Extrahepatic Portal Vein Thrombosis Research Articles

Attention span deticits in children with extrahepatic portal vein thrombosis

Attention span deticits in children with extrahepatic portal vein thrombosis

Coagulation abnormalities in pediatric extrahepatic portal vein thrombosis- is REX shunt the cure?

Coagulation abnormalities in pediatric extrahepatic portal vein thrombosis- is REX shunt the cure?

Coagulation abnormalities in pediatric extrahepatic portal vein thrombosis- is REX shunt the cure?

Coagulation abnormalities in pediatric extrahepatic portal vein thrombosis- is REX shunt the cure?

Attention span deticits in children with extrahepatic portal vein thrombosis

Attention span deticits in children with extrahepatic portal vein thrombosis

Portosystemic shunting in children during the era of endoscopic therapy: improved postoperative growth parameters.

Surgical portosystemic shunting has been performed less frequently in recent years. In this retrospective study, recent outcomes of portosystemic shunting in children are described, to evaluate its role in the era of endoscopic therapy. Retrospective chart review of children who underwent surgical portosystemic shunt procedures between October 1994 and October 1997. Twelve children (age range, 1-16 years) underwent shunting procedures. The causes of portal hypertension were extrahepatic portal vein thrombosis (n = 6), congenital hepatic fibrosis (n = 2), hepatic cirrhosis (n = 2), and other (n = 2). None of the patients were immediate candidates for liver transplantation. Types of shunt included: distal splenorenal (n = 10), portocaval (n = 1), and other (n = 1). Median follow-up was 35 months (range, 24-48 months). All patients are currently alive and well with patent shunts. The mean hospital stay was 8 days. Three patients required readmission for further interventions because of shunt stenosis in two and small bowel obstruction in the other. Mild portosystemic encephalopathy was seen in one child with pre-existing neurobehavioral disturbance. Excluding a patient who underwent placement of a portosystemic shunt for a complication of liver transplantation, mean weight-for-age z score in nine prepubertal patients improved from -1.16 SD to +0.15 SD (P = 0.023), and mean height-for-age z score from -1.23 SD to 0.00 SD (P = 0.048) by 2 years after surgery. Surgical portosystemic shunting is a safe and effective method for the management of portal hypertension in childhood. Patients show significant improvements in growth parameters after the procedure. Surgical portosystemic shunting should be actively considered in selected children with portal hypertension.

Experience with the Rex shunt (mesenterico-left portal bypass) in children with extrahepatic portal hypertension

Extrahepatic portal vein thrombosis (EPVT) in children can lead to severe bleeding from gastrointestinal varices, ascites, thrombocytopenia from hypersplenism, and other coagulation disorders. The authors have used the superior mesenteric vein to intrahepatic left portal vein (Rex) shunt in 5 children with symptomatic EPVT and report their results with this novel technique. Children with symptomatic portal hypertension were screened for the underlying cause. All children with essentially normal livers and obstruction of the extrahepatic portal vein were considered for the Rex shunt. Evaluation included liver function tests, liver biopsy, and radiological evaluation of the intrahepatic vascular anatomy. Five patients between the ages of 2.8 and 10.5 years underwent evaluation for portal hypertension secondary to extrahepatic portal vein obstruction. Three patients had idiopathic extra hepatic portal vein thrombosis with cavernous transformation, 1 had thrombosis after a living-related liver transplant, and 1 had compression and obstruction of the main portal vein from enlarged lymph nodes after treatment of systemic histoplasmosis. All patients were symptomatic. Three patients had intermittent bleeding from esophageal and gastric varices, and all 5 had relative degrees of hypersplenism with enlarged spleens and thrombocytopenia (11,000 to 77,000). Three patients had significant leukopenia. Results of imaging studies suggested that 3 patients had inadequate intrahepatic portal veins for shunting, but all patients at exploration underwent successful shunting. There were no serious intraoperative complications. Postoperative complications included ascites in 2 patients that resolved within 1 month. There were no early shunt thromboses. The median postoperative length of stay was 7 days. Clinical follow-up ranged from 7 to 21 months. Gastrointestinal bleeding did not recur in any patient, and ascites resolved in all. Spleen size decreased significantly (P < .01) from 9.4 +/- 4.0 cm to 5.0 +/- 3.7 cm below the left costal margin. Mean platelet count and white blood cell count rose after shunting from 79 +/- 42 to 176 +/- 73 (P < .02) and 5.4 +/- 2.3 to 7.5 +/- 3.9 (P = .06), respectively. All shunts were studied at 1 and 7 days, and 3 and 6 months after the procedure. Shunt patency was documented in all cases. Subsequently, shunt blockage occurred in 2 patients. The Rex shunt has proven to be an effective method of resolving portal hypertension caused by EPVT including thrombosis after living donor transplantation. This shunt is preferable to other surgical procedures because it eliminates portal hypertension and its sequelae by restoring normal portal flow to the liver.

