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Table of Contents    
CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 4  |  Page : 930-933

Neurological Symptoms of Congenital Portosystemic Shunt Reversed by Venous Endovascular Intervention: A Six Years Follow-Up Study


Hospital Nacional “Profesor Doctor Alejandro Posadas”, El Palomar, Buenos Aires, Argentina

Date of Web Publication26-Aug-2020

Correspondence Address:
Dr. José Antonio Díaz
Hospital Nacional Profesor Doctor Alejandro Posadas, El Palomar, Buenos Aires
Argentina
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.293475

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 » Abstract 


Congenital portosystemic shunt (CPSS) is a rare disorder characterized by a diversion of Porto-mesenteric blood into systemic veins. Type I is an end to side fistula between the portal vein and the inferior vena cava. Type II is a side to side fistula between the main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. Clinical presentation of these patients varies. Treatment and management are guided by the type of malformation and clinical presentation. Herein, we present a case of CPSS Type IIb with neurological symptoms, treated with endovascular occlusion with 6-year follow-up without remission.


Keywords: Hypermangnesemia, portocaval shunt, portosystemic shunt, secondary parkinsonism
Key Messages: The CPSS is a rare congenital condition that can go undiagnosed until adulthood. Treatment requires a medical center with areas of complex specialty care. An interdisciplinary group of experts is necessary not only for the diagnosis but also for the treatment. Neurological symptoms could be related to a CPSS.


How to cite this article:
Riolo FM, Alvarez DP, Villegas MO, Díaz JA. Neurological Symptoms of Congenital Portosystemic Shunt Reversed by Venous Endovascular Intervention: A Six Years Follow-Up Study. Neurol India 2020;68:930-3

How to cite this URL:
Riolo FM, Alvarez DP, Villegas MO, Díaz JA. Neurological Symptoms of Congenital Portosystemic Shunt Reversed by Venous Endovascular Intervention: A Six Years Follow-Up Study. Neurol India [serial online] 2020 [cited 2020 Sep 29];68:930-3. Available from: http://www.neurologyindia.com/text.asp?2020/68/4/930/293475




Congenital portosystemic shunt (CPSS) is a rare disorder (1:25,000/1:30,000 at birth) described by John Abernethy 1793.[1] Type I CPSS is characterized by absent intrahepatic portal circulation where the portal blood is diverted completely into the systemic circulation, predominantly observed in females, and it is associated with other congenital malformations such as polysplenia, intestinal malrotation and cardiopathies. Type I is further classified into Type Ia, where the splenic vein (SV) and superior mesenteric vein (SMV) drain separately, and Type Ib where both veins drain together after joining in a common trunk.[2] Type II affects both sexes and is usually not associated with malformations. Type II is classified based on the anatomic location of the shunt as IIa, IIb, and IIc [Figure 1] by Lautz et al.[3] Hepatic encephalopathy is commonly observed in both types. Liver transplant is the current treatment for the symptomatic Type I and shunt interruption (ligature or endovascular occlusion) is the treatment for Type II. Herein, we present a case of CPSS Type IIb that presented important neurological symptoms that disappeared after endovascular occlusion.
Figure 1: Portosystemic shunt Classification. (A) Normal. (B) Type I: Morgan and Superina Classification: Intrahepatic Portal Vein absent. Liver not perfused with portal blood-total shunt. Ia: SMV and splenic vein do not joint to form confluence. Ib: SMV and splenic vein join to form confluence trunk. (C) Type II: (Lautz et al.). Liver perfused with portal blood-partial shunt: IIa: Arising from left or right portal vein to the IVC. IIb: Arising from the main portal vein (including its bifurcation or splenomesenteric confluence) to the IVC. IIc: Arising from the IMV, gastric, or splenic veins into the IVC

