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Agenesis of gallbladder and multiple anomalies of the biliary tree in a patient with portal thrombosis: a case report.

A 55-year-old male patient was admitted to hospital because of splenomegaly. Abdominal ultrasonography and computed tomography failed to demonstrate the gallbladder. The diagnosis of right portal vein thrombosis was established by Doppler ultrasonography, splenoportography and computed tomography angiography. To investigate the biliary tree and evaluate the effect on the biliary tree of portal changes, endoscopic retrograde cholangiopancreatography was performed. Endoscopic retrograde cholangiopancreatography study revealed the absence of gallbladder, cystic duct and common bile duct together with the junction of the right and left hepatic ducts at the pancreatic head, with predominant left hepatic duct. To our knowledge, this is the first reported case of multiple congenital anomalies of the extrahepatic biliary tree associated with right portal vein thrombosis. The presence of these rare pathologies in two viscera running together in a patient with right portal vein thrombosis is a very rare condition.

Abnormalities, Multiple↗

Spontaneous splenorenal shunt in a patient with liver cirrhosis and hypertrophic caudal lobe.

Spontaneous splenorenal shunt is a rare condition, sometimes causing complications in cirrhotic patients. We report a 30-year old man with liver cirrhosis, hypertrophic caudal lobe and spontaneous splenorenal shunt. Real-time and color Doppler ultrasonography evidenced enlarged caudal lobe (130 x 95 mm) with direct veins draining into dilated inferior cava vein (diameter 25 mm, flow 52 cm/sec). In the left renal hilus a large vein with a flow typical for portal vein system was found, velocity 25-37 cm/sec. Indirect splenoportography noticed splenomegaly, dilated lienal and portal vein with hepatopetal blood flow, perisplenic varices, and large spontaneous splenorenal shunt. Whole inferior caval vein was dilated, while hepatic veins were intact. Hemodynamic consequences of this large shunt were dilation of inferior cava vein with hyperkinetic systemic flow, and secondary hypertrophy of liver caudal lobe.

Adult↗

Ultrasonography and computed tomography in the diagnosis of portomesenteric vein thrombosis.

This study was undertaken to evaluate ultrasonography (US) and computed tomography (CT) in the diagnostics of portomesenteric vein thrombosis (PMT). Between 1972-1988 we found 40 cases with PMT, all verified in angiography and/or autopsy. Between 1972-79 US and CT were never used, when 19 cases with PMT were found (1 premortem diagnosis with splenoportography). Between 1980-88 there were 21 patients with PMT. US was performed in 13, detecting PMT in 2. CT was performed in 5 patients confirming US diagnosis in 2 cases and revealing one further PMT. One PMT was diagnosed in the venous phase of an arteriography. The sensitivities of US and CT to detect PMT were 15% (2/13) and 60% (3/5) (p less than 0.05). The delay until diagnosis decreased from 27 +/- 28 days in 1972-79 to 12 +/- 9 days in 1980-88 (p less than 0.02). However, neither the rate of correct antemortem diagnosis (5% vs 19%) nor the survivals (68% vs 65%, 95% vs 81%, 95% vs 100% at 1, 12 and 60 months) were improved. Because only the patients with correct diagnosis received appropriate therapy, followed by longest survivals, the importance of the diagnostic attempts should not be questioned. CT may be more sensitive than US for detecting PMT, but neither method have yet replaced angiographic techniques.

Evaluation Studies as Topic↗

[Characteristics of angiohepatography using an oily contrast medium in the diagnosis of hepatic echinococcosis].

A comparative assessment of different methods of angiography for diagnostics of echinococcosis of the liver is given. Conditions of prolonged contrast of vessels and parenchyma of the organ were created by splenoportography and arteriography of the liver with maiodyl which allows to determine the presence and degree of morphofunctional changes and spread of the pathological process in echinococcosis of the liver.

Contrast Media↗

[Angiography in abdominal diseases (author's transl)].

Angiography of arteries and veins in the abdominal and retroperitoneal compartement is a valuable routine roentgenologic procedure. Its value in the diagnosis of vascular disorders is apparent. Regarding space occupying lesions in the abdomen angiography is an aid in diagnosis and differential diagnosis and provides information on the curability. Visualization of the venous system will give mainly information on the extent of a tumor. For an evaluation of portal hypertension the arterial indirect visualization of the portal venous system is superior to the direct percutaneous splenoportography. In the acute gastrointestinal bleeding selective angiography is able to demonstrate the bleeding site.

Abdomen↗

Sinistral (left-sided) portal hypertension.

