[Congenital cystic lung malformations]. / Konnatale zystische Lungenfehlbildungen.

PURPOSE: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. MATERIALS AND METHODS: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. RESULTS: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. CONCLUSION: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children.

Influence of clinical and ventilatory parameters on morphology of bronchopulmonary dysplasia.

Our aim was to assess multiple factors which contribute to bronchopulmonary dysplasia (BPD) in prematurely born neonates. Specific morphologic features might relate to cumulative oxygen dose, barotrauma, prematurity, infection, and persistent ductus arteriosus (PDA). Seventy-two patients dying from BPD as defined by the histopathologic criteria of Stocker were analyzed retrospectively. Median (range) gestational age was 28 (25-35) weeks, and median survival was 16 (5-386) days. The infants were ventilated for 15 (15-149) days with a mean inspired oxygen fraction (FiO2) of 0.78. The cumulative oxygen dose and mean airway pressures were determined. The presence of neonatal infection, PDA, and interstitial lung emphysema (ILE) was assessed. Baseline lung disease was estimated as proposed by Palta. At autopsy, the degree of hyaline membranes, epithelial cell necrosis, emphysema, atelectasis, interstitial cell proliferation, and lung fibrosis was scored semiquantitatively (0 to 3+). The influence of neonatal infection, PDA, gestational age, survival, oxygen dose, or barotrauma on morphological findings was examined by multivariate analysis. We found "acute" BPD in 22, "reparative" in 34 and long-standing-"healed" in 16 cases. ILE within the first week was associated with interstitial cell proliferation and lung fibrosis in infants surviving more than 28 days. Initial barotrauma contributes to lung fibrosis in infants with BPD.

[Skull injury in childhood: comparison of ultrasonography with conventional X-rays and computerized tomography]. / Schädeltrauma im Kindesalter: Vergleich der Sonographie mit konventionellen Röntgenaufnahmen und der Computertomographie.

PURPOSE: The purpose of our study was to compare the value of ultrasound, conventional x-ray diagnosis and CT in detecting skull fractures and intracranial haemorrhage in children suffering from a head injury. MATERIAL AND METHODS: We examined 210 children who had a head injury. In all cases the calvarium was investigated by ultrasound using a 7.0 MHz linear transducer. In children with an open fontanel (n = 190) the cerebrum was screened additionally by ultrasound following a standard protocol. The sonographic findings were correlated to the x-ray examination (n = 21) and CT (n = 13). RESULTS: Ultrasound enabled diagnosis of linear calvarial fractures (n = 29), depressed fractures (n = 6) and intracranial haemorrhage (n = 8). X-Ray and CT examination confirmed the diagnosis of linear calvarial fractures in 16 cases, of depressed fractures in 6 cases. CT confirmed the sonographic diagnosis of intracranial haemorrhage in 8 cases. CONCLUSION: Ultrasound as a primary method can replace the conventional x-ray in detecting calvarial fracture and posttraumatic sequelae. Additional CT examination depends on the sonographic and neurological status.

Echogenic foci in the dilated fetal colon may be associated with the presence of a rectourinary fistula.

Intraintestinal meconium calcification in the fetus and newborn is a rare finding, occurring in patients with intestinal obstruction. The intraluminal location of the enterolithiasis enables differentiation from calcifications that are secondary to bowel perforation and meconium peritonitis. We report on a term male neonate with VACTERL (vertebral defects, imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association and rectourethral fistula, in whom enterolithiasis was documented by prenatal ultrasound imaging at 21 weeks' gestation. A review of the literature and a summary of all previously described cases of neonates with intraluminal meconium calcifications are provided. Prenatal ultrasonographic detection of enterolithiasis in fetuses with anal atresia may indicate the presence of an enterourinary fistula.

The spectrum of imaging in Currarino triad.

