Congenital Portosystemic Shunt Research Articles

2215 An Interesting Case of Intrahepatic Porto-Hepatic Shunt as a Cause of Unexplained Encephalopathy

INTRODUCTION: Congenital portosystemic shunts or Abernethy malformations are rare anatomical abnormalities shunting blood from the portal venous system to the systemic venous system, thereby bypassing passage through the hepatic acinus. The presentation may range from incidental finding on imaging to hepatic encephalopathy and liver failure depending on the degree and type of shunt. Here, we describe an interesting case of unexplained encephalopathy in a patient without liver cirrhosis found to have congenital intrahepatic Type II Abernethy malformation. CASE DESCRIPTION/METHODS: A 73-year-old female with a past medical history of Rheumatoid arthritis and Gilbert syndrome was admitted to hospital with clinical evidence of encephalopathy. Vitals were stable and physical examination was unremarkable except for encephalopathy. Laboratory investigations were unremarkable except for elevated ammonia levels (165 µmol/L). Computed tomography (CT) of the brain was unremarkable. Abdominal ultrasonography revealed the normal size and echotexture of the liver. Abdominal CT angiography showed the presence of dilated portal vein measuring up to 1.8 cm at the porta-hepatis along with dilated superior mesenteric and splenic veins. Multiple dilated vascular channels were seen within the right hepatic lobe. An intrahepatic portosystemic shunt between an enlarged middle hepatic vein and 2 separate branches of the right portal vein was visualized. Pressure gradients were measured and were normal. She received conservative management with lactulose and rifaximin and discharged on day 6 after the resolution of encephalopathy. DISCUSSION: Encephalopathy associated with portal-systemic bypass and no intrinsic hepatocellular disease is often referred to as Type B hepatic encephalopathy as was in our patient. Ammonia which is the culprit for the neuropsychiatric manifestation is usually metabolized by the liver in normal conditions, however in cases of portosystemic shunting, whether congenital or not, ammonia bypasses the liver and enters the systemic circulation and directly reaches the brain. The degree of symptomatology associated with these venous shunts depends on the shunt ratio and patient age. Treatment options include medical management including use of lactulose and non-absorbable antibiotics or endovascular shunt closure. Our patient was managed medically with the plan to refer her for shunt closure if she continues to have recurrent episodes.

A case of 23-year-old man with hepatocellular carcinoma on the background of a congenital portosystemic shunt

A case of 23-year-old man with hepatocellular carcinoma on the background of a congenital portosystemic shunt

Outcomes of 50 Cases of Pediatric Liver Transplantation for Intrapulmonary Shunt While Considering the Deteriorated Prognosis of Severe Intrapulmonary Shunt Patients

BackgroundIntrapulmonary shunt (IPS) is recognized in 10% of chronic liver disease patients. Liver transplantation (LT) is associated with a high risk of morbidity and mortality in patients with IPS. Patients and methodsOf 519 pediatric LT cases between November 2005 and October 2018, 50 patients with IPS were enrolled in this study. The patients were divided into 3 groups, according to the shunt ratio, calculated by scintigraphy: mild (15%-20%, n = 26), moderate (20%–40%, n = 19), and severe (> 40%, n = 5). We compared the patients’ characteristics before LT and the outcomes of LT between these groups. ResultsThe major original disease resulting in LT in the mild and moderate groups was biliary atresia (73.1% and 52.6%, respectively), while that in the severe group was congenital portosystemic shunt (60%). The median ages at LT were 7.5, 6.1, and 8.3 years in the mild, moderate, and severe groups, respectively. All of the mild and moderate IPS patients lived; however, 3 patients with severe IPS (60.0%) died within 3 months. The shunt ratios of the mild and moderate IPS patients normalized within 2 years after LT, while the 2 surviving severe IPS patients showed a slight improvement. The autopsy findings of the lung in 1 deceased severe IPS patient showed medial hypertrophy and proliferation of intimal cells of the pulmonary arteries, suggesting a diagnosis of portopulmonary hypertension. ConclusionsLT can be safely performed for mild and moderate IPS patients; however, LT for severe IPS patients should be carefully indicated because concomitant portopulmonary hypertension may be masked by IPS.

