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Sarangi Y, Behari A, Malage S, Kumar A, Singh RK. Left Lateral Sectionectomy With Intrahepatic Cholangiojejunostomy for Portal Cavernoma Cholangiopathy (PCC)-A Novel Approach. J Clin Exp Hepatol 2025; 15:102535. [PMID: 40236521 PMCID: PMC11995097 DOI: 10.1016/j.jceh.2025.102535] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2024] [Accepted: 02/21/2025] [Indexed: 04/17/2025] Open
Abstract
The development of portal cavernoma cholangiopathy (PCC) in cases of extrahepatic portal vein obstruction (EHPVO) presents significant management challenges. Strictures, stones, and extensive collaterals at the porta hepatis contribute to considerable surgical complexity. The traditional surgical approach for such patients involves a portosystemic shunt, followed by hepaticojejunostomy when indicated. In carefully selected cases, left lateral sectionectomy combined with intrahepatic cholangiojejunostomy (Longmire's procedure) offers a viable and durable long-term solution. We present two cases where this approach was successfully employed. Both the patients had sub-hilar strictures, and a large stone burden localized to the left lateral section of the liver. Conventional hepaticojejunostomy was contraindicated due to a heavily collateralized hilum, which precluded a safe landing zone. In these two cases, left lateral sectionectomy was performed to clear the large intrahepatic stone burden, with intrahepatic cholangiojejunostomy providing effective biliary drainage while avoiding the heavily collateralized hilar and pericholedochal regions.
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Affiliation(s)
- Yajnadatta Sarangi
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, India
| | - Anu Behari
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, India
| | - Somanath Malage
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, India
| | - Ashok Kumar
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, India
| | - Rajneesh K. Singh
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow-226014, India
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Lim C, Saliba F, Salloum C, Azoulay D. Revisiting the place of surgical portal decompression for adults with noncirrhotic portal hypertension due to chronic extrahepatic portal vein obstruction: a scoping review. HPB (Oxford) 2025; 27:434-444. [PMID: 39863431 DOI: 10.1016/j.hpb.2025.01.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2024] [Revised: 11/22/2024] [Accepted: 01/07/2025] [Indexed: 01/27/2025]
Abstract
BACKGROUND Liver cirrhosis accounts for more than 90 % of portal hypertension cases, and the other cases are due to noncirrhotic portal hypertension (NCPH). Variceal bleeding is the most life-threatening complication of portal hypertension and its primary treatment is medical according to the Baveno VII guidelines. This review discusses the evidence on surgical portal decompression for adult patients with NCPH secondary to chronic extrahepatic portal vein obstruction (EHPVO). METHODS This is a scoping review of the evidence for the feasibility and effectiveness of surgical portal decompression in adults with NCPH secondary to EHPVO. RESULTS This scoping review yielded 17 studies, including a total of 110 patients. Patient age(s) ranged from 19 to 68 years, with the majority undergoing nonphysiological (i.e., portosystemic shunts) shunts (N = 84, 76.4 %), mostly for variceal bleeding refractory to medical and endoscopic treatments. Physiological shunts (i.e., Rex shunts) had a potential advantage over nonphysiological shunts in postoperative rebleeding (5 % vs. 10 %) and hepatic encephalopathy rates (0 % vs. 13 %). Conversely, nonphysiological shunts had a potential advantage over physiological shunts in postoperative shunt thrombosis (8 % vs. 22 %). DISCUSSION This scoping review reported that surgical portal decompression is feasible in adults with NCPH due to EHPVO with favorable outcomes and long-term patency.
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Affiliation(s)
- Chetana Lim
- Department of Digestive, Hepato-Biliary and Pancreatic Surgery and Liver Transplantation, AP-HP Pitié-Salpêtrière Hospital, Paris, France; Research Unit, Université de Picardie-Jules Verne, UR UPJV 7518 SSPC, Amiens, France
| | - Faouzi Saliba
- Hepato-Biliary Center, AP-HP Paul Brousse Hospital, Paris-Saclay University, INSERM Unit 1193, 94800 Villejuif, France
| | - Chady Salloum
- Hepato-Biliary Center, AP-HP Paul Brousse Hospital, Paris-Saclay University, INSERM Unit 1193, 94800 Villejuif, France
| | - Daniel Azoulay
- Hepato-Biliary Center, AP-HP Paul Brousse Hospital, Paris-Saclay University, INSERM Unit 1193, 94800 Villejuif, France.
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Goel P, Bhatnagar V, Chennur VS. Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population. J Indian Assoc Pediatr Surg 2024; 29:152-158. [PMID: 38616824 PMCID: PMC11014182 DOI: 10.4103/jiaps.jiaps_21_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2021] [Revised: 05/23/2021] [Accepted: 10/25/2021] [Indexed: 04/16/2024] Open
Abstract
Background and Objectives More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. Materials and Methods During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Results Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Conclusions Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.
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Affiliation(s)
- Prabudh Goel
- Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
| | - Veereshwar Bhatnagar
- Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India
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Lal R, Behari A, Sarma MS, Yachha SK, Mandelia A, Srivastava A, Poddar U. Portosystemic Shunt Surgery for Extrahepatic Portal Venous Obstruction Beyond Endoscopic Variceal Eradication: Two Decades of Pediatric Surgical Experience. J Clin Exp Hepatol 2023; 13:997-1007. [PMID: 37975042 PMCID: PMC10643506 DOI: 10.1016/j.jceh.2023.06.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/21/2023] [Accepted: 06/06/2023] [Indexed: 11/19/2023] Open
Abstract
Background This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome. Methods EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV. Results One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism (n = 132, 95%); ii) growth retardation (GR, n = 95, 68%); iii) isolated gastric (IGV) and ectopic varices (n = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) (n = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy (n = 104); side-to-side splenorenal shunt (n = 4); mesocaval shunt (n = 1); inferior mesenteric vein (IMV) to left renal vein shunt (n = 2); IMV to inferior vena cava shunt (n = 3); H-graft interposition splenorenal shunt (n = 1); spleno-adrenal shunt (n = 3); makeshift shunt (n = 1). Esophagogastric devascularization (n = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly. Conclusions Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.
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Affiliation(s)
- Richa Lal
- Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Anu Behari
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Moinak S. Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Surender K. Yachha
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Ankur Mandelia
- Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Anshu Srivastava
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
| | - Ujjal Poddar
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India
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Ochoa G, Marino C, Dib M, Briceño E, Martinez JA, Jarufe N. Roux-en-Y biliary reconstruction as a definitive treatment for serious complications of portal biliopathy. Case series. Int J Surg Case Rep 2023; 110:108571. [PMID: 37574629 PMCID: PMC10448269 DOI: 10.1016/j.ijscr.2023.108571] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Revised: 07/15/2023] [Accepted: 07/23/2023] [Indexed: 08/15/2023] Open
Abstract
INTRODUCTION AND IMPORTANCE Portal biliopathy (PB) is an abnormality of the biliary tree wall due to extrahepatic portal hypertension. Among the complications of portal biliopathy are digestive bleeding, jaundice, and cholangitis. Surgical treatment is an exception when medical management is not possible. CASE PRESENTATION This is a case series study of four patients with severe PB complications requiring surgical management in our center from 2005 to 2016. Two of them had previous surgical procedures related to portal hypertension. All presented with severe biliary stenosis and recurrent cholangitis, and two also had massive upper gastrointestinal bleeding. Because of endoscopic management failure, a Roux-en-Y hepaticojejunostomy was performed in all cases. Two patients presented morbidity Clavien-Dindo>IIIA, requiring reoperation. During follow-up, no one developed other complications related to PB. DISCUSSION Surgical treatment for PB complications is a challenge and mainly implies a portosystemic shunt as a first step. When it fails, an alternative is perform a biliodigestive anastomoses, with high risk of bleeding given the prominent collaterals present in the hepatoduodenal pedicle secondary to portal cavernomatosis. CONCLUSION Our patients after YRGB didn't present new complications due to PB. The surgery could be a definite solution for PB complications. It has only been made for selective cases because it implies high complexity and risk.
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Affiliation(s)
- Gabriela Ochoa
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile
| | - Carlo Marino
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile
| | - Martín Dib
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile
| | - Eduardo Briceño
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile
| | - Jorge A Martinez
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile
| | - Nicolas Jarufe
- Department of Digestive Surgery, Pontificia Universidad Católica de Chile's Hospital, Santiago, Chile.
