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1/71. bronchopulmonary sequestration and dextrocardia.

    bronchopulmonary sequestration (BPS) is usually a rare congenital anomaly, which is most frequently extralobar or intralobar. The case of a patient with positional congenital anomaly--dextrocardia (situs thoracalis inversus) and intrapulmonary sequestration (IPS) is presented. Clinical and radiological characteristics of EPS and IPS are discussed, and new combinations of congenital anomalies with bronchopulmonary sequestration are described, dextrocardia and intrapulmonary sequestration. The importance of the algorithm of diagnostic examinations is emphasized, from detection of bronchopulmonary sequestration on the chest roentgenogram to establishing a definite diagnosis by means of angiography.
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keywords = bronchopulmonary
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2/71. Radiologic differential diagnosis. Radiologic pattern: solitary cavity.

    The differential diagnosis of a left lower lobe cavity in this young patient with a history of productive cough should include hiatal hernia, pulmonary abscess, bronchiectatic cyst and bronchopulmonary sequestration. Hiatal hernia should be ruled out by barium swallow; acute pulmonary abscess by the lack of a history suggestive of a necrotizing pneumonia; bronchiectasis by bronchogram; and intralobar bronchopulmonary sequestration should be confirmed by aortography.
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keywords = bronchopulmonary
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3/71. Direct intrauterine fetal therapy in a case of bronchopulmonary sequestration associated with non-immune hydrops fetalis.

    bronchopulmonary sequestration associated with non-immune hydrops fetalis is generally recognized as a uniformly fatal fetal condition without fetal surgical intervention. We describe here the first case of such a condition treated successfully with direct intrauterine fetal therapy using digoxin and frusemide.
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ranking = 2
keywords = bronchopulmonary
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4/71. 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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ranking = 2
keywords = bronchopulmonary
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5/71. Pulmonary sequestration diagnosed by contrast enhanced three-dimensional MR angiography.

    Pulmonary sequestration is a congenital bronchopulmonary foregut malformation in which a segment of lung parenchyma is not connected to the tracheobronchial tree. This abnormal segment receives a blood supply from the systemic circulation. Multiple imaging modalities have been used to demonstrate the vascular anatomy of the sequestration. Different magnetic resonance angiography (MRA) techniques have been employed in the identification of these anomalous vessels. We report a case of pulmonary sequestration diagnosed by MRI with the use of contrast enhanced three-dimensional MRA.
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ranking = 0.5
keywords = bronchopulmonary
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6/71. Extralobar pulmonary sequestration receiving its arterial supply from the innominate artery.

    Pulmonary sequestration is a rare bronchopulmonary foregut malformation, for which the arterial blood supply is usually derived from the descending thoracic or abdominal aorta. A 5-week-old infant is described with an extralobar pulmonary sequestration supplied by a large artery originating from the innominate artery, an arrangement only described once previously. Helical CT scan now makes arteriography unnecessary in the evaluation of these malformations.
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ranking = 0.5
keywords = bronchopulmonary
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7/71. Intralobar bronchopulmonary sequestration evaluated by contrast-enhanced three-dimensional MR angiography.

    bronchopulmonary sequestration (PS) is characterized by non-functioning lung tissue fed from one or several aberrant systemic arteries. The condition is diagnosed by visualizing the feeding arteries using non-invasive CT, MRI, colour Doppler sonography or conventional angiography. We present a 5-year-old boy in whom intralobar sequestration was diagnosed using contrast-enhanced 3D MR angiography, which visualised fine blood vessels in the thoraco-abdominal region without arterial puncture. This technique is useful for diagnosing PS.
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ranking = 2
keywords = bronchopulmonary
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8/71. association of bronchopulmonary sequestration with expression of the homeobox protein Hoxb-5.

    bronchopulmonary sequestration (BPS) is caused by the abnormal development of an accessory lung diverticulum from the foregut very early in embryogenesis. The developmental abnormalities seen with BPS suggest that this anomaly is caused by abnormal expression of homeobox genes, which control axial identity and organ-specific patterning during embryogenesis. The authors previously have shown that the homeobox gene Hoxb-5 is necessary for normal airway branching during lung development. The authors now report that BPS is associated with aberrant developmental expression of Hoxb-5 protein, suggesting that this Hox gene is involved in the development of BPS.
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ranking = 2
keywords = bronchopulmonary
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9/71. Bronchial trifurcation in a congenital pulmonary venolobar syndrome.

    Congenital malformations of the tracheobronchal tree and the related arterial blood supply are a complex group of lesions in which there are abnormalities of the venous drainage and lung parenchyma. These malformations are examples of congenital pulmonary venolobar syndrome (CPVS). Tracheal trifurcation is an extremely rare anomaly associated with CPVS. We report on an unusual case of lower right extralobar sequestration connected to the trachea, plus a type I posterior laryngeal cleft, an aberrant systemic artery, and an anomalous route of the phrenic nerve. This paper discusses the place of this unusual abnormality in the spectrum of congenital bronchopulmonary vascular malformations.
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ranking = 0.5
keywords = bronchopulmonary
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10/71. 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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ranking = 3
keywords = bronchopulmonary
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