Extralobar Bronchopulmonary Sequestration Research Articles

Association of Prenatal Congenital Lung Malformation Volume Ratio to Postnatal Computed Tomography Characteristics

IntroductionCongenital lung malformations (CLMs) are diverse and readily diagnosed on prenatal ultrasound. Postnatal computed tomography (CT) characteristics, including volume, are used in centers for the clinical decision-making of asymptomatic CLM. We aim to evaluate the relationship of prenatal CLM volume ratio (CVR) to postnatal CT characteristics by suspected prenatal diagnosis and postnatal radiological diagnosis. MethodsWe performed a single-center retrospective review of all patients evaluated with prenatally diagnosed CLM (May 2015-December 2022). Demographics, prenatal imaging findings at initial evaluation, and postnatal radiological diagnosis/imaging findings were analyzed with descriptive statistics. Pairwise correlation coefficient tests were performed to analyze the correlation between prenatal CVR and postnatal CT lesion size stratified by diagnosis. ResultsOf 123 patients referred and evaluated, suspected prenatal diagnoses consisted of 68 bronchial atresia (BA), 20 intralobar bronchopulmonary sequestration (iBPS), 20 extralobar bronchopulmonary sequestration (eBPS), and nine congenital pulmonary airway malformation (CPAM). Postnatal radiological diagnoses consisted of 53 BA, 22 iBPS, 14 eBPS, and 20 CPAM. Overall correlation coefficient of prenatal CVR to postnatal CT lesion size volume was 0.56. By suspected prenatal diagnosis, correlation coefficients were 0.61 (BA), 0.59 (iBPS), 0.29 (eBPS), and 0.51 (CPAM). For postnatal radiological diagnosis, correlation coefficients were 0.58 (BA), 0.56 (iBPS), 0.33 (eBPS), and 0.62 (CPAM). ConclusionsOur results indicate that initial CVR is overall consistent with the postnatal CT lesion size. This correlation is present in suspected prenatal diagnoses of BA and iBPS and postnatal radiological diagnoses of BA, iBPS, and CPAM. Additional studies analyzing long-term follow-up should be conducted to specify the safety of patients who undergo observation rather than surgical intervention.

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.

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.

ЭНДОХИРУРГИЧЕСКИЕ ТЕХНОЛОГИИ ЛЕЧЕНИЯ БРОНХОЛЕГОЧНОЙ СЕКВЕСТРАЦИИ У ДЕТЕЙ

The study systematized the long-term experience of thoracoscopic and laparoscopic treatment of intra- and extrapleural forms of bronchopulmonary sequestration (BPS). Materials and methods of research: a retrospective study analysed clinical data of 20 patients with BPS who have underwent thoracoscopic and laparoscopic surgeries for 15 years, starting from 2005. The main surgical methods included thoracoscopic lobectomy, thoracoscopic sequestrectomy, laparoscopic sequestrectomy and combined thoracoscopic transdiaphragmatic access in a patient with multiple forms of BPS. Results: a total of 20 patients with BPS had an intralobar form, 5 had an extralobar form, 4 had an extrapleural type, and 1 had a combined (extralobar + extrapleural) form. Intralobar form of BPS was found in the left lower lobe in 6 patients, in the right lower lobe – in 4. Extralobar BPS was found near the left lower lobe in 3 patients, near the lower right lobe – in 2. Extrapleural BPS was observed in the abdominal cavity under the diaphragm in 3 patients, in one patient – in the thickness of the diaphragmatic muscle. One patient had a combined form of BPS in the form of an association of extralobaric and intra-abdominal types. Thoracoscopy was performed in 16 patients, laparoscopy in 4 patients. The mean operative time was 60 min (range: 40–100 min). Intraoperative blood loss was not observed. A drainage tube was installed in 10 patients with intralobaric BPS. In all other cases, the pleural and abdominal cavity was not drained. A histological examination of all resected pulmonary sequesters showed that in half of the cases (10/20) the anomaly was associated with other malformations of the lungs (cystoadenomatous malformation of the lungs, bronchogenic cyst or intestinal duplication). During the 5 year average observation period after surgery (range: 1–15 years), musculoskeletal deformities of the chest and adhesive intestinal obstruction were not recorded. Conclusion: the series of minimally invasive treatment of patients with BPS presented in this study demonstrates the effectiveness and safety of this approach, especially for the surgical correction of those forms of pulmonary sequestration when they are located in hard-to-reach anatomical spaces of the body.

Non-immune hydrops fetalis complicating bronchopulmonary sequestration: A case report

Background: Non-immune hydrops (NIH) associated with bronchopulmonary sequestration (BPS) is quite rare with a 95% risk of intrauterine fetal death, without a fetal intervention. Case Presentation: We describe a case of an antenatally diagnosed extralobar BPS with severe NIH, who underwent fetal thoracentesis, but had worsening of NIH requiring an emergency cesarean section. Postnatally, the baby required skillful intensive care management and timely surgical management in the form of a sequestrectomy. Conclusion: We report the smallest neonate with antenatally diagnosed NIH complicating BPS treated successfully by early neonatal surgery.