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Poddar U, Reddy DVU. Non-Cirrhotic Portal Hypertension in Children: Current Management Strategies. CURRENT HEPATOLOGY REPORTS 2023; 22:158-169. [DOI: 10.1007/s11901-023-00608-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 06/08/2023] [Indexed: 01/05/2025]
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Bhavsar R, Yadav A, Nundy S. Portal cavernoma cholangiopathy: Update and recommendations on diagnosis and management. Ann Hepatobiliary Pancreat Surg 2022; 26:298-307. [PMID: 36168271 PMCID: PMC9721250 DOI: 10.14701/ahbps.22-029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2022] [Accepted: 06/07/2022] [Indexed: 12/15/2022] Open
Abstract
Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.
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Affiliation(s)
- Ruchir Bhavsar
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India,Corresponding author: Ruchir Bhavsar, MS, Fellowship in Surgical Gastroenterology and Liver Transplantation Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi 110060, India Tel: +91-9898269932, E-mail: ORCID: https://orcid.org/0000-0002-7026-5245
| | - Amitabh Yadav
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India
| | - Samiran Nundy
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India
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8
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Conticchio M, Salloum C, Allard MA, Golse N, Pittau G, Ciacio O, Vibert E, Sa Cunha A, Cherqui D, Adam R, Azoulay D. The rex shunt for left portal vein reconstruction during hepatectomy for malignancy using of rex-shunt in adults for oncoliver surgery. Surg Endosc 2022; 36:8249-8254. [PMID: 35441315 DOI: 10.1007/s00464-022-09270-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2022] [Accepted: 04/09/2022] [Indexed: 01/06/2023]
Abstract
BACKGROUND Immediate portal reperfusion is mandatory following hepatectomy combined with portal vein (PV) resection. This retrospective study analyzes the feasibility and the outcomes of the Rex shunt (RS) for reconstruction of the left portal vein (LPV) and reperfusion of the remnant left liver or lobe following hepatectomy for cancer combined with resection of the PV in adult patients. METHODS From 2018 to 2021, an RS was used in the above setting to achieve R0 resection or when the standard LPV reconstruction failed or was deemed technically impossible. RESULTS There were 6 male and 5 female patients (median age, 58 years) with perihilar cancer (5 cases) or miscellaneous cancers invading the PV (6 cases). A major hepatectomy was performed in 10/11 patients. The RS was indicated to achieve R0 resection or for technical reasons in 8 and 3 cases, respectively, and was feasible in all consecutive attempts with (10 cases) or without an interposed synthetic graft (1 case). Two fatal complications (PV thrombosis and pulmonary embolism) and three non-severe complications occurred in four patients within 90 days of surgery. Two patients died of tumor recurrence with a patent RS at 13 and 29 months, and 7 were recurrence free with a patent shunt with a follow-up of 1 to 37 months (median, 15 months). CONCLUSION In case of remnant left liver or lobe following hepatectomy combined with resection of the PV, the RS may help to achieve R0 resection and is a valuable option to perform technically satisfying portal reperfusion of the remnant left liver or lobe.
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Affiliation(s)
- Maria Conticchio
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France
| | - Chady Salloum
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France
| | - Marc Antoine Allard
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Nicolas Golse
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Gabriella Pittau
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Oriana Ciacio
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Eric Vibert
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Antonio Sa Cunha
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Daniel Cherqui
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - René Adam
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France.,Université Paris-Saclay, Saclay, France.,Institut National de la Santé et de la Recherche Médicale (INSERM) Unité 935, Villejuif, France
| | - Daniel Azoulay
- Centre Hépato-Biliaire, Assistance Publique - Hôpitaux de Paris, Hôpital Universitaire Paul Brousse, 94000, Villejuif, France. .,Université Paris-Saclay, Saclay, France.
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Plessier A. Extrahepatic Portal Vein Obstruction: Recent Portal Vein Thrombosis and Portal Cavernoma in the Absence of Cirrhosis. VASCULAR DISORDERS OF THE LIVER 2022:93-109. [DOI: 10.1007/978-3-030-82988-9_7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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10
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Sarma MS, Seetharaman J. Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations. World J Hepatol 2021; 13:1269-1288. [PMID: 34786165 PMCID: PMC8568571 DOI: 10.4254/wjh.v13.i10.1269] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2021] [Revised: 06/27/2021] [Accepted: 09/19/2021] [Indexed: 02/06/2023] Open
Abstract
Non-cirrhotic portal hypertension (NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias (hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure, especially in extra-hepatic portal vein obstruction (EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life (QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis (NCPF) is a less common cause of portal hypertension in children in developing nations. Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis (CHF) presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli’s disease, renal cysts, and syndromes associated with neurological defects. Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplantation is required when there is decompensation or recurrent cholangitis, especially in Caroli’s syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.
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Affiliation(s)
- Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Jayendra Seetharaman
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
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11
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Srisajjakul S, Prapaisilp P, Bangchokdee S. Imaging features of vascular compression in abdomen: Fantasy, phenomenon, or true syndrome. Indian J Radiol Imaging 2021; 27:216-224. [PMID: 28744083 PMCID: PMC5510320 DOI: 10.4103/ijri.ijri_7_17] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022] Open
Abstract
Vascular structures in the abdomen can compress or be compressed by adjacent structures. Classic imaging findings of vascular compressions, including median arcuate ligament syndrome, superior mesenteric artery syndrome, nutcracker syndrome, portal biliopathy, May-Thurner syndrome, and ureteropelvic junction obstruction will be discussed here. It is important to correlate imaging findings and clinical data to identify asymptomatic vascular compression which requires no treatment, intermittent vascular compression with nonspecific or vague clinical manifestation, and the subset of patients with true syndromes who will benefit from treatment.
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Affiliation(s)
- Sitthipong Srisajjakul
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
| | - Patcharin Prapaisilp
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand
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12
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Current Indications and Long-Term Outcomes of Surgical Portosystemic Shunts in Adults. J Gastrointest Surg 2021; 25:1437-1444. [PMID: 32424687 DOI: 10.1007/s11605-020-04643-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2020] [Accepted: 05/03/2020] [Indexed: 01/31/2023]
Abstract
BACKGROUND Surgical portosystemic shunts are rare. We reviewed indications, operative details, and outcomes of patients undergoing surgical portosystemic shunt procedures. METHODS We retrospectively reviewed clinical data of consecutive patients between 1997 and 2018 from a single institution. Clinical characteristics and outcomes were compared between two groups: patients with portomesenteric venous thrombosis (PMVT) vs those with cirrhosis. Endpoints included 30-day mortality, shunt-related complications, patency, and survival. RESULTS There were 99 patients, 45 male and 54 female, with a mean age of 46 ± 18 years, enrolled in the study. There were 63 patients (63%) with PMVT and 36 patients (36%) with cirrhosis. Both groups had similar demographics, cardiovascular risk factors, and aneurysm extent, except for more diabetes among those with cirrhosis (p < 0.05). There were no significant differences in procedural metrics and intra-procedure complications between groups, except that patients with PMVT underwent more non-selective shunts than those with cirrhosis (63% vs. 30%, p < 0.001). There were two 30-day deaths (2%), with no difference in mortality and MAEs between groups. On univariate analysis, cholangiopathy and PMVT were associated with graft thrombosis (HR = 9.22, 95% CI 1.22-70.27) while race, smoking, cardiac comorbidity, type of operative shunt, configuration of the shunt, and use of conduit were not (p > 0.05). Patients with PMVT had significantly lower 1-, 5-, and 10-year primary (77%, 71%, and 71% vs. 97%, p = 0.009) and secondary patency (88%, 76%, and 72% vs. 96%, p = 0.027) compared with those with cirrhosis. The 1-, 5-, and 10-year survival rates were 94%, 84%, and 61% for patients with PMVT compared with 88%, 58%, and 26% for those with cirrhosis (non-adjusted HR 0.40, 95% CI 0.19-0.84, p = 0.01, age-adjusted HR 0.51, 95% CI 0.24-1.09, p = 0.08). The survival of patients with PMVT without liver disease trended higher than those with liver disease; however, when adjusted for age, the survival gap narrowed, and the difference was not statistically significant (p = 0.19), survival being lowest for those with PMVT and liver disease. CONCLUSIONS Surgical portosystemic shunts are safe and effective for symptom relief in selected patients with portal hypertension. The odds of graft thrombosis is 9 times higher in patients with PMVT. Overall survival is similar in patients with PMVT or cirrhosis.
