Congenital Extrahepatic Portosystemic Shunt Research Articles

Significance of a multidisciplinary approach to congenital extrahepatic portosystemic shunt: A changing paradigm for the treatment.

To review the current institutional practice to treat patients with congenital extrahepatic portosystemic shunt (CEPS) and to determine the optimal strategy. We retrospectively reviewed the records of 55 patients diagnosed with CEPS at our center between December 2008 and March 2022. Among these 55 patients, 44 (80.0%) received treatment for CEPS at a median age of 4.7years. The most common indication for treatment was cardiopulmonary complications (45.5%). Therapeutic intervention included shunt closure by endovascular techniques (50.0%) or surgery (40.9%), and liver transplantation (9.1%). A total of 11 were classified as short shunt types, and surgical ligation was performed in all to preserve the major vascular system and prevent complications (p<0.001). Children who received a surgical ligation were more likely to develop complications after shunt closure (p=0.02). Among seven patients with portopulmonary hypertension (POPH), one patient, who received a shunt ligation at <1year-of-age, was only able to completely discontinue medication. Most other CEPS-related complications were completely resolved. Post-treatment complications, including thrombosis and symptoms of portal hypertension, were seen in 16 patients. After shunt closure, one patient was scheduled to undergo liver transplantation for progressive POPH and large residual hepatocellular adenoma. During follow-up, one patient without any treatment for CEPS developed POPH 16years from the diagnosis. Earlier therapeutic interventions should be strongly considered for patients with POPH related to CEPS. However, in view of the invasiveness and treatment complications, special attention should be paid to the management of patients with short shunt types.

Congenital porto-pulmonary shunt in a dog

Congenital extra-hepatic porto-systemic shunts (CEPS) are a non-rare vascular anomaly observed in dogs, most commonly in small and toy pure breeds. Computed tomographic angiography (CTA) examination is considered the gold standard imaging modality for the diagnosis of anomalous vascular connections. An anomalous congenital porto-pulmonary shunt was incidentally diagnosed in a 5-year-old French Bulldog. The anomalous vessel originated from the ventral aspect of the portal vein and went cranially towards the esophageal hiatus, entering the lobar vein of the caudal left pulmonary lobe. The dog did not show any significant clinical or computed tomography angiography-perceived hepatic abnormalities and no signs of portal hypertension were evidenced. No case of porto-pulmonary shunt in veterinary medicine have ever been reported, while in humans it was rarely described secondarily to portal hypertension, severe hepatopathies or complex cardiac malformations. CTA must be considered the best imaging modality for the diagnosis also of unusual CEPS and in the author's opinion the congenital porto-pulmonary shunt described in the patient was of little or no clinical relevance.

Abernethy Syndrome Type 1B in a Young Male presenting with acute gastrointestinal bleeding: A Case report

Abernethy syndrome is a rare congenital extrahepatic portosystemic shunt (CEPS) in which splanchnic blood flows straightly into the inferior vena cava (IVC). Compared to type 1, in which the intra hepatic portal tree is not developed, in type 2 the portal vein is intact, but forms an anastomosis with the IVC. In the subtype 1a, superior mesenteric and splenic veins do not merge into the portal vein, while in type 1b they anatomically form the portal vein. CEPS can cause a wide spectrum of clinical manifestations including hepatic, pulmonary, metabolic and neurological symptoms and is usually diagnosed before adulthood. The diagnosis is established using the radiological imaging methods. Whileliver transplantation is the treatment of choice for type 1, surgical or minimally invasive endovascular shunt closure are treatment options for type 2 Abernethy syndome.

Attenuation of right gastrocaval extrahepatic shunt with cellophane band in a dog: Case report

