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Patel S, Thompson D, Sharshar M, Crawford JM, Dagher N, Fahmy AE. Liver Transplantation as a Treatment for Unresectable Hepatic Adenoma in a Patient With Abernethy Syndrome. Cureus 2024; 16:e60683. [PMID: 38903310 PMCID: PMC11187450 DOI: 10.7759/cureus.60683] [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] [Accepted: 05/20/2024] [Indexed: 06/22/2024] Open
Abstract
Abernethy syndrome is a rare congenital anomaly characterized by an intrahepatic or extrahepatic portosystemic shunt. Most patients are asymptomatic; however, due to the alteration in, or lack of, a portovenous flow, patients with Abernethy syndrome are at high risk of developing sequelae of liver failure. Once these complications develop, the only definitive treatment is transplantation. Patients with Abernethy syndrome are also at a higher risk of developing benign and malignant liver lesions, including hepatic adenomas. Here, we describe the first case of deceased donor liver transplantation as a treatment for a patient with type 1 Abernethy syndrome complicated by large, unresectable hepatic adenoma, found to have focal hepatocellular carcinoma on pathologic examination. Our male patient was found to have elevated liver enzymes at age 33, during a routine outpatient medical appointment. Despite being asymptomatic, his history of prior liver resection prompted CT imaging, which revealed two large liver lesions concerning for hepatic adenomas. When surveillance imaging showed a significant growth of the liver lesions, biopsy was pursued, which confirmed a diagnosis of hepatic adenomas. However, given the size of these lesions, resection was not a viable option for the patient. Instead, the patient underwent liver transplantation at age 41, which he tolerated well. Our case demonstrates the utility of deceased donor liver transplantation as a treatment for patients with Abernethy syndrome complicated by unresectable adenomas.
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Affiliation(s)
- Shreeja Patel
- Division of Transplant Surgery, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA
| | - Dane Thompson
- Department of Surgery, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA
| | - Mohamed Sharshar
- Division of Transplant Surgery, Department of Surgery, Northwell Health, New York, USA
| | - James M Crawford
- Department of Pathology and Laboratory Medicine, Northwell Health, New York, USA
- Department of Pathology and Laboratory Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA
| | - Nabil Dagher
- Division of Transplant Surgery, Department of Surgery, Northwell Health, New York, USA
| | - Ahmed E Fahmy
- Division of Transplant Surgery, Department of Surgery, Northwell Health, New York, USA
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Malik A, Patel ME, Ganger D, Hohlastos E, Riaz A. Percutaneous sequential closure of an Abernethy malformation: A case report. Radiol Case Rep 2023; 18:3135-3139. [PMID: 37388532 PMCID: PMC10302158 DOI: 10.1016/j.radcr.2023.06.019] [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: 04/27/2023] [Revised: 06/05/2023] [Accepted: 06/08/2023] [Indexed: 07/01/2023] Open
Abstract
Abernethy malformation (congenital extrahepatic portosystemic shunt [CEPS]) is rare and is characterized by an aberrant connection between the portal and systemic veins, bypassing the liver. It can have varying presentations and can lead to severe complications if left untreated. It is usually diagnosed incidentally on abdominal imaging. Occlusion venography and measurement of portal pressures (pre- and postocclusion) is an important step in management. Complete occlusion of the malformation in cases where the portal veins in the liver are very small and the gradient is more than 10 mm Hg, can potentially lead to acute portal hypertensive complications, such as porto-mesenteric thrombosis. We report a case of Abernethy malformation diagnosed on an abdominal computed tomography scan that presented with neurological symptoms and was successfully managed by interventional radiology via endovascular closure through placement and sequential occlusion of 2 metal stents.
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Affiliation(s)
- Asad Malik
- Division of Interventional Radiology, Department of Radiology, Northwestern University, 676 N. St. Clair, Suite 800, Chicago, IL, USA
| | - Muhammed Ebrahim Patel
- Division of Interventional Radiology, Department of Radiology, Northwestern University, 676 N. St. Clair, Suite 800, Chicago, IL, USA
| | - Daniel Ganger
- Department of Medicine, Section of Gastroenterology/Hepatology, Northwestern University, Chicago, IL, USA
| | - Elias Hohlastos
- Division of Interventional Radiology, Department of Radiology, Northwestern University, 676 N. St. Clair, Suite 800, Chicago, IL, USA
| | - Ahsun Riaz
- Division of Interventional Radiology, Department of Radiology, Northwestern University, 676 N. St. Clair, Suite 800, Chicago, IL, USA
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Nagy RD, Cernea N, Dijmarescu AL, Manolea MM, Zorilă GL, Drăgușin RC, Vrabie SC, Dîră LM, Sîrbu OC, Novac MB, Drăgoescu NAM, Gheonea M, Stoica GA, Căpitănescu RG, Iliescu DG. Ductus Venosus Agenesis and Portal System Anomalies-Association and Outcome. BIOLOGY 2022; 11:548. [PMID: 35453747 PMCID: PMC9031854 DOI: 10.3390/biology11040548] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 03/06/2022] [Revised: 03/28/2022] [Accepted: 03/30/2022] [Indexed: 06/14/2023]
Abstract
To evaluate the prenatal diagnosis of agenesis of ductus venosus (ADV) and portal venous system (PVS) anomalies and describe the outcome of these cases, either isolated or associated. We evaluated the intrahepatic vascular system regarding the presence of normal umbilical drainage and PVS characteristics in the second and third trimester of pregnancy. The associated anomalies and umbilical venous drainage were noted. Follow-up was performed at six months follow-up. Ultrasonography was performed in 3517 cases. A total of 19 cases were prenatally diagnosed: 18 ADV cases, seven abnormal PVS cases, and six associations of the two anomalies. We noted an incidence of 5.1‱ and 1.9‱ for ADV and PVS anomalies, respectively. Out of the 18 ADV cases, 27.7% were isolated. Five cases (26.3%) presented genetic anomalies. PVS anomalies were found in 33.3% of the ADV cases. ADV was present in 85.7% of the PVS anomalies. DV and PVS abnormalities were found with a higher than reported frequency. Normal DV is involved in the normal development of the PVS. Additional fetal anomalies are the best predictor for the outcome of ADV cases. Evaluation of PVS represents a powerful predictor for ADV cases and addresses the long-term prognosis.
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Affiliation(s)
- Rodica Daniela Nagy
- Doctoral School, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (R.D.N.); (L.M.D.)
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Nicolae Cernea
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
| | - Anda Lorena Dijmarescu
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Department of Obstetrics and Gynecology, Clinical Municipal Hospital “Filantropia” of Craiova, 200143 Craiova, Romania
| | - Maria-Magdalena Manolea
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Department of Obstetrics and Gynecology, Clinical Municipal Hospital “Filantropia” of Craiova, 200143 Craiova, Romania
| | - George-Lucian Zorilă
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Roxana Cristina Drăgușin
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Sidonia Cătălina Vrabie
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
- Department of Obstetrics and Gynecology, Clinical Municipal Hospital “Filantropia” of Craiova, 200143 Craiova, Romania
| | - Laurențiu Mihai Dîră
- Doctoral School, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (R.D.N.); (L.M.D.)
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Ovidiu Costinel Sîrbu
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Marius Bogdan Novac
- Department of Anesthesia and Intensive Care, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (M.B.N.); (N.A.M.D.)
- Department of Anesthesia and Intensive Care, Clinical Municipal Hospital “Filantropia” of Craiova, 200143 Craiova, Romania
| | - Nicoleta Alice Marinela Drăgoescu
- Department of Anesthesia and Intensive Care, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (M.B.N.); (N.A.M.D.)
