Neonatal portal vein thrombosis (PVT) is uncommon with potentially serious complications that may manifest in infancy and childhood.

The primary aim of our study was to describe the short-term and long-term outcomes of neonatal PVT.

A retrospective chart review was conducted from 2008 to 2016 of neonates diagnosed with PVT. A systematic review was also performed from 2000 to 2018 to evaluate anticoagulant therapy (ACT) in neonatal PVT.

Forty-four premature and 30 term infants (mean gestational age 30.7 vs 39.1 weeks, respectively) had PVT. Sixty-eight involved the left portal vein, one involved only the main portal vein, and 5 involved ≥1 vein. PVT was catheter associated in 46 (62%); none of the 7 neonates tested had thrombophilia. Of 74 neonates, 19 (26%) received ACT and 55 (74%) were untreated. The mean follow-up duration was 16.6 months (SD = 17.62; range, 0-89.6); 59.5% were followed for ≥6 months. On last ultrasound examination, thrombus resolution was documented in treated (ACT; n = 19) and nontreated (n = 55) neonates: 12 (63%) versus 32 (58%) with complete resolution, 1 (5%) versus 6 (11%) partial, 0 versus 1 (2%) extension, and 6 (32%) versus 16 (29%) had nonprogressive lesions, respectively. Seventy-one (96%) had no complications. Seventy-one articles met inclusion criteria for the systematic review and 19 were retained for analysis after assessment.

PVT resolution rate was similar to previous reports. Although a low complication rate was detected, longer follow-up is necessary to determine the need for early treatment and the precise incidence of outcomes such as portal hypertension.

The aim of the study was to better describe incidence, risk factors, and the natural evolution of neonatal portal vein thrombosis (PVT).

One hundred and twenty-three premature newborns or with birth weight <1.5 kg were prospectively included in a single center during a one-year period. Three systematic abdominal ultrasound examinations at day 3, day 10, and day 45 (and 1 year in case of persistent PVT) were performed. Clinical and biological data were recorded.

Seventy neonates (57%) had three normal US examinations. Fifty-three neonates (43%) had a clinical and biological asymptomatic left PVT. No right or extrahepatic portal venous thrombosis was observed. Umbilical vascular catheter (UVC) was removed in case of PVT. No anticoagulation therapy was required. No risk factor was significantly associated with PVT. At 1 year of follow-up, five infants had persistent isolated left PVT (4%).

A spontaneous favorable evolution of left PVT occurred in more than of 95%.

Interventional radiology's role in the management of portal hypertension in the pediatric population differs from the management of adult portal hypertension. In the pediatric population, portal hypertension is frequently secondary to thrombosis and cavernous transformation of the extrahepatic portion of the portal vein. Transjugular intrahepatic portosystemic shunt can be utilized to manage portal hypertension in children with intrinsic liver disease that results in cirrhosis and portal hypertension, and is often used as a bridge to transplant. While technically feasible in extrahepatic portal vein occlusion, the sequelae of portosystemic shunting are less desirable in a child. The Meso-Rex bypass procedure, which represents the mainstay of management for pediatric portal hypertension, provides surgical relief of extrahepatic portal vein obstruction and restores mesenteric venous blood flow to the liver. This article aims to review management of portal hypertension in children as it pertains to the interventional radiologist, including preoperative assessment, postoperative evaluation, and the management of complications of the Meso-Rex bypass.

Abdominal venous thrombosis is a rare form of venous thromboembolic disease in children. While mortality rates are low, a significant proportion of affected children may suffer long-term morbidity. Additionally, given the infrequency of these thrombi, there is lack of stringent research data and evidence-based treatment guidelines. Nonetheless, pediatric hematologists and other subspecialists are likely to encounter these problems in practice. This review is therefore intended to provide a useful guide on the clinical diagnosis and management of children with these rare forms of venous thromboembolic disease. Herein, we will thus appraise the current knowledge regarding major forms of abdominal venous thrombosis in children. The discussion will focus on the epidemiology, presentation, diagnosis, management, and outcomes of (1) inferior vena cava, (2) portal, (3) mesenteric, (4) hepatic, and (5) renal vein thrombosis.

Neonates have the highest risk for pathologic thrombosis among pediatric patients. A combination of genetic and acquired risk factors significantly contributes to this risk, with the most important risk factor being the use of central venous catheters. Proper imaging is critical for confirming the diagnosis. Despite a significant number of these events being life- and limb-threatening, there is limited evidence on what the appropriate management strategy should be. Evaluation and treatment of any neonate with a clinically significant thrombosis should occur at a tertiary referral center that has proper support.

Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication - the upper gastrointestinal bleeding.

