Congenital extrahepatic portosystemic shunts
A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopath...
Gespeichert in:
| Veröffentlicht in: | Pediatric radiology 2003-09, Vol.33 (9), p.614-620 |
|---|---|
| Hauptverfasser: | , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 620 |
|---|---|
| container_issue | 9 |
| container_start_page | 614 |
| container_title | Pediatric radiology |
| container_volume | 33 |
| creator | MURRAY, Conor P YOO, Shi-Joon BABYN, Paul S |
| description | A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy.
To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature.
The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed.
Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver ( n=25), cardiac anomalies ( n=19), portosystemic encephalopathy ( n=10), polysplenia ( n=9), biliary atresia ( n=7), skeletal anomalies ( n=5), and renal tract anomalies ( n=4).
MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. |
| doi_str_mv | 10.1007/s00247-003-1002-x |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_73555643</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>73555643</sourcerecordid><originalsourceid>FETCH-LOGICAL-c420t-89a5599c3e38404e52b787ffd0b8390d07a4b1cb1dfd71839a466301b3d8a02f3</originalsourceid><addsrcrecordid>eNpdkEtLAzEUhYMotlZ_gBspgq6M3rwmk6UUX1Bwo-uQyWTslHnUJAPtvzelAwVXl3P5zuFwELom8EgA5FMAoFxiAIaTpnh7gqaEM4qJUvkpmgIDgoFzNUEXIawhgYKwczQhNJeKETZFD4u--3FdHU0zd9vozcptTKztfNP72IddiK5NKqyGLoZLdFaZJrir8c7Q9-vL1-IdLz_fPhbPS2w5hYhzZYRQyjLHcg7cCVrIXFZVCUXOFJQgDS-ILUhZlZKkl-FZlqoWrMwN0IrN0P0hd-P738GFqNs6WNc0pnP9ELRkQoiMswTe_gPX_eC71E1TSrOMZJwmiBwg6_sQvKv0xtet8TtNQO931IcddZpnr6neJs_NGDwUrSuPjnG4BNyNgAnWNJU3na3DkROcCCKB_QEV2Xlw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>222661642</pqid></control><display><type>article</type><title>Congenital extrahepatic portosystemic shunts</title><source>MEDLINE</source><source>SpringerLink Journals - AutoHoldings</source><creator>MURRAY, Conor P ; YOO, Shi-Joon ; BABYN, Paul S</creator><creatorcontrib>MURRAY, Conor P ; YOO, Shi-Joon ; BABYN, Paul S</creatorcontrib><description>A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy.
To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature.
The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed.
Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver ( n=25), cardiac anomalies ( n=19), portosystemic encephalopathy ( n=10), polysplenia ( n=9), biliary atresia ( n=7), skeletal anomalies ( n=5), and renal tract anomalies ( n=4).
MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested.</description><identifier>ISSN: 0301-0449</identifier><identifier>EISSN: 1432-1998</identifier><identifier>DOI: 10.1007/s00247-003-1002-x</identifier><identifier>PMID: 12879313</identifier><identifier>CODEN: PDRYA5</identifier><language>eng</language><publisher>Berlin: Springer</publisher><subject>Biological and medical sciences ; Blood and lymphatic vessels ; Cardiology. Vascular system ; Child, Preschool ; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous ; Female ; Focal Nodular Hyperplasia - pathology ; Heart Defects, Congenital - pathology ; Humans ; Infant ; Liver Diseases - pathology ; Magnetic Resonance Imaging ; Male ; Medical sciences ; Portal System - abnormalities</subject><ispartof>Pediatric radiology, 2003-09, Vol.33 (9), p.614-620</ispartof><rights>2004 INIST-CNRS</rights><rights>Springer-Verlag 2003</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c420t-89a5599c3e38404e52b787ffd0b8390d07a4b1cb1dfd71839a466301b3d8a02f3</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=15415170$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12879313$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>MURRAY, Conor P</creatorcontrib><creatorcontrib>YOO, Shi-Joon</creatorcontrib><creatorcontrib>BABYN, Paul S</creatorcontrib><title>Congenital extrahepatic portosystemic shunts</title><title>Pediatric radiology</title><addtitle>Pediatr Radiol</addtitle><description>A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy.
To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature.
The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed.
Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver ( n=25), cardiac anomalies ( n=19), portosystemic encephalopathy ( n=10), polysplenia ( n=9), biliary atresia ( n=7), skeletal anomalies ( n=5), and renal tract anomalies ( n=4).
MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested.</description><subject>Biological and medical sciences</subject><subject>Blood and lymphatic vessels</subject><subject>Cardiology. Vascular system</subject><subject>Child, Preschool</subject><subject>Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous</subject><subject>Female</subject><subject>Focal Nodular Hyperplasia - pathology</subject><subject>Heart Defects, Congenital - pathology</subject><subject>Humans</subject><subject>Infant</subject><subject>Liver Diseases - pathology</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Portal System - abnormalities</subject><issn>0301-0449</issn><issn>1432-1998</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2003</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><recordid>eNpdkEtLAzEUhYMotlZ_gBspgq6M3rwmk6UUX1Bwo-uQyWTslHnUJAPtvzelAwVXl3P5zuFwELom8EgA5FMAoFxiAIaTpnh7gqaEM4qJUvkpmgIDgoFzNUEXIawhgYKwczQhNJeKETZFD4u--3FdHU0zd9vozcptTKztfNP72IddiK5NKqyGLoZLdFaZJrir8c7Q9-vL1-IdLz_fPhbPS2w5hYhzZYRQyjLHcg7cCVrIXFZVCUXOFJQgDS-ILUhZlZKkl-FZlqoWrMwN0IrN0P0hd-P738GFqNs6WNc0pnP9ELRkQoiMswTe_gPX_eC71E1TSrOMZJwmiBwg6_sQvKv0xtet8TtNQO931IcddZpnr6neJs_NGDwUrSuPjnG4BNyNgAnWNJU3na3DkROcCCKB_QEV2Xlw</recordid><startdate>20030901</startdate><enddate>20030901</enddate><creator>MURRAY, Conor P</creator><creator>YOO, Shi-Joon</creator><creator>BABYN, Paul S</creator><general>Springer</general><general>Springer Nature B.V</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7TK</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>LK8</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20030901</creationdate><title>Congenital extrahepatic portosystemic shunts</title><author>MURRAY, Conor P ; YOO, Shi-Joon ; BABYN, Paul S</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c420t-89a5599c3e38404e52b787ffd0b8390d07a4b1cb1dfd71839a466301b3d8a02f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2003</creationdate><topic>Biological and medical sciences</topic><topic>Blood and lymphatic vessels</topic><topic>Cardiology. Vascular system</topic><topic>Child, Preschool</topic><topic>Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous</topic><topic>Female</topic><topic>Focal Nodular Hyperplasia - pathology</topic><topic>Heart Defects, Congenital - pathology</topic><topic>Humans</topic><topic>Infant</topic><topic>Liver Diseases - pathology</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Portal System - abnormalities</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>MURRAY, Conor P</creatorcontrib><creatorcontrib>YOO, Shi-Joon</creatorcontrib><creatorcontrib>BABYN, Paul S</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>ProQuest Biological Science Collection</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Biological Science Database</collection><collection>Nursing & Allied Health Premium</collection><collection>Advanced Technologies & Aerospace Database</collection><collection>ProQuest Advanced Technologies & Aerospace Collection</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric radiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>MURRAY, Conor P</au><au>YOO, Shi-Joon</au><au>BABYN, Paul S</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Congenital extrahepatic portosystemic shunts</atitle><jtitle>Pediatric radiology</jtitle><addtitle>Pediatr Radiol</addtitle><date>2003-09-01</date><risdate>2003</risdate><volume>33</volume><issue>9</issue><spage>614</spage><epage>620</epage><pages>614-620</pages><issn>0301-0449</issn><eissn>1432-1998</eissn><coden>PDRYA5</coden><abstract>A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy.
To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature.
The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed.
Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver ( n=25), cardiac anomalies ( n=19), portosystemic encephalopathy ( n=10), polysplenia ( n=9), biliary atresia ( n=7), skeletal anomalies ( n=5), and renal tract anomalies ( n=4).
MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested.</abstract><cop>Berlin</cop><pub>Springer</pub><pmid>12879313</pmid><doi>10.1007/s00247-003-1002-x</doi><tpages>7</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0301-0449 |
| ispartof | Pediatric radiology, 2003-09, Vol.33 (9), p.614-620 |
| issn | 0301-0449 1432-1998 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_73555643 |
| source | MEDLINE; SpringerLink Journals - AutoHoldings |
| subjects | Biological and medical sciences Blood and lymphatic vessels Cardiology. Vascular system Child, Preschool Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous Female Focal Nodular Hyperplasia - pathology Heart Defects, Congenital - pathology Humans Infant Liver Diseases - pathology Magnetic Resonance Imaging Male Medical sciences Portal System - abnormalities |
| title | Congenital extrahepatic portosystemic shunts |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-22T10%3A25%3A42IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Congenital%20extrahepatic%20portosystemic%20shunts&rft.jtitle=Pediatric%20radiology&rft.au=MURRAY,%20Conor%20P&rft.date=2003-09-01&rft.volume=33&rft.issue=9&rft.spage=614&rft.epage=620&rft.pages=614-620&rft.issn=0301-0449&rft.eissn=1432-1998&rft.coden=PDRYA5&rft_id=info:doi/10.1007/s00247-003-1002-x&rft_dat=%3Cproquest_cross%3E73555643%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=222661642&rft_id=info:pmid/12879313&rfr_iscdi=true |