Pulmonary Sequestration Research Articles

The Clinical Value of Prenatal Ultrasonography in the Differential Diagnosis of Fetal Suprarenal Space-Occupying Lesions.

ObjectiveThis study aimed to investigate the value of prenatal ultrasonography in the differential diagnosis of fetal space-occupying lesions in the adrenal gland.MethodsThirty-six fetuses with adrenal gland space-occupying lesions diagnosed by prenatal ultrasonography between January 2019 and July 2021 were included in this retrospective study. The clinical data, ultrasonographic features, treatments, and prognoses of the fetuses were analyzed. Postnatal diagnoses were made using computed tomography (CT), magnetic resonance imaging, and surgical resection.ResultsOf the 36 fetuses, 10 were diagnosed with adrenal hematomas, eight with adrenal neuroblastomas, seven with adrenal cysts, seven with subphrenic pulmonary sequestration, and four with adrenal teratomas. The accuracy of prenatal diagnosis was highest in those with adrenal cysts and subphrenic pulmonary sequestration, with the accuracy being 85.7% for both conditions. The mean gestational age at first detection of subphrenic pulmonary sequestration was 22.5 ± 3.1 weeks, which was significantly lower than that of other diagnoses (P < 0.05), and the mean diameter of adrenal cysts was 15.1 ± 4.2 mm, which was significantly smaller than that of other lesions (P < 0.05). All newborns with adrenal teratomas and neuroblastomas were treated surgically. Five of the seven patients with subphrenic pulmonary sequestration and three of the seven patients with cysts were also treated surgically. Follow-ups of the remaining cases were carried out by enhanced CT examination, and the prognoses were good.ConclusionPrenatal ultrasonography can help differentiate between different types of fetal adrenal space-occupying lesions, and there is a high coincidence rate between the diagnosis of adrenal cysts and subphrenic pulmonary sequestration.

VATS surgical anatomical resection of bronchopulmonary sequestration presenting as chest sepsis

BackgroundBronchopulmonary 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.MethodsHere 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).ResultsBetween 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.ConclusionsDissection 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.

Social media communities for patients and families affected by congenital pediatric surgical conditions.

BackgroundSocial media has become a means to allow individuals affected by rare diseases to connect with others. This study aimed to characterize the use of three popular social media platforms by individuals affected by common pediatric surgery index conditions.MethodsA systematic search of Instagram, Facebook, and Twitter was performed using standardized terms for the following pediatric surgical conditions: anorectal malformation, biliary atresia, bronchopulmonary sequestration, congenital diaphragmatic hernia (CDH), congenital pulmonary airway malformation, duodenal atresia, esophageal atresia/tracheoesophageal fistula (EA/TEF), gastroschisis, Hirschsprung disease (HD), and omphalocele. Accounts active within the last year were analyzed and assessed.ResultsA total of 666 accounts were identified. Instagram was the most common platform, but accounts dedicated to support and story sharing were most common on Facebook. Biliary atresia and CDH had the largest communities identified. Support groups were most common among those dedicated to EA/TEF (43.3%, p < 0.001). Most accounts were created by parents, but accounts dedicated to HD had the greatest proportion founded by patients (22.4%, p = 0.04).ConclusionsSocial media use is common among patients and families affected by congenital surgical conditions. Certain diseases have larger communities and support networks. Knowledge about their existence may allow surgeons to direct patients towards supportive communities and resources.Level of evidenceIV.

Pulmonary sequestration and concomitant pectus excavatum in adulthood

Pulmonary sequestration and concomitant pectus excavatum in adulthood

Pulmonary sequestration

Pulmonary sequestration

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.

Case Report: 43-years old-Male with Pulmonary Sequestration which Mimics Lung Tumor and Aspergillosis

Background: Pulmonary sequestration is a very rare congenital abnormality of the lung, which was responsible for 0.15-6.4% of all cases of congenital lung formation disorders. Pulmonary sequestration is often seen as recurrent pneumonia, accompanied by complaints of chest pain, shortness of breath and hemoptysis. Conventional angiography is the gold standard for diagnosing pulmonary sequestration and for demonstrating arterial supply and venous drainage. The optimal therapy for pulmonary sequestration is operative resection to avoid infection and damage to the lung parenchyma. Case Report: We reported a case of 43-years old-male with pulmonary sequestration, previously diagnosed with a lung tumor with massive left pleural effusion and pneumonia. A series of diagnostic tests found no malignant cells. During FNAB with CT scan guiding, we found an image of a fungus ball, thus, he was diagnosed with Aspergillosis. The patient was planned to undergo VATS. There were complications during the operation, thus, a lobectomy with an open thoracotomy was performed, and an arterial anomaly was found from the abdominal aorta. The operation was successful and the patient was able to carry on with his normal activities.Conclusion: Establishing a definite diagnosis of pulmonary sequestration is very difficult, and in this case, it was done through lobectomy with thoracotomy and through a multidisciplinary approach.