Autonomic neuropathy in extra-hepatic portal vein thrombosis: evidence for impaired autonomic reflex arc

Background/Aims: Automatic dysfunction is common in cirrhosis, and may be associated with increased mortality and hyperdynamic circulatory changes. Our aim was to investigate whether autonomic disturbances occur in extrahepatic portal vein thrombosis and their correlation with hemodynamic abnormalities. Patients and Methods: Heart rate variation in response to standing, deep breathing, and Valsalva maneuver, and blood pressure response to sustained handgrip and to standing, were studied in 16 subjects with portal vein thrombosis (10 males, 30.8±2.8 years: mean±SE), 12 with cirrhosis (7 males, 52±2.3 years), and 10 healthy controls (7 males, 30.8±3.0 years). Supine resting, and 10- and 30-min standing epinephrine and norepinephrine levels were measured and results correlated with cardiac output. Results: Automatic dysfunction occurred in 62% of portal vein thrombosis and 75% of cirrhosis subjects, but in no controls ( p<0.02). Similarly, postural hypotension occurred in portal vein thrombosis (−10.25±0.65 mmHg, p+0.003) and cirrhosis (−7.42±0.82 mmHg, p=0.007) but not in controls. All groups had similar baseline epinephrine and norepinephrine concentrations. Epinephrine increased significantly in controls (45%, p<0.01 and 49%, p<0.02) after 10 of 30 min standing but not in the portal vein thrombosis of the cirrhotic groups, and norepinephrine increased after 10 and 30 min standing in cirrhotics (128%, p<0.004 and 130%, p<0.008) and controls (129%, p<0.002 and 116%, p<0.004), but not portal vein thrombosis (34.5% and 39%, NS vs baseline). Portal vein thrombosis and cirrhosis groups had increased cardiac output (4441±509 and 3262±292) vs controls (1763±212 ml/min/m 2, p<0.002), but there was no correlation with autonomic neuropathy or with catecholamine levels. Conclusions: Automatic dysfunction and impaired techolamine response to orthostatic stress occur commonly in portal vein thrombosis and suggest an impairment of the authonomic reflex arc, but changes do not correlate with hemodynamic abnormalities.

Selective distal splenorenal shunts for intractable variceal bleeding in pediatric portal hypertension

The treatment of portal hypertension in the pediatric population has undergone an evolution toward less invasive methods of care. With the advent of endoscopic sclerotherapy, surgery is less common in the acute care of these patients. Few reports deal with the role of portosystemic shunting in the emergent management of variceal hemorrhage in children. To address this issue, the authors studied the medical records of all pediatric patients at their institution who underwent placement of a shunt for portal hypertension during the last 10 years. Nine patients underwent a total of 10 emergent or semiurgent shunting procedures. Seven were boys and two were girls. Six patients had portal hypertension as a result of intrahepatic disease. Two had extrahepatic portal vein thrombosis. Five children had abnormal hepatic function. The median age at the time of the procedure was 9 years. The indication for surgical shunting in all cases was gastrointestinal hemorrhage not responsive to sclerotherapy. Eight patients underwent emergent distal splenorenal shunts (DSRS), and two underwent a nonselective mesocaval shunt, with one undergoing both. Postoperatively all patients had cessation of bleeding. Operative mortality was zero. Early complications included ascites (3), small bowel obstruction (1), and hepatorenal syndrome (1). The child who underwent a nonselective shunt procedure had encephalopathy. Two DSRS thrombosed, requiring reexploration; eight shunts remained patent. Three patients eventually had orthotopic liver transplantation (OLT) because of progressive hepatic failure. Two children died; neither death was shunt related. This study suggests that selective shunts still have a role in the care of pediatric patients with portal hypertension. The DSRS is well tolerated and is associated with a high survival rate among children with variceal bleeding unresponsive to medical management. It has minimal morbidity, with good patency, and can be a life-sustaining measure in children before OLT.