- Department of Anesthesia and Intensive Care, University Emergency County Hospital, 200642 Craiova, Romania
| | - Mihaela Gheonea
- Department of Neonatology, University Emergency County Hospital, 200642 Craiova, Romania;
- Department of Neonatology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
| | - George Alin Stoica
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
- Department of Pediatric Orthopedic Surgery, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania
- Department of Pediatric Orthopedic Surgery, University Emergency County Hospital, 200642 Craiova, Romania
| | - Răzvan Grigoraș Căpitănescu
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
| | - Dominic-Gabriel Iliescu
- Department of Obstetrics and Gynecology, University Emergency County Hospital, 200642 Craiova, Romania; (N.C.); (R.C.D.); (O.C.S.); (R.G.C.); (D.-G.I.)
- Department of Obstetrics and Gynecology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania; (A.L.D.); (M.-M.M.); (S.C.V.)
- Ginecho Clinic, Medgin SRL, 200333 Craiova, Romania;
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Prenatal Diagnosis and Outcome of Umbilical–Portal–Systemic Venous Shunts: Experience of a Tertiary Center and Proposal for a New Complex Type. Diagnostics (Basel) 2022; 12:diagnostics12040873. [PMID: 35453921 PMCID: PMC9027129 DOI: 10.3390/diagnostics12040873] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2022] [Revised: 03/28/2022] [Accepted: 03/29/2022] [Indexed: 02/04/2023] Open
Abstract
Aims: To share our experience in the prenatal diagnosis of umbilical–portal–systemic venous shunts (UPSVS) and to study the prognostic factors for proper prenatal and perinatal management. Material and Methods: A five-year prospective study regarding the detection of UPSVS was conducted in two referral centers, Medgin Ginecho Clinic and the Prenatal Diagnostic Unit of the tertiary center, University Emergency County Hospital Craiova, Romania. We included in the analysis a series of agenesis of ductus venosus (ADV) cases previously reported by our center. We analyzed the incidence of the UPSVS types, their associations, and outcome predictors. Results: UPSVS were diagnosed in all 16 cases that were presented to our center at the time of first trimester anomaly scan, except one (94.12%). We diagnosed: 19 type I (61.2%), 4 type II (12.9%) and 5 type IIIa (16.1%) UPSVS. In three cases (9.6%) we noted multiple shunts, which we referred to as type IV (a new UPSVS type). Type IIIa-associated fetal growth restriction (FGR) was found in 60% of cases. Major anomalies worsened the outcome. Of the UPVSS cases, 57.1% were associated with PVS anomalies. Genetic anomalies were present in 40% of the tested cases. Conclusions: The incidence of UPSVS in our study was 0.2%. Early detection is feasible. The postnatal outcome mainly depends on the presence of structural, genetic and PVS anomalies. FGR may be associated. The new category presented a poor outcome secondary to poor hemodynamic and major associated anomalies.
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Feasibility of Fetal Portal Venous System Ultrasound Assessment at the FT Anomaly Scan. Diagnostics (Basel) 2022; 12:diagnostics12020361. [PMID: 35204452 PMCID: PMC8871164 DOI: 10.3390/diagnostics12020361] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2021] [Revised: 01/13/2022] [Accepted: 01/29/2022] [Indexed: 02/04/2023] Open
Abstract
Objective: To investigate the feasibility of the first trimester (FT) ultrasound scan (US) for the evaluation of the fetal portal venous system (PVS) anatomy, and to evaluate the potential of microcopy for a proper pathology evaluation for the PVS in the FT. Methods: We evaluated the PVS in 200 scan examinations performed in FT pregnancy. Half of the cases were scanned by two operators with extensive experience in obstetric ultrasound—Group I, and the other half was evaluated by two sonographers with less experience—Group II. Second-trimester US and autopsy in terminated pregnancies were used as follow-up. The pathologic evaluation was supported by microscopy. Results: all PVS features were successfully assessed by transabdominal ultrasound (TAUS) in 27% of the Group I cases and 14% in Group II. These rates increased to 88% in Group I and in 72% in Group II, after rescanning and using transvaginal ultrasound (TVUS). The conditions that led to rescanning and TVUS were: BMI greater than 24 in 26% cases, unfavorable fetal position (12.32%), retroverted uterus (12.32%), abdominal scar (10.96%), fibroids (4.11%), and combinations of the above (34.23%). The L-shaped UV confluence was identified transabdominally in 91% in Group I and in 79% in Group II and increased to 98% and 95%, respectively, following reevaluations. Microscopy represented a useful audit in all FT investigated cases. Conclusions: At the end of the FT, the visualization of a normal L-shaped UV confluence, that excludes major PVS abnormalities, is achievable in approx. 80%, indifferently the examiners experience. The sonographers experience, pregnant women BMI, and uterine anomalies as fibroids or retroversion significantly affect the rate of visualization, and necessitates vaginal approach and reexamination. The FT pathology, the audit of the ultrasound findings can only be performed microscopically, with relatively little resources involved and good results.
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Namgoong JM, Hwang S, Park GC, Kwon H, Kim KM, Oh SH. Living donor liver transplantation in a pediatric patient with congenital absence of the portal vein. Ann Hepatobiliary Pancreat Surg 2021; 25:401-407. [PMID: 34402443 PMCID: PMC8382859 DOI: 10.14701/ahbps.2021.25.3.401] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2021] [Revised: 02/11/2021] [Accepted: 02/14/2021] [Indexed: 11/25/2022] Open
Abstract
Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. We report a case of pediatric living donor liver transplantation (LDLT) for CAPV combined with focal nodular hyperplasia (FNH) and hepatocellular adenoma. A 9-year-old girl who had been diagnosed with multiple FNH had CAPV. Her blood ammonia level was raised to 137 μg/dL. However, she did not complain of any symptoms. To treat CAPV and FNH, we decided to perform LDLT. The graft was a left liver graft from 39-year-old mother of the patient. Recipient hepatectomy was performed according to standard procedures of pediatric LDLT. Portal vein reconstruction was performed using interposition of an iliac vein homograft conduit to the superior mesenteric vein-splenic vein confluence. The CAPV-associated congenital splenorenal shunt was securely ligated. The pathology report of the explant liver showed a 2 cm-sized hepatocellular adenoma and multiple FNH lesions measuring up to 7.1 cm. The patient recovered uneventfully from the LDLT operation. The reconstructed portal vein was maintained well without any hemodynamic abnormalities. In conclusion, as CAPV patients can have various vascular anomalies, combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.
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Affiliation(s)
- Jung-Man Namgoong
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gil-Chun Park
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Hyunhee Kwon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Kyung Mo Kim
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seak Hee Oh
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Laverdure N, Lallier M, Dubois J, Paganelli M. Congenital absence of the portal vein: Define the portosystemic shunt, avoid liver transplantation. CANADIAN LIVER JOURNAL 2021; 4:322-327. [DOI: 10.3138/canlivj-2020-0011] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/19/2020] [Accepted: 06/02/2020] [Indexed: 11/20/2022]
Abstract
Liver transplantation has been historically recommended for patients with congenital absence of the portal vein associated with extrahepatic congenital portosystemic shunts. Here, based on a case report of a 2-year-old girl and a thorough review of all published cases from 1974 to 2020, we show that such a diagnosis most often conceals a hypoplastic portal vein, which can be successfully re-permeabilized through the closure of the shunt in order to re-establish a physiological vascular anatomy. This highlights the importance of achieving a detailed anatomical description of extrahepatic congenital portosystemic shunts with a balloon occlusion test in order to plan the best surgical approach and avoid unnecessary liver transplantation.