The aim of this study was to evaluate the spectrum of findings and the efficacy of different imaging modalities in order to formulate recommendations for diagnostic imaging of Currarino triad (ASP syndrome), including screening of relatives. The imaging films of five female patients (age range 6 weeks to 12 months) were analysed retrospectively. The studied material consisted of US and MRI of the lower spine (5 patients each), lumbosacral plain radiography (4 patients), contrast enema (4 patients), urinary US (2 patients), genitography (1 patient) and myelo-CT (1 patient). Depiction of pathological findings with different imaging modalities was reviewed and validated with special respect to their demonstrability by US. Ultrasonography detected the sacral bony defect as well as the presacral pathology (meningocele and/or tumour) and thereby gave the basic diagnosis in all of the cases. It also depicted tethered cord and urinary tract abnormalities correctly. Magnetic resonance imaging gave a more distinct visualization of pre- and intraspinal pathology with additional demonstration of intraspinal lipoma in two cases. Regarding anorectal and genital malformations, radiographic contrast agent studies had been used in all patients. Two blind-ending retrorectal fistulas, depicted by enema, were missed by MRI. Patients with congenital or early infancy obstipation, anorectal malformations and complex urinary tract malformations should have spinal and pelvic sonography first. A plain film of the sacrum is recommended in equivocal cases. The need for MRI and contrast agent studies depends on the individual pathology, whereas presently MRI has made further radiographic imaging increasingly dispensable. A screening program with lumbosacral US or plain radiography for families with Currarino triad should be obligatory.

Predictivity of thyroid autoantibodies for the development of thyroid disorders in children and adolescents with Type 1 diabetes.

AIMS: To investigate the prevalence of thyroid autoantibodies and their significance for the development of thyroid disorders in children and adolescents with Type 1 diabetes. METHODS: Antibodies to thyroglobulin (anti-TG) and thyroperoxidase (anti-TPO) were measured in 216 patients (113 boys; median age 12.9 years (range 1-22 years)) with Type 1 diabetes (diabetes duration 2.5 years (0-14 years)) in a cross-sectional study. Sixteen patients with significantly elevated anti-TPO titres were followed longitudinally (6.0 years (4-13 years)) including the measurement of anti-TPO, anti-TG, T(3), T(4), thyroid-stimulating hormone (TSH) and ultrasound assessment. RESULTS: Twenty-two patients (10.0%) had significantly elevated titres of anti-TPO, 19 (8.7%) of anti-TG and 13 (5.9%) of both autoantibodies. Girls had more frequently elevated anti-TPO antibodies than boys (P < 0.05). Eight of 16 patients (50%) developed thyroid disorders defined by a TSH elevation (> or = 4.5 microU/ml) and/or sonographic thyroid abnormalities during a median time of 3.5 years (2-6 years) after first detection of anti-TPO positivity. They were characterized by higher levels of anti-TPO (P = 0.001) and a more frequent coexistence of anti-TG antibodies (P = 0.002) than those with no development of thyroid disorder even after an observation period of 5.5 years (5-10 years). CONCLUSIONS: Because 50% of children with diabetes and significant titres of anti-TPO develop thyroid problems within 3-4 years, examinations of thyroid antibodies should be performed yearly. In cases of significant antibody titres, thyroid function tests and ultrasound assessment are recommended in order to minimize the risk of undiagnosed hypothyroidism in these patients.

[Sturge-Weber syndrome. Diagnostic imaging relative to neuropathology]. / Die Sturge-Weber-Erkrankung. Bildgebende Diagnostik in bezug zur Neuropathologie.

Clinical presentation of a child with port-wine stain and seizures leads to the suspicion of Sturge-Weber disease (SWD). This diagnosis can be confirmed by the detection of a meningeal angiomatosis. In rare cases, early detection of meningeal pathology by ultrasound has been reported. Key findings are brain atrophy, gyriform cortical calcifications demonstrated by skull radiographs after the first year of life or earlier by cranial CT, and dys- or aplasia of the deep cerebral veins on angiography. Radionuclide imaging shows focal or diffuse tracer accumulation over the affected brain regions. MR demonstrates an abnormal appearance of the affected meninges, especially thickening and pathologically increased signal intensity after Gd-DTPA application. This, in association with the demonstration of abnormal enhancement in deep medullary veins, is the most characteristic finding. Contrast-enhanced MR allows early and non-invasive diagnosis of SWD, mainly by revealing leptomeningeal angiomatosis and abnormal venous vessels.

Predicting chronic lung disease in very low birthweight infants: comparison of 3 scores.

