M4p to mp3 converter mac. This new edition of 'Squire's Fundamentals of Radiology' describes and illustrates these new techniques to prepare medical students and other radiology learners to provide the most optimal and up-to-date imaging management for their patients. Not only are new diagnostic techniques outlined, such as the multidetector computed tomography.
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Andrew Murphy ◉ and A.Prof Frank Gaillard ◉ ◈ et al.
Scimitar syndrome,also known as hypogenetic lung syndrome,is characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.
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Clinical presentation
Infants present with signs of heart failure, which may be due to associated congenital heart disease 13. Adults are most frequently asymptomatic but may have recurrent pulmonary infection or dyspnea on exertion.
Pathology
It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). It almost exclusively occurs on the right side.
Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into:
- inferior vena cava: most common
In some variants, the abnormal lung segment may receive arterial blood supply from the aorta or its branches 1,4,11,12. Some cases may show the anomalous systemic arteries supplying an area of associated pulmonary sequestration 11.
Associations
- congenital heart disease, e.g. ASD, VSD, tetralogy of Fallot, patent ductus arteriosus
- ipsilateral diaphragmatic anomalies, e.g. accessory diaphragm, diaphragmatic hernia
- vertebral anomalies, e.g. hemivertebrae 4
- genitourinary tract abnormalities 4
- pulmonary sequestration10
Radiographic features
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography.
Plain radiograph
Chest radiographic findings are that of a small, hypoplastic lung with ipsilateral mediastinal shift, and in one-third of cases, the anomalous draining vein may be seen as a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). The right heart border may be blurred.
Treatment and prognosis
Surgical correction should be considered in the presence of significant left to right shunting and pulmonary hypertension. This involves the creation of an interatrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.
Complications
The presence of a left-to-right shunt may lead to the development of pulmonary hypertension and Eisenmenger phenomenon.
History and etymology
The term was first given by C A Neill et al. in 1960 5.
Differential diagnosis
General imaging differential considerations include:
Squire S Radiology Pdf Free
- right middle lobe atelectasis (on AP plain radiograph)
See also
- 1. Dahnert WF. Radiology Review Manual. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 2. Melduni RM, Mookadam F, Mulligan ME. Scimitar syndrome: another one for the radiologists. Radiology. 2006;241 (2): 629. doi:10.1148/radiol.2412051952 - Pubmed citation
- 2. Olson MA, Becker GJ. The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return. Radiology. 1986;159 (1): 25-6. Radiology (citation) - Pubmed citation
- 4. Berrocal T, Madrid C, Novo S et-al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24 (1): e17. doi:10.1148/rg.e17 - Pubmed citation
- 5. Schramel FM, Westermann CJ, Knaepen PJ et-al. The scimitar syndrome: clinical spectrum and surgical treatment. Eur. Respir. J. 1995;8 (2): 196-201. Eur. Respir. J. (link) - Pubmed citation
- 6. Baskar karthekeyan R, Saldanha R, Sahadevan MR et-al. Scimitar syndrome: experience with 6 patients. Asian Cardiovasc Thorac Ann. 2009;17 (3): 266-71. doi:10.1177/0218492309104750 - Pubmed citation
- 7. Woodring JH, Howard TA, Kanga JF. Congenital pulmonary venolobar syndrome revisited. Radiographics. 1994;14 (2): 349-69. doi:10.1148/radiographics.14.2.8190958 - Pubmed citation
- 8. Konen E, Raviv-Zilka L, Cohen RA et-al. Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting. Radiographics. 2003;23 (5): 1175-84. doi:10.1148/rg.235035004 - Pubmed citation
- 9. Casha AR, Sulaiman M, Cale AJ. Repair of adult Scimitar syndrome with an intra-atrial conduit. Interact Cardiovasc Thorac Surg. 2003;2 (2): 128-30. doi:10.1016/S1569-9293(02)00120-2 - Pubmed citation
- 10. Vida VL, Padalino MA, Boccuzzo G et-al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122 (12): 1159-66. doi:10.1161/CIRCULATIONAHA.109.926204 - Pubmed citation
- 11. Do KH, Goo JM, Im JG, Kim KW, Chung JW, Park JH. Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics : a review publication of the Radiological Society of North America, Inc. 21 (2): 387-402. doi:10.1148/radiographics.21.2.g01mr06387 - Pubmed
- 12. Tortoriello TA, Vick GW, Chung T, Bezold LI, Vincent JA. Meandering right pulmonary vein to the left atrium and inferior vena cava: the first case with associated anomalies. Texas Heart Institute journal. 29 (4): 319-23. Pubmed
- 13. Gupta ML, Bagarhatta R, Sinha J. Scimitar syndrome: A rare disease with unusual presentation. (2009) Lung India : official organ of Indian Chest Society. 26 (1): 26-9. doi:10.4103/0970-2113.45202 - Pubmed
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