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A case of two shunts in the endovascular treatment of type II Abernethy syndrome

A case of two shunts in the endovascular treatment of type II Abernethy syndrome 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. Keywords: Abernethy syndrome, Portosystemic shunt, Portal vein, Shunt embolization, Venous malformations Introduction mesenteric vein drain separately into the systemic circu- Abernethy malformation is a rare condition defined by a lation via the inferior vena cava, and type 1b, where the congenital extrahepatic portosystemic shunt. Two types splenic vein and superior mesenteric vein form a com- of Abernethy syndrome exist: Type I malformations have mon trunk before draining into the systemic circulation a congenital absence of the portal vein which leads to via the inferior vena cava. Type II malformations have a complete diversion of portal blood into the systemic cir- hypoplastic portal vein which leads to at least some of culation. Type I malformations can be further classified the portal blood flow diverting into the systemic circula- into type 1a, where the splenic vein and superior tion (Fig. 1). (Papamichail et al. 2018; Alonso-Gamarra et al. 2011). The treatment for Type I Abernethy malformations is * Correspondence: bbombardier@kumc.edu University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas liver transplant. (Papamichail et al. 2018; Alonso- City, KS 66160, USA © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Bombardier et al. CVIR Endovascular (2022) 5:3 Page 2 of 5 Fig. 1 Diagram demonstrating normal hepatic anatomy and hepatic anatomy in Abernethy Syndrome Type Ia, Ib, and II. PV: portal vein, IVC: inferior vena cava, SV: splenic vein, SMV: superior mesenteric vein Gamarra et al. 2011; Xiang et al. 2019) Although there Case report are no consensus treatment guidelines for Type II Aber- A 20-year-old female patient presented with neurocogni- nethy malformations, they are now commonly treated by tive behavioral difficulty, voracious appetite, and chronic endovascular methods. The current literature describes encephalopathy. Despite maximal medical treatment, the closure of extrahepatic portal shunts using a variety of patient had persistently elevated serum ammonia levels, materials including atrial septal occluder devices, VSD with pre-operative levels of 135 and 98 mcmol/L, along occluder devices, Amplatzer vascular plugs, n-butyl with clinical signs and symptoms of encephalopathy. cyanoacrylate, coils, and microcoils. (Papamichail et al. Contrast-enhanced computed tomography (CT) demon- 2018; Venkateshwaran et al. 2014; Rajeswaran et al. strated a hypoplastic intrahepatic portal venous system. 2020; Loureiro et al. 2021; Grimaldi et al. 2012; Suzuki The superior mesenteric vein and splenic vein joined to et al. 2013; Yoshimatsu et al. 2006) Rajeswaran et al. form a short extrahepatic portal vein, with dominant retrospectively studied patients with congenital portosys- flow into two parallel shunts into the suprahepatic infer- temic shunts treated by either operative ligation, endo- ior vena cava (IVC) (Fig. 2). The patient was previously vascular occlusion, combined surgical & endovascular evaluated for candidacy for a liver transplant at a local closure, and observation. They found that at 1 month, children’s hospital but was denied eligibility. Thus, the serum ammonia levels decreased from 82.5 ± 10.3 μmol/ patient and her family agreed to an endovascular pro- L to 38.4 ± 4.6 μmol/L after shunt closure and no differ- cedure to increase blood flow into the intrahepatic por- ence was observed in the decrease between patients tal venous system and decrease blood flow through the treated surgically versus endovascularly. Mean occluded portosystemic shunts. to non-occluded portal pressure gradients were sig- The embolization of the patient’s two extrahepatic nificantly lower for endovascular closure (5.3 + − 1.8 portosystemic shunts was performed via a right internal mmHg) than for surgical closure (12.3 + − 3.3 mmHg). jugular approach. The IVC was selected with an 0.035″ (Rajeswaran et al. 2020) Symptom regression was re- Amplatz wire (Boston Scientific, Marlborough, Massa- ported in most patients following occlusion including chusetts) with the aid of a 5F JB1 catheter (Angiody- improvement in neurologic symptoms and hepatopul- namics, Latham, New York). The catheter was then monary syndrome.