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Tanji Y, Furukawa K, Igarashi Y, Yanagaki M, Haruki K, Shirai Y, Taniai T, Gocho T, Okui N, Ikegami T. Living donor liver transplantation for idiopathic portal hypertension with focal nodular hyperplasia. Surg Case Rep 2022; 8:73. [PMID: 35445894 PMCID: PMC9023646 DOI: 10.1186/s40792-022-01428-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2022] [Accepted: 04/12/2022] [Indexed: 11/10/2022] Open
Abstract
The patient was a 61-year-old woman with a history of diabetes mellitus who had undergone ileocecal resection for ascending colon carcinoma 5 years earlier, followed by a postoperative adjuvant chemotherapy with XELOX (capecitabine + oxaliplatin). During follow-up, the liver gradually atrophied, and radiological imaging showed suspicious findings of 20 × 14 mm hepatocellular carcinoma (HCC) in the right lobe of the liver. The patient also underwent endoscopic variceal ligation for the esophageal varices. She was referred to our hospital for living donor liver transplantation (LDLT) due to decompensated liver cirrhosis with HCC. The patient did not have hepatitis B or C, and history of alcohol, suggesting that her liver cirrhosis was caused by a non-alcoholic steatohepatitis. The Child-Pugh score was 10 points (class C) and the Model for End-Stage Liver Disease (MELD) score was 8 points. The possibility of HCC could not be ruled out, and LDLT was performed. Postoperative pathological examination revealed idiopathic portal hypertension (IPH), and the mass lesion was diagnosed as focal nodular hyperplasia (FNH). The postoperative course was uneventful and the patient was discharged on postoperative day 14. This is the first case of liver transplantation for IPH with FNH.
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Affiliation(s)
- Yoshiaki Tanji
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Kenei Furukawa
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan.
| | - Yosuke Igarashi
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Mitsuru Yanagaki
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Koichiro Haruki
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Yoshihiro Shirai
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Tomohiko Taniai
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Takeshi Gocho
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Norimitsu Okui
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
| | - Toru Ikegami
- Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8, Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan
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Bombardier B, Alli A, Rohr A, Collins Z, Raval K. A case of two shunts in the endovascular treatment of type II Abernethy syndrome. CVIR Endovasc 2022; 5:3. [PMID: 34985620 PMCID: PMC8733101 DOI: 10.1186/s42155-021-00279-7] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2021] [Accepted: 12/21/2021] [Indexed: 11/25/2022] Open
Abstract
Background Abernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population. Case presentation We report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT. Conclusions This report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.
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Affiliation(s)
- Brenden Bombardier
- University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas City, KS, 66160, USA.
| | - Adam Alli
- University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas City, KS, 66160, USA
| | - Aaron Rohr
- University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas City, KS, 66160, USA
| | - Zachary Collins
- University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas City, KS, 66160, USA
| | - Kavi Raval
- University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas City, KS, 66160, USA
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Uchida H, Sakamoto S, Kasahara M, Kudo H, Okajima H, Nio M, Umeshita K, Ohdan H, Egawa H, Uemoto S. Longterm Outcome of Liver Transplantation for Congenital Extrahepatic Portosystemic Shunt. Liver Transpl 2021; 27:236-247. [PMID: 37160013 DOI: 10.1002/lt.25805] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2019] [Revised: 04/01/2020] [Accepted: 05/01/2020] [Indexed: 02/07/2023]
Abstract
Liver transplantation (LT) is often viewed as the last resort for the treatment of congenital extrahepatic portosystemic shunt (CEPS) due to advancement of imaging and interventional radiology techniques. However, some patients still require LT, and criteria for LT are yet to be determined. We conducted a national survey of patients undergoing LT for CEPS between June 1998 and August 2018 and evaluated the clinical data and outcomes with a review of previously reported patients from the English-language medical literature. A total of 26 patients underwent LT in Japan at a median age of 5.2 years old. The most common indications for LT were persistent hyperammonemia (54%) and liver mass (50%), followed by pulmonary complications (38%). Pulmonary complications in all patients, including intrapulmonary shunt and pulmonary hypertension (PH), were improved after LT. Regarding the 29 previously reported patients in the English-language literature, a liver nodule (49%), including hepatoblastoma and hepatocellular carcinoma, was the most common indication for LT, followed by pulmonary complications (34%). A total of 25 (96%) patients in our survey and 26 (90%) patients in the literature review were alive with a median follow-up period of 9.5 and 1.6 years, respectively. Although LT has a limited role in management of CEPS, our study indicated that LT was safe as an alternative treatment for select patients with malignant tumor or pulmonary complications and those with complications related to new portosystemic collateral vessels after shunt closure, such as PH or hepatopulmonary syndrome.
