Interventional radiology procedures in pediatric patients with extra-hepatic portal vein obstruction and complications of portal hypertension

This poster is published under an open license. Please read the disclaimer for further details.

Congress:

ECR 2014

Poster Number:

C-1879

Type:

Scientific Exhibit

Keywords:

Paediatric, Liver, Interventional vascular, MR, CT, Catheter venography, Diagnostic procedure, Recanalisation, Balloon occlusion, Education and training, Haemodynamics / Flow dynamics, Haemorrhage

Authors:

R. Miraglia¹, L. Maruzzelli², K. Cortis², S. Caruso², A. Luca²; ¹Palermo /IT, ²Palermo/IT

DOI:

10.1594/ecr2014/C-1879

DOI-Link:

https://dx.doi.org/10.1594/ecr2014/C-1879

Fig. 2: Main portal vein cavernoma, very thin intra-hepatic portal vessels,...

Fig. 3: Retrograde portography, anteroposterior and oblique projections (a and b...

Fig. 5: Selected axial (a) and coronally-reformatted (b) MDCT images in a 10 year old...

Fig. 8: Selective catheterization of the shunt from the vena cava side with mechanical...

Fig. 12: 12 year old child with EHPVO secondary to neonatal umbilical vein...

Fig. 13: 12 year old child with EHPVO secondary to neonatal umbilical vein...

Fig. 15: 6 year old child with EHPVO secondary to neonatal umbilical vein...

Fig. 18: 5 year old child with a partial liver transplant (segment II/III, two separate...

$Fig. 20: Emergency TIPS performed due to massive refractory acute bleeding from a varix sited at the posterior duodenal wall. The Colapinto needle was advanced from the right hepatic vein a portal vein radicle (a). Injection of contrast shows tracking along the portal vein remnant, with stasis at the caudal aspect due to absence of communication between the remnant and the spleno-mesenteric confluence (b). A hydrophilic wire was successfully introduced through the remnant, and into the spleno-mesenteric confluence following multiple attempts (c). This was substituted by a stiff wire, over which two TIPS stents were deployed in series (d).$

Fig. 20: Emergency TIPS performed due to massive refractory acute bleeding from a varix...

Aims and objectives

To define the role of interventional radiology in the management of pediatric patients with extra-hepatic portal vein obstruction complicated by portal hypertension.

Methods and materials

Background Extra-hepatic portal vein obstruction (EHPVO) can be defined as obliteration of the extra-hepatic portal vein with or without extension to the intra-hepatic portal veins. EHPVO is an important cause of portal hypertension in developing countries, where it is the most common cause of upper gastrointestinal bleeding in children (up to 70% in some series). In EHPVO the normal hepatopetal flow from the superior mesenteric vein and splenic vein is impeded by obstruction of the main portal vein. This results in cavernomatous transformation of the...

Results

Case 1 3 year old child with portal vein cavernoma and EHPVO most likely due to umbilical vein cannulation after birth. This patient had previous episodes of gastro-intestinal bleeding and five sessions of endoscopic scleroterapy for esophageal varices. MDCT shows main portal vein cavernoma (Figure 2a), very thin intra-hepatic portal vessels (Figure 2b-c). Extensive collateralization and splenomegaly are also noted. Retrograde portography through the left hepatic vein (Figure 3) was performed, since the Rex segment could not be identified with confidence on non-invasive imaging. Anteroposterior...

Conclusion

In pediatric patients with EHPVO, axial and reformatted MDCT allows delineation of the portal venous anatomy in most cases, allowing for proper planning prior to surgical shunt creation. Retrograde portography is a safe and effective technique to show the intrahepatic portal venous branches and identify the Rex segment in children with EHPVO and indeterminate findings on non-invasive imaging (US, MDCT or MR). The role of interventional radiology depends on the clinical presentation. Chemical and mechanical thrombectomy in patients with thrombosed porto-systemic surgical shunts might prevent...

Personal information

Theauthorsdeclare that theyhave no conflicts of interest or financial disclosures.

References

Bernard O, Alvarez F, Brunelle F et al. (1985) Portal hypertension in children. Clin Gastroenterol; 14:33-5. Lykavieris P, Gauthier F, Hadchouel P et al. (2000) Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction. J Pediatr; 136:805-8. Sarin SK, Sollano JD, Chawla YK et al. (2006) Consensus on extra-hepatic portal vein obstruction. Liver International: 26: 512–519. Chiu B, Superina R. (2004) Extrahepatic portal vein thrombosis is associated with an increased incidence of cholelithiasis. J Pediatr Surg. 39:1059-1061. Fuchs J,...