(Venkateshwaran et al. 2014;Lour- removed, and a 10F RAABE Flexor sheath (Cook Med- eiro et al. 2021; Grimaldi et al. 2012;Yoshimatsu ical, Bloomington, Indiana) was advanced into the IVC. et al. 2006; Franchi-Abella et al. 2018) Complications An inferior vena cavagram was performed, demonstrat- of closure include reported cases of acute portal ing wide patency. Next, a JB1 catheter and a .035 angled hypertension and/or thrombosis. However, these re- glidewire (Terumo, Tokyo, Japan) were used to select solved with anticoagulation therapy. (Franchi-Abella the right hepatic vein. Digital subtraction angiography et al. 2018) This report describes a case of a type II (DSA) was performed demonstrating a patent right hep- Abernethy malformation with not one, but two extra- atic vein. The left hepatic vein was then selected, dem- hepatic portosystemic shunts, treated with endovascu- onstrating a massively dilated vein. Next the portal vein lar occlusion. This case report was exempt from was selected and venography was performed, confirming institutional board approval. dominant flow into shunts and a small caliber portal Bombardier et al. CVIR Endovascular (2022) 5:3 Page 3 of 5 Fig. 2 Coronal CT showing two extrahepatic portal shunts (black arrows) and a hypoplastic portal vein (red arrow) vein. The splenic vein, inferior mesenteric vein, and su- occlusion balloon (Edwards LifeSciences, Irvine, Califor- perior mesenteric vein were all evaluated and were pa- nia) was inflated in the larger shunt to measure pres- tent with flow into the two portosystemic shunts rather sures prior to plug deployment. The IVC pressure than the diminutive portal vein (Fig. 3). Next, an measured 16 mmHg, while the portal venous pressure Fig. 3 Splenic and superior mesenteric venography showed preferential flow through the shunts into the IVC without flow visualized into the intrahepatic portal vein Bombardier et al. CVIR Endovascular (2022) 5:3 Page 4 of 5 with occlusion of the shunt measured 18 mmHg prior to measuring 13.1 cm. The main and intrahepatic portal intervention. A 16 mm AVPII Amplatzer plug (Abbot, veins were diminutive, though all appeared patent with Chicago, Illinois) was deployed in the larger portosyste- antegrade flow with a peak velocity of 29 cm/sec in the mic shunt. After selection of the second shunt, an occlu- portal vein. 1 year follow up ammonia level was 97 sion balloon was again inflated, and the portal venous mcmol/L. pressure was found to be 18 mmHg. Next a 12 mm Amplatzer plug was deployed. Following embolization, Discussion/conclusion flow into the hypoplastic portal vein improved with near This case demonstrated the feasibility and safety of treat- complete occlusion of flow into the portosystemic ing two portosystemic shunts with a single staged endo- shunts (Fig. 4). The final portal venous gradient was 2 vascular closure with Amplatzer plugs. The increase in mmHg. liver volume post-procedure is consistent with expected Enoxaparin was administered at a dose of 40 mg daily increase in trophic flow through the liver following clos- for 90 days as prophylaxis against portal venous throm- ure of the two extrahepatic shunts. Previous reports de- bosis. At 3 month follow up, the ammonia level was scribe both single and multi-staged closure, both by 133mcmol/L. Although still elevated, the patient’s family open surgery and by endovascular techniques. Previous and educators both subjectively noted mild improve- experience suggests that a single stage approach may be ments in her behavioral symptoms and described her as safely performed if the portosystemic gradient remains being more affectionate and less confrontational. A fol- < 10 mmHg to avoid complications of acute portal low up CT at 3 months demonstrated complete occlu- hypertension, and the hemodynamics measured in this sion of the two portosystemic shunts, and a portal vein patient allowed for closure of both shunts without sta- diminutive in caliber. The portal vein measured 7 mm in ging. (Rajeswaran et al. 2020) diameter on both pre and post-procedure CT scans. The In conclusion, the management strategy of Type II total volume of the liver was found to be 843 cm3 on Abernethy Syndrome should be to redirect blood flow pre-procedure CT & 1191 cm3 on post-procedure CT into the hypoplastic native portal system, allowing for (Fig. 1d). The patient had no complications at 3 and 6 physiologic hepatic metabolism of splanchnic blood, months of follow up. At 6 months follow up the patient’s hypertrophy of the portal system, and growth of the liver family continued to endorse modest improvement in the from the increased trophic flow. patient’s behavioral symptoms. A 1 year follow up ab- dominal ultrasound showed a normal sized liver Fig. 4 Portal venography following embolization showed increased flow into the intrahepatic portal vein with nearly complete occlusion of the portosystemic shunts Bombardier et al. CVIR Endovascular (2022) 5:3 Page 5 of 5 Abbreviations a case report. World J Clin Cases 7(7):903–907. https://doi.org/10.12998/wjcc. CT: Computed tomography; IVC: Inferior vena cava; F: French; DSA: Digital v7.i7.903[published Online First: Epub Date] subtraction angiography Yoshimatsu R, Takeuchi Y, Morishita H et al (2006) Successful embolisation of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes. Br J Radiol 79(947):e162–e165. Acknowledgements