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 » Case Top


A 52-year-old male patient presented with hypertension and dyslipidemia with neurological symptoms for the past 4 years. The neurological symptoms were disorientation, lethargy, slurred speech, upper extremities tremor, comprehension aphasia, dizziness, dysmetria, and amnesia. Presumptive diagnosis was encephalitis. The laboratory profile was normal including liver panel and cerebrospinal fluid. A brain CT scan showed hypodensity at the level of the dentate nuclei of the cerebellum and left occipital lobe at the subcortical area. MRI of the brain showed altered white matter in both cerebellum hemispheres, observed as hypodense in T1 and hyperintense in T2. Interestingly, the MRI also showed increased intensity in T1 located at the lentiform nucleus, suggesting a metabolic process. The electroencephalogram results suggested diffuse encephalopathy. The abdominal ultrasound showed heterogenous liver, with a small left lobe. Regardless, the liver panel was normal, thus the differential diagnosis was metabolic encephalopathy secondary to liver dysfunction or CPSS. However, the liver biopsy was normal. New laboratory analysis revealed: Manganese (Mn + 2) in blood 0.85 microg/dl (ref: Up to 0.05) in urine 2.6 microg/dl (ref: Up to 3); Copper in plasma, 71.7 microg/dl (ref: 100-200 microg/dl) and in urine 20 microg/24 hs (ref: 20-50 microg/24 hs); Ammonia in plasma 25 μg/dl (ref: 15-45 μg/dl). The final diagnosis was hepatic encephalopathy due to increased Mn + 2 levels in the blood and Mn + 2 deposit at the basal ganglia level secondary to CPSS.


 » Endovascular Procedure Diagnosis and Treatment Top


After informed consent was obtained, we proceeded to safely access the left and right femoral vein using the Seldinger technique with a 6F sheath. Using the left access, we positioned a 6F catheter Cobra (Merit Medical, South Jordan UT USA) in the inferior vena cava (IVC) at the level of the right supra-hepatic vein and measured a pressure of 5 mmHg [Figure 2]. Then we position a pigtail catheter (Merit Medical, South Jordan UT USA) in the left renal vein using the right femoral access [Figure 2]. We visualized the left real vein, the shunt and the portal system using 20 ml of non-ionic contrast injected with a pump infusion. We advanced the pigtail up to the Portal vein and measured a pressure of 10 mmHg with a 5-mmHg gradient pressure at the Portal vein level (ref: 2-6 mmHg). We also performed selective venography of the Splenic vein. Thus, and extrahepatic shunt was diagnosed. Using the right femoral access, we interchanged the pigtail for a 6 F peripheral guiding sheath Destination® (Terumo, Shibuya, Tokyo, Japan) and advanced the AMPLATZER®, 10 × 7 mm, Vascular Plug II (St. Jude Medical, Saint Paul, Minnesota, USA). Successful occlusion was obtained as shown in [Figure 3]. A blood test post-procedure found manganese in blood 0.07 microg/dl (ref: Up to 0.05), and in urine 0.0 microg/dl (ref: Up to 3). Following the procedure, the patient recovered from the neurological symptoms, including total absence of tremor and amnesia. Six years after the procedure, the patient still remained without neurological symptoms.
Figure 2: Diagnosis. (a) Selective angiography showing a large congenital shunt between the left renal vein and the Porta. (b) Digital subtraction angiography showing small development of the left Portal vein compared to the right Portal vein. (c) Catheter located on the Splenic vein that allowed us to perform pressure measurements

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Figure 3: Treatment. Post-procedure angiography with AMPLATZER® Vascular Plug II, using Delivery System AMPLATZER Torq Vue® 45°

