"New" ectodermal dysplasia syndrome with distinctive facial appearance and preaxial polydactyly of feet.

We describe a 2-year-old girl with virtual absence of body and scalp hair, rounded nails, thin dental enamel, preaxial polydactyly of feet, and unusual facial appearance. This combination of findings is not similar to that of any of the previously described ectodermal dysplasia syndromes and may represent a new disorder.

Holoprosencephaly and interstitial deletion of 2(p2101p2109).

We report on a girl with holoprosencephaly and a small, de novo interstitial deletion of most of band 2(p21). The similarity between the cytogenetic findings and CNS malformations in our patient and those recently reported by Münke et al. [Am J Med Genet 30:929-938, 1988] suggests a phenotypic relationship between deletion of this band and holoprosencephaly.

A comparative evaluation of rare earth screen-film systems. Free-response operating characteristic analysis and anatomic criteria analysis.

Diagnostic accuracy was evaluated in a signal detection experiment that used low-contrast acute lesions in a living dog model. At the high levels of certainty normally used by radiologists, rare earth screen-film systems provided accuracy comparable to that of a reference calcium tungstate screen-film system. Additionally subjective image quality evaluation for a given imaging task (clinical pediatric anterior-posterior chest films) based on visualization of anatomic landmarks and physical parameters has been conducted for several rare earth and one calcium tungstate screen-film system. The correspondence of subjective physical ranking with physical and psychophysical measurements was investigated. Evaluating the visualization of anatomic parameters can provide a clear and objective distinction among systems with comparable physical and psychophysical properties.

The child with acute abdominal pain and vomiting.

Evaluation of the child who presents with abdominal pain and vomiting is difficult. Close cooperation between pediatricians, surgeons, and radiologists is necessary to identify situations requiring urgent intervention. Close attention to history and physical findings will allow appropriate, judicious use of modern imaging techniques to arrive at an early diagnosis and initiate proper treatment when necessary.

An approach to the asymmetric neonatal chest radiograph.

Conditions causing respiratory distress in the neonate, which may require surgery or an interventional procedure, typically have asymmetric radiographic findings. The findings are conveniently categorized from the plain radiographs as those producing a large lucent-appearing hemithorax, those producing a large cystic-appearing hemithorax, and those producing a large opaque-appearing hemithorax. Additional imaging with ultrasound, CT, MR imaging, or fluoroscopy can be planned based on the initial radiographic appearance.

Current status of residency programs: survey of program directors.

RATIONALE AND OBJECTIVES: This survey was compiled to provide current data on the structure and content of radiology residency programs and the role of the residency program director. METHODS: A survey, created in electronic form and on paper, was distributed to all United States academic, private, and military radiology residency program directors. RESULTS: Of the 202 survey forms distributed, 168 (83%) were completed and returned. Eighty percent of respondents support national curriculum guidelines, but most do not favor national curriculum requirements. About half (53%) of programs spend 6 months in "view box" nuclear medicine; others provide some experience by lectures and on-call time. Most programs (60%) relieve residents of some call during the senior year. Some programs (28%) allow seniors time away from clinical duties to study for the oral board exam, and 17% permit time away to study for the written boards. Seventy-eight percent of programs have had a "problem resident