Long term outcome after surgery for extrahepatic portal vein thrombosis: P.D. Losty, M.J. Lynch, and E.J. Guiney. Arch Dis Child 71:436–440, (November), 1994

Long term outcome after surgery for extrahepatic portal vein thrombosis: P.D. Losty, M.J. Lynch, and E.J. Guiney. Arch Dis Child 71:436–440, (November), 1994

Long term outcome after surgery for extrahepatic portal vein thrombosis.

The long term outcome of 21 children with extrahepatic portal hypertension secondary to portal vein thrombosis managed by surgical intervention was evaluated. Portosystemic shunts, used primarily in nine patients (eight central splenorenal, one mesocaval) after conservative treatment had failed, had no associated mortality and a 56% patency rate. Five of these shunted patients had no further bleeding episodes and did not show encephalopathic impairment. Direct attack procedures-portoazygos operation (four patients) was associated with significant complications, including one fatality. Other direct approaches-oesophageal transection and variceal plication (five patients) had variable outcome. Splenectomy alone (three patients) ameliorated hypersplenism; however, further surgery for recurrent haemorrhage (two patients) was necessary. Endoscopic sclerotherapy controlled recurrent variceal bleeding (three patients) when it became available to the unit. Conservative treatment practised in five children had little success: two patients survived, two died from further haemorrhage, and one was lost to follow up. These results suggest that in centres without endoscopic expertise, and for patients who are sclerotherapy 'failures', surgery can be performed safely and achieve reasonable long term success rates in childhood extrahepatic portal hypertension.

A Comparative Clinical Study of Idiopathic Portal Hypertension, Extrahepatic Portal Vein Thrombosis, and Cirrhosis

The relationship between idiopathic portal hypertension (IPH) and extrahepatic portal vein thrombosis (EPVT) remains controversial. In a retrospective study, we compared the clinical features of 21 patients with IPH, 27 with EPVT, and 38 with liver cirrhosis (LC). The variables with statistical difference between IPH and EPVT were age (38 +/- 17 years versus 29 +/- 14, respectively; p < 0.05), and 5-year survival (72 versus 92%, respectively; p < 0.05). The other clinical and laboratory variables had similar distribution in IPH and EPVT. LC patients were older than those in the other groups (48 +/- 13 years, p < 0.05), and had a poorer 5-year survival (60%, p < 0.05). Furthermore, LC patients had clinical features of liver failure in a higher proportion than the other groups did. The results suggest that IPH, EPVT, and LC are separate clinical entities.

Surgical management of extrahepatic portal hypertension and variceal bleeding.

Variceal bleeding remains an important complication in extrahepatic portal vein thrombosis (EPVT). As for portal hypertension due to other etiologies, an elective treatment to decrease the risk of subsequent rebleeding is warranted. The results of the Sugiura-Futagawa procedure (SP) in 38 patients with variceal bleeding secondary to EPVT are reported: 20 women and 18 men, with a mean age of 28 +/- 2 years (SEM). Thirty-seven patients were classified as Child-Pugh class A, and one patient as class B. In terms of diagnosis, 45% of patients had idiopathic EPVT, and 18% had associated hypercoagulability disorders; 52% of patients had associated splenic vein thrombosis. The SP was completed in two surgical stages in 18 patients and in one surgical stage in 14; 6 patients had only the abdominal stage. One patient had mild postoperative encephalopathy, and three patients rebled at long-term follow-up study. There were two operative deaths. Actuarial survival was 70% at 64 months. It is concluded that the SP is an excellent alternative for patients with variceal bleeding secondary to EPVT.

Use of distal splenorenal shunt in children referred for liver transplant evaluation

