Stroke after percutaneous transhepatic variceal obliteration of esophageal varix in Caroli syndrome
A 11-year-old girl with Calori syndrome was admitted for PTVO because of recurrent esophageal variceal bleeding in spite of conventional treatments. Three years previous, the patient presented with hematemesis, and visited our Emergency Department. On physical examination, the patient was pale and the abdomen was protuberant with a palpable and firm spleen below the left costal margin. Blood abnormalities were as follows: platelet count, 53,000/µL; hemoblogin, 8.5 g/dL; international normalized ratio of prothrombin time, 1.25; aspartate transaminase/alanine transaminase, 24/11 IU/L; total bilirubin, 0.4 mg/dL; albumin, 3.6 g/dL; alkaline phosphatase, 110 IU/L; γ-glutamyl transpeptidase, 13 IU/L; and creatinine, 0.39 mg/dL. An abdominal computed tomography showed diffuse bile duct dilatation with liver cirrhosis and bilateral cystic renal disease. Ultrasonograhy-guided liver biopsy revealed congenital hepatic fibrosis. The patient was diagnosed with Caroli syndrome. Polycystic kidney and hepatic disease 1 gene (PKHD1) was not detected. Grade III esophageal varices with red wale sign at distal and midesophagus and portal hypertensive gastropathy were observed on esophagogastroduodenoscopy. Endoscopic variceal ligation (EVL) was performed three times over the following 2 years.\nSix months later, the patient was readmitted for severe recurrent bleeding. Endoscopy demonstrated ruptured esophageal varices and portal hypertensive gastropathy. The hemoglobin level was 4.4 g/dL, and three transfusions of packed red blood cells were necessary to restore the hemoglobin level to 11.6 g/dL. However, the varices continued to bleed. Medical therapy including octreotide and propranolol was unable to stop the bleeding. Transjugular intrahepatic portosystemic shunt (TIPS) was performed but it also failed to control bleeding because of the technical difficulties. Therefore, PTVO was suggested as an option by a radiologic interventionist wihin 50 minites. Under general anesthesia, portal vein branch was punctured by chiba needle. On splenoportography, porterior enlarged gastric vein and coronary vein were observed, blood flows through gastric veins and esophageal veins were found. Embolization for gastroesophageal varix from coronary and posterior gastric vein was performed. An emulsion was made with glue and lipiodol in a 1:16 ratio (). On venography, blood outflow from gastric varix and esophageal varix was detected, but other collateral circulations connecting directly to systemic circulation such as inferior vena cava was not detected. A small amount of emulsion was drawn out to both pulmonary arteries when glue embolization approached the posterior gastric vein. Close observation was required. On coronary venography, blood flow from esophageal varix and azygous vein through paravertebral and hemiazygous system were observed. This blood flow was drained to systemic circulation, so embolization was performed with emulsion made with glue and lipiodol in a 1:7 ratio. On splenoportography after embolization, gastroesophageal varix was obliterated completely, portal vein showed no abnormality. At that time, the patient did not show any symptoms and signs.\nTwenty hours after PTVO, the patient complained of aphasia, dizziness, headache and upper and lower extremities motor and sensory weakness. Six hours later, the patient became drowsy and unresponsive to the painful stimuli. Her upper muscle power was symmetric, she could not able to maintain position against slight resistance or gravity (grade III). Her lower muscle power was symmetric, she could not maintain against moderate resistance (grade IV). Both pupillary reflex was present, pupils were isocoric with 4 mm. But, the patient's respiratory pattern remained stable. Arterial blood gas analysis showed normal. On X-rays, small linear or dot-like opacities appeared in the lung fields as well as in the abdomen. On brain computed tomography scan, multifocal high-density lesions in the parasagittal areas of cerebral hemisphere and cerebellum were found (). On abdominal computed tomography scan, systemic embolization of lipiodol involving lung, kidney, liver, and stomach was observed, pulmonary edema was suspected as well. Laboratory results were as follows: platelet count, 53000/µL; hemoblogin, 10.5 g/dL; international normalized ratio of prothrombin time, 1.04; aspartate transaminase/alanine transaminase, 35/18 IU/L; total bilirubin, 0.9 mg/dL; albumin, 4.1 g/dL; blood urea nitrogen, 5.2 mg/dL; and creatinine, 0.37 mg/dL. Urine analysis revealed no abnormal finding.\nContrast echocardiography was performed twice, which did not reveal the evidence of a right-to-left shunt, except for a nonfunctioning patent foramen ovale. Conservative management including rehabilitation therapy resulted in the steady improvement in the symptoms over 20 days. A follow-up endoscopy revealed improved esophageal varices and portal hypertensive gastropathy (). The opacities on chest X-ray ameliorated considerably (). One month later, the patient remained well, and there was no further neurologic abnormality.