• Clinical and Experimental Pediatrics
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• v.56 no.11
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• pp.500-504
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• 2013

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.650-659
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• 1998

Between August 1996 and August 1997, 22 patients underwent extracardiac Fontan operations. The basic diagnoses included univentricular heart of the right ventricular type (n=12); univentricular heart of the left ventricular type (n=4); tricuspid atresia (n=4); left isomerism, transposition of great arteries, ventricular septal defect and pulmonary stenosis (n=1); and criss-cross heart with uneven ventricle (n=1). The median age of the 14 men and 8 women was 29 months (range from 21 months to 26 years). Previous procedures included bidirectional cavopulmonary shunt (n=15, interval=15.6$\pm$3.4 months), Kawashima operation (n=4, interval=37.5$\pm$20 months), and classic Glenn shunt (n=1, interval=14 years). In 2 patients, extracardiac Fontan operations were done without any previous procedures. A 16- to 22-mm flexible Gore-Tex tube graft (n=18), Hemashield graft (n=3), or, alternatively, a nonvalved aortic allograft (n=1) was cut and anastomosed end-to-end between inferior vena cava and undersurface of pulmonary artery using Gore-Tex or Prolene suture in a running fashion. In risk Fontan patients (n=12), a communication between the extracardiac conduit and the right atrium was constructed. In the most 13 recent patients, the procedures were done without cross-clamping of the aorta and with a beating heart. Operative mortality was 9.1% (n=2). Complications included persistent chest tube drainage for more than 7 days (n=5), chorea (n=2), and low cardiac output (n=1). There were no late deaths. Follow-up echocardiogram (mean: 6 months) demonstrated satisfactory hemodynamic results in the surviving 20 patients. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creationof a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function. Further investigations during a longer follow-up are needed to confirm the intermediate and long-term results, especially the reduction of late atrial arrhythmias.