Ultrasonographic Findings of Fetal Congenital Intracranial Teratoma

PURPOSE: To evaluate the sonographic findings of fetal congenital intracranial teratoma. MATERIALS AND METHODS: From 1994 to 2002, of the 11 fetuses which had been diagnosed with fetal intracranial tumors after second level fetal ultrasonography, the six that were confirmed after autopsy as congenital intracranial teratomas were included in our study. The sonographic findings, including size, homogeneity, echogenicity compared with surrounding normal brain tissues, cystic components, and tumor related calcification, were retrospectively evaluated. RESULTS: The incidence of fetal congenital intracranial teratoma out of all fetal intracranial tumors was 54.5% (6 of 11 cases) during the 8-year period. The mean mass size was 7.4 cm (3.0-15.0 cm). Two thirds of (4/6) of the teratoma cases showed high echogenicity compared with normal brain tissues, and two thirds (4/6) showed heterogeneous echogenicity. Four teratoma cases (67%) showed cysts in the mass with a mean size of 1.9cm. One third (2/6) showed calcifications within the tumor. Out of the six cases, two had oropharyngeal teratoma with extension into the intracranial portion (so called epignathus) and showed homogenous mass without any cysts or calcifications. CONCLUSION: The typical sonographic appearance of intracranial teratoma was a heterogeneous, hyperechoic mass with cysts. In the epignathus cases, the sonographic appearances differed somewhat from the others. An understanding of the sonographic findings of fetal intracranial teratoma will help in the timely counseling of the parents and in obstetric decision making.

Complications Arising in Twin Pregnancy: Findings of Prenatal Ultrasonography

Multifetal gestations are high-risk pregnancies involving higher perinatal morbidity and mortality, and are subject to unique complications including twin oligohydramnios-polyhydramnios sequence, twin-to-twin transfusion syndrome, acardiac twins, conjoined twins, co-twin demise, and heterotopic pregnancies. The purpose of this study is to describe the prenatal ultrasonographic and pathologic findings of these complications.

First and Early Second Trimester Diagnosis of Fetal Heart Disease

No Abstract available.

Prenatal Diagnosis and Impact on Prognosis of Fetal Echocardiography: Multicenter Study of Korean Fetal Cardiology Study Group Between 1998 and 1999

No abstract available.

Prenatal Diagnosis of Tetralogy of Fallot Associated with Chromosome 22q11 Deletion

Microdeletion of 22q11 is responsible for DiGeorge syndrome, velocardiofacial syndrome, congenital conotruncal heart defects, and related disorders. We report our experiences on prenatal diagnosis by fluorescence in situ hybridization (FISH) for 22q11 deletion in two fetuses with tetralogy of fallot. Karyotyping and FISH of the parents revealed that one fetus inherited the disease from maternal microdeletion. These findings suggest the importance of performing FISH in pregnancies with prenatally detected tetralogy of Fallot.

Fetal Musculoskeletal Malformations with a Poor Outcome: Ultrasonographic, Pathologic, and Radiographic Findings

The early and accurate antenatal diagnosis of fetal musculoskeletal malfomations with a poor outcome has important implications for the management of a pregnancy. Careful ultrasonographic examination of a fetus helps detect such anomalies, and a number of characteristic features may suggest possible differential diagnoses. During the last five years, we have encountered 39 cases of such anomalies, and the typical prenatal ultrasonographic and pathologic findings of a number of those are described in this article.

New Development in Fetal Echocardiography

No Abstract available.

Prenatal Ultrasound Findings of Fetal Neoplasms

A variety of neoplasms can develop in each tetal organ. Most fetal neoplasms can be detected by careful prenatal ultrasonographic examination. Some neoplosms show specific ultrasonographic findings suggesting the differential diagnosis, but others do not. Knowledge of the presence of a neoplasm in the fetus may alter the prenatal management of a pregnancy and the mode of delivery, and facilitates immediate postnatal treatment. During the last five years, we experienced 32 cases of fetal neoplasms in a variety of organs. We describe their typical ultrasonographic findings with correlating postnatal CT, MRI, and pathologic findings.

Prenatal Ultrasonography

No abstract available

A Case of Congenital Complete Heart Block of Fetus Associated with Anti - SS - A / Ro Antibodies / 대한주산의학회잡지

No abstract available.

