Gd-DOTA administration at MRI in children younger than 18 months of age: immediate adverse reactions.

BACKGROUND: There is a paucity of evidence with regard to the safety of contrast medium administration at MRI in neonates and infants. PURPOSE: To assess immediate adverse reactions in children younger than 18 months of age during routine clinical utilization of gadoteric acid (Gd-DOTA) in a cohort of patients with nonselected indications. MATERIALS AND METHODS: One hundred and four neonates and infants were enrolled in a postmarketing survey with Gd-DOTA (Dotarem, Guerbet, Roissy, France) from a single pediatric hospital. A standardized questionnaire was used to collect the patient information. RESULTS: All included children, ages 3 days to 18 months, received one injection of Gd-DOTA (volume 0.6-4 ml). No immediate adverse event was reported. CONCLUSION: This postmarketing study involving neonates and infants suggests a favorable safety profile of Gd-DOTA in routine practice.

Familial interstitial disease with I73T mutation: A mid- and long-term study.

OBJECTIVES: To describe the long-term course and the management in children of chronic interstitial lung disease associated with I73T mutation. MATERIALS AND METHODS: Clinical, radiological, and histological data from one family including five children and two adults were analyzed retrospectively for three patients and prospectively for the others. RESULTS: Mean age of onset of respiratory symptoms for children was 6 months (2-15 months). The follow up was 14 months to 15 years (mean 55 months). The children were treated by intravenous high dose methylprednisolone pulses (6-15, mean 12). Four received oral prednisolone (mean 16 months) and hydroxychloroquine, one of these had additional mycophenolate mofetil. One adult with mild respiratory symptoms in infancy and another who was symptom free were also diagnosed. Both of them received no treatment. BAL fluids were obtained in all children: pro-SPC and SPB were positive in all. Lung biopsies were performed in two children respectively at 7 months, showing interstitial pneumonia features with endoluminal macrophage and type II alveolar cells hyperplasia, and at 33 months, showing subpleural microbullae, areas of interstitial pneumonia and type II alveolar cells hyperplasia. Immunohistochemistry showed for both an increased SPB and TTF1 staining in type II cells nuclei and a faint staining for pro-SPC and for ABCA3. Genetic diagnosis obviated the need for biopsy in other cases. The clinical status progressively improved and oxygen supplementation could be stopped after 3-14 months (mean 9 months). The CT scans initially showed ground glass opacities, then reduction in the ground glass pattern associated with clinical improvement and development of cysts. CONCLUSION: This kindred illustrates the variability of respiratory involvement and prognosis. It confirms the value of genetic screening for surfactant protein genes mutations.

Neonatal bilateral ovarian sex cord stromal tumors.

A child was referred for evaluation after prenatal diagnosis with macrosomia, clitoromegaly, labial hypertrophy, and a left ovarian cyst. The karyotype was 46,XX. The postnatal pelvic ultrasound was normal. High levels of anti-mullerian hormone and testosterone led to a hCG stimulation test, which was followed by isosexual precocious puberty and the appearance of a bilateral ovarian enlargement with a left tumoral mass. A left ovarian tumorectomy revealed a fibrothecoma. Six weeks later, a tumoral relapse occurred and completion of oophorectomy revealed a juvenile granulosa cell tumor (JGCT). Whereas hormonal levels decreased after surgery, a new rise associated with an enlargement of the right ovary led to the diagnosis of right JGCT. A right oophorectomy was proposed to the parents, who declined further surgery. After 2 months, the hormonal levels normalized. This case illustrates the confusing overlap between developmental and neoplastic biology in neonates.

Diagnostic value of high-resolution CT in the evaluation of chronic infiltrative lung disease in children.

OBJECTIVE: The purpose of this study was to evaluate the accuracy of CT in the diagnosis of chronic infiltrative lung disease in children. MATERIALS AND METHODS: Fifty-nine patients selected over a 14-year period (29 girls, 30 boys; mean age, 6 +/- 4.9 years; range, 2 months-18 years) had nine disorders. CT scans were evaluated independently by two experienced chest radiologists, who were unaware of pathologic or clinical data. The radiologists recorded specific CT findings of infiltrative lung disease and were asked to give the most likely diagnosis and up to two differential diagnoses. Descriptive statistic analysis was followed by logistic regression analysis for each elementary lesion on the grid of abnormalities. RESULTS: A correct first-choice diagnosis was made in 38% of CT observations. The correct diagnosis was among the three main choices in 59% of CT observations. Pulmonary alveolar proteinosis (n = 18) was most frequently correctly diagnosed; it was the first-choice diagnosis 47% of the time and among the three main choices 72% of the time. The correct first-choice diagnosis of idiopathic pulmonary fibrosis (n = 16) was made 43% of the time; of hypersensitivity pneumonitis (n = 4), 37% of the time; of sarcoidosis (n = 7), 28% of the time; of idiopathic pulmonary hemosiderosis (n = 6), 16% of the time; and of connective tissue diseases (n = 5), 10% of the time. All single cases of pulmonary fibrosis with calcification, lymphangiectasia, and Langerhans' cell histiocytosis were correctly diagnosed. CONCLUSION: Our results showed there are limitations to diagnosing chronic infiltrative lung disease in children on the basis of CT data alone. We suppose that these differences are explained by the technical difficulties of high-resolution CT in children, the insufficient number of cases of and data on high-resolution CT of children, and the heterogeneity of lesions of a given cause.