Between 1953 and 1988, 21 patients with splenic vein thrombosis (SVT), 12 of whom had sinistral portal hypertension (SPH) were treated at our institution. SVT was identified at autopsy in nine patients. Twelve additional patients presented with SPH: bleeding esophageal varices, SVT and normal hepatic function. SVT was caused by pancreatic neoplasm (5), chronic pancreatitis (5), and pancreatic pseudocyst (2). SVT was diagnosed by splanchnic angiography, splenoportography, computerized tomography, and ultrasonography. Gastric varices were diagnosed by endoscopy (10) and barium swallow (2). Splenectomy was performed as primary therapy in 10 patients. Three of these 10 had en block distal pancreatectomy. Two high-risk patients had splenic artery embolization, one as a prelude to splenectomy performed 48 hours later and the other as definitive therapy. One splenectomized patient continued to bleed. No further bleeding occurred in 10 splenectomized patients in follow-up from 1 week to 14 years. Sinistral portal hypertension is a clinical syndrome of splenic vein thrombosis caused by pancreatic pathology and manifests as bleeding gastric varices in patients with a patent portal vein and normal hepatic function. Splanchnic arteriography is necessary for accurate diagnosis. Splenectomy is the effective treatment of choice.

Adult↗

[Segmental portal hypertension caused by splenic vein thrombosis].

Seventeen cases of segmental portal hypertension due to splenic vein thrombosis are reported. This syndrome may be asymptomatic for a long time and then present suddenly in the form of a serious picture of high digestive haemorrhage due to rupture of gastric fundus varices as a result of hypertrophied submucous collateral drainage circulation. Useful for diagnosis are oesophagogastroduodenoscopy, which points to stomach varices, and splenoportography or splenic artery angiography with venous phase, which highlight pathognomonic dilatation and tortuosity of the gastroepiploic veins. Surgical exploration typically shows: presence of large epiploic vessels, splenomegaly, absence of changes in the liver and in the portal and mesenteric circulation. Curative treatment of choice is splenectomy.

Adult↗

Gastrointestinal tract angiography in infants and children.

In the past 10 years since the development of newer imaging modalities, the method of evaluation of gastrointestinal diseases has changed to less invasive examinations. Angiography of the gastrointestinal tract and its accessory organs, which once was one of the primary procedures for hepatic tumors especially, is now considered nonessential except for occasional demonstration of vascular distribution prior to attempted surgery. As for gastrointestinal bleeding, although the incidence of ulcer disease is not as common in children as in adults, a child with bleeding who is at high risk for surgery will benefit occasionally from intra-arterial infusion of vasopression. As the major cause of gastrointestinal bleeding in children is from esophageal varices, though great anatomical detail of portal circulation can be seen in computed tomography and ultrasound, the coronal mapping of vascular anatomy of portal circulation is of utmost benefit prior to any attempt for surgery. Intra-arterial portography and splenoportography can be very helpful to delineate the anatomy and hemodynamics of portal hypertension, as well as for evaluation of suspected shunt thrombosis. Briefly, an update of information on digital subtraction angiography in gastrointestinal pathology will be given though the pediatric application has not been as popular as in adults.

Abdominal Injuries↗

[Correlations between splenoportographic findings and risk of digestive hemorrhage in portal hypertension].

In 102 cases examined by splenoportography (93 cirrhosis with patent splenoportal axis and 9 splenoportal thrombosis without appreciable liver changes), the occurrence of episodes of digestive haemorrhage was statistically correlated with age, sex, type of portal impediment, type of collateral circulation, and the presence or otherwise of ascites, and with portal pressure values measured by splenomanometry. The only statistically significant correlation was that concerning the type of hepatofugal collateral circulation, as digestive haemorrhage proved gradually less frequent in patients with gastro-oesophageal collateral circulation, in those with so-called infraportal collateral circulation and, finally, in those where angiography was unable to demonstrate the presence of any collateral circulation at all.

Adolescent↗

[Problems in emergency surgery: segmental portal hypertension].

Three typical cases of segmental portal hypertension due to occlusion of the splenic vein are reported. This syndrome may be asymptomatic for a very long time and then present suddenly in the form of a serious picture of high digestive haemorrhage due to rupture of the varices of the fundus of the stomach as a result of hypertrophized submucous collateral drainage circulation. Useful for diagnosis are oesophagogastroduodenoscopy, which points to stomach varices, and splenoportography or superselective arteriography of the splenic artery with venous phase, which highlight pathognomonic dilatation and tortuosity of the gastroepiploic veins. Surgical exploration typically shows: presence of large epipolic vessels, splenomegaly, absence of changes in the liver and in the portal and mesenteric circulation. Resolutive treatment of choice is splenectomy.

Adult↗

[Hemodynamic studies of portal hypertension with esophageal varices; a role of left gastric artery and vein].

Hemodynamic states of portal hypertension with esophageal varices were studied by scintiphoto splenoportography (SSP) and left gastric angiographies in relation to endoscopic findings. The cases were classified into two groups by SSP. The flow of left gastric vein was hepatofugal in Group I (77.3%), and it was hepatopetal or "to and fro" in Group II (22.7%). Endoscopically, the varices were more severe in Group I than those in Group II. The diameter of left gastric vein was significantly larger in Group I. The values of K.ICG and liver function by blood analysis were also poor in Group I. Moreover, the cases with varices supplied by both left gastric artery and vein showed more severe endoscopic findings and history of hematemesis than those in the cases with varices supplied by left gastric artery alone. In conclusion, the results suggested that the flow of left gastric vein was closely related to the severity of esophageal varices.