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 » Discussion Top


The CPSS are uncommon vascular anomalies that occur secondary to abnormal development or involution of fetal vasculature, between the 4th and the 8th weeks of embryonic development.[1] These abnormalities, uncommonly diagnosed in adulthood, allow for portal circulation to reach the systemic circulation, bypassing the liver and resulting in a variety of symptoms.[1],[2],[3],[4] A number of CPSS are diagnosed when patients consult about their complications such as cardiopathies or hepatopathies.[4] Sometimes, the patient presents with neurological symptoms such as in our case, with MRI signs of increased intensity in T1 located at the lentiform nucleus, suggesting a metabolic process.[4] Increased circulating levels of Manganese (Mn+2) in blood is an electrolyte disorder that is responsible for neurological symptoms such as weakness, nausea and vomiting, impaired breathing, low blood pressure, low blood calcium, abnormal heart rhythms and asystole, and neurological symptoms as well. Our patient, who did not present with symptoms during childhood, had symptoms with strong neurological components: Disorientation, lethargy, slurred speech, upper extremities tremor, comprehension aphasia, dizziness, dysmetria, and amnesia. The diagnosis included the symptoms, blood tests and images.[5] Since Rothuizen et al., a group that studied CPSS in dogs and cats, which involved neurological symptoms, there have been several animal studies that revealed the currently observed associated symptoms in humans with CPSS [Table 1].[6] It is mandatory to perform imaging and histology to diagnose CPSS. The ultrasound and MRI are critical to understanding the clinical presentation and formulate a presumptive diagnosis of CPSS. Studying the liver with biopsy, we can observe reduced vasculature with parenchymal atrophy. Type I CPSS is characterized by the absence of portal branches and the reduction of the size observed by CT scan.[7] Initially, medical treatment should be initiated to control the symptoms, except if the patient presents neurological symptoms in which a more aggressive approach should be considered.[8] Type II requires surgical or endovascular treatment.[9] In our case, the left liver lobe was reduced in size associated with the small left portal vein and was treated with an endovascular approach using an occlusion device. The symptoms improved dramatically and in a very short period of time.
Table 1: Table showing publications (including cases with neurological symptoms)

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 » Conclusions Top


The CPSS is a rare congenital condition that can go undiagnosed until adulthood. Treatment requires a medical center with areas of complex specialty care. An interdisciplinary group of experts is necessary not only for the diagnosis but also for the treatment. We present a case of CPSS with neurological symptoms treated with endovascular occlusion in 2012, with rapid relief of symptoms and without remission.

Significance of this work

The CPSS is a rare congenital condition that can go undiagnosed until adulthood. An interdisciplinary group of experts is necessary not only for the diagnosis but also for the treatment. The treatment requires a medical center with areas of complex specialty care.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Paganelli M, Lipsich JE, Sciveres M, Alvarez F. Predisposing factors for spontaneous closure of congenital portosystemic shunts. J Pediatr 2015;167:931-935.e12.  Back to cited text no. 1
    
2.
Morgan G, Superina R. Congenital absence of the portal vein: Two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994;29:1239-41.  Back to cited text no. 2
    
3.
Lautz TB, Tantemsapya N, Rowell E, Superina RA. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 2011;46:308-14.  Back to cited text no. 3
    
4.
Córdoba J. New assessment of hepatic encephalopathy. J Hepatol 2011;54:1030-40.  Back to cited text no. 4
    
5.
Aschner M, Guilarte TR, Schneider JS, Zheng W. Manganese: Recent advances in understanding its transport and neurotoxicity. Toxicol Appl Pharmacol 2007;221:131-47.  Back to cited text no. 5
    
6.
Rothuizen J, Van Den Ingh TSGAM, Voorhoutm G, Van Der Luer RJT, Wouda W. Congenital porto-systemic shunts in sixteen dogs and three cats. J Small Anim Pract 1982;23:67-81. doi:10.1111/j.1748-5827.1982.tb01638.x.   Back to cited text no. 6
    
7.
Shinkai M, Ohhama Y, Nishi T, Yamamoto H, Fujita S, Take H, et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001;36:1026-31.  Back to cited text no. 7
    
8.
Avila LF, Luis AL, Encinas JL, Hernández F, Olivares P, Fernández Cuadrado J, et al. [Congenital portosystemic shunt. The abernethy malformation]. Cir Pediatr 2006;19:204-9.  Back to cited text no. 8
    
9.
Kitagawa S, Gleason WA, Northrup H, Middlebrook MR, Ueberschar E. Symptomatic hyperammonemia caused by a congenital portosystemic shunt. J Pediatr 1992;121:917-9.  Back to cited text no. 9
    


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