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Ectopia of the spleen

MedGen UID:
451027
•Concept ID:
C0266632
•
Congenital Abnormality
Synonyms: Aberrant spleen; Abnormal spleen location; Ectopic spleen
SNOMED CT: Ectopic spleen (65146007); Aberrant spleen (65146007)
 
HPO: HP:0010452

Definition

An abnormal (non-anatomic) location of the spleen. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEctopia of the spleen

Conditions with this feature

Heterotaxy, visceral, 4, autosomal
MedGen UID:
462407
•Concept ID:
C3151057
•
Disease or Syndrome
Heterotaxy ('heter' meaning 'other' and 'taxy' meaning 'arrangement'), or situs ambiguus, is a developmental condition characterized by randomization of the placement of visceral organs, including the heart, lungs, liver, spleen, and stomach. The organs are oriented randomly with respect to the left-right axis and with respect to one another (Srivastava, 1997). Heterotaxy is a clinically and genetically heterogeneous disorder. For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).
See: Condition Record
Heterotaxy, visceral, 4, autosomal

Professional guidelines

PubMed

Intrapancreatic accessory spleen: CT appearance and differential diagnosis.
Kawamoto S, Johnson PT, Hall H, Cameron JL, Hruban RH, Fishman EK
Abdom Imaging 2012 Oct;37(5):812-27. doi: 10.1007/s00261-011-9830-x. PMID: 22160284

Recent clinical studies

Etiology

Ectopias of the kidney, urinary tract organs, and male genitalia.
Mikuz G
Pathologe 2019 Jun;40(Suppl 1):1-8. doi: 10.1007/s00292-018-0505-z. PMID: 30446779
Intrapancreatic accessory spleen (IPAS): A single-institution experience and review of the literature.
Bhutiani N, Egger ME, Doughtie CA, Burkardt ES, Scoggins CR, Martin RC 2nd, McMasters KM
Am J Surg 2017 Apr;213(4):816-820. Epub 2016 Nov 23 doi: 10.1016/j.amjsurg.2016.11.030. PMID: 27894508
Pancreatic and gastric heterotopy in the gastrointestinal tract.
Wlaź J, Mądro A, Kaźmierak W, Celiński K, Słomka M
Postepy Hig Med Dosw (Online) 2014 Sep 3;68:1069-75. doi: 10.5604/17322693.1119720. PMID: 25228515
Epidermoid cyst of the spleen.
Chen YY, Shyr YM, Wang SE
J Gastrointest Surg 2013 Mar;17(3):555-61. Epub 2012 Nov 27 doi: 10.1007/s11605-012-2088-y. PMID: 23188221
Splenogonadal fusion.
Alalayet YF, Mansoor K, Shiba NA, Khan AM, Al Kasim F
Eur J Pediatr Surg 2008 Oct;18(5):342-4. Epub 2008 Oct 15 doi: 10.1055/s-2008-1038588. PMID: 18924073

Diagnosis

Intrapancreatic accessory spleen.
Santos MPD, Rezende AP, Santos PVD Filho, Gonçalves JE, Beraldo FB, Sampaio AP
Einstein (Sao Paulo) 2017 Jul-Sep;15(3):366-368. Epub 2017 Jun 12 doi: 10.1590/S1679-45082017RC3942. PMID: 28614428Free PMC Article
Heterotopic Pancreas: Histopathologic Features, Imaging Findings, and Complications.
Rezvani M, Menias C, Sandrasegaran K, Olpin JD, Elsayes KM, Shaaban AM
Radiographics 2017 Mar-Apr;37(2):484-499. doi: 10.1148/rg.2017160091. PMID: 28287935
Pancreatic and gastric heterotopy in the gastrointestinal tract.
Wlaź J, Mądro A, Kaźmierak W, Celiński K, Słomka M
Postepy Hig Med Dosw (Online) 2014 Sep 3;68:1069-75. doi: 10.5604/17322693.1119720. PMID: 25228515
Epidermoid cyst of the spleen.
Chen YY, Shyr YM, Wang SE
J Gastrointest Surg 2013 Mar;17(3):555-61. Epub 2012 Nov 27 doi: 10.1007/s11605-012-2088-y. PMID: 23188221
Intrapancreatic accessory spleen: CT appearance and differential diagnosis.
Kawamoto S, Johnson PT, Hall H, Cameron JL, Hruban RH, Fishman EK
Abdom Imaging 2012 Oct;37(5):812-27. doi: 10.1007/s00261-011-9830-x. PMID: 22160284