Variceal bleeding remains a common cause of morbidity for children with both intrahepatic and extrahepatic portal hypertension. Occasionally, patients referred for liver transplant evaluation have significant variceal bleeding, despite adequate synthetic liver function. During a 7-year period, 322 children were referred for liver transplant evaluation. Six underwent distal splenorenal shunt surgery after evaluation. There were four boys and two girls. The average age was 11 ± 4 years, and the average weight was 39 ± 15 kg. The etiology of variceal bleeding was intrahepatic portal hypertension in five (1 biliary atresia, 2 chronic hepatitis, 2 congenital hepatic fibrosis) and extrahepatic portal vein thrombosis in one. Two patients had no previous attempts at sclerotherapy (one because of an abnormality in platelet function, the other because of extensive gastric varices), and four had multiple previous sclerotherapy treatments. No patient had preoperative encephalopathy. Three cases were Child's class A, and three were Child's class B. Preoperative evaluation of the portasystemic system was performed with magnetic resonance (MR) imaging or splenoportography. All patients underwent a distal splenorenal shunt procedure, four of whom also had splenopancreatic disconnection. One patient required 100 mL of blood replacement, and five required no blood. The average length of hospital stay was 9.8 ± 2.2 days. Postoperative complications were minimal. All patients are alive, without recurrent gastrointestinal bleeding or encephalopathy, and they have patent shunts, which was confirmed by MR or Doppler ultrasound at a mean of 25 ± 20 months after shunt surgery. Children who have significant cirrhosis, portal hypertension, recurrent variceal bleeding refractory to sclerotherapy, and adequate synthetic function can be effectively treated with a distal splenorenal shunt. While the choice between liver transplantation of shunting may be difficult, use of the distal splenorenal shunt does not preclude future liver transplantation for such children.

Idiopathic acute portal vein thrombosis: A case report

There are few reported cases of acute portal vein thrombosis presenting as an acute abdomen in adolescent age group. Most published series concern chronic extrahepatic portal vein thrombosis. Acute portal vein thrombosis is rare, but can develop into serious complications. Hence, prompt diagnosis and heparinization can prevent the development of lethal complications such as venous gangrene of the bowel and portal hypertension.

Hepatic encephalopathy induced by small bowel obstruction in a noncirrhotic child with portal vein thrombosis

A case of hepatic encephalopathy in a noncirrhotic child, who has undergone a previous mesocaval shunt for extrahepatic portal vein thrombosis, is presented. Hepatic encephalopathy developed 5 years after the operation and is believed to have been precipitated by the presence of small bowel obstruction. Exploratory laparotomy and lysis of an adhesion relieved the obstruction and led to the resolution of the encephalopathy.

Management of variceal bleeding in patients with extrahepatic portal vein thrombosis

Patients with extrahepatic portal vein thrombosis may present from infancy through adulthood with variceal bleeding. Physiologically, such patients differ from patients with cirrhosis and variceal bleeding in that they have a normal liver and maintain good portal perfusion through hepatopedal collaterals. Complete evaluation of these patients requires identification of the bleeding site by endoscopy, definition of the anatomy by angiography, and confirmation of a normal liver by biopsy examination. Causative factors, including hypercoagulable states, should be evaluated. Therapeutic options range from noninterventive, through ablative procedures, to shunt operations. The goal should be definitive control of bleeding and return to a normal lifestyle. Distal splenorenal shunt offers the best option if technically feasible, but if no shuntable veins are patent, ablative procedures and sclerotherapy may be required. A noninterventive, noninvestigational approach is inappropriate in patients who can be offered definitive therapy. Splenectomy for hypersplenism should not be done in these patients.

Management of variceal bleeding in patients with noncirrhotic portal vein thrombosis.

Since 1971, 70 patients have been seen at Emory University Hospital with gastroesophageal varices secondary to extrahepatic portal vein thrombosis (PVT). Thirty-seven of these patients had had prior major operative therapy. In only three patients (8%) was shunt surgery successful, and there was a high incidence of rebleeding, other morbidity, and mortality. Of especial note are the serious consequences of simple splenectomy; splenomegaly and thrombycytopenia should rarely, if ever, be used as indication for splenectomy in portal hypertension. In 1977, the use of selective distal splenorenal shunt (DSRS) was begun at Emory in this population and a selective shunt has been possible in 24 of 29 patients (83%) who had had no prior operative therapy. Results have been excellent with a greater than 90% patency rate, long-term portal perfusion in all, no encephalopathy, and late rebleeding in one patient. Quantitative studies at 3-6 years show stability of liver function, significant decrease in spleen size, and rise in platelet count. However, long-term follow-up (greater than 15 years) is required in PVT patients before definitive assessment can be obtained. A specific problem of the PVT patient is late shunt stenosis which requires close observation; dilatation of the shunt was performed in six of the 24 patients with a patent shunt. Poor results with non-shunt operative procedures in PVT were again documented. The proper role of endoscopic variceal sclerotherapy is not yet clear, but appears to be an excellent addition to the therapeutic options. In conclusion, for patients with a patent splenic vein, initial therapy should be a selective shunt; for patients without a patent splenic venous system, endoscopic sclerotherapy is the procedure of choice.