Pancreatic neuroendocrine tumour resection in circumportal pancreas: a rare anatomical anomaly with important surgical implications.

Various congenital anomalies of the pancreas have been reported due to its complex embryological development involving the fusion of two separate buds. Circumportal pancreas is a rare anatomical anomaly where the pancreatic head and uncinate process fuse abnormally with the pancreatic body, encasing the portal vein and/or superior mesenteric vein completely. This anomaly poses several challenges to hepatobiliary surgeons, as the encasement of the portal vein by the abnormal pancreatic tissue makes an additional parenchymal transection necessary. Vascular variants have also been reported with circumportal pancreas, which, if not recognised preoperatively, can be catastrophic. Therefore, careful preoperative evaluation and planning are essential, to ensure safe pancreatic resection and recovery in a patient with circumportal pancreas. We present a case of a successful subtotal pancreatectomy and splenectomy in a patient with circumportal pancreas, for a suspected pancreatic duct adenocarcinoma. The aim of this case report is to contribute valuable insights that can aid hepatobiliary surgeons in enhancing their preoperative planning when encountered with patients with similar anatomical variances.

Successful surgical attenuation of portosystemic shunt in a dog with imaging-diagnosed portal vein aplasia.

An 8-month-old female Maltese dog was referred for examination with a history of circling, dullness, and drooling. Serum biochemical analysis revealed hyperammonemia, with microhepatica observed on radiography. Computed tomography angiography revealed a portosystemic shunt originating from the right gastric vein and inserting into the prehepatic caudal vena cava. Portal blood flow to the liver was not observed. Based on computed tomography angiography, the dog was tentatively diagnosed with portosystemic shunt with portal vein aplasia. An exploratory laparotomy was done to obtain a definitive diagnosis. The dog had no subjective clinical signs of portal hypertension during a temporary occlusion test of the portosystemic shunt. A thin-film band was placed around the portosystemic shunt to achieve partial attenuation. There was no evidence of hepatic encephalopathy in the long term after surgery, and the dog's liver volume increased over time. Computed tomography angiography at 6 mo after surgery identified well-visualized intrahepatic portal branches. Key clinical message: We inferred that a direct occlusion test is a reliable diagnostic technique that overcomes the limitations of diagnostic imaging methods, including computed tomography angiography, and is a good technique for determining whether surgical attenuation is possible in dogs with suspected portal vein aplasia.

Atténuation chirurgicale réussie d'un shunt porto-systémique chez un chien avec une aplasie de la veine porte diagnostiquée par imagerie. Une femelle bichon maltais âgée de 8 mois a été référée pour examen avec une histoire de tournis, apathie et salivation excessive. L'analyse biochimique du sérum a révélé une hyperammionémie, avec un petit foie observé lors des radiographies. Une angiographie par tomodensitométrie a révélé un shunt porto-systémique prenant son origine de la veine gastrique droite et s'insérant dans la veine cave caudale pré-hépatique. Le flot sanguin porte au foie n'était pas observé. Sur la base de l'angiographie par tomodensitométrie, un diagnostic présumé de shunt porto-systémique avec aplasie de la veine porte a été émis. Une laparotomie exploratoire a été effectuée afin d'obtenir un diagnostic définitif. Le chien ne présentait pas de signe clinique subjectif d'hypertension portale durant un test d'occlusion temporaire du shunt porto-systémique. Une bande de film mince a été placée autour du shunt porto-systémique pour causer une réduction partielle. Il n'y avait aucune évidence d'encéphalopathie hépatique à long terme après la chirurgie, et le volume du foie du chien a augmenté dans le temps. Une angiographie par tomodensitométrie effectuée 6 mo après la chirurgie a permis de bien visualiser des branches portes intra-hépatiques.Message clinique clé :Nous avons déduit qu'un test d'occlusion est une technique diagnostique fiable qui surpasse les limites des méthodes d'imagerie diagnostique, incluant l'angiographie par tomodensitométrie, et est une bonne technique pour déterminer si une réduction chirurgicale est possible chez des chiens chez qui on soupçonne une aplasie de la veine porte.(Traduit par Dr Serge Messier).

