Inequalities in diagnosis and registration of pediatric very rare tumors: a European study on pleuropulmonary blastoma.

Very rare tumors (VRTs) account for up to 11% of childhood cancers. Dedicated national groups and registries only exist in some European countries. Pleuropulmonary blastoma (PPB) is a very rare intrathoracic pediatric tumor with a potentially severe prognosis. Due to its rarity, it sometimes goes unrecognized. We investigated PPB diagnostic capability and possible correlations between diagnostic performance and VRT-dedicated activities. The number of cases of PPB registered between 2000 and 2014 at pediatric oncology centers in Europe was compared with the number of expected cases. Data sources included VRT registries, population-based cancer registries, and hospital registries. Data were obtained for 25 countries, grouped into 4 geographical regions. The expected cases were 111, and the observed cases were 129. The observed-to-expected ratio was 1.86 for Northern Europe, 1.33 for Southern Europe, 1.22 for Central Europe, and 0.65 for Eastern Europe. More cases than expected were registered in all countries with an official VRT registry.Conclusion: The number of cases observed is consistent with expectations, but disparities exist across Europe. Difficulties in diagnosing PPB emerged in most Eastern countries. The incidence rate of PPB may be underestimated. The creation of VRT-dedicated groups and a European Registry for VRTs could help to reduce inequalities.What is Known:• Very rare pediatric tumors are often not recognized, despite representing almost 11% of childhood cancers .• Pleuropulmonary blastoma is a rare pediatric tumor with a poor prognosis.What is New:• The ability to diagnose and register pleuropulmonary blastoma varies in Europe.Registries dedicated to very rare pediatric tumors improve the diagnostic rates.• The incidence rate of pleuropulmonary blastoma may currently be underestimated.

Non-fermentative Gram-negative rods bacteremia in children with cancer: a 14-year single-center experience.

PURPOSE: Data on non-fermentative Gram-negative rods (NFGNR) bacteremia in children with malignancies are limited. The aim of this study was to present clinical picture, antimicrobial susceptibility pattern, risk factors for resistance and outcome in NFGNR bacteremia in children with cancer. METHODS: All episodes of NFGNR bacteremia occurring during 2001-2014 in children with cancer in a tertiary-care hospital were retrospectively analyzed. Pseudomonas and Acinetobacter spp. resistant to three or more antibiotic classes and all Stenotrophomonas maltophilia (SM) were defined as multidrug-resistant bacteria (MDR). RESULTS: A total of 80 children (44 males, 0.8-18 years, median 5 years) developed 107 episodes (116 pathogens) of NFGNR bacteremia; Pseudomonas aeruginosa (PA) (51; 43.9%), Acinetobacter baumannii (AB) (21, 18.1%), SM (18, 15.5%); and others (27, 25.2%). The rate of NFGNR bacteremia in children with certain solid tumors (e.g. sarcoma, 12/134 (9.0%)) was comparable to that of hematological malignancies (52/429 (12.2%). Focal infection and septic shock occurred in 16 (14.9%) and four (3.7%) episodes, respectively. Thirty (25.8%) of 116 NFGNR were MDR. The most significant predictors of bacteremia with MDR PA or AB were severe neutropenia (<100 cells/mm3; OR 7.8, p = 0.002), hospital-acquired (OR 16.9, p < 0.0001) and breakthrough (OR 11.2, p < 0.0001) infection. Infection with MDR bacteria was associated with inappropriate empirical therapy. The 30-day mortality was 3/107 (2.8%), all in neutropenic patients with hematological malignancies. CONCLUSIONS: NFGNR bacteremia can present with nonspecific signs or symptoms. MDR NFGNRs are common and compromise treatment options, but mortality is relatively low. Knowledge of local epidemiology, pattern and risk factors for resistance is important to guide empirical therapy.

Implications of early sonographic evaluation of parapneumonic effusions in children with pneumonia.