Therapy

Superparamagnetic iron-oxide-enhanced diffusion-weighted magnetic resonance imaging for the diagnosis of intrapancreatic accessory spleen.
Ishigami K, Nishie A, Nakayama T, Asayama Y, Kakihara D, Fujita N, Ushijima Y, Okamoto D, Ohtsuka T, Mori Y, Ito T, Mochidome N, Honda H
Abdom Radiol (NY) 2019 Oct;44(10):3325-3335. doi: 10.1007/s00261-019-02189-8. PMID: 31420705
The prevalence and morphometry of an accessory spleen: A meta-analysis and systematic review of 22,487 patients.
Vikse J, Sanna B, Henry BM, Taterra D, Sanna S, Pękala PA, Walocha JA, Tomaszewski KA
Int J Surg 2017 Sep;45:18-28. Epub 2017 Jul 15 doi: 10.1016/j.ijsu.2017.07.045. PMID: 28716661
Pediatric laparoscopic splenectomy.
Moores DC, McKee MA, Wang H, Fischer JD, Smith JW, Andrews HG
J Pediatr Surg 1995 Aug;30(8):1201-5. doi: 10.1016/0022-3468(95)90022-5. PMID: 7472983
Injuries of the spleen.
Perry JF Jr
Curr Probl Surg 1988 Dec;25(12):749-859. doi: 10.1016/0011-3840(88)90022-6. PMID: 3061747
Malign effects of splenectomy--the place of conservative treatment.
Werbin N, Lodha K
Postgrad Med J 1982 Feb;58(676):65-9. doi: 10.1136/pgmj.58.676.65. PMID: 7048272Free PMC Article

Prognosis

Pancreatic Incidentalomas: A Management Algorithm for Identifying Ectopic Spleens.
Baugh KA, Villafane N, Farinas C, Dhingra S, Silberfein EJ, Massarweh NN, Cao HT, Fisher WE, Van Buren G 2nd
J Surg Res 2019 Apr;236:144-152. Epub 2018 Dec 17 doi: 10.1016/j.jss.2018.11.032. PMID: 30694749
Ectopias of the kidney, urinary tract organs, and male genitalia.
Mikuz G
Pathologe 2019 Jun;40(Suppl 1):1-8. doi: 10.1007/s00292-018-0505-z. PMID: 30446779
Intrapancreatic accessory spleen: a diagnostic dilemma.
Li BQ, Xu XQ, Guo JC
HPB (Oxford) 2018 Nov;20(11):1004-1011. Epub 2018 May 26 doi: 10.1016/j.hpb.2018.04.004. PMID: 29843985
Epidermoid cysts are a characteristic feature of intrapancreatic but not of extrapancreatic accessory spleens.
Hirabayashi K, Yamada M, Kono H, Hadano A, Kawanishi A, Takanashi Y, Kawaguchi Y, Nakagohri T, Mine T, Nakamura N
Virchows Arch 2017 Jul;471(1):91-98. Epub 2017 May 24 doi: 10.1007/s00428-017-2139-6. PMID: 28540449
Splenogonadal fusion.
Alalayet YF, Mansoor K, Shiba NA, Khan AM, Al Kasim F
Eur J Pediatr Surg 2008 Oct;18(5):342-4. Epub 2008 Oct 15 doi: 10.1055/s-2008-1038588. PMID: 18924073

Clinical prediction guides

Intrapancreatic accessory spleen: a diagnostic dilemma.
Li BQ, Xu XQ, Guo JC
HPB (Oxford) 2018 Nov;20(11):1004-1011. Epub 2018 May 26 doi: 10.1016/j.hpb.2018.04.004. PMID: 29843985
Significance of imaging findings in the diagnosis of heterotopic spleen-an intrapancreatic accessory spleen (IPAS): Case report.
Yang B, Valluru B, Guo YR, Cui C, Zhang P, Duan W
Medicine (Baltimore) 2017 Dec;96(52):e9040. doi: 10.1097/MD.0000000000009040. PMID: 29384897Free PMC Article
Asymptomatic heterotopic pancreas in Meckel's diverticulum: a case report and review of the literature.
Kilius A, Samalavicius NE, Danys D, Zaldokas G, Seinin D
J Med Case Rep 2015 May 9;9:108. doi: 10.1186/s13256-015-0576-x. PMID: 25956067Free PMC Article
Frequency and characterization of benign lesions in patients undergoing surgery for the suspicion of solid pancreatic neoplasm.
Vitali F, Hansen T, Kiesslich R, Heinrich S, Kumar A, Mildenberger P, Amodio A, Benini L, Vantini I, Frulloni L
Pancreas 2014 Nov;43(8):1329-33. doi: 10.1097/MPA.0000000000000193. PMID: 25058888
Differentiation of an intrapancreatic accessory spleen from a small (<3-cm) solid pancreatic tumor: value of diffusion-weighted MR imaging.
Jang KM, Kim SH, Lee SJ, Park MJ, Lee MH, Choi D
Radiology 2013 Jan;266(1):159-67. Epub 2012 Oct 23 doi: 10.1148/radiol.12112765. PMID: 23093681

Recent systematic reviews

Intrapancreatic accessory spleen: a diagnostic dilemma.
Li BQ, Xu XQ, Guo JC
HPB (Oxford) 2018 Nov;20(11):1004-1011. Epub 2018 May 26 doi: 10.1016/j.hpb.2018.04.004. PMID: 29843985
The prevalence and morphometry of an accessory spleen: A meta-analysis and systematic review of 22,487 patients.
Vikse J, Sanna B, Henry BM, Taterra D, Sanna S, Pękala PA, Walocha JA, Tomaszewski KA
Int J Surg 2017 Sep;45:18-28. Epub 2017 Jul 15 doi: 10.1016/j.ijsu.2017.07.045. PMID: 28716661

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