Pulmonary Calcifying Fibrous Tumor in a Pediatric Patient: A Case Report.

A calcifying fibrous tumor (CFT), also known as calcifying fibrous pseudotumor, is an uncommon non-cancerous neoplasm usually located in the gastrointestinal tract. Its location in the lung is extremely rare, and only a few case reports have been published. This case report describes our diagnostic approach in a 9-year-old male patient with an incidental pulmonary mass. The mass was initially misdiagnosed, requiring multiple imaging tests and interventions to obtain the definitive diagnosis of pulmonary CFT. This paper aims to contribute to the limited information available on pulmonary CFT by presenting detailed findings from computed tomography and magnetic resonance imaging.

Presentation and Management of Pulmonary Sequestration With an Aneurysmal Aberrant Pulmonary Artery.

Bronchopulmonary sequestration presents rarely in adults and less frequently with an aneurysmal aberrant feeding artery. Treatment of bronchopulmonary sequestration generally involves lung resection with vascular ligation; however, aneurysmal disease increases the risk of intra- and postoperative hemorrhage and often necessitates more extensive surgery for vascular control. A 39-year-old female patient with a history of prior abdominal surgery presented with sudden onset epigastric and back pain. Computed tomography demonstrated an aneurysmal aberrant pulmonary artery originating from the abdominal aorta, adjacent to the celiac artery, supplying an intralobar pulmonary sequestration in the inferior right lower lung lobe. She also had evidence of cholelithiasis, with confusing symptom correlation. She was treated with a minimally invasive hybrid approach, which involved endovascular arterial embolization prior to delayed thoracoscopic lung resection. This is a safe and effective approach that reduces the risk of intraoperative bleeding while safely achieving vascular control proximal to the aneurysmal disease.

Interlobar pulmonary sequestration with celiac aberrant artery in an elderly patient treated with combined endovascular and video-assisted thoracoscopic approach.

Pulmonary sequestration is a rare congenital pulmonary anomaly where a portion of the lung parenchyma is supplied by an anomalous systemic artery, usually originating from the thoracic or abdominal aorta. Traditionally surgical resection and ligation of the aberrant feeding vessel are the gold standard treatments of this disease. Hybrid operations consisting in endovascular arterial embolization and surgical resection is a promising treatment option. We report a case of a 69-years-old man with symptomatic intralobular sequestration successfully treated by hybrid approach.

Neonatal pleural effusion associated with pulmonary sequestration: A case report.

Pleural effusion is rare during neonatal period with an estimated prevalence of 0.06%. It may sometimes uncommonly be secondary to pulmonary sequestration. Besides common conditions like hydrops fetalis, congenital heart disease, congenital chylothorax, chromosomal abnormalities; pulmonary sequestration should also be considered while evaluating the cause for neonatal pleural effusion.

Imaging Diagnosis for Intradiaphragmatic Pulmonary Sequestration: A Pilot Study.

OBJECTIVES: Extrapulmonary sequestration (EPS) within the diaphragm (ID-EPS) is rare and requires additional procedures such as incision or detachment of the diaphragm from the lesion for diagnosis. This study aimed to describe the imaging findings and evaluate the diagnostic accuracy of ultrasonography and computed tomography (CT) for ID-EPS. MATERIAL AND METHODS: Split diaphragm sign, shape of lesion edge, drainage vein into intra-abdomen on ultrasound and CT, and lesion characteristics on ultrasound were compared between patients with ID-EPS and with above-diaphragm EPS (AD-EPS) using Fisher's exact test. RESULTS: Three and nine patients were diagnosed with ID-EPS and AD-EPS, respectively. Significant differences were observed between the two groups in the split diaphragm sign on ultrasound (presence/absence in patients with ID-EPS vs AD-EPS, 2/1 vs 0/9, P = .046), shape of lesion edge on ultrasound/CT (round/beak in patients with ID-EPS vs AD-EPS, 3/0 vs 0/9, P = .005 on both CT and ultrasound), lesion characteristics on ultrasound (presence/absence of cystic area within lesion in patients with ID-EPS vs AD-EPS, 0/3 vs 7/2, P = .046), and the drainage vein into the abdomen on CT (presence/absence in patients with ID-EPS vs AD-EPS; 2/1 vs 0/9, P = .046). No drainage veins were visualized in the abdomen on ultrasonography and no significant differences in the presence/absence of the split-diagram sign on CT (presence/absence in patients with ID-EPS vs AD-EPS and ID-EPS; 0/3 vs 0/9, P > .999; 1/2 vs 0/9, P = .250) were observed between the two groups. CONCLUSION: A combination of postnatal ultrasonography and CT was useful in predicting EPS located within the diaphragm.

