situs inversus

Summary

Summary: A congenital abnormality in which organs in the THORAX and the ABDOMEN are opposite to their normal positions (situs solitus) due to lateral transposition. Normally the STOMACH and SPLEEN are on the left, LIVER on the right, the three-lobed right lung is on the right, and the two-lobed left lung on the left. Situs inversus has a familial pattern and has been associated with a number of genes related to microtubule-associated proteins.

Top Publications

  1. Choi D, Park J, Kim B, Han K, Hong C, Sohn D, et al. Colonoscopy in situs inversus totalis patients. Am J Gastroenterol. 2008;103:1311-2 pubmed publisher
  2. Bergmann C, Fliegauf M, Brüchle N, Frank V, Olbrich H, Kirschner J, et al. Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia. Am J Hum Genet. 2008;82:959-70 pubmed publisher
    ..is responsible for the cystic kidney disease phenotype, whereas complete loss of Nphp3 function results in situs inversus, congenital heart defects, and embryonic lethality in mice...
  3. Galiatsatos P, Kasprzak L, Chong G, Jass J, Foulkes W. Multiple primary malignancies in a patient with situs ambiguus. Clin Genet. 2006;69:528-31 pubmed
  4. Shiraishi K, Ono N, Eguchi S, Mohri J, Kamiryo Y, Takihara H. Young's syndrome associated with situs inversus totalis. Arch Androl. 2004;50:169-72 pubmed
    ..A 34-year old man with bronchectasis and situs inversus totalis was investigated because of azoospermia...
  5. Hoyos S, Guzman C, Correa G, Restrepo J, Franco H, Cardenas A. Orthotopic liver transplantation in an adult with situs inversus: an easy way to fit the liver. Ann Hepatol. 2006;5:53-5 pubmed
    b>Situs inversus (SI) is a rare congenital disorder with a complete mirror image of thoracic and abdominal organs. In adults with SI and decompensated cirrhosis experience with liver transplantation is limited...
  6. Frank L, Yu Q, Francis R, Tian X, Samtani R, Sahn D, et al. Ventricular rotation is independent of cardiac looping: a study in mice with situs inversus totalis using speckle-tracking echocardiography. J Am Soc Echocardiogr. 2010;23:315-23 pubmed publisher
    ..fide model of primary ciliary dyskinesia, with surviving homozygous mice showing either situs solitus (SS) or situs inversus totalis (SI)...
  7. Aydinoz S, Ersen A, Karademir F, Suleymanoglu S, Ozkaya H, Gocmen I. A case of cerebello-oculo-renal syndrome with situs inversus totalis: a new phenotype. J Child Neurol. 2007;22:204-7 pubmed
    ..The authors present a case of cerebello-oculo-renal syndrome with situs inversus totalis from a consanguineous family...
  8. Perles Z, Cinnamon Y, Ta Shma A, Shaag A, Einbinder T, Rein A, et al. A human laterality disorder associated with recessive CCDC11 mutation. J Med Genet. 2012;49:386-90 pubmed publisher
    ..processes that lead to laterality defects, such as heterotaxy syndrome (HS, also known as situs ambiguous) and situs inversus totalis (SIT)...
  9. Sutherland M, Ware S. Disorders of left-right asymmetry: heterotaxy and situs inversus. Am J Med Genet C Semin Med Genet. 2009;151C:307-17 pubmed publisher
    ..This review focuses on the clinical manifestations, molecular mechanisms, and human genetics of these disorders of laterality. ..

More Information

Publications62

  1. Vogel P, Read R, Hansen G, Freay L, Zambrowicz B, Sands A. Situs inversus in Dpcd/Poll-/-, Nme7-/- , and Pkd1l1-/- mice. Vet Pathol. 2010;47:120-31 pubmed publisher
    b>Situs inversus (SI) is a congenital condition characterized by left-right transposition of thoracic and visceral organs and associated vasculature...
  2. Sparrow D, Faqeih E, Sallout B, Alswaid A, Ababneh F, Al Sayed M, et al. Mutation of HES7 in a large extended family with spondylocostal dysostosis and dextrocardia with situs inversus. Am J Med Genet A. 2013;161A:2244-9 pubmed publisher
    ..Mutation of genes in the Notch signaling pathway cause SCD types 1-5. Dextrocardia with situs inversus is a rare congenital malformation in which the thoracic and abdominal organs are mirror images of normal...
