[Right upper lobe pulmonary sequestration as a rare cause of recurrent spontaneous pneumothorax]. / Sekvestratsiya verkhnei doli pravogo legkogo ­ redkaya prichina pravostoronnego retsidiviruyushchego spontannogo pnevmotoraksa.

A 19-year-old patient after previous wedge resection of the right upper pulmonary lobe a year ago urgently admitted with recurrent right-sided spontaneous pneumothorax. According to standard management of spontaneous pneumothorax, we performed diagnostic thoracoscopy and drainage of the right pleural cavity with regular X-ray examinations. However, these measures were ineffective. The patient was scheduled for surgery, and we intraoperatively observed an unusual cause of pneumothorax. Thus, we present spontaneous pneumothorax following right upper lobe pulmonary sequestration. The uniqueness of this case is associated with unusual manifestation and non-standard localization of rare lesion. A few cases of pneumothorax in similar patients are described in the world literature. The key limiting factor in diagnosis of such defects (identification of aberrant vessel supplying abnormal lung parenchyma) is the lack of routine CT angiography in patients diagnosed with pneumothorax. That is why CT changes were interpreted as postoperative ones, and the true cause was established only during redo surgery. A thorough inspection of the pleural cavity and alertness regarding unusual appearance of the right upper pulmonary lobe made it possible to suggest a non-standard diagnosis, avoid complications (bleeding from afferent vessel) and perform adequate lung resection.

Congenital diaphragmatic hernia with associated broncho-pulmonary sequestration: A report of two cases and a literature review.

BACKGROUND: Congenital Diaphragmatic Hernia (CDH) is a severe congenital anomaly with significant morbidity and mortality. It can be isolated or can be associated with other congenital anomalies, including broncho-pulmonary sequestration (BPS). The association of CDH with BPS (CDH+BPS) is uncommon but has been previously reported, and it can complicate the course of the disease in patients with CDH. We report two cases of CDH+BPS that were recently treated at our CDH-Qatar (CDH-Q) program. METHODS: We reviewed CDH-Q program registry to search for CDH+BPS and extracted the data for the identified cases. We also reviewed the previously published literature available on PubMed for similar cases. RESULTS: Out of 53 cases of CDH referred to CDH-Q from January 2018 to December 2022, two cases of CDH+BPS were identified, with an estimated prevalence of 3.8% of this association in our CDH population. Both cases were born at term. Case 1 was diagnosed with CDH+BPS postnatally, while case 2 was diagnosed with CDH antenatally but BPS was diagnosed after birth. Both cases underwent a surgical repair of the CDH with resection of the associated BPS, and the histopathology of the resected lung tissue confirmed the presence of BPS in both. Both cases survived to discharge. CONCLUSION: The association of CDH+BPS is uncommon; however, it can have significant consequences on the management and the prognosis of patients with CDH. Reporting these cases is important to provide a better understanding of this association and its impact on CDH patients.

Bilateral pulmonary sequestrations: Distinct types sharing a common celiac artery origin, presented with pneumonia and associated findings in a pediatric case.

A 14-year-old boy presented with shortness of breath, cough, and mild chest pain, with a history of intermittent milder symptoms. Physical examination and initial tests showed mild iron deficiency anemia, elevated C-reactive protein, and normal vital signs. Chest radiograph revealed abnormalities in the left lower zone, leading to contrast-enhanced chest CT. The CT scan revealed bilateral intrapulmonary sequestrations, both deriving blood supply from a common trunk originating from the celiac artery. The patient's symptoms initially attributed to a pulmonary infection improved with antibiotic therapy. Pulmonary sequestration is a congenital anomaly characterized by aberrant lung tissue lacking connections to bronchial tree or pulmonary arteries. It can lead to recurrent pulmonary infections and postinfectious sequelae. This case presented a unique bilateral sequestration, both originating from the celiac artery. Radiologists should be aware of sequestration types and associated anomalies, even in atypical locations. Blood supply can originate from various arteries, not just the aorta.

Unexpected pulmonary sequestration in a pregnant patient.

A pregnant woman in her early 30s, at 20 weeks of gestational age, presented with recurrent haemoptysis, pleuritic chest pain and a productive cough of 6 months duration. She underwent CT pulmonary angiogram which demonstrated right pulmonary sequestration and right-sided consolidation. Pre-existing pulmonary comorbidities such as chronic inflammation, structural abnormalities or weakened blood vessels within the lungs can encourage the growth of abnormal blood vessels. During pregnancy, these dynamics can be further aggravated by increasing cardiac output to promote blood flow to the placenta and increasing oxygen delivery to the developing foetus. These changes likely cause increased blood flow to the pulmonary sequestration, resulting in haemoptysis. The patient was treated conservatively for community-acquired pneumonia with a course of oral amoxicillin 500 mg three times a day for 5 days, and she is doing well on follow-up.

Torsed extralobar pulmonary sequestration associated with congenital lobar emphysema: a rare entity.