https://doi.org/10.1259/bjr/10360703[published Online First: Epub Date] none. Authors’ contributions Publisher’sNote Brenden Bombardier MD: Manuscript lead author. Adam Alli MD: Manuscript Springer Nature remains neutral with regard to jurisdictional claims in co-author. Aaron Rohr MD: Manuscript co-author. Zachary Collins MD: At- published maps and institutional affiliations. tending interventional radiologist performing the case. Manuscript reviewer. Kavi Raval DO: Fellow interventional radiologist performing the case. Manu- script reviewer. All authors read and approved the final manuscript. Funding These authors did not receive funding for this case report. Availability of data and materials not applicable. Declarations Ethics approval and consent to participate This case report was exempt of institutional board approval at the University of Kansas Medical Center. Consent for publication Consent obtained from patient’s family. Competing interests The authors have no financial or non-financial competing interests to disclose. Received: 19 August 2021 Accepted: 21 December 2021 References Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M (2011) Clinical and Radiologic Manifestations of Congenital Extrahepatic Portosystemic Shunts: a Comprehensive Review. RadioGraphics 31(3):707– 722. https://doi.org/10.1148/rg.313105070[published Online First: Epub Date] Franchi-Abella S, Gonzales E, Ackermann O et al (2018) Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 43(8):2023–2036. https://doi.org/10.1007/s00261-018-1619-8[published Online First: Epub Date] Grimaldi C, Monti L, Falappa P, d'Ambrosio G, Manca A, de Ville de Goyet J (2012) Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 47(2): e27–e31. https://doi.org/10.1016/j.jpedsurg.2011.10.079[published Online First: Epub Date] Loureiro P, Georgiev S, Ewert P et al (2021) Successful percutaneous treatment with the Konar MF-VSD Occluder in an infant with Abernethy syndrome-case report. Cardiovasc Diagn Ther 11(2):631–636. https://doi.org/10.21037/cdt-2 0-380[published Online First: Epub Date] Papamichail M, Pizanias M, Heaton N (2018) Congenital portosystemic venous shunt. Eur J Pediatr 177(3):285–294. https://doi.org/10.1007/s00431-017-3058- x [published Online First: Epub Date] Rajeswaran S, Johnston A, Green J et al (2020) Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts. J Vasc Interv Radiol 31(5):788–794. https://doi.org/10.1016/j.jvir.2019.08. 007[published Online First: Epub Date] Suzuki K, Shimohira M, Hashizume T, Suzuki Y, Shibamoto Y (2013) Dual microcather-dual detachable coil technique in embolization for a congenital intrahepatic portosystemic venous shunt (IPSVS). Minim Invasive Ther Allied Technol 22(5):316–318. https://doi.org/10.3109/13645706.2013. 769006[published Online First: Epub Date] Venkateshwaran S, Krishnamoorthy KM, Sivasankaran S (2014) Percutaneous device closure of Abernethy malformation--a treatable cause of hepatopulmonary syndrome. Catheter Cardiovasc Interv 83(6):968–970. https://doi.org/10.1002/ccd.25275[published Online First: Epub Date] Xiang W, Wang H, Si ZZ, Chen GS, Wang GW, Li T (2019) Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png CVIR Endovascular Springer Journals

A case of two shunts in the endovascular treatment of type II Abernethy syndrome

A case of two shunts in the endovascular treatment of type II Abernethy syndrome

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. Keywords: Abernethy syndrome, Portosystemic shunt, Portal vein, Shunt embolization, Venous malformations Introduction mesenteric vein drain separately into the systemic circu- Abernethy malformation is a rare condition defined by a lation via the inferior vena cava, and type 1b, where the congenital extrahepatic portosystemic shunt. Two types splenic vein and superior mesenteric vein form a com- of Abernethy syndrome exist: Type I malformations have mon trunk before draining into the systemic circulation a congenital absence of the portal vein which leads to via the inferior vena cava. Type II malformations have a complete diversion of portal blood into the systemic cir- hypoplastic portal vein which leads to at least some of culation. Type I malformations can be further classified the portal blood flow diverting into the systemic circula- into type 1a, where the splenic vein and superior tion (Fig. 1). (Papamichail et al. 2018; Alonso-Gamarra et al. 2011). The treatment for Type I Abernethy malformations is * Correspondence: bbombardier@kumc.edu University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas liver transplant. (Papamichail et al. 2018; Alonso- City, KS 66160, USA © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Bombardier et al. CVIR Endovascular (2022) 5:3 Page 2 of 5 Fig. 1 Diagram demonstrating normal hepatic anatomy and hepatic anatomy in Abernethy Syndrome Type Ia, Ib, and II. PV: portal vein, IVC: inferior vena cava, SV: splenic vein, SMV: superior mesenteric vein Gamarra et al. 2011; Xiang et al. 2019) Although there Case report are no consensus treatment guidelines for Type II Aber- A 20-year-old female patient presented with neurocogni- nethy malformations, they are now commonly treated by tive behavioral difficulty, voracious appetite, and chronic endovascular methods. The current literature describes encephalopathy. Despite maximal medical treatment, the closure of extrahepatic portal shunts using a variety of patient had persistently elevated serum ammonia levels, materials including atrial septal occluder devices, VSD with pre-operative levels of 135 and 98 mcmol/L, along occluder devices, Amplatzer vascular plugs, n-butyl with clinical signs and symptoms of encephalopathy. cyanoacrylate, coils, and microcoils. (Papamichail et al. Contrast-enhanced computed tomography (CT) demon- 2018; Venkateshwaran et al. 2014; Rajeswaran et al. strated a hypoplastic intrahepatic portal venous system. 2020; Loureiro et al. 2021; Grimaldi et al. 2012; Suzuki The superior mesenteric vein and splenic vein joined to et al. 2013; Yoshimatsu et al. 2006) Rajeswaran et al. form a short extrahepatic portal vein, with dominant retrospectively studied patients with congenital portosys- flow into two parallel shunts into the suprahepatic infer- temic shunts treated by either operative ligation, endo- ior vena cava (IVC) (Fig. 2). The patient was previously vascular occlusion, combined surgical & endovascular evaluated for candidacy for a liver transplant at a local closure, and observation. They found that at 1 month, children’s hospital but was denied eligibility. Thus, the serum ammonia levels decreased from 82.5 ± 10.3 μmol/ patient and her family agreed to an endovascular pro- L to 38.4 ± 4.6 μmol/L after shunt closure and no differ- cedure to increase blood flow into the intrahepatic por- ence was observed in the decrease between patients tal venous system and decrease blood flow through the treated surgically versus endovascularly. Mean occluded portosystemic shunts. to non-occluded portal pressure gradients were sig- The embolization of the patient’s two extrahepatic nificantly lower for endovascular closure (5.3 + − 1.8 portosystemic shunts was performed via a right internal mmHg) than for surgical closure (12.3 + − 3.3 mmHg). jugular approach. The IVC was selected with an 0.035″ (Rajeswaran et al. 2020) Symptom regression was re- Amplatz wire (Boston Scientific, Marlborough, Massa- ported in most patients following occlusion including chusetts) with the aid of a 5F JB1 catheter (Angiody- improvement in neurologic symptoms and hepatopul- namics, Latham, New York). The catheter was then monary syndrome.(Venkateshwaran et al. 2014;Lour- removed, and a 10F RAABE Flexor sheath (Cook Med- eiro et al. 2021; Grimaldi et al. 2012;Yoshimatsu ical, Bloomington, Indiana) was advanced into the IVC. et al. 2006; Franchi-Abella et al. 2018) Complications An inferior vena cavagram was performed, demonstrat- of closure include reported cases of acute portal ing wide patency. Next, a JB1 catheter and a .035 angled hypertension and/or thrombosis. However, these re- glidewire (Terumo, Tokyo, Japan) were used to select solved with anticoagulation therapy. (Franchi-Abella the right hepatic vein. Digital subtraction angiography et al. 2018) This report describes a case of a type II (DSA) was performed demonstrating a patent right hep- Abernethy malformation with not one, but two extra- atic vein. The left hepatic vein was then selected, dem- hepatic portosystemic shunts, treated with endovascu- onstrating a massively dilated vein. Next the portal vein lar occlusion. This case report was exempt from was selected and venography was performed, confirming institutional board approval. dominant flow into shunts and a small caliber portal Bombardier et al. CVIR Endovascular (2022) 5:3 Page 3 of 5 Fig. 2 Coronal CT showing two extrahepatic portal shunts (black arrows) and a hypoplastic portal vein (red arrow) vein. The splenic vein, inferior mesenteric vein, and su- occlusion balloon (Edwards LifeSciences, Irvine, Califor- perior mesenteric vein were all evaluated and were pa- nia) was inflated in the larger shunt to measure pres- tent with flow into the two portosystemic shunts rather sures prior to plug deployment. The IVC pressure than the diminutive portal vein (Fig. 3). Next, an measured 16 mmHg, while the portal venous pressure Fig. 3 Splenic and superior mesenteric venography showed preferential flow through the shunts into the IVC without flow visualized into the intrahepatic portal vein Bombardier et al. CVIR Endovascular (2022) 5:3 Page 4 of 5 with occlusion of the shunt measured 18 mmHg prior to measuring 13.1 cm. The main and intrahepatic portal intervention. A 16 mm AVPII Amplatzer plug (Abbot, veins were diminutive, though all appeared patent with Chicago, Illinois) was deployed in