OBJECTIVE: To devise a clinically relevant grading system for the sonographic evaluation of parapneumonic effusions, and to evaluate length of hospital stay as a function of treatment approach and sonographic grades. METHODS: Chest sonograms of 46 pediatric patients diagnosed with empyemas and admitted to two medical centers in the last 8 years were retrospectively evaluated using a grading system based on the degree of fibrinous organization within the parapneumonic effusions. Hospital charts were reviewed to determine the method of treatment and length of hospital stay. Patients were divided into two treatment groups: nonoperative (n = 26) (antibiotics alone, or combined with thoracentesis, or tube thoracostomy) and operative (n = 20) (open decortication, or video thoracoscopy and pleural debridement). Patients in the nonoperative group were further subdivided into two groups: those who received antibiotics alone (n = 11) and those who received antibiotics plus nonoperative drainage thoracentesis and/or tube thoracostomy (n = 15). Within each treatment group, patients were subdivided into two ultrasound grades: low (no evidence of organization) and high (evidence of organization such as fronds, septations, or loculations). Student's t test was performed to compare the lengths of hospital stay for each of the treatment groups and ultrasound grades. RESULTS: The length of hospitalization was no different for patients with low-grade ultrasounds in the nonoperative (9.8 days) and operative groups (8.0 days). In contrast, length of hospitalization was significantly shorter for patients with high-grade sonograms in the operative group (8.6 days), when compared with the nonoperative group (16.4 days). Length of hospitalization for patients in the nonoperative group with high-grade sonograms was significantly longer (16.4 days) than for those with low-grade ultrasounds (9.8 days). Furthermore, when the nonoperative patients were divided into an antibiotics alone group and a nonoperative drainage group, the patients with low-grade sonograms had no difference in the length of hospitalization (9.0 days vs. 10.4 days), whereas those patients with high-grade sonograms in the nonoperative drainage group had a significantly longer hospitalization (19.9 days) than the antibiotics alone (high-grade) group (11.4 days). CONCLUSIONS: Patients with a low-grade sonogram had similar length of hospitalization if treated with either nonoperative or operative measures. Patients with high-grade sonograms had significantly shorter length of hospitalization when treated with decortication. Our retrospective study suggests that patients with high-grade ultrasound studies treated nonoperatively do not benefit from pleural drainage procedures or chest tube placement. This study demonstrates the usefulness of early sonographic evaluation of parapneumonic effusions. A prospective study evaluating the usefulness of sonographic assessment of severity of disease in the treatment of children with parapneumonic effusions is warranted on the basis of our retrospective data.

Possible heterogeneity in spondyloenchondrodysplasia: quadriparesis, basal ganglia calcifications, and chondrocyte inclusions.

We report on 6 patients with short stature and progressive enchondromatous-like changes of the vertebral bodies and the metaphyses of the long bones. Parental consanguinity was observed in 5 of 6 cases, supporting autosomal recessive inheritance. In spite of the similarity in radiographic changes and body proportions, genetic heterogeneity is suggested by the presence of CNS calcifications in 3 patients. Two of the latter had progressive quadriparesis. We tentatively classified these patients into 2 provisional types. An iliac crest biopsy in one of the patients with "type I" disease did not demonstrate enchondromatosis. Light and transmission electron microscopic studies demonstrated large cisterns and small inclusion bodies containing a flocculent material within the rough endoplasmic reticulum of the chondrocytes. Based on the histological and radiographic findings, we propose to classify these conditions among the spondylometaphyseal skeletal dysplasias.

Sonography of the chest in children.

Sonography is an extremely important tool in the evaluation of the chest in children. Its easy availability, versatility, and portability make it an obvious choice as the next diagnostic examination after an abnormal chest radiograph. Patience and meticulous technique coupled with good anatomic knowledge will produce useful studies. Sonography is equivalent and sometimes superior to CT because of its Doppler ability and superior fluid and tissue characterization. It is most helpful in the assessment of anterior and middle mediastinal masses, opaque chest, and pleural and juxta-diaphragmatic abnormalities; in the classification of perplexing radiographs; and in the assessment of peripheral chest lesions. Sonography provides guidance for diagnostic and therapeutic aspiration, providing not only excellent anatomic demonstration, tissue characterization, and vascular information but also immediate access to bacteriologic and tissue diagnosis when required.