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Itare VB, Imanirad D, Almaghraby A. Portal Cholangiopathy: An Uncommon Cause of Right Upper Quadrant Pain. Cureus 2020; 12:e10281. [PMID: 33042716 PMCID: PMC7538209 DOI: 10.7759/cureus.10281] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2020] [Accepted: 09/06/2020] [Indexed: 11/05/2022] Open
Abstract
Portal cholangiopathy is one of the complications of the chronic portal vein thrombosis (PVT). Chronic PVT can occur in a patient with acute PVT that usually does not resolve regardless of the treatment. There is a development of collateral blood vessels that bring blood from the portal system towards the liver around the obstruction area, known as the cavernous transformation of the portal vein or portal cavernoma, in a patient with chronic PVT. The appearance and location of collateral channels depends on the extent and location of thrombus in the portomesenteric venous system. If the portomesenteric venous system is occluded near the formation of the portal vein, blood tends to flow through collateral channels that form varices in and around the common bile duct. Portal cholangiopathy (also referred to as portal biliopathy) is common in patients with long-standing chronic PVT. It is due to compression of the large bile ducts by the venous collaterals that form in patients with chronic PVT. Most of the patients with long-standing PVT have portal cholangiopathy. Typically, symptoms of portal cholangiopathy include jaundice, biliary colic, and pruritus. Portal cholangiopathy is a rare complication of chronic portal hypertension, and it is an important differential diagnosis of biliary colic secondary to cholelithiasis. The patient can also present with the sharp right upper quadrant pain, which is atypical by nature for biliary colic.
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Affiliation(s)
- Vikram B Itare
- Internal Medicine, Smolensk State Medical University, Smolensk, RUS
| | - Donya Imanirad
- Internal Medicine, University of South Florida, Tampa, USA
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Sarma MS, Ravindranath A. Portal Cavernoma Cholangiopathy in Children and the Management Dilemmas. J Clin Transl Hepatol 2020; 8:61-68. [PMID: 32274346 PMCID: PMC7132017 DOI: 10.14218/jcth.2019.00041] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/02/2019] [Revised: 12/08/2019] [Accepted: 01/01/2020] [Indexed: 12/19/2022] Open
Abstract
Portal cavernoma cholangiopathy (PCC) is one of the most harrowing complications of extrahepatic portal venous obstruction, as it determines the long-term hepatobiliary outcome. Although symptomatic PCC is rare in children, asymptomatic PCC is as common as that in adults. However, there are major gaps in the literature with regard to the best imaging strategy and management modality in children. Moreover, natural history of PCC and effect of portosystemic shunt surgeries in children are unclear. Neglected PCC would lead to difficult or recalcitrant biliary strictures that will require endoscopic therapy or bilioenteric anastomosis, both of which are challenging in the presence of extensive collaterals. There are limited studies on the effect of portosystemic shunt surgeries on the outcome of PCC in children compared to adults. In this review, we aimed to collate all existing literature on PCC in childhood and also compare with adult studies. We highlight the difficulties of this disease to provide a comprehensive platform to foster further research on PCC exclusively in children.
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Affiliation(s)
- Moinak Sen Sarma
- Correspondence to: Moinak Sen Sarma, Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India. Tel: +91- 522-2495379, E-mail:
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Ravindranath A, Sen Sarma M, Yachha SK, Lal R, Singh S, Srivastava A, Poddar U, Neyaz Z, Behari A. Outcome of portosystemic shunt surgery on pre-existing cholangiopathy in children with extrahepatic portal vein obstruction. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2019; 27:141-148. [PMID: 31654467 DOI: 10.1002/jhbp.692] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
BACKGROUND This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal vein obstruction (EHPVO). METHODS Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification. Those with patent shunt were re-evaluated at least 6 months after surgery with MRC, MRPV and compared with pre-shunt images. RESULTS Twenty-five children underwent PSS (central end to side splenorenal shunt with splenectomy [n = 24], mesocaval shunt [n = 1]). Pre-surgery MRC showed PCC grade I in 11, grade II in 1 and grade III in 13. MRPV showed superior mesenteric vein (SMV) block in 20. Re-assessment for PCC 18 (6 to 54) months after surgery showed grade I in 6 and grade III in 19. Thus, PCC was progressive in 6 and static in 19. Density of peribiliary collaterals decreased in 5 (SMV patent, static PCC), increased in 3 and remained unchanged in 17. Splenomegaly-related problems, gastroesophageal varices and other intra-abdominal (esophageal, perisplenic and perigastric) collaterals ameliorated in all. CONCLUSION Non-selective PSS decompresses esophago-gastro-splenic venous circuit effectively but fails to ameliorate cholangiopathy and peribiliary collaterals. Persistence of cholangiopathy is attributable to SMV block.
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Affiliation(s)
- Aathira Ravindranath
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Surender Kumar Yachha
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Richa Lal
- Department of Pediatric Surgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Somesh Singh
- Department of Radiodiagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Anshu Srivastava
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Ujjal Poddar
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Zafar Neyaz
- Department of Radiodiagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Anu Behari
- Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
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Saied Abdelgawad M, Abouyoussef RA. Portal biliopathy; multidetector CT (MDCT) imaging features. THE EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE 2018. [DOI: 10.1016/j.ejrnm.2018.01.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/17/2022] Open
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Shukla A, Gupte A, Karvir V, Dhore P, Bhatia S. Long Term Outcomes of Patients with Significant Biliary Obstruction Due to Portal Cavernoma Cholangiopathy and Extra-Hepatic Portal Vein Obstruction (EHPVO) With No Shuntable Veins. J Clin Exp Hepatol 2017; 7:328-333. [PMID: 29234198 PMCID: PMC5715445 DOI: 10.1016/j.jceh.2017.04.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2017] [Accepted: 04/27/2017] [Indexed: 12/12/2022] Open
Abstract
AIMS The natural history of portal cavernoma cholangiopathy (PCC) in patients with significant biliary obstruction (SBO) who cannot undergo shunt surgery, is not known. We therefore, analyzed data of patients of extra-hepatic portal venous obstruction (EHPVO) with PCC. METHODS Prospectively recorded details of 620 (age 21.2 [11.4] years; 400 [65%] males) patients with primary EHPVO were reviewed. Outcomes (hepatic decompensation/mortality) of patients with PCC and SBO without shuntable veins were noted at follow up of 7 [4-11] years. RESULTS Ninety-seven of 620 (15.6% [60 men]) EHPVO patients had PCC-SBO. Of these 57 did not have shuntable veins. The median duration from any index symptom to symptomatic PCC was 7 (0-24) years and from index bleed to symptomatic PCC was and 12 (5-24) years, respectively. Thirteen patients underwent endoscopic retrograde cholangiography; nine repeatedly over 7 (4-10) years. Decompensation was seen in 5 patients. Presentation other than variceal bleed was associated with hepatic decompensation (5/19 versus 0/38, P = 0.003). CONCLUSIONS Majority of patients with PCC-SBO do not have shuntable veins, and may have good long-term outcomes. Patients presenting with variceal bleed have low chance of decompensation. Symptomatic PCC appears to be a late event in EHPVO.
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Affiliation(s)
| | | | | | | | - Shobna Bhatia
- Address for correspondence: Shobna Bhatia, Professor and Head, Department of Gastroenterology, 11th Floor, Multi-Storey Building, KEM Hospital and Seth GS Medical College, Mumbai 400012, Maharashtra, India. Tel.: +91 9869072213; fax: +91 22 4103057.Shobna Bhatia, Professor and Head, Department of Gastroenterology, 11th Floor, Multi-Storey Building, KEM Hospital and Seth GS Medical CollegeMumbai400012India
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Lal R, Sarma MS, Gupta MK. Extrahepatic Portal Venous Obstruction: What Should be the Mainstay of Treatment? Indian J Pediatr 2017; 84:691-699. [PMID: 28612224 DOI: 10.1007/s12098-017-2390-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2017] [Accepted: 05/15/2017] [Indexed: 02/07/2023]
Abstract
The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system. While endotherapy obliterates natural porto-systemic collaterals in the gastroesophageal region, persistently raised portal pressures manifest as an increase in secondary isolated gastric varices, ectopic varices, portal hypertensive vasculopathy, issues related to massive splenomegaly, portal biliopathy, growth retardation and hence impaired quality of life (QOL). An ideal management strategy should address both bleed and non-bleed consequences of EHPVO and translate into a near normal QOL. Further, MRB has opened up new dimensions to the management philosophy of EHPVO. This review article critically evaluates the role of surgery and endotherapy based on available literature and authors' own experience.Surgery and endotherapy are complementary. However, with increasing duration of follow-up post EEEV, it is evident that there is resurgence in the role of surgery (PSS/MRB) as a single one time definitive procedure for alleviating all bleed and delayed non bleed sequelae of EHPVO.Surgery for EHPVO (PSS/MRB) should not be allowed to become a dying art and future generations of surgeons should continue to receive training in this specialized area of surgery.