  3. Halasz Z, Bertalan R, Toke J, Patocs A, Toth M, Fekete G, et al. Laterality disturbance and hypopituitarism. A case report of co-existing situs inversus totalis and combined pituitary hormone deficiency. J Endocrinol Invest. 2008;31:74-8 pubmed
    ..of a 52-yr-old male patient with a unique association of combined pituitary hormone deficiency (CPHD) and situs inversus totalis...
  4. Aydin U, Unalp O, Yazici P, Gurcu B, Sozbilen M, Coker A. Laparoscopic cholecystectomy in a patient with situs inversus totalis. World J Gastroenterol. 2006;12:7717-9 pubmed
    ..What about performing this procedure on a patient with situs inversus totalis and what are the difficulties of this operation for a right-handed surgeon? We presented a 35-year-old ..
  5. Kim Y, Ryu H, Kim D, Kim I. Double primary malignancies associated with colon cancer in patients with situs inversus totalis: two case reports. World J Surg Oncol. 2011;9:109 pubmed publisher
    b>Situs inversus totalis (SIT) is not itself a premalignant condition, however, rare synchronous or metachronous multiple primary malignancies have been reported...
  6. Benhammane H, Kharmoum S, Terraz S, Berney T, Nguyen Tang T, Genevay M, et al. Common bile duct adenocarcinoma in a patient with situs inversus totalis: report of a rare case. BMC Res Notes. 2012;5:681 pubmed publisher
    b>Situs inversus totalis represents an unusual anomaly characterized by a mirror-image transposition of the abdominal and thoracic viscera...
  7. Kang S, Han H. Laparoscopic exploration of the common bile duct in a patient with situs inversus totalis. J Laparoendosc Adv Surg Tech A. 2004;14:103-6 pubmed
    b>Situs inversus totalis (SIT) is a rare anomaly that can present technical difficulties during laparoscopic surgery...
  8. McManus I, Martin N, Stubbings G, Chung E, Mitchison H. Handedness and situs inversus in primary ciliary dyskinesia. Proc Biol Sci. 2004;271:2579-82 pubmed
    ..primary ciliary dyskinesia (PCD) (also known as immotile cilia syndrome or Siewert-Kartagener syndrome) have situs inversus, which is thought to result from absent nodal ciliary rotation and failure of normal symmetry breaking...
  9. Koga H, Yamataka A, Kobayashi H, Lane G, Miyano T. Laparoscopy-assisted gastropexy for gastric volvulus in a child with situs inversus, asplenia, and major cardiac anomaly. J Laparoendosc Adv Surg Tech A. 2007;17:513-6 pubmed
    The aim of this study was to report on laparoscopy-assisted gastropexy in a child with situs inversus, asplenia, and major cardiac anomaly...
  10. Schertler T, Lardinois D, Boehm T, Weder W, Wildermuth S, Alkadhi H. Lung transplantation in Kartagener syndrome and situs inversus: potential of multidetector row computed tomography and three-dimensional postprocessing. J Thorac Cardiovasc Surg. 2007;134:814-5 pubmed
  11. Delhaas T, Kroon W, Bovendeerd P, Arts T. Left ventricular apical torsion and architecture are not inverted in situs inversus totalis. Prog Biophys Mol Biol. 2008;97:513-9 pubmed publisher
    Occasionally, individuals have a complete, mirror-image reversal of their internal organ position, called situs inversus totalis (SIT)...
  12. Atasoy H, Yavuz T, Altunrende S, Guven M, Kılıçgün A, Polat O, et al. A unique case of right-sided Poland syndrome with true dextrocardia and total situs inversus. Eur J Pediatr. 2013;172:269-72 pubmed publisher
    Poland syndrome has been reported to be associated with true dextrocardia, but not with true situs inversus. In this report, we describe the first patient with total situs inversus in medical literature and try to highlight the syndrome's ..
  13. Docimo G, Manzi F, Maione L, Canero A, Veneto F, Lo Schiavo F, et al. Case report: laparoscopic cholecystectomy in situs viscerum inversus. Hepatogastroenterology. 2004;51:958-60 pubmed
  14. Romano G, Grande G, Romano F, Di Luna G, Musto L, Saldutti L. [Laparoscopic cholecystectomy in situs viscerum inversus totalis: technical note]. G Chir. 2009;30:369-73 pubmed
    ..Laparoscopic cholecystectomy was performed. The Authors discuss the clinical case and the particular technique used. ..
  15. Hussain S, Ahmed W. Percutaneous transvenous mitral commissurotomy in mitral restenosis with situs inversus. J Coll Physicians Surg Pak. 2009;19:117-9 pubmed publisher
    A woman, aged 30 years with situs inversus, dextrocardia and severe rheumatic mitral restenosis was referred to AFIC/NIHD for commissurotomy...