the larger portosyste- antegrade flow with a peak velocity of 29 cm/sec in the mic shunt. After selection of the second shunt, an occlu- portal vein. 1 year follow up ammonia level was 97 sion balloon was again inflated, and the portal venous mcmol/L. pressure was found to be 18 mmHg. Next a 12 mm Amplatzer plug was deployed. Following embolization, Discussion/conclusion flow into the hypoplastic portal vein improved with near This case demonstrated the feasibility and safety of treat- complete occlusion of flow into the portosystemic ing two portosystemic shunts with a single staged endo- shunts (Fig. 4). The final portal venous gradient was 2 vascular closure with Amplatzer plugs. The increase in mmHg. liver volume post-procedure is consistent with expected Enoxaparin was administered at a dose of 40 mg daily increase in trophic flow through the liver following clos- for 90 days as prophylaxis against portal venous throm- ure of the two extrahepatic shunts. Previous reports de- bosis. At 3 month follow up, the ammonia level was scribe both single and multi-staged closure, both by 133mcmol/L. Although still elevated, the patient’s family open surgery and by endovascular techniques. Previous and educators both subjectively noted mild improve- experience suggests that a single stage approach may be ments in her behavioral symptoms and described her as safely performed if the portosystemic gradient remains being more affectionate and less confrontational. A fol- < 10 mmHg to avoid complications of acute portal low up CT at 3 months demonstrated complete occlu- hypertension, and the hemodynamics measured in this sion of the two portosystemic shunts, and a portal vein patient allowed for closure of both shunts without sta- diminutive in caliber. The portal vein measured 7 mm in ging. (Rajeswaran et al. 2020) diameter on both pre and post-procedure CT scans. The In conclusion, the management strategy of Type II total volume of the liver was found to be 843 cm3 on Abernethy Syndrome should be to redirect blood flow pre-procedure CT & 1191 cm3 on post-procedure CT into the hypoplastic native portal system, allowing for (Fig. 1d). The patient had no complications at 3 and 6 physiologic hepatic metabolism of splanchnic blood, months of follow up. At 6 months follow up the patient’s hypertrophy of the portal system, and growth of the liver family continued to endorse modest improvement in the from the increased trophic flow. patient’s behavioral symptoms. A 1 year follow up ab- dominal ultrasound showed a normal sized liver Fig. 4 Portal venography following embolization showed increased flow into the intrahepatic portal vein with nearly complete occlusion of the portosystemic shunts Bombardier et al. CVIR Endovascular (2022) 5:3 Page 5 of 5 Abbreviations a case report. World J Clin Cases 7(7):903–907. https://doi.org/10.12998/wjcc. CT: Computed tomography; IVC: Inferior vena cava; F: French; DSA: Digital v7.i7.903[published Online First: Epub Date] subtraction angiography Yoshimatsu R, Takeuchi Y, Morishita H et al (2006) Successful embolisation of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes. Br J Radiol 79(947):e162–e165. Acknowledgements https://doi.org/10.1259/bjr/10360703[published Online First: Epub Date] none. Authors’ contributions Publisher’sNote Brenden Bombardier MD: Manuscript lead author. Adam Alli MD: Manuscript Springer Nature remains neutral with regard to jurisdictional claims in co-author. Aaron Rohr MD: Manuscript co-author. Zachary Collins MD: At- published maps and institutional affiliations. tending interventional radiologist performing the case. Manuscript reviewer. Kavi Raval DO: Fellow interventional radiologist performing the case. Manu- script reviewer. All authors read and approved the final manuscript. Funding These authors did not receive funding for this case report. Availability of data and materials not applicable. Declarations Ethics approval and consent to participate This case report was exempt of institutional board approval at the University of Kansas Medical Center. Consent for publication Consent obtained from patient’s family. Competing interests The authors have no financial or non-financial competing interests to disclose. Received: 19 August 2021 Accepted: 21 December 2021 References Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M (2011) Clinical and Radiologic Manifestations of Congenital Extrahepatic Portosystemic Shunts: a Comprehensive Review. RadioGraphics 31(3):707– 722. https://doi.org/10.1148/rg.313105070[published Online First: Epub Date] Franchi-Abella S, Gonzales E, Ackermann O et al (2018) Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 43(8):2023–2036. https://doi.org/10.1007/s00261-018-1619-8[published Online First: Epub Date] Grimaldi C, Monti L, Falappa P, d'Ambrosio G, Manca A, de Ville de Goyet J (2012) Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 47(2): e27–e31. https://doi.org/10.1016/j.jpedsurg.2011.10.079[published Online First: Epub Date] Loureiro P, Georgiev S, Ewert P et al (2021) Successful percutaneous treatment with the Konar MF-VSD Occluder in an infant with Abernethy syndrome-case report. Cardiovasc Diagn Ther 11(2):631–636. https://doi.org/10.21037/cdt-2 0-380[published Online First: Epub Date] Papamichail M, Pizanias M, Heaton N (2018) Congenital portosystemic venous shunt. Eur J Pediatr 177(3):285–294. https://doi.org/10.1007/s00431-017-3058- x [published Online First: Epub Date] Rajeswaran S, Johnston A, Green J et al (2020) Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts. J Vasc Interv Radiol 31(5):788–794. https://doi.org/10.1016/j.jvir.2019.08. 