Computed tomography in children with esophageal and airway trauma.

Computed tomography (CT) was used in five children, four with esophageal and one with airway trauma. The examination contributed valuable information that aided in planning and evaluating therapy: (1) it assessed mediastinal and pleural cavity involvement prior to surgery or drainage; (2) it evaluated the efficacy of drainage; (3) it gave excellent information about the position of chest tubes; and (4) it demonstrated unsuspected pneumothoraces, pleural effusion, pulmonary infiltrates, and lung perforation by a chest tube. In the postoperative assessment of laryngotracheal fracture, neck radiographs were useless since the airways were obliterated by hematomas and edema. In this situation, CT showed the position and state of the laryngeal cartilages. However, CT findings were not pathognomonic for esophageal tears or airway fractures. The primary diagnosis was still made by conventional radiography. Esophageal tears were accurately demonstrated by gastrografin swallow and the tracheolaryngeal fracture was diagnosed by a lateral neck radiograph--the state of the child permitting no lengthy workup. CT and conventional radiography with contrast studies play a complementary role in esophageal and airway trauma in children.

Sclerosis of the mastoid air cells as an indicator of undiagnosed otitis media in children with Down's syndrome.

We hypothesized that many children with Down's syndrome have undiagnosed otitis media. In a 1989 study of lateral neck radiographs of 22 children with Down's syndrome, we found that 64% had sclerosis of the mastoid air cells, indicating untreated or inadequately treated otitis media. We reviewed the lateral neck radiographs of 53 children with Down's syndrome and interviewed their parents regarding the diagnosis of otitis media. Mastoid air cells were found to be sclerotic in 22 (42%) of study subjects; 32% of these had no known history of otitis media. For the 68% of children diagnosed as having had otitis media, the most common symptoms were fever (61%) and cough or coryza (58%). Sclerosis of the mastoid processes was not associated with a high frequency of otitis media (> 20 episodes) or a higher frequency of hearing loss, but was associated with subsequent myringotomy and insertion of a ventilatory tube (P = .038). Our finding of sclerotic mastoids in 42% of children with Down's syndrome raises the possibility that children with Down's syndrome have unidentified or inadequately treated episodes of otitis media.

Trends in pediatric uroradiology.

Excretory urography, conventional and radionuclide voiding cysto(urethro)graphy, ultrasonography, radionuclide renal imaging and computed tomography are all currently readily available in most locales for the evaluation of the child's urinary tract. It is the responsibility of the radiologist to determine which studies are best for which children and the order in which they should be performed to minimize "cost" while maximizing the information obtained. A description of such an approach and its consequences are presented.

Traumatic prenatal sigmoid perforation due to amniocentesis.

A variety of fetal injuries, including those inflicted to the gastrointestinal tract by amniocentesis, have been reported before. This brief report describes the first documented case of sigmoid perforation owing to the common procedure of amniocentesis that manifested as abdominal distention at birth. A potential link between this complication and a recent increased incidence of "intrauterine spontaneous perforation" of the gastrointestinal tract has been mentioned. Practicing radiologists are encouraged to inquire directly about the history of amniocentesis in unexplained cases of intrauterine intestinal perforation.

Fused labia. Clinical and radiological findings.

The clinical and radiological findings in fused labia are described. Some of the patients were asymptomatic. The others were referred because of a suspicion of urinary infection. Micturating cystourethrography showed collection of contrast material above the labia and marked reflux into the vagina in all patients examined. These findings were not seen after separation of the labia. Although the diagnosis is usually a clinical one, it is suggested that labial fusion may first be recognized by the radiologist through these cystourethrographic findings and while catheterizing the child.

Vesicoureteral reflux in boys: review of 196 cases.

