Extrahepatic Portocaval Shunt Research Articles

Pulmonary arterial hypertension associated with type II Abernethy malformation in an adolescent: a case report

The article provides a case of pulmonary arterial hypertension (PAH) associated with congenital extrahepatic portocaval shunt (CEPS), or Abernethy malformation, in a 17-year-old female patient. CEPS, which remained undiagnosed for a long time, manifested with severe encephalopathy at an early age. By the age of 6 years, it was complicated by PAH, and by the age of 9 years — by hepatic focal nodular hyperplasia. In the absence of timely surgical treatment and adequate therapy of CEPS, PAH progressed. Combined dual therapy for PAH, prescribed only at age 14, was ineffective. Despite the potential for clinical and functional improvement documented with combination triple therapy at 17 years of age, the patient continued to meet criteria for a high risk of adverse events, including mortality. Two months after the initiation of triple therapy for PAH, not achieving the target hemodynamic characteristics that would reduce operative and perioperative risks lead to an attempt of surgical treatment of CEPS, which led to the patient death in the early postoperative period. The description of this case report shows the difficulties of diagnosing a rare form of portopulmonary hypertension, the modern possibilities of drug therapy for a severe, potentially curable PAH in the early stages.

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

ObjectiveAbernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. MethodsThis is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. Results10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. ConclusionCongenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.

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.

Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report

BackgroundCongenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension.Case presentationWe report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient’s condition improved in the first year after discharge. However, one year later, the patient’s conditions start to deteriorate.ConclusionThis article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.

Detection of trisomy 18 with Abernethy malformation by prenatal ultrasound: a case report

We reported a case of trisomy 18 with Abernethy malformation diagnosed by prenatal ultrasound, which was confirmed by chromosome karyotyping after cordocentesis and fetal autopsy. At 24 gestational weeks, fetal sonography revealed a fetus with multiple malformations (cleft lip/cleft palate of left side, left radial dysplasia, hooked hands, bilateral pes valgus, ventricular septal defect, and congenital extrahepatic portocaval shunt), which was diagnosed as trisomy 18 syndrome with Abernethy malformation. The fetus's karyotype was 47, XX+18. Key words: Trisomy 18 syndrome; Vascular malformations; Portal vein; Ultrasonography, prenatal

Distribution of extrahepatic congenital portosystemic shunt morphology in predisposed dog breeds

BackgroundAn inherited basis for congenital extrahepatic portosystemic shunts (EHPSS) has been demonstrated in several small dog breeds. If in general both portocaval and porto-azygous shunts occur in breeds predisposed to portosystemic shunts then this could indicate a common genetic background. This study was performed to determine the distribution of extrahepatic portocaval and porto-azygous shunts in purebred dog populations.ResultsData of 135 client owned dogs diagnosed with EHPSS at the Faculty of Veterinary Medicine of Utrecht University from 2001 – 2010 were retrospectively analyzed. The correlation between shunt localization, sex, age, dog size and breed were studied. The study group consisted of 54 males and 81 females from 24 breeds. Twenty-five percent of dogs had porto-azygous shunts and 75% had portocaval shunts. Of the dogs with porto-azygous shunts only 27% was male (P = 0.006). No significant sex difference was detected in dogs with a portocaval shunt. Both phenotypes were present in almost all breeds represented with more than six cases. Small dogs are mostly diagnosed with portocaval shunts (79%) whereas both types are detected. The age at diagnosis in dogs with porto-azygous shunts was significantly higher than that of dogs with portocaval shunts (P < 0.001).ConclusionThe remarkable similarity of phenotypic variation in many dog breeds may indicate common underlying genes responsible for EHPSS across breeds. The subtype of EHPSS could be determined by a minor genetic component or modulating factors during embryonic development.

Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation

A seven-yr-old boy presented with persistent oxygen requirement following a respiratory infection. Physical exam was remarkable for orthodeoxia and digital clubbing. Laboratory evaluation showed elevated A-a oxygen gradient of 48 mmHg and mildly elevated transaminases. Sonography showed a 13 cm multilobulated liver mass and a biopsy revealed histological findings consistent with focal nodular hyperplasia. MAA scan revealed 23% right to left shunting. Abdominal CTA and MRV demonstrated the absence of the intrahepatic portal vein with an extrahepatic portocaval shunt. Abernethy malformation is a rare anomalous intra- or extrahepatic communication between portal blood flow and systemic venous return. In rare cases, Abernethy malformation results in HPS. Ours is the sixth case report to describe the co-existence of these two entities. Surgical correction of anomalous hepatic vasculature or liver transplant is imperative to restoration of lung function and also to prevent progression of possible malignant liver tumors. We describe the second patient with Abernethy and HPS who underwent liver transplant with complete resolution of HPS.

