MR appearance of osteogenic sarcoma of the calvaria.
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Biomedical subjects
Publications and source records attributed to E G Kassner.
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Iatrogenic disorders continue to be an important cause of morbidity and mortality in infants and children. Most adverse drug reactions have no distinctive radiologic features. Of those with radiologic manifestations, it is usually impossible to differentiate iatrogenic disorders from their spontaneously occurring counterparts. However, certain iatrogenic disorders have distinctive imaging characteristics that allow their recognition in specific patient populations. This review considers a variety of drug-related disorders and phenomena that have been the subject of original articles in the English language literature since 1984.
Triphalangeal thumb (TPT), a rare malformation of uncertain pathogenesis, may occur as an isolated defect, in association with other malformations of the hands, or as a feature of a syndrome or sequence. Isolated TPT occurs in two functional types: opposable and non-opposable. The latter appears to be inherited as a simple autosomal dominant trait, while the former is generally sporadic. TPT is associated with a number of specific malformations of the hand or foot, several of which have a well documented autosomal dominant pattern of inheritance. TPT is a feature of a number of specific syndromes. In this setting it may be associated with radial hypoplasia, bone marrow dysfunction, congenital heart disease, lung hypoplasia or agenesis, anorectal malformations, sensorineural hearing loss, onychodystrophy, mental retardation, and other disorders. TPT serves as a useful marker in such patients; in conjunction with the clinical and radiological findings, it can help to establish the correct diagnosis, leading to appropriate management and genetic counselling.
The sonographic records of 27 children who underwent a Cohen ureteroneocystostomy were reviewed. In 13 children (48%) sonograms demonstrated an echogenic, nonacoustically shadowing structure at or just above the trigone that was fixed in position within the bladder wall and covered by intact mucosa. This constellation of sonographic findings, which represent the submucosal segment of the reimplanted ureter, produces a "tunnel sign." Awareness of this appearance will enable the sonographer to better evaluate children who have undergone surgical procedures on the bladder.
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Although systemic candidiasis is common in hospitalized patients, invasive pulmonary candidiasis is rare and generally considered of secondary importance when found at autopsy. Autopsy records for a 12 year period were reviewed and 15 infants were found in whom systemic candidiasis was considered the primary or a major contributory cause of death. Significant pulmonary involvement was found in 14. There were three characteristic histologic patterns of pulmonary candidiasis: (1) embolic (arterial-invasive) (seven cases); (2) disseminated (capillary-invasive) (four cases); and (3) bronchopulmonary (air space-invasive) (three cases, including one congenital infection). An indwelling vascular catheter or infected cutdown wound was the portal of entry in every case of the embolic form of pulmonary candidiasis. Systemic and pulmonary Candida infections were rarely diagnosed during life. The typical radiographic appearance was progressive air space consolidation, although two infants with the embolic form of pulmonary candidiasis had focal cavitation. In general, there was poor correlation between the radiologic and pathologic findings, and pathologic findings other than pulmonary candidiasis undoubtedly accounted for many of the radiologic abnormalities observed. There were no radiologic findings that could be used to differentiate the three histologic forms of lung involvement in these patients. Small lung nodules, the earliest histologic lesion of pulmonary candidiasis, were not seen in any patient owing to the presence of other lung disease and suboptimal radiographic technique.
Diffuse cortical calcification is an unusual form of nephrocalcinosis most often seen in chronic glomerulonephritis (CGN). The authors report the youngest patient with nephrocalcinosis of CGN, a 13-year-old boy, and briefly review the radiologic and pathologic features of this entity.
A case of bilateral kidney herniation through cutaneous pyelostomy stomas is reported. The child is doing well after successful closure of the pyelostomies and repair of both ureteropelvic junctions obstructions.
Sonography was used to evaluate 28 patients (age range, newborn to 16 years) with mediastinal and juxtacardiac masses and disease processes, partial or complete opacification of a hemithorax, and various juxtadiaphragmatic abnormalities. Technically adequate studies were obtained in 93% of the cases. Sonography was of greatest value in determining the cause of an opaque hemithorax, characterizing fluid collections, localizing fluid collections prior to percutaneous aspiration or drainage, and demonstrating the position and integrity of the diaphragm. Sonography was least successful in depicting and characterizing mediastinal disorders. The authors' method is described and the technical limitations and pitfalls of pediatric chest sonography are discussed.