Hidrotórax fetal secundario a secuestro pulmonar intralobar: terapia fetal mediante escleroterapia percutánea bajo guía ecográfica

Pulmonary sequestration is a mass of non-functioning lung tissue that does not communicate with the tracheobronchial tree and has an aberrant blood supply that comes from the systemic circulation. We present the case of a 34-year-old pregnant woman, with a pregnancy of 25 weeks, with an antenatal diagnosis of pulmonary sequestration with pleural effusion, mediastinal compression, cardiac dextroposition and polyhydramnios. Fetal intervention was performed using percutaneous sclerotherapy, successfully. After 4 weeks, there was evidence of absence of pleural effusion, heart in normal position. The pregnancy ended satisfactorily with a newborn in stable condition. Pulmonary sequestration can present a rapid evolution of mass growth, which produces mediastinal compression, fetal hydrops and polyhydramnios, which produces approximately 100% fetal mortality. It is important to know all available therapeutic options, including percutaneous sclerotherapy under ultrasound guidance, to improve fetal prognosis.

Hidden Infection in Asymptomatic Congenital Lung Malformations-A Decade Retrospective Study.

BackgroundWhether to operate on asymptomatic patients with congenital lung malformations (CLMs) remains controversial. Our study intended to find out the proportion of hidden infection in CLMs and its effect on surgery, to provide help for the management of asymptomatic CLMs patients.MethodsA retrospective review of the medical records of patients with asymptomatic CLMs from January 2011 to December 2020 was performed in our center. Selected asymptomatic patients were divided into a non-hidden infection group (NHI) and a hidden infection group (HI).ResultsA total of 581 asymptomatic CLMs patients were included in this study. Thirty-two percent of asymptomatic CLMs patients had hidden infection in the lesion. Among various CLMs diseases, intralobular pulmonary sequestration had the highest percentage of hidden infection (48.8%). With age, the proportion of HI gradually increased. Patients in the HI and NHI groups were 223 and 121. The incidence of pleural adhesion and focal abscess in the HI group were 14.9 and 7.4%. Statistical significances were shown between the two groups in intraoperative blood loss (p = 0.002), operation time (p = 0.045), chest tube drainage time (p < 0.001), postoperative hospital stay (p < 0.001), and air leak (p = 0.012).ConclusionThe proportion of HI detected by postoperative pathological results was high and they could increase the difficulty and risk of surgery. Therefore, early surgery may be a more appropriate choice for the management of asymptomatic CLMs patients.

Comparison of Endobronchial Intubation Versus Bronchial Blockade for Elective Pulmonary Lobectomy of Congenital Lung Anomalies in Small Children.

Purpose: Resection of many congenital lung lesions is commonly performed under single-lung ventilation, which helps collapse the lung being manipulated and enables a thoracoscopic approach in most cases. We set out to determine whether lung isolation achieved by either main stem intubation or usage of a bronchial blocker was associated with superior clinical outcomes. Materials and Methods: A retrospective review of all patients aged <2 years undergoing elective pulmonary lobectomy for congenital lung malformations at a tertiary-care pediatric hospital from 2011 through 2020 was performed. Demographic data, diagnosis type, type of lung isolation method employed, and perioperative outcomes were recorded. Continuous variables were analyzed with Student's t-tests, whereas categorical variables were analyzed with Fisher's exact tests and chi-square tests. Results: Thirty-two patients were analyzed-17 were managed with a bronchial blocker while 15 underwent main stem intubation. The most common diagnoses were congenital pulmonary airway malformations (53.1%) and intralobar bronchopulmonary sequestrations (34.4%). Patients managed with main stem intubation were slightly younger (P = .06) than those for which a bronchial blocker was used. Thirty-one (96.9%) resections were initiated thoracoscopically. Main stem intubation was associated with shorter operative times (P = .01), shorter anesthetic times (P = .02), and less blood loss (P = .04). No differences in length of stay (P = .64), conversation to thoracotomy (P = .35), intraoperative complications (P = .23), or postoperative complications (P = .49) were observed. Conclusion: Lung isolation through main stem intubation, when compared with bronchial blockers, is associated with shorter operative time, shorter anesthetic exposure, and diminished blood loss in pediatric patients undergoing lobectomy for congenital lung anomalies.

