in the TASP1 gene is presented with the long-term follow-up. Although the majority of the patient's clinical characteristics were similar to those of previously reported SULEHS patients, this study was the first to describe some additional anomalies, such as cystic hygroma, increased nuchal thickness, coarctation of the aorta, pulmonary stenosis, pulmonary sequestration anomaly, chronic constipation, encephalomalacia, and aggressive behavior. Because of the remarkable similarities between the clinical features of Baraitser-Winter syndrome (BRWS) and the patient, BRWS was considered the most likely diagnosis before the molecular diagnosis. Network analysis also supported that the interaction of the SULEHS-associated TASP1 gene with the BRWS-associated ACTB and ACTG1 genes through common intermediate molecules. Overall, despite the existence of differences in clinical features, inheritance patterns, and underlying pathophysiology between BRWS and SULEHS, both diseases could be considered in the differential diagnosis due to the high clinical similarities, including the dysmorphic features, growth parameters, neurodevelopmental phenotype, neurological problems, and multisystem involvement. Additionally, this report could contribute to a better understanding of the genotypic and clinical features of SULEHS by describing a novel pathogenic variant and new clinical features, such as prenatal manifestations.