Congenital extrahepatic portosystemic shunt is a vascular anomaly that consists in the communication between the portal and systemic circulation, causing substances normally metabolized and excreted by the liver to be diverted directly to the systemic circulation and triggering changes in neurological, gastrointestinal and urinary systems. Diagnosis is through imaging tests, such as ultrasound and computed tomography. For patient stabilization, initial clinical treatment is highly indicated, especially for those who manifest signs of hepatic encephalopathy, and the definitive treatment is gradual surgical attenuation. Currently, the most indicated techniques for shunt occlusion are the ameroid constrictor ring and the cellophane band, which have good long-term results. The prognosis of patients undergoing the surgical procedure varies according to the general condition of the patient at the time of diagnosis and the complications that may occur in the trans and postoperative periods, such as portal hypertension, seizures, hypoglycemia and failure to close the shunt. This report aims to describe a case of surgical attenuation of a right extrahepatic gastrocaval shunt using the cellophane band technique. The canine patient, pug, aged eight months, was treated at the Veterinary Hospital of the Federal University of Paraná, presenting neurological clinical signs and a previous diagnosis of portosystemic shunt. To determine the origin of the anomalous vessel and optimize surgical planning, computed angiotomography was indicated. Clinical treatment was instituted and, after 15 days, surgical attenuation of the shunt was performed using the cellophane band fixed with titanium clips. The patient showed signs of portal hypertension, ascites and postoperative hypoglycemia, which were treated during hospitalization. After 15 days of hospital discharge, the patient returned to the Veterinary Hospital with good general condition, absence of ascites and neurological signs and no significant changes were observed in laboratory tests, demonstrating the effectiveness of the chosen surgical technique.

Shear wave elastography measurements in dogs treated surgically for congenital extrahepatic portosystemic shunts.

Assessing the postoperative surgical success of congenital extrahepatic portosystemic shunt (EHPSS) attenuation can be challenging and involve invasive imaging methods. Elastography is an ultrasound technique that allows qualitative and quantitative estimation of tissue stiffness and has extensively been used in people with liver disease. In recent years, increased interest in this technique has developed in veterinary medicine due to its non-invasive nature, availability, and low cost. The objective of this study was to compare liver stiffness values between dogs with closed EHPSS and those with multiple acquired portosystemic shunts (MAPSS) after gradual surgical attenuation and to assess whether shear wave elastography could be used to determine EHPSS closure. As a secondary objective, measurements obtained from both intercostal and subxiphoidal views were compared. Mean values for the average, median, and maximum two-dimensional shear wave velocities (2D SWV) for the closed EHPSS were 2.88 +/−0.11 m/s; 2.83 +/−0.11 m/s; and 3.75 +/−0.16 m/s, respectively. In the MAPSS dogs, mean values for the average, median, and maximum 2D SWV were 2.77 +/– 0.17 m/s; 2.71 +/– 0.17 m/s; and 3.66 +/−0.24 m/s, respectively. No significant differences in 2D SWV were present between dogs with closed EHPSS and those with MAPSS (P = 0.33; P = 0.33; P = 0.42, respectively). When assessing potential differences between intercostal and subxiphoidal 2D SWV measurements, no effect was observed for the average and median 2D SWV (P = 0.06; P = 0.07, respectively). Yet, a significant difference was identified for the maximum 2D SWV between intercostal 4.00 +/−0.20 m/s and subxiphoidal 3.41 +/−0.17 m/s measurements (P = 0.02). The relevance of this finding is uncertain as many other studies about liver elastography only report mean and not maximum values.

Short- and long-term outcomes of polyethylene band attenuation of congenital extrahepatic portosystemic shunts in dogs: 60 cases (2010-2020).

To report the short- and long-term outcomes following attenuation of congenital extrahepatic portosystemic shunts in dogs using a novel polyethylene band. Records were retrospectively reviewed for dogs that underwent congenital extrahepatic portosystemic shunt attenuation by a polyethylene banding technique, at a single institution between 2010 and 2020. Short-term outcome data were collected from peri-operative clinical records with follow-up examinations, scheduled at 6 and 18 weeks post-operatively, and post-operative imaging when performed. Long-term follow-up was collected by validated owner questionnaire, telephone interview or medical records. Long-term outcomes were categorised by "excellent", "good" or "poor". Sixty dogs were included. Post-operative complications occurred in 10 of 60 dogs (16.7%), four major and six minor, with a peri-operative mortality of 6.7%. Persistent shunting was identified in nine of 53 dogs (17%) available for follow-up examination and four dogs underwent a revision surgery. Long-term follow-up was available for 44 dogs at a median of 75 months post-operatively (range 7 to 128). Long-term outcomes were "excellent" (26) or "good" (8) in 81.8% of dogs and "poor" (8) in 18.2%. At the time of follow-up, 30 of 44 (68.2%) dogs were not receiving any medical treatment and 27 of 28 (96.4%) questionnaire respondents were satisfied with the response to surgery. Polyethylene band attenuation of congenital extrahepatic portosystemic shunts provides comparable outcomes to cellophane. The material used in this study is widely available and consistent while being pre-sterilised and pre-folded makes it easy to use.

Comparison of diet, lactulose, and metronidazole combinations in the control of pre-surgical clinical signs in dogs with congenital extrahepatic portosystemic shunts.