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Affiliation(s)
- Richa Lal
- Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, 226014, India.
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, India
| | - Manish K Gupta
- Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh, 226014, India
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Portal Hypertensive Biliopathy Presents with Massive Bleeding during ERCP after Balloon Sphincteroplasty in a Noncirrhotic Saudi Sickler Patient. Case Rep Med 2017; 2017:4163919. [PMID: 28588619 PMCID: PMC5446870 DOI: 10.1155/2017/4163919] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2017] [Accepted: 04/19/2017] [Indexed: 02/03/2023] Open
Abstract
Portal hypertensive biliopathy (PHB) is described as abnormalities of the walls of the biliary tree secondary to portal hypertension. Gastrointestinal bleeding caused by PHB is rare. PHB as a cause of serious bleeding after sphincteroplasty during ERCP is extremely rare. Here, we report a case of PHB in a young Saudi male with cell sickle anemia who developed massive hemorrhage during ERCP after balloon dilation of the ampulla of Vater. We further discussed the diagnosis and management. To the best of our knowledge, no such case has been reported.
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Outcomes of surgical shunts and transjugular intrahepatic portasystemic stent shunts for complicated portal hypertension. Br J Surg 2017; 104:443-451. [DOI: 10.1002/bjs.10431] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2016] [Revised: 10/09/2016] [Accepted: 10/15/2016] [Indexed: 12/14/2022]
Abstract
Abstract
Background
Transjugular intrahepatic portasystemic stent shunt (TIPSS), instead of surgical shunt, has become the standard treatment for patients with complicated portal hypertension. This study compared outcomes in patients who underwent TIPSS or surgical shunting for complicated portal hypertension.
Methods
This was a retrospective study of all consecutive patients who received portasystemic shunts from 1994 to 2014 at a single institution. Patients who underwent surgical shunting were compared with those who had a TIPSS procedure following one-to-one propensity score matching. The primary study endpoints were overall survival and shunt failure, defined as major variceal rebleeding, relapse of refractory ascites, irreversible shunt occlusion, liver failure requiring liver transplantation, or death.
Results
A total of 471 patients received either a surgical shunt or TIPSS. Of these, 334 consecutive patients with cirrhosis who underwent elective surgical shunting (34) or TIPSS (300) for repeated variceal bleeding or refractory ascites were evaluated. Propensity score matching yielded 31 pairs of patients. There were no between-group differences in morbidity and 30-day mortality rates. However, shunt failure was less frequent after surgical shunting than TIPSS (6 of 31 versus 16 of 31; P = 0·016). The 5-year shunt failure-free survival (77 versus 15 per cent; P = 0·008) and overall survival (93 versus 42 per cent; P = 0·037) rates were higher for patients with surgical shunts. Multivariable analysis revealed that a Model for End-Stage Liver Disease (MELD) score exceeding14 and TIPSS were independently associated with shunt failure. In patients with MELD scores of 14 or less, the 5-year overall survival rate remained higher after surgical shunting than TIPSS (100 versus 40 per cent; P < 0·001).
Conclusion
Surgical shunting achieved better results than TIPSS in patients with complicated portal hypertension and low MELD scores.
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Franceschet I, Zanetto A, Ferrarese A, Burra P, Senzolo M. Therapeutic approaches for portal biliopathy: A systematic review. World J Gastroenterol 2016; 22:9909-9920. [PMID: 28018098 PMCID: PMC5143758 DOI: 10.3748/wjg.v22.i45.9909] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/26/2016] [Revised: 10/12/2016] [Accepted: 11/14/2016] [Indexed: 02/06/2023] Open
Abstract
Portal biliopathy (PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/non-neoplastic extrahepatic portal vein obstruction (EHPVO) and portal cavernoma (PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC (77%-100%), only a part of these (5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic (Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical (bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.
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Khuroo MS, Rather AA, Khuroo NS, Khuroo MS. Portal biliopathy. World J Gastroenterol 2016; 22:7973-7982. [PMID: 27672292 PMCID: PMC5028811 DOI: 10.3748/wjg.v22.i35.7973] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2016] [Revised: 06/22/2016] [Accepted: 08/01/2016] [Indexed: 02/06/2023] Open
Abstract
Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures (hepaticojejunostomy or choledechoduodenostomy).
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Safety of supramesocolic surgery in patients with portal cavernoma without portal vein decompression. Large single centre experience. HPB (Oxford) 2016; 18:623-9. [PMID: 27346144 PMCID: PMC4925800 DOI: 10.1016/j.hpb.2016.05.009] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/15/2016] [Revised: 05/15/2016] [Accepted: 05/21/2016] [Indexed: 12/12/2022]
Abstract
BACKGROUND Supra-mesocolic surgery (SMS) is complicated in patients with portal vein cavernoma (PC) and portal decompression is recommended. The aim of this study was to report a large single centre of SMS in patients with PC without portal decompression. METHODS Between 2006 and 2013, all patients who met inclusion criteria were analyzed retrospectively. The primary endpoint was the feasibility rate, surgical and postoperative outcome. The secondary endpoints were the long-term outcome of patients who underwent biliary bypass for cholangitis. Risk factors for complications were studied. RESULTS Thirty patients underwent 51 procedures. Pancreatitis was the main etiology of PC (19/30) and biliary obstruction was mainly related to the underlying disease and not to portal cholangiopathy (12/14). All planned procedures were successfully completed. Fourteen patients underwent biliary bypass. Median blood loss (250 ml), transfusion (n = 7), mortality (n = 0), overall morbidity (n = 12) and the median hospital stay (10 days). Good long-term control of cholangitis was achieved in the 9 patients alive with available follow-up. Significant risk factors for complications were a previous abdominal wall scar, previous intra-abdominal surgical field and liver fibrosis. CONCLUSION SMS can be safely performed in patients with PC. In patients with risk factors for complications, portal decompression should be discussed.
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Portal Vein Stenting for Portal Biliopathy with Jaundice. Cardiovasc Intervent Radiol 2015; 39:620-3. [PMID: 26518011 DOI: 10.1007/s00270-015-1222-0] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2015] [Accepted: 09/19/2015] [Indexed: 12/14/2022]
Abstract
Portal biliopathy refers to obstruction of the bile duct by dilated peri- or para-ductal collateral channels following the main portal vein occlusion from various causes. Surgical shunt operation or endoscopic treatment has been reported. Herein, we report a case of portal biliopathy that was successfully treated by interventional portal vein recanalization.
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Le Roy B, Gelli M, Serji B, Memeo R, Vibert E. Portal biliopathy as a complication of extrahepatic portal hypertension: etiology, presentation and management. J Visc Surg 2015; 152:161-6. [PMID: 26025414 DOI: 10.1016/j.jviscsurg.2015.04.003] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Portal biliopathy (PB) refers to the biliary abnormalities of the biliary ducts observed in patients with extrahepatic portal hypertension. Although majority of patients are asymptomatic, approximately 20% of these patients present with biliary symptoms (pain, pruritus, jaundice, cholangitis). The pathogenesis of PB is uncertain but compression by dilated veins into or around common bile duct may play the main role. CT-scan, MR cholangiopancreatography with MR portography should be the initial investigations in the evaluation of PB. Treatment is limited to symptomatic cases and is dictated by clinical manifestations and complications of the disease. Treatment of PB could be done by endoscopy (sphincterotomy, stone extraction or biliary stenting of the common bile duct) or surgery (definitive decompression by porto-systemic shunt followed by bilioenteric anastomosis, if necessary). This review describes pathogenesis, clinical features, investigation and management of portal biliopathy.
