Portal Hypertension

Question 1

What is the definition of portal hypertension (PHT) in pediatric patients?

Answer 1

There is no formal definition for pediatric PHT, but a porto-mesenteric pressure above 10 mmHg is arbitrarily used as the cut-off.

Question 2

What are the three main causes of portal hypertension?

Answer 2

The three main causes are:
1. Portal vein obstruction (PVO) - usually synonymous with thrombosis.
2. Liver cirrhosis.
3. Liver fibrosis.

Question 3

How can the causes of portal hypertension be categorized?

Answer 3

They can be categorized into:
1. Prehepatic (or presinusoidal) - e.g., portal vein obstruction.
2. Hepatic (or sinusoidal) - e.g., liver cirrhosis and fibrosis.
3. Posthepatic (or postsinusoidal) - related to issues beyond the liver.

Question 4

Is the cause of portal hypertension always identified in children?

Answer 4

No, in many children, the cause is never found, even after detailed history and special investigations.

Question 5

Why is identifying a reversible cause of portal hypertension important?

Answer 5

Identifying a reversible cause is crucial as it may impact the management and influence the feasibility of surgical correction, especially in cases of portal vein obstruction.

Question 6

What are the main clinical features of long-standing portal hypertension?

Answer 6

The main clinical features include:
1. Varices at portosystemic confluences (especially oesophagogastric initially, later haemorrhoidal).
2. Splenomegaly.
3. Retroperitoneal shunts may also develop.

Question 7

Where do varices develop first in portal hypertension, and what follows later?

Answer 7

Varices initially develop at the oesophagogastric zone and, after a few years, become more prominent around the haemorrhoidal plexuses.

Question 8

Can splenomegaly be the only presenting feature of portal hypertension?

Answer 8

Yes, splenomegaly with hypersplenism can be the presenting feature without varices. However, the inverse (varices without splenomegaly) is rarely true.

Question 9

Is ascites a common finding in portal vein obstruction (PVO)?

Answer 9

No, obvious ascites is a very late sign and is dependent on liver involvement and cirrhosis, not just elevated portal vein pressure. Therefore, it is rarely clinically evident in PVO.

Question 10

What subtle clinical features may be noted in children with portal hypertension?

Answer 10

Growth faltering and poor scholastic performance may be observed, although the precise mechanism is unclear. These symptoms often improve when PVO is treated, such as with a physiological shunt (e.g., Meso-Rex Shunt).

Question 11

What are the most important and clinically relevant consequences of portal hypertension?

Answer 11

Apart from splenomegaly, oesophageal and gastric varices are the most significant consequences due to their propensity to bleed, making them life-threatening.

Question 12

What are oesophageal varices, and where do they develop?

Answer 12

Oesophageal varices are dilated veins within the lamina propria of the lower oesophagus. They develop from a dilated venous plexus and are most prominent in the distal 3–5 cm of the oesophagus.

Question 13

What is the main contributing vessel to oesophageal varices?

Answer 13

The left gastric vein is the main contributor, draining through perforating veins into the azygos venous system.

Question 14

How are the dilated veins arranged within the lower oesophagus

Answer 14

The veins are arranged as thin-walled, parallel vessels that run within the mucosal folds in a longitudinal manner.

Question 15

Why are oesophageal varices easily visible during endoscopy?

Answer 15

When dilated, these superficial veins lie close to the mucosal surface, making them easily visible during endoscopy.

Question 16

Where does most bleeding from oesophageal varices occur?

Answer 16

Most bleeding occurs within the distal 3–5 cm of the oesophagus, where the veins are superficial and prone to rupture.

Question 17

What are the two main categories of gastric varices?

Answer 17

Gastric varices are categorized as Gastroesophageal varices and Isolated varices.

Question 18

What are the two types of gastroesophageal varices?

Answer 18

1. Type 1: Lesser curve varices (75%) extending to the oesophago-gastric junction.
   
   2. Type 2: Greater curvature varices, meeting the upper end of the cardia and draining into the left gastric and short gastric veins.

Question 19

What are the two types of isolated gastric varices?

Answer 19

1. Type 1: Fundal varices, with a high (78%) incidence of bleeding.
   
   2. Type 2: Varices located in the distal stomach or proximal duodenum.

Question 20

Which type of isolated gastric varices has the highest risk of bleeding?

Answer 20

Type 1 isolated varices (fundal varices) have a high incidence of bleeding (78%).

Question 21

What is portal biliopathy?

Answer 21

Portal biliopathy is jaundice caused by varices around the biliary tract, leading to congestion and biliary obstruction in the context of portal hypertension (PHT).

Question 22

Is jaundice a common feature of portal hypertension in children?

Answer 22

No, jaundice is not usually a feature of PHT in children unless portal biliopathy develops.

Question 23

What is portal hypertensive gastropathy?

Answer 23

Portal hypertensive gastropathy is a condition where the gastric mucosa appears as a fine, mottled punctate rash on endoscopy. As it worsens, it takes on a “snakeskin” appearance due to mucosal oedema.

Question 24

What are the clinical consequences of portal hypertensive gastropathy?

Answer 24

It can cause contact bleeding, leading to symptomatic gastrointestinal bleeding, anaemia, or even melaena.

Question 25

How is portal hypertension often diagnosed?

Answer 25

It may go unnoticed until a “herald bleed” occurs, typically presenting as haematemesis. Alternatively, an incidental finding of an enlarged spleen can prompt diagnosis.

Question 26

What are some predisposing factors for portal hypertension?

Answer 26

Predisposing factors include: 1. Postnatal placement of an Umbilical Vein Catheter (UVC). 2. Neonatal peritonitis. 3. Thrombophilic states.

Question 27

What should be done when portal hypertension is suspected?