BackgroundHepatic supportive diet (HSD), lactulose, and antimicrobials are medical treatments for dogs with congenital extrahepatic portosystemic shunts (cEHPSS). The relative contribution of these treatment components is currently unknown.ObjectivesTo determine which treatment combinations are most efficacious in pre‐surgical control of clinical signs of cEHPSS in dogs.AnimalsThirty‐six dogs with untreated cEHPSS.MethodsThree‐arm randomized clinical trial. At inclusion (T0), dogs were divided into 3 groups: HSD (n = 12), HSD + lactulose (n = 12), or HSD + metronidazole (n = 12) and received the randomized treatment for 4 weeks (T1) followed by combined treatment of HSD + lactulose + metronidazole for 2 weeks or until cEHPSS attenuation (T2). Clinical score as well as fasting ammonia (FA) and C‐reactive protein (CRP) concentrations were compared among groups and time points.ResultsThirty‐four dogs were evaluated. Thirty‐four dogs reached T1 and 29 dogs T2. At T1, clinical scores decreased in the HSD + lactulose (n = 11; P = .001), but not in the HSD (n = 8; P = .96) and HSD + metronidazole (n = 10; P = .06) groups. Adding metronidazole to HSD + lactulose (n = 11) did not result in further clinical score improvement (T2; P = 1.000). Moderate and weak correlation between clinical score and FA and clinical score and CRP was present (ρ = .35, P < .001; ρ = .27, P = .01, respectively) with FA decreasing over time on medical treatment (P = .001).Conclusions and Clinical ImportanceCombined HSD + lactulose seems sufficient for pre‐surgical cEHPSS stabilization unlike sole HSD or HSD + metronidazole. Medical treatment of cEHPSS clinical signs decreases FA.

Presentation, Management, and Outcome of Congenital Portosystemic Shunts in Children: The Boston Children's Hospital Experience.

Congenital portosystemic shunts (CPSS) are rare vascular malformations. We describe presentations, complications, associations, and outcomes of CPSS at Boston Children's Hospital (BCH). This was a retrospective review of children with CPSS at BCH from 2000 to 2020. Twenty-nine patients had CPSS (17 girls): 14 extrahepatic (EH) and 15 intrahepatic (IH). At diagnosis, 15 were ≤5 days, 7 <1 year, and 7 >1 year (range 1-19). Median follow-up duration was 5.2 years (interquartile range [IQR] 1.6-10.9) in EH and 2.2 years (0.2-4.2) in IH CPSS. The most common presentation was antenatal ultrasound 13 (45%) followed by hyperammonemia 10 (34%), whereas 6 (21%) were asymptomatic. Complications were noted in 17 (12/14 EH vs 6/15 IH, P = 0.008). Associated anomalies were present in 25 (14/14 EH vs 11/15 IH, P = 0.10). Spontaneous closure was observed in 8 (28%) patients with IH CPSS, all <12 months of age. Ten patients underwent shunt closure 3 (30%) by interventional radiology (IR) and 5 (50%) by surgery, whereas 2 (20%) required both. After therapeutic closure; 8 had improvement, 1 had portal hypertension, and 1 had sepsis and thrombosis. The remaining 11 patients, 8 (42%) were followed without closure: 6 of 8 (75%) EH versus 2 of 11 (18%) IH ( P = 0.02), 2 lost follow-up and 1 with complicated EH CPSS died, unsuitable for therapeutic closure. CPSS may be asymptomatic or present with complications. Spontaneous closure of IH shunts may occur in infancy, thus therapeutic closure may be deferred until age ≥ 2 years. IR and surgical closure of CPSS are associated with improvement in the majority of cases.

Surgical shunt ligation for a congenital extrahepatic portosystemic shunt with pulmonary hypertension: A case report.