Abernethy Malformation – a Rare but Important Diagnosis Prior to Liver-Directed Therapy for Hepatocellular Carcinoma

A 45-year-old man with incidentally discovered, unresectable HCC were treated with TACE to the left hepatic lobe and TARE to the right hepatic lobe. Upon retrospective review, he was found to have a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy malformation). This case report discusses variant splanchnic and portal anatomy in the setting of rare, congenital portosystemic shunts and evaluates types of liver-directed therapies for HCC in this setting.

Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.

Congenital portosystemic shunt (CPSS) is a rare malformation in which splanchnic venous flow bypasses the liver. CPSS is associated with other congenital anomalies and syndromes and can be associated with life-threatening complications. CPSS and their management remain underreported in the literature. Here, we review the clinical characteristics, management, and outcomes of a cohort of children and young adults with CPSS from two pediatric centers. Cases of CPSS from Cincinnati Children's Hospital Medical Center and C.S. Mott Children's Hospital were reviewed to define CPSS anatomy, associated anomalies, complications, interventions, and outcomes. The imaging features and histopathology of liver lesions were characterized in detail. A total of 11 cases were identified. Median age was 10years (range 0-26); 8 (73%) cases were female. Associated anomalies included six patients with heterotaxy (55%), five patients with congenital heart disease (45%), three patients with Turner syndrome (27%), and two patients with omphalocele, exstrophy, imperforate anus, spinal defects (OEIS) complex (18%). Eight (73%) cases had hyperammonemia ± encephalopathy. A 4-month-old presented with hepatopulmonary syndrome, and 12-year-old presented with pulmonary hypertension. Eight patients (73%) had liver lesions including five with premalignant adenomas and three with well-differentiated hepatocellular carcinoma (HCC). Four children underwent successful CPSS occlusion/ligation. Three children underwent liver transplant (2) or resection (1) for HCC without recurrence at extended follow-up. CPSS is associated with multiple anomalies (heterotaxy, congenital heart disease) and syndromes (Turner syndrome). CPSS liver lesions should be very carefully evaluated due to risk of premalignant adenomas and HCC. Serious complications of CPSS can occur at a young age but can be managed endovascularly or with open surgery.

Congenital portosystemic shunt in dogs: review

Portosystemic shunt is a condition commonly seen in dogs. This disease is a vascular anomaly that makes communication between the systemic circulation and the portal circulation. Because of this anomalous vascular communication, portal blood from the drainage of organs such as the stomach, intestine, pancreas and spleen, passes directly into the systemic circulation, diverting from the liver that is responsible for detoxification of various substances. Therefore, the patient will manifest numerous clinical signs with possible neurological or systemic involvement only due to the accumulation of toxins in the blood. The treatment of choice for the portosystemic shunt is the ligation of the anomalous vessel, to which the ameroid constricting ring will be the most approached in this article.

P: 62 Neurometabolism in Grey Matter of Children With Chronic Liver Disease or Portosystemic Shunting: A 1H-MRS Study at 7T