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Affiliation(s)
- Hajime Uchida
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Seisuke Sakamoto
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Mureo Kasahara
- Organ Transplantation Center, National Center for Child Health and Development, Tokyo, Japan
| | - Hironori Kudo
- Department of Pediatric Surgery, Tohoku University, Miyagi, Japan
| | - Hideaki Okajima
- Department of Pediatric Surgery, Kanazawa Medical University, Ishikawa, Japan
| | - Masaki Nio
- Department of Pediatric Surgery, Tohoku University, Miyagi, Japan
| | - Koji Umeshita
- Department of Surgery, Osaka University, Osaka, Japan
| | - Hideki Ohdan
- Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Hiroto Egawa
- Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, Tokyo, Japan
| | - Shinji Uemoto
- Department of Surgery, Kyoto University, Kyoto, Japan
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Peček J, Fister P, Homan M. Abernethy syndrome in Slovenian children: Five case reports and review of literature. World J Gastroenterol 2020; 26:5731-5744. [PMID: 33088165 PMCID: PMC7545390 DOI: 10.3748/wjg.v26.i37.5731] [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: 05/30/2020] [Revised: 08/11/2020] [Accepted: 09/17/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Abernethy syndrome is a congenital vascular anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt. Although the number of recognized and reported cases is gradually increasing, Abernethy syndrome is still a rare disease entity, with an estimated prevalence between 1 per 30000 to 1 per 50000 cases. With this case series, we aimed to contribute to the growing knowledge of potential clinical presentations, course and complications of congenital portosystemic shunts (CPSS) in children.
CASE SUMMARY Five children are presented in this case series: One female and four males, two with an intrahepatic CPSS and three with an extrahepatic CPSS. The first patient, who was diagnosed with an intrahepatic CPSS, presented with gastrointestinal bleeding, abdominal pain and hyperammonaemia at six years of age. He underwent a percutaneous embolization of his shunt and has remained asymptomatic ever since. The second patient presented with direct hyperbilirubinemia in the neonatal period and his intrahepatic CPSS later spontaneously regressed. The third patient had pulmonary hypertension and hyperammonaemia due to complete portal vein agenesis and underwent liver transplantation at five years of age. The fourth patient was diagnosed immediately after birth, when evaluated due to another congenital vascular anomaly, and the last patient presented as a teenager with recurrent bone fractures associated with severe osteoporosis. In addition, the last two patients are characterised by benign liver nodules; however, they are clinically stable on symptomatic therapy.
CONCLUSION Abernethy syndrome is a rare anomaly with diverse clinical features, affecting almost all organ systems and presenting at any age.
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Affiliation(s)
- Jerneja Peček
- Division of Paediatrics, University Medical Centre Ljubljana, Ljubljana 1000, Slovenia
| | - Petja Fister
- Department of Neonatology, Division of Paediatrics, University Medical Centre Ljubljana; Faculty of medicine, University of Ljubljana, Ljubljana 1000, Slovenia
| | - Matjaž Homan
- Department of Gastroenterology, Hepatology and Nutrition, Division of Paediatrics, University Medical Centre Ljubljana; Faculty of Medicine, University of Ljubljana, Ljubljana 1000, Slovenia
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