To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension.

This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound.

Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding.

The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.

The meso-Rex bypass is a physiological and anatomical bypass procedure for relief of extrahepatic portal vein obstruction and restoration of mesenteric venous return to the liver. Most patients who are candidates for the bypass are children or young adults with portal hypertension and hypersplenism secondary to cavernous transformation of the portal vein. Most frequently, the bypass utilizes an autologous venous graft to connect the intrahepatic left portal vein to the infrapancreatic superior mesenteric vein (SMV) re-establishing first-pass portal perfusion. We describe the preoperative imaging of the 92 bypass candidates, the surgical anatomy as reflected in postoperative imaging, and the imaging of bypass complications at our institution.Preoperative imaging with US, CT and MR is directed to demonstrate patency and size of the left portal vein and SMV, to define the extent of cavernous transformation and splanchnic collaterals, and to assess for any associated abdominal vascular or solid organ abnormalities. Postoperative imaging is aimed at diagnosing meso-Rex bypass stenosis or occlusion and the interventional management of these complications.

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.

The study aims to determine childhood outcomes of neonates diagnosed with portal vein thrombosis (PVT).

A retrospective chart review of neonates diagnosed with PVT who had documented follow up (physical examination, laboratory tests or ultrasonographic examination) at the age of 2 years or beyond was conducted.

Data were available for 70 children (71% of eligible neonates) at a median age of 5 years (range 2-8 years). Physical examination was unremarkable in all who were examined (n= 68). Twenty-five children had liver functions assessed and only mild abnormalities were detected in nine children. Six of 25 patients tested for thrombophilia showed abnormalities. Thirty-seven children had ultrasonographic follow up. Left lobar atrophy (LLA) was noted in 20 children (16 had isolated LLA, two had LLA and splenomegaly, and two had LLA and portal hypertension). Five children who had normal ultrasound examinations at discharge had splenomegaly (two with additional finding of LLA) at follow up. Two children with portal hypertension diagnosed during neonatal period needed porta-caval shunting.

Our results show that among patients, who had PVT as neonates, 28% still had asymptomatic LLA in childhood, 7% had slowly progressive splenomegaly and 3% required shunting because of progression of portal hypertension. Ultrasonographic follow up was the most sensitive method in detecting progression associated with neonatal PVT. Until more data are available, periodic long-term ultrasonographic follow up should be considered for neonates diagnosed with PVT.

To determine catheter-associated thrombosis by color Doppler ultrasound and to detect duration of catheter placement as a risk factor for thrombosis.

All newborn infants with umbilical vascular catheterization for more than 6 h duration were included in this study. Color Doppler ultrasound examination was performed within 24-48 h of catheter insertion, 48-72 h after its withdrawal and weekly until hospital discharge or clot resolution.

Portal vein thrombosis (PVT) was determined in five cases (3.04%) of 164 infants received umbilical vascular catheterization. The mean duration of catheter placement in patients with PVT was 3.4 ± 1.94 days, which was not significantly different from infants without thrombosis (3.5 ± 2.03). Thrombosis was completely recanalized and resolved after 3-6 weeks in three survived neonates. There was history of exchange transfusion for hyperbilirubinemia via umbilical vein in two neonates with PVT.

Catheter-associated portal venous thrombosis was uncommon in our study. The duration of catheter placement was not longer in patients with portal vein thrombosis than those without thrombosis.

Cardiovascular and renal malformations are well-known in Turner syndrome. However, gastrointestinal bleeding is less frequent. The possible etiologies of gastrointestinal bleeding in Turner syndrome are intestinal teleangiectasia, inflammatory bowel disease and portal hypertension. The authors report a 3-year-old girl with Turner syndrome who presented with severe gastrointestinal bleeding requiring transfusion. The radiological examination indicated prehepatic portal hypertension as a reason for haematochezia. The liver biopsy demonstrated the anomaly of intrahepatic arteries and veins. In this report we describe a case of congenital portal vein obstruction and we reviewed liver abnormalities associated with Turner syndrome and causes of obstruction of vena portae in childhood.

The use of umbilical catheters in the care of critically ill neonates has become standard practice. Both arterial and venous umbilical catheters are a vital component of care, providing a stable route for fluid and medication administration, vascular pressure monitoring, and frequent blood sampling. Although commonplace, umbilical catheters are not without complications. Neonatal caregivers must be aware of and monitor carefully for associated complications. This article, Part 1 of a 2-part series, provides a systematic guide to the physical assessment of infants with umbilical venous catheters with an emphasis on early and ongoing recognition of complications related to this device. Part 2 will focus on umbilical arterial catheters, their use, and assessment of the infant to detect device-specific complications.