A rare tale of an extralobar pulmonary sequestration as a cause of hemoptysis not often contemplated: a case report.

Background: A rare congenital malformation of the respiratory tract, bronchopulmonary sequestration (BPS), may present symptomatically early on in childhood or adolescent years. Adult BPS is typically an incidental finding found on thoracic imaging. There are currently four known types of BPS. Intralobar sequestrations (ILSs) are the most common of them and the most commonly reported form in literature. In our case report, we report of a healthy adult female who presented with hemoptysis that resulted in the diagnosis of the rarest form of BPSs; extralobar sequestration (ELS). One that is not commonly described in literature, especially of one reported in late adulthood. This case report aims to educate and elude clinicians to this rare cause as a differential and guidance on its investigation and management. Case Description: In this case report, a 56-year-old woman who presented to an outpatient respiratory clinic after being referred by her general practitioner (GP) of a queried BPS. A thorough workup was done by the respiratory team that derived at the final diagnosis of an ELS. The patient's autonomy was adhered to along with risks and benefits which resulted in a non-surgical approach to management. One that she remarkably achieved a resolution of her symptoms. Conclusions: An awareness and familiarity of this rare disease, ELS, should prompt one to consider its' diagnosis when no other common causes are apparent. Often the diagnosis can be made radiologically. Treatment of ELSs should depend on multiple factors.

Scimitar Syndrome in a Pediatric Cohort.

BACKGROUND: Scimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome. METHODS: We performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography. RESULTS: Ten patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up. CONCLUSIONS: All patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.

Separating Out Pulmonary Sequestration.

Pulmonary sequestration (PS) is a rare congenital anomaly that accounts for 1% to 6% of all pulmonary malformations at birth. It is characterized by a focal area of pulmonary tissue that does not have direct communication with the tracheobronchial tree and does not get blood supply from the pulmonary circulation. We present the case of a 28-year-old female with a history of recurrent pulmonary infections who was found to have intralobar sequestration and underwent curative surgical excision. Because pulmonary sequestration is commonly misdiagnosed, as it can mimic other conditions on chest X-rays, this case illustrates the importance of recognizing pulmonary sequestration as a separate entity and diagnosing/treating it appropriately. The patient presented to the hospital with a one-week history of upper chest pain. Chest radiograph showed mild hyperinflated right lung. Computed tomography angiogram (CTA) revealed an 8.9 x 8.3 cm area of hyper-lucency and decreased normal lung architecture in the right lower lobe with an aberrant arterial blood supply suggestive of intralobar pulmonary sequestration. The patient was referred to cardiothoracic surgery and underwent preoperative outpatient pulmonary function testing, which was unremarkable. The patient subsequently underwent successful robotic resection of the right lower lobe sequestration and the pathology report confirmed PS. A diagnosis of pulmonary sequestration is commonly missed, as it can mimic other conditions on chest X-rays. It can present as a solitary nodule or mass, cystic lesion, consolidation, or an air-fluid level. The period between symptom onset and diagnosis is typically more than five years. While digital subtraction angiography is considered the gold standard for imaging, CTA is now preferred because it allows for clear visualization of lung parenchyma and vascular assisting in surgical planning. For our patient, CTA demonstrated a prominent tubular vessel, which showed less enhancement than the opacified pulmonary artery and pulmonary veins, suggestive of an abnormal vascular supply for the right lower lobe sequestration. Management of intralobar sequestration is curative surgical excision. Both video-assisted thoracoscopic surgery (VATS) and posterolateral thoracotomy are viable options for resection. It should be noted that in symptomatic patients, it is recommended to proceed with surgical resection. However, in asymptomatic individuals with intralobar sequestration (ILS), surgical resection is not required but could be considered as prophylaxis to prevent recurrent infections. Asymptomatic individuals with extralobar sequestration (ELS), on the other hand, should undergo serial monitoring as non-operative management is appropriate. This case highlights the importance of including pulmonary sequestration, especially intralobar sequestration in the differential diagnosis of recurrent localized pulmonary infections, especially in a patient who is otherwise healthy. Although rare, it is important to consider this congenital anomaly when evaluating patients with recurrent localized pulmonary infections, chest pain, or hemoptysis.