Large focal nodular hyperplasia and extrahepatic portosystemic shunt in a male patient: Multi-modality imaging features

Congenital extrahepatic portocaval shunt is a rare condition that is described mostly in female patients. We report an unusual case of a young adult male patient with type 1 congenital extrahepatic portocaval shunt with associated development of a focal nodular hyperplasia on a background of regenerative nodules. With multi-slice CT utilisation, there is increased detection of portocaval malformation in asymptomatic patients. This congenital variant is clinically significant with associated development of hepatocellular lesions, hepatic dysfunction and/or encephalopathy.

Complex extrahepatic portocaval shunt with unusual caval features in a cat: computed tomographic characterisation

A two-year-old, neutered male domestic shorthair cat was evaluated for a history of urate calculi, and neurologic signs. Diagnostic imaging revealed an elongated and tortuous single extrahepatic portosystemic shunt which appeared to receive normal tributaries of the caudal vena cava. Surgical correction of the shunt was carried out using cellophane banding. Eight months following surgery, clinical signs had resolved. Computed tomographic angiography allows thorough, rapid imaging of complex vascular anomalies to aid proper surgical correction. Errors in the formation of the portal vein and caudal vena cava can produce complex anomalies of the abdominal vasculature. Persistence of the embryologic left subcardinal vein is proposed to account for the lesion.

Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation

In this report, we describe a living donor liver transplantation (LDLT) in a patient (7-year-old boy) with Abernethy type 2 congenital extrahepatic portocaval shunts (CEPS). This patient underwent a surgical shunt ligation as the first treatment for pulmonary hypertension; pulmonary hypertension was improved and controlled successfully 4 years after the first operation. However, pulmonary hypertension recurred gradually because of multiple intrahepatic portosystemic shunts; therefore, LDLT was performed as a radical treatment of intrahepatic portosystemic shunts. His pulmonary arterial pressure was also controlled 22 months after LDLT, the postoperative continuous intravenous prostaglandin I(2) (PGI(2)) treatment could be withdrawn successfully. We suggest that clinicians carefully follow up the recurrent portosystemic shunt and cardiopulmonary disorders secondary to Abernethy type 2 CEPS.

A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect

저자들은 간의 거대 종괴와 심방 중격 결손으로 수술을 받았던 환아에서 복부 CT와 간 조직 검사를 통해 확인된 Abernethy 기형 2형 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. 【Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.】

Unklare Leberrundherde bei angeborenem portokavalem Shunt einer jungen Patientin

The Abernethy malformation is a rare anomaly with partial or complete congenital absence of the portal vein and subsequent development of extrahepatic portocaval shunts. Diagnosis is usually made in childhood following the finding of liver neoplasms combined with slightly increased liver enzymes. There is a female predominance. Further findings are malformations of the heart, skeletal system and kidneys. Short-term follow-up of the liver lesions is recommended. Progressive disease can be treated by partial liver resection or transplantation. Diagnosis of the Abernethy malformation is based on various imaging modalities. We report the MRI and MRA findings of an Abernethy malformation in a 35-year-old woman with multiple liver cell adenomas and review the 32 previously published cases.

Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in abernethy malformation. A case report

Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9-year-old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation. HPS in Abernethy syndrome is a manifestation of normal portal blood flow bypassing the liver and can be ameliorated by redirecting blood flow to a segment of liver with normal portal anatomy. APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS.

A Flexible Stent with Small Intestinal Submucosa Covering for Direct Intrahepatic Portocaval Shunt: Experimental Pilot Study in Swine

The suitability of the flexible sandwich Zilver stent-graft (SZSG) with a biologically active tissue layer (small intestinal submucosa) for creation of the intravascular ultrasound (IVUS)-guided direct intrahepatic portocaval shunt (DIPS) was explored in six young swine in a search for a flexible system to replace the rigid polytetrafluoroethylene (PTFE) stent originally used by this group with limited success. The portal vein was punctured from the inferior vena cava through the caudate lobe of the liver using IVUS guidance. After balloon dilation of the puncture tract, DIPS was successfully created in all animals with use of an SZSG 9 mm in diameter and 6 cm or 8 cm long. Only one DIPS remained well patent at 14 days when the animal had to be killed because of encephalopathy. DIPS in the other five animals were found to be either severely stenosed (3 animals) or occluded (2 animals) at 4 weeks due to accelerated formation of neointimal hyperplasia (NIH) in the liver parenchymal portion of the shunt and superimposed thrombosis. The lack of high pressure in the portal system contributed to early endograft closure. The flexible stent and the covering fail badly. The reason for this could be due to either component. More work is required to find a reliable flexible system with long-term patency. Exploration of the IVUS-guided direct extrahepatic portocaval shunt is suggested.

