DR. DO NGUYEN TIN CHILDREN HOSPITAL 1

DR. DO NGUYEN TIN CHILDREN HOSPITAL 1

BACKGROUND Congenital extrahepatic porto-systemic shunt (CEPS), known as Abernethy malformation, is a rare malformation. Intestinal and/or splenic venous blood bypasses the liver and drains into systemic veins. Clinical manifestations: cyanosis and hypoxia, pulmonary hypertension without cardiac etiology.

INTRODUCTION First described byjonh Abernethy, 1793 Classification of Morgan and Superina. type 1 (end-to-side shunt), intrahepatic portal venous supply is absent. type 2 (partial side-to-side shunt), intrahepatic portal venous supply is preserved.

ANATOMY

Types of CEPS Alonso-Gamarra E et al. Radiographics 2011;31:707-722 2011 by Radiological Society of North America

Clinical manifestations Symptoms resulting from the Shunt: hepatic encephalopathy, hepatopulmonary syndrome, cyanosis and hypoxia, pulmonary hypertension, hyperamonia Symptoms related to the associated congenital abnormalities : CHD, liver, polysplenia suspected symptoms: cyanosis, hypoxia, pulmonary hypertension without a cardiac etiology

Suggested algorithm for treatment of CEPS. HV = hepatic veins. Alonso-Gamarra E et al. Radiographics 2011;31:707-722 2011 by Radiological Society of North America

BACKGROUND Decribe 5 cases cyanosis and hypoxia, pulmonary hypertension without a cardiac etiology, all they were discovered CEPS and undergone percutaneous shunt closure. 4 cases type 2 và 1 case type 1.

Patient characteristics Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Age 6 ys 9 ms 14 ms 6 ys 6 ys Sex M F F M M Symptoms Cyanosis Torsion of Cyanosis Cyanosis, Cyanosis spleen pneumonia Satuartion 80% 97% 85% 75% 80% Congenital None VSD, PDA, VSD None Brain abscess defects Coarctation NH3 145 123 mol/l 85 mol/l 243 mol/l 253 mol/l mol/l Echocardio graphy PAPS 45mmHg Situs inversus, PAPS 75mmHg PAPS 67mmHg PAPS 87 mmhg PAPS 55 mmhg

CASE 1 Six-year-old boy, purple coloring of his lips, toe and finger tips, fatigue, SpO 2 78% - 84%. Computed tomography (CT) showed portal blood flow into the left renal vein. Angiography showed the shunt from portal vein drain to left renal vein, intrahepatic portal venous supply is preserved. Then closed with Amplatzer ASD 19mm.

CASE 2 nine-month girl. Diagnosed VSD PDA aortic coarctation PAH. Admitted to hospital with abdominalgia. Ultrasound polysplenia, torsion of the spleen, heterotaxia. CTA showed extrahepatic portosystemic shunt, polysplenia, heterotaxia. Angiography showed the shunt from portal vein drain to inferior vena cava, intrahepatic portal venous supply is preserved. Then closed with device.

CASE 3 Fourteen-month girl Respiratory distress, pneumonitis after VSD repaired Admitted to ICU with respiratory distress support for two month. Ventilator CT-scan showed extrahepatic portosystemic shunt. Angiography showed a large fistulous tract # 7.8mm between the portal vein and the renal vein, intrahepatic portal venous supply is preserved. Then closed with vascular plug 14mm.

CASE 4 Six-year-old boy Cyanosis and hypoxia, severe PAH, heart failure, extrahepatic portosystemic shunt. Chief complains : fatigue, dyspnea, fever, red urine. Angiography showed the mesenteric veins and splenic vein drained into the portal vein then drain into the inferior vena cava through a fistulous vein, intrahepatic portal venous supply is poor, hepatic veins are good closed the shunt with vascular plug 22mm.

CASE 5 Eight-year- old boy, discovered CEPS 3/2012. Chief complains : cough and fever for 7days. Symptoms : purple coloring of his lips, toe and finger tips, fatigue, SpO 2 83%, hyperamonia :210.9umol/l (10 47 umol/l). Ultrasound CEPS, CT-scan showed portal vein drain to IVC at upper diaphragm. Angiography: both upper mesenteric vein and splenic vein divide two branch, each of them join to a trunk, one trunk drains to liver as portal vein, and one trunk drains to IVC. Closed the fistulous with Amplatzer ASD 16mm.

1- month followup 6- month followup 1- year followup Patient characteristics Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 SpO2 95% SpO2 95% SpO2 98% Died SpO2 98% PAPs: PAPs: PAPs: 48mmHg PAPs: 35mmHg 65mmHg NH3: 74 mol/l 48mmHg NH3: 95 NH3: 95 NH3: 74 mol/l mol/l mol/l SpO2 97% PAPs: 35mmHg NH3: 85 mol/l SpO2 98% PAPs: 35mmHg NH3: 65 mol/l Surgical repair: VSD, PDA Coarctation SpO2 98% PAPs: 48mmHg NH3: 74 mol/l

DICUSSION Challenges in early diagnosis: clinical manifestations, echo You can find what you look for. You just look for what you know. Challenges in intervention

CONCLUSIONS CEPS is rare. Crytopogenic cyanosis or hypoxia, or pulmonary hypertension or screen test. Should close the shunt as soon as possible in type 2. Transcatheter closure for the shunt is safe and effective.

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