Portal Vein Thrombosis in Children and Adolescents HoJin Kim

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

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)

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%

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

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

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

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

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

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

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)

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

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