Pulmonary sequestration (PS) is a rare congenital malformation, which mainly contains two variants involving extralobar and intralobar sequestrations. Extralobar sequestrations (ELS) are isolated from the remaining lung tissue and have their visceral pleura. Herein, we report the first case of a torsed ELS associated with congenital lobar emphysema. We described a boy who mainly presented with abdominal and chest pain. A contrast-enhanced computed tomography (CT) scan revealed a mildly enhanced posterior mediastinal mass with left lower lobar emphysema. Thoracoscopic surgery identified a dark and hemorrhagic mass that was connected to the thoracic aorta by a twisted feeding vessel and had its visceral pleura. Pathological findings were consistent with an ELS that had undergone torsion and infarction. The postoperative recovery was uneventful. For the left lower lobar emphysema, he was required for a regular outpatient follow-up. In conclusion, although extremely rare, physicians should maintain a high index of suspicion for torsed ELS when a posterior mediastinal mass with abdominal or chest pain is presented in children.

Intrapulmonary sequestration with destructive pneumonia and life-threatening hemoptysis in an adult patient: a case report.

The article reports the case of a patient with bronchopulmonary sequestration complicated by destructive actinomycotic inflammation leading to life-threatening hemoptysis. It was an adult patient with the history of repeated right-sided pneumonia the cause of which had not been investigated in detail in the past. Only hemoptysis, which appeared as a complication, led to a closer investigation of the background of repeated right-sided pneumonia. CT scan of the chest revealed a lesion of the middle lobe of the right lung with anomalous vascularization - compatible with intralobar sequestration. Initially, conservative antibiotic treatment of pneumonia was provided at a local clinic. Embolization of the afferent vessels of the sequestrum was indicated due to persistent hemoptysis; this led to a reduction of its blood supply, proven by a follow-up CT examination of the chest. Clinically, the hemoptysis subsided. Three weeks later, the hemoptysis reocurred. The patient was acutely hospitalized at a specialized thoracic surgery department where shortly after admission, hemoptysis progressed to life-threatening hemoptea. Urgent middle lobectomy of the right lung was approached via thoracotomy to treat the source of bleeding. The case describes unrecognized bronchopulmonary sequestration as a possible cause of recurrent ipsilateral pneumonia in adulthood; additionally, it emphasizes the possible risks associated with a pathologically altered tissue microenvironment of pulmonary sequestration, and the need for surgical removal in all indicated cases.

[Scimitar syndrome with pulmonary sequestration containing carcinoma: a case report].

We reported the manifestations, auxiliary examination, and treatment courses of a case of scimitar syndrome with pulmonary sequestration containing carcinoma. The clinical characteristics of scimitar syndrome with pulmonary sequestration, pulmonary sequestration containing carcinoma were summarized based on the data of this case and the related literatures before January 2022. Scimitar syndrome can coexist with ipsilateral pulmonary sequestration. Because sequestered lung tissue has a risk of malignant transformation, a cancer screening test is useful for early diagnosis and timely treatment.

Pulmonary sequestration in adult patients: a single-center retrospective study.

BACKGROUND: Pulmonary sequestration (PS) is a rare congenital lower airway malformation. This study presents the clinical and imaging features and surgical outcomes of PS in adults, and compare the safety and feasibility of minimally invasive surgery versus open thoracotomy for PS. METHODS: Adult patients with PS treated at our center from July 2011 to September 2021 were included. Information regarding the patient demographics, clinical and CT features, arterial supply and venous drainage, and surgical outcomes were collected. RESULTS: Ninety seven patients were included. The most common CT findings were mass lesions (50.5%) and cystic lesions (20.6%). The vast majority of the lesions (96 out of 97) were located close to the spine in the lower lobes (left vs. right: 3.6 vs. 1). Arterial supply was mainly provided by the thoracic aorta (87.4%) and abdominal aorta (10.5%). Intralobar and extralobar PS accounted for 90.7% and 9.3% of the patients, respectively. Three (4.5%) patients who underwent minimally invasive surgery were converted to open thoracotomy due to dense adhesions. Though no significant differences regarding operative time (P = 0.133), the minimally invasive surgery group was significantly better than the open thoracotomy group regarding intraoperative blood loss (P = 0.001), drainage volume (P = 0.004), postoperative hospital days (P = 0.017) and duration of chest drainage (P = 0.001). There were no cases of perioperative mortality. Only four (4.1%) patients developed postoperative complications, and no significant difference existed between the two groups. CONCLUSION: Our study revealed PS can present with a variety of different clinical and radiologic manifestations. Clinicians should consider the possibility of PS when diagnosing a lesion in the lower lobes close to the spine. Moreover, minimally invasive surgery is a safe and effective treatment modality for the treatment of PS in an experienced center.

Thoracoscopic repair of neonatal left diaphragmatic hernia with sac combined with both extralobar pulmonary sequestration and congenital pulmonary airway malformation.