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Affiliation(s)
- Noémie Laverdure
- Pediatric Gastroenterology Hepatology and Nutrition, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
- Service d’Hépato-gastroenterologie et nutrition pediatrique, Hopital Femme Mere Enfant, Lyon, France
| | - Michel Lallier
- Pediatric Surgery, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
| | - Josée Dubois
- Pediatric Radiology, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
| | - Massimiliano Paganelli
- Pediatric Gastroenterology Hepatology and Nutrition, CHU Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
- Liver Tissue Engineering and Cell Therapy Laboratory, CHU Sainte-Justine Research Centre, Montréal, Québec, Canada
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Namgoong JM, Hwang S, Kim DY, Ha TY, Song GW, Jung DH, Kim KM, Oh SH. Pediatric liver transplantation using a hepatitis B surface antigen-positive donor liver graft for congenital absence of the portal vein. KOREAN JOURNAL OF TRANSPLANTATION 2021; 35:59-65. [PMID: 35769617 PMCID: PMC9235344 DOI: 10.4285/kjt.20.0038] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/04/2020] [Revised: 10/01/2020] [Accepted: 10/02/2020] [Indexed: 11/13/2022] Open
Abstract
Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into systemic circulation. Herein, we report a case of pediatric deceased donor liver transplantation (DDLT) for symptomatic CAPV with whole liver graft from a hepatitis B surface antigen (HBsAg)-positive donor. A 4-year-old boy suffered from CAPV and secondary portal hypertension. He was also diagnosed with DiGeorge syndrome and heart anomalies. After waiting for 4 months, a 5-year-old donor weighing 19 kg with positive HBsAg was allocated to this 4-year-old patient weighing 15 kg. Recipient operation was performed according to the standard procedures of pediatric DDLT. Portal vein reconstruction was performed using interposition of a vascular homograft conduit to the superior mesenteric vein-splenic vein confluence. The patient recovered uneventfully from DDLT. He has been administered with lamivudine to prevent hepatitis B virus infection. This patient has been doing well for 5 years after DDLT without growth retardation. In conclusion, CAPV patients can have various vascular anomalies, thus combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.
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Affiliation(s)
- Jung-Man Namgoong
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Dae-Yeon Kim
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Tae-Yong Ha
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gi-Won Song
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong-Hwan Jung
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Kyung Mo Kim
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seak Hee Oh
- Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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9
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Sato T, Ishii T, Kato M, Oyanagi T, Nakatsuka S, Kuroda T, Hasegawa T. Oral sodium phenylbutyrate for hyperammonemia associated with congenital portosystemic shunt: a case report. J Pediatr Endocrinol Metab 2021; 34:407-410. [PMID: 33675205 DOI: 10.1515/jpem-2020-0603] [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: 10/17/2020] [Accepted: 11/22/2020] [Indexed: 11/15/2022]
Abstract
OBJECTIVES The efficacy of sodium phenylbutyrate (SPB) for hyperammonemia associated with congenital portosystemic shunt (CPSS) remains unknown. We show the effectiveness of oral SPB. CASE PRESENTATION Our patient had CPSS with severe hypoplasia of extrahepatic portal veins. At 9 months of age, to assess the efficacy of oral SPB, we evaluated the 24 h fluctuations of venous ammonia levels. In the first two days without SPB, ammonia levels were above 80 μmol/L for half a day. On the third and fourth days, administration of oral SPB three times a day decreased ammonia to acceptable levels, except at midnight. On the fifth day, another oral SPB administration at 8 pm decreased ammonia at midnight. Low levels of branched-chain amino acids, as well as coagulation disturbances, were observed without apparent symptoms. At 12 months of age, he showed normal psychomotor development. CONCLUSIONS Oral SPB may be effective for hyperammonemia associated with CPSS.
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Affiliation(s)
- Takeshi Sato
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Tomohiro Ishii
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Mototoshi Kato
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Takayuki Oyanagi
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
| | - Seishi Nakatsuka
- Department of Diagnostic Radiology, Keio University School of Medicine, Tokyo, Japan
| | - Tatsuo Kuroda
- Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan
| | - Tomonobu Hasegawa
- Department of Pediatrics, Keio University School of Medicine, Tokyo, Japan
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Wu J, Lu Y, Zhao W, Shen J, Li F, Zhang H, Chen Q, Fu L. Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts. Eur J Pediatr 2021; 180:929-936. [PMID: 33011830 DOI: 10.1007/s00431-020-03817-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/25/2020] [Revised: 08/13/2020] [Accepted: 09/18/2020] [Indexed: 12/01/2022]
Abstract
The aim of this retrospective study was to investigate the clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension (PAH) secondary to congenital portosystemic shunts (CPSS). Thirty-three pediatric patients diagnosed in our institution with CPSS between 2012 and 2019 were enrolled in this study. The patients were divided into PAH and non-PAH groups. The PAH group included 15 patients who presented with unexplained PAH when CPSS was diagnosed. Two patients with microangiopathic hemolytic anemia died of right heart failure shortly after diagnosis. One patient received a liver transplant at the age of 4.3 years and showed a mild decrease in pulmonary artery pressure (PAP) 4 years after the operation. Seven patients underwent one-stage shunt closure at a median age of 2.8 years (1.4-13 years). Follow-up examinations, from 1.6 to 4.1 years after intervention, showed marked reduction of PAP in one patient and stabilization of PAH in six others. However, in one patient who underwent two-stage shunt closure, a marked increase in PAP was noted after partial ligation of the shunt. The remaining four patients received only pulmonary vasodilator therapy, and one of them died of right heart failure 12 years after the PAH diagnosis. The non-PAH group included 18 patients without evidence of PAH upon CPSS diagnosis. Shunt closure was carried out in eight of these patients, but one patient subsequently developed PAH after the resolution of hepatopulmonary syndrome.Conclusion: CPSS may be a more likely cause of unexplained PAH in pediatric patients than previously thought. Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.
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Affiliation(s)
- Jinjin Wu
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Yi Lu
- Department of Internal Medicine, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Wenzhuo Zhao
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Jie Shen
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Fen Li
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Hao Zhang
- Department of cardiothoracic surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China
| | - Qimin Chen
- Department of Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China.
| | - Lijun Fu
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, China.
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11
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He X, Zhu Y, Fu H, Feng C, Liu Z, Gu W, Jin Y, Yang B, Shen H. Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type II. Front Pediatr 2021; 9:647364. [PMID: 33816407 PMCID: PMC8010253 DOI: 10.3389/fped.2021.647364] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2020] [Accepted: 02/22/2021] [Indexed: 11/29/2022] Open
Abstract
This report describes an 8-year-old male who presented with clinical manifestations including systemic edema, heavy proteinuria, hypoproteinemia, and persistent hypocomplementemia. Arachnoid cysts and focal nodular hyperplasia were also detected. Imaging examination and renal biopsy were performed, and Abernethy malformation type II with immune complex-mediated membranoproliferative glomerulonephritis was considered the diagnosis. Due to the persistence of embryonic vessels, Abernethy malformation is a rare congenital vascular malformation of the splanchnic venous system, which can be classified as type I (end-to-side shunt) and type II (side-to-side shunt). Abernethy malformation with glomerulonephritis remains extremely rare. In the patient described, glomerulonephritis mediated by immune complex with "full-house" positive immunohistochemistry was confirmed on renal biopsy. In addition, he was treated with glucocorticoids and tacrolimus. Whether surgical treatment is necessary should be determined according to the state of the disease in the later stages. The present case reflects the association between the congenital portosystemic shunt and the renal region and, to the authors' knowledge, may be the first report to describe arachnoid cysts as a symptom of Abernethy malformation.
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Affiliation(s)
- Xue He
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Yueling Zhu
- Department of Traditional Chinese Medicine, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Haidong Fu
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Chunyue Feng
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Zhixia Liu
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Weizhong Gu
- Department of Pathology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Yanyan Jin
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Binbin Yang
- Department of General Surgery, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Huijun Shen
- Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China
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12
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Bobhate P, Garg S, Sharma A, Roy D, Raut A, Pawar R, Karande T, Kulkarni S. Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension. Indian Heart J 2020; 73:99-103. [PMID: 33714417 PMCID: PMC7961257 DOI: 10.1016/j.ihj.2020.12.015] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2020] [Revised: 11/24/2020] [Accepted: 12/23/2020] [Indexed: 10/25/2022] Open
Abstract
OBJECTIVE Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. METHODS This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. RESULTS 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. CONCLUSION Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.