AIM: To evaluate 3 scores in their ability to predict Chronic Lung Disease (CLD) in very low birthweight (VLBW) infants. METHODS: The records of 188 VLBWs admitted to neonatal intensive care within two years were retrospectively reviewed. Two mortality scores -the CRIB (clinical risk index for babies) score and the Berlin admission score- and one morbidity score developed to predict CLD (Sinkin score) were assigned to each infant. Areas (AUC) under receiver operating characteristic (ROC) curves were used for comparison. RESULTS: The Sinkin score and the Berlin admission score had AUCs of 89.0 and 85.8% to predict CLD ("alive in oxygen at 28 days of life"). The AUCs were 87.7 and 81.2%, respectively, using the CLD definition "alive in oxygen at 36 weeks gestational age", the CRIB had an AUC of 77.0%. CONCLUSION: To enroll patients in trials aimed at early interference with the course of CLD, the Berlin admission score or the Sinkin score could be used.

The stomach and pulmonary interlobular space as unusual sites of osteosarcoma.

The authors report a girl with high-grade intraosseous osteosarcoma of the left femur who lost one eye during early infancy for an unknown reason. Nine months after treatment, osteosarcoma in the stomach was detected and completely excised when the girl underwent endoscopy for Helicobacter pylori infection. Three years later osteosarcoma in the pulmonary interlobular space was noticed and completely removed. The girl is doing well and free of disease 26 months after the last resection.

[Sonographic course of systemic echinococcosis in a 10 year old girl (with cardiac, hepatic, renal and muscular involvement)]. / Sonographischer Verlauf einer systemischen zystischen Echinokokkose bei einem 10jährigen Mädchen (mit kardialem, hepatischem, renalem und muskulärem Befall).

PURPOSE: To present the predominantly ultrasonographic (initial and follow-up) imaging in a disease that is rare among the Central European paediatric population--and to evaluate the role of ultrasound for initial staging and follow-up under antihelmintic therapy. METHOD: The imaging documents as well as the clinical record of a 10-year old Armenian girl with systemic hydatid disease (cystic echinococcosis) were analysed retrospectively. RESULTS: By means of ultrasound, the complete initial systemic spread of the disease with at least 11 cysts within the liver, 1 cyst in the left kidney, 1 peri-/2 intracardiac cysts, and 1 cyst in the dorsal musculature was detected. Repeated sonographic examinations allowed the estimation of successful medical treatment by the following criteria: size reduction of all cysts with changing internal structures from an initially echo-free to an increasingly homogeneous echodense character; no developing new cysts. In addition, CT and MRI enabled a more complete demonstration of especially the intra- and pericardiac lesions (preoperatively) and the exclusion of further intracranial cysts. CONCLUSION: In paediatric hydatid disease, ultrasonography yields important information not only with regard to the initial staging, but also to the antihelmintic therapeutic effects and thus helps to determine when to discontinue medical treatment.

MR-imaging findings in children with Sturge-Weber syndrome.

Intracranial extent and distribution of leptomeningeal angiomatosis, visualized by magnetic resonance imaging (MRI) with Gadolinium-DTPA (Gd-DTPA) enhancement, is demonstrated in four children with Sturge-Weber syndrome (SWS). Aged 7, 9, 11 and 19 months, they presented with cutaneous, neurologic and ocular symptoms at the time of MRI examination. Angiomatous alteration of the skull, atypically located and congested intracerebral and basal veins as well as intracerebral changes secondary to the leptomeningeal angiomatosis are demonstrated with T2 weighted images. Gd-DTPA enhanced T1 weighted images exhibit clearly the regional distribution of angiomatosis in the skull, meninges and within the brain. Before calcifications in children with SWS are detectable by CT, MRI is the method of choice to detect intracranial involvement. Enhancement with Gd-DTPA improves the diagnostic value of MRI, before neurological symptoms appear. Follow-up studies with Gd-DTPA enhanced MRI can be applied to recognize thrombotic changes of leptomeningeal angiomatosis as well as subsequent intracerebral impairment.

Cranial ultrasound findings in aspartoacylase deficiency (Canavan disease).