Intradiaphragmatic extralobar pulmonary sequestration (IDEPS) is considered to be very rare among extralobar pulmonary sequestration (EPS), and IDEPS combined with congenital diaphragmatic hernia (CDH) with sac is extremely rare. The patient was a neonatal girl. Left-sided CDH with a hernia sac was diagnosed based on computed tomography (CT). Thoracoscopic repair was planned for the 5th day after birth. Left CDH with sac was recognized on the posterolateral side and isolated EPS was recognized on the sac. The hernia sac, including the EPS, was resected, and the diaphragm defect was closed. The resected specimen consisted of fibrous connective tissue and pulmonary sequestration (PS) connected with the sac. It was diagnosed as the hybrid form, with features of both type 2 congenital pulmonary airway malformation (CPAM) and EPS. The postoperative course was uneventful. At 1 year and 9 mo after the operation, no recurrence of herniation or laterality of the diaphragm was recognized.

Thoracoscopic resection of pulmonary sequestration with carbon dioxide insufflation and indocyanine green.

We encountered a rare case of pulmonary sequestration supplied from the right renal artery, which was resected by video-assisted thoracic surgery with carbon dioxide insufflation and indocyanine green-guided technique. A 41-year-old woman with intralobar pulmonary sequestration supplied from the right renal artery was referred to our department. At the time of surgery, we used carbon dioxide insufflation to improve the manoeuvrable workspace for shutting off aberrant arteries and indocyanine green fluorescence guidance to differentiate the boundary of the sequestered lung from the normal lung. These procedures helped in the efficient resection of the lesion.

Two unusual variants of pulmonary intra-lobar sequestration.

A pulmonary sequestration is a congenital malformation characterized by non-functional lung tissue with abnormal arterial systemic supply and abnormal connection to the bronchial tree. This may lead to recurrent infections rendering a surgical intervention more demanding. Because of multiple anatomic variations, it is important to obtain high-quality preoperative radiological clarification to determine the most suitable surgical approach. Although a non-surgical technique, consisting of embolization of the aberrant artery has been described, a surgical technique remains the treatment of choice in operable patients. Preoperative embolization of the aberrant artery may reduce the risk of haemorrhage but could cause technical challenges in a hybrid approach and therefore unforeseen peroperative stress to the surgical team. We report 2 adult patients with unusual intra-lobar sequestration with aberrant vascular rare anatomy. Both were treated by surgery. In the latter patient, we performed a hybrid approach. This was complicated by peroperative coils exposure making it a technical challenge to proceed.

VATS surgical anatomical resection of bronchopulmonary sequestration presenting as chest sepsis.

BACKGROUND: Bronchopulmonary 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. METHODS: Here 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). RESULTS: Between 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. CONCLUSIONS: Dissection 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.

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

INTRODUCTION: 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. METHODS: Retrospective review of patients treated in our center. Literature review and combined analysis of perinatal data were performed. RESULTS: 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. CONCLUSION: 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.

Impact of the bronchopulmonary sequestration on endobronchial tuberculosis: the case report and the review of literature.

INTRODUCTION: We describe the rare case of endobronchial tuberculosis (EBTB) and chronic pulmonary atelectasis with mediastinal distortion. Finding of the concomitant venous anomaly of inferior vena cava revealed the diagnosis of bronchopulmonary sequestration. CASE REPORT: A 22-year-old Caucasian woman presented with a history of chronic cough, initially treated as bronchial asthma for a year. Chest X-ray showed fibrocaseous cavernous tuberculosis on the right lung. Acid Fast Bacilli (AFB) were found in sputum samples. Patient was treated for 6 months with usual antituberculous regiment. Control chest X-ray showed subatelectasis of the upper right lobe. Six months later the first thorax computed tomography (CT) showed complete atelectasis of the right lung. Patient was admitted to the hospital again after 6 years due to the persistent fever and cough. Endoscopic finding and histopathological analysis confirmed EBTB. Thoracic CT scan revealed duplication of inferior vena cava which led to profound vascular analysis and aberrant arterial vascularization of aortic origin that contributed to the diagnosis of bronchopulmonary sequestrations. Antituberculous treatment was initiated (streptomycin, isoniazid, rifampicin, ethambutol and pyrazinamide) and lasted for 8 months. After 8 months a follow-up fiberoptic bronchoscopy showed the progression of endoscopic finding with 60-70% tracheal stenosis. Histopathological finding of the mid-trachea showed non-specific granulations. During 7 years of follow-up repeated bronchoscopy and thoracic CT scans were unchanged and patient was well-shaped. CONCLUSIONS: The clinician should consider bronchopulmonary sequestration in the cases of recurrent EBTB.

Intralobar pulmonary sequestration associated with left main coronary artery obstruction and mitral regurgitation.

A 4-year-old boy with left intralobar pulmonary sequestration associated with left main coronary artery obstruction (LMCAO) and severe mitral regurgitation (MR) was admitted to our hospital. Since the patient presented with dyskinesia of the cardiac apex and increased left ventricular end-diastolic volume (LVEDV), left main coronary artery reconstruction and mitral annuloplasty were performed. The enlargement of the left ventricle was improved after sequential surgeries. There was a risk of deterioration of MR and regrowth of LVEDV due to shunt blood flow; therefore, left lower lobectomy and aberrant artery division were performed. This is a very rare case of a patient with pulmonary sequestration associated with LMCAO and severe MR.