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Affiliation(s)
- Prashant Bobhate
- Children's' Heart Center, Second Floor, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India.
| | - Sandeep Garg
- Department of Pediatrics, Bhaktivednata Hospital and Research Institute, Bhaktivendanta Swami Marg, Srishti Sector 1, Mira Road, Thane, 401107, India
| | - Anuj Sharma
- Children's' Heart Center, Second Floor, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India
| | - Diptiman Roy
- Department of Radiodiagnosis and Interventional Radiology, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India
| | - Abhijeet Raut
- Department of Radiodiagnosis and Interventional Radiology, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India
| | - Ravindra Pawar
- Consultant Pediatric Cardiologist, 15, Sawali Bunglow, Shivaji Park, Kolhapur, 416005, India
| | - Tanuja Karande
- Children's' Heart Center, Second Floor, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India
| | - Snehal Kulkarni
- Children's' Heart Center, Second Floor, Kokilaben Dhirubai Ambani Hospital and Research Center, Rao Achyut Rao Patwardhan Marg, Four Bunglows, Andheri West, Mumbai, Maharashtra, 400053, India
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13
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Schmalz MJ, Radhakrishnan K. Vascular anomalies associated with hepatic shunting. World J Gastroenterol 2020; 26:6582-6598. [PMID: 33268948 PMCID: PMC7673960 DOI: 10.3748/wjg.v26.i42.6582] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/12/2020] [Revised: 10/14/2020] [Accepted: 10/26/2020] [Indexed: 02/06/2023] Open
Abstract
Congenital vascular anomalies affecting the liver have been described in the scientific literature for decades. Understanding these malformations begins with knowledge of hepatic vascular embryology. Surgeons have applied numerous classification systems to describe both intrahepatic and extrahepatic shunts, which can confuse the reader and clinician. In our experience, focusing on one classification system for extrahepatic shunts and one for intrahepatic shunts is better. Today many patients with these shunts carry good long-term prognosis thanks to advances in imaging to better detect shunts earlier and classify them. Timely intervention by skilled radiologists and surgeons have also limited complications arising from dynamic shunts and can avoid a liver transplant. Congenital hepatic shunts are not the only vascular condition affecting the liver. Hereditary hemorrhagic telangiectasia, also known as Osler Weber Rendu syndrome, particularly type 2, may have varying severity of hepatic involvement which warrants longitudinal care from an experienced hepatologist. Lastly, congenital hemangiomas, often first identified on the skin and oral mucosa, also can affect the liver. While most will resolve in infancy and childhood, the pediatric hepatologist must understand how and when to treat persistent lesions and their complications. This article serves as a concise reference to help clinicians better care for patients with these rare conditions.
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Affiliation(s)
- Michael J Schmalz
- Department of Pediatric Gastroenterology, Cleveland Clinic, Cleveland, OH 44106, United States
| | - Kadakkal Radhakrishnan
- Department of Pediatric Gastroenterology, Children's Hospital, Cleveland Clinic, Cleveland, OH 44106, United States
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14
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Guérin F, Franchi Abella S, McLin V, Ackermann O, Girard M, Cervoni JP, Savale L, Hernandez-Gea V, Valla D, Hillaire S, Dutheil D, Bureau C, Gonzales E, Plessier A. Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol 2020; 44:452-459. [PMID: 32279979 DOI: 10.1016/j.clinre.2020.03.004] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Accepted: 03/03/2020] [Indexed: 02/07/2023]
Affiliation(s)
- Florent Guérin
- Department of Visceral Pediatric surgery, FHU Hépatinov, Bicêtre Hospital, AP-HP, Paris-Saclay, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; UMRS-1193, Inserm, Paris-Saclay University, 91400 Orsay, France.
| | - Stéphanie Franchi Abella
- Department of Pediatric Radiology, FHU Hépatinov, Bicêtre Hospital, AP-HP, Paris-Saclay, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France
| | - Valérie McLin
- Swiss Pediatric Liver Center, Pediatric Gastroenterology, Hepatology, and Nutrition Unit, Department of Pediatrics, Gynecology and Obstetrics, University of Geneva, Geneva, Switzerland
| | - Oanez Ackermann
- UMRS-1193, Inserm, Paris-Saclay University, 91400 Orsay, France; Department of Pediatric Hepatology, FHU Hépatinov, Bicêtre Hospital, AP-HP, Paris-Saclay, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France
| | - Muriel Girard
- French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France; Department of Pediatric Hepatology, Hôpital Necker - enfants maladies, AP-HP, 149, rue de Sèvres, 75743 Paris cedex 15, France
| | - Jean Paul Cervoni
- Department of Hepatology, Jean Minjoz Hospital, University Hospital of Besançon, 3, boulevard Alexandre-Fleming, 25030 Besançon cedex, France
| | - Laurent Savale
- Department of Pulmonary Medicine, Bicêtre Hospital, AP-HP, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France
| | - Virginia Hernandez-Gea
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Institut de Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona. Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas (CIBERehd). Health Care Provider of the European Reference Network onRare Liver Disorders (ERN-Liver), Spain
| | - Dominique Valla
- French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France; Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Inserm, center for research in inflammation, University of Paris, 75018 Paris, France
| | - Sophie Hillaire
- Department of Internal medicine, Foch Hospital, 40, rue Worth, 92150 Suresnes, France
| | - Danielle Dutheil
- Department of Hepatology, Association of patients with vascular liver diseases (AMVF), Beaujon Hospital, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Christophe Bureau
- Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur-Jean-Poulhès, 31400 Toulouse, France
| | - Emmanuel Gonzales
- UMRS-1193, Inserm, Paris-Saclay University, 91400 Orsay, France; Department of Pediatric Hepatology, FHU Hépatinov, Bicêtre Hospital, AP-HP, Paris-Saclay, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France; French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France
| | - Aurélie Plessier
- French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France; Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France
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15
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Riolo FM, Alvarez DP, Villegas MO, Díaz JA. Neurological Symptoms of Congenital Portosystemic Shunt Reversed by Venous Endovascular Intervention: A Six Years Follow-Up Study. Neurol India 2020; 68:930-933. [PMID: 32859847 DOI: 10.4103/0028-3886.293475] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022]
Abstract
Congenital portosystemic shunt (CPSS) is a rare disorder characterized by a diversion of Porto-mesenteric blood into systemic veins. Type I is an end to side fistula between the portal vein and the inferior vena cava. Type II is a side to side fistula between the main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. Clinical presentation of these patients varies. Treatment and management are guided by the type of malformation and clinical presentation. Herein, we present a case of CPSS Type IIb with neurological symptoms, treated with endovascular occlusion with 6-year follow-up without remission.
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Affiliation(s)
- Federico Martin Riolo
- Hospital Nacional "Profesor Doctor Alejandro Posadas", El Palomar, Buenos Aires, Argentina
| | - Derwin Plazas Alvarez
- Hospital Nacional "Profesor Doctor Alejandro Posadas", El Palomar, Buenos Aires, Argentina
| | | | - José Antonio Díaz
- Hospital Nacional "Profesor Doctor Alejandro Posadas", El Palomar, Buenos Aires, Argentina
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16
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Nam HD. Living-donor liver transplantation for Abernethy malformation - case report and review of literature. Ann Hepatobiliary Pancreat Surg 2020; 24:203-208. [PMID: 32457268 PMCID: PMC7271108 DOI: 10.14701/ahbps.2020.24.2.203] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2019] [Revised: 01/23/2020] [Accepted: 01/29/2020] [Indexed: 11/17/2022] Open
Abstract
Abernethy malformation was named for the rare congenital absence of the portal vein (CAPV), also known as congenital extrahepatic portal-systemic shunts (CEPS). This could be classified as complete (type 1) or incomplete shunt (type 2) according to Morgan-Superina classification. Its presentation may show under variable signs and symptoms such as hepatopulmonary syndrome, hyper-ammonia, hepatic masses and liver failure…. This usually combined with other congenital anomalies (cardiac anomaly, trisomy…). This report presented a 10 year-old boy with growth retardation and mild mental recognition, intermittent hyperammonia, elevated liver enzymes, huge inoperable mass in the right liver. MS CT and MRI findings: hyperplasia of liver parenchyma with superior mesenteric vein confluenced with splenic vein before draining directly into the inferior vena cava (Abernethy anomaly type 1b). Living donor liver transplantation underwent using right lobe from his mother. Anatomopathology findings of the native liver showed chronic hepatitis with cirrhosis 4/6 Knodel-Ishak. Postoperatively, the patient still attained optimal liver function and has returned to normal life at 12-month follow-up. Liver transplantation was a reasonable indication for CAPV type 1. Living donor liver transplantation was effective and practical in the scarcity of donor organ.