Introduction and importanceCongenital extrahepatic portosystemic shunt (CEPS) presents with various symptoms due to abnormal communication between the portal venous system and inferior vena cava. And Klippel-Trenaunay-Weber syndrome is another rare congenital disorder characterized by vascular malformations.Case presentationA 16-year-old male with Klippel-Trenaunay-Weber syndrome was referred to our hospital for surgical treatment of pulmonary hypertension due to CEPS since childhood. Dyspnea had developed about two years before presentation at our hospital and gradually worsened. Right heart catheterization had revealed pulmonary hypertension and a high cardiac output state associated with a portosystemic shunt. Although pulmonary vasodilators improved the dyspnea, pulmonary hypertension remained. The patient was then referred to our hospital for surgical shunt occlusion. The results of the preoperative and intraoperative shunt occlusion tests were within acceptable limits. Therefore, primary shunt ligation was performed. There were no postoperative complications. Continuous intravenous vasodilator was tapered off four months after discharge. No additional or increased doses of medications were required for four years after surgery.Clinical discussionSurgical shunt ligation for CEPS is effective but can cause acute portal hypertension. Primary shunt ligation could be performed without complications according to the criteria for safe shunt occlusion.ConclusionSurgical portosystemic shunt ligation could be performed safely and ameliorated pulmonary hypertension. Patients with CEPS, especially young patients, should be referred to a tertiary center while asymptomatic, and early therapeutic intervention is necessary.

Dogs ≥ five years of age at the time of congenital extrahepatic portosystemic shunt diagnosis have better long-term outcomes with surgical attenuation than with medical management alone.

To determine the outcome in dogs diagnosed with congenital extrahepatic portosystemic shunts (EHPSS) at ≥ 5 years of age treated with medical management only (M) or with surgical attenuation (S). The hypothesis was that dogs undergoing surgical attenuation would have a longer survival time than dogs undergoing medical management only. 351 dogs definitively diagnosed with EHPSS at ≥ 5 years of age. Medical records from 2009 to 2019 at 16 veterinary teaching hospitals were evaluated. Data collected included signalment, clinical signs at diagnosis, clinicopathologic data, surgical and medical treatments, shunt morphology, clinical signs and medical treatments at 6 to 12 months after diagnosis, and survival time. 351 dogs (M, 119 [33.9%]; S, 232 [66.1%]) were included in the study. Survival time was longer with surgery than medical management (hazard ratio, 4.2; M, 3.4 years; S, 10.9 years). Continued clinical signs at 6 to 12 months after diagnosis were more common with medical management (M, 40% [33/88]; S, 14% [21/155]). Continued medical treatments at 6 to 12 months after diagnosis were more common in the medical management group (M, 78% [69/88]; S, 34% [53/155]). Perioperative mortality rate was 7.3%. Dogs diagnosed at ≥ 5 years of age with EHPSS have significantly better survival times and fewer clinical signs with surgical attenuation, compared with medical management. Older dogs have similar surgical mortality rates to dogs of all ages after surgical EHPSS attenuation.

Use of an ameroid constrictor ring for gradual occlusion in canine congenital extrahepatic portosystemic shunts: Review

Congenital extrahepatic portosystemic shunts may be caused by embryological failure and are related to normal abdominal vascular development. They are noteworthy due to the higher prevalence of portosystemic vascular shunts and small to miniature breeds are the most affected. In this literature review, we carefully compiled in international articles in the field on the use of the ameroid constrictor ring in surgery for the gradual occlusion of unique congenital extrahepatic portosystemic shunts in dogs. In this sense, aspects of pathophysiology, etiopathogenesis, clinical symptoms and technical aspects regarding the surgical area were addressed. It is concluded that the use of the constrictor ring becomes a safe and highly recommended method, resulting in low rates of morbidity and mortality and prolonged postoperative survival in dogs.

Status of congenital extrahepatic portosystemic shunt.

Status of congenital extrahepatic portosystemic shunt.

Laparoscopic portosystemic shunt attenuation in 20 dogs (2018-2021).

To describe the technique, complications, and outcome of laparoscopic portosystemic shunt attenuation (LPSSA) in dogs. Retrospective study. Twenty client-owned dogs. Medical records were searched for dogs with a single congenital extrahepatic portosystemic shunt (CEPSS) that was treated with LPSSA. Signalment, clinical signs, CEPSS location, diagnostic imaging, laparoscopic approach, operative technique, complications, and clinical outcome were reviewed. Fourteen dogs with CEPSS located in the epiploic foramen had a right (13/14) or left (1/14) paramedian approach. In 6 dogs a CEPSS was not located in the epiploic foramen, and a left paramedian approach was used. A 3 or 4-port technique was used in 7 and 13 dogs, respectively. A thin film band was used for CEPSS attenuation in all dogs. The median operating time for LPSSA was 62 min (range 27-98 min). Intraoperative complications requiring conversion to an open technique occurred in 5 dogs. Mild perioperative self-limiting portal hypertension occurred in 3 dogs, while severe portal hypertension with surgical revision occurred in 1 case. The complications were resolved, and all dogs had a good outcome. Laparoscopic portosystemic shunt attenuation can be performed in dogs, in particular for a CEPSS located in the epiploic foramen using a right paramedian approach. For CEPSS not located in the epiploic foramen, a left paramedian approach is recommended. Conversion to open celiotomy was required in around a third of cases. Laparoscopic attenuation of CEPSSs can be performed in dogs and has a good clinical outcome, particularly for CEPSS located in the epiploic foramen.