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...

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Meeting abstracts from the 15th international conference on neonatal and childhood pulmonary vascular disease

Minimally-invasive surgery for concurrent intralobar pulmonary sequestration and bronchogenic cyst in an adult: a case report

Minimally-invasive surgery for concurrent intralobar pulmonary sequestration and bronchogenic cyst in an adult: a case report

The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations

BackgroundThe natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery. Material and methodsA retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed. ResultsSixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery. ConclusionPrenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms. Level of evidenceIII

Surgical resection for congenital lung malformation: Lessons learned from thoracotomy to biportal thoracoscopy under one-lung ventilation

The purpose of this study is to compare the clinical characteristics and surgical outcomes of thoracotomy and video-assisted thoracoscopic surgery (VATS) in children with congenital lung malformations (CLMs) in a tertiary referring center and to report our modified biportal VATS setting. This is a single-center retrospective chart review study including children who underwent surgical resection for CLMs between January 2007 and December 2020. Patient characteristics and surgical outcomes were compared between open and thoracoscopy, as well as conventional VATS and biportal VATS. Biportal setting included an anterior utility wound and a camera trocar wound with one-lung ventilation. A total of 100 patients were identified. Twenty patients received thoracotomy, and 80 patients received VATS (67 conventional and 13 biportal VATS). The median age at operation was 0.4 months in the thoracotomy group and 4.7 months in the VATS group. More patients in the thoracotomy group had preoperative symptoms, comorbidities, and emergent operations. The patients who underwent thoracotomy had significantly longer postoperative ICU stays, chest tube durations, hospital stays, and more complications. The pathological analysis revealed 67 congenital pulmonary airway malformations, 27 pulmonary sequestration, 6 hybrid lesions, and one accompanying pleuropulmonary blastoma. Compared to conventional VATS, the ICU stay was shorter in the biportal VATS group, with comparable operative durations, hospital stay and complications. VATS for CLMs is associated with better postoperative recovery and fewer complications. Biportal VATS is also a safe and feasible approach.

Management Options for Fetal Bronchopulmonary Sequestration.

To evaluate the prenatal course and perinatal outcome of fetuses with bronchopulmonary sequestration (BPS) managed expectantly or using minimally invasive methods. This was a retrospective study of 29 fetuses with suspected BPS managed between 2010 and 2021 in three fetal medicine centers in Poland. Medline was searched to identify cases of BPS managed expectantly or through minimally-invasive methods. In 16 fetuses with BPS, there was no evidence of cardiac compromise. These fetuses were managed expectantly. Thirteen hydropic fetuses with BPS qualified for intrauterine intervention: a thoraco-amniotic shunt (TAS) was inserted in five fetuses, laser coagulation of the feeding vessel was performed in seven cases, and one fetus had combined treatment. In the combined data from the previous and the current study of various percutaneous interventions for BPS associated with hydrops, the survival rate was 91.2% (31/34) for TAS, 98.1% (53/54) for laser coagulation, and 75% (3/4) for intratumor injection of sclerosant. After taking into account cases with available data, the rate of preterm birth before 37 weeks in the group treated with laser coagulation was 14.3% (7/49) compared to 84.6% (22/26) in the group treated with TAS. The need for postnatal sequestrectomy was lower in the group of fetuses treated with laser coagulation 23.5% (12/51) in comparison to fetuses treated with TAS 84% (21/26). In fetuses with BPS without hydrops, progression of the lesion’s volume, leading to cardiac compromise, is unlikely. In hydropic fetuses with BPS, intrauterine therapy using minimally invasive methods prevents fetal demise. Both, the rate of preterm birth and the need for postnatal surgery is significantly lower in the group treated with laser coagulation compared to the group treated with TAS.