  16. Kroon W, Delhaas T, Bovendeerd P, Arts T. Structure and torsion in the normal and situs inversus totalis cardiac left ventricle. II. Modeling cardiac adaptation to mechanical load. Am J Physiol Heart Circ Physiol. 2008;295:H202-10 pubmed publisher
    ..adaptation to mechanical load on their ability to estimate a realistic myocardial structure of the normal and situs inversus totalis (SIT) left ventricle (LV)...
  17. Oms L, Badia J. Laparoscopic cholecystectomy in situs inversus totalis: The importance of being left-handed. Surg Endosc. 2003;17:1859-61 pubmed
    ..standard procedure for the treatment of gallstone disease, several cases have been reported in patients with situs inversus. These cases require more technically demanding procedures due to the symmetrical disposition of the anatomy...
  18. Jobanputra S, Safar B, Wexner S. Laparoscopic diverticular resection with situs inversus totalis (SIT): report of a case. Surg Innov. 2007;14:284-6 pubmed publisher
    b>Situs inversus totalis (SIT) is a rare condition where the abdominal and thoracic cavity structures are opposite of their usual position...
  19. Iino K, Watanabe G, Ishikawa N, Tomita S. Total endoscopic robotic atrial septal defect repair in a patient with dextrocardia and situs inversus totalis. Interact Cardiovasc Thorac Surg. 2012;14:476-7 pubmed publisher
    b>Situs inversus with mirror-image of the heart is a rare condition. The present report describes a case of a patient with dextrocardia with situs inversus who had atrial septal defect with multiple holes in the fossa ovalis...
  20. Pitiakoudis M, Tsaroucha A, Katotomichelakis M, Polychronidis A, Simopoulos C. Laparoscopic cholecystectomy in a patient with situs inversus using ultrasonically activated coagulating scissors. Report of a case and review of the literature. Acta Chir Belg. 2005;105:114-7 pubmed
    b>Situs inversus totalis is a rare defect with a genetic predisposition, which can present difficulties in the management of abdominal pathology, especially in laparoscopic surgery, due to the mirror-image anatomy...
  21. Makiyama K, Sakata R, Yamanaka H, Tatenuma T, Sano F, Kubota Y. Laparoscopic nephroureterectomy in renal pelvic urothelial carcinoma with situs inversus totalis: preoperative training using a patient-specific simulator. Urology. 2012;80:1375-8 pubmed publisher
    ..the first case of preoperative training using a patient-specific laparoscopic simulator for a patient with situs inversus totalis (SIT)...
  22. Marion V, Stutzmann F, Gerard M, De Melo C, Schaefer E, Claussmann A, et al. Exome sequencing identifies mutations in LZTFL1, a BBSome and smoothened trafficking regulator, in a family with Bardet--Biedl syndrome with situs inversus and insertional polydactyly. J Med Genet. 2012;49:317-21 pubmed publisher
    ..The 16 BBS genes known to date are implied in the primary cilia related cellular pathways...
  23. Tan S, Rosenthal J, Zhao X, Francis R, Chatterjee B, Sabol S, et al. Heterotaxy and complex structural heart defects in a mutant mouse model of primary ciliary dyskinesia. J Clin Invest. 2007;117:3742-52 pubmed
    Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder associated with ciliary defects and situs inversus totalis, the complete mirror image reversal of internal organ situs (positioning)...
  24. Bohun C, Potts J, Casey B, Sandor G. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol. 2007;100:305-9 pubmed
    ..Twenty-seven cases were situs solitus, 30 situs inversus, and 24 situs ambiguous or isomerism...
  25. Afzelius B. Cilia-related diseases. J Pathol. 2004;204:470-7 pubmed
    ..malfunctioning, there is a random determination of asymmetry of the heart and visceral organs ('a 50% risk of situs inversus')...
  26. Lee S, Kim H, Jung S, Lee S, Park K, Kim W. Situs anomalies and gastrointestinal abnormalities. J Pediatr Surg. 2006;41:1237-42 pubmed
    ..The aim of the study was to review the gastrointestinal abnormalities occurring in association with situs anomalies...
  27. Witsberger T, Dismukes D, Kelmer E. Situs inversus totalis in a dog with a chronic diaphragmatic hernia. J Am Anim Hosp Assoc. 2009;45:245-8 pubmed
    b>Situs inversus totalis is a rare, congenital condition that is characterized by the development of the thoracic and abdominal viscera in a mirror image to their normal orientation...