To determine the risk factors, clinical features, and outcome of infants diagnosed with portal vein thrombosis (PVT).

A retrospective chart review was conducted of all consecutive infants admitted to the Hospital for Sick Children, Toronto, between January 1999 and December 2003 diagnosed with PVT.

PVT was diagnosed in 133 infants, all but 5 of whom were neonates, with a median age at time of diagnosis of 7 days. An umbilical venous catheter (UVC) was inserted in 73% of the infants and was in an appropriate position in 46% of them. Poor outcome, defined as portal hypertension or lobar atrophy, was diagnosed in 27% of the infants and was significantly more common in those with an initial diagnosis of grade 3 PVT and in those with a low or intrahepatically placed UVC. Anticoagulation treatment did not appear to have a significant effect on outcome.

PVT occurs early in life; major risk factors in addition to the neonatal period are placement of UVC and severe neonatal sickness. Poor outcome is associated with an improperly placed UVC and with grade 3 thrombus.

Extra-hepatic portal hypertension (EHPH) defined as non cirrhotic, presinusoidal and prehepatic portal hypertension, with obstruction and cavernomatous transformation of the main portal vein, entails a high, early and prolonged risk of gastro-intestinal bleeding (GIB) mainly from esophageal and/or gastric varices, and less often a risk of cholangiopathy or protein-losing enteropathy. Diagnosis of EHPH may be done with non invasive imaging techniques. Assessment of bleeding risk is based on results of endoscopic examination. Occurence of a bleeding episode or onset during follow-up of endoscopic signs of high risk of GIB require radical eradication of varices. Radical cure of EHPH is achieved at best by bypass surgery restoring a physiological portal flow, and as a second choice by shunt surgery. Endoscopic therapy has a place as first line treatment of GIB episodes, and also in a few cases with poor extrahepatic portal network contra-indicating efficient vascular surgery.

To establish, by means of serial ultrasonography (US), the incidence and natural history of neonatal portal venous thrombosis associated with catheterization of the umbilical vein and to evaluate the potential risk factors predisposing patients to thrombus formation.

Neonates who had undergone umbilical vein catheterization were studied. US was performed at 2-7-day intervals, before and after the removal of the catheter, until clot resolution or hospital discharge. The presence of portal venous thrombosis and temporal evolution were noted, and various risk factors were analyzed.

US demonstrated clinically silent portal venous thrombosis in 43 (43%) of 100 neonates. Follow-up US revealed complete or partial resolution in 20 (56%) of 36 babies. A significant (P =.024) correlation was found between the initial size of the thrombi and spontaneous clot resolution. Statistically significant risk factors were catheterization for more than 6 days (P =.001) and transfusion (P =.019).

Portal venous thrombosis is frequently associated with the placement of an umbilical venous catheter, and spontaneous resolution is expected in many cases. The duration of catheter placement should be minimized, and US monitoring is recommended as a guide to catheter removal.

The factor V (FV) G1691A mutation, the prothrombin (PT) G20210A variant, the methylenetetrahydrofolate reductase (MTHFR) T677T genotype, together with fasting homocysteine (HCY) concentration, lipoprotein (Lp)(a), anti-thrombin (AT), protein C (PC), protein S (PS) and anti-cardiolipin antibodies were investigated in 65 consecutively recruited infants (neonate to < 12 months) with renal venous thrombosis (RVT; n = 31), portal vein thrombosis (PVT; n = 24) or hepatic vein thrombosis (HVT n = 10), and 100 age- and sex-matched healthy controls. FV G1691A was found in 14 babies (heterozygous: RVT n = 9, PVT n = 4; homozygous HVT n = 1) and five controls, the MTHFR TT677 genotype together with increased HCY in four infants with thrombosis (RVT n = 2; PVT n = 1; HVT n = 1) compared with one control, and the PT G20210A variant was present in one control only. PC type I deficiency was diagnosed in three patients (RVT n = 2; PVT n = 1) and AT deficiency in two patients (RVT n = 1; PVT n = 1). Three neonates with spontaneous thrombosis showed FV G1691A combined with Lp(a) and the FV G1691A was combined with the PT G20210A genotype in two infants. Additional triggering factors were reported in 27 patients (41.5%). The overall odds ratios (ORs) and 95% confidence intervals (CIs) with respect to the different thrombosis locations were: RVT (OR/CI: 10.9/3.85-31.1; P < 0.0001), PVT (5.47/1.7-17.6; P < 0.0007) and HVT (3.3/0.58-18.7; P = 0.18). The data presented here suggest that genetic prothrombotic risk factors also play an important role in abdominal venous thrombosis during infancy.