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Affiliation(s)
- B Le Roy
- Service de chirurgie et oncologie digestive, CHU de Clermont-Ferrand, 63003 Clermont-Ferrand, France; Service de chirurgie hépatobiliaire, centre hépato-bilaire Paul-Brousse, 94800 Villejuif, France
| | - M Gelli
- Service de chirurgie hépatobiliaire, centre hépato-bilaire Paul-Brousse, 94800 Villejuif, France
| | - B Serji
- Service de chirurgie hépatobiliaire, centre hépato-bilaire Paul-Brousse, 94800 Villejuif, France; Faculté de médecine, université Mohammed Premier Oujda, Morocco
| | - R Memeo
- Service de chirurgie hépatobiliaire, centre hépato-bilaire Paul-Brousse, 94800 Villejuif, France
| | - E Vibert
- Service de chirurgie hépatobiliaire, centre hépato-bilaire Paul-Brousse, 94800 Villejuif, France.
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Cardoso R, Casela A, Lopes S, Agostinho C, Souto P, Camacho E, Almeida N, Mendes S, Gomes D, Sofia C. Portal Hypertensive Biliopathy: An Infrequent Cause of Biliary Obstruction. GE-PORTUGUESE JOURNAL OF GASTROENTEROLOGY 2015; 22:65-69. [PMID: 28868376 PMCID: PMC5579995 DOI: 10.1016/j.jpge.2015.01.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/08/2014] [Accepted: 01/20/2015] [Indexed: 11/26/2022]
Abstract
Introduction Biliary obstruction is usually caused by choledocholithiasis. However, in some circumstances, alternative or concurring unusual ethiologies such as portal hypertensive biliopathy (PHB) must be considered. Clinical case We present the case of a 36-year-old female complaining of jaundice and pruritus. Liver function tests were compatible with biliary obstruction and the ultrasound scan of the abdomen showed dilatation of the intrahepatic biliary ducts, a dilated common bile duct (CBD) and biliary calculi. The computed tomography of the abdomen revealed a portal cavernoma encasing the CBD. Discussion Portal cavernoma, the hallmark of extrahepatic portal venous obstruction, can cause PHB. When symptomatic, chronic cholestasis is present if a dominant stricture exists whereas biliary pain and acute cholangitis occur when choledocholithiasis prevails. Management must be individualized and usually includes endoscopic therapy to address choledocholithiasis and shunt surgery for definitive treatment.
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Affiliation(s)
- Ricardo Cardoso
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Adriano Casela
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Sandra Lopes
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Cláudia Agostinho
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Paulo Souto
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Ernestina Camacho
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Nuno Almeida
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Sofia Mendes
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Dário Gomes
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
| | - Carlos Sofia
- Gastroenterology Department, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal
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Varma V, Behera A, Kaman L, Chattopadhyay S, Nundy S. Surgical management of portal cavernoma cholangiopathy. J Clin Exp Hepatol 2014; 4:S77-84. [PMID: 25755599 PMCID: PMC4244827 DOI: 10.1016/j.jceh.2013.07.005] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/03/2013] [Accepted: 07/09/2013] [Indexed: 12/12/2022] Open
Abstract
The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5-38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure.
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Key Words
- CBD, common bile duct
- EHBRD, extrahepatic biliary radical dilatation
- EHPVO, extrahepatic portal venous obstruction
- ERCP, endoscopic retrograde cholangiopancreatography
- GB, gall bladder
- HJ, hepaticojejunostomy
- IHBRD, intrahepatic biliary radical dilatation
- LFT, liver function tests
- NCPF, non cirrhotic portal fibrosis
- NPSS, non-portosystemic shunt
- PB, portal biliopathy
- PCC, portal cavernoma cholangiopathy
- PSS, portosystemic shunt
- PTBD, percutaneous transhepatic biliary drainage
- UGI, upper gastrointestinal
- biliary obstruction
- extrahepatic portal venous obstruction
- portal cavernoma cholangiopathy
- portal hypertension
- portosystemic shunt surgery
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Affiliation(s)
- Vibha Varma
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India,Address for correspondence: Vibha Varma, Consultant, Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India.
| | - Arunanshu Behera
- Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Leileshwar Kaman
- Department of Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
| | - Somnath Chattopadhyay
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India
| | - Samiran Nundy
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi 110060, India
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Maia MCA, Amaro AP, Oliveira EC, Melo JRDC, Sanches MD, Silva RAPD. Portal cholangiopathy: case report. Radiol Bras 2014. [DOI: 10.1590/s0100-39842014000100016] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
The present report describes the case of a child that after blunt abdominal trauma presented with portal thrombosis followed by progressive splenomegaly and jaundice. Ultrasonography and percutaneous cholangiography revealed biliary dilatation secondary to choledochal stenosis caused by dilated peribiliary veins, characterizing a case of portal biliopathy. The present case report is aimed at presenting an uncommon cause of this condition.
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Saraswat VA, Rai P, Kumar T, Mohindra S, Dhiman RK. Endoscopic management of portal cavernoma cholangiopathy: practice, principles and strategy. J Clin Exp Hepatol 2014; 4:S67-76. [PMID: 25755598 PMCID: PMC4244828 DOI: 10.1016/j.jceh.2013.08.011] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2013] [Accepted: 08/24/2013] [Indexed: 02/06/2023] Open
Abstract
Portal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied.
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Affiliation(s)
- Vivek A. Saraswat
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh 226014, India
| | - Praveer Rai
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh 226014, India
| | - Tarun Kumar
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh 226014, India
| | - Samir Mohindra
- Department of Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, Uttar Pradesh 226014, India
| | - Radha K. Dhiman
- Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India
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Puri P. Pathogenesis of Portal Cavernoma Cholangiopathy: Is it Compression by Collaterals or Ischemic Injury to Bile Ducts During Portal Vein Thrombosis? J Clin Exp Hepatol 2014; 4:S27-33. [PMID: 25755592 PMCID: PMC4244823 DOI: 10.1016/j.jceh.2013.05.015] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/08/2013] [Accepted: 05/31/2013] [Indexed: 12/12/2022] Open
Abstract
The pathogenesis of portal cavernoma cholangiopathy (PCC) is important as it can impact the choice of treatment modalities. PCC consists of a reversible component, which resolves by decompression of collaterals as well as a fixed component, which persists despite the decompression of collaterals. The reversible component is due to compression by large collaterals located adjacent to the bile duct as well as possibly intracholedochal varices. The fixed component is likely to be due to ischemia at the time of portal vein thrombosis, local ischemia by compression as well as encasement by a solid tumor-like cavernoma comprising of fibrous hilar mass containing multiple tiny collateral veins rather than markedly enlarged portal collaterals. Although cholangiographic abnormalities in portal hypertension are common, the prevalence of symptomatic PCC is low. This is likely to be related to the cause of portal hypertension, the duration of portal hypertension and possibly the pattern of occlusion of the splenoportal axis. There may possibly be higher prevalence of symptomatic PCC in extension of the thrombosis to the splenomesentric veins.
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Affiliation(s)
- Pankaj Puri
- Address for Correspondence: Pankaj Puri, Department of Gastroenterology, Army Hospital (Research and Referral), Dhaula Kuan, New Delhi 110010, India. Tel.: +91 9717233996.
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Portal cavernoma cholangiopathy: consensus statement of a working party of the Indian national association for study of the liver. J Clin Exp Hepatol 2014; 4:S2-S14. [PMID: 25755591 PMCID: PMC4274351 DOI: 10.1016/j.jceh.2014.02.003] [Citation(s) in RCA: 68] [Impact Index Per Article: 6.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2014] [Accepted: 02/02/2014] [Indexed: 12/12/2022] Open
Abstract
Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery.
