AVM in LIVER, Dr TRAN NGAN CHAU-Dr PHAN THANH HAI

ANEURYSMAL PORTO-HEPATIC ANEURYSMAL PORTO-HEPATIC VENOUS SHUNT: A CASE REPORT VENOUS SHUNT: A CASE REPORT

Tran Ngan Chau, Phan Thanh Hai

Medic Medical Center, HCMC, Vietnam

Case descriptionCase descriptionHistory

A 31-year-old male patient complained about 3-day right subcostal abdominal pain. He did not have any other symptoms such as vomiting, diarrhea. He also had no history of abdominal surgery, trauma, liver biopsy or alcohol abuse.

Physical examination The patient had no fever. He was found no

mass, no peritoneal induction in the right subcostal.

Laboratory results were normal.Abdominal ultrasound and CT angiography

were done.

Fig.1 (A) cystic structure 21x21mm in size, communicated Fig.1 (A) cystic structure 21x21mm in size, communicated with 2 dilated veins with 2 dilated veins

(A)

(B)

Fig.1 (B) portal vein flow in the lesion with yin-yang sign

(C)

Fig.1 (C) hepatic vein flow in the lesion with yin-yang sign

+ B-mode ultrasound (US) findings showed a cystic structure ( 21x21mm) in the sixth segment, it communicated with 2 parallel –dilated - tubular - structure (d = 8 and 9mm) originated from the right portal vein and right hepatic vein. Doppler US showed Yin-yang sign, portal vein flow and hepatic vein flow in the cystic structure ( Fig.1)

Fig. 2: Contrast enhanced CT on venous phase (A) density of Fig. 2: Contrast enhanced CT on venous phase (A) density of the lesion is the same to vein.the lesion is the same to vein.

(A)

(B)

Fig. 2: Contrast enhanced CT on venous phase (B) the lesion with dilated afferent and efferent veins

Fig. 2: Contrast enhanced CT on venous phase (C ) the lesion with dilated afferent and efferent veins on three dimensional reconstruction.

(C)

+ MSCT Angiography showed aneurysmal right portohepatic venous shunt in the sixth segment 21x31mm in size with dilated afferent and efferent veins (Fig 2).

One week later, the patient underwent an abdominal laparoscopic surgery for resection the aneurysm to prevent a rupture. He recovered well after surgery.

The aneurysm in the sixth segment

vein

The sac flatted when the operator lifted it up

Microscopic result : there are normal hepatic cells and some abnormal vascular cells. It is concordant with vascular malformation.

Macroscopic result: sinusoid aspect in the sac

DiscussionDiscussionIntrahepatic portosystemic venous shunt

(IVPS) is rare, and considered to be created as congenital or acquired condition as: portal hypertension, cirrhosis, trauma, liver biopsy. Our patient was regarded to be congenital because he had no history of abdominal surgery, trauma, liver biopsy or alcohol abuse.

In this case, no pulse or arterial signal was found in this abnormal structure so it was thought to be a venous shunt.

Congenital portosystemic venous shunt is due to the conglutination of the umbillico-vitelline venous plexus.

There are 4 types of intrahepatic portosystemic venous shunt according to Park et al.

- Type I: a single large shunt that connects the right portal vein to the inferior vena cava.

- Type II: single or multiple communications of the portal and hepatic veins in one hepatic segment.

- Type III: an aneurysm of the connection of peripheral portal and hepatic veins.

- Type IV: multiple communications between the peripheral portal and hepatic veins are found diffuse in both lobes.

We presented a case of type III of IVPS according to Park’s classification. In our knowledge, 25 cases of IVPS have been reported in the literature up to 2007 and there were only 7 cases of aneurysmal IPVS.

Imaging findings in aneurysmal IVPS:

- Abdominal ultrasound

+ Gray-scale findings shows abnormal cystic structure communicating with two dilated tubular structures.

+ Color Doppler detects a direct connection of flows between the portal vein and hepatic vein. The spectral pattern at each point in this structure shows portal vein or aneurysm or hepatic vein signal.

Complications

- IVPS can lead to hyperammonaemia because of decreasing ammonia elimination that brings the patient to risk of encephalopathy. It also can causes liver failure, cirrhosis, pulmonary arterial hypertension.

- Aneurysm IVPS can be ruptured.

Conclusion Conclusion We reported a venous malformation

case that belongs to Park type III of IVPS. IVPS is rare and considered to be created as congenital or acquired condition. IVPS patients can be with or without symptoms.

Color Doppler is the primary imaging modality to find out IVPS and confirmed by CT angiography. The patient underwent via laparoscopy for removing the aneurysmal sac and recovered well.

Clinical implicationClinical implication

Ultrasound plays an important role in diagnosis, B-mode detects deformities. Color doppler contributes to identify vascular malformation.

ReferencesReferences1. P Naidoo, MB BCh, FCRad Diag (SA), N Maharaj, MB ChB, FCRad Diag (SA);V Naidu, MB

ChB, FCRad Diag (SA); J Maharajh, MB ChB, FFRad (D) SA, MMed Rad (D), an unusual case of intrahepatic portosystemic venous shunt, S Afr J Rad 2013;17(2):57-58. DOI:10.7196/SAJR.761

2. Carmen Gallego, MD; Carlos Marin, MD ; Enrique Garcia-Hidalgo, MD ;Maria Miralles, MD; Purificacion Muyor, MD; Gabino Gonzalez, MD, Congenital Hepatic Shunts, EDUCATION EXHIBIT - Continuing Medical Education

3. Akshay Kumar Saxena1, Kushaljit Singh Sodhi1, Julie Arora1, Babu Ram Thapa2 and Sudha Suri1

Congenital Intrahepatic Portosystemic Venous Shunt in an Infant with Down Syndrome , Pediatric Imaging, Case Report, December 2004, Volume 183, Number 6.

4. H Mori,  M Nagasaki, M Mutsukura, N Matsunaga, K Hayashi, T Fukuda, S Futagawa, Intrahepatic portosystemic venous shunt: occurrence in patients with and without liver cirrhosis, October 1987, Volume 149, Number 4.