Case Report
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Sol izomerizmim çekum malpozisyonu, dorsal pankreas agenezi ve reroaortik sol renal ven varlığı ile birlikteliği: Olgu sunumu

Year 2019, Volume: 3 Issue: 11, 812 - 814, 01.11.2019
https://doi.org/10.28982/josam.573375

Abstract

Polispleni / heterotaksi sendromu, torasik ve abdominal organların yanlış dağılımı sonucu olarak ortaya çıkan çok nadir görülen bir durumdur. Farklı klinik özelliklere ve anatomik varyasyonlara sahip iki grupta incelenmiştir; sağ ve sol izomerizm. Sağ yan ağrısı ve böbrek taşı şüphesi nedeniyle kontrastsız bilgisayarlı tomografi (BT) çekimi yapılan 47 yaşındaki kadın hastada, sol izomerizm – polispleni sendromu ve eşlik eden abdominal organ varyasyonları saptandı. Abdominal BT incelemesinde sağ üst kadranda yerleşimli mide ve çok sayıda dalak, orta hat yerleşimli karaciğer tespit edildi. İnferior vena kava devamlılığında kesilme ve azigos devamlılığı tespit edildi. Hepatik venler doğrudan sağ atriyuma drene oluyordu. Tespit edilen bulgular, polispleni-sol izomerizm ile uyumlu olarak değerlendirildi. Retroaortik sol renal ven, çekum malpozisyonu ve dorsal pankreas agenezisine izomerizm eşlik ediyordu. Literatürde ayrı ayrı rapor edilen sol izomerizme eşlike eden retroaortik sol renal ven ve dorsal pankreas agenezisi olgu sunumları mevcuttu. Ancak bu üç anomalinin eşlik ettiği olgu sunumu yoktu ve bu özellikleriyle olgumuz tekdi. Anatomik varyasyonları ve klinik durumu bilmek yanlış tanıyı önleyebilir ve olası bir cerrahi işlemin planlanmasında görüntüleme bulgularını bilmek çok önemlidir.

References

  • 1. Jung JE, Hur JH, Jung MK, Kwon A, Chae HW, Kim DH, et al. Diabetes mellitus due to agenesis of the dorsal pancreas in a patient with heterotaxy syndrome. Ann Pediatr Endocrinol Metab. 2017 Jun;22(2):125-8.
  • 2. Gupta AC, Herts B. Heterotaxia with polysplenia. J Urol. 2015 Sep;194(3):801-2
  • 3. Yildiz AE, Ariyurek MO, Karcaaltincaba M. Splenic anomalies of shape, size, and location: pictorial essay. Scientific World Journal. 2013 Apr 21;2013:321810.
  • 4. De Wailly P, Metzler P, Sautot-Vial N, Olivier D, Meunier B, Faure JP. Pre-duodenal portal vein in polysplenia syndrome: clinical effects and surgical application. Surg Radiol Anat. 2011 Jul;33(5):451-4.
  • 5. Gupta R, Soni V, Valse PD, Goyal RB, Gupta AK, Mathur P. Neonatal intestinal obstruction associated with situs inversus totalis: two case reports and a review of the literature. J Med Case Rep. 2017 Sep 18;11(1):264.
  • 6. Douard R, Feldman A, Bargy F, Loric S, Delmas V. Anomalies of lateralization in man: a case of total situs inversus. Surg Radiol Anat. 2000;22(5-6):293-7.
  • 7. Varga I, Babala J, Kachlik D. Anatomic variations of the spleen: current state of terminology, classification, and embryological background. Surg Radiol Anat. 2018 Jan;40(1):21-9.
  • 8. Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol. 2008 Nov 7;14(41):6418-20.
  • 9. Pagkratis S, Kryeziu S, Lin M, Hoque S, Bucobo JC, Buscaglia JM, et al. Case report of intestinal non-rotation, heterotaxy, and polysplenia in a patient with pancreatic cancer. Medicine (Baltimore). 2017 Dec;96(49):e8599.

Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report

Year 2019, Volume: 3 Issue: 11, 812 - 814, 01.11.2019
https://doi.org/10.28982/josam.573375

Abstract

Polysplenia / heterotaxy syndrome is a very rare condition that occurs as a result of the maldistribution of the thoracic and abdominal organs. It is examined in two groups with different clinical features and anatomical variations: right and left isomerism. This paper reports the case of a 47-year-old female patient who underwent a non-contrast computed tomography (CT) examination due to right flank pain and suspicion of a renal stone and presented with the findings of left isomerism; i.e., accompanied by intraabdominal variations. On abdominal CT examination, stomach and multiple spleen were localized in the right upper quadrant, and the liver had a midline localization. The interruption of the inferior vena cava with azygos continuation were also detected. The hepatic veins were draining directly into the right atrium. In the literature, a retroaortic left renal vein and dorsal pancreatic agenesis have been individually reported to coexist with left isomerism, but no other case of the coincidence of these three anomalies has been described, which makes the current case report significant. Having knowledge of anatomical variations and clinical status can prevent misdiagnosis, and imaging findings are crucial in the planning of a possible surgical procedure.