  28. Goi T, Kawasaki M, Yamazaki T, Koneri K, Katayama K, Hirose K, et al. Ascending colon cancer with hepatic metastasis and cholecystolithiasis in a patient with situs inversus totalis without any expression of UVRAG mRNA: report of a case. Surg Today. 2003;33:702-6 pubmed
    b>Situs inversus totalis refers to an inverted position of part or all of the viscera, which represents a mirror-image of the normal location, and it is a relatively rare condition that occurs in one in 4,000-8,000 people in Japan...
  29. Jesudason S, Vyas F, Jesudason M, Govil S, Muthusami J. Laparoscopic cholecystectomy in a patient with situs inversus. Indian J Gastroenterol. 2004;23:79-80 pubmed
  30. Sceusi E, Wray C. Pancreatic adenocarcinoma in a patient with situs inversus: a case report of this rare coincidence. World J Surg Oncol. 2009;7:98 pubmed publisher
    b>Situs inversus (SI) is a relatively rare occurrence in patients with pancreatic adenocarcinoma. Pancreatic resection in these patients has rarely been described...
  31. Tsunoda S, Miyashita T, Murata M. Pancreaticoduodenectomy for common bile duct cancer in a patient with situs inversus totalis: a case report. Int Surg. 2006;91:24-7 pubmed
    ..case of successfully performed pancreaticoduodenectomy to treat common bile duct cancer in a patient with situs inversus totalis. A 65-year-old man was admitted to our hospital with the working diagnosis of obstructive jaundice...
  32. Greene Q, Cheadle W. Ascending colon cancer in a patient with situs inversus. Am Surg. 2007;73:831-2 pubmed
  33. Kulesza R, Kalmey J, Dudas B, Buck W. Vascular anomalies in a case of situs inversus. Folia Morphol (Warsz). 2007;66:69-73 pubmed
    b>Situs inversus is a developmental condition in which the thoracic and abdominal organs fail to negotiate their normal migration patterns and the result is a mirror-image arrangement of these viscera...
  34. Hoang C, Bakman Y, Ikramuddin S, Maddaus M. Situs inversus totalis: giant hiatal hernia repair by laparoscopic Collis gastroplasty and Nissen fundoplication. Surg Endosc. 2004;18:345-9 pubmed
    ..of a giant hiatal hernia by laparoscopic Collis gastroplasty and Nissen fundoplication in a patient with situs inversus totalis, highlighting the unique anatomic challenges in this case...
  35. Kobus C, Targarona E, Bendahan G, Alonso V, Balague C, Vela S, et al. Laparoscopic surgery in situs inversus: a literature review and a report of laparoscopic sigmoidectomy for diverticulitis in situs inversus. Langenbecks Arch Surg. 2004;389:396-9 pubmed
    b>Situs inversus (SI) is a rare autosomal recessive congenital defect in which the position of abdominal and/or thoracic organs is a "mirror image" of the normal one, in the sagittal plain...
  36. Pego Fernandes P, de Serro Azul J, Matheus F, Maehara B. Myocardial revascularization in a patient with situs inversus totalis. Arq Bras Cardiol. 2007;88:e103-6 pubmed
    We report the case of a patient with dextrocardia and situs inversus totalis associated with obstructive coronariopathy in the anterior and posterior descending arteries, right coronary artery, first diagonal branch and left marginal ..
  37. Bedioui H, Chebbi F, Ayadi S, Makni A, Fteriche F, Ksantini R, et al. [Laparoscopic cholecystectomy in a patient with situs inversus]. Ann Chir. 2006;131:398-400 pubmed
    ..The diagnosis of acute cholecystitis with situs inversus totalis was made following clinical examination and radiological investigations...
  38. Mano Y, Adachi N, Murakami G, Yokoyama T, Dodo Y. Human situs inversus of the thoracoabdominal structures. Anat Sci Int. 2006;81:7-20 pubmed
    b>Situs inversus totalis is well known, but its comprehensive description has been rare, especially on the internal view of the heart...
  39. Rayhill S, Scott D, Orloff S, Horn J, Schwartz J, Zaman A, et al. Orthotopic, but reversed implantation of the liver allograft in situs inversus totalis-a simple new approach to a difficult problem. Am J Transplant. 2009;9:1602-6 pubmed publisher
    b>Situs inversus totalis is a rare congenital anomaly in which the heart and abdominal organs are oriented in a mirror image of normal...
  40. Ichioka K, Kohei N, Okubo K, Nishiyama H, Terai A. Obstructive azoospermia associated with chronic sinopulmonary infection and situs inversus totalis. Urology. 2006;68:204.e5-7 pubmed
    We describe 2 cases of obstructive azoospermia associated with situs inversus and sinopulmonary infection due to ciliary defects...