Simultaneous occurrence of extralobar pulmonary sequestration, esophageal duplication, and bronchogenic cysts in a Chinese child: a rare case report.

The occurrence of simultaneous extralobar pulmonary sequestration, esophageal duplication, and bronchogenic cysts is relatively low. We report the case of a 9-month-old Chinese child who had a right lung cyst, detected in utero and was closely monitored until birth. At age 9 months, contrast-enhanced computed tomography revealed right mediastinal extralobar pulmonary sequestration and two cysts. The patient did not exhibit any abnormalities. However, the parents were concerned about the disease. Following positive psychological counseling to the parents, surgery was the strong desire. Subsequently, successful thoracoscopic surgery was performed, excising the three lesions. No postoperative complications occurred. Postoperative pathology confirmed extralobar pulmonary sequestration syndrome combined with esophageal duplication and bronchogenic cysts. The patient was followed-up at 1 and 12 months postoperatively and recovered well with no abnormal space occupation. In such cases, preoperative imaging examinations should be carefully performed, and intraoperative exploration should correspond to that before surgery to avoid lesion omission.

Endovascular embolization for massive hemoptysis in intralobar pulmonary sequestration with celiac artery supply: A comprehensive case report.

Pulmonary sequestration (PS) is a rare congenital anomaly characterized by noncommunicative lung tissue supplied by an abnormal systemic vessel. We present a case of a 30-year-old male with intralobar PS, receiving arterial supply from the celiac artery, manifesting as massive hemoptysis. After urgent stabilization, endovascular embolization using polyvinyl alcohol particles was successfully employed. The patient's symptoms resolved, and follow-up confirmed satisfactory recovery. Our case underscores the diverse arterial origins of PS and the efficacy of endovascular embolization as a minimally invasive treatment. The complexity of PS, its diagnostic imaging, and alternative therapeutic options are discussed, emphasizing tailored approaches for optimal outcomes in managing this uncommon congenital anomaly.

Intralobar pulmonary sequestration presenting as multiple nodular pulmonary lesions and focal emphysema.

The features of intralobar pulmonary sequestration vary on computed tomography (CT). Many cases demonstrate a mass or cystic lesion within a lower lobe. We report herein a case of a 55-year-old, female patient presenting with right back pain. Contrast enhanced (CE) CT revealed multiple, nodular, pulmonary lesions suggesting recurrent infections with surrounding focal emphysema. Three-dimensional (3D) reconstruction demonstrated a sequestrated lung segment with a systemic, arterial blood supply. Based on these findings, intralobar pulmonary sequestration was diagnosed. Intralobar pulmonary sequestration can present as multiple, nodular, pulmonary lesions with focal emphysema rather than as a mass or cyst. CE-CT with 3D reconstruction is useful for diagnosing this condition. Patients with recurrent pulmonary infections have a high index of suspicion of intralobar pulmonary sequestration.

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.

Lingular intralobar pulmonary sequestration supplied by the left inferior phrenic artery arising from the left gastric artery - A case report and literature review.

Pulmonary sequestration is characterized by a nonfunctional mass of lung tissue with an aberrant blood supply. Intralobar pulmonary sequestration (IPS) typically affects lower lung lobes and receives its blood supply from systemic arteries. Here, we present a unique case of a 51-year-old woman presented with recurrent nonmassive hemoptysis. Contrast-enhanced computed tomography angiography (CTA) of the chest showed uniform consolidation in the inferior lingular segment of the left upper lobe. Maximal intensity projection (MIP) and three-dimensional volume rendering (3D-VR) showed the affected area's blood supply from unusual arterial branches originating from the left inferior phrenic artery arising from the left gastric artery, consistent with IPS. A multidisciplinary approach utilized endovascular intervention (coil embolization) before successful surgical resection. Detecting IPS in unusual sites, like the lingular region, poses a diagnostic challenge. Clinicians and radiologists may not initially consider this diagnosis when evaluating patients with respiratory symptoms or incidental imaging findings. A comprehensive grasp of their anatomy and vascular variations is vital for precise diagnosis and effective treatment planning.

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.

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.