BACKGROUND: Neurocognitive deficits in children with chronic liver disease (CLD) or congenital porto-systemic shunts (CPSS) are incompletely understood. Understanding the molecular underpinnings by non-invasive means could inform management. Aims To characterize the neurometabolic profile in the grey matter (GM) of children with CLD or CPSS and analyze correlations with neurocognitive and biological results. METHODS: Children aged 8–14 were enrolled if they presented with CLD or CPSS. Short-echo-time (16 ms) using 1H-MRS at 7T in GM dominated medial prefrontal cortex was performed, and neurocognitive testing and routine labs were obtained within 3 months of each other following informed consent. RESULTS: 5 patients (8–14 y) including 4 with CLD (2 girls), 1 with CPSS (1 girl), and 4 controls (10–14 y, 2 girls) underwent 1H-MRS. Causes of CLD: congenital disorder of glycosylation (1), progressive familial intrahepatic cholestasis type-2 (1), portal obliterative venopathy (1), autoimmune hepatitis (1). Mean plasma ammonium in patients was 26 umol/l, mean serum bilirubin was in normal range and mean platelet count was 201 G/L (59–346). The 3 patients with CLD showed scores in average or above average on Total Intellectual Quotient measures (WISC-IV). One of the 3 scored below average on the working memory sub-scale of the WISC-IV (<1, 65 SD), while the intellectual profiles were homogenous and above average for the 2 others patients. One of these 2 scored below average on 6/10 parameters on the Conners Continuous Performance Test, suggesting attention deficit. The other two were in range. The patient with CPSS displayed Total Intellectual Quotient below average (<1, 65 SD on the WISC-IV), with additional deficits (<1, 65 SD) in executive and attentional functioning as well as expressive and receptive language. 1H-MRS results: 13 metabolites were reliably quantified. Figure 1 illustrates the differences between CLD and CPSS: the expected increase of brain glutamine and decrease of brain osmolytes (inositol, taurine, total choline) together with a previously unreported decrease in the neurotransmitters glutamate, GABA and N-acetylaspartate. No statistically significant differences were observed between the CLD patients and controls. CONCLUSIONS: In patients with compensated CLD, there were no significant neurometabolic alterations as assessed by high resolution 1H-MRS. In CPSS, however, neurometabolic changes were clear, and likely related to measurably impaired neurocognitive functioning. Together, these results suggest that in CPSS (type B encephalopathy) the brain is likely exposed to a higher load of neurotoxic substances than in patients who have some degree of portal flow (type C).

Hypernatraemia following surgery for a congenital extrahepatic portosystemic shunt in a Shih Tzu presenting with marked polyuria and polydipsia

A 3-year-old male Shih Tzu was referred to the University Veterinary Hospital of the University College Dublin with a suspicion of congenital hepatopathy based on investigations performed by the referring...

Treatment of congenital extrahepatic portosystemic shunts in dogs: A systematic review and meta-analysis.

BackgroundSeveral options have been proposed for the treatment of congenital extrahepatic portosystemic shunts (cEHPSS) in dogs, but formal comparisons among different treatment options are currently unavailable. A previous evidence‐based review (2012) found low quality of evidence for papers assessing the treatment of cEHPSS in dogs.ObjectivesTo assess the quality of evidence available in the treatment of cEHPSS, summarize the current state of knowledge with respect to outcome after cEHPSS management, and compare different treatment techniques.AnimalsNot used.MethodsA bibliographic search was performed without date or language restrictions. Studies were assessed for quality of evidence (study design, study group sizes, subject enrollment quality, and overall risk of bias) and outcome measures reported (perioperative outcome, clinical outcome, and surgical or interventional outcome), all reported with 95% confidence intervals. A network meta‐analysis was performed.ResultsForty‐eight studies were included. Six retrospective studies (grade 4b) compared 2 techniques and 7 were abstracts (grade 5). The quality of evidence was low and risk of bias high. Regarding surgical outcome, statistically significant superiority of ameroid constrictor over thin film band was observed (P = .003). No other comparisons were statistically significant.Conclusions and Clinical ImportanceThe evidence base of choice of treatment of cEHPSS in dogs remains weak despite recent publications on the subject. Ameroid is superior to thin film band in causing EHPSS closure. Blinded randomized studies comparing different treatment modalities, which routinely include postoperative imaging to assess cEHPSS closure and acquired portosystemic shunt development are essential.

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study.

