Introduction And Importance: Congenital lung malformations (CLMs) are rare congenital abnormalities resulting from abnormal development of the foregut and tracheobronchial tree. Pulmonary sequestration (PS) and bronchogenic cysts (BC) are two such anomalies, and while they can occasionally coexist, the combination of intralobar pulmonary sequestration (ILS) and BC is exceptionally rare. Only a limited number of cases have been reported in the literature. This case presents a unique occurrence of ILS and BC in the left lower lobe of an elderly woman, contributing to the understanding of these anomalies' shared origin.
Case Presentation: A 57-year-old woman with no significant medical history presented with left-sided chest pain and cough lasting for one month. Chest CT revealed a well-circumscribed cystic lesion in the left lower lobe, initially suspected to be a hydatid cyst. During surgery, a feeding artery originating from the descending thoracic aorta was found, confirming ILS. The cyst contained a thick chocolate-like fluid, suggesting a BC. Pathological analysis confirmed the presence of a unilocular cyst lined by respiratory epithelium, with features consistent with BC and ILS. The postoperative recovery was uncomplicated.
Clinical Discussion: CLMs include various malformations such as foregut duplication cysts, PS, and congenital pulmonary airway malformations. BCs arise from the ventral foregut and are usually asymptomatic but may present with symptoms due to infection or complications. PS is characterized by non-functioning lung tissue supplied by an aberrant systemic artery. CT features of PS vary, and angiography remains the gold standard for diagnosis. Two types of sequestration exist: extralobar and intralobar. ILS, typically considered acquired, has been occasionally linked to congenital anomalies, suggesting an embryological origin. The coexistence of BC and ILS is extremely rare, with only 10 cases reported in the literature.
Conclusion: The coexistence of Bronchogenic Cyst (BC) and Intralobar Pulmonary Sequestration (ILS) is a rare condition, with only a few cases documented. This case highlights the complexity of diagnosing these pulmonary malformations and suggests a potential shared congenital origin, challenging previous theories that ILS arises from chronic inflammation. Further studies are needed to elucidate the embryological link between these malformations and their clinical implications.
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http://dx.doi.org/10.1016/j.ijscr.2025.111008 | DOI Listing |
Ital J Pediatr
March 2025
Pediatric Pulmonology & Cystic Fibrosis Unit, Respiratory Research Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Introduction: Children with congenital lung disease (CLD) may suffer from long-term complications, such as impairments in lung growth, decreased total lung volume, recurrent lower respiratory tract infections and, in some cases, malignant transformation.
Objective And Methods: we described retrospective data on diagnostic process, clinical and functional data regarding a cohort of symptomatic and asymptomatic children with CLD followed in a single third level center in the last twenty years.
Results: 91 children were included in the study.
Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations, bronchopulmonary sequestration (BPS), congenital lobar overinflation, bronchogenic cyst, and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. BPS can be intralobar or extralobar sequestration.
View Article and Find Full Text PDFInt J Surg Case Rep
February 2025
General and Digestive Surgery Department, Mancha Centro University Hospital, Alcázar de San Juan, Ciudad Real, Spain; Instituto de Investigación Sanitaria de Castilla-La Mancha (IDISCAM), Spain.
Introduction: Epiphrenic diverticula are rare entities, generally asymptomatic. Most symptomatic ones occur in the form of dysphagia and are related to primary hypertensive oesophageal motor disorders of the distal oesophagus or the lower oesophageal sphincter. Exceptionally, they have been associated with congenital abnormalities in the formation of the primitive foregut.
View Article and Find Full Text PDFJ Cardiothorac Surg
March 2025
Department of Thoracic Surgery and Thoracic Oncology Institute, Peking University People's Hospital, Beijing, China.
Background: Anomalous systemic arterial supply to the left basal segment of the lung is a rare congenital pulmonary vascular malformation, historically classified as a variant of intra-lobar pulmonary sequestration. The standard surgical approach has typically involved ligation of the anomalous artery in combination with lobectomy or segmentectomy.
Case Description: We present three cases of anomalous systemic arterial supply to the left basal segment of the lung, all successfully treated with thoracoscopic anomalous arterial ligation alone.
Int J Surg Case Rep
March 2025
Al-Ahli Hospital, Hebron, Palestine.
Introduction: The patient showed an uneventful recovery post-surgery, underscoring the significance of early intervention in managing rare congenital anomalies. Bronchogenic cysts constitute 13-15 % of congenital cystic lung diseases and 6 % of childhood mediastinal masses. Arising from abnormal foregut development during embryogenesis, these cysts can be asymptomatic or cause serious complications, such as airway or vascular compression.
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