007[published Online First: Epub Date] Suzuki K, Shimohira M, Hashizume T, Suzuki Y, Shibamoto Y (2013) Dual microcather-dual detachable coil technique in embolization for a congenital intrahepatic portosystemic venous shunt (IPSVS). Minim Invasive Ther Allied Technol 22(5):316–318. https://doi.org/10.3109/13645706.2013. 769006[published Online First: Epub Date] Venkateshwaran S, Krishnamoorthy KM, Sivasankaran S (2014) Percutaneous device closure of Abernethy malformation--a treatable cause of hepatopulmonary syndrome. Catheter Cardiovasc Interv 83(6):968–970. https://doi.org/10.1002/ccd.25275[published Online First: Epub Date] Xiang W, Wang H, Si ZZ, Chen GS, Wang GW, Li T (2019) Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation:
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10.1186/s42155-021-00279-7
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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. Keywords: Abernethy syndrome, Portosystemic shunt, Portal vein, Shunt embolization, Venous malformations Introduction mesenteric vein drain separately into the systemic circu- Abernethy malformation is a rare condition defined by a lation via the inferior vena cava, and type 1b, where the congenital extrahepatic portosystemic shunt. Two types splenic vein and superior mesenteric vein form a com- of Abernethy syndrome exist: Type I malformations have mon trunk before draining into the systemic circulation a congenital absence of the portal vein which leads to via the inferior vena cava. Type II malformations have a complete diversion of portal blood into the systemic cir- hypoplastic portal vein which leads to at least some of culation. Type I malformations can be further classified the portal blood flow diverting into the systemic circula- into type 1a, where the splenic vein and superior tion (Fig. 1). (Papamichail et al. 2018; Alonso-Gamarra et al. 2011). The treatment for Type I Abernethy malformations is * Correspondence: bbombardier@kumc.edu University of Kansas Medical Center, 3901 Rainbow Blvd, MS 4032, Kansas liver transplant. (Papamichail et al. 2018; Alonso- City, KS 66160, USA © The Author(s). 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. Bombardier et al. CVIR Endovascular (2022) 5:3 Page 2 of 5 Fig. 1 Diagram demonstrating normal hepatic anatomy and hepatic anatomy in Abernethy Syndrome Type Ia, Ib, and II. PV: portal vein, IVC: inferior vena cava, SV: splenic vein, SMV: superior mesenteric vein Gamarra et al. 2011; Xiang et al. 2019) Although there Case report are no consensus treatment guidelines for Type II Aber- A 20-year-old female patient presented with neurocogni- nethy malformations, they are now commonly treated by tive behavioral difficulty, voracious appetite, and chronic endovascular methods. The current literature describes encephalopathy. Despite maximal medical treatment, the closure of extrahepatic portal shunts using a variety of patient had persistently elevated serum ammonia levels, materials including atrial septal occluder devices, VSD with pre-operative levels of 135 and 98 mcmol/L, along occluder devices, Amplatzer vascular plugs, n-butyl with clinical signs and symptoms of encephalopathy. cyanoacrylate, coils, and microcoils. (Papamichail et al. Contrast-enhanced computed tomography (CT) demon- 2018; Venkateshwaran et al. 2014; Rajeswaran et al. strated a hypoplastic intrahepatic portal venous system. 2020; Loureiro et al. 2021; Grimaldi et al. 2012; Suzuki The superior mesenteric vein and splenic vein joined to et al. 2013; Yoshimatsu et al. 2006) Rajeswaran et al. form a short extrahepatic portal vein, with dominant retrospectively studied patients with congenital portosys- flow into two parallel shunts into the suprahepatic infer- temic shunts treated by either operative ligation, endo- ior vena cava (IVC) (Fig. 2). The patient was previously vascular occlusion, combined surgical & endovascular evaluated for candidacy for a liver transplant at a local closure, and observation. They found that at 1 month, children’s hospital but was denied eligibility. Thus, the serum ammonia levels decreased from 82.5 ± 10.3 μmol/ patient and her family agreed to an endovascular pro- L to 