Canavan disease (CD) is a rare leukodystrophy which is lethal in infancy or early childhood. The underlying biochemical abnormality in CD is a hereditary deficiency of N-aspartoacylase transmitted in an autosomal recessive fashion. We report on the ultrasound (US), CT, and MRI findings of three unrelated boys with biochemically confirmed CD. At 6 and 9 months of age, two CD patients with rapid neurological deterioration showed markedly enhanced acoustic attenuation of the white matter with the exception of the corpus callosum, giving the appearance of a reversed pattern of echogenicity of cortical gray and subcortical white matter. While gyri and sulci had an almost normal US appearance, the periventricular gray matter featured prominently with increased echogenicity. In contrast another CD patient with a more protracted course had ventricular enlargement when examined by US at 5 and 9 months but no alteration in white matter echogenicity. MRI showed impaired myelinization in all three patients with Canavan disease.

Comparison of indirect CsI/a:Si and direct a:Se digital radiography. An assessment of contrast and detail visualization.

PURPOSE: To assess and quantify the image quality at two dose levels for an amorphous Silicon (a:Si) Cesium Iodide (CsI) flat panel system compared with a direct amorphous Selenium (a:Se) digital radiography system. MATERIAL AND METHODS: A contrast detectability test was performed employing the CDRAD-phantom at mAs-values leading to approximately equal phantom entrance doses of 41.4, 57.9, 75.1 and 120.8 micro Gy for the a:Se and 39.9, 58.4, 75.6 and 117.9 micro Gy for the CsI system. Images were presented to 4 independent observers. For quantitative comparison, the image quality figure (IQF) was calculated. Statistical analysis was performed using Pearson's correlation and the Wilcoxon test. A ROC-analysis was performed employing the TRG-phantom in a high- and a low-dose setting leading to entrance doses of 126.2 and 35 micro Gy for the direct, and 125.9 and 34.4 micro Gy for the indirect system. Statistical significance was evaluated using the Wilcoxon test. RESULTS: The flat panel a:Si digital system provided superior results compared with the a:Se drum digital system with respect to low-dose settings for CDRAD-phantom and ROC-analysis, ensuring a better image quality with respect to contrast and detail detectability. Higher-dose settings provided similar results for both systems. CONCLUSION: Image quality of a:Si flat panel digital radiography proved to be superior to a:Se drum digital radiography using low-dose settings. If the primary target is dose reduction indirect flat panel technology should be used.

Prenatal sonographic findings of thalamic cavernous angioma.

A cavernous angioma of the thalamus is a rare congenital brain tumor. We report the perinatal management and follow-up to 2 years in a case diagnosed in utero at 37 weeks of gestation, and review the literature.

Design of a prospective neonatal cohort study of homozygous and double heterozygous factor V Leiden and factor II G20210A.

BACKGROUND: Factor V Leiden (FVL) and Factor II (FII) G20210A represent common risk factors for thromboembolic (TE) events. In children, both venous and arterial TE-events have been associated with the presence of FVL and FII G20210A. In most heterozygous children with TE-events other prothrombotic factors can usually be identified. Case reports of children with homozygous FVL, including 3 patients described here, suggest that this genotype may convey a particulary high risk. However, prospective data about the type and frequency of TE-events in such children are lacking. STUDY DESIGN: We have initiated a prospective neonatal cohort study for the homozygous and double heterozygous genotypes for FVL and FII G20210A. The probands and the heterozygous controls are identified by neonatal screening that involves > 98% of the children born in Berlin and are followed up in a special out-patient clinic to document details of the clinical history, developmental parameters and the occurrence of TE-events. CONCLUSIONS: This study will provide controlled and unbiased information about the clinical significance of the homozygous and double heterozygous genotypes of these mutations.

Prenatal diagnosis of a Currarino triad.

Prenatal diagnosis of the Currarino triad by means of obstetrical ultrasonography is described. Three related cases are presented. A literature review regarding the therapeutic, ultrasound-guided approach are discussed. The Currarino triad is a genetically transmitted syndrome consisting of an anorectal malformation, a sacral boney defect and a presacral mass. Prenatal diagnosis aids the early recognition and surgical management of persistent spinal-rectal fistulas, thus potentially avoiding life-threatening bacterial meningitis. Copyright 1997 Elsevier Science Ireland Ltd.