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.

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.

Retroperitoneal abscess and mycotic aortic aneurysm: unusual septic complications of central vascular line placement in premature infants.

OBJECTIVE: To describe the sonographic appearance of unusual septic complications after central vascular line placement in premature infants. METHODS: Two case reports are presented. RESULTS: The first patient had a retroperitoneal abscess after percutaneous central venous catheter placement. The second patient had a ruptured mycotic aneurysm of the abdominal aorta after umbilical arterial catheter placement. CONCLUSIONS: Retroperitoneal abscess and aortic aneurysm should be considered in patients with histories of long-standing catheters or line sepsis. Both of these complications are readily diagnosed on the basis of sonography.

Manual epigastric compression during upper gastrointestinal examination of neonates: value in diagnosis of intestinal malrotation and volvulus.

OBJECTIVE: This paper describes the value of manual epigastric compression during upper gastrointestinal examination in establishing the diagnosis of intestinal malrotation and volvulus in five neonates. CONCLUSION: In two patients, the location of the duodenojejunal junction was near normal. Manual compression revealed abnormal mobility of the duodenojejunal junction, indicating malrotation. In three other patients, the duodenum was completely obstructed. Manual compression unequivocally displayed the anatomy of midgut volvulus by allowing contrast material to pass beyond the point of obstruction.

Duodenum and duodenal-jejunal junction in children: position and appearance after liver transplantation.

PURPOSE: To correlate upper gastrointestinal study findings of the position of the duodenum and duodenal-jejunal junction in children after liver transplantation with transplant type, age at transplantation, indication for transplantation, and history of surgery or malrotation. MATERIALS AND METHODS: Upper gastrointestinal studies in 23 children with a liver transplant were reviewed by two pediatric radiologists, and appearance and position of the duodenum and duodenal-jejunal junction were recorded. Findings were correlated with transplant type, age at transplantation, indication for transplantation, and history of surgery or malrotation. RESULTS: The duodenum and duodenal-jejunal junction were visualized on anteroposterior spot radiographs in 18 children. In 10 children, the duodenum and the junction were elevated and to the right of the spine; in two, the first and second portions of the duodenum were elevated, but the junction was normally located. These 12 children had undergone segmental liver transplantation. In the remaining six children, the duodenum and junction were normally positioned; three of these children had a whole liver transplant, and three had a segmental transplant. CONCLUSION: The duodenum and duodenal-jejunal junction are often malpositioned in children with a left lobe or left lateral segmental liver transplant. Without documented bowel obstruction, however, these children should be observed and followed up clinically.

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.

Extrahepatic portal vein stenosis in recipients of living-donor allografts: Doppler sonography.

OBJECTIVE: The aim of this study was to describe the appearances obtained and the pitfalls involved with the use of Doppler sonography for detecting portal vein stenoses after surgery in 198 recipients of pediatric reduced-size transplants. SUBJECTS AND METHODS: We analyzed sonographic and Doppler studies after surgery for 167 children (average, 2.5 years old) who were recipients of 198 left lobe or left lateral segment liver segments (79 living-donor allografts and 119 cadaveric grafts). Sonographic and Doppler studies were performed either on the basis of clinical evidence of portal hypertension or as part of a screening protocol. Demographic and surgical data were compared with the incidence of portal vein structure. We calculated pressure gradients from Doppler jet velocities and compared them with gradients measured manometrically from direct portography in 12 patients. Imaging criteria that indicated portal vein stenoses were (1) a visualized portal vein diameter of 2.5 mm or less, (2) an acceleration of flow at the stricture or a poststenotic jet of portal vein flow revealed by Doppler imaging, or (3) both. Stenoses meeting these criteria were verified by surgical or angiographic identification. RESULTS: Seventeen (22%) portal vein stenoses were detected in recipients of the 79 living-donor liver transplants, whereas three (3%) were detected in recipients of the 119 cadaveric grafts (p < .005). The use of cryopreserved venous extension grafts was the most significant parameter of correlation (p < .025). Doppler sonography predicted the stenoses in all cases, although it overestimated the pressure gradients in all but one of the verified cases. Intrahepatic portal vein flow was frequently normal in the presence of significant extrahepatic portal vein stenosis. CONCLUSION: Diagnosis of portal vein stenosis in recipients of living-donor allografts requires real-time visualization of the entire length of the portal vein, combined with spectral and color Doppler investigations of the portal and splenic veins and a search for collateral vessels. Visualization of each component alone may be insufficient. In our study, when care was taken to follow this procedure, sonography accurately showed all angiographically verified portal vein stenoses, although pressure gradients frequently were inaccurate. A protocol for periodic follow-up with real-time and Doppler sonography is crucial for pediatric patients to permit early identification of portal vein stenoses.

Sonographic demonstration of a normal thyroid gland excludes ectopic thyroid in patients with thyroglossal duct cyst.

