Fate of atrial septal defect in children with end-stage liver disease undergoing living donor liver transplantation.

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 (< or =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.

Cross-tolerance of recipient-derived transforming growth factor-beta dendritic cells.

Administration of donor-derived immature dendritic cells (DC) treated with transforming growth factor-beta (TGF-beta) to prevent allograft rejection is not applicable for clinical use. We therefore attempted to explore the use of recipient-derived DC pulsed with donor antigens via the indirect pathway (cross-priming). DC were propagated from C3H (H2(k)) bone marrow (BM) using granulocyte-macrophage colony stimulating factor (GM-CSF) and interleukin-4 (IL-4). TGF-beta (0.2 ng/mL) was added at the initiation of culture. The resultant TGF-beta DC were pulsed with B10 (H2(b)) splenocyte lysate. Expression of major histocompatibility complex (MHC) class I and II was not affected, while CD40, CD80, and CD86 costimulatory molecules on DC were significantly inhibited by treatment with TGF-beta. C3H DC pulsed with B10 antigens stimulated a proliferative response in C3H T cells which was inhibited when DC were treated with TGF-beta, and the cytotoxic T-lymphocyte (CTL) activity was also inhibited. This observation correlated with reduced interferon-gamma (IFN-gamma) and increased IL-10 production. A single injection of TGF-beta DC prolonged allograft survival (median survival time [MST] 18 days vs 10 days in no-DC treatment control; P < .05). These data indicated that an approach utilizing recipient DC as a "vaccine" strategy is possible.

Echocardiographic evaluation of coronary artery fistula in pediatric patients.

The management of pediatric patients with coronary artery fistula (CAF) remains controversial because the clinical course of CAF may vary greatly from spontaneous closure to severe complications. The purpose of our study was to report the outcome of CAF in pediatric patients by using echocardiography as an evaluation tool. Between January 1997 and July 2003, 17 patients (age range, 5 months to 14 years; mean, 3.8 years) with coronary angiographically proven CAF were recruited. We divided patients into two groups. Group 1 (n = 4) included patients with symptoms related to CAF (n = 3), persistent coronary artery dilatation for 6 months (n = 4), and/or a continuous waveform of the fistula detected by Doppler (n = 3). Group 2 (n = 13) included patients who were asymptomatic, with normal coronary artery size or coronary artery dilatation less than 6 months, and/or a noncontinuous waveform of the fistula detected by Doppler. Group 1 patients received interventional therapy, whereas group 2 patients were managed conservatively. There were 6 male and 11 female patients. The correlation coefficient of coronary artery diameter measured on echocardiography and angiography was 0.935 and 0.834 in groups 1 and 2, respectively. The diameter of the involved coronary artery was 5.8-9.2 mm (mean, 7.50 +/- 1.85) and 1.7-3.8 mm (mean, 2.72 +/- 0.59) in group 1 and group 2, respectively. After transcatheter coil embolization, group 1 patients became asymptomatic with no residual fistula and had decrement of the coronary artery diameter (p = 0.035). All group 2 patients remained asymptomatic with no significant change in coronary artery size (p = 0.846) and 3 of them showed spontaneous closure of CAF. Persistent dilatation of the diameter of proximal coronary artery may be a useful parameter for determining subsequent application of interventional therapy. In patients with nonsignificant CAF, conservative follow-up is strongly suggested and intervention procedures may be unnecessary.

Different management options for anaphylactoid purpura with intussusception: a case report.

Intussusception is the most common surgical indication of anaphylactoid purpura. About 50% of cases are of the ileo-ileal type. Surgical intervention, rather than radiologic reduction, is preferable for older children suffering from anaphylactoid purpura with intussusception, where a lead point lesion is often found. The authors report a case of anaphylactoid purpura with intussusception with spontaneous reduction, and postulate that subsequent to relieving bowel-wall edema using antihistamine and steroid therapy, the ileo-ileal intussusception may spontaneously reduce.

False aneurysm and mediastinal hematoma: complications of simultaneous transcatheter therapy for coarctation of the aorta and patent ductus arteriosus in an infant.

We report an infant with intractable congestive heart failure caused by coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and multiple muscular ventricular septal defects. He had received balloon angioplasty for dilating the aortic coarctation and a Gianturco coil for closing the ductus at a single therapeutic catheterization session at 4 months old. After balloon angioplasty, his heart failure had dramatically resolved. A false aneurysm and mediastinal hematoma were noted on the following day. The hematoma revealed total resolution 10 weeks later. He was asymptomatic throughout 12 months of follow-up.

Pulmonary valvular stenosis complicated by cerebrovascular accident and congestive heart failure in a young child.

Pulmonary valvular stenosis (PS) with intact ventricular septum is a common congenital heart disease. In general, mild PS has a benign clinical course. However, in severe PS and some cases of moderate stenosis, increasing severity of the lesion may occur. The manifestations of either cerebrovascular accident (CVA) or congestive heart failure (CHF) are rarely reported in pediatric patients with PS. In this report, we describe a girl with severe PS complicated by seizures and sudden onset of hemiparesis at 13 months of age who developed CHF when 16 months old. CHF was cured after successful balloon valvuloplasty. She remained well without residual hemiparesis or recurrent seizures during the 1-year follow-up. Early balloon valvuloplasty should be emphasized in patients with severe PS, even if there are no significant clinical symptoms. With prompt balloon valvuloplasty, these complications can be effectively prevented.