Clinical presentation and short-term outcomes of dogs ≥15 kg with extrahepatic portosystemic shunts.

OBJECTIVE: To describe demographics, clinical presentation, shunt anatomy, clinical progression, and complications in large dogs ≥15 kg with single extrahepatic portosystemic shunts (EHPSS) treated with or without surgery. STUDY DESIGN: Multicenter retrospective (10 university hospitals, one private referral institution). ANIMALS: Dogs ≥15 kg (n = 63). METHODS: Medical records of dogs ≥15 kg diagnosed with EHPSS between January 01, 2005 and December 31, 2020 were reviewed. Dogs had a minimum follow-up of 90 days. Signalment, clinical signs, diagnostics, shunt anatomy, treatment interventions, and perioperative complications were assessed. RESULTS: Median age was 21.9 months (IQR: 9-36.8). The breed most represented was the Golden retriever (17/63 dogs). Portocaval (17/63) and splenocaval (15/63) shunt configurations were most common. Portal vein hypoplasia was noted in 18 imaging reports. Of the surgically treated dogs, 14/45 (35.6%) had short-term complications, and 3/45 (6.7%) had shunt-related deaths. Medical management was discontinued in 15/40 and reduced in 9/40 of surviving dogs who had surgical attenuation. All medically managed, nonattenuated dogs (18/18) were maintained on their original shunt-related medication regimens. CONCLUSIONS: Clinical presentation of dogs ≥15 kg with extrahepatic portosystemic shunts was similar to the more commonly reported small breed dogs. Surgical management of single EHPSS in large dogs ≥15 kg had similar clinical short-term outcomes as small breed dogs. CLINICAL SIGNIFICANCE: Clinicians should be aware that large breed dogs with EHPSS share similar characteristics and clinical outcomes to small breed dogs. The significance of the presence of a hypoplastic portal vein warrants further research. Surgical treatment is a viable option for large breed dogs with EHPSS.

Diagnosis and management of Abernethy syndrome.

Abernethy syndrome (AS or extrahepatic portosystemic shunt) is an uncommon congenital malformation consisting of agenesis or hypoplasia of the portal vein (PV) in such a way that splanchnic venous blood drains directly into the systemic circulation through aberrant communications, resulting in a portosystemic shunt that bypasses the liver AS is an underdiagnosed condition with unknown incidence and complication rate given that symptoms are usually absent. AS identification is increasingly common because of improved imaging techniques, hence prognostic implications and clinical management need be understood. This editorial reviews the natural history of AS and its diagnostic-therapeutic implications, illustrating the process with a series of cases from our institution.

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.

Abernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins. Though rare, this anomaly can lead to pulmonary hypertension. Drainage of Abernethy malformation into coronary sinus is extremely rare. We describe a child with Abernethy malformation with unusual drainage into coronary sinus. The abnormal channel was successfully closed by trans-catheter technique with normalisation of pulmonary arterial pressures.

The Portal Vein: A Comprehensive Review.

Radiologists are familiar with the appearances of a normal portal vein; variations in its anatomy are commonplace and require careful consideration due to the implications for surgery. These alterations in portal vein anatomy have characteristic appearances that are clearly depicted on CT, MR, and US images. Similarly, there are numerous congenital and acquired disorders of the portal vein that are deleterious to its function and can be diagnosed by using imaging alone. Some of these conditions have subtle imaging features, and some are conspicuous at imaging but poorly understood or underrecognized. The authors examine imaging appearances of the portal vein, first by outlining the classic and variant anatomy and then by describing each of the disorders that impact portal vein function. The imaging appearances of portal vein abnormalities discussed in this review include (a) occlusion from and differentiation between bland thrombus and tumor in vein and the changes associated with resultant hepatic artery buffer response changes, cavernous transformation of the portal vein, and portal biliopathy; (b) ascending thrombophlebitis of the portal vein (pylephlebitis); (c) portal hypertension and its causes and sequelae; (d) the newly described disease entity portosinusoidal vascular disorder; and (e) intra- and extrahepatic shunts of the portal system, both congenital and acquired (including Abernethy malformations), and the associated risks. Current understanding of the pathophysiologic processes of each of these disorders is considered to aid the approach to reporting. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Diagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts.