We report two sibs with the SC phocomelia syndrome with typical facial appearance and bilateral absence or extreme hypoplasia of the fibula, radius, and thumb. One sib had bilateral humero-ulnar and femoro-tibial synostosis (absence of the elbow and knee joints). Application of the nosologic criteria of Herrmann and Opitz showed that there was no significant intrafamilial variation in phenotype. Chromosome analyses in both patients showed heterochromatic puffing and centromere separation involving many chromosomes, an observation that has previously been reported in patients with SC phocomelia and Roberts syndromes. More important, this finding will have significance in prenatal detection of a certain proportion of cases with these syndromes without resorting to the use of radiographic examinations.
Impassible strictures of the posterior urethra are frequent sequelae of injuries of the prostatomembranous urethra. To select the appropriate type of urethral reconstructive procedure, the surgeon must know the length of the stricture, whether local complications are present, and whether the anterior urethra is strictured or bound down by fibrosis. The authors describe the radiographic techniques they use to plan urethral reconstruction and evaluate the operative result. The principles of the one-stage transperineal and combined transpubic-transperineal repairs and the two-stage Turner-Warwick scrotal skin inlay urethroplasty are presented to enable radiologists and urologists to interpret contrast studies of the lower urinary tract in patients who have undergone these operation.
We evaluated the long-term use of synthetic salmon calcitonin in the management of osteogenesis imperfecta tarda and congenita. Forty-eight children, ranging in age from 6 months to 15 years, and two young adults, received synthetic salmon calcitonin 2 MRC units/kg three days a week and a daily oral calcium supplement of 230 to 345 mg. The annual fracture rate was decreased during calcitonin therapy as compared to the period preceding therapy. There was an increase in the ability of the patient to stand and move and in the subjective feeling of strength in the lower extremities during calcitonin therapy. There was also a significant improvement in radiographic bone density, as determined by the method of photodensitometry, in patients under 5 years of age. Long-term administration of synthetic salmon calcitonin may be beneficial to young children with osteogenesis imperfecta.
We favor initial non-operative treatment (suprapubic cystostomy drainage only) for prostatomenbranous urethral injuries in children and adolescents. Non-operative treatment usually results in uncomplicated strictures that can be corrected by a 1-stage transperineal or transpublic operation 4 to 6 months later. A hands-off diagnostic approach, which relies on excretory urography and retrograde injection urethrography to demonstrate partial and complete tears, eliminates the need for blind passage of catheters, an invasive procedure that may lead to complicated strictures unsuitable for a 1-stage repair. If a 1-stage repair is planned it is necessary to determine the length of the stricture, whether there are local complications and whether the anterior urethra can be widely mobilized. The radiographic techniques used to plan a corrective operation and to evaluate the results are described.
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Masses in the mesenteries and omentum are often difficult to diagnose by conventional radiographic techniques. Gray scale sonography was a valuable adjunct to radiographic vector analysis in four children with such masses. Masses that are clearly separable from the liver and spleen and do not distort identifiable extraperitoneal structures are probably intraperitoneal. In children most cystic intraperitoneal masses are related to the mesenteries, omentum, ovary, or bile ducts. An anterior fluid collection with internal septa (which might be mistaken for loculated ascites) is the typical sonographic appearance of an omental cyst. Echogenic masses are more difficult to evaluate: careful study of the acoustical features yielded important information in cases of omental lipoma and rhabdomyosarcoma metastatic to the mesenteries and omentum.
During the last five years we have applied diagnostic ultrasound to more than 350 girls under the age of 15 for suspected obstetric and gynecologic disorders. More than 60% of these patients were pregnant and were studied before elective abortion. Many other children were studied for amenorrhea or for the evaluation of a pelvic mass. Sonography has allowed us to successfully distinguish pelvic masses of ovarian origin from those of uterine origin. We have also been able to rule out functioning endocrine tumors in children with precocious puberty. In cases of intersex, sonography has been useful in the older child prior to reconstructive surgery but has played an ancillary role to more invasive procedures in the infant.
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