Extrahepatic portal-venous obstruction

Gibson, James B.; Johnston, G. W.; Fulton, T. T.; Rodgers, H. W.

doi:10.1002/bjs.1800520211

Cited by 83 publications

(36 citation statements)

References 32 publications

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“…1 Only sporadic reports appeared over the next few decades [7][8][9] illustrating the rarity of this condition in the West. Though surgical hazards posed by common bile duct (CBD) varices were recognized in the late 1970s, 10 it was the seminal paper by Webb and Sherlock 11 that focussed attention on this condition.…”

Section: Historical Perspective Nomenclature and Definitionmentioning

confidence: 99%

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Dhiman

Saraswat

Valla

et al. 2014

Journal of Clinical and Experimental Hepatology

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Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery. ( J CLIN EXP HEPATOL 2014;4:S2-S14)

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Section: Historical Perspective Nomenclature and Definitionmentioning

confidence: 99%

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Dhiman

Saraswat

Valla

et al. 2014

Journal of Clinical and Experimental Hepatology

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“…Several investigators believe that umbilical vein catheterization and umbilical sepsis are responsible for portal vein thrombosis (PVT) others disagree. [8][9][10][11][12] The discrepancies between the observations are probably because of the studies that have been retrospective and clinical. In an Indian prospective ultrasonographic study up to 24 months, none of the 11 patients with septicaemia or umbilical sepsis developed portal vein thrombosis.…”

Section: Resultsmentioning

confidence: 99%

A study on demographic and clinical profile of children with extra hepatic portal venous obstruction and with special reference to thrombophilic factors

Jena

Mohanty

Patra

et al. 2017

Int J Community Med Public Health

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Background: Extra-hepatic portal vein obstruction (EHPVO) due to portal vein thrombosis is an important cause of portal hypertension in several region including India. The cause of thrombosis in these patients remains unclear. Objective of the study was to study the demographic features, etiology, clinical, laboratory findings with special reference to thrombophilic factors like protein C, protein S and antithrombin III deficiency in children with EHPVO.Methods: The prospective analysis of 62 patients of EHPVO (<14 years of age) was done in the Department of Hepatology, SCB medical College, Cuttack. After detailed history, clinical examination, Ultrasound abdomen /color Doppler and Upper GI endoscopy, the subjects were analyzed for any deficiency of thrombophilic factors like protein C, protein S and antithrombin III.Results: A total of 62 patients (37 Male, 25 Female) with mean age of 8.3+3.1 years were studied. Growth retardation was present in the form of wasting (alone) 20.9%, stunting (alone) 25.8% and both wasting and stunting was found in 9.8% cases. History of neonatal, umbilical sepsis and umbilical vein catheterization was found in 15.9% and 10.2% of cases respectively. Haemorrhage from oesophageal varices was prevalent symptoms in 85.9% patients. Splenomegaly was found in 91.9% patients and ascites in 9.4% patients. 47 patients studied for protein C, S and antithromibin III. 14 patients were found to have thrombophilia: protein C deficiency in 9, protein S deficiency in 8, Antithrombin III deficiency in 6.Conclusions: The etiology of EHPVO in the majority of patients remain still unclear. It is commonly associated impaired somatic growth. The risk of EHPVO increases in the presence of thrombophilia, resulting from deficiency of naturally occurring anticoagulant proteins like Protein C, Protein S and Antithrombin III.

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“…In a review of 13 major retrospective studies (1, 6, 9, 12-14, 17, 19, 20, 22, 23, 26, 29), a positive history of neonatal infection was available in a percentage varying from 3.5 to 56.2%, in most of them, the origin being the umbilicus. In only six of these studies (9,13,19,23,26,29) was a positive history of cannulation of umbilical vein available, with a percentage varying from 4.2 to 38.4%. Some characteristics of these studies have limited their generalizability.…”

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confidence: 99%

NeonatalCandida albicansSeptic Thrombosis of the Portal Vein followed by Cavernous Transformation of the Vessel

et al. 2004

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We report two premature neonates with Candida albicans septic thrombosis of the portal vein who developed, in very early childhood, the sonographic appearance of cavernous transformation of the vessel and/or clinical signs of extrahepatic portal hypertension. CASE REPORTS Case 1. A 1,980-g male infant, who was the second infant delivered, was born by emergency cesarean section for fetal distress after a 34-week-triplet gestation to a 35-year-old primigravida mother. Except for premature labor, her pregnancy had been otherwise normal, without prolonged rupture of membranes or maternal fever. The infant had respiratory distress immediately after birth and was therefore admitted to the neonatal intensive care unit (NICU), where he was treated for 2 days with nasal continuous positive airway pressure (CPAP) and for 5 days with oxygen therapy. On admission to the NICU, a polyvinyl chloride 5 French catheter was inserted into the umbilical vein, and intravenous ampicillin and netilmicin were started for suspected sepsis. The catheter tip was noted to be in the inferior vena cava. Blood culture obtained on admission gave negative test results, and antibiotics were discontinued after 3 days. On postnatal day 8, he developed apnea and bradycardia, irritability, feeding difficulties, and abdominal distension. The same day, the catheter was removed, the cathetertip, blood from peripheral veins, and urine cultures were taken, and the infant was started on teicoplanin therapy. While the infant continued to be symptomatic, on postnatal day 10, the laboratory report for the positive cultures of the catheter tip indicated a pure growth of Candida albicans and treatment was changed to oral fluconazole. On postnatal day 11, the blood culture performed 3 days earlier was reported to have yielded a heavy (defined as Ն50 CFU/ml) (24) growth of C. albicans (sensitive to fluconazole and amphotericin B). He continued treatment with fluconazole for 6 weeks and on day 11 started a 6-week intravenous amphotericin B treatment. Soon after the positive blood culture result, a disseminated fungal workup including abdominal and cranial ultrasound, echocardiogram, and ophthalmologic evaluation was performed. The echocardiogram, cranial ultrasound, and ophthalmologic evaluation were negative. Abdominal gray-scale ultrasound showed that both right and main portal veins were completely filled with hyperechoic thrombus. The spleen was of normal size, but the liver appeared slightly enlarged without sonographic abnormalities of the hepatic parenchyma or of the biliary structures. A second cranial ultrasound revealed, in the parietal and temporal lobes, multiple, bilateral, round hypoechoic lesions with echogenic rims, which were consistent with intracranial abscesses (18). A peripheral venipuncture blood culture obtained 1 week after initiation of antifungal therapy was still positive for C. albicans. However, it became sterile 11 days after antifungal treatment was started. At 35 days of age, the cranial ultrasound became normal, but the abdominal ...

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Extrahepatic portal-venous obstruction

Cited by 83 publications

References 32 publications

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

A study on demographic and clinical profile of children with extra hepatic portal venous obstruction and with special reference to thrombophilic factors

NeonatalCandida albicansSeptic Thrombosis of the Portal Vein followed by Cavernous Transformation of the Vessel

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