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Affiliation(s)
- Hoang Duc Nam
- HBP-Liver Transplant Center, Vinmec Times City International Hospital, Hanoi, Vietnam
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17
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Tang H, Song P, Wang Z, Han B, Meng X, Pan Y, Meng X, Duan W. A basic understanding of congenital extrahepatic portosystemic shunt: incidence, mechanism, complications, diagnosis, and treatment. Intractable Rare Dis Res 2020; 9:64-70. [PMID: 32494552 PMCID: PMC7263991 DOI: 10.5582/irdr.2020.03005] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
Extrahepatic portosystemic shunt belongs to a family of rare vascular abnormalities. The clinical importance and manifestations of this vascular abnormality range from asymptomatic cases to liver or metabolic dysfunctions of various degrees. Congenital extrahepatic portosystemic shunt, also termed as Abernethy malformation, is a very rare congenital vascular malformation in which splenomesenteric blood drains into a systemic vein, bypassing the liver through a complete or partial extrahepatic shunt. So far, limited cases of congenital extrahepatic portosystemic shunt have been reported. In this review, incidence, mechanisms, complications, diagnoses and treatments of congenital extrahepatic portosystemic shunt are described.
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Affiliation(s)
- Haowen Tang
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Peipei Song
- The Institute for Global Health Policy Research, Bureau of International Health Cooperation, National Center for Global Health and Medicine, Tokyo, Japan
| | - Zhiqiang Wang
- Department of Hepatobiliary Surgery, Qinghai Red Cross Hospital, Xining, China
| | - Bing Han
- Department of General Surgery, Shanxian Central Hospital, Heze, China
| | - Xiangfei Meng
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Yingwei Pan
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
| | - Xuan Meng
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
- Address correspondence to:Weidong Duan and Xuan Meng, Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Haidian, Beijing 100853, China. E-mail: (Duan WD), (Meng X)
| | - Weidong Duan
- Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China
- Address correspondence to:Weidong Duan and Xuan Meng, Department of Hepatobiliary Surgery, First Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, 28 Fuxing Road, Haidian, Beijing 100853, China. E-mail: (Duan WD), (Meng X)
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18
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Le Van T, Duc DD, Duc NH, Van QV. The first report of living donor liver transplantation for abernethy malformation (congenital absence of the portal vein) in Vietnam. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2020.101419] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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19
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Kemp SR. Sonography Aids in Diagnosis of Congenital Absence of Portal Vein in Patient With Marfan Syndrome: A Case Study. JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY 2019. [DOI: 10.1177/8756479319848747] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Skylar R. Kemp
- Department of Radiology Ultrasound, University of Iowa Hospitals and Clinics, Iowa City, IA, USA
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20
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Lin KY, Chen H, Yu L. Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report. BMC Cardiovasc Disord 2019; 19:141. [PMID: 31196005 PMCID: PMC6567657 DOI: 10.1186/s12872-019-1124-1] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2019] [Accepted: 06/02/2019] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Congenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension. CASE PRESENTATION We report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient's condition improved in the first year after discharge. However, one year later, the patient's conditions start to deteriorate. CONCLUSION This article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.
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Affiliation(s)
- Kai-Yang Lin
- Department of Cardiology, Fujian Provincial Hospital, Fujian Medical University, Fujian Cardiovascular Institute, Fuzhou, 350001, China
| | - Hui Chen
- Department of Cardiology, Fujian Provincial Hospital, Fujian Medical University, Fujian Cardiovascular Institute, Fuzhou, 350001, China
| | - Ling Yu
- Department of Cardiology, Fujian Provincial Hospital, Fujian Medical University, Fujian Cardiovascular Institute, Fuzhou, 350001, China.
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21
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Jain V, Sangdup T, Agarwala S, Bishoi AK, Chauhan S, Dhua A, Jana M, Kandasamy D, Malik R, Kothari SS, Patcharu R, Varshney A, Bhatnagar V. Abernethy malformation type 2: varied presentation, management and outcome. J Pediatr Surg 2019; 54:760-765. [PMID: 30262201 DOI: 10.1016/j.jpedsurg.2018.08.053] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/17/2018] [Revised: 06/28/2018] [Accepted: 08/29/2018] [Indexed: 12/16/2022]
Abstract
PURPOSE To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation. MATERIAL AND METHODS Children with Type 2 Abernethy who had had been operated between 2013 and 2017 were included in the study. The diagnosis had been confirmed on ultrasonography, CECT or angiography. All patients underwent laparotomy. The shunt was identified, clamped and the bowel congestion was noted. The shunt was ligated if the bowel congestion was not significant or had improved. Relevant follow-up investigations were done to document the resolution or amelioration of symptoms and the patency of the shunt. RESULTS Five patients were included in the study with a median age of 6 years. Hepatopulmonary syndrome was the presentation in 4 patients while one patient presented with liver tumor. Ultrasonography and CECT were able to diagnose Type 2 malformation in 4 patients whereas in 1 patient the distal portal vein was not seen. The postoperative period was complicated in 3 patients. At the median follow up at 14 months, good intrahepatic portal flow in all patients. All patients demonstrated improvement/ resolution of symptoms. CONCLUSION Abernethy is rare malformation which can have a varied presentation. Additional investigations may be needed to confirm the diagnosis of Type 2 variety. Most patients have gradual improvement of symptoms. LEVEL OF EVIDENCE Level IV/ Treatment study.
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Affiliation(s)
- Vishesh Jain
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Tsering Sangdup
- Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Sandeep Agarwala
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029.
| | - Akshay Kumar Bishoi
- Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Sandeep Chauhan
- Department of Cardiac-Anaesthesia, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Anjan Dhua
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Manisha Jana
- Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India 110029
| | | | - Rohan Malik
- Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Shyam Sunder Kothari
- Department of Cardiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Ravi Patcharu
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Abhimanyu Varshney
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029
| | - Veereshwar Bhatnagar
- Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India 110029
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Sanada Y, Mizuta K. Congenital absence of the portal vein: translated version. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2018; 25:359-369. [PMID: 30160060 DOI: 10.1002/jhbp.572] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward.
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Affiliation(s)
- Yukihiro Sanada
- Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan
| | - Koichi Mizuta
- Department of Transplant Surgery, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke City, Tochigi 329-0498, Japan
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Franchi-Abella S, Gonzales E, Ackermann O, Branchereau S, Pariente D, Guérin F. Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 2018; 43:2023-2036. [PMID: 29730740 DOI: 10.1007/s00261-018-1619-8] [Citation(s) in RCA: 52] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Congenital portosystemic shunts (CPSS) are rare vascular malformations that create an abnormal connection between portal and systemic veins resulting in complete or partial diversion of the portal flow away from the liver to the systemic venous system. Different anatomic types exist and several classifications have been proposed. They can be associated with other malformations especially cardiac and heterotaxia. The main complications include hepatic encephalopathy, liver tumors, portopulmonary hypertension, and pulmonary arteriovenous shunts. Diagnosis relies on imaging, and prenatal diagnosis is possible. Spontaneous closure of the CPSS is possible in some anatomic forms during the first year of life. When the CPSS remains patent, radiologic or surgical closure of the CPSS may prevent, resolve, or stabilize complications. Interventional radiology plays a key role for both the preoperative evaluation with occlusion test to assess the exact anatomy and to measure portal pressure after occlusion of the CPSS. Endovascular closure is the first option for treatment when possible.