To determine the frequency of vesicoureteral reflux (VUR) in boys, the authors retrospectively studied 724 boys who underwent voiding cystourethrography for the first time. VUR was identified in 196 of these patients (27.0%). Urinary tract infection was the indication for cystourethrography in 188 patients (25.9%), 80 of whom (42.5%) had VUR. Hypospadias was the second most frequent indication (179 patients [24.7%]), with VUR present in 32 (17.8%). A significant frequency of VUR was demonstrated in boys studied for various other conditions. Excretory urography in 588 boys revealed congenital anomalies of the upper urinary tract in 72, with VUR in 26 (36.1%), which was significantly higher than that in boys with hypospadias (P greater than .01). There was no significant difference between the frequency of VUR in boys with hypospadias with or without meatal stenosis (P greater than .9) and in boys with meatal stenosis with or without hypospadias (P greater than .9); thus, VUR seems to be independent of mild urethral obstruction and hypospadias. VUR was more frequent in boys with posterior urethral valves (62.5%, P less than .002). VUR in boys with urinary tract infection is as common as in girls. In most cases, its frequency in many apparently unrelated conditions is suggestive of its primary nature.

Pneumatic rupture of the esophagus caused by carbonated drinks.

Pneumatic rupture of the esophagus occurs when gas under pressure is accidentally delivered into the oral cavity. To the 4 cases previously described we add 2 pediatric patients and in both the source of the offending gas was a bottle of carbonated drink. The mild initial symptoms were followed in both by physical and radiographic findings suggesting pharyngoesophageal perforation. Early radiologic findings included free subcutaneous and mediastinal air, followed later by hydropneumothorax and mediastinal widening as well as leak of contrast material on gastrografin swallow. CT findings contributed to patient evaluation and management.

Pre-suppurative phase of retropharyngeal infection: contribution of ultrasonography in the diagnosis and treatment.

Pre-suppurative phase of retropharyngeal infection can't be differentiated from retropharyngeal abscess clinically or radiographically. Ultrasound, however, can diagnose this common infection in early phase. Early antibacterial treatment prevents the suppurative phase, obviates surgery and shortens the hospitalization phase.

Imaging of children with urinary tract infection: a tailored approach.

We reviewed the initial imaging studies of 283 children with urinary tract infection in order to decide on the most effective approach. A voiding cystogram was performed in all, complemented by either urography (147 patients), ultrasonography (65), or both (71 patients). In the latter group there was good correlation between sonographic and urographic findings, except for a more accurate delineation of renal scars and anomalies on urography, all in children with vesicoureteral reflux and/or abnormal sonograms. From these data we derived two imaging sequences tailored to the clinical circumstances: cystography as the initial study in ambulatory patients after infection has subsided, and sonography followed by cystography in children hospitalised with severe infection. The upper tracts are studied by ultrasonography and/or urography in patients with significant vesicoureteral reflex or an abnormal sonogram. Both these sequences reduce radiation by omitting the urogram in roughly half of the patients.

Partial duplication of the lower limb with agenesis of ipsilateral kidney--a new syndrome: report of a case and review of the literature.

A detailed account is given of a 19-month-old female infant with partial duplication of the left lower limb and aplasia of the ipsilateral kidney, plus other congenital malformations. Although the etiology is unknown, we believe this constellation of findings, which has been reported previously, represents a new congenital malformation syndrome.

Internal mammary compartment: window to the mediastinum.

PURPOSE: To determine the role of internal mammary ultrasonography (US) in assessment of the mediastinum. MATERIALS AND METHODS: In 66 patients (age range, 2 days to 18 years old; mean age 6.5 years) US imaging with spectral and color Doppler was used to examine the internal mammary region. Forty-four patients were suspected to have mediastinal masses; 22, vascular abnormalities. RESULTS: Five patients had abnormal internal mammary arterial flow (reversed in one). Internal mammary vein flow was reversed or absent in 10 patients with vein obstruction or Glenn shunts. Eight of 23 patients with lymphoma had internal mammary adenopathy. None of six patients with infectious mediastinal adenopathy and no patients with other benign mediastinal masses or vascular abnormalities had visualized internal mammary nodes. CONCLUSION: US and Doppler imaging of the internal mammary vessels and nodes provide valuable information about the nature of mediastinal masses and vascular disease. Flow pattern in the internal mammary vessels may elucidate abnormalities of central vessels.