38.4 ± 4.6 μmol/L after shunt closure and no differ- cedure to increase blood flow into the intrahepatic por- ence was observed in the decrease between patients tal venous system and decrease blood flow through the treated surgically versus endovascularly. Mean occluded portosystemic shunts. to non-occluded portal pressure gradients were sig- The embolization of the patient’s two extrahepatic nificantly lower for endovascular closure (5.3 + − 1.8 portosystemic shunts was performed via a right internal mmHg) than for surgical closure (12.3 + − 3.3 mmHg). jugular approach. The IVC was selected with an 0.035″ (Rajeswaran et al. 2020) Symptom regression was re- Amplatz wire (Boston Scientific, Marlborough, Massa- ported in most patients following occlusion including chusetts) with the aid of a 5F JB1 catheter (Angiody- improvement in neurologic symptoms and hepatopul- namics, Latham, New York). The catheter was then monary syndrome.(Venkateshwaran et al. 2014;Lour- removed, and a 10F RAABE Flexor sheath (Cook Med- eiro et al. 2021; Grimaldi et al. 2012;Yoshimatsu ical, Bloomington, Indiana) was advanced into the IVC. et al. 2006; Franchi-Abella et al. 2018) Complications An inferior vena cavagram was performed, demonstrat- of closure include reported cases of acute portal ing wide patency. Next, a JB1 catheter and a .035 angled hypertension and/or thrombosis. However, these re- glidewire (Terumo, Tokyo, Japan) were used to select solved with anticoagulation therapy. (Franchi-Abella the right hepatic vein. Digital subtraction angiography et al. 2018) This report describes a case of a type II (DSA) was performed demonstrating a patent right hep- Abernethy malformation with not one, but two extra- atic vein. The left hepatic vein was then selected, dem- hepatic portosystemic shunts, treated with endovascu- onstrating a massively dilated vein. Next the portal vein lar occlusion. This case report was exempt from was selected and venography was performed, confirming institutional board approval. dominant flow into shunts and a small caliber portal Bombardier et al. CVIR Endovascular (2022) 5:3 Page 3 of 5 Fig. 2 Coronal CT showing two extrahepatic portal shunts (black arrows) and a hypoplastic portal vein (red arrow) vein. The splenic vein, inferior mesenteric vein, and su- occlusion balloon (Edwards LifeSciences, Irvine, Califor- perior mesenteric vein were all evaluated and were pa- nia) was inflated in the larger shunt to measure pres- tent with flow into the two portosystemic shunts rather sures prior to plug deployment. The IVC pressure than the diminutive portal vein (Fig. 3). Next, an measured 16 mmHg, while the portal venous pressure Fig. 3 Splenic and superior mesenteric venography showed preferential flow through the shunts into the IVC without flow visualized into the intrahepatic portal vein Bombardier et al. CVIR Endovascular (2022) 5:3 Page 4 of 5 with occlusion of the shunt measured 18 mmHg prior to measuring 13.1 cm. The main and intrahepatic portal intervention. A 16 mm AVPII Amplatzer plug (Abbot, veins were diminutive, though all appeared patent with Chicago, Illinois) was deployed in the larger portosyste- antegrade flow with a peak velocity of 29 cm/sec in the mic shunt. After selection of the second shunt, an occlu- portal vein. 1 year follow up ammonia level was 97 sion balloon was again inflated, and the portal venous mcmol/L. pressure was found to be 18 mmHg. Next a 12 mm Amplatzer plug was deployed. Following embolization, Discussion/conclusion flow into the hypoplastic portal vein improved with near This case demonstrated the feasibility and safety of treat- complete occlusion of flow into the portosystemic ing two portosystemic shunts with a single staged endo- shunts (Fig. 4). The final portal venous gradient was 2 vascular closure with Amplatzer plugs. The increase in mmHg. liver volume post-procedure is consistent with expected Enoxaparin was administered at a dose of 40 mg daily increase in trophic flow through the liver following clos- for 90 days as prophylaxis against portal venous throm- ure of the two extrahepatic shunts. Previous reports de- bosis. At 3 month follow up, the ammonia level was scribe both single and multi-staged closure, both by 133mcmol/L. Although still elevated, the patient’s family open surgery and by endovascular techniques. Previous and educators both subjectively noted mild improve- experience suggests that a single stage approach may be ments in her behavioral symptoms and described her as safely performed if the portosystemic gradient remains being more affectionate and less confrontational. A fol- < 10 mmHg to avoid complications of acute portal low up CT at 3 months demonstrated complete occlu- hypertension, and the hemodynamics measured in this sion of the two portosystemic shunts, and a portal vein patient allowed for closure of both shunts without sta- diminutive in caliber. The portal