CASE 17443 Published on 27.10.2021

Intrahepatic portosystemic shunt: A rare cause of pediatric jaundice

Section

Paediatric radiology

Case Type

Clinical Cases

Authors

Dr Neeti Gupta, Dr Antariksh Vijan

Department of Radiology, Bai Jerbai Wadia Hospital for Children, Parel, Mumbai, India

Patient

7 years, female

Categories
Area of Interest Abdomen, Interventional vascular ; Imaging Technique CT
Clinical History

A 7-year-old girl presented with complaints of jaundice. She underwent an ultrasound of the abdomen as a part of the evaluation for jaundice and was detected with a large intra-hepatic porto-systemic shunt connecting the main portal vein and IVC. The liver, however, showed normal morphology on imaging. She also had a history of developmental dysplasia of left hip and congenital unilateral radial head dislocation.

Imaging Findings

CT abdomen was performed, which demonstrated a large intrahepatic shunt vessel connecting the main portal vein and the IVC (Figure 1).  The liver was non-cirrhotic, and no other portosystemic collaterals were noted. These features were consistent with a congenital intrahepatic shunt owing to the lack of evidence for any cause of portal hypertension or history of trauma, surgery, or biopsy.

Invasive fluoroscopy-guided porto-venography confirmed the findings on CT, following which transcatheter closure device was deployed (Figure 2).

Successful placement of the occluder device is demonstrated by the post-procedure contrast CT study. (Figure 3).

Discussion

Background

Congenital portosystemic shunts are rare malformations comprising of anomalous communication(s) between the portal system and the caval venous system. The shunting may occur within the substance of the liver – an intrahepatic portosystemic shunt (IHPSS); or outside the liver parenchyma, i.e., extrahepatic portosystemic shunt (also referred to as an Abernethy malformation) [1]

Etio-pathophysiology

Aberrant persistence of communicating channels in the porto-hepatic venous systems during embryonal development results in this malformation.

Clinical Features

IHPSS are incidentally detected most times. When symptomatic, they present with jaundice, hyperammonemia and features of hepatic encephalopathy. Type IV shunts may lead to heart failure (due to massive multisegmental shunting).

In the neonate, these may undergo auto-resolution in the first year of life. Additional anomalies such as a cutaneous hemangioma and cardiac malformations may be associated with any of the types[1,2]. They may occasionally be associated with other congenital anomalies, including malrotation, congenital heart diseases, polysplenia, annular pancreas and musculoskeletal anomalies among others.

Imaging Perspective  

  • Ultrasound and Doppler: [3,4] 
  • Direct communication showing colour flow between portal and caval branches.
  • Portal venous system may demonstrate a biphasic or triphasic spectral waveform reflecting the shunting.
  • Non-uniform size of the portal vein and hepatic vein sizes may aid in locating additional communications.
  • CT :  
  • Abdominal CT in the portovenous phase readily demonstrates any comunications, with precise anatomic characterisation.
  • Also useful in depiction of parenchymal changes such as regenerative nodules.
  • MRI: Flow void on T2WI at the shunt site. May be helpful in demonstrating multiple communications. 
  • Best modality for accurate analysis is transvenous or transarterial Porto-Venography.

Classification of Intra-Hepatic Porto-systemic Shunts (Park et al)

Type I – Single large vein connecting the right portal vein and the IVC.

Type II – Peripheral shunting, localised to a single hepatic segment.

Type III – An aneurysm connecting peripheral branches of the portal vein and hepatic vein.

Type IV – Multiple communications in several segments.

An additional type has been added to the original Park classification: Type V – Persistent ductus venosus. [1,2]

Outcome

Endovascular repair with transcatheter embolization remains the treatment of choice in large symptomatic shunts such as ours, with use of an appropriate occluder device such as the Amplatz device.  A short interval follow-up of the patient at 2 months showed improvement of the jaundice.  

A wait-and-watch approach with serial doppler ultrasound follow up studies and dietary restrictions may be adopted in patients that are completely asymptomatic with only mild metabolic derangements. [5]

Our patient was treated with endovascular transcatheter placement of an occluder device across the posto-systemic shunt, following which there was resolution of jaundice.

Take-Home Message / Teaching Points

Congenital portosystemic shunts can be intrahepatic or extrahepatic. They are commonly associated with other congenital anomalies, including malrotation, congenital heart diseases, polysplenia, annular pancreas, skeletal anomalies among others. Hence, a thorough workup must be performed in such cases to rule out other anomalies. These patients usually do not have other signs of portal hypertension, which helps distinguish them from secondary shunts.

Differential Diagnosis List
Intra Hepatic Congenital Porto-systemic Shunt, Park type I
Abernethy malformation: porto-systemic shunt vessels lying entirely = outside the liver
Acquired porto-systemic shunts: in the presence of underlying cirrhosis. Occasionally may be seen secondary to trauma or other hepatobiliary interventions (for e.g. biopsy)
Higher porto-systemic shunting and encephalopathy; almost always require transcatheter closure
Final Diagnosis
Intra Hepatic Congenital Porto-systemic Shunt, Park type I
Case information
URL: https://www.eurorad.org/case/17443
DOI: 10.35100/eurorad/case.17443
ISSN: 1563-4086
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