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Affiliation(s)
- Stéphanie Franchi-Abella
- Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 rue du Général Leclerc, 94278, Le Kremlin-Bicêtre, France.
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France.
- Hepatinov, University Paris -Sud, Orsay, France.
- IR4 M-UMR 8081- University Paris -Sud, Orsay, France.
| | - Emmanuel Gonzales
- Pediatric Hepatology, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, National Centre for Biliary Atresia, Le Kremlin-Bicêtre, France
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France
- Hepatinov, University Paris -Sud, Orsay, France
- INSERM UMR-S1174, Orsay, France
| | - Oanez Ackermann
- Pediatric Hepatology, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, National Centre for Biliary Atresia, Le Kremlin-Bicêtre, France
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France
- Hepatinov, University Paris -Sud, Orsay, France
| | - Sophie Branchereau
- Pediatric Surgery Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France
- Hepatinov, University Paris -Sud, Orsay, France
| | - Danièle Pariente
- Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, 78 rue du Général Leclerc, 94278, Le Kremlin-Bicêtre, France
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France
- Hepatinov, University Paris -Sud, Orsay, France
| | - Florent Guérin
- Pediatric Surgery Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France
- Pediatric Liver Transplantation Unit, National Reference Centre for Rare Pediatric Liver Diseases and Filfoie, Paris, France
- Hepatinov, University Paris -Sud, Orsay, France
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24
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Sharma S, Bobhate PR, Sable S, Kumar S, Yadav K, Maheshwari S, Amin S, Chauhan A, Varma V, Kapoor S, Kumaran V. Abernethy malformation: Single-center experience from India with review of literature. Indian J Gastroenterol 2018; 37:359-364. [PMID: 30187299 DOI: 10.1007/s12664-018-0884-3] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2018] [Accepted: 08/05/2018] [Indexed: 02/04/2023]
Abstract
Abernethy malformation is a rare congenital anomaly in which there is direct communication between the portal and systemic venous circulation. The clinical presentation ranges from asymptomatic with incidental detection on imaging to secondary complications of disease or related to associate anomalies. This is a retrospective analysis of data from nine patients with Abernethy malformation at a single center. This is a referral center for Pediatric Cardiology and for Hepatobiliary and Pancreatic Surgery. The patients presented to the Pulmonary Hypertension Clinic/the Hepatobiliary Surgery Clinic. Out of nine patients, four were male. Type II Abernethy malformation was present in five patients whereas three patients had type I malformation. One of the patients had communication between inferior mesenteric vein and internal iliac vein. Five out of nine patients were erroneously diagnosed as idiopathic primary pulmonary hypertension and were treated with vasodilators. One patient required living donor liver transplant. One patient was managed with surgical shunt closure whereas two patients required transcatheter shunt closure. The rest of the patients were managed conservatively. Abernethy malformation is more common than previously thought and the diagnosis is often missed. There are various management options for Abernethy malformation, which includes surgical or transcatheter shunt closure and liver transplant. Management of Abernethy malformation depends upon type, presentation, and size of shunt.
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Affiliation(s)
- Swapnil Sharma
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India.
| | - Prashant R Bobhate
- Department of Pediatric Cardiology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Shailesh Sable
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Suneed Kumar
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Kapildev Yadav
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Sharad Maheshwari
- Department of Radiology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Saista Amin
- Department of Pediatric Hepatology, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Ashutosh Chauhan
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Vibha Varma
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Sorabh Kapoor
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
| | - Vinay Kumaran
- Department of Liver Transplant and HPB Surgery, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Achutrao Patwardhan Marg, Andheri (West), Mumbai, 400 053, India
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25
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Christou N, Dib N, Chuffart E, Taibi A, Durand-Fontanier S, Valleix D, Mathonnet M. Stepwise management of hepatocellular carcinoma associated with Abernethy syndrome. Clin Case Rep 2018; 6:930-934. [PMID: 29744090 PMCID: PMC5930207 DOI: 10.1002/ccr3.1384] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2017] [Revised: 09/05/2017] [Accepted: 10/18/2017] [Indexed: 12/22/2022] Open
Abstract
Patients with congenital agenesis of the portal vein may develop hepatocellular tumors due to enhanced arterial blood flow. These tumors may be benign (FNH, adenomas) or malignant (hepatoblastoma, HCC). Liver resection can be proposed, and preoperative arterial embolization may decrease blood loss during surgery. Liver transplantation with PV reconstruction is also an option.
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Affiliation(s)
- Niki Christou
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Nabil Dib
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Etienne Chuffart
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Abdelkader Taibi
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Sylvaine Durand-Fontanier
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Denis Valleix
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
| | - Muriel Mathonnet
- Department of Digestive and Endocrine Surgery University Hospital of Limoges 2 avenue Martin Luther King 87042 Limoges Cedex France
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26
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Benedict M, Rodriguez-Davalos M, Emre S, Walther Z, Morotti R. Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) With Associated Hepatocellular Carcinoma: Case Report and Literature Review. Pediatr Dev Pathol 2017; 20:354-362. [PMID: 28727971 DOI: 10.1177/1093526616686458] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein, we report a case of Abernethy malformation, type Ib, in a 12-month-old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years with Abernethy malformation.
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Affiliation(s)
- Mark Benedict
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
| | | | - Sukru Emre
- 2 Department of Surgery, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Zenta Walther
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
| | - Raffaella Morotti
- 1 Department of Pathology, Yale University School of Medicine, New Haven, Connecticut, USA
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27
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Liver transplantation in an adult with adenomatosis and congenital absence of the portal vein: a case report. Transplant Proc 2015; 46:2418-21. [PMID: 25242797 DOI: 10.1016/j.transproceed.2014.04.012] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2014] [Accepted: 04/01/2014] [Indexed: 02/07/2023]
Abstract
Congenital absence of the portal vein (CAPV) is a rare congenital anomaly in which the superior mesenteric veins (SMV) and splenic veins converge and bypass the liver, effectively draining directly into the systemic venous circulation via the inferior vena cava (IVC), or alternatively the renal or iliac vein, creating a native portosystemic shunt. Portosystemic shunting results in clinical manifestations of hepatic encephalopathy as well as a predisposition to focal nodular hyperplasia and tumors, including adenomas, hepatoblastoma, and hepatocellular carcinoma (HCC), by the disruption of enterohepatic blood flow. Historically, CAPV has been thought to be a rare condition found mainly at autopsy, however, in recent years due to advances in radiological techniques, CAPV detection has increased. Herein we describe a patient with known CAPV who initially underwent hepatic resection for HCC. During surveillance, additional masses were discovered and were identified as recurrent HCC. Unfortunately, this patient was not a candidate for further resection or locoregional therapy. We demonstrate that transplantation is a challenging but technically viable option for treatment of HCC complicating adenomatosis-associated CAPV.
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28
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Nakazawa R, Sato Y, Sasaki H, Shibagaki Y, Kimura K, Chikaraishi T. Pharmacokinetic analysis of cyclosporine in a renal transplant recipient with congenital absence of the portal vein. Int J Urol 2015; 22:785-7. [DOI: 10.1111/iju.12830] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2014] [Accepted: 04/29/2015] [Indexed: 11/28/2022]
Affiliation(s)
- Ryuto Nakazawa
- Department of Urology; St. Marianna University School of Medicine; Kawasaki Japan
| | - Yuichi Sato
- Department of Urology; St. Marianna University School of Medicine; Kawasaki Japan
| | - Hideo Sasaki
- Department of Urology; St. Marianna University School of Medicine; Kawasaki Japan
| | - Yugo Shibagaki
- Division of Nephrology and Hypertension; Department of Internal Medicine; St. Marianna University School of Medicine; Kawasaki Japan
| | - Kenjiro Kimura
- Division of Nephrology and Hypertension; Department of Internal Medicine; St. Marianna University School of Medicine; Kawasaki Japan
| | - Tatsuya Chikaraishi
- Department of Urology; St. Marianna University School of Medicine; Kawasaki Japan
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29
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Epelman M, Johnson C, Hellinger JC, Darge K, Newman B. Vascular Lesions—Congenital, Acquired, and Iatrogenic: Imaging in the Neonate. Semin Ultrasound CT MR 2015; 36:193-215. [DOI: 10.1053/j.sult.2015.01.009] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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30
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Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature. Cardiol Young 2015; 25:208-17. [PMID: 24848245 DOI: 10.1017/s1047951114000729] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Agenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.