  41. Luo C, Lin J, Lien R, Chu S. A new variant of esophageal atresia with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus. J Pediatr Surg. 2003;38:E25-7 pubmed
    ..To our knowledge, this is the first case report of EA with distal tracheo-antral fistula associated with congenital intrathoracic stomach and situs inversus (SI).
  42. Kosaki K, Ikeda K, Miyakoshi K, Ueno M, Kosaki R, Takahashi D, et al. Absent inner dynein arms in a fetus with familial hydrocephalus-situs abnormality. Am J Med Genet A. 2004;129A:308-11 pubmed
    ..This figure is much higher than the general risk of 1-2% for siblings of a patient with isolated hydrocephalus. ..
  43. Bielecki K, Gregorczyk M, Baczuk L. Visceral situs inversus in three patients. Wiad Lek. 2006;59:707-9 pubmed
    Three examples of patients with complete visceral situs inversus are presented from nineteen years of clinical experience...
  44. Krishnamurthy S, Kapoor S, Sharma V, Prakash A. Tectocerebellar dysraphia and occipital encephalocele: an unusual association with abdominal situs inversus and congenital heart disease. Indian J Pediatr. 2008;75:1178-80 pubmed publisher
    ..infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.
  45. Machado N, Chopra P. Laparoscopic cholecystectomy in a patient with situs inversus totalis: feasibility and technical difficulties. JSLS. 2006;10:386-91 pubmed
    b>Situs inversus is a rare anomaly characterized by transposition of organs to the opposite side of the body. In patients with this anomaly, cholelithiasis is observed with a frequency similar to that in the normal population...
  46. Kamitani S, Tsutamoto Y, Hanasawa K, Tani T. Laparoscopic cholecystectomy in situs inversus totalis with "inferior" cystic artery: a case report. World J Gastroenterol. 2005;11:5232-4 pubmed
    A 76-year-old man with known situs inversus totalis presented with left-sided discomfort...
  47. Park S, Min B, Kim W, Han H, Yong H, Kim S, et al. Double cancer of the stomach and oesophagus with situs ambiguus with polysplenia: the importance of preoperative evaluation. Dig Liver Dis. 2005;37:799-802 pubmed
    ..This is the first case presentation of a double carcinoma occurring in a patient with situs ambiguus with polysplenia. The literature is reviewed and the importance of preoperative evaluation is discussed. ..
  48. Nawaz A, Matta H, Hamchou M, Jacobez A, Trad O, Al Salem A. Situs inversus abdominus in association with congenital duodenal obstruction: a report of two cases and review of the literature. Pediatr Surg Int. 2005;21:589-92 pubmed
    ..Their radiological investigations revealed the existence of situs inversus and duodenal obstruction...
  49. Chui W, Sarkar P. Coronary artery bypass grafting in dextrocardia with situs inversus totalis. J Cardiovasc Surg (Torino). 2003;44:617-9 pubmed
    Dextrocardia with situs inversus totalis is a rare congenital abnormality of development involving a left-handed malrotation of the visceral organs...
  50. Fujiwara Y, Fukunaga Y, Higashino M, Tanimura S, Takemura M, Tanaka Y, et al. Laparoscopic hemicolectomy in a patient with situs inversus totalis. World J Gastroenterol. 2007;13:5035-7 pubmed
    As among persons with normal anatomy, occasional patients with situs inversus develop malignant tumors. Recently, several laparoscopic operations have been reported in patients with situs inversus...
  51. Maruyama Y, Horiuchi H, Okabe Y, Kawahara R, Uchida S, Sakai T, et al. Perioperative challenges associated with a pancreaticoduodenectomy and distal pancreatectomy for pancreatic cancer in patients with situs inversus totalis: report of two cases. Surg Today. 2010;40:79-82 pubmed publisher
    b>Situs inversus totalis is a rare anatomic variant of a complete mirror-image transposition of the thoracic and abdominal viscera...
  52. Palmers K, van Loon G, Jorissen M, Verdonck F, Chiers K, Picavet M, et al. Situs inversus totalis and primary ciliary dyskinesia (Kartagener's syndrome) in a horse. J Vet Intern Med. 2008;22:491-4 pubmed publisher
  53. Macafee D, Armstrong D, Hall R, Dhingsa R, Zaitoun A, Lobo D. Pancreaticoduodenectomy with a "twist": the challenges of pancreatic resection in the presence of situs inversus totalis and situs ambiguus. Eur J Surg Oncol. 2007;33:524-7 pubmed