Intrauterine Ultrasound-Guided Laser Coagulation as a First Step for Treatment of Prenatally Complicated Bronchopulmonary Sequestration: Our Experience and Literature Review

Prenatal ultrasound-guided laser coagulation (USLC) for complicated bronchopulmonary sequestrations has been described but a consensus on the procedure and on the following management is still lacking. We present our experience and provide a literature review. Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed. Five cases were treated at our center, all presenting with severe hydrothorax. Four met the criteria for fetal hydrops. Four cases underwent postnatal computed tomography (CT) scan: in one case, there was no evidence of persistent bronchopulmonary sequestration. The other three underwent thoracoscopic resection, in two, a viable sequestration was found. Including our series, 57 cases have been reported, with no mortality and a success rate of 94.7%. Mean gestational age (GA) at the procedure was 28 ± 3.4 weeks and mean GA at birth and birth weight (BW) were 38.6 ± 2.3 weeks and 3,276 ± 519.8 g, respectively. In 80.6% of the cases investigated postnatally, a residual mass was found, 50% of cases who showed prenatal arterial flow cessation had a persistent sequestration postnatally, and 26.3% of cases underwent postnatal sequestrectomy. Both patients in our series had pathology examination confirming a viable bronchopulmonary sequestration. Prenatal USLC seems to be a valid option for bronchopulmonary sequestration complicated by severe hydrothorax and/or fetal hydrops. Authors believe that this procedure should aim to reverse fetal distress and allow pregnancy continuation, and it should not be considered a definitive treatment. The currently available data do not support changes of the common postnatal management.

The Role of Fetal MRI-based Texture Analysis in Differentiating Congenital Pulmonary Airway Malformation and Pulmonary Sequestration

The Role of Fetal MRI-based Texture Analysis in Differentiating Congenital Pulmonary Airway Malformation and Pulmonary Sequestration

Pulmonary sequestration: a rare cause of false-positive uptake in Iodine-131 whole-body scan

BackgroundHigh-dose radioactive iodine-131 (I-131) is generally used post-total or subtotal thyroidectomy in differentiated thyroid cancer (DTC) patients. I-131 whole-body scan (WBS) is performed to localize I-131 accumulation in the body and for restaging after therapy. Nonetheless, there are numerous causes of false-positive uptake in I-131 WBS in the absence of residual thyroid tissue or thyroid cancer metastasis.Case presentationWe present a case of 51-year-old lady with false-positive uptake in I-131 WBS mimicking lung metastasis. Patient underwent total thyroidectomy, and histopathological examination showed well-differentiated papillary thyroid microcarcinoma. Diagnostic I-131 WBS followed by two subsequent high-dose radioiodine ablation showed persistent I-131 uptake in the left lower lung field. However, the serum thyroglobulin (Tg) is incessantly undetectable (< 0.04 μg/ml) with negative anti-thyroglobulin antibody (anti-TgAb). Three serial CT scans over the course of treatment coupled with MRI of the thoracolumbar region revealed stable lung lesion in the posterior segment of left lower lobe which signifies pulmonary sequestration. Based on the clinical assessment, imaging findings and blood investigations, patient was no longer subjected to I-131 therapy and is maintained on TSH suppression with L-thyroxine. After due consideration of the pros and cons of surgical intervention, the patient opted against surgical removal of the pulmonary sequestration.ConclusionsMeticulous assessment of patient with suspected disease recurrence or metastasis in thyroid carcinoma is essential to avoid unnecessary I-131 ablation or surgical intervention. In this case, the positive I-131 imaging is associated with undetectable serum Tg (with negative anti-TgAb). Albeit uncommon, pulmonary sequestration should be taken into consideration as one of the differentials in false-positive findings of an I-131 WBS.

Congenital Malformations of the Lung - an Overview

Congenital pulmonary malformations comprise a heterogenous group of rare developmental diseases. The most common malformations are the tracheal bronchus, bronchial atresia, bronchogenic cyst, pulmonary sequestration, congenital lobar emphysema, and congenital pulmonary airway malformation. Due to their space-consuming effect, patients suffer early postnatal respiratory distress which generally requires immediate surgical resection. The management of asymptomatic lesions remains subject to debate, but early elective surgery is generally recommended to avoid respiratory and infectious complications at a later time point.We here provide a comprehensive review in which we present causes, clinical presentation and therapeutic options for the most prominent congenital malformations of the airways and lung parenchyma.