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31
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Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein--Case report and a review of literature. Clin Anat 2015; 23:750-8. [PMID: 20533511 DOI: 10.1002/ca.21007] [Citation(s) in RCA: 41] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2010] [Revised: 04/13/2010] [Accepted: 04/20/2010] [Indexed: 01/30/2023]
Abstract
Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and the splenic venous drainage bypass the liver and drain into systemic veins through various venous shunts. To our knowledge, we have reviewed all 83 cases of CAPV, since first described in 1793. This equates to a rate of almost 2.5 cases per year over the last 30 years. Morgan and Superina (1994, J. Pediatr. Surg. 29:1239-1241) proposed the following classification of portosystemic anomalies; either the liver is not perfused with portal blood because of a complete shunt (Type I) or the liver is perfused with portal blood due to the presence of a partial shunt (Type II). In our case, abdominal venous blood drained into the suprarenal inferior vena cava via the left renal vein and dilated left gastric veins. After analyzing all reported cases, we recognize that more than 65% of patients are females and more than 30% of all published cases had been diagnosed by the age of 5 years. Additional anomalies are common in CAPV. In the reported cases, more then 22% of patients had congenital heart disease. Other commonly found anomalies include abnormalities of the spleen, urinary and male genital tract, brain as well as skeletal anomalies. Hepatic changes such as focal nodular hyperplasia, hepatocellular carcinoma, and hepatoblastoma are diagnosed in more then 40% of patients. This article also illustrates the radiological findings of CAPV. Radiological evaluation by ultrasound, CT, and MRI is helpful to detect coexisting abnormalities.
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Affiliation(s)
- Jana Mistinova
- Department of Radiology, Faculty of Medicine and University Hospital, Comenius University Bratislava, Slovak Republic
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32
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Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review. Oncol Lett 2014; 9:695-700. [PMID: 25624897 PMCID: PMC4301562 DOI: 10.3892/ol.2014.2767] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2014] [Accepted: 10/29/2014] [Indexed: 11/13/2022] Open
Abstract
Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases.
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33
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Velayutham P, Dev A, Arora A. Congenital extrahepatic portocaval shunt: growth in vain. J Pediatr 2013; 162:1076.e1. [PMID: 23295141 DOI: 10.1016/j.jpeds.2012.11.066] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/02/2012] [Accepted: 11/13/2012] [Indexed: 12/13/2022]
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Abstract
BACKGROUND Congenital extrahepatic portosystemic shunt (CEPS) is reported more frequently because of advances in imaging techniques. Liver transplantation (LT) is a therapeutic option, although the indications for LT are still controversial. METHODS This study reviewed 34 cases of LT for CEPS, including 30 cases reported in the English medical literature and the patients treated in our department, to collect the clinical data associated with LT. RESULTS The median age at diagnosis and LT was 3.7 and 6.8 years, respectively. Hepatic encephalopathy, including persistent hyperammonemia, was the most common indication of LT. Pulmonary complications, including hepatopulmonary syndrome and pulmonary hypertension, were the second most common indications of LT, and those patients underwent LT soon after the diagnosis. Although a shunt directly draining into the inferior vena cava was the most common type and managed by a simple direct anastomosis of the portal vein at LT, some cases required the modification of the portal vein reconstruction, such as interposition. Thirty patients were alive with a median follow-up period of 18 months. CONCLUSIONS LT for CEPS showed an excellent outcome. The development of pulmonary complications is an early indication for LT. Precise planning of portal vein reconstruction is required before LT.
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35
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Guérin F, Blanc T, Gauthier F, Abella SF, Branchereau S. Congenital portosystemic vascular malformations. Semin Pediatr Surg 2012; 21:233-44. [PMID: 22800976 DOI: 10.1053/j.sempedsurg.2012.05.006] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.
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Affiliation(s)
- Florent Guérin
- Department of Pediatric Surgery, Bicêtre Hospital, Paris, France.
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36
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Nagata H, Yamamura K, Ikeda K, Ihara K, Hara T. Balloon-occluded retrograde transvenous obliteration for congenital portosystemic venous shunt: report of two cases. Pediatr Int 2012; 54:419-21. [PMID: 22631572 DOI: 10.1111/j.1442-200x.2011.03459.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Affiliation(s)
- Hazumu Nagata
- Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
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37
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Pathak A, Agarwal N, Mandliya J, Gehlot P, Dhaneria M. Abernethy malformation: a case report. BMC Pediatr 2012; 12:57. [PMID: 22642663 PMCID: PMC3441307 DOI: 10.1186/1471-2431-12-57] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2011] [Accepted: 05/10/2012] [Indexed: 01/22/2023] Open
Abstract
Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4 weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients.
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Affiliation(s)
- Ashish Pathak
- Department of Pediatrics, R.D. Gardi Medical College, Ujjain, India.
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38
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Loomba RS, Telega GW, Gudausky TM. Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 2012; 47:E25-31. [PMID: 22595606 DOI: 10.1016/j.jpedsurg.2011.12.031] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/21/2011] [Revised: 12/12/2011] [Accepted: 12/29/2011] [Indexed: 11/18/2022]
Abstract
We report the case of a child with Abernethy malformation with an anomalous connection between the portal vein and the coronary sinus. After 30 months of close follow-up, the patient developed hepatoblastoma, a previously documented complication of the Abernethy malformation. This case reports a unique variant of Abernethy malformation and documents the first reported case of hepatoblastoma in a patient with type 2 Abernethy malformation.
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Affiliation(s)
- Rohit S Loomba
- Children's Hospital of Wisconsin/Herma Heart Center, Milwaukee, WI 53226, USA.
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Sanada Y, Urahashi T, Ihara Y, Wakiya T, Okada N, Yamada N, Egami S, Hishikawa S, Kawano Y, Ushijima K, Otomo S, Sakamoto K, Nakata M, Yasuda Y, Mizuta K. The role of operative intervention in management of congenital extrahepatic portosystemic shunt. Surgery 2011; 151:404-11. [PMID: 21962400 DOI: 10.1016/j.surg.2011.07.035] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2010] [Accepted: 07/08/2011] [Indexed: 01/07/2023]
Abstract
BACKGROUND AND AIMS Congenital extrahepatic portosystemic shunt (CEPS) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. It is still a matter of debate whether conservative or operative strategies should be used to treat symptomatic CEPS. The aim of this study was to evaluate the role of operative intervention in the management of CEPS. METHODS Between June 2004 and August 2010, 6 consecutive patients with symptomatic CEPS were treated in our department. There were 3 male and 3 female patients, with a median age of 3.5 years (range, 1-8). Their demographic, clinical, and laboratory data were analyzed. All patients were scheduled to undergo shunt ligation or liver transplantation (LT). RESULTS Living donor LT was carried out in 4 patients, and shunt ligation in 2. After a median follow-up of 25 months, all the patients are alive currently with marked relief of symptoms. CONCLUSION Shunt ligation or LT for symptomatic CEPS is potentially curative.
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Affiliation(s)
- Yukihiro Sanada
- Department of Transplant Surgery, Jichi Medical University, Tochigi, Japan.