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

Computed tomography angiography of a congenital extrahepatic splenocaval shunt in a foal

Congenital portosystemic shunts in foals are rare and only a small number of cases have been described. Detailed description of the course of the shunt is lacking in earlier reports. This is the first detailed description of a computed tomography angiography (CTA) displaying an extra-hepatic splenocaval shunt. A 1-month old colt showing increasing signs of dullness, ataxia, circling, lip-smacking and coordination problems was presented. Hyperammonemia was detected and abdominal CTA revealed an extra-hepatic portocaval shunt. During surgery, ligation of the abnormal vessel could not be achieved, and the foal was euthanized because of complications during surgery. CTA provided a detailed overview of portal vasculature. If a portosystemic shunt is suspected in a foal, CTA can be used to confirm the diagnosis and for surgical planning.

Outcomes of dogs treated for extrahepatic congenital portosystemic shunts with thin film banding or ameroid ring constrictor.

To compare the outcomes of dogs treated at a single institution for single extrahepatic congenital portosystemic shunts (CPSS) by thin film banding (TFB) or by placement of an ameroid constrictor (AC). Retrospective case series. Seventy-six client-owned dogs with CPSS treated with TFB (n = 53) or AC (n = 23). Records were reviewed for signalment, preoperative, intraoperative, and postoperative management and short-term outcomes. Data on second surgeries were reviewed. Long-term outcomes were obtained via an owner-directed health-related quality of life questionnaire. The rates of complications, mortality, and revision surgery were compared between the treatment groups. Postoperative complications occurred in 15 (28%) dogs with TFB (9% mortality, n = 5) and 8 (35%) dogs with an AC (4% mortality, n = 1). Long-term follow-up was available in 41 of 56 dogs at a median of 55 months (range, 15-89). Revision surgery for persistent shunting was performed in 14 (29%) dogs treated initially with TFB and in no dogs treated initially with AC (P = .007). Median long-term outcome scores were good in both groups; nine of 14 revision surgeries led to favorable outcomes. Persistent shunting requiring revision surgery was more common when CPSS were treated with TFB than with an AC, but both treatments achieved favorable long-term outcomes. Treatment of CPPS by placement of an AC rather than TFB seems more reliable for shunt attenuation and prevention of revision surgeries.

Portal venous deprivation in patients with portosystemic shunts and its effect on liver tumors

AimsTo outline the use of radiological investigations in patients with congenital portosystemic shunts (CPS) and to assess the degree of portal venous deprivation in relation to the development of liver tumors. MethodsSingle center retrospective cohort study of all patients with CPS referred from 1990 to 2016. Radiological investigations were reviewed for the presence of intrahepatic portal veins. Two groups were defined: Group 1 — without evidence of preservation of an intrahepatic portal venous system and, Group 2 — with evidence of intrahepatic portal venous flow. These groups were compared for the development of liver tumors, serum ammonia, and nature of subsequent surgical intervention. The ratio of infraceliac to supraceliac aortic diameter and hepatic enhancement in biphasic CT scans were also used to infer hepatic arterial in-flow and compared to age matched controls. Nonparametric tests were used throughout. A P value of 0.05 was considered significant. Data are quoted as median (IQR). Results45 patients (Group 1, n = 12: Group 2, n = 33) were investigated for CPS at a median age of 8 months (1 month–14 years). Liver tumors were more common in Group 1 than Group 2 [11/12 (92%) versus 10/33 (29%); P < 0.001]. Aortic ratio was significantly lower in patients with CPS compared to control (0.82 versus 0.96; P < 0.001), but there was no difference between patients with or without tumors (0.82 versus 0.82; P = 0.52). Enhancement of the liver parenchyma was greater in portal venous rather than arterial phases by a median difference of 28 (15–50) Hounsfield units in controls, compared to 15 (8.5–23.5) in CPS patients (P = 0.04). A single stage closure was possible in 2/6 (33%) operated patients in Group 1 and 14/20 (70%) in Group 2 (P = 0.32). ConclusionsPatients without radiological evidence of intrahepatic portal venous flow were significantly more likely to have associated hepatic tumors with a relative risk 3.1. Levels of evidenceThis manuscript includes data that are III and IV Levels-of-Evidence.