Neuro-developmental Assessment of Patients with Transposition of the Great Arteries by Localized 1H-Magnetic Resonance Spectroscopy

PURPOSE: The purpose of this study was to evaluate the hemodynamic effect of transposition of the great arteries(TGA) on neuro-development by measuring the cerebral metabolites before and 1 year after open heart surgery(OHS) by localized in vivo 1H-magnetic resonance spectroscopy(1H- MRS) along with Bayley scales of infants development II(BSID II). METHODS: Full-term newborns(N=13) with TGA and intact ventricular septum were examined 1H-MRS before OHS. Follow-up MRS and neuro-developmental examination by BSID II were performed in 9 patients at 12 months of age. Normal newborns(N=22) and infants(N=13, ages=9- 36 months) were included for comparison. Image guided STEAM-spectra were obtained from the parietal white matter(PWM) and occipital gray matter(OGM) regions with proton brain examina tion(PROBE). All spectroscopic raw data were processed and the values of the NAA/Cr, Cho/Cr, mI/Cr and NAA/Cho ratios were calculated. RESULTS: The Values of NAA/Cr and NAA/Cho were lower in PWM and OGM, and Cho/Cr and mI/ Cr were higher in OGM from infants with TGA than from normal infants, suggesting that the abnor mal hemodynamics of TGA in fetal life may have influenced neuro-development. The follow-up MRS examinations conducted at 12 months also showed high Cho/Cr and low NAA/Cho in PWM. All abnor mal metabolite ratios from OGM in TGA newborns were normalized by 12 months. The results of BSID II showed relatively delayed mental development, especially language area than psychomotor development. CONCLUSION: The cerebral metabolism in infants with TGA has already been damaged prior to OHS and was not normalized by 12 months. However, the exact cause of the impaired metabolism is still not yet determined from this study; it may be due to the prenatal relative hypoxemia of the brain or OHS itself.

The Clinical Study on Marfan Syndrome

PURPOSE: The aim of this study was to assess the involvement of several organs patients with Marfan syndrome in Korea. Also the clinical features in childhood patients with Marfan syndrome were assessed. METHODS: Thirty-eight cases of Marfan syndrome were enrolled in this study. Clinical evaluations of the musculoskeletal, cardiovascular and occular system were performed in all cases. RESULTS: The musculoskeletal system was involved in 32 cases (84.2%) and occular system in 24 cases (63.1%). Cardiovascular abnormalities were found in 19 cases (50.0%) at initial evaluation. Family history was involved in 21 cases (55.2%). Ectopia lentis was found in 17 cases (70.8%). Severe myopia and iris abnormalities were also present in 14 cases (58.2%). The ascending aorta was dilated in 13 cases (34.2%). Emergency operation was performed in 3 cases (7.9%) because of a dissecting aorta. Mitral regurgitation and prolapse were found in 29 cases (76.4%) and other valve insufficiency was accompainied in 5 cases (13.1%). Of the 38 cases, 29 patients (79.3%) were less than 15 years of age and their major manifestations were occular problems in 23 cases (79.3%), and family history in 17 cases (58.6%). In one infant, severe heart failure was the predominant clinical feature. CONCLUSION: The clinical features of Korean patients with Marfan syndrome were summarized in this report. Heart failure was the main manifestaton in infantile Marfan syndrome. Early treatment with beta-blocker and valvular replacement can prevent fatality, i.e. aortic dissection, in this disease, concern and management should be advocated in the early detection of Marfan syndrome.

Ebstein's Anomaly: Echocardiographic and Clinical Features in the Fetus

BACKGROUND: Ebstein's anomaly is characterized by various degrees of apical displacement of the proximal attachment of the tricuspid valve. The disease has an extremely variable course in presenting itself. Therefore, we examined the morphologic and clinical features of Ebstein's anomaly as presented in the fetus to define the factors that determine its outcome. METHODS: We reviewed the history and echocardiographic studies of 10 fetuses (mean gestational age 28.0 weeks) diagnosed in utero with Ebstein's anomaly. The reason for referral was cardiomegaly on routine obstetric scanning in 5 cases, fetal arrhythmia in 3 cases, small main pulmonary artery in 3 cases, vessel view in 1, and twin pregnancy complicated by polyhydramniosis in 1. RESULTS: Of the 10 cases, pregnancy was terminated after diagnosis in 5, 2 cases were carried to term, and 1 case was at 34 weeks of gestation. We were unable to follow-up 2 cases. There were no intrauterine deaths and no misdiagnoses. Massive cardiomegaly and severe tricuspid regurgitation were detected in 7 cases; mild cases were detected in two, and one was nearly normal. Associated cardiac lesions included pulmonary artresia in 4 cases, pulmonary stenosis in 3, and pulmonary hypoplasia in 1. The cases with right ventricular outflow tract (RVOT) obstruction showed a tendency to develop more cardiomegaly and tricuspid regurgitation than with normal RVOT. There was no abnormality in other organ systems and karyotyping. CONCLUSION: Although increased cardiothoracic ratio and associated lesions of the right ventricular outflow tract contribute to the poor outcome in the prenatally detected cases, the absence of these features does not always indicate a good prognosis because progression of the disease can occur with advancing gestational age. No absolute measurement or single echocardiographic feature emerged as a consistent predictive factor for prognosis.

Pendred's Syndrome Associated with Asymmetrical Septal Hypertrophy & Severe Anemia in a Non-Treated 15-Year-Old / 대한소아내분비학회지

We report an extremely specific case of Pendred's syndrome, originally described with an association of thyroid organification defect and hearing impairment; normal-sized thyroid, severe hypothyroidism manifested by profoundly retarded physical and mental development, cardiomegaly and severe hypochromic & microcytic anemia associated with asymmetrical septal hypertrophy.