References

  • 1. Jung JE, Hur JH, Jung MK, Kwon A, Chae HW, Kim DH, et al. Diabetes mellitus due to agenesis of the dorsal pancreas in a patient with heterotaxy syndrome. Ann Pediatr Endocrinol Metab. 2017 Jun;22(2):125-8.
  • 2. Gupta AC, Herts B. Heterotaxia with polysplenia. J Urol. 2015 Sep;194(3):801-2
  • 3. Yildiz AE, Ariyurek MO, Karcaaltincaba M. Splenic anomalies of shape, size, and location: pictorial essay. Scientific World Journal. 2013 Apr 21;2013:321810.
  • 4. De Wailly P, Metzler P, Sautot-Vial N, Olivier D, Meunier B, Faure JP. Pre-duodenal portal vein in polysplenia syndrome: clinical effects and surgical application. Surg Radiol Anat. 2011 Jul;33(5):451-4.
  • 5. Gupta R, Soni V, Valse PD, Goyal RB, Gupta AK, Mathur P. Neonatal intestinal obstruction associated with situs inversus totalis: two case reports and a review of the literature. J Med Case Rep. 2017 Sep 18;11(1):264.
  • 6. Douard R, Feldman A, Bargy F, Loric S, Delmas V. Anomalies of lateralization in man: a case of total situs inversus. Surg Radiol Anat. 2000;22(5-6):293-7.
  • 7. Varga I, Babala J, Kachlik D. Anatomic variations of the spleen: current state of terminology, classification, and embryological background. Surg Radiol Anat. 2018 Jan;40(1):21-9.
  • 8. Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome with preduodenal portal vein detected in adults. World J Gastroenterol. 2008 Nov 7;14(41):6418-20.
  • 9. Pagkratis S, Kryeziu S, Lin M, Hoque S, Bucobo JC, Buscaglia JM, et al. Case report of intestinal non-rotation, heterotaxy, and polysplenia in a patient with pancreatic cancer. Medicine (Baltimore). 2017 Dec;96(49):e8599.
There are 9 citations in total.

Details

Primary Language English
Subjects Radiology and Organ Imaging
Journal Section Case report
Authors

Emre Emekli 0000-0001-5989-1897

Elif Gündoğdu 0000-0002-1729-6958

Mahmut Kebapçı This is me 0000-0002-2856-9923

Publication Date November 1, 2019
Published in Issue Year 2019 Volume: 3 Issue: 11

Cite

APA Emekli, E., Gündoğdu, E., & Kebapçı, M. (2019). Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report. Journal of Surgery and Medicine, 3(11), 812-814. https://doi.org/10.28982/josam.573375
AMA Emekli E, Gündoğdu E, Kebapçı M. Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report. J Surg Med. November 2019;3(11):812-814. doi:10.28982/josam.573375
Chicago Emekli, Emre, Elif Gündoğdu, and Mahmut Kebapçı. “Coincidence of Left Isomerism, Malposition of Cecum, Dorsal Pancreatic Agenesis, and Retroaortic Left Renal Vein: A Case Report”. Journal of Surgery and Medicine 3, no. 11 (November 2019): 812-14. https://doi.org/10.28982/josam.573375.
EndNote Emekli E, Gündoğdu E, Kebapçı M (November 1, 2019) Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report. Journal of Surgery and Medicine 3 11 812–814.
IEEE E. Emekli, E. Gündoğdu, and M. Kebapçı, “Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report”, J Surg Med, vol. 3, no. 11, pp. 812–814, 2019, doi: 10.28982/josam.573375.
ISNAD Emekli, Emre et al. “Coincidence of Left Isomerism, Malposition of Cecum, Dorsal Pancreatic Agenesis, and Retroaortic Left Renal Vein: A Case Report”. Journal of Surgery and Medicine 3/11 (November 2019), 812-814. https://doi.org/10.28982/josam.573375.
JAMA Emekli E, Gündoğdu E, Kebapçı M. Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report. J Surg Med. 2019;3:812–814.
MLA Emekli, Emre et al. “Coincidence of Left Isomerism, Malposition of Cecum, Dorsal Pancreatic Agenesis, and Retroaortic Left Renal Vein: A Case Report”. Journal of Surgery and Medicine, vol. 3, no. 11, 2019, pp. 812-4, doi:10.28982/josam.573375.
Vancouver Emekli E, Gündoğdu E, Kebapçı M. Coincidence of left isomerism, malposition of cecum, dorsal pancreatic agenesis, and retroaortic left renal vein: A case report. J Surg Med. 2019;3(11):812-4.