Presentation of Congenital Portosystemic Shunts in Children.

Background: Congenital portosystemic shunts (CPSS) are rare vascular anomalies resulting in communications between the portal venous system and the systemic venous circulation, affecting an estimated 30,000 to 50,000 live births. CPSS can present at any age as a multi-system disease of variable severity mimicking both common and rare pediatric conditions. Case presentations: Case A: A vascular malformation was identified in the liver of a 10-year-old girl with tall stature, advanced somatic maturation, insulin resistance with hyperinsulinemia, hyperandrogenemia and transient hematuria. Work-up also suggested elevated pulmonary pressures. Case B: A young girl with trisomy 8 mosaicism with a history of neonatal hypoglycemia, transient neonatal cholestasis and tall stature presented newly increased aminotransferase levels at 6 years of age. Case C: A 3-year-old boy with speech delay, tall stature and abdominal pain underwent abdominal ultrasound (US) showing multiple liver nodules, diagnosed as liver hemangiomas by hepatic magnetic resonance imaging (MRI). Management and outcome: After identification of a venous malformation on liver Doppler US, all three patients were referred to a specialized liver center for further work-up within 12 to 18 months from diagnosis. Angio-computed tomography (CT) scan confirmed the presence of either an intrahepatic or extrahepatic CPSS with multiples liver nodules. All three had a hyperintense signal in the globus pallidus on T1 weighted cerebral MRI. Right heart catheterization confirmed pulmonary hypertension in cases A and C. Shunts were closed either using an endovascular or surgical approach. Liver nodules were either surgically removed if there was a risk of malignant degeneration or closely monitored by serial imaging when benign. Conclusion: These cases illustrate most of the common chief complaints and manifestations of CPSS. Liver Doppler US is the key to diagnosis. Considering portosystemic shunts in the diagnostic work-up of a patient with unexplained endocrine, liver, gastro-intestinal, cardiovascular, hematological, renal or neurocognitive disorder is important as prompt referral to a specialized center may significantly impact patient outcome.

Does surgical or medical management of extrahepatic portosystemic shunts in dogs carry a better prognosis for the resolution and reduction of neurological dysfunction?

PICO question&#x0D; In dogs with congenital extrahepatic portosystemic shunts that are treated with surgical attenuation what is the persistency, frequency, severity and outcome of neurological signs when compared to dogs that are treated medically?&#x0D; &#x0D; Clinical bottom line&#x0D; Category of research question&#x0D; Prognosis&#x0D; The number and type of study designs reviewed&#x0D; Ten papers were critically reviewed&#x0D; Strength of evidence&#x0D; Weak&#x0D; Outcomes reported&#x0D; For short-term success, owners reported an overall decrease in neurological signs associated with neurological dysfunction and an increase in quality of life after the initiation of either medical management or surgical management. Surgical management has a weak association with higher mortality or severe neurological signs in the immediate postoperative period&#x0D; Conclusion&#x0D; It is challenging to make a direct comparison between medical and surgical management but, overall, both strategies seemed to decrease neurological signs in the short term. There was a lack of evidence and available data about the efficacy of each for long-term control of neurological signs&#x0D; &#x0D; How to apply this evidence in practice&#x0D; The application of evidence into practice should take into account multiple factors, not limited to: individual clinical expertise, patient’s circumstances and owners’ values, country, location or clinic where you work, the individual case in front of you, the availability of therapies and resources.&#x0D; Knowledge Summaries are a resource to help reinforce or inform decision making. They do not override the responsibility or judgement of the practitioner to do what is best for the animal in their care.&#x0D; &#x0D;

Type II Abernethy Malformation: A Rare Cause of Hepatic Encephalopathy in Adulthood.