Adult↗

Unselective end-to-side proximal left-gastric-caval shunt without splenectomy. Indications and technique.

From 1976 to 1982, 104 patients with bleeding esophageal varices were treated surgically. In three patients a wide left gastric vein without portal flow was demonstrated by means of arterial splenoportography. Ascites estimated at between 4-to-6 liters was observed in two patients. Anemia, leukopenia and platelet counts below 85 X 10(9)/l were shown in all of three. Liver panangiography was performed on all three patients. Given the absence of portal flow, the presence of intractable ascites and the demonstration of a wide left gastric vein, we dissect this vein disconnecting it from the stomach, and performing the anastomosis between the proximal end of this vessel and the inferior vena cava (proximal unselective left gastric caval shunt). Ascites and esophageal varices disappeared in our three patients between one and three weeks after the operation. The shunts were patent between eight months to eight and a half years after the operation. All three patients are still living.

Esophageal and Gastric Varices↗

An unusual portosystemic shunt in a dog.

An unusual portosystemic shunt was diagnosed in a 4-month-old male Miniature Poodle examined because of stunted growth and episodes of dementia characterized by hysteria, ataxia, and staggering. Operative splenoportography revealed a portosystemic shunt. Exploratory surgery to identify and correct the shunt was attempted. Evidence of any portal circulation was not detected, and the dog was euthanatized. The possible embryologic basis for this vascular anomaly is discussed.

Animals↗

[Diagnostic up-date in thrombosis of the portal vein].

Portal thrombosis is a rare acquired condition with various etiology. Clinical manifestations are portal hypertension or nine. The main technics to diagnostic are: CT, indirect splenoportography and ultrasound examination. The first and the second are reliable but the third only is useful to explore the portal system and to evaluate patients with suspect portal vein thrombosis.

Aged↗

Modified distal splenorenal shunt with expanded polytetrafluoroethylene interposition.

Two types of modified distal splenorenal shunt with expanded polytetrafluoroethylene (Gore-Tex; WL Gore & Associates Inc., Elkton, Md.) interposition were performed in 18 consecutive patients with esophageal or esophagogastric varices. There were 12 men and six women ranging in age from 32 to 76 years. The causes of portal hypertension were cirrhosis of the liver in 15 patients, chronic hepatitis in two, and idiopathic portal hypertension in one. In five patients the left gastric vein branched off from the splenic vein; bilateral gastric venous decompression was achieved by preserving the splenic vein. Porta-azygos disconnection was routinely performed by confirming repeated intraoperative direct splenoportography. The operations were elective in seven and were emergencies in five patients. Six patients underwent a prophylactic shunt; all patients had "red color signs" endoscopically, and three of them had concomitant hepatocellular carcinoma. Postoperative morbidity was minimal and there was no mortality. Shunt patency was confirmed angiographically in all patients 14 to 56 days after surgery. The varices disappeared or significantly improved in all patients. No patients had variceal bleeding postoperatively. Hepatic encephalopathy was transiently seen in one (the oldest) patient.

Adult↗

[Diagnosis and treatment of non-parasitic cysts of the spleen in children].

The experience with the treatment of non-parasitic cysts of the spleen in children has shown that although careful assessment of the clinical and case-history data facilitate the correct diagnosis the leading role should be given to laparoscopy fulfilled if necessary in combination with splenoportography or puncture of the splenic cyst. Splenectomy is the operation of choice for cysts of the spleen in children.

Child↗

[Radiology of portal hypertension (author's transl)].

Description of the applications of radiology to portal hypertension, mainly of the 2 most common conditions, the secondary prehepatic block and the intra-hepatic block in cirrhosis of the liver. In prehepatic block the veins in the splanchnic area which will have to be anastomosed to relieve pressure must be shown. This can be done with direct and indirect spleno-portography and by contrasting the proximal and, possibly, the distal mesenteric veins. For intrahepatic postsinusoidal block of cirrhosis of the liver the portal vein and its major contributaries must be shown. In addition, circulation and pressure in the liver and its surroundings have to be elucidated. For this, catheterisation of the umbilical vein and the veins of the liver are suitable. In addition splenoportography and mesentericography have to be used, when transumbilical portohepatography cannot be done.

Catheterization↗

Hepatoportography via the umbilical vein: a superior approach to diagnosis in liver disease.

The umbilical vein in adults is patent but collapsed. There is a membranous valve at its entrance into the left portal vein. Cannulation of the portal vein via the umbilical vein permits direct access to the portal system for portography and hepatography. This procedure was performed under local or general anesthesia in 30 patients and was successful in 22. It is useful in the investigation of patients with portal hypertension, and suspected intrahepatic tumours or abscesses. It gives excellent contrast visualization of the liver and definition of lesions as small as 1.0 cm. This technique is superior to both hepatic scanning and splenoportography.

Aged↗