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1/19. Intralobar pulmonary sequestration supplied by the right coronary artery.

    Bronchopulmonary sequestrations are malformations that are often congenital; they consist of isolated nonfunctioning lung segments having no communication with functional tracheobronchial elements of the surrounding lung. They are supplied by single or multiple branches from the distal thoracic or proximal abdominal aorta, or from the celiac, splenic, intercostal, subclavian, or pulmonary artery. Due to the absence of ventilation, the lung tissue can become chronically infected. We describe an intralobar pulmonary sequestration with arterial supply from the right coronary artery.
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2/19. Epiphrenic diverticulum composed of airway components attributed to a bronchopulmonary-foregut malformation: report of a case.

    Bronchopulmonary-foregut malformation (BPFM), defined originally as pulmonary sequestration with or without communication to the esophagus, has been acknowledged to include congenital foregut diverticula. We present herein the case of a 43-year-old woman with a 9-year history of dysphagia, in whom a barium meal examination demonstrated a 2.5-cm epiphrenic diverticulum and several fistulae. A laparotomy was performed and the lower esophagus without communication to the lung was pulled down and resected, followed by an esophagogastrostomy carried out with fundopexy. Since her operation, the patient has been free of symptoms. Histologically, the diverticulum was observed to be lined by stratified squamous cells, but its shape was formed by mural cartilage, smooth muscle cells, and three ciliated-cell cysts. The dysphagia was considered to have been derived from the kinked esophagus created by the rigid diverticulum, being the possible developmental arrest of a supernumerary lung bud. These findings indicate that this case may involve BPFM in the broad sense. Although several cases of bronchogenic cysts located beneath or across the diaphragm have been reported as a subgroup of BPFM, congenital epiphrenic diverticula has rarely been described.
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3/19. Atypical extralobar sequestration: CT-pathological correlation.

    Pulmonary sequestration is a malformation comprised of dysplastic lung tissue without normal communication with the tracheobronchial tree and with an anomalous systemic arterial supply. Pulmonary sequestration is classified into two types, intralobar and extralobar based on the location of the malformation and the venous drainage. Extralobar sequestration is less common than intralobar sequestration and usually has systemic venous drainage into the azygos system. Most patients with extralobar sequestration are diagnosed before the age of 10. We present an interesting and unusual case of extralobar sequestration which presented as a retroperitoneal mass in an asymptomatic adult.
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4/19. Complex bronchopulmonary foregut malformation: extralobar pulmonary sequestration associated with a duplication cyst of mixed bronchogenic and oesophageal type.

    We report a 13-year-old girl with an unusual, complex bronchopulmonary foregut malformation. The malformation included extralobar pulmonary sequestration and a duplication cyst of mixed bronchogenic and oesophageal type. Preoperative CT and MRI demonstrated the cystic and solid portions of the mass and indicated an aberrant vascular supply, suggesting the possibility of bronchopulmonary foregut malformation and several other differential diagnoses. A direct communication between the cyst and the bronchus of the sequestrated lung was found on pathological examination. This unusual combination of an extralobar pulmonary sequestration and a foregut cyst points to a common embryological pathogenesis.
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5/19. The multiple facets of pulmonary sequestration.

    PURPOSE: The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. methods: All charts of patients with pulmonary sequestration admitted at 2 children's hospitals from 1982 to July 1999 were reviewed retrospectively. The authors included all anomalies with a systemic arterial supply or without bronchial connection. RESULTS: Only 22 of the 39 patients (56%) had a classic isolated extralobar or intralobar sequestration, whereas the others presented with a spectrum of anomalies. Of the 13 cases diagnosed prenatally, 85% were asymptomatic at birth. In contrast, 26 cases diagnosed postnatally were all symptomatic, with those patients less than 2 weeks old presenting with various degrees of respiratory distress, and those older than 2 weeks old presenting with respiratory infections. The correct diagnosis was made preoperatively in 59% of cases. Only 4 patients did not undergo resection of their lesion, of which, 1 underwent interventional radiology with embolization of the anomalous arterial supply. Follow-up issues of importance included pneumonia, asthma, gastroesophageal reflux, and pectus excavatum. CONCLUSIONS: Sequestrations represent a spectrum of anomalies that overlap with other lung lesions. To facilitate management, they should be described according to their (1) connection to the tracheobronchial tree, (2) visceral pleura, (3) arterial supply, (4) venous drainage, (5) foregut communication, (6) histology, (7) mixed/multiple lesions, and (8) whether there are associated anomalies. Surgeons should be aware that approximately 50% of sequestrations could be atypical or associated with other anomalies. This should be kept in mind when weighing the benefits of resection versus conservative management of pulmonary sequestrations.
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6/19. Communicating bronchopulmonary foregut malformation involving a mixed sequestration/cystic adenomatoid malformation: a case report.

    The authors report the case of a baby girl with an unusual communicating bronchopulmonary foregut malformation consisting of extralobar pulmonary sequestration and cystic adenomatoid malformation. A well-formed bronchus was the communication between the sequestration and lower esophagus.
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7/19. Bronchopulmonary foregut malformation: a large bronchogenic cyst communicating with an esophageal duplication cyst.

    A 1-year-old boy with a bronchopulmonary foregut malformation presented with a large mediastinal bronchogenic cyst associated with pulmonary sequestration, a cervical esophageal duplication cyst, a bronchial communication between these cysts, and 2 small bronchogenic cysts around the communication. These lesions were resected followed by an uneventful recovery.
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8/19. Sclerosing haemangioma arising within extralobar pulmonary sequestration.

    Extralobar pulmonary sequestration is a rare anomaly of abnormal pulmonary tissue without any communication to the bronchial tree. Sclerosing haemangioma is a rare lung tumour, generally seen in middle-aged women. The combination of these two rare pathologies has not been previously reported. We describe the CT and CT angiographic findings of sclerosing haemangioma arising within an extralobar pulmonary sequestration in a 2-year-old girl.
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9/19. Pulmonary sequestration presenting as fetal hydrops.

    Pulmonary sequestration is a rare developmental anomaly of the lung characterized by nonfunctional pulmonary tissue without communication with the tracheobronchial tree and receiving an aberrant systemic arterial blood supply. Few cases of non-immune hydrops fetalis associated with this entity have been reported. A 2 day old male baby born by cesarean section at 31 weeks gestational age due to fetal hydrops is presented. autopsy revealed a hydropic baby with extralobar pulmonary sequestration and bilateral pulmonary hypoplasia. The clinicopathologic presentation of this unusual pulmonary developmental anomaly is discussed.
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10/19. Pulmonary sequestration and aspergillosis.

    OBJECTIVE: Pulmonary sequestration (PS) is an uncommon congenital disease. Symptoms when present are usually secondary to pyogenic infection. Our objective was to draw attention on superimposed fungal infection. methods: During the last 20 years, we operated upon 19 intralobar PS. Four of six patients operated during the last decade proved to have intralobar PS containing aspergillus. RESULTS: In one patient aspergillosis presented as an aspergilloma and communications between the sequestration and small bronchi were present. In another patient diagnosis was made before operation because of positive precipitins test. In two patients aspegillosis was discovered by the pathologist. CONCLUSIONS: review of literature demonstrates PS fungal colonization to be a rare but recently reported entity (14 cases reported). The observation of four consecutive patients leads us to suggest that PS aspergillosis must be considered in order to evaluate its incidence.
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