Chest computed tomography findings in bronchopulmonary dysplasia and correlation with lung function.

OBJECTIVE: With changes in the predominant pathogenic factors in the new form of bronchopulmonary dysplasia (BPD), a different pattern of CT findings may be expected. This study aimed to (1) describe CT findings in infants with BPD and (2) correlate the CT findings with lung function abnormalities. STUDY DESIGN AND METHOD: Retrospective review of 41 very low birthweight infants with BPD, who were referred for pulmonary investigations at between 10 and 20 months after birth because of persistent respiratory symptoms, and underwent CT and lung function tests. RESULTS: None of the infants had normal CT findings. The most frequent abnormalities were hyperlucent areas (n = 36; 88%), linear opacities (n = 39; 95%), and triangular subpleural opacities (n = 26; 63%). Bronchiectasis was not seen. None of the CT abnormalities correlated with the maximum expiratory flow at functional residual capacity (VmaxFRC). In contrast, increased number of subpleural opacities and limited linear opacities were associated with low FRC and longer duration of neonatal oxygen exposure. The numbers of triangular subpleural opacities also correlated with duration of mechanical ventilation. CONCLUSIONS: Despite advances in neonatal care, many CT findings in infants with BPD are similar to those observed in the pre-surfactant era, and are still associated with duration of supplemental oxygen and mechanical ventilation. The absence of bronchial involvement in the present study was the most striking difference from previous studies.

Congenital disorders of glycosylation type I: a rare but new cause of hyperechoic kidneys in infants and children due to early microcystic changes.

BACKGROUND: There are numerous causes of bilateral hyperechoic kidneys. Congenital disorders of glycosylation (CDGs) are a rapidly growing family of inherited disorders due to defects in the synthesis of the glycans of glycoproteins or other glycoconjugates. OBJECTIVE: To describe renal sonographic abnormalities in CDG type I in infants and children. MATERIAL AND METHODS: A retrospective study of renal US in 12 infants and children: 8 CDG-Ia (6 multivisceral forms, 2 neurological forms), 2 CDG-Ib, and 2 CDG-Ix, with detailed functional renal tests in 6. Histology of the kidneys of one 35-week fetus with CDG-Ia was available. RESULTS: Renal US was normal in the two children with the neurological form of CDG-Ia. All patients with the multivisceral form of CDG-Ia or with CDG-Ib showed increased cortical echogenicity, and/or abnormal pyramids (small +/- hyperechoic). The two patients with CDG-Ix showed predominant involvement of the medulla, with inverted corticomedullary differentiation in one. Kidney size was normal in all but two patients. The fetal kidneys exhibited diffuse microcysts arising from the distal tubules. CONCLUSIONS: Hyperechoic kidneys are common in CDG-I patients, contrasting with grossly preserved renal function. The US pattern seems to differ slightly according to the type of CDG-I, and is consistent with microcystic changes of the renal parenchyma, which occur prenatally, and may be due to ciliary dysfunction secondary to altered glycosylation of tubular glycoproteins. CDG-I, which remains largely underdiagnosed at present, should be added to the causes of hyperechoic kidneys in children, especially in cases of multivisceral involvement, after ruling out other more frequent causes.

High-resolution computed tomography scan and airway remodeling in children with severe asthma.