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Key Words
- CBD, common bile duct
- CHD, common hepatic duct
- CT, computed tomography
- EHPVO, extrahepatic portal venous obstruction
- ERC, endoscopic retrograde cholangiography
- EUS, endoscopic ultrasound
- GRADE, Grading of Recommendations, Assessment, Development and Evaluation
- INASL, Indian National Association for Study of the Liver
- MRC, magnetic resonance cholangiography
- MRI, magnetic resonance imaging
- NCPF, non-cirrhotic portal fibrosis
- PSS, portosystemic shunt
- PVT, portal vein thrombosis
- UDCA, ursodeoxycholic acid
- USG, ultrasound
- cholestasis
- extrahepatic portal venous obstruction
- gallbladder varices
- obstructive jaundice
- portal hypertensive biliopathy
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Cellich PP, Crawford M, Kaffes AJ, Sandroussi C. Portal biliopathy: multidisciplinary management and outcomes of treatment. ANZ J Surg 2013; 85:561-6. [PMID: 24237891 DOI: 10.1111/ans.12436] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/12/2013] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Portal biliopathy (PB) is a rare condition in which portal hypertension because of extrahepatic portal vein obstruction can lead to biliary abnormalities, with some patients developing obstructive jaundice. At present, there is no international consensus on the management of PB. We present the experience of an Australian tertiary referral hospital with the diagnosis and management of PB, and compare this with reported international experience. METHODS The records of nine patients presenting with PB between June 2003 and March 2012 were reviewed and analysed. RESULTS All patients had portal hypertension because of portal vein thrombosis, with seven patients showing cavernous transformation of the portal vein. Biliary abnormality presented with jaundice (3/9), abdominal pain (2/9) or without symptoms (3/9). All patients developed a cholestatic pattern of liver function tests (LFTs). First-line endoscopic management was employed in 7 of 8 symptomatic patients. Four patients required endoscopic management alone (sphincterotomy alone (1/9), single stent (2/9), repeated stent changes (1/9) ), while four required second-line surgical intervention (portosystemic shunt (1/9), bilioenteric anastomosis (3/9) ). All patients were well, with stable LFTs, at median 18-month follow-up, with two patients undergoing regular stent changes, and the remainder requiring no further intervention. CONCLUSION PB can be managed successfully with endoscopic therapy as the first-line option, but a multidisciplinary approach is necessary, with second-line surgical intervention often required. We recommend a management algorithm similar to that presented in the UK PB literature, and confirm that bilioenteric anastomosis can be performed successfully without prior portal decompression.
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Affiliation(s)
| | - Michael Crawford
- Australian National Liver Transplantation Unit, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Department of Upper Gastrointestinal and Hepatobiliary Surgery, Royal Prince Alfred Hospital, Sydney, NSW, Australia
| | - Arthur John Kaffes
- AW Morrow Gastroenterology and Liver Centre, Royal Prince Alfred Hospital, Sydney, NSW, Australia
| | - Charbel Sandroussi
- Department of Upper Gastrointestinal and Hepatobiliary Surgery, Royal Prince Alfred Hospital, Sydney, NSW, Australia.,Surgical Outcomes Research Centre (SOuRCe), The University of Sydney, Sydney, NSW, Australia
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Farid WRR, de Jonge J, Zondervan PE, Demirkiran A, Metselaar HJ, Tilanus HW, de Bruin RWF, van der Laan LJW, Kazemier G. Relationship between the histological appearance of the portal vein and development of ischemic-type biliary lesions after liver transplantation. Liver Transpl 2013; 19:1088-98. [PMID: 23843296 DOI: 10.1002/lt.23701] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2012] [Accepted: 06/09/2013] [Indexed: 12/13/2022]
Abstract
Ischemic-type biliary lesions (ITBLs) are a major cause of morbidity after liver transplantation (LT). Their assumed underlying pathophysiological mechanism is ischemia/reperfusion injury of the biliary tree, in which the portal circulation has been proposed recently to have a role. The aim of this study was to investigate whether early histological changes, particularly in the portal vein, predispose patients to ITBLs. A case-control study of 22 LT recipients was performed through a retrospective assessment of more than 30 histological parameters in 44 intraoperative liver biopsy samples taken after cold ischemia (time 0) and portal reperfusion (time 1). Eleven grafts developed ITBLs requiring retransplantation (the ITBL group), and 11 matched controls had normally functioning grafts 11 years after LT on average (the non-ITBL group). Additionally, 11 liver biopsy samples from hemihepatectomies performed for metastases of colorectal cancer (CRC) were assessed similarly. Analyses showed no significant histological differences at time 0 between the ITBL and non-ITBL groups. However, the time 1 biopsy samples from the ITBL group showed smaller portal vein branches (PVBs) significantly more often than the samples from the non-ITBL group, which also showed persisting paraportal collateral vessels. Larger PVBs and paraportal collateral vessels were also found in the CRC group. A morphometric analysis confirmed these findings and showed that PVB measurements were significantly lower for the ITBL group at time 1 versus the ITBL group at time 0 and the non-ITBL and CRC groups (they were largest in the CRC group). Thus, the PVB dimensions decreased in the ITBL group in comparison with the time 0 biopsy samples, and they were significantly smaller at time 1 in comparison with the dimensions for the non-ITBL and CRC groups. In conclusion, a smaller PVB lumen size in postreperfusion biopsy samples from liver grafts, suggesting a relatively decreased portal blood flow, is associated with a higher incidence of ITBLs. These findings support recent clinical studies suggesting a possible pathophysiological role of portal blood flow in the oxygenation of the biliary tree after LT.
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Affiliation(s)
- Waqar R R Farid
- Departments of Surgery, Erasmus University Medical Center, Rotterdam, the Netherlands
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Suárez V, Puerta A, Santos LF, Pérez JM, Varón A, Botero RC. Portal hypertensive biliopathy: A single center experience and literature review. World J Hepatol 2013; 5:137-44. [PMID: 23556047 PMCID: PMC3612573 DOI: 10.4254/wjh.v5.i3.137] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2012] [Revised: 09/25/2012] [Accepted: 11/14/2012] [Indexed: 02/06/2023] Open
Abstract
Portal hypertensive biliopathy (PHB) is characterized by anatomical and functional abnormalities of the intrahepatic, extrahepatic and pancreatic ducts, in patients with portal hypertension associated to extrahepatic portal vein obstruction and less frequently to cirrhosis. These morphological changes, consisting in dilatation and stenosis of the biliary tree, are due to extensive venous collaterals occurring in an attempt to decompress the portal venous blockage. It is usually asymptomatic until it progresses to more advanced stages with cholestasis, jaundice, biliary sludge, gallstones, cholangitis and finally biliary cirrhosis. Imaging modalities of the biliary tree such as Doppler ultrasound, computed tomography, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are essential to establish the diagnosis and the need of therapeutical interventions. Once the diagnosis is established, treatment with ursodesoxycholic acid seems to be beneficial. Decompression of the biliary tree to dilate, remove stones or implant biliary prosthesis by endoscopic or surgical procedures (hepato-yeyunostomy) usually resolves the cholestatic picture and prevents septic complications. The ideal treatment is the decompression of the portal system, with transjugular intrahepatic porto-systemic shunt or a surgical porto-systemic shunt. Unfortunately, few patients will be candidates for these procedures due to the extension of the thrombotic process. The purpose of this paper is to report the first 3 cases of PHB seen in a Colombian center and to review the literature.
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Affiliation(s)
- Vanessa Suárez
- Vanessa Suárez, Luisa Fernanda Santos, Adriana Varón, Rafael Claudino Botero, Gastroenterology and Hepatology Service, Department of Internal Medicine, Fundación Cardioinfantil, Bogotá 110131, Colombia
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Abstract
Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.
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Aguirre DA, Farhadi FA, Rattansingh A, Jhaveri KS. Portal biliopathy: imaging manifestations on multidetector computed tomography and magnetic resonance imaging. Clin Imaging 2012; 36:126-34. [PMID: 22370133 DOI: 10.1016/j.clinimag.2011.07.001] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2011] [Accepted: 07/21/2011] [Indexed: 12/11/2022]
Abstract
Portal biliopathy refers to biliary abnormalities secondary to extrahepatic portal vein obstruction and cavernous transformation and is caused by vascular compression from peribiliary collateral vessels, producing segmental stenoses of the common bile duct and abnormal liver function test (LFT) results. A review of imaging studies yielded 18 patients with abnormal LFT results, biliary tract dilatation, and extrahepatic portal vein obstruction with cavernous transformation. Multidetector computed tomography and magnetic resonance imaging showed biliary stenotic segments in 11 patients secondary to extrinsic compression from enlarged peribiliary collaterals. Clinical and imaging follow-up demonstrated improvement in LFT results with minimal decrease in bile duct dilatation, eliminating percutaneous or endoscopic biliary intervention.
