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SCİMİTAR SENDROMU

Year 2017, Volume: 31 Issue: 3, 193 - 196, 01.12.2017

Abstract

Scimitar sendromu sağ pulmoner venin, vena cava inferior veya sağ atriuma açılmasıyla karakterize nadir görülen bir pulmoner venöz dönüş anomalisidir. Sağ akciğer hipoplazisi, pulmoner sekestrasyon, atriyal septal defekt (ASD) ve kalbin dextropozisyonu eşlik eden diğer anomalilerdir. Olgu: 36 yasında bayan hasta halsizlik ve nefes darlığında 2 aydır artma şikayetiyle göğüs hastalıkları polikliniğimize başvurdu. Hastanın çocukluk çağından beri birkaç kez öksürük ve nefes darlığı ile hastaneye basvurusu olduğu öğrenildi. Hastanın başvurusunda yapılan fizik muayenesinde akciğer ve kalp sesleri normal olarak değerlendirildi. Çekilen akciğer filminde sağ akciğer parakardiyak alanda opasite artışı görüldü. Bilgisayarlı tomografik anjiyografide (BT anjografi) sağ alt ve üst pulmoner venlerin doğrudan hepatik ven- inferior vena cava düzeyine açıldığı görüldü. Olgu radyolojik olarak Scimitar Sendromu olarak tanı aldı. Nadir görülen bir pulmoner venöz dönüş anomalisi olması nedeniyle literatür eşliğinde sunuldu.

References

  • 1. Sinzig M, Scheer J, Willi UV. LangerGiedionsyndromeassociatedwithscimitarsyndro me. Pediatr Radiol 1999; 29: 218-20
  • 2. Fraser RS, Müller NL, Colman N, Parè PD. Diagnosis of Diseases of theChest. 4th ed. Philadelphia: WB SaundersCompany, 1999: 653-5.
  • 3. Geggel RL. Scimitar syndrome associated with parti alanomalous pulmonary venous connection at the supracardiac, cardiacan dinfracardiac levels. Pediatr Cardiol 1993; 14: 234-7
  • 4. Dupuis C, Charaf LAC, Brevière GM, et al. Theadult form of scimitarsyndrome. Am J Cardiol 1992; 70: 502-7.
  • 5. Foreman MG, Rosa U. Thescimitarsyndrome. SouthernMed J 1991; 84: 489-93.
  • 6. Takeda S, Imachi T, Arimitsu K, et al. Twocases of scimitarvariant. Chest 1994; 105: 292-3.
  • 7. Cooper G. Case of malformation of the thoracicvis cera: consisting of imperfect development of rightlungandtransposition of theheart. LondonMedGazzette 1836; 18: 600-1.
  • 8. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial suppl yand venousdrainage “scimitarsyndrome.’’ Bull Johns Hopkins Hosp 1960; 107;7:1-21.
  • 9. Midyat L, Demir E, Aşkın M, Gülen F, Ülger Z, Tanaç R, et al. Eponym. Scimitarsyndrome. Eur J Pediatr 2010;169:1171-7.
  • 10. Juraszek AL, Cohn H, Van Praagh R, Van Praagh S. Isolatedleft-sideds cimitar vein connecting allleft pulmonary veins to therightinferior vena cava. Pediatr Cardiol 2005;26:846-7.
  • 11. Gudjonsson U, Brown JW. Scimitarsyndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006; 9:56-62.
  • 12. Schramel FM, Westermann CJ, Knaepen PJ, van den Bosch JM. Thescimitarsyndrome: clinical spectrum and surgicaltreatment. EurRespir J 1995;8:196-201.
  • 13. Dupuis C, Charaf LA, Brevière GM, Abou P. “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 1993;71:1326-30.
  • 14. Clements BS, Warner JO. Pulmonary seques trationandrelatedcongenitalbronchopulmonaryvascularmalformations: nomen clature and class ification based on anatomic alan dem bryological considerations. Thorax 1987; 42:401–8
  • 15. Osama Elsallabi, Aiman Smer, Bradley De Vrieze, Gopi Sirineni Scimitar Syndrome Journal of General Internal Medicine February 2016;31(2): 253–4