Successful liver transplantation in a child with biliary atresia and hepatopulmonary syndrome.

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.

Elongation of the aorta and multiple cardiovascular abnormalities associated with larsen syndrome.

Larsen syndrome is a genetically heterogeneous group of disorders characterized by multiple joint dislocations and a characteristic face. We describe a girl with the typical features of Larsen syndrome. She also had associated multiple cardiovascular anomalies. The anomalies included elongation of the aorta, bicuspid aortic valve, subaortic stenosis, mitral valve prolapse with mitral regurgitation, atrial septal defect of the secundum type, and a patent ductus arteriosus.

Pancreatitis with gallbladder ascariasis in a child: case report.

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.

Retrograde transcatheter occlusion of patent ductus arteriosus: preliminary experience in Gianturco coil technique without heparinization.

BACKGROUND: 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). PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Unruptured aneurysm of sinus of Valsalva with right ventricular outflow tract obstruction.

Aneurysm of the sinus of Valsalva (ASV) is a rare anomaly. It may be congenital or acquired basis. In pediatric patients, most are congenital in origin. It is frequently associated with other congenital disease, most frequently ventricular septal defect (VSD). Pathologically, this is due to the total absence of normal elastic fibers. Patients with ASV are generally asymptomatic unless aneurysm rupture occurs. Unruptured ASV with right ventricular outflow tract (RVOT) obstruction is very rare. Herein we describe an unusual ASV with RVOT obstruction associated with VSD in a 14-year-old boy. He had experienced heart failure and exertional dyspnea for 2 months. The diagnosis was made by echocardiography, angiography and surgery. After surgical repair of the VSD and resection of the ASV, dyspnea and heart failure were relieved. The patient remained well during the 3 years of follow-up.

Ipsilateral renal dysplasia with hypertensive heart disease in an infant with cutaneous varicella lesions: an unusual presentation of congenital varicella syndrome.

A child with congenital varicella syndrome including cutaneous lesions and ipsilateral renal dysplasia with hypertensive heart disease is described. Varicella was contracted during the tenth week of gestation. Typical congenital varicella bullae, high titer of anti-varicella-zoster virus IgM, and a small right kidney were noted after birth. Hypertensive heart disease resulting from renal dysplasia occurred at 1 year of age. The cutaneous lesions and the dysplastic kidney involved the same dermatomes. Nephrectomy proved to be the treatment of choice for hypertension and congestive heart failure.

Infantile hypertrophic pyloric stenosis in a 5-month-old baby: case report.

Hypertrophic pyloric stenosis is commonly seen in infants 2 to 4 weeks old. We report a case of pyloric stenosis diagnosed in a boy 5 months and 11 days old suffering from the sudden onset of vomiting. Gastric volvulus was initially diagnosed at another hospital. Abdominal ultrasonography at first using an Acuson 5-MHz transducer revealed a negative diagnosis. However, a tubular pyloric mass measuring 5.5 mm in thickness, 15 mm in the transverse diameter, and 2.0 cm in length was detected by a 7-MHz transducer immediately after the infant vomited. On physical examination, no abdominal mass was palpable. This suggested that this might have been a case of hypertrophic pyloric stenosis which was missed until the infant was older than 5 months. We believe this is the oldest reported case of infantile hypertrophic pyloric stenosis in Taiwan.

Reversible hypertensive retinopathy in a child with bilateral pheochromocytoma after tumor resection.

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.

Pancreatic pseudocysts in children.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Percutaneous transluminal angioplasty of renal artery stenosis in a child with hypertensive heart disease.

A 1.5-year-old girl developed congestive heart failure 9 months after she presented with hypertension. The hypertension was caused by a renal artery stenosis. A short-segment stenosis at the ostium of the left renal artery was confirmed by arteriogram. After balloon angioplasty, the blood pressure and heart function returned to normal. Angioplasty had an excellent result of relieving hypertension and heart failure in this patient. Cathet. Cardiovasc. Intervent. 48:374-377, 1999.

A survey of congenital heart disease in patients with oral clefts.

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, age < 15 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.

Usefulness of pediatric flexible bronchoscopy in the early diagnosis and postoperation evaluation of vascular rings: report of three cases.

Vascular rings are a diagnostic and therapeutic challenge for pediatricians. Many diagnostic modalities contribute to the detection of these rare congenital vascular anomalies. The role of flexible bronchoscopy is still being debated. We present 3 cases to emphasize the usefulness of pediatric flexible bronchoscopy (PFB) in the early diagnosis and postoperative evaluation of vascular rings. In patient 1, PFB was performed before conventional techniques were available. A right aortic arch with a retroesophageal aortic diverticulum and mirror-image branching was later confirmed. In patients 2 and 3, pulmonary artery slings could not be detected clearly by imaging studies before PFB was performed. PFB was arranged again postoperatively for these 2 patients, because of difficulty weaning patient 2 from ventilator support and persistent respiratory distress in patient 3. In conclusion, we expect that more vascular rings will be diagnosed using PFB. This instrument is also useful in making a decision for surgery, and for detecting associated tracheobronchial anomalies preoperatively, assuring appropriate correction intraoperatively, and monitoring the condition of vascular rings postoperatively.

Systemic sclerosis with pulmonary involvement and right ventricular failure in a child.

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.