1/5. 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.- - - - - - - - - - ranking = 1keywords = scimitar syndrome, scimitar (Clic here for more details about this article) |
2/5. Pulmonary sequestration--a review of 8 cases treated with lobectomy.BACKGROUND AND AIMS: Pulmonary sequestration (PS) is a rare congenital malformation where non-functioning lung tissue is separated from the bronchial tree and vascularised with an aberrant artery from the systemic circulation. The aim of this report was to study all patients who were treated for PS at Lund University Hospital between 1994 and 2004, with emphasis on clinical presentation of the disease and evaluate the results of surgical treatment. MATERIAL AND methods: 8 cases were identified, 7 females and one male, with a mean age of 7.3 years (range 25 days -17 years) at the time of diagnosis. RESULTS: Out of 8 patients, seven presented with respiratory symptoms and two with congestive heart failure. Five patients had other congenitial malformations; including scimitar syndrome and congenital heart disease. All the patients underwent a successful lobectomy. There were no major postoperative complications. At a medium follow-up of 77 months all of the fully treated children were doing well. CONCLUSION: Respiratory and cardiovascular symptoms are the most common symptoms related to PS. The wide range of clinical symptoms may cause diagnostic problems, especially in children and young adults with concomitant congenital heart disease. Therefore PS should be considered as a differential diagnosis in children with unexplained respiratory symptoms or with signs of congestive heart failure. In patients with PS, lobectomy seems to be a good therapeutic option.- - - - - - - - - - ranking = 1keywords = scimitar syndrome, scimitar (Clic here for more details about this article) |
3/5. scimitar syndrome and associated pulmonary sequestration: report of a successfully corrected case.A case of scimitar syndrome with pulmonary sequestration is reported. Anomalous pulmonary venous return from the right lung to the infradiaphragmatic vena cava inferior was diagnosed by pulmonary angiogram and sequestration of the right lower lobe was confirmed by aortogram. Venous return from the sequestrated lung was partly into the vena cava inferior and partly into the left atrium. Successful repair was achieved by resection of the sequestrated lobe and direct reimplantation of the scimitar vein into the left atrium. Accurate preoperative diagnosis and intraoperative evaluation of the anatomy is mandatory to correct this rare anomaly.- - - - - - - - - - ranking = 1.1978679116342keywords = scimitar syndrome, scimitar (Clic here for more details about this article) |
4/5. Scimitar sign with normal pulmonary venous drainage and systemic arterial supply. scimitar syndrome or bronchopulmonary sequestration?The classic definition of the scimitar syndrome is a triad of hypoplasia of the right lung with anomalous venous drainage and a systemic arterial supply of a variable degree. We report a case in which a scimitar-shaped anomalous vein was observed on the plain chest radiograph, but subsequently a pulmonary angiogram showed that it drained normally into the left atrium.- - - - - - - - - - ranking = 1.1978679116342keywords = scimitar syndrome, scimitar (Clic here for more details about this article) |
5/5. Shoe, scimitar or sequestration: a shifting spectrum.congenital abnormalities of the tracheobronchial tree are rare causes of recurrent chest infections in childhood. A case is described which shows some typical features of horseshoe lung. More detailed imaging revealed complete separation of the right and left pleural cavities and the malformation represents part of the sequestration spectrum. The case emphasises the need for careful evaluation of the pulmonary arteries, veins, bronchi and oesophagus, particularly prior to surgery.- - - - - - - - - - ranking = 0.79147164653664keywords = scimitar (Clic here for more details about this article) |