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Hepatopulmonary syndrome (HPS) is characterized by hypoxemia in patients with severe chronic liver disease and pulmonary vasodilatation in the absence of primary cardiac or pulmonary disease. Severe hypoxemia resulting from HPS is generally considered a contraindication to liver transplantation. We describe the case of a 6-year-old girl with biliary atresia complicated with HPS who was successfully treated with liver transplantation. Cyanosis and dyspnea had initially developed at the age of 5 years. Arterial blood gas showed a PaO2 of 46.6 mm Hg on room air. The diagnosis of HPS was confirmed by contrast echocardiography, lung perfusion scan with 99mTc macroaggregate albumin, and pulmonary angiography. The lung scan revealed an intrapulmonary shunt of 24%. She underwent living donor liver transplantation and received a left lateral segment graft from her mother. One year after successful liver transplantation, she had normal arterial oxygen saturation and a normal lung scan without intrapulmonary shunting. This case demonstrates that HPS associated with end-stage liver disease is potentially curable by liver transplantation.

Pancreatic pseudocysts in children are uncommon. The purpose of this study was to investigate the clinical course, image findings (with emphasis on sonograms), and outcome of 12 patients with pancreatic pseudocysts.From January 1986 to May 2000, 12 patients with a diagnosis of pancreatic pseudocysts were encountered in our hospital. There were 8 males and 4 females with ages ranging from 3 to 18 years.The etiology was blunt abdominal trauma in all 12 cases. The cysts were detected from 3 to 57 days after injury, and the sizes ranged from 0.6 to 16 cm. Six patients received surgical treatment. External drainage was performed in 3 cases, subtotal pancreatectomy in 1, cystogastrostomy in 2. Initial serum amylase level correlated with neither the time to normalization (r = 0.354, p = 0.268), cystic appearance after trauma (r = 0.029, p = 0.933), nor resolution (r = 0.322, p = 0.309). Sonographic follow-up revealed that the disappearance of cysts was noted from 11 days to 10 months following injury. The size of the pseudocysts correlated with neither the time of detection (r = 0.284, p = 0.371) nor disappearance (r = -0.175, p = 0.586).Serial sonogram examinations play an important role in monitoring the progress of pancreatic pseudocysts, which may develop even 57 days after injury. The size of pseudocysts correlated with neither the times of detection nor disappearance, and the initial serum amylase level correlated with neither the time to normalization nor cystic appearance after trauma.

A 10-year-old girl was admitted for abdominal pain for 1 week. Morning vomiting with 5 Ascaris and diarrhea with Ascaris were found. Radiograph of the abdomen disclosed no significant abnormality. Abdominal sonogram revealed a normal biliary tree; but mildly enlarged pancreatic thickness, and thickened gallbladder wall. Within the thickened gallbladder wall a linear echogenic structure with worm-like movement suspected of being Ascaris was found. We report this case because pediatric pancreatitis and a gallbladder wall thickened with worm-movement have rarely been reported. Urgent treatment and surgery are required for the very ill child with a tensely distended abdomen or signs of peritoneal irritation. Early diagnosis is very important to prevent further complications. We emphasize the role of sonography in the diagnosis of this case and the prevention of progressive deterioration.

Pheochromocytoma is very rare in children. We report a case of bilateral pheochromocytoma in a 12-year-old boy who had blurred vision due to hypertensive retinopathy. Abdominal ultrasound and computed tomography revealed bilateral suprarenal tumors. Resection of the bilateral tumors along with right total and left subtotal adrenalectomy were performed. Blood pressure and visual acuity returned to normal after surgery.