PURPOSE: To investigate the diagnostic and monitoring value of ultrasound (US), computed tomography angiography (CTA), and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts (CEPS) in children. METHODS: We retrospectively analysed different imaging examinations of 15 children with CEPS. Development of the portal vein before shunt occlusion, shunt location, portal vein pressure, main symptoms, diameter of the main portal vein, and location of secondary thrombosis after shunt occlusion were recorded. Final classification diagnosis was made by portal venography after shunt occlusion, and consistency with other imaging examinations in diagnosing development of the portal vein was calculated using Cohen's kappa. RESULTS: Compared with portal venography after shunt occlusion, US, CTA, and portal venography before shunt occlusion had poor consistency in showing the development of hepatic portal veins (Kappa value 0.091-0.194, P > 0.05). Six cases developed portal hypertension (40-48 cmH2O) during the temporary occlusion test, and US showed that portal veins gradually expanded after shunt ligation. Eight patients with haematochezia had inferior mesenteric vein (IMV)-iliac vein (IV) shunts. After surgery, secondary IMV thrombosis was observed in eight cases and secondary splenic vein thrombosis in four cases. CONCLUSION: Portal venography with occlusion testing is very important to accurately evaluate the development of the portal vein in CEPS. The portal vein needs to expand gradually, and partial shunt ligation surgery is necessary in cases diagnosed as portal vein absence or hypoplasia before occlusion testing to avoid severe portal hypertension. After shunt occlusion, US is effective in monitoring portal vein expansion, and both US and CTA can be used to monitor secondary thrombi. IMV-IV shunts can cause haematochezia and are prone to secondary thrombosis after occlusion.

Incidental Preduodenal Portal Vein Causing Partial Duodenal Obstruction and Hypertrophied Pre-Pyloric Gastric Polyp.

Preduodenal portal vein (PDPV) is a rare congenital anomaly in which the portal vein lies anterior to the duodenum rather than its normal posterior position. It is a known rare cause of duodenal obstruction and can be associated with other anomalies such as malrotation with or without jejunal atresia. Presented is an incidentally found PDPV causing partial duodenal obstruction during exploration for the resection of a gastric mass and placement of open gastrostomy tube for feeding. This was managed with duodenoduodenostomy, re-creating normal anatomy with portal.

Jejunal arteriovenous malformation and multiple acquired extrahepatic portosystemic shunts in a juvenile dog, presenting with melena.

A juvenile dog referred with a 1-month history of persistent melena and severe anaemia, was diagnosed with a jejunal arteriovenous malformation, and multiple acquired extrahepatic portosystemic shunts. A midline coeliotomy was performed, the jejunal arteriovenous malformation was localised intraoperatively and was successfully removed via an enterectomy. Histopathology confirmed a true arteriovenous malformation. Despite the initial improvement, the patient developed seizure episodes secondary to hepatic encephalopathy 8 months after surgery. Fifteen months after surgery, the owner opted for euthanasia due to the ongoing seizure episodes. Post-mortem histologic examination of the liver showed features consistent with portal vein hypoplasia. A congenital arteriovenous malformation should be considered as a differential diagnosis in juvenile patients with a chronic history of haemorrhage from the gastrointestinal tract. In addition, acquired portosystemic shunts may occur in patients with portal vein hypoplasia and jejunal arteriovenous malformations.

Unique case of pelvic congestion syndrome caused by a new communication branch of the portal-vena cava system.

Congenital portosystemic shunt (CPS) is a developmental anomaly of the portal vein system. The disease can cause blood from the portal vein to flow into the vena cava, resulting in various atypical clinical manifestations. Pelvic congestion syndrome (PCS) caused by CPS is particularly rare. A young woman with PCS had an abnormal communicating branch of the left ovarian vein (OV). Her left OV drained normally into the left renal vein, and at the same time communicated with the portal vein, forming an extrahepatic portosystemic shunt. With embolization of her left OV, the patient was cured of PCS.

Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Traumatic high flow arterioportal fistula. Correction by a covered stent.