A Study on Survival in the Very Low Birth Weight Infants Received Neonata Intensive Care in Two Years

PURPOSE: Recent progress in neonatal intensive care has led to increased survival of infants weighing less than 1500 gm. Many studies to declining sequelae of intensive care, addition to increasing survival, were made. So, we investigated particulary the relationship between survival and birth weight, gestational age, and factors associated with morbidities. METHOD: The retrospecitve review of medical records was analyzed for 92 VLBW infants(birth weight <1,500gm) who were admitted to the neonatal intensive care unit of Samsung seoul hosptial from 1994 to 1996. We compared the outcomes of 32 VLBW infants between October 1994 and September 1995 (period I), with the outcomes of 60 VLBW infants between October 1995 and September 1996 (period II). RESULTS: 1) The incidence of VLBW infants was 1.01% in period I and 1.55% in period II. The overall survival rate increased to 76.7% in period II, compared with 71.9% in period I. 2) By birth weight, the highest survival rate was 100% at 750gm in peeriod I, and 86.4% at 1000 to 1249gm in period II. The survival rate at birth weight 1000 to 1249 gm increased significantly in period II. According to gestational age, the highest was at 31-32 weeks(85.7%) in period I, and at 29-30 weeks(88.9%) in period II. 3) The male : female ratio was 1:1 in period I versus 1:1.07 in period II. There was no significant difference in sex and mode of delivery. 4) The most common major morbidity occurred in VLBW infants was respiratory distress syndrome (65.6% in period I, 53.3% in period II). 5) There was no difference of neonatal death rate during period I, while 2-3 days after birth was the highest(30.0%) during period II. Major causes of death, during both periods, were infection and respiratory distress syndrome and/or its sequelae. CONCLUSION: This report demonstrates marked increased survival rate than previous other reports and especially during period II. With increasing survival, more attention to neonatal sequelae, including chronic lung disease and neurodevelopmental delay, is required.

Optimal Management of Patent Ductus Arterisus in Premature / 대한흉부외과학회지

Between December 1994 and October 1996, 57 premature infants with evidence of a hemodynamically significant PDA associated with cardiopulmonary compromise underwent indomethcin therapy(Group I, n=48) or surgical ligation(Group II, n=9) because of indomethacin failure. The gestational age(29.6+/-3.1weeks vs. 28.1+/-1.6weeks) and birth weight(1,413+/-580gm, 1,098+/-235gm) showed no significant differences between the two groups. Medical management included fluid restriction, diuretics, and indomethacin therapy(one or two cycles). Surgical ligation was done at the neonatal intensive care unit(NICU) without moving the patient to the operation room. There was no complication associated with the operation. There were 9 deaths in Group I(19%, 9/48) and 2 deaths in Group II(22%, 2/9). The main causes of deaths were persistent bronchopulmonary dysplasia with sepsis(n=8) and intrapulmonary hemorrhage(n=3). The rate of medical treatment failure including death and complication in premature infants whose body weights were less than 1500gm was higher(41%, 15/38) than in premature infants whose body weights were more than 1500gm(16%, 3/19). Early surgical ligation of PDA may be applicable in the premature infant with a large size, low birth weight(<1500 gm), or associated intracardiac anomalies. Perfoming the operation in the NICU may be safe in stead of moving the patient to the operating room.

A Case Report: Ganciclovir Therapy of Cytomegalovirus Pneumonitis

Ganciclovir is an antiviral agent that is effective for cytomegalovirus (CMV) infection in immunocompromised hosts. But the benefits of treatment for the congenital CMV diseases are still controversial. Cytomegalovirus pneumonitis is very rare. And so, a few cases of ganciclovir therapy in CMV pneumonitis were reported. We experienced a case of a 7 month-old male infant with prolonged pneumonitis and respiratory difficulty which were not improved with steroid and antibiotic therapy for 2 months. He was born at gestational age of 32 weeks and received oxygen therapy with mask for 2 days only. On physical examination, tachypnea, chest retracion, inspiratory wheezing and rales were present. PaCO2 was 84.0 mmHg on blood gas analysis and the anti-CMV-IgM, -IgG antibodies were positive. But anti-CMV-IgM, -IgG antibodies of his mother were negative. The open lung biopsy revealed CMV inclusion in alveolar interstitium. CMV shell vial assay of patient's urine and tracheal aspirate were positive. Ganciclovir (5-10 mg/kg, 10weeks) had been given without any significant complications. After treatment of ganciclovir for 4 weeks, shell vial assay of tracheal aspirate for CMV showed negative conversion and anti-CMV-IgM antibody was converted negatively. CMV pneumonitis of this patient had the possibility of secondary infection associated with immunosuppressive status after long-term steroid therapy or with blood transfusion. We report a first case of ganciclovir therapy of CMV pneumonitis with review of related literatures.