SCİMİTAR SYNDROME

Year 2017, Volume: 31 Issue: 3, 193 - 196, 01.12.2017

Abstract

Scimitar Syndrome is a rare congenital disorder that is characterised by an anomalous connection of the pulmonary vein with the inferior vena cava. The other components of the syndrome include hypoplasia of the right lung, hypoplasia of the right pulmonary artery, pulmonary sequestration, dextrocardia, atrial septal defect (ASD). A 36 years old woman suffering from weakness and shortness of breath was admitted in our clinic. It has been learned that the patient has been referred to the hospital with coughing and shortness of breath several times since childhood. Physical examination was normal. The chest radiograh demonstrated increased opacity along the right paracardiac area. Computed tomography angiography (CT angiography) was evaluated. It was detected that the right pulmonary veins drained in to the inferior vena cava named as Scimitar Syndrome. This case was presented with the review of literature.

References

  • 1. Sinzig M, Scheer J, Willi UV. LangerGiedionsyndromeassociatedwithscimitarsyndro me. Pediatr Radiol 1999; 29: 218-20
  • 2. Fraser RS, Müller NL, Colman N, Parè PD. Diagnosis of Diseases of theChest. 4th ed. Philadelphia: WB SaundersCompany, 1999: 653-5.
  • 3. Geggel RL. Scimitar syndrome associated with parti alanomalous pulmonary venous connection at the supracardiac, cardiacan dinfracardiac levels. Pediatr Cardiol 1993; 14: 234-7
  • 4. Dupuis C, Charaf LAC, Brevière GM, et al. Theadult form of scimitarsyndrome. Am J Cardiol 1992; 70: 502-7.
  • 5. Foreman MG, Rosa U. Thescimitarsyndrome. SouthernMed J 1991; 84: 489-93.
  • 6. Takeda S, Imachi T, Arimitsu K, et al. Twocases of scimitarvariant. Chest 1994; 105: 292-3.
  • 7. Cooper G. Case of malformation of the thoracicvis cera: consisting of imperfect development of rightlungandtransposition of theheart. LondonMedGazzette 1836; 18: 600-1.
  • 8. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial suppl yand venousdrainage “scimitarsyndrome.’’ Bull Johns Hopkins Hosp 1960; 107;7:1-21.
  • 9. Midyat L, Demir E, Aşkın M, Gülen F, Ülger Z, Tanaç R, et al. Eponym. Scimitarsyndrome. Eur J Pediatr 2010;169:1171-7.
  • 10. Juraszek AL, Cohn H, Van Praagh R, Van Praagh S. Isolatedleft-sideds cimitar vein connecting allleft pulmonary veins to therightinferior vena cava. Pediatr Cardiol 2005;26:846-7.
  • 11. Gudjonsson U, Brown JW. Scimitarsyndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006; 9:56-62.
  • 12. Schramel FM, Westermann CJ, Knaepen PJ, van den Bosch JM. Thescimitarsyndrome: clinical spectrum and surgicaltreatment. EurRespir J 1995;8:196-201.
  • 13. Dupuis C, Charaf LA, Brevière GM, Abou P. “Infantile” form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol 1993;71:1326-30.
  • 14. Clements BS, Warner JO. Pulmonary seques trationandrelatedcongenitalbronchopulmonaryvascularmalformations: nomen clature and class ification based on anatomic alan dem bryological considerations. Thorax 1987; 42:401–8
  • 15. Osama Elsallabi, Aiman Smer, Bradley De Vrieze, Gopi Sirineni Scimitar Syndrome Journal of General Internal Medicine February 2016;31(2): 253–4
There are 15 citations in total.

Details

Other ID JA42MF49TV
Journal Section Case Report
Authors

Seda Beyhan Sağmen This is me

Coşkun Doğan This is me

Sevda Cömert This is me

Elif Torun Parmaksız This is me

Banu Salepçi This is me

Ali Fidan This is me

Publication Date December 1, 2017
Published in Issue Year 2017 Volume: 31 Issue: 3

Cite

APA Sağmen, S. B., Doğan, C., Cömert, S., Parmaksız, E. T., et al. (2017). SCİMİTAR SENDROMU. İzmir Göğüs Hastanesi Dergisi, 31(3), 193-196.