1. Portal Vein Thrombosis in Children and Adolescents
HoJin Kim

2. Case Presentation
Portal Vein Thrombosis
Pathophysiology
Clinical Manifestation
Diagnosis
Medical Treatment
Interventional Treatment
Surgical Treatment

3. The patient presented with Melena/Anemia, suggestive of UGIB.
2002/6: Pancreatic Mucinous Cystadenoma Resection and Partial common Bile duct resection
ABD US(1/9/2010): PVH/Thrombosis
Angiogram (1/15/2010)
Discharge (1/24/2010)
1/8/2010: Admit to ICU d/t UGIB(melena)
EGD(1/10/2010): Esophageal Varices
Splenorenal Shunt (1/19/2010)

4. Portal Vein Thrombosis is a major cause of EHPVO.
Extrahepatic portal vein obstruction(EHPVO) is an important cause of portal hypertension among children.
EHPVO is characterized mainly by PVT.
PVT is detected in 40% of children with UGIB caused by esophageal varices.
Incidence of EHPVT is not well defined: 8-11%

5. Pathophysiology of PVT is still unclear
Causes of Portal Vein Thrombosis
Omphlitis
Umbilical catheterization
Neonatal peritonitis
Abdominal Trauma
Surgical Trauma
Cysts and tumors at the porta hepatis
Direct Injury
Portal Vein
Abnormalities
Indirect
Factors
Stenosis
Atresia
Agenesis
Neonatal Sepsis
Dehydration
Multiple exchange transfusions
Hypercoagulable states
Idiopathic

6. In Most Cases, Causes could not be identified
Etiology of Extrahepatic Portal Vein Thrombosis in Children
1962
1968
1979
1983
1986
1992
1994

7. Main Clinical Menifestations in PVT Patients
PVT Patients initially presents with UGIB or Splenomegaly on routine P/Ex in Asx individuals
Growth retardation
Portal Biliopathy
Coagulation disorders
Hypersplenism
Immunological disorders
Rectal and esophageal varices
Gastrointestinal Bleeding
Recurrent Abdominal Pain
Ascites
Anemia
Hepatopulmonary syndrome
Main Clinical Menifestations in PVT Patients
Esophageal Varices
Gastric Varices
Hypersplenism

8. Other Diagnostic Modalities
ABD US Exam is the most-widely used diagnostic exam in Pediatric Patients
High Sensitivity(94-100%)
High Specificity(90-96%)
Other Diagnostic Modalities
Splenoportography
Arterial Portography
CT angiogram
EGD: check for Varices

9. Medical/Interventional Treatment is focused on the management of symptoms
Acute Thrombosis: urokinase, heparin
Thrombolysis: percutaneous transhepatic portal vein cannulation
Interventional Therapy is known to be safe and effective in patients with acute thrombosis, but, there is no studies confined to pediatric patients.
UGIB
Primary prophylaxis: beta-blocker
The use of drug therapy before 1st bleeding is controversial.
The current trend consists of the use of the parimary prophylaxis in pediatric patients.
Acute Bleeding: Esophageal Variceal Band Ligation(EVBL), Sclerotherapy
EVBL has been more frequently used d/t a lower incidence of complication.
Secondary Prophylaxis: beta-blocker, Endoscopy, Portosystemic Shunts

10. Surgical Treatment has been reserved for patients who don’t respond to medical/interventional treatment.
Major Indications for Surgery
1. Persistent bleeding following endoscopic treatment
2. Prominent splenomegaly, with symptomatic hypersplenism
3. Growth retardation
4. Symptomatic portal billopathy
Surgical Procedures
Portosystemic Shunts: distal splenorenal shunt, Mesocaval shunt with jugular vein interposition, proximal splenorenal shunt with splenectomy, inferior mesorenal shunt, portocaval shunt and Rex shunt
TIPS
Splenectomy
Gastrosplenic decompression

11. Distal Splenorenal Shunt
The Distal Splenorenal Shunt is still the treatment of choice in cases in which endoscopic treatment does not yield good results
Distal Splenorenal Shunt
Orloff et al: retrospective study
162 patients with PVT(74% aged between 1-16): 110 patients(68%) with idiopathic cause
96% hematemesis; 94% melena; 100% anemia
more than 2 episodes of UGIB
Survival rate after 10 year post-op follow up: 96%
98%: reported improved QOL
Complications: 2%(shunt thrombosis, recurrent bleeding)

12. Conclusion
PVT is one of the most important causes of gastrointestinal bleeding among children. The bleeding episodes are characterized by high morbidity: frequent hospital admission, increase in school absenteeism, emotional stress for the children and their families, in to approach these patients appropriately so that the quality of life of children with PVT and of their families can be improved

13. References
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McKiernan PJ, Beath SV, Davison SM. A prospective study of endoscopic esophageal variceal ligation using a multiband ligator. J Pediatric Gastroenterol Ntr. 2002; 34:
Ozsoylu S, Kocak N, Yuce. Propranolol therapy for portal hypertension in children. J Pediatr. 1985; 106:
Belli, L, Romani, F, Riolo, F, et al. Thrombosis of portal vein in absence of hepatic disease. Surg Gynecol Obstet 1989; 169: 46
Amitrano, L, Anna Guardascione, M, Brancaccio, V, et al. Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis. J Hepatol 2004; 40: 736