OBJECTIVE: Preoperative thyroid scintigraphy has been performed in patients with presumed thyroglossal duct cyst to document a normal thyroid and to exclude the possibility of an ectopic thyroid mimicking a thyroglossal duct cyst. Often, an ectopic thyroid is the patient's only functioning thyroid tissue, and its removal will result in hypothyroidism. The purpose of this study was to determine whether demonstration of a normal thyroid gland by sonography in children with thyroglossal duct cyst can exclude ectopic thyroid and thereby obviate routine preoperative thyroid scintigraphy. MATERIALS AND METHODS: We studied 30 patients with pathologically proved thyroglossal duct cysts who had neck sonograms. The sonograms were evaluated for the presence or absence of a normal thyroid gland. The medical records of these children were also reviewed. Three children had normal preoperative radionuclide thyroid scans. All the children were clinically euthyroid preoperatively. Follow-up was available in 15 of the 30 patients, and all of these patients were clinically euthyroid postoperatively. RESULTS: A sonographically normal thyroid gland was detected in all patients. CONCLUSION: Preoperative sonographic identification of a normal thyroid gland in patients with thyroglossal duct cyst confirms a source of thyroid hormone separate from the thyroglossal duct cyst and thus excludes ectopic thyroid. Routine thyroid scintigraphy is not necessary.

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.

Septic arthritis of the elbow in children: the role of sonography.

Twelve children with suspected septic arthritis of the elbow were prospectively studied with plain-film radiography and ultrasound. Imaging data were correlated with clinical history and final diagnosis. Joint effusion was seen by sonography in six patients, four of whom underwent ultrasound-guided joint aspiration, confirming the diagnosis of septic arthritis in two patients and excluding it in two. Of nine patients whose plain films showed only soft tissue swelling, seven had one or more significant findings with sonography: joint effusion (without fat pad elevation on lateral plain films) in three patients, periosteal reaction in two, and epitrochlear mass in three. Sonography confirmed soft tissue swelling alone in two patients, thus excluding the diagnosis of septic arthritis and obviating unnecessary attempts at joint aspiration. Sonography of the elbow is an informative, easily performed examination, which is capable of showing both intra- and extra-articular abnormalities not apparent by plain radiography. Both the demonstration of pathologic changes and the failure to show joint fluid may affect clinical management.

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.

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.

Anatomic features of reduced-size liver transplant: postsurgical imaging characteristics.

In this retrospective review, the authors evaluated the anatomic and imaging features of 140 reduced-size liver transplants in 120 boys and girls; 100 of these patients (83.3%) were 2 years old or younger. The authors reviewed the surgical reports, plain radiographs, contrast material-enhanced studies of the gastrointestinal tract, duplex Doppler and color Doppler ultrasound (US) scans, and computed tomographic (CT) scans. Understanding the anatomic features associated with transplantation was crucial to understanding the post-surgical imaging characteristics. Changes in visceral anatomic structure caused a variety of imaging appearances, some of them misleading: (a) Some patients had unusual bowel gas patterns on plain radiographs because of a shift in location by bowel loops; (b) gastrointestinal studies showed gastric compression by an oversized left lobe that mimicked mass effect; and (c) displacement or migration of the duodenum, jejunum, or cecum into the right hepatic fossa sometimes mimicked malrotation. Pitfalls were created by fluid and gas collections at the transected edge. US and CT were often used to further assess anatomic structures after surgery; color Doppler was especially helpful in vascular localization.

Menetrier's disease associated with formula protein allergy and small intestinal injury in an infant.

Menetrier's disease in infancy is extremely rare, and its natural course has not been studied in detail. The present case report describes an infant whose initial diagnosis was formula protein allergy and who developed gastric outlet obstruction by 3 months of age. The diagnosis of Menetrier's disease was suggested by characteristic radiological, pathological, and functional abnormalities of the stomach. Small intestinal partial villous atrophy, malabsorption, and protein loss from both the stomach and the intestine were documented. Cytomegalovirus infection was excluded. There was no evidence for an immune deficiency. The described features and an unrelenting course suggest that infantile Menetrier's disease may be an entity distinct from the childhood and adult forms.

Hypoplastic posterior arch of C-1 in children with Down syndrome: a double jeopardy.

Radiographs of 38 children with Down syndrome (aged 2-3 years) were retrospectively evaluated for hypoplasia of the posterior arch of the C-1 vertebra. The anteroposterior dimensions of the posterior arch of C-1 were measured and compared with those of an age- and sex-matched control group. The posterior arches of C-2 to C-5 were similarly evaluated. Radiographs demonstrated hypoplasia of the posterior arch of C-1 in 26%. This anomaly of C-1 was an isolated event; sagittal diameters of C-2 to C-5 were within normal limits compared with those of the control group and published standards. Because of the known, potentially devastating atlantoaxial dislocation in Down syndrome, the increased prevalence of a second C-1 anomaly causing preexistent narrowing of the vertebral canal is of clinical concern. A hypoplastic posterior arch of C-1 may amplify the risk of spinal cord damage following atlantoaxial subluxation, as the subsequently smaller vertebral canal has less room for dorsal movement of the odontoid process. This potential "double jeopardy" of C-1 vertebral abnormalities should be recognized and assessed on cervical spine screening radiographs in children with Down syndrome.