Abernethy malformation is a rare congenital extrahepatic portosystemic shunt with variable clinical manifestations, mainly associated with the portosystemic shunt. Hepatic encephalopathy can be the first manifestation.We present the case of a 50-year-old woman with hepatic encephalopathy without liver dysfunction. Further evaluation with contrast-enhanced abdominal computed tomography was performed, showing a large-calibre portocaval shunt from the left portal branch, suggestive of type 2 Abernethy malformation. The patient improved with lactulose and rifaximin but maintained rare encephalopathy episodes. She is therefore currently awaiting surgical treatment.Despite being a rare cause of hepatic encephalopathy, Abernethy malformation should be considered in patients with unexplained hyperammonaemia. Since it is potentially reversible, and early diagnosis and treatment may improve outcome, raising awareness of this malformation is essential.LEARNING POINTSAbernethy malformation is a rare congenital extrahepatic portosystemic shunt with variable clinical manifestations, some associated with the portosystemic shunt; hepatic encephalopathy can be the first manifestation.Initial recognition of the presence of encephalopathy in non-cirrhotic individuals can be challenging, and patients may have symptoms for a long period of time before being diagnosed.This entity, although rare, should be looked for in patients with hepatic encephalopathy without evidence of liver disease, mainly because it can be submitted to targeted treatment.

Persistent hypercobalaminemia three months after successful gradual attenuation of extrahepatic shunts in dogs: a prospective cohort study

BackgroundDeficiencies in vitamin A and D and disorders in the vitamin B complex are often present in people with chronic liver diseases. So far, the serum concentrations of these vitamins have not yet been studied in dogs with congenital extrahepatic portosystemic shunts (EHPSS), who also have some degree of liver dysfunction. The objective was to assess serum vitamin concentrations in dogs with EHPSS from diagnosis to complete closure. A prospective cohort study was performed using ten client-owned dogs with EHPSS, closed after gradual surgical attenuation. Serum concentrations of vitamin A, 25-hydroxyvitamin D, folic acid, cobalamin and methylmalonic acid (MMA) were measured at diagnosis prior to institution of medical therapy, prior to surgery, and three months after gradual attenuation and complete closure of the EHPSS.ResultsAt diagnosis, median serum concentrations of vitamin A, 25-hydroxyvitamin D and folic acid were 18.2 μg/dL (8.8 - 79.5 μg/dL), 51.8 ng/mL (19.4 - 109.0 ng/mL), and 8.1 μg/L (5.2 - 14.5 μg/L), respectively, which increased significantly postoperatively (88.3 μg/dL (51.6 - 182.2 μg/dL, P=0.005), 89.6 ng/mL (49.3 - >150.0 ng/mL, P =0.005), and 14.8 μg/L (11.5 - 17.7 μg/L, P <0.001), respectively). Median serum cobalamin concentrations were 735.5 ng/L (470 - 1388 ng/L) at diagnosis and did not significantly decrease postoperatively (P =0.122). Both at diagnosis and three months postoperatively 7/10 dogs had hypercobalaminemia.ConclusionsSerum concentrations of vitamin A, 25-hydroxyvitamin D and folic acid significantly increase after surgical attenuation. Nevertheless, persistent hypercobalaminemia is suggestive of ongoing liver dysfunction, despite successful surgery.

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

BackgroundAbernethy 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 presentationWe 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.ConclusionsThis 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.

Pulmonary arterial hypertension secondary to congenital extrahepatic portosystemic shunt - a serious complication of a rare disease.

Pulmonary arterial hypertension secondary to congenital extrahepatic portosystemic shunt - a serious complication of a rare disease.

Multistage closure of a congenital extrahepatic portosystemic shunt

BackgroundCongenital extrahepatic portosystemic shunts (CEPS) are rare shunts connecting the extrahepatic portal system with the inferior vena cava. Shunt dimensions and the risk of portal hypertension determines the closure strategy. Endovascular treatment is indicated for single stage occlusion of longer length shunts, whereas the remaining shunt types are preferentially surgically occluded. Herein we describe the technical details of a novel endovascular treatment for short length CEPS.Case presentationA 15-years-old male with a short length CEPS complicated with multinodular liver disease was submitted to a multistage closure, as indicated by the high portal pressure values during shunt balloon occlusion venography. Initially a transjugular intrahepatic portosystemic shunt (TIPS) was created and the CEPS occluded with an atrial septal defect occluder. In a second procedure the TIPS was embolized with a flow reductor stent and an amplatzer vascular plug II. At a 1 year follow up the liver nodules size reduced, the patient remains asymptomatic, and the shunt adequately closed.ConclusionThis paper outlines the potential use of a TIPS and an atrial septal defect occluder combination in complex CEPS, supporting its usage as an alternative to the standard surgical treatment.Level of Evidence: Level 4, Case report.