USE OF99MTCO TRANS‐SPLENIC PORTAL SCINTIGRAPHY FOR DIAGNOSIS OF PORTOSYSTEMIC SHUNTS IN 28 DOGS

Ultrasound-guided percutaneous trans-splenic portal scintigraphy (TSPS) using 99mTcO4(-) has been used to image the portal venous system in normal dogs. Compared with per-rectal portal scintigraphy, it provides higher count density, consistent nuclear venograms of the splenic and portal vein, and significantly decreased radiation exposures. This paper describes the use of TSPS for the diagnosis of portosystemic shunts in 28 dogs. TSPS was performed injecting 70 +/- 28 MBq of 99mTcO4(-) (mean +/- SD) into the splenic parenchyma with ultrasound guidance. A dynamic acquisition at a frame rate of four frames/s for 5 min was initiated after placement of the needle and approximately 2s prior to injection. All dogs had diagnoses confirmed via exploratory laparotomy or ultrasonographic identification of the shunting vessel(s). Three studies (10.7%) were nondiagnostic because of intraperitoneal rather than intrasplenic injection of the radionuclide. Three pathways were recognized on the scintigraphic images: (1) portoazygos shunts--the 99mTcO4(-) bolus traveled dorsally, running parallel to the spine and entering the heart craniodorsally; (2) single portocaval or splenocaval shunts--the 99mTcO4(-) bolus ran from the area of the portal vein/splenic vein junction in a linear fashion toward the caudal vena cava entering the heart caudally; (3) internal thoracic shunt-the 99mTcO4 bolus traveled ventrally along the thorax and abdomen entering the cranial aspect of the heart. Single and multiple shunts were easily distinguished. There were no distinguishing features between single intra and extrahepatic portocaval shunts.

Hematochezia and Congenital Extrahepatic Portocaval Shunt with Absent Portal Vein: Successful Treatment by Liver Transplantation

Hematochezia and Congenital Extrahepatic Portocaval Shunt with Absent Portal Vein: Successful Treatment by Liver Transplantation

Re‐Evaluation of a Portocaval Venograft Without an Ameroid Constrictor as a Method for Controlling Portal Hypertension After Occlusion of Intrahepatic Portocaval Shunts in Dogs

To evaluate the use of a portocaval venograft without an ameroid constrictor in the surgical management of intrahepatic portosystemic shunts (PSS). Prospective clinical study. Seven dogs with intrahepatic PSS. Portal pressure was measured after temporary suture occlusion of the intrahepatic PSS. In dogs with an increase in portal pressure > or =8 mm Hg or signs of portal hypertension, a single extrahepatic portocaval shunt was created using a jugular vein. Clinical outcome and complications were recorded. The mean (+/-SD) portal pressure increased from 5.9+/-1.6 to 17.9+/-4.1 mm Hg with PSS occlusion. There were no intraoperative complications and, after creation of the portocaval shunt, the intrahepatic PSS could be completely ligated in all dogs. The final portal pressure was 9.6+/-1.9 mm Hg. Complications developed during postoperative hospitalization in 5 dogs and included incisional discharge (4 dogs), ascites (3), ventricular premature contractions (2), and melena, bloody diarrhea, neurologic signs, coagulopathy, and aspiration pneumonia (each in 1 dog). Six dogs died or were euthanatized with clinical signs related to depression, inappetance, abdominal pain, vomiting, melena, and abdominal distention, with a median survival of 82 days (range, 20-990 days). One dog was clinically normal at 33 months after surgery. Clinical signs observed in 6 dogs after surgery were consistent with portal hypertension. Use of a portocaval venograft without an ameroid constrictor may reduce the likelihood of hepatic vascular development, thereby increasing the risk of life-threatening portal hypertension should the venograft suddenly occlude. Use of a portocaval venograft without an ameroid constrictor to control portal hypertension after ligation of an intrahepatic PSS cannot be recommended.

Ultrasonographic evaluation of partially attenuated congenital extrahepatic portosystemic shunts in 14 dogs

Doppler ultrasonography was used to evaluate the portal vein in 14 dogs before, immediately after and four weeks after a partial ligation of a congenital extrahepatic portocaval shunt. By four...

Congenital atresia of the portal vein and extrahepatic portocaval shunt associated with benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and atrial septal defect

A rare case of congenital atresia of the portal vein and ductus venosus, extrahepatic portocaval shunt, benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and an atrial septal defect is reported. Twenty-two cases of congenital extrahepatic end-to-side shunts have been described before. Although additional anomalies are common in this type of shunt, hemangiomatosis has been described only once. Adrenal hyperplasia has never been reported in this anomaly.

Congenital extrahepatic portocaval shunt (Abernethy Type 2), huge liver mass, and patent ductus arteriosus-a case report of its rare clinical presentation in a young girl

The authors report a case of congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This type of shunt is called an Abernethy type 2 shunt, where the portal vein and inferior vena cava are connected side to side, and a hypoplastic portal flow exists in the liver parenchyma. This case of a young girl is complicated with a cardiac anomaly and a large hyperplastic nodule in the liver. This phenotype usually is seen in the Abernethy type 1 shunt, which is a side-to-end shunt with the complete absence of a portal vein. In this context, the current case is very unique in its clinical manifestation, and this phenotype has never been reported. Therefore, it is important to speculate the etiology and treatment of this kind of abnormality and its associated hepatic mass.