vein measured 7 mm in ging. (Rajeswaran et al. 2020) diameter on both pre and post-procedure CT scans. The In conclusion, the management strategy of Type II total volume of the liver was found to be 843 cm3 on Abernethy Syndrome should be to redirect blood flow pre-procedure CT & 1191 cm3 on post-procedure CT into the hypoplastic native portal system, allowing for (Fig. 1d). The patient had no complications at 3 and 6 physiologic hepatic metabolism of splanchnic blood, months of follow up. At 6 months follow up the patient’s hypertrophy of the portal system, and growth of the liver family continued to endorse modest improvement in the from the increased trophic flow. patient’s behavioral symptoms. A 1 year follow up ab- dominal ultrasound showed a normal sized liver Fig. 4 Portal venography following embolization showed increased flow into the intrahepatic portal vein with nearly complete occlusion of the portosystemic shunts Bombardier et al. CVIR Endovascular (2022) 5:3 Page 5 of 5 Abbreviations a case report. World J Clin Cases 7(7):903–907. https://doi.org/10.12998/wjcc. CT: Computed tomography; IVC: Inferior vena cava; F: French; DSA: Digital v7.i7.903[published Online First: Epub Date] subtraction angiography Yoshimatsu R, Takeuchi Y, Morishita H et al (2006) Successful embolisation of intrahepatic portosystemic venous shunt using coils and n-butyl cyanoacrylate through two approach routes. Br J Radiol 79(947):e162–e165. Acknowledgements https://doi.org/10.1259/bjr/10360703[published Online First: Epub Date] none. Authors’ contributions Publisher’sNote Brenden Bombardier MD: Manuscript lead author. Adam Alli MD: Manuscript Springer Nature remains neutral with regard to jurisdictional claims in co-author. Aaron Rohr MD: Manuscript co-author. Zachary Collins MD: At- published maps and institutional affiliations. tending interventional radiologist performing the case. Manuscript reviewer. Kavi Raval DO: Fellow interventional radiologist performing the case. Manu- script reviewer. All authors read and approved the final manuscript. Funding These authors did not receive funding for this case report. Availability of data and materials not applicable. Declarations Ethics approval and consent to participate This case report was exempt of institutional board approval at the University of Kansas Medical Center. Consent for publication Consent obtained from patient’s family. Competing interests The authors have no financial or non-financial competing interests to disclose. Received: 19 August 2021 Accepted: 21 December 2021 References Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M (2011) Clinical and Radiologic Manifestations of Congenital Extrahepatic Portosystemic Shunts: a Comprehensive Review. RadioGraphics 31(3):707– 722. https://doi.org/10.1148/rg.313105070[published Online First: Epub Date] Franchi-Abella S, Gonzales E, Ackermann O et al (2018) Congenital portosystemic shunts: diagnosis and treatment. Abdom Radiol (NY) 43(8):2023–2036. https://doi.org/10.1007/s00261-018-1619-8[published Online First: Epub Date] Grimaldi C, Monti L, Falappa P, d'Ambrosio G, Manca A, de Ville de Goyet J (2012) Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 47(2): e27–e31. https://doi.org/10.1016/j.jpedsurg.2011.10.079[published Online First: Epub Date] Loureiro P, Georgiev S, Ewert P et al (2021) Successful percutaneous treatment with the Konar MF-VSD Occluder in an infant with Abernethy syndrome-case report. Cardiovasc Diagn Ther 11(2):631–636. https://doi.org/10.21037/cdt-2 0-380[published Online First: Epub Date] Papamichail M, Pizanias M, Heaton N (2018) Congenital portosystemic venous shunt. Eur J Pediatr 177(3):285–294. https://doi.org/10.1007/s00431-017-3058- x [published Online First: Epub Date] Rajeswaran S, Johnston A, Green J et al (2020) Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts. J Vasc Interv Radiol 31(5):788–794. https://doi.org/10.1016/j.jvir.2019.08. 007[published Online First: Epub Date] Suzuki K, Shimohira M, Hashizume T, Suzuki Y, Shibamoto Y (2013) Dual microcather-dual detachable coil technique in embolization for a congenital intrahepatic portosystemic venous shunt (IPSVS). Minim Invasive Ther Allied Technol 22(5):316–318. https://doi.org/10.3109/13645706.2013. 769006[published Online First: Epub Date] Venkateshwaran S, Krishnamoorthy KM, Sivasankaran S (2014) Percutaneous device closure of Abernethy malformation--a treatable cause of hepatopulmonary syndrome. Catheter Cardiovasc Interv 83(6):968–970. https://doi.org/10.1002/ccd.25275[published Online First: Epub Date] Xiang W, Wang H, Si ZZ, Chen GS, Wang GW, Li T (2019) Type I congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation:

Journal

CVIR EndovascularSpringer Journals

Published: Jan 5, 2022

Keywords: Abernethy syndrome; Portosystemic shunt; Portal vein; Shunt embolization; Venous malformations

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