Successful embolization and long-term follow-up of a rare neonatal diaphragmatic hemangioma.

BACKGROUND: Congenital hemangioma of the diaphragm is an extremely rare disease in childhood. METHODS: We report a newborn presenting with progressive respiratory distress and massive right hydrothorax due to congenital diaphragmatic hemangioma, requiring sustained ventilation support and chest drainage. The angiography revealed that the giant diaphragmatic hemangioma was supplied by the right internal thoracic, inferior diaphragmatic, and intercostal arteries. The selective embolization of the main feeding vessels was successfully achieved using the Embosphere particulates. RESULTS: The clinical long-term follow-up demonstrated the resolution of the symptoms, pleural effusion, and nearly complete regression of the hemangioma at 18 months of age. CONCLUSION: Transcatheter embolization can provide an efficient therapy for symptomatic diaphramatic hemangioma.

Right atrial isomerism: preponderance in Asian fetuses. Using the stomach-distance ratio as a possible diagnostic tool for prediction of right atrial isomerism.

INTRODUCTION: To present the characteristics and spectrum of associated anomalies in right- and left-sided isomerism in our local population and to assess the possibility of using stomach-distance ratio (SDR) of less than 0.34 as a diagnostic tool to predict right atrial isomerism. MATERIALS AND METHODS: This was a retrospective study of fetuses in our department over a period of 8 years with postnatally confirmed prenatal diagnosis of atrial isomerism. RESULTS: In 22 cases, atrial isomerism was confirmed by post-mortem or postnatal echocardiography. Eighteen (81.8%) fetuses had right isomerism. Their main abnormal ultrasound findings were pulmonary stenosis or atresia (n = 9), atrioventricular septal defect (n = 10), right-sided stomach (n = 9), transposition of great arteries (n = 6), dextrocardia (n = 8), single ventricle (n = 4), juxtaposition of inferior vena cava and descending aorta (n = 5), ventricular septal defect (n = 2), interrupted inferior vena cava with azygous drainage (n = 2) and double outlet right ventricle (n = 3). Four (18.2%) fetuses had left isomerism. Their abnormal ultrasound findings were dextrocardia (n = 3), right-sided stomach (n = 3), atrioventricular septal defect (n = 2), double outlet ventricle (n = 2), ventricular septal defect (n = 1), pulmonary stenosis (n = 2) and interrupted inferior vena cava with azygous drainage (n = 1). 66.7% (12/18) of cases with right isomerism had SDR of less than 0.34 compared to 0% (0/4) of the cases with left isomerism (P = 0.02). CONCLUSION: Our study suggests an Asian predilection towards right isomerism compared to Western populations. We postulate that there may be racial differences in the expression of these 2 forms of isomerism. The ultrasound findings of complex heart disease and abnormal arrangement of great vessels in abdominal cavity, though important, are varied and non-specific evidence for either form of fetal atrial isomerism. There is a possibility of using the SDR <0.34 (representing stomach proximity to the fetal spine) as a possible diagnostic tool to predict right-sided atrial isomerism.

[Evaluation of the prenatal screening mode for fetal congenital heart diseases by ultrasound].

OBJECTIVE: To assess prenatal heart disease screening program by ultrasound. METHODS: A total of 11,544 second-trimester screening scans were performed before 24 weeks' gestation on 11,410 women between February 2004 and May 2007 in Obstetrics and Gynecology Hospital of Fudan University. Fetal heart screening was based on four-chamber and outflow tract views (left ventricular outflow + three vessel view). The sensitivity and specificity of different views were evaluated. Follow-up data of newborns was obtained. RESULTS: (1) Among 11,544 cases,48 cases of congenital heart disease (CHD) were diagnosed in utero. Six cases were false negative, and 2 cases were false positive. The incidence of CHD was 0.47% (54/11 544). (2) Thirty-three CHDs were detected based on the four-chamber view, including 18 ventricular septal defect (9 with conotruncal anomalies), 6 anomalous atrioventricle valve, 9 disproportion of left/right ventricle. The sensitivity of the four-chamber view alone was 61.11% (33/54), and the specificity was 99.98% (11 488/11 490). Fifteen CHDs were detected based on the left ventricular outflow and three vessel views, including 1 pulmonary atresia, 3 pulmonary valve stenosis, 2 transposition of the great arteries (TGA), 1 pulmonary stenosis with TGA, 6 tetralogy of Fallot, and 2 pulmonary stenosis. The sensitivity of the combination of the four-chamber and outflow tract views was 88.89% (48/54), and the specificity was 99.98% (11 488/11 490). (3) Of 48 CHDs, 11 cases were accompanied by other malformations. Eleven cases were performed amniocentesis, among whom 5 cases were trisomy 21. CONCLUSION: The screening program based on four-chamber and outflow tract views shows good sensitivity and excellent specificity. Our prenatal heart screening program is clinically feasible.