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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Asplenia (ASP), polysplenia (PSP)
Synonyms
- Heterotaxy syndromes
- ASP: ASP syndrome, Ivemark syndrome, bilateral right-sidedness
- PSP: PSP syndrome, bilateral left-sidedness
Definitions
- Complex inherited syndromes associated with absence (ASP) or multiplicity (PSP) of spleens as well as many other anomalies
Associated Syndromes
- Heterotaxy: Abnormal embryologic placement of thoracoabdominal structures across right-left axis of body
- Situs solitus: Normal placement of thoracoabdominal organs in right-left axis
- Situs inversus: Reversal of normal positions of thoracoabdominal organs across right-left axis (mirror image of situs solitus)
- Can be subdivided into situs inversus with dextrocardia or levocardia
- Situs ambiguus (heterotaxy syndrome): Abnormal placement of thoracoabdominal structures without situs inversus
- Situs ambiguus with PSP: Left isomerism or bilateral left-sidedness
- Situs ambiguus with ASP: Right isomerism or bilateral right-sidedness
IMAGING
General Features
Radiographic Findings
CT Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Ashorobi D et al: Asplenia. StatPearls, 2021
- Lagrotta G et al: Heterotaxy polysplenia syndrome in adulthood: focused review and a case report. Cureus. 12(1):e6822, 2020
- Varga I et al: Anatomic variations of the spleen: current state of terminology, classification, and embryological background. Surg Radiol Anat. 40(1):21-9, 2018
- Tawfik AM et al: Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 35(8):647-53, 2013
- Yildiz AE et al: Splenic anomalies of shape, size, and location: pictorial essay. ScientificWorldJournal. 2013:321810, 2013
- Kim SW et al: Duplication of the spleen with a short pancreas. Br J Radiol. 82(975):e42-3, 2009
- Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
- De La Villeon B et al: Polysplenia syndrome. J Visc Surg. 148(5):e395-6, 2011
- Davit-Spraul A et al: CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr. 46(1):111-2, 2008
- Halac M et al: Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT. Clin Nucl Med. 33(2):142-3, 2008
- Maier M et al: Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 188(2):W150-3, 2007
- Elsayes KM et al: MR imaging of the spleen: spectrum of abnormalities. Radiographics. 25(4):967-82, 2005
- Combs LS et al: Evaluation of spleen in children with heterotaxia and congenital heart disease. Tenn Med. 97(4):161-3, 2004
- Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
- Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
- Paterson A et al: A pattern-oriented approach to splenic imaging in infants and children. Radiographics. 19(6):1465-85, 1999
- Ruscazio M et al: Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol. 81(1):111-6, 1998
- Nakada K et al: Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg. 32(1):91-4, 1997
- Freeman JL et al: CT of congenital and acquired abnormalities of the spleen. Radiographics. 13(3):597-610, 1993
- Chitayat D et al: Prenatal diagnosis of asplenia/polysplenia syndrome. Am J Obstet Gynecol. 158(5):1085-7, 1988
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Asplenia (ASP), polysplenia (PSP)
Synonyms
- Heterotaxy syndromes
- ASP: ASP syndrome, Ivemark syndrome, bilateral right-sidedness
- PSP: PSP syndrome, bilateral left-sidedness
Definitions
- Complex inherited syndromes associated with absence (ASP) or multiplicity (PSP) of spleens as well as many other anomalies
Associated Syndromes
- Heterotaxy: Abnormal embryologic placement of thoracoabdominal structures across right-left axis of body
- Situs solitus: Normal placement of thoracoabdominal organs in right-left axis
- Situs inversus: Reversal of normal positions of thoracoabdominal organs across right-left axis (mirror image of situs solitus)
- Can be subdivided into situs inversus with dextrocardia or levocardia
- Situs ambiguus (heterotaxy syndrome): Abnormal placement of thoracoabdominal structures without situs inversus
- Situs ambiguus with PSP: Left isomerism or bilateral left-sidedness
- Situs ambiguus with ASP: Right isomerism or bilateral right-sidedness
IMAGING
General Features
Radiographic Findings
CT Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Image Interpretation Pearls
Selected References
- Ashorobi D et al: Asplenia. StatPearls, 2021
- Lagrotta G et al: Heterotaxy polysplenia syndrome in adulthood: focused review and a case report. Cureus. 12(1):e6822, 2020
- Varga I et al: Anatomic variations of the spleen: current state of terminology, classification, and embryological background. Surg Radiol Anat. 40(1):21-9, 2018
- Tawfik AM et al: Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat. 35(8):647-53, 2013
- Yildiz AE et al: Splenic anomalies of shape, size, and location: pictorial essay. ScientificWorldJournal. 2013:321810, 2013
- Kim SW et al: Duplication of the spleen with a short pancreas. Br J Radiol. 82(975):e42-3, 2009
- Balan A et al: Atrial isomerism: a pictorial review. J Cardiovasc Comput Tomogr. 6(2):127-36, 2012
- De La Villeon B et al: Polysplenia syndrome. J Visc Surg. 148(5):e395-6, 2011
- Davit-Spraul A et al: CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr. 46(1):111-2, 2008
- Halac M et al: Appearance of situs inversus totalis and polysplenia syndrome on FDG PET/CT. Clin Nucl Med. 33(2):142-3, 2008
- Maier M et al: Annular pancreas and agenesis of the dorsal pancreas in a patient with polysplenia syndrome. AJR Am J Roentgenol. 188(2):W150-3, 2007
- Elsayes KM et al: MR imaging of the spleen: spectrum of abnormalities. Radiographics. 25(4):967-82, 2005
- Combs LS et al: Evaluation of spleen in children with heterotaxia and congenital heart disease. Tenn Med. 97(4):161-3, 2004
- Fulcher AS et al: Abdominal manifestations of situs anomalies in adults. Radiographics. 22(6):1439-56, 2002
- Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 19(4):837-52; discussion 853-4, 1999
- Paterson A et al: A pattern-oriented approach to splenic imaging in infants and children. Radiographics. 19(6):1465-85, 1999
- Ruscazio M et al: Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities. Am J Cardiol. 81(1):111-6, 1998
- Nakada K et al: Digestive tract disorders associated with asplenia/polysplenia syndrome. J Pediatr Surg. 32(1):91-4, 1997
- Freeman JL et al: CT of congenital and acquired abnormalities of the spleen. Radiographics. 13(3):597-610, 1993
- Chitayat D et al: Prenatal diagnosis of asplenia/polysplenia syndrome. Am J Obstet Gynecol. 158(5):1085-7, 1988