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Affiliation(s)
- Diego A Aguirre
- Department of Radiology, Fundacion Santa Fe de Bogota, University Hospital, Colombia
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Chattopadhyay S, Govindasamy M, Singla P, Varma V, Mehta N, Kumaran V, Nundy S. Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome? HPB (Oxford) 2012; 14:441-7. [PMID: 22672545 PMCID: PMC3384873 DOI: 10.1111/j.1477-2574.2012.00473.x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
OBJECTIVES After portosystemic anastomoses for biliopathy, some patients continue to suffer biliary obstruction. The effects of splenectomy and devascularization of the abdominal oesophagus and upper stomach are unclear. The aim of the current study was to determine the features of portal biliopathy (PB) in patients with non-cirrhotic portal hypertension, and to investigate outcomes in these patients after surgical procedures. METHODS A retrospective study of 56 patients who underwent surgery for PB during 1996-2010 was conducted. Data on presenting features, treatment received and outcomes were analysed. RESULTS In total, 41 of these patients had extrahepatic portal venous obstruction and 15 had non-cirrhotic portal fibrosis. Forty patients underwent shunt surgery and 16 underwent splenectomy and devascularization. Median bilirubin levels fell from 1.8 mg/dl (range: 0.4-5.9 mg/dl) to 1.0 mg/dl (range: 0.3-5.4 mg/dl) after shunt surgery and from 1.9 mg/dl (range: 0.6-4.0 mg/dl) to 1.2 mg/dl (range: 0.6-5.2 mg/dl) after splenectomy-devascularization. On follow-up, five of 33 patients had persistent jaundice after successful shunt surgery. These patients had a history of multiple endoscopic stentings and three patients had demonstrated a dominant common bile duct stricture preoperatively. CONCLUSIONS Portal biliopathy was reversed in 38 of 43 patients by either portosystemic shunting or splenectomy-devascularization. In five patients, direct biliary decompressive procedures were required because of shunt blockage or a non-reversible biliary stricture.
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Affiliation(s)
- Somnath Chattopadhyay
- Department of Surgical Gastroenterology and Liver Transplantation, Sir Ganga Ram Hospital, New Delhi, India.
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Justo LA, Olcina JRF, Tallón AG, Carbonell SP, Rivera JIG, Vicente VM. [Cholangiopathy associated with portal hypertension]. GASTROENTEROLOGIA Y HEPATOLOGIA 2011; 34:619-23. [PMID: 21862180 DOI: 10.1016/j.gastrohep.2011.05.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/06/2011] [Revised: 05/18/2011] [Accepted: 05/26/2011] [Indexed: 11/28/2022]
Abstract
Portal cholangiopathy encompasses a group of abnormalities of the biliary system and gallbladder that occur secondary to chronic portal vein thrombosis and collateral venous circulation. Chronic obstruction of the portal vein is a frequent cause of gastrointestinal variceal bleeding, but data on biliary tract abnormalities are limited. We report the case of a male patient with obstructive jaundice secondary to portal cholangiopathy. We describe the pathogenesis of this entity, and the various diagnostic and therapeutic options available.
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Affiliation(s)
- Linette Achécar Justo
- Servicio de Gastroenterología, Hospital Universitario Ramón y Cajal, Madrid, España.
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40
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Agarwal AK, Sharma D, Singh S, Agarwal S, Girish SP. Portal biliopathy: a study of 39 surgically treated patients. HPB (Oxford) 2011; 13:33-39. [PMID: 21159101 PMCID: PMC3019539 DOI: 10.1111/j.1477-2574.2010.00232.x] [Citation(s) in RCA: 44] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/23/2009] [Accepted: 03/03/2010] [Indexed: 02/06/2023]
Abstract
BACKGROUND Portal biliopathy (PBP) denotes intra- and extrahepatic biliary duct abnormalities that occur as a result of portal hypertension and is commonly seen in extrahepatic portal vein obstruction (EHPVO). The management of symptomatic PBP is still controversial. METHODS Prospectively collected data for surgically managed PBP patients from 1996 to 2007 were retrospectively analysed for presentation, clinical features, imaging and the results of surgery. All patients were assessed with a view to performing decompressive shunt surgery as a first-stage procedure and biliary drainage as a second stage-procedure if required, based on evaluation at 6 weeks after shunt surgery. RESULTS A total of 39 patients (27 males, mean age 29.56 years) with symptomatic PBP were managed surgically. Jaundice was the most common symptom. Two patients in whom shunt surgery was unsuitable underwent a biliary drainage procedure. A total of 37 patients required a proximal splenorenal shunt as first-stage surgery. Of these, only 13 patients required second-stage surgery. Biliary drainage procedures (hepaticojejunostomy [n= 11], choledochoduodenostomy [n= 1]) were performed in 12 patients with dominant strictures and choledocholithiasis. One patient had successful endoscopic clearance of common bile duct (CBD) stones after first-stage surgery and required only cholecystectomy as a second-stage procedure. The average perioperative blood product transfusion requirement in second-stage surgery was 0.9 units and postoperative complications were minimal with no mortality. Over a mean follow-up of 32.2 months, all patients were asymptomatic. Decompressive shunt surgery alone relieved biliary obstruction in 24 of 37 patients (64.9%) and facilitated a safe second-stage biliary decompressive procedure in the remaining 13 patients (35.1%). CONCLUSIONS Decompressive shunt surgery alone relieves biliary obstruction in the majority of patients with symptomatic PBP and facilitates endoscopic or surgical management in patients who require second-stage management of biliary obstruction.
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Affiliation(s)
- Anil Kumar Agarwal
- Department of Gastrointestinal Surgery, Govind Ballabh Pant Hospital and Maulana Azad Medical College, Delhi University, New Delhi, India.
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Takamatsu M, Furutake M, Hisa T, Ueda M. Obstructive jaundice caused by a portal cavernoma. Jpn J Radiol 2010; 28:754-8. [PMID: 21191741 DOI: 10.1007/s11604-010-0480-7] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2010] [Accepted: 06/24/2010] [Indexed: 12/12/2022]
Abstract
A 69-year-old Japanese man was admitted to our hospital because of acute cholangitis with biliary obstruction. The cause of obstruction was either compression by a portal cavernoma or cavernous transformation. Multidetector row computed tomography (MDCT) and abdominal ultrasonography (US) revealed a portal cavernoma around the common bile duct. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiography (ERC) demonstrated characteristic short, smooth narrowing of the bile duct. Endoscopic US and intraductal US demonstrated collateral vessels around the bile duct and were helpful for ruling out a neoplastic lesion. Thus, a combination of imaging modalities was useful for diagnosing this hepatobiliary complication, portal biliopathy.
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MESH Headings
- Aged
- Cholangiopancreatography, Magnetic Resonance/methods
- Cholangitis/complications
- Common Bile Duct/diagnostic imaging
- Common Bile Duct/pathology
- Contrast Media
- Diagnosis, Differential
- Hemangioma, Cavernous/complications
- Hemangioma, Cavernous/diagnosis
- Humans
- Hypertension, Portal/diagnosis
- Hypertension, Portal/etiology
- Jaundice, Obstructive/diagnosis
- Jaundice, Obstructive/etiology
- Liver Cirrhosis/complications
- Male
- Portal Vein/diagnostic imaging
- Portal Vein/pathology
- Radiographic Image Enhancement/methods
- Tomography, X-Ray Computed/methods
- Ultrasonography, Interventional/methods
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Affiliation(s)
- Masato Takamatsu
- Department of Internal Medicine, Saku Central Hospital, 197 Usuda, Saku, Nagano, 384-0301, Japan.
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Petillon S, Vibert E, Gorden DL, de la Serna S, Salloum C, Azoulay D. Hepatectomy and intrahepatic biliary enteric anastomosis: A rescue surgery for obstructed metallic biliary stents in chronic pancreatitis. ACTA ACUST UNITED AC 2010; 34:310-3. [DOI: 10.1016/j.gcb.2010.03.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/07/2010] [Revised: 03/04/2010] [Accepted: 03/05/2010] [Indexed: 11/29/2022]
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Chabchoub I, Maalej B, Turki H, Aloulou H, Aissa K, Ben Mansour L, Kamoun T, Hachicha M. [Cholelithiasis associated with portal cavernoma in children: 2 case reports]. Arch Pediatr 2010; 17:507-10. [PMID: 20303244 DOI: 10.1016/j.arcped.2010.01.023] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2009] [Revised: 04/23/2009] [Accepted: 01/24/2010] [Indexed: 11/16/2022]
Abstract
The association of cholelithiasis and portal cavernoma is rarely described in adult or pediatric patients. We report 2 cases of gallstone associated with portal cavernoma in 2 girls. The first one suffered from Evans syndrome associated with congenital immune deficiency. The portal cavernoma was discovered with gallstone after splenectomy indicated because of high steroid dependence. In the second case, the cavernoma complicated neonatal umbilical catheterism. The gallstone was asymptomatic and discovered on annual ultrasonography. Septicemia, profound thrombocytopenia, and acute anaemia led to rapid death in the first case. However, the progression was favourable under celioscopic treatment in the second one. Our original observations suggest systematically searching for gallstone in children with portal cavernoma.
