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Portal vein thrombosis in children and young adults?

Portal vein thrombosis in children and young adults?

Portal vein thrombosis in children and young adults?

Patient’s information

The portal vein is a blood vessel that carries blood from the gastrointestinal tract, gallbladder, pancreas, and spleen to the liver. This blood contains nutrients and toxins extracted from digested contents. Approximately 75% of total liver blood flow is through the portal vein, with the remainder coming from the hepatic artery. The blood leaves the liver to the heart in the hepatic veins.

gastrointestinal tract

Portal vein thrombosis

 

Portal vein thrombosis (PVT) is the blockage of the portal vein with blood clots. In fact some believe that there is 1% lifetime risk of PVT in general population. The diagnosis can now be readily established by using ultrasound scan or CT or scans.

There are various causes of PVT, but whatever the cause, the sequence of events are very serious. Patients may present with severe bleeding from esophageal or gastric varices. The two main types of PVT are acute and chronic.

Portal vein thrombosis

PVT is not very uncommon in Pakistan. One of the common causes is the belly button infection in infancy. The infection leads to clotting in the portal vein. Most of the time this goes unnoticed and the diagnosis is only made when child presents with gastrointestinal bleeding. Other causes of portal vein thrombosis are;

 

Causes of congenital portal vein thrombosis

Deficiency or access of certain chemical in the body can increase the risk of blood clotting in the veins. Some of these abnormalities are present form birth.

Inherited prothrombotic disorders

·         ☐ Factor V Leiden mutation

·         ☐ Factor II gene mutation

·         ☐ Protein C deficiency

·         ☐ Protein S deficiency

·         ☐ Antithrombin III deficiency

Acquired thrombophilic disorders

·         ☐ Primary myeloproliferative disorders

·         ☐ Paroxysmal nocturnal hemoglobinemia

·         ☐ Antiphospholipid syndrome

·         ☐ Hyperhomocysteinemia

·         ☐ Increased factor VIII levels

·         ☐ Thrombin activatable fibrinolysis inhibitor gene

Portal vein thrombosis in children and young adults

Symptoms of portal vein thrombosis

Portal hypertension and its consequence of bleeding varices are usually seen in people with portal vein thrombosis.

As the blood tries to find another way back to the heart, new blood vessels open up. Among these vessels are those that run along the wall under the lining of the upper part of the stomach and the lower end of the oesophagus (food pipe). These veins protrude into the gullet and the stomach and can bleed. The dilated veins in the gullet are known as varices. Bleeding may be a gentle ooze in which case anaemia is the commonest symptom. Sometimes there can be a major bleed; a child may have a haemorrhage and either vomits blood or passes blood through their bowels. This blood may appear to be black, since it is often changed as it passes through the body.

Oesophageal varices represent a significant complication of portal vein thrombosis.

Detection of varices

Unless they bleed, varices do not produce any complications or symptoms. The only way they can be detected is by a process called endoscopy. During endoscopy a small flexible tube is put into the gullet and the endoscopist can see not only where the varices are present but also their size.

Prevention of bleeding

In general, small varices rarely bleed and bigger ones may bleed. Small varices however, may well develop into large varices over time. For those children who have varices and are likely to bleed, treatment with drugs can sometimes reduce the risk of bleeding and reduce the severity of any bleed should it occur. The drug most commonly used is propranolol. As with all drugs, not everyone is suitable and some have side-effects. Alternative methods may sometimes be used for those who are at risk of bleeding.

Treatment

Current treatments do allow for early identification of those people who are at risk of variceal bleeding, and treatments can greatly reduce the risk and severity of the bleeds. For those who have bleeding oesophageal varices, this is a medical emergency but early treatment is usually highly effective. There are a variety of approaches to treat bleeding varices and the treatment used will depend on the overall condition of the patient.

Propranolol is used both for the prevention of bleeding and also in those people who have bled. It may be used in the prevention of re-bleeding. A number of treatment options are available for the treatment and prevention of bleeding.

Drugs

Several drugs are useful in the treatment of the variceal bleed. These drugs, such as glypressin or octreotide, are given by injection.

Endoscopic techniques

  • Banding– with banding techniques a single vein (the varix) is sucked into a ring at the end of the endoscope. A small band is placed around the base of the varix. After one or two days this will result in the formation of a clot which will control the bleeding.
  • Injection scleropathy – this is a sclerosant (special chemical) material injected into the veins of the gullet. It is done after you have been given some sedation and a camera (endoscope) has been passed into your gullet. A fine flexible needle is passed through the endoscope and used to inject the sclerosant material into the oesophageal veins or the adjacent veins. These injections cause clotting and will also stimulate some scarring to reduce the recurrence of varices.

Treatment of portal vein thrombosis (PVT)

There are various forms of treatment modalities for PVT, these include medical and surgical.

Medical treatment

The mainstay of treatment is to minimize portal hypertension and to avoid progression of thrombosis (clot in the portal vein) by;

1-Anticoagulation (blood thinning medications). Spontaneous recovery with re-canalization of the portal vein can be achieved in near half of the patients with anticoagulation. 

2-Endoscopic intervention (banding or sclerotherapy) for bleeding varices

3-Drug therapy (propranolol) to reduce portal hypertension

Surgical shunts

The patients that do not respond to the medical therapy may require surgical intervention if they suffer from persistent complications of portal hypertension. Variceal bleed is common and disastrous complication

Surgical options for PVT are portosystemic shunts. The choice of procedure depends upon patients overall condition and liver status. Liver transplant is only considered for patients who has severe scarring (cirrhosis) of the liver. Those patient whose liver function is normal; they are good candidate for surgical shunts.

The shunting procedures available are;

  • Splenorenal shunt
  • Mesocaval shunt
  • Portocaval shunt

It is believed that the shunt operation are very effective in controlling the risk of bleeding in long term.

Outlook 

Portal vein thrombosis is not a rare disease but it carries serious complication if left untreated. The surgical options for PVT are simple and carry a good prognosis when performed at right time. All the procedures carry certain risks but the procedure of choice is dependent on the patient‘s condition. Variceal bleeding is a serious complication that requires urgent management and monitoring. These patients should be looked after only by experienced doctors at specialist centers.

If you have any further questions or worries please feel free to ask the staff on your ward, your surgical team or your clinical nurse specialist.

 

Patient Help Line (PHL) is a service that offers support, information and assistance to patients, relatives and visitors. They can also provide help and advice if you have a concerns.

The PHL office is located on the ground 6th floor of the “Quaid e Azam International Hospital”, Islamabad our staff will be happy to help and assist you.

Transplant coordinator:

Mr. Touseef Ahmed 0333-1027353

Transplant Coordinator: (for post-transplant follow up patients):

M. Zaheen Hashmi 0333-1027354

Hepatobiliary & Pancreatic Surgery Services:

Muhammad Asif 0341-0543883

24 Hours Patients emergency Helpline: 0333-1027356

Email: info@faisaldar.com

Counselling service: The counselling service offers free and confidential sessions to our inpatients, outpatients, their relatives and friends. If you would like to meet with one our counsellors you can contact us on 0333-1027356

New Life Health Services at Quaid e Azam International Hospital”, Islamabad, Pakistan

Transplant coordinator:

Mr. Touseef Ahmed 0333-1027353

Transplant Coordinator: (for post-transplant follow up patients):

M. Zaheen Hashmi 0333-1027354

Hepatobiliary & Pancreatic Surgery Services:

Muhammad Asif 0341-0543883

24 Hours Patients emergency Helpline: 0333-1027356

Email: info@faisaldar.com

Website: www.faisaldar.com

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