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Extrahepatic portal hypertension

MedGen UID:
870811
•Concept ID:
C4025268
•
Disease or Syndrome
HPO: HP:0004941

Definition

Increased pressure in the pre-hepatic portal vein. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic portal hypertension

Conditions with this feature

Portal vein, cavernous transformation of
MedGen UID:
331396
•Concept ID:
C1832917
•
Disease or Syndrome
See: Condition Record
Portal vein, cavernous transformation of

Professional guidelines

PubMed

Portal biliopathy as a complication of extrahepatic portal hypertension: etiology, presentation and management.
Le Roy B, Gelli M, Serji B, Memeo R, Vibert E
J Visc Surg 2015 Jun;152(3):161-6. Epub 2015 May 27 doi: 10.1016/j.jviscsurg.2015.04.003. PMID: 26025414
Management of portal hypertension in children: a retrospective study with long-term follow-up.
Erkan T, Cullu F, Kutlu T, Emir H, Yeşildağ E, Sarimurat N, Senyüz OF, Erginöz E, Tümay GT
Acta Gastroenterol Belg 2003 Jul-Sep;66(3):213-7. PMID: 14618951
Pregnancy and extrahepatic portal hypertension. Review and report on the management.
Pajor A, Lehoczky D
Gynecol Obstet Invest 1990;30(4):193-7. doi: 10.1159/000293268. PMID: 2289698

Recent clinical studies

Etiology

Surgical Treatment of Portal Hypertension in Children.
Alkhasov A, Komina E, Ratnikov S, Saveleva M, Romanova E, Gusev A, Lochmatov M, Yatsyk S, Dyakonova E
J Laparoendosc Adv Surg Tech A 2023 Dec;33(12):1231-1235. Epub 2023 Oct 16 doi: 10.1089/lap.2022.0404. PMID: 37844079
An Audit of Extrahepatic Portal Vein Obstruction: Experience from Tertiary Referral Center.
Rathi P, Nair S
J Assoc Physicians India 2023 Jul;71(7):11-12. doi: 10.59556/japi.71.0281. PMID: 37449688
Angiographic patterns of portal venous system in children with extrahepatic portal hypertension and its etiological and clinical relevance.
Yuldashev RZ, Aliev MM, Maksudov MF, Khaydarov UO, Shokhaydarov S
Pediatr Surg Int 2023 Feb 1;39(1):97. doi: 10.1007/s00383-023-05384-3. PMID: 36723662
Optimal Rex shunt procedures as a treatment for pediatric extrahepatic portal hypertension.
Zhang YQ, Wang Q, Wu M, Ruan Z-, Li Y, Wei X-, Zhang FX, Li Y, Shao GR, Xiao J
Pediatr Surg Int 2021 May;37(5):597-606. Epub 2021 Jan 10 doi: 10.1007/s00383-020-04847-1. PMID: 33423101
Distal Splenorenal Shunt (DSRS) in Children with Extrahepatic Portal Hypertension.
Rehman ZU, Nazir Z
J Coll Physicians Surg Pak 2019 Dec;29(12):1228-1229. doi: 10.29271/jcpsp.2019.12.1228. PMID: 31839103

Diagnosis

Recurrent variceal haemorrhage managed with splenic vein stenting.
El Kininy W, Kearney L, Hosam N, Broe P, Keeling A
Ir J Med Sci 2017 May;186(2):323-327. Epub 2016 Feb 25 doi: 10.1007/s11845-016-1420-z. PMID: 26911860
Portal biliopathy as a complication of extrahepatic portal hypertension: etiology, presentation and management.
Le Roy B, Gelli M, Serji B, Memeo R, Vibert E
J Visc Surg 2015 Jun;152(3):161-6. Epub 2015 May 27 doi: 10.1016/j.jviscsurg.2015.04.003. PMID: 26025414
Pulmonary vascular abnormalities in cirrhosis.
Herve P, Le Pavec J, Sztrymf B, Decante B, Savale L, Sitbon O
Best Pract Res Clin Gastroenterol 2007;21(1):141-59. doi: 10.1016/j.bpg.2006.07.011. PMID: 17223502
Segmental portal hypertension.
Madsen MS, Petersen TH, Sommer H
Ann Surg 1986 Jul;204(1):72-7. doi: 10.1097/00000658-198607000-00010. PMID: 3729585Free PMC Article
"Sinistral" (left-sided) extrahepatic portal hypertension.
Turrill FL, Mikkelsen WP
Arch Surg 1969 Sep;99(3):365-8. doi: 10.1001/archsurg.1969.01340150073014. PMID: 5306330

