Renal vein thrombosis in newborn infants

The authors reviewed renal venous thrombosis (RVT), a frequent cause of acute renal failure in newborns. Clinical manifestations and prognosis are unsteady and, although there is agreement about the management of underlying conditions and general abnormalities, the relative role of anticoagulant and fibrinolytic therapy has not been clearly established. The 5 RVT described cases were treated in our hospital in the last 5 years. Four out of five were followed and one of these was submitted to nefrectomy to control arterial hypertension. The findings are compared to literature and present therapy is discussed.     

Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis?

The aetiopathology of extrahepatic portal vein obstruction is unknown. In retrospective studies, umbilical vein cannulation and sepsis have been alleged to cause portal thrombosis. This prospective study was undertaken to detect whether thrombosis and consequent obstruction of the splenoportal venous system develops after umbilical vein catheterization for exchange transfusion in newborns using Doppler ultrasound. Forty children (M = 24; F = 16) who had undergone exchange transfusion for hyperbilirubinaemia were studied at school age. Maximal duration of the venous umbilical cannulation was 120 min and sepsis did not occur. Clinical, biological and sonographic examinations were normal, except in 3 children. In 2 the left branch of portal vein could not be identified (normal variant). Conclusion Our results show that, in these children, umbilical vein catheterization did not lead to development of portal vein thrombosis. However, when other risk factors such as umbilical infection, traumatic catheterization are associated, children should be screened for obstruction of the portal vein. Received: 27 August 1997 and in revised form: 28 March 1997 / Accepted: 5 September 1997     

Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach

OBJECTIVES: Current management of extrahepatic portal vein thrombosis (EPVT) comprises endoscopic eradication therapy of esophageal varices and conventional shunt surgery. The authors have used the novel technique of mesenterico-left portal bypass (Rex shunt) in seven children with symptomatic EPVT, and report their results here. METHODS: Median age of the children was 12 years (range, 2-16 years). All children had portal hypertension with hypersplenism and recurrent bleeding from esophageal varices. Furthermore, one patient suffered from a severe hepatopulmonary syndrome. Preoperative evaluation included liver function tests, liver biopsy, hepatic duplex ultrasonography, and radiologic evaluation of the intrahepatic and extrahepatic vascular anatomy. The internal jugular vein was used as vein graft in all patients. RESULTS: Median follow-up period was 15 months (range, 3-28 months). Ultrasound scans revealed sufficient perfusion in all shunts (median, 35 cm/s; range, 28-60 cm/s). The intrahepatic portal perfusion in segment 4 improved from a median of 6 cm/s before surgery to 18 cm/s postoperatively. The platelet count increased within 3 months from a mean of 50,625/microL to 137,750/microL. The clinical signs of hypoxemia in the child with hepatopulmonary syndrome disappeared within 6 months. CONCLUSIONS: In accordance with the limited experience published by others, the authors' data confirmed the mesenterico-portal Rex shunt as the therapy of choice for children with EPVT. Furthermore, this report is the first to show that a hepatopulmonary syndrome can be abolished by mesenterico-portal Rex shunt.     

Portal vein thrombosis complicating neonatal hepatic abscess

Hepatic abscess in a neonate is a rare but serious disorder. Diagnosis of hepatic abscess requires a high index of suspicion in any septic neonate. CT scan and ultrasound of liver are the most sensitive diagnostic tests in detection of hepatic abscess. Portal vein thrombosis and portal cavernoma formation is hitherto unreported complication of neonatal hepatic abscess in English literature. Present case report highlights the difficulty in diagnosis of neonatal hepatic abscess and describes the development of portal vein thrombosis and cavernoma during its treatment.     

Renal vein thrombosis in the newborn infant.]

In a child born with traumatism to a diabetic mother, an acute thrombosis of the left renal vein occurred. An arteriography performed at the acute stage, established the diagnosis. The newborn received urokinase and heparin administered through an arterial catheterism. An arteriography, repeated at the 3rd day, showed an improvement in the vascularization of the kidney, which returned to normal functions. The radiological symptons of renal vein thrombosis are reviewed including the arteriography which is never used for the diagnosis in newborns. The interest of the use of urokinase and its efficiency in this disorder are discussed. This therapy harmless and efficient seems of interest. Such a therapy has to be tried because of the failure of isolated heparin-therapy and of the sequellae which may be observed after spontaneous recovery or after anticoagulant treatment.     

Renal vein thrombosis and response to therapy in a newborn due to protein C deficiency

A newborn infant was diagnosed as having renal vein thrombosis due to heterozygous protein C deficiency and no other predisposing factor for thrombosis. He responded adequately to treatment with streptokinase, fresh frozen plasma and subsequent anticoagulation with heparin and warfarin (Coumadin). Four years from diagnosis he remains well with no recurrence of thrombosis and with normal renal function.     

Extra hepatic portal vein thrombosis in a child associated with lupus anticoagulant

The presence of lupus anticoagulant has been implicated in venous as well as arterial thrombosis. We report here a 10-year-old boy who presented to us with hematemesis, malaena and splenomegaly. An ultrasound showed a recanalized portal vein with collaterals suggestive of portal vein thrombosis. He had grade IV esophageal varices. The liver function tests were normal. Investigations for prothrombotic factors showed that tests for PNH and for APC resistance were negative. Levels of anti-thrombin II and protein C were normal. There was a prolonged activated partial thromboplastin time with a normal prothrombin time. Presence of lupus anticoagulant was confirmed with dilute Russell viper venom time and platelet neutralization test. Repeat tests after 10 weeks showed persistence of the lupus anticoagulant. ELISA test for anti-phospholipid antibody was negative. The association of lupus anticoagulant with portal vein thrombosis in the pediatric age group is very rare.     

Neonatal portal vein thrombosis: diagnosis and management

Neonatal portal vein thrombosis (PVT) is an increasingly recognized event. Patients are generally asymptomatic in the neonatal period. The diagnosis is made with Doppler ultrasound. Umbilical catheterization, exchange transfusion and sepsis are risk factors for neonatal PVT. Thrombophilia is possibly a contributing risk factor. Although there are potential serious acute complications such as hepatic necrosis, the outcome is good in the majority of cases, followed up to 8 years of age. Thrombus resolution occurs in 30-70% in days to months. Liver lobe atrophy may occur following PVT, and does not appear to be associated with any impairment of liver function. Non-occlusive thrombosis is more likely to resolve than non-occlusive thrombosis. A subset of patients without resolution is at risk for developing portal hypertension over the next decade of life. There are no current defining features present during the neonatal period to enable identification of neonates at risk for portal hypertension. There is no evidence that anticoagulation therapy improves time to resolution or decreases the likelihood of portal hypertension. Anticoagulation therapy may be considered. A management algorithm is proposed.     

Acute intrahepatic portal vein thrombosis complicating cholangitis in biliary atresia

A 2-year-old boy with a Kasai portoenterostomy had systemic features suggestive of cholangitis and acute portal vein obstruction. The rapid rise in portal pressure caused a transient, severe decompensation of hepatic function. A superior mesenteric arterial angiogram confirmed the presence of diffuse intrahepatic portal vein thrombosis. Conclusion This case provides clinical and radiological evidence supporting an association between ascending cholangitis and acute intrahepatic portal vein thrombosis. Received: 7 May 1996 / Accepted: 3 September 1996