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Chandler TM, Heran MK, Chang SD, Parvez A, Harris AC. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging 2011; 29:881-6. [DOI: 10.1016/j.mri.2011.03.001] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2011] [Accepted: 03/07/2011] [Indexed: 12/12/2022]
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Congenital absence of the portal vein in a middle-aged man. Surg Radiol Anat 2010; 33:369-72. [DOI: 10.1007/s00276-010-0711-8] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2009] [Accepted: 08/03/2010] [Indexed: 10/18/2022]
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Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010; 51:322-30. [PMID: 20601902 DOI: 10.1097/mpg.0b013e3181d9cb92] [Citation(s) in RCA: 159] [Impact Index Per Article: 10.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
BACKGROUND AND OBJECTIVE Congenital portosystemic shunts are rare vascular malformations that lead to severe complications. Their management is controversial. The aim of this study was to propose a clear definition of the risks and management of congenital portosystemic shunts in children according to our experience and a review of the literature. PATIENTS AND METHODS Twenty-two children with a complicated congenital portosystemic shunt were studied in our institution. When necessary, management included portal pressure measurement and portal vein angiography during an occlusion test and closure of the shunt by surgical and/or endovascular methods. RESULTS Five neonates with intrahepatic shunts presented with cholestasis that resolved spontaneously, and 17 older children presented with liver tumors (13) and/or hepatopulmonary syndrome (2), pulmonary artery hypertension (3), portosystemic encephalopathy (3), heart failure (1), and glomerulonephritis (1). The portosystemic shunt was extrahepatic (11) or intrahepatic (6). Portosystemic shunts were closed by endovascular methods in 5 children and surgically in 10, 4 of whom had portal pressure during occlusion above 35 mmHg and extremely hypoplastic or undetectable portal veins requiring banding of the fistula before closure. Shunt closure resulted in restoration of intrahepatic portal flow in all, with complete or partial regression of benign liver masses, and regression or stabilization of pulmonary, cardiac, neurological, and renal complications. CONCLUSIONS Congenital portosystemic shunt carries risks of severe complications in children. Closure of a shunt persisting after age 2 years should be considered preventively. Intrahepatic portal flux restoration can be expected, even when intrahepatic portal veins are extremely hypoplastic or undetectable.
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Afzal S, Nair A, Grainger J, Latif S, Rehman AU. Spontaneous thrombosis of congenital extrahepatic portosystemic shunt (Abernethy malformation) simulating inguinal hernia incarceration. Vasc Endovascular Surg 2010; 44:508-10. [PMID: 20551093 DOI: 10.1177/1538574410373666] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Tender lumps in the inguinal region are often explored emergently to treat suspected hernial strangulation. We discuss the case of an adult male who presented acutely with a tender inguinal swelling and raised inflammatory markers and was therefore deemed as requiring surgical exploration. However preoperative abdominal computerized tomography (CT) revealed an extensive thrombosing congenital venous malformation of portosystemic origin with extension into the symptomatic inguinal canal. A potentially lethal exsanguination from surgery was thus avoided.
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Affiliation(s)
- Samara Afzal
- Department of Vascular Surgery, Russells Hall Hospital, Dudley, West Midlands, United Kingdom
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Kuo MD, Miller FJ, Lavine JE, Peterson M, Finch M. Exploiting Phenotypic Plasticity for the Treatment of Hepatopulmonary Shunting in Abernethy Malformation. J Vasc Interv Radiol 2010; 21:917-22. [DOI: 10.1016/j.jvir.2010.01.038] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2009] [Revised: 01/10/2010] [Accepted: 01/18/2010] [Indexed: 10/19/2022] Open
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Singhal A, Srivastava A, Goyal N, Vij V, Wadhawan M, Bera M, Gupta S. Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting. Pediatr Transplant 2009; 13:1041-7. [PMID: 19254272 DOI: 10.1111/j.1399-3046.2009.01092.x] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
Abstract
Congenital portosystemic shunts are the anomalies in which the mesenteric venous drainage bypasses the liver and drains directly into the systemic circulation. This is a report of a rare case of LDLT in a four-yr old male child suffering with biliary atresia (post-failed Kasai procedure) associated with (i) a large congenital CEPSh from the spleno-mesentric confluence to the LHV, (ii) intrapulmonary shunts, (iii) perimembranous VSD. The left lobe graft was procured from the mother of the child. Recipient IVC and the shunt vessel were preserved during the hepatectomy, and the caval and shunt clamping were remarkably short while performing the HV and portal anastomosis. Post-operative course was uneventful; intrapulmonary shunts regressed within three months after transplantation and currently after 18 months following transplant child is doing well with normal liver functions. CEPSh has been extensively discussed and all the published cases of liver transplantation for CEPSh were reviewed.
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Affiliation(s)
- Ashish Singhal
- Department of Surgical Gastroenterolgy & Liver Transplant, Indraprastha Apollo Hospital, New Delhi, India
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Sanada Y, Mizuta K, Kawano Y, Egami S, Hayashida M, Wakiya T, Mori M, Hishikawa S, Morishima K, Fujiwara T, Sakuma Y, Hyodo M, Yasuda Y, Kobayashi E, Kawarasaki H. Living Donor Liver Transplantation for Congenital Absence of the Portal Vein. Transplant Proc 2009; 41:4214-9. [DOI: 10.1016/j.transproceed.2009.08.080] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2008] [Revised: 02/19/2009] [Accepted: 08/17/2009] [Indexed: 01/08/2023]
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Yu CY, Chen CL, Huang TL, Chen TY, Tsang LLC, Ou HY, Concejero AM, Wang CC, Wang SH, Cheng YF. Preoperative imaging evaluation of the hepatic vasculature in biliary atresia patients undergoing LDLT: comparison of MDCT and MRI. Pediatr Transplant 2009; 13:984-9. [PMID: 19032411 DOI: 10.1111/j.1399-3046.2008.01100.x] [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] [Indexed: 12/21/2022]
Abstract
LT is the definitive treatment option in the management of end-stage liver disease. Preoperative vascular evaluation plays an important role for a safe and successful operation in LDLT. The purpose of this study is to assess the usefulness and accuracy of CTA and MRA in evaluating vascular anomalies in BA patients undergoing LDLT. Images of CTA and MRA for preoperative vascular evaluation in 57 BA patients undergoing LDLT were reviewed with the operative and pathologic findings. All underwent preoperative CTA and MRA. Pathologic PV (n = 20), interruption of the retro-hepatic vena cava (n = 1), aberrant right HA from the SMA (n = 2) were confirmed during the transplant operation. The success rate of CTA and MRA in identifying vascular anomalies was 96% and 82%, respectively (p = 0.01). The IQR scores were 3.25 +/- 0.53 for CTA and 2.91 +/- 0.70 for MRA (p = 0.001). The sensitivity, specificity and accuracy of CTA were 85%, 97% and 93%, respectively; and for MRA, were 65%, 95% and 84%, respectively. CTA is superior than MRA in the preoperative evaluation of the vascular anatomy in pediatric BA LDLT candidates.
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Affiliation(s)
- Chun-Yen Yu
- Liver Transplantation Program and Department of Diagnostic Radiology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, and Chang Gung University College of Medicine, Taiwan
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Kasahara M, Nakagawa A, Sakamoto S, Tanaka H, Shigeta T, Fukuda A, Nosaka S, Matsui A. Living donor liver transplantation for congenital absence of the portal vein with situs inversus. Liver Transpl 2009; 15:1641-3. [PMID: 19877232 DOI: 10.1002/lt.21839] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Mureo Kasahara
- Department of Transplant Surgery, National Center for Child Health and Development, Tokyo, Japan.
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Tan Y, Xiao EH. Application of computed tomography for diagnosis and treatment of liver diseases in children. Shijie Huaren Xiaohua Zazhi 2009; 17:1485-1488. [DOI: 10.11569/wcjd.v17.i15.1485] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Computed tomography (CT) has become an extremely valuable tool for disease diagnosis since it was invented. With the rapid development of technology in recent years, CT has become more widely used, especially in this particular group of children. This article sums up on the application of CT for liver cancer, portal lesions, liver trauma and the application of low-dose CT in children.
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