Therapy

Surgical Treatment of Portal Hypertension in Children.
Alkhasov A, Komina E, Ratnikov S, Saveleva M, Romanova E, Gusev A, Lochmatov M, Yatsyk S, Dyakonova E
J Laparoendosc Adv Surg Tech A 2023 Dec;33(12):1231-1235. Epub 2023 Oct 16 doi: 10.1089/lap.2022.0404. PMID: 37844079
The clinical applicability of percutaneous splenic vein stent implantation for pancreatic portal hypertension.
Liu J, Wang Q, Ding X, Liu Q, Huang W, Gu J, Wang Z, Wu W, Wu Z
BMC Gastroenterol 2022 Mar 25;22(1):136. doi: 10.1186/s12876-022-02214-z. PMID: 35337294Free PMC Article
Functional side-to-side splenorenal shunts to treat extrahepatic portal vein thrombosis in children.
Yang AY, Slatnick BL, Durgin J, Truche P, Kim HB, Cuenca AG
Am J Surg 2022 Jul;224(1 Pt B):530-534. Epub 2022 Feb 9 doi: 10.1016/j.amjsurg.2022.01.031. PMID: 35164959
Optimal Rex shunt procedures as a treatment for pediatric extrahepatic portal hypertension.
Zhang YQ, Wang Q, Wu M, Ruan Z-, Li Y, Wei X-, Zhang FX, Li Y, Shao GR, Xiao J
Pediatr Surg Int 2021 May;37(5):597-606. Epub 2021 Jan 10 doi: 10.1007/s00383-020-04847-1. PMID: 33423101
Extrahepatic portal hypertension in chronic pancreatitis: an old problem revisited.
Izbicki JR, Yekebas EF, Strate T, Eisenberger CF, Hosch SB, Steffani K, Knoefel WT
Ann Surg 2002 Jul;236(1):82-9. doi: 10.1097/00000658-200207000-00013. PMID: 12131089Free PMC Article

Prognosis

Altered von Willebrand Factor and ADAMTS13 Levels in Children With Cirrhosis and Extrahepatic Portal Hypertension.
Islek A, Ilhan D, Ozturk N, Guven B, Sag E
J Pediatr Hematol Oncol 2021 Oct 1;43(7):e951-e956. doi: 10.1097/MPH.0000000000002038. PMID: 33369998
Distal Splenorenal Shunt (DSRS) in Children with Extrahepatic Portal Hypertension.
Rehman ZU, Nazir Z
J Coll Physicians Surg Pak 2019 Dec;29(12):1228-1229. doi: 10.29271/jcpsp.2019.12.1228. PMID: 31839103
Recurrent variceal haemorrhage managed with splenic vein stenting.
El Kininy W, Kearney L, Hosam N, Broe P, Keeling A
Ir J Med Sci 2017 May;186(2):323-327. Epub 2016 Feb 25 doi: 10.1007/s11845-016-1420-z. PMID: 26911860
Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance.
Bertocchini A, Falappa P, Grimaldi C, Bolla G, Monti L, de Ville de Goyet J
J Pediatr Surg 2014 Aug;49(8):1268-75. Epub 2013 Nov 15 doi: 10.1016/j.jpedsurg.2013.10.029. PMID: 25092088
Extrahepatic portal hypertension: quality of life and somatic growth after surgery.
Menon P, Rao KL, Bhattacharya A, Thapa BR, Chowdhary SK, Mahajan JK, Samujh R
Eur J Pediatr Surg 2005 Apr;15(2):82-7. doi: 10.1055/s-2004-830341. PMID: 15877255

Clinical prediction guides

Angiographic patterns of portal venous system in children with extrahepatic portal hypertension and its etiological and clinical relevance.
Yuldashev RZ, Aliev MM, Maksudov MF, Khaydarov UO, Shokhaydarov S
Pediatr Surg Int 2023 Feb 1;39(1):97. doi: 10.1007/s00383-023-05384-3. PMID: 36723662
Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults (with video).
Brichard M, Iesari S, Lerut J, Reding R, Goffette P, Coubeau L
Hepatobiliary Pancreat Dis Int 2022 Feb;21(1):25-32. Epub 2021 Aug 14 doi: 10.1016/j.hbpd.2021.08.003. PMID: 34426078
Portal biliopathy as a complication of extrahepatic portal hypertension: etiology, presentation and management.
Le Roy B, Gelli M, Serji B, Memeo R, Vibert E
J Visc Surg 2015 Jun;152(3):161-6. Epub 2015 May 27 doi: 10.1016/j.jviscsurg.2015.04.003. PMID: 26025414
Extrahepatic portal hypertension: quality of life and somatic growth after surgery.
Menon P, Rao KL, Bhattacharya A, Thapa BR, Chowdhary SK, Mahajan JK, Samujh R
Eur J Pediatr Surg 2005 Apr;15(2):82-7. doi: 10.1055/s-2004-830341. PMID: 15877255
Extrahepatic portal hypertension in chronic pancreatitis: an old problem revisited.
Izbicki JR, Yekebas EF, Strate T, Eisenberger CF, Hosch SB, Steffani K, Knoefel WT
Ann Surg 2002 Jul;236(1):82-9. doi: 10.1097/00000658-200207000-00013. PMID: 12131089Free PMC Article

Recent systematic reviews

Pancreatic Surgery in Chronic Pancreatitis Complicated by Extrahepatic Portal Hypertension or Cavernous Transformation of the Portal Vein: A Systematic Review.
Latorre Fragua RA, Manuel Vázquez A, López Marcano AJ, Gijón De La Santa L, de la Plaza Llamas R, Ramia Ángel JM
Scand J Surg 2020 Sep;109(3):177-186. Epub 2019 Jun 23 doi: 10.1177/1457496919857260. PMID: 31232190

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