The arterio-portal fistula is a rare entity, generally associated with different causes, among which penetrating trauma stands out. They can occur at the beginning asymptomatic or manifest by a wide spectrum of signs and symptoms with severe limitation of the patient's quality of life and even compromise it. Immediate therapeutic action, whether surgical or endovascular, is vital for the definitive solution of the primary triggering cause. We present the case of a patient with a stab wound to the abdomen who developed an arterioportal fistula with associated portal hypertension as a complication. It is treated by placing a covered stent at the level of the anomalous communication, with definitive clinical and imaging resolution.

Congenital Absence of the Portal Vein.

A 59-year-old woman presented with abdominal bloating, elevated alkaline phosphatase and transaminases, and computed tomography abdomen/pelvis demonstrating large right-sided hepatic masses. A percutaneous fine needle aspiration demonstrated hepatocellular neoplasm concerning for hepatocellular carcinoma. Preoperative imaging demonstrated possible porto-caval shunt. She underwent uneventful right hepatic lobectomy with confirmation of porto-systemic shunt. Congenital porto-systemic shunt, or Abernethy malformation, is rare and is associated with congenital cardiac and gastrointestinal abnormalities. Additionally, congenital porto-systemic shunt is associated with increased risk of hepatic neoplasms including hepatocellular carcinoma. Recommended surveillance for these patients is not well defined.

Deep brain stimulation for the abernethy malformation related tremor.

Deep brain stimulation (DBS) is introduced for the surgical treatment of movement disorders such as Parkinson's disease, tremor, dystonia, and tics. Electrostimulation of the ventral thalamus or subthalamic area has been found effective in different types of tremors that have different etiologies. Abernethy malformation is a rare congenital abnormality characterized by the presence of a congenital extrahepatic portosystemic shunt between the portal vein and systemic circulation. In this report, we present as a case of Abernethy malformation that caused hyperammonemia congenitally and presented as action and resting tremor in the hands and, treated with DBS.

Abernethy malformation with unusual cardiac malformation: Case report and literature review.

Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly, which is characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type II in a 9-year-old boy, whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries (MAPCAs) but laboratory examinations were normal 5 years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that physicians should be aware of the possibility of Abernethy malformation in children with enlargement of the left ventricular due to systemic-pulmonary collateral circulation.

Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report. / Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Abernethy Malformation: Possible Diagnosis for Patients with Congenital Heart Disease and Persistent Cyanosis.

CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. ECHOCARDIOGRAM: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. COMPUTED TOMOGRAPHY: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. DIAGNOSIS: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. OPERATION: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.

[Importance of prenatally diagnosed portosystemic vascular shunts in clinical outcomes]. / Importancia de las derivaciones vasculares portosistémicas de diagnóstico prenatal en la evolución clínica.

Portosystemic venous shunts (PSVS) are malformations that result from abnormal communications between the portal and hepatic veins or inferior vena cava. Prenatal diagnosis is made by evaluating the fetal venous circulation and it is classified as intrahepatic and extrahepatic, with different evolution and complications. OBJECTIVE: To report two cases of prenatal diagnosis of portosystemic vascular shunts and review the importance of this rare pathology in its neonatal and pediatric evolution. CLINICAL CASES: Case 1: pregnancy with fetal growth restriction, 2nd percentile, polyhydramnios, without fetal malformations and abnormal patterns on fetal Doppler. Abnormal blood flow through the ductus venous and abnormal venous communication in the liver were identified. Normal genetic study. Male newborn (NB) delivered at 36 weeks, because of severe fetal growth restriction, by emergency cesarean section. He evolved asymptomatic, with normal liver function, and did not continue follow-up. Case 2: pregnancy with fetal growth restriction < percentile 1. Agenesis of the ductus venous and abnormal communication between the portal vein and the left suprahepatic vein were identified with no other malformations or signs of heart failure. Severe SGA newborn was delivered by induction of labor at 35 weeks. He evolved asymptomatic. Normal complementary study. A home check-up at 2 months showed persistent vascular anomaly without systemic involvement. CONCLUSIONS: Even though in the cases presented there were no neonatal complications, this kind of malformations require a high index of suspicion in cases with fetal growth restriction, as well as a long-term multidisciplinary follow-up.