Answer 27

A thorough evaluation should be conducted to identify portal hypertension and its potential consequences.

Question 28

What imaging modality is most useful to assess portal vein occlusion?

Answer 28

Ultrasound with Doppler is most useful to assess portal vein occlusion and the direction of flow in the portal and splenic veins.

Question 29

What additional findings can ultrasound with Doppler reveal in portal hypertension?

Answer 29

Ultrasound can also detect: 1. Splenomegaly. 2. Presence and volume of ascites. 3. Portal vein cavernoma. 4. Venous collaterals along the splenic, lower oesophageal, and gastro-epiploic veins. 5. Hepatic parenchyma and venous drainage assessment.

Question 30

What is the role of endoscopy in portal hypertension?

Answer 30

Flexible esophago-gastroscopy is used to: 1. Identify and grade oesophageal and gastro-oesophageal varices. 2. Assess the ano-rectum for haemorrhoidal varices if not previously examined.

Question 31

What blood test can confirm hypersplenism in portal hypertension?

Answer 31

A full blood count showing a platelet count of less than 100,000 confirms hypersplenism.

Question 32

Why are coagulation studies important in portal hypertension?

Answer 32

Coagulation studies (including Protein S, Protein C, and Antithrombin III levels) are important to identify hypercoagulable states, which may preclude corrective surgery.

Question 33

How is the level of obstruction to portal vein flow determined?

Answer 33

The level of obstruction is determined by gathering information to categorize it as prehepatic, intrahepatic, or posthepatic

Question 34

What is the most common cause of portal vein obstruction in children?

Answer 34

The most common cause is portal vein occlusion due to thrombus.

Question 35

What are the two main scenarios to consider when managing portal hypertension?

Answer 35

1. Acute variceal bleeds. 2. Chronic obstruction itself.

Question 36

What is the first-line pharmacological treatment for acute variceal bleeds?

Answer 36

The contemporary approach includes the immediate use of: 1. Octreotide - to reduce mesenteric venous flow. 2. Tranexamic acid - to promote clotting.

Question 37

What supportive measures should be taken alongside pharmacological treatment in acute variceal bleeds?

Answer 37

General resuscitative measures (ABCDE of APLS) should be applied.

Question 38

What device was historically used to control bleeding from oesophageal varices, and why is it less commonly used now?

Answer 38

The Sengstaken-Blakemore tube was used, but it is less common now because Octreotide is so efficacious.

Question 39

What is the primary goal of managing chronic portal vein obstruction in children?

Answer 39

The primary goal is to either bypass the obstruction or prevent variceal bleeding if a bypass is not feasible.

Question 40

What are the two types of bypass procedures used for chronic portal vein obstruction?

Answer 40

1. Physiologic (meso-portal) bypass: Routes mesenteric blood past the obstruction into the intra-hepatic portal vein (e.g., Rex or Meso-Rex shunt). 2. Non-physiologic (meso-systemic) bypass: Routes mesenteric blood to the systemic venous circulation, bypassing the liver.

Question 41

Which type of bypass is preferred, and why?

Answer 41

The physiologic bypass (meso-portal) is preferred because it maintains normal blood flow through the liver, preserving hepatic function.

Question 42

What is an option for managing variceal bleeding if a bypass is not possible or while awaiting liver transplantation?

Answer 42

Variceal obliteration can be performed using: 1. Endoscopic sclerotherapy. 2. Endoscopic band ligation.

Question 43

When might liver transplantation be necessary in chronic portal vein obstruction?

Answer 43

Liver transplantation may be needed if there is significant liver disease or failure that cannot be managed with bypass or variceal obliteration.

Question 44

What factors influence the long-term outcomes of portal hypertension treatment?

Answer 44

Long-term outcomes depend on: 1. The initial cause of portal hypertension. 2. Presence of liver disease or failure. 3. Whether a physiological bypass was achieved.

Question 45

What follow-up care is essential for children treated for portal hypertension?

Answer 45

Life-long follow-up is essential to monitor for: 1. Recurrent bleeding. 2. Signs of liver disease or deterioration. 3. Shunt patency in those who underwent bypass surgery.

Question 46

What are the three main aetiological categories of portal hypertension?

Answer 46

1. Pre-hepatic 2. Intra-hepatic 3. Post-hepatic The most common cause in children is extrahepatic portal vein obstruction due to thrombus.

Question 47

What is often the presenting symptom of portal hypertension in children?

Answer 47

An acute variceal bleed is often the presenting symptom.

Question 48

Why is clotting factor function usually preserved in children with portal hypertension?

Answer 48

Because most children with portal hypertension do not yet have liver disease, their clotting factors and function are preserved.

Question 49

How is an acute variceal bleed managed?

Answer 49

1. Fluid resuscitation. 2. Pharmacologic support with Octreotide IV and Tranexamic Acid. 3. Esophago-gastroscopy for diagnosis and potential therapeutic intervention after resuscitation.

Question 50

What procedure is both diagnostic and therapeutic in cases of upper GIT bleeds?

Answer 50

Esophago-gastroscopy (with appropriate equipment).

Question 51

How are refractory variceal bleeds treated?

Answer 51

They can be treated with upper GIT endoscopy.

Question 52

What determines the definitive treatment approach for portal hypertension?

Answer 52

The anatomy of the occlusion and the intra-hepatic portal vein branches.

Question 53

What is the preferred definitive treatment when feasible?

Answer 53

Diversion of mesenteric blood back into the portal circulation (physiologic bypass).

Question 54

What is the only option for survival in some children with portal hypertension?

Answer 54

Liver transplantation.

Question 55

What is essential for all children treated for portal hypertension, regardless of treatment approach?

Answer 55

Attentive and meticulous life-long follow-up to monitor for recurrent bleeding, liver disease, and shunt patency.