BACKGROUND: Children with severe asthma have a significantly higher bronchial wall thickness (BWT) on high-resolution computed tomography scan than control children. OBJECTIVE: We sought to determine whether a BWT score correlates with markers of airway remodeling and inflammation. METHODS: In 37 children with severe asthma, we determined reticular basement membrane thickness; number of intraepithelial neutrophils and eosinophils on bronchial biopsy; IFN-gamma, IL-4, IL-5, and eosinophil cationic protein levels and IFN-gamma/IL-4 ratio on bronchoalveolar lavage specimen; and alveolar nitric oxide (NO) concentration and the maximum airway wall NO flux. RESULTS: The BWT score significantly correlated with reticular basement membrane thickening (r = 0.34; P = .04) and NO production by the airway wall (r = 0.45; P = .02). The correlation with the eosinophil cationic protein level was just significant (r = 0.40; P = .05), whereas there was no correlation with IFN-gamma/IL-4 ratio (r = -0.31; P = .08). The BWT score did not correlate with FEV(1) or forced expiratory flow at 25% to 75% of forced vital capacity. CONCLUSION: High-resolution computed tomography scan is a noninvasive technique that might be valuable for quantifying airway remodeling in children with severe asthma. The new generations of multislice computed tomography scanners will allow higher definition and lower radiation exposure and probably give a better assessment of airway remodeling and efficacy of treatment in children with asthma.

Unusual presentation of GM2 gangliosidosis mimicking a brain stem tumor in a 3-year-old girl.

We report a case of GM2 gangliosidosis revealed by MR imaging of an isolated brain stem abnormality in a 3-year-old girl referred for gait difficulties related to ataxia and pyramidal signs. Brain MR imaging displayed a brain stem lesion with high signal intensity on fluid-attenuated inversion recovery and T2-weighted images, suggesting either a tumor or an inflammatory process. Stereotactic biopsy findings showed the presence of swollen neurons with storage material in lysosomes. Enzyme study revealed deficiency of hexosaminidase A, variant B1. Gangliosidoses should be considered in the differential diagnosis of isolated infiltrating brain stem lesions in childhood.

Management of asymptomatic neonatal cystic adenomatoid malformations.

BACKGROUND/PURPOSE: Although much is known about the prenatal course of cystic adenomatoid malformations (CCAM), the postnatal course of asymptomatic lesion is less well documented. The authors studied the pre- and postnatal course and treatment of asymptomatic CCAM. METHODS: The authors reviewed the files of all patients referred to Necker-Enfants Malades hospital with an antenatal diagnosis of CCAM and asymptomatic at birth. RESULTS: Files of 29 patients were studied. The first x-ray film was considered normal in 12 cases (41.3%). Computed tomography was normal in 4 cases and showed cystic lung malformations in the other patients. Postnatally, clinical manifestations occurred in 3 patients (10.3%). CCAM vanished in 6 cases. Surgical resection of CCAM was performed in 17 cases (58.6%). All the patients currently are asymptomatic. CONCLUSIONS: CCAM can shrink or vanish during pregnancy and antenatal ultrasound findings are not predictive of the postnatal course. Thus, all infants with prenatal diagnosis of CCAM require postnatal evaluation. Normal radiographic findings at birth do not rule out CCAM persistence on CT. The treatment of asymptomatic CCAM is controversial. Surgery may be advocated because of the low morbidity and the prevention of late complications, above all, cancer. The surgical indications of small (<3 cm) and asymptomatic lesions should be discussed on a case-by-case basis with the parents.

Thoracic CT in pediatric patients with difficult-to-treat asthma.

OBJECTIVE: . The aim of this study was to establish objective, simple criteria for bronchial wall thickening in children with difficult-to-treat asthma. SUBJECTS AND METHODS: . Bronchial sections were counted at five levels in both lungs and at three levels in the right lung on high-resolution CT and plotted against lung function. Findings from 27 children with persistent symptoms of asthma (mean age, 11.4 years; SD, +/-3.1 years) that were severe (group A, n = 15) or moderate (group B, n = 12) were compared with findings from 21 control subjects (mean age, 10.8 years; SD, +/-3.0 years) using the Student's t test, analysis of variance, and Dunn-Bonferroni test. RESULTS: . A bronchial wall thickening score based on the number of visible bronchi at three levels (three-level score) proved to be as valuable as and simpler to obtain than a score based on the number of bronchi at five levels (five-level score). The three-level scores for groups A and B were similar (mean +/- SD, 16.8 +/- 4.2 vs 18.4 +/- 3.4, respectively; p = not significant), but these scores were significantly higher than those for the control subjects (mean +/- SD, 8.2 +/- 3.4, respectively; p < 0.001). There was no correlation between the three-level score and forced expiratory volume in 1 sec or forced expiratory flow between 25% and 75% of forced vital capacity. In contrast with adults with severe asthma, our pediatric patients with difficult-to-treat asthma did not have CT evidence of mucoid impaction, emphysema, areas of hyperlucency, bronchiectasis, or sequellar line shadows. CONCLUSION: . Bronchial wall thickening visible on high-resolution CT may constitute an additional criterion of asthma severity in children. CT evidence of bronchial wall thickening might help to identify patients with a higher risk of airway remodeling.