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Affiliation(s)
- I Chabchoub
- Service de pédiatrie générale, hôpital Hédi Chaker, Sfax, Tunisie.
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Trombosis portal. GASTROENTEROLOGIA Y HEPATOLOGIA 2010; 33:179-90. [DOI: 10.1016/j.gastrohep.2009.04.002] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/07/2009] [Accepted: 04/14/2009] [Indexed: 12/31/2022]
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45
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Parikh S, Shah R, Kapoor P. Portal vein thrombosis. Am J Med 2010; 123:111-9. [PMID: 20103016 DOI: 10.1016/j.amjmed.2009.05.023] [Citation(s) in RCA: 125] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2009] [Revised: 04/23/2009] [Accepted: 05/18/2009] [Indexed: 02/07/2023]
Abstract
Portal vein thrombosis is a condition not infrequently encountered by clinicians. It results from a combination of local and systemic prothrombotic risk factors. The presentation of acute thrombosis varies widely from an asymptomatic state to presence of life-threatening intestinal ischemia and infarction. In the chronic stage, patients typically present with variceal bleeding or other complications of portal hypertension. Abdominal ultrasound color Doppler imaging has a 98% negative predictive value, and is considered the imaging modality of choice in diagnosing portal vein thrombosis. Controlled clinical trials to assist with clinical decision-making are lacking in both acute and chronic portal vein thrombosis. Oral anticoagulant therapy is initiated if the risks of bleeding are low, but long-term anticoagulation is generally not recommended in patients with concomitant hepatic cirrhosis. The roles of invasive therapeutic approaches such as thrombolysis and transjugular intrahepatic portosystemic shunt continue to evolve. This review conflates dissenting views into a rational approach of managing patients with portal vein thrombosis for the general internist.
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Affiliation(s)
- Sameer Parikh
- Department of Medicine, Baylor College of Medicine, Houston, TX, USA
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Abstract
BACKGROUND Portal vein thrombosis (PVT) is an important cause of portal hypertension. It may occur as such with or without associated cirrhosis and hepatocellular carcinoma. Information on its management is scanty. AIM To provide an update on the modern management of portal vein thrombosis. Information on portal vein thrombosis in patients with and without cirrhosis and hepatocellular carcinoma is also updated. METHODS A pubmed search was performed to identify the literature using search items portal vein thrombosis-aetiology and treatment and portal vein thrombosis in cirrhosis and hepatocellular carcinoma. RESULTS Portal vein thrombosis occurs because of local inflammatory conditions in the abdomen and prothrombotic factors. Acute portal vein thrombosis is usually symptomatic when associated with cirrhosis and/or superior mesenteric vein thrombosis. Anticoagulation should be given for 3-6 months if detected early. If prothrombotic factors are identified, anticoagulation should be given lifelong. Chronic portal vein thrombosis usually presents with well tolerated upper gastrointestinal bleed. It is diagnosed by imaging, which demonstrates a portal cavernoma in place of a portal vein. Anticoagulation does not have a definite role, but bleeds can be treated with endotherapy or shunt surgery. Rarely liver transplantation may be considered. CONCLUSION Role of anticoagulation in chronic portal vein thrombosis needs to be further studied.
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Affiliation(s)
- Y Chawla
- Department of Hepatology, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
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Zhang M, Guo C, Pu C, Ren Z, Li Y, Kang Q, Jin X, Yan L. Adult to pediatric living donor liver transplantation for portal cavernoma. Hepatol Res 2009; 39:888-97. [PMID: 19467022 DOI: 10.1111/j.1872-034x.2009.00526.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
AIM Portal cavernoma (PC) is an important cause of non-cirrhotic portal hypertension with severe complications, such as variceal hemorrhage in pediatric patients. With the development of new surgical techniques, living donor liver transplantation (LDLT) has recently been recognized as a viable but challenging treatment option for PC. The purpose of the present study was to summarize the efficacy of LDLT in PC patients and to carry out a follow-up study of pediatric recipients. METHODS The primary indication for LDLT in our research was PC with severe variceal bleeding and liver function decompensation. Three patients were diagnosed with PC following evaluation with computed tomography angiography and abdominal color Doppler ultrasonography (CDU). RESULTS Various surgical techniques, including jump bypass grafting for portal vein anastomosis, were carried out according to the range and degree of cavernous transformation within the splenic vein and superior mesenteric vein. Postoperative CDU confirmed the early integrity of the portal vein (PV) in each patient. PV rethrombosis occurred in one patient 7 days after LDLT, despite anticoagulation therapy with coumadin. Two of the three patients had no further episodes of variceal hemorrhage during the 2-year follow-up period. CONCLUSIONS The present study is the first report of the successful use of LDLT to treat pediatric PC patients. We conclude that LDLT is effective for the majority of pediatric patients with PC.
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Affiliation(s)
- Mingman Zhang
- Department of Hepatobiliary Surgery, Children's Hospital, Chongqing Medical University, Chongqing, China
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Bile duct stricture due to caused by portal biliopathy: Treatment with one-stage portal-systemic shunt and biliary bypass. Indian J Gastroenterol 2009; 28:35-7. [PMID: 19529903 DOI: 10.1007/s12664-009-0010-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/04/2008] [Revised: 07/07/2008] [Accepted: 08/26/2008] [Indexed: 02/06/2023]
Abstract
Portal biliopathy is a rare complication of extrahepatic portal vein obstruction. Jaundice occurs in symptomatic patients with fibrotic strictures. Short-term improvement in such patients can be achieved with endoscopic retrograde cholangio-pancreatography with balloon dilatation and stent placement. Surgery in these patients is traditionally two staged. We report the results of a one-stage procedure combining non-selective portal-systemic shunt surgery with biliary bypass, performed successfully on a 24-year-old man with a tight biliary stricture resulting from portal biliopathy. At 18-month follow up, the patient shows he is doing well, with normal liver function tests.
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Right porto-ovarian H-shunt for the surgical treatment of symptomatic portal biliopathy: a case report and literature review. HPB SURGERY : A WORLD JOURNAL OF HEPATIC, PANCREATIC AND BILIARY SURGERY 2009; 2009:152195. [PMID: 19584934 PMCID: PMC2703746 DOI: 10.1155/2009/152195] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/05/2009] [Accepted: 05/08/2009] [Indexed: 01/10/2023]
Abstract
Portal hypertension, especially when it is caused by extrahepatic portal vein thrombosis, is commonly followed by the development of an abnormal periportal and pericholedochal variceal network, which form a portal cavernoma. This may exert extrinsic pressure on the adjacent biliary ducts and gallblader, causing morphologic abnormalities, termed portal biliopathy, which is usually leading to asymptomatic cholestasis, while less frequently it can be associated with obstructive jaundice, gallstone formation, and cholangitis. Endoscopic stone extraction can effectively treat portal biliopathy when cholangitis is associated with common bile duct stones. Portosystemic shunts are indicated in cases of disease recurrence as they can achieve regression of portal cavernoma and usually relieve symptomatic portal biliopathy. This case describes an alternative partial portosystemic shunt that utilizes the right ovarian vein as an autologous conduit for the surgical treatment of symptomatic portal biliopathy.
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El-Matary W, Roberts EA, Kim P, Temple M, Cutz E, Ling SC. Portal hypertensive biliopathy: a rare cause of childhood cholestasis. Eur J Pediatr 2008; 167:1339-42. [PMID: 18270735 DOI: 10.1007/s00431-008-0675-4] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/26/2007] [Accepted: 01/17/2008] [Indexed: 12/24/2022]
Abstract
Portal hypertensive biliopathy (PHB) is defined as abnormal biliary changes that take place most likely secondary to extrahepatic portal vein obstruction (EHPVO) with portal hypertension. This condition may be asymptomatic or could lead to a cholestatic state, which is not well-described in children. We report a child who developed a cholestatic nature with portal hypertension some time after having neonatal surgery for duodenal atresia. We discuss the differential diagnosis and management of this rare condition. Symptomatic PHB has been only rarely reported in children. It should be suspected in patients with portal hypertension and having features of biliary obstruction. Hepaticojejunostomy may have a therapeutic role in selected patients in whom endoscopic or percutaneous manipulation of the biliary tree is unsuccessful and who have not responded to a surgical portal-systemic shunt procedure.
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Affiliation(s)
- Wael El-Matary
- Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada.
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