Retrospective Liver Histomorphological Analysis in Dogs in Instances of Clinical Suspicion of Congenital Portosystemic Shunt.

IntroductionThe clinical symptoms of portosystemic shunts (PSSs) and hepatic microvascular dysplasia (HMD) – portal vein hypoplasia (PVH) in dogs are similar. PSSs are abnormal vascular connections between the portal vein system and systemic veins. HMD is a very rare developmental vascular anomaly, recognisable during histopathological examination. The study aim was to assess the prevalence of HMD–PVH and hepatocellular and vascular pathologies in the liver.Material and MethodsLiver biopsies from 140 dogs (of different breeds and both sexes) arousing clinical suspicion of PSS were examined histopathologically.ResultsAn initial PSS diagnosis was confirmed in 125 dogs (89.29%). HMD–PVH was found in 12.32% of dogs, as an isolated disease in 9.29%, especially in Yorkshire terriers, and with extrahepatic PSS in 6.67%. Histopathological analysis of muscles around sublobular veins showed that HMD cases presented hypertrophy or hypertrophy with fibrosis. In 2.17% of all dogs with liver vascular developmental disorders calcification was visible around vessels (without correlation by degenerative changes in those vessels), suggesting prior onset of deep metabolic disorders. Clinical suspicion of PSS was also formed upon quite different pathological processes in young dogs.ConclusionHistopathological findings diagnosed the type of vascular anomalies (PSS or HMD–PVH) or other pathological changes conclusively, therefore detailed hepatic histopathology is an indispensable component of the clinical diagnostic process.

Untargeted metabolomic profiling of urine from healthy dogs and dogs with chronic hepatic disease.

Chronic hepatic disease can present a diagnostic challenge with different etiologies being associated with similar clinical and laboratory findings. The histopathological assessment of a liver biopsy specimen is usually required in order to make a definitive diagnosis and the availability of non-invasive prognostic biomarkers is limited. The emerging science of metabolomics is used to detect changes in endogenous low molecular weight metabolites in biological samples and offers the possibility of identifying noninvasive markers of disease. The objective of this study was to investigate differences in the urine metabolome between healthy dogs, dogs with chronic hepatitis, dogs with hepatocellular carcinoma, and dogs with a congenital portosystemic shunt. Stored urine samples from 10 healthy dogs, 10 dogs with chronic hepatitis, 6 dogs with hepatocellular carcinoma, and 5 dogs with a congenital portosystemic shunt were analyzed. The urine metabolome was analyzed by gas chromatography–quadrupole time of flight mass spectrometry and 220 known metabolites were identified. Principal component analysis and heat dendrogram plots of the metabolomics data showed clustering between groups. Random forest analysis showed differences in the abundance of various metabolites including putrescine, gluconic acid, sorbitol, and valine. Based on univariate statistics, 37 metabolites were significantly different between groups. In, conclusion, the urine metabolome varies between healthy dogs, dogs with chronic hepatitis, dogs with hepatocellular carcinoma, and dogs with a congenital portosystemic shunt. Further targeted assessment of these metabolites is needed to assess their diagnostic utility.

Congenital extrahepatic portosystemic shunt with progressive myelopathy and encephalopathy

Portosystemic encephalopathy commonly occurs in patients with portal hypertension caused by end-stage liver disease or portal vein thrombosis. Congenital extrahepatic portosystemic shunt (CEPS) is an underdiagnosed and treatable condition that...

Congenital Portosystemic Shunts: Current Diagnosis and Management.

Congenital portosystemic shunts are increasingly recognized in several settings and at any age. The following are some of the most common presentations: prenatal ultrasound, neonatal cholestasis, incidental finding on abdominal imaging, or systemic complications such as unexplained cardiopulmonary or neurological disease, or the presence of liver nodules in a noncirrhotic liver. The purpose of the present review is to summarize clinical presentation and current recommendations for management, and highlight areas of future research. Illustrative examples from the veterinary literature complement our current lack of knowledge of this rare malformation often masquerading as a multisystem disease.

Abernethy Malformation – Congenital Extra-hepatic Portosystemic Shunt Associated with Multiple Liver Adenomatosis: Case Report

In this article we report a case of Abernethy malformation, also known as congenital extrahepatic portosystemic shunt (CEPS). It is a rare vascular malformation in which the portal vein drains into a systemic vein, diverting it from its normal path to the liver. The clinical presentation is variable and most commonly the shunt is diagnosed during the propaedeutic for investigation of the symptoms. Discussion and diagnosis: R.B.S.R, 21 years old, male, with history of liver disease and previous diagnosis of multiple liver adenomatosis from 9 years of age, was hospitalized for pre-transplant liver evaluation. In the medical history, he presented delayed neuropsychomotor development, congenital scoliosis, neurogenic bladder and single kidney, and had also undergone two surgeries. His initial laboratory tests showed increased transaminases and canalicular enzymes. Ultrasonography, radiography, computed tomography (CT) and magnetic resonance imaging (MRI) were performed, which enabled us to identify and confirm important points for diagnosis. Conclusions: It is important that the radiologist recognize the findings early. CT and MRI are fundamental in the management of the syndrome, since they provide the information for diagnosis, planning, intervention and follow-up, as well as the identification of complications.

Laparoscopic Partial Closure for Congenital Portosystemic Shunt-Indications, Postoperative Management, and Subsequent Complete Closure.

Extrahepatic congenital portosystemic shunts (CPSSs) can be occluded by surgical or endovascular approaches. However, when the estimated portal vein (PV) pressure after the closure is high enough to induce symptoms associated with portal hypertension, partial closure is recommended to avoid life-threatening events. In this study, we attempted laparoscopic partial closure of a CPSS in two patients. Along with intraoperative real-time measuring of the PV pressure and angiography, laparoscopic partial closure was performed to achieve a PV pressure of ≤25 mmHg. Subsequently, the intrahepatic portal system grew in both patients. The partially ligated CPSS closed spontaneously in the first patient. In the second patient, laparoscopic complete closure was performed for the residual CPSS 6 months after the first operation. To our knowledge, this is the first report of laparoscopic partial closure for CPSS. Minimally invasive laparoscopic partial ligation of CPSS is technically feasible and useful when the estimated PV pressure is too high to tolerate one-step complete closure.

Untargeted metabolomic profiling of serum from dogs with chronic hepatic disease

BackgroundChronic hepatopathies present a diagnostic challenge, with different diseases being associated with similar clinical and laboratory findings. Characterization of dogs with chronic hepatopathies can be difficult and require costly diagnostic procedures such as acquisition of a liver biopsy specimen. Noninvasive and inexpensive biomarkers that reliably characterize chronic hepatopathies such as chronic hepatitis or a congenital portosystemic vascular anomaly may decrease the need for costly or invasive diagnostic testing and guide novel therapeutic interventions.ObjectiveTo investigate differences in the serum metabolome among healthy dogs, dogs with congenital portosystemic shunts, and dogs with chronic hepatitis.AnimalsStored serum samples from 12 healthy dogs, 10 dogs with congenital portosystemic shunts, and 6 dogs with chronic hepatitis were analyzed.MethodsThe serum metabolome was analyzed with an untargeted metabolomics approach using gas chromatography–quadrupole time of flight mass spectrometry.ResultsPrincipal component analysis and heat dendrogram plots of the metabolomics data showed clustering among individuals in each group. Random forest analysis showed differences in the abundance of various metabolites including increased aromatic amino acids and xylitol in dogs with congenital portosystemic shunts. Based on the univariate statistics, 50 metabolites were significantly different among groups.Conclusions and Clinical ImportanceThe serum metabolome varies among healthy dogs, dogs with congenital portosystemic shunts, and dogs with chronic hepatitis. Statistical analysis identified several metabolites that differentiated healthy dogs from dogs with vascular or parenchymal liver disease. Further targeted assessment of these metabolites is needed to confirm their diagnostic reliability.