Association of Communicating Extralobar Bronchopulmonary Sequestration with Intralobar Sequestration in a Patient Who had Anal Atresia

Association of Communicating Extralobar Bronchopulmonary Sequestration with Intralobar Sequestration in a Patient Who had Anal Atresia

‘Hybrid’ Bronchopulmonary Malformation – Lobar Emphysema and Extra Lobar Sequestration

Background: Idiopathic lobar emphysema (ILE) and bronchopulmonary sequestration (BPS) are two of the well-characterized pulmonary malformations. Case report: An antenatally detected case of a left bronchopulmonary malformation (BPM) was clinicoradiologically diagnosed to be a left upper lobar emphysema with isolated dextrocardia in the neonatal period. Besides the emphysematous left upper lobe, an accessory lobe akin to an extra lobar BPS was an operative surprise. Histopathological examination of both excised lobes led to a revised diagnosis of a ‘hybrid’ malformation comprising lobar emphysema and extra lobar BPS. The postoperative recovery was uneventful. Discussion/conclusion: The observations suggest that BPS and ILE may be interim entities in a continuum of abnormal embryogenesis. Such a hybrid malformation has not been hitherto reported.

Congenital pulmonary airway malformation and bronchopulmonary sequestration in infants - a rare diagnosis

Herein is described the case of a two-month-old female infant with recurrent respiratory infections, admitted to the Pediatric Emergency Department with productive cough, respiratory distress, and fever. Chest radiography showed an heterogenous hypotransparent image in the left pulmonary field and contralateral deviation of the mediastinum. Angiotomography revealed bilateral bronchopulmonary sequestration and a systemic vessel contributing to area irrigation. The patient was submitted to vessel occlusion and lacquering, left inferior lobectomy, and resection of a right lower lobe segment. Histological examination confirmed intralobar bronchopulmonary sequestration and type 2 congenital pulmonary airway malformation in the left lung, and extralobar bronchopulmonary sequestration in the right lung.With this report, the authors intend to emphasize the importance of clinical suspicion of congenital anomalies in infants with recurrent respiratory infections.

Concurrent extrapulmonary bronchopulmonary sequestration and bronchogenic cyst

Abstract A prenatal ultrasound (US) at 21 weeks estimated gestational age (EGA) identified a left intrathoracic homogenously echogenic microcystic mass with mediastinal shift and a CPAM volume ratio (CVR) of 0.78 (Fig. 1). A fetal magnetic resonance imaging (MRI) study confirmed the US findings. MRI and US were repeated at 35 weeks EGA demonstrating a CVR of 0.36 and a spontaneous decrease in size of the previously identified mass. Moreover, an intralesional, fluid filled dilated bronchus was identified that connected to the esophagus and showed mild branching. This finding was thought to represent an esophageal bronchus. Prenatal echocardiogram demonstrated normal cardiac anatomy and function. The patient was delivered vaginally at 38 weeks EGA with no respiratory distress after delivery. The patient was discharged at this time with planned outpatient follow-up. Outpatient contrast CT at three months of age demonstrated a left BPS supplied by the celiac axis and a fluid filled branching structure within the sequestration suspicious for esophageal bronchus. At four months of age, the patient underwent a left, muscle sparing thoracotomy, where the bronchopulmonary sequestration was excised and an adjacent cyst was enucleated. Pathology demonstrated extralobar bronchopulmonary sequestration with a separate foregut malformation most consistent with bronchogenic cyst.

Subsegmental consolidation masquerading as extralobar bronchopulmonary sequestration presenting as pyrexia of unknown origin

Acinetobacter baumannii infection is common among inpatients and is a major cause of hospital-acquired or ventilator-associated pneumonia. It is rare for this pathogen to cause community-acquired pneumonia. Eighty percent of the patients with community-acquired Acinetobacter present with lobar consolidation. Bronchopulmonary sequestration is a rare congenital disorder with incidence of 1 in 10,000–35,000 live births. Intralobar sequestration is more common than extralobar. Affected individual generally presents with respiratory distress at an early age. Late manifestation with the lower respiratory tract infection has also been reported. We report an interesting case of subsegmental consolidation masquerading as extralobar sequestration of the lung in an adult who presented with pyrexia of unknown origin.

Mixed Form of Congenital Cystic Adenomatoid Malformation and Extralobar Bronchopulmonary Sequestration: A Case Report

Bronchopulmonary sequestration(BPS) and congenital cystic adenomatoid malformation (CCAM) are rare, but both should be included in the differential diagnosis of fetal lung mass. We experienced a mixed form of Stocker type-III CCAM and extralo-bar BPS, and present this case, together with a review of the related literature.

Spontaneous hemorrhage into an extralobar bronchopulmonary sequestration.

A case of hemorrhage into an extralobar bronchopulmonary sequestration is presented. In a patient with a previously normal chest film, the appearance of a smooth mass lesion in the paravertebral area, particularly when adjacent to the diaphragm, should raise the possibility of this entity.