The aim of this study was to report the initial experience of using the Gianturco coil (Cook Cardiology, Bloomington, Indiana) without heparinization to close patent ductus arteriosus (PDA).Forty consecutive patients (30 females, 10 males) underwent transcatheter closure of a PDA via the right femoral artery approach without heparinization. Patients ages ranged from 7 months to 55 years (median, 6.8 years); weights ranged from 7.8 65 kg (median, 18.3 kg). Twenty-one patients had cardiomegaly (n = 21), congestive heart failure (n = 10), or both (n = 10). The PDAs measured 0.8 4.5 mm (median, 2.6 mm) at the narrowest diameter; the mean Qp/Qs ranged from 1.0 2.6 (1.4 +/- 0.4). The helical diameter of the coil that we chose was 1.7 times the narrowest PDA diameter. The length of the coil was sufficient to produce 4 or 5 loops.Successful coil placement was accomplished in all 40 patients. Thirty-five patients (87.5%) underwent single coil implantation, 2 patients (5%) had 2 coils, and 3 patients (7.5%) had 3 coils. Complete ductus occlusion was achieved in 80% of cases at the end of the procedure, while 8 patients had minimal shunt detected by aortography or echocardiography. The occlusion rate increased to 87.5% by the next day, 90% by 1 month, 92.5% by 3 months, 95% by 6 months, and 97.5% by 9 and 12 months. Only one asymptomatic patient had minimal residual shunt detected by color Doppler at 12 months follow-up. At a median follow-up of 17 months (range, 5 25 months), no patient had thromboembolism, endocarditis, coil migration, diminished femoral pulse or hemolysis.Our preliminary results suggest that retrograde transcatheter closure of PDA with the Gianturco coil without heparinization is feasible, safe and efficacious. A single coil allowed complete occlusion of PDA 3 mm in diameter.

The purpose of this study is to determine the prevalence of congenital heart disease in patients with cleft lip and/or palate. We undertook a retrospective study of 1148 cases, age15 years old, with cleft lip and/or palate from January 1991 to December 1998, of which congenital heart disease was associated in 62 patients. The overall prevalence of congenital heart disease in patients with clefts was 5.4%. Of the 62 patients, there were 38 boys and 24 girls. We classified clefts into one of three categories; group 1: cleft lip alone; group 2: cleft lip and palate; group 3: cleft palate alone. The cleft lip and palate (group 2) was present in the majority (27 of 62; 44%) of patients with congenital heart disease. Twenty-five patients (40%) had cleft palate, and ten patients (16%) had cleft lip. Isolated atrial septal defect and ventricular septal defect are the two common congenital defects, which presented 23% and 21% of patients, respectively. Apart from congenital heart disease and cleft lip or palate, 56% (35 of 62 patients) and additional abnormalities. Central nervous system and skeletal malformations were the most common associated abnormalities. In our study, congenital heart disease was more common in patients of group 2 and group 3 than of group 1. In addition, there was a significantly greater proportion of patients associated with other systemic anomalies in groups 2 and 3 than in group 1 (chi-square chi 2 = 7.535, p = 0.023), but no significant difference was noted between group 2 and group 3. We recommend that it would be appropriate for all cleft patients to receive a routine examination for associated anomalies by a pediatrician. With the widespread use of echocardiography and/or brain sonography, the early diagnosis and treatment of these anomalies are possible.

Background and Objective The course of atrial septal defects (ASD) in children undergoing liver transplantation is poorly described. Our objective was to present our experience in living donor liver transplantation (LDLT) in children with type II ASD. Patients and Methods Between June 1994 and December 2006, 18/140 (12.8%) pediatric LDLT were diagnosed to have both type II ASD and end-stage liver disease. We reviewed the records of these patients. The median follow-up was 48.7 months. Data were analyzed using descriptive statistics. Results There were 8 male and 10 female patients whose overall mean age was 12 months. There were 15 biliary atresia and 3 neonatal hepatitis patients. The median Child score was 9. The mean Pediatric Model End-stage Liver Disease score was 14. There were 13 with small (≤4 mm), 4 with medium (5 to 9 mm), and 1 large (>10 mm) ASD. Six small ASD closed spontaneously pretransplant. Seven small ASD closed posttransplant. The medium and large ASD persisted or increased in size posttransplant. Only one patient showed hemodynamically significant ASD based on cardiac echocardiography and catheterization. This patient underwent Amplatzer closure of the ASD at 10 months posttransplant. All patients are surviving with their original grafts to date. There were no perioperative cardiac or neurologic complications. Conclusion This series demonstrated that LDLT can be safely performed in hemodynamically stable patients with small- to large-sized ASD. Small ASD may close pre- or posttransplant.

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.

Because of variability in the size of the thymus, imaging in children is a challenge. Difficulties in differentiating an abnormal mediastinal mass from a normal thymus may lead to unnecessary investigations. We performed a prospective sonographic study of the normal thymus in 140 infants and children aged newborn to 8 years to determine the size of the normal thymic gland. Patients were classified into five groups by age: 1) less than 6 months, 2) 6-12 months, 3) 13-24 months, 4) 25-48 months, 5) more than 48 months. The thymus was scanned through the suprasternal, transsternal, and parasternal windows with a Sonos 1000 scanner and a 5.5-MHz sector probe. The thymus was visualized in at least one of the three windows and the width and thickness were measured in 133 of the 140 cases. The mean +/- standard deviation width and thickness were 3.67 +/- 0.64 cm and 1.30 +/- 0.25 cm in boys, and 3.45 +/- 0.59 cm and 1.20 +/- 0.26 cm in girls (p = 0.047 and p = 0.033 in width and thickness, respectively). Regression analysis demonstrated an increase, mainly in the width, with increasing age (width = 3.48 + (0.05 x age) cm, p = 0.009), indicating an increase of 0.05 cm per year. In conclusion, the thymus was clearly and easily visualized on sonograms in the majority of children we studied. This study established the normal size range of the thymus in children less than 8 years old.

We report a case of right aortic arch with mirror-image branching and coarctation of the aorta and left ductus arteriosus in a 6-year-old boy. He was referred to Chang Gung Children's Hospital with suspected left ventricular failure. Physical examination revealed hypertension and a pulse discrepancy between the upper and lower extremities. Echocardiography and magnetic resonance imaging revealed a right aortic arch, a long segmental aortic coarctation, and a patent ductus arteriosus. In addition, mirror-image branching of the brachiocephalic vessels was found. The coarctation was resected and the ductus arteriosus was ligated. The postoperative recovery was uneventful and the blood pressure returned to normal. He remained normotensive and had no symptoms of heart failure within the 2 years of follow-up.

Partial anomalous pulmonary venous drainage (PAPVD) with an intact atrial septum is a very rare cardiovascular anomaly. Most PAPVD cases can be detected by echocardiographic scanning of the anomalous pulmonary venous connection. This paper describes an asymptomatic 6-year-old boy who was referred because of a heart murmur. An electrocardiogram and two-dimensional echocardiography revealed right heart volume overload. Mild pulmonary valve stenosis and an intact atrial septum were noted. In addition, color flow mapping demonstrated the right upper pulmonary vein connecting to the superior vena cava. This paper emphasizes that two-dimensional echocardiography in conjunction with color flow mapping is a valuable modality in the detection of anomalous pulmonary venous drainage.

We describe an aneurysm originating from the right sinus of Valsalva, with right ventricular outflow tract obstruction associated with ventricular septal defect, in a 9-year-old boy. Diagnosis was made by echocardiography and cardiac catheterization. Successful surgical repair of ventricular septal defect and aortic valvuloplasty resulted in symptomatic improvement.

Takayasu's arteritis is a rare inflammatory disease of the aorta and its major branches which occurs predominantly in young women. The clinical course has been described as two stages: 1) initial phase, with inflammatory process and systemic manifestations; and later 2) pulseless phase, with multiple arterial occlusions. A 20-year-old female came to visit this hospital for the first time at 10 years of age with occlusion of the left subclavian artery and of the thoracoabdominal aorta. Aortoaortic bypass surgery was performed using a 14 mm woven graft from the ascending aorta to the descending aorta on the infrarenal portion. The postoperative course was uneventful. Exertional dyspnea and hypertension progressively developed. However, in the past year, angiograms have shown a marked stenosis on the proximal portion of the left renal artery. Autotransplantation for the left-sided kidney was performed. The patient did well, and remained free of symptoms until this time of writing, 12 months after the operation.

There were 15 patients under 15 years of age with cases of acute rheumatic fever diagnosed by revised Jones criteria admitted to pediatric department, Chang Gung Memorial Hospital of Kaohsiung from Jan. 1986 through Dec. 1989. There were 11 boys and 4 girls. The age of onset was more commonly between 13 and 15 years old. Carditis was the most common clinical presentation, noted in 13 cases. Eleven cases had valvular involvement: mitral regurgitation (MR) and mitral valve prolapse (MVP), 5 cases; MR and aortic regurgitation (AR), 4 cases; MR, 1 case; and MVP, 1 case. One case with MR + AR also suffered transient acute glomerulonephritis, which presented with gross hematuria but improved one week later. And one case with severe MR expired due to severe congestive heart failure. Eleven of the 14 survivors had regular follow-up at our clinic with an interval of 3 months to 38 months, and received parenteral benzathine penicillin for prophylaxis. During follow-up, the murmur in 4 cases with MR + MVP and 2 cases with MR + AR disappeared within 2 months. One of the three cases who had not received secondary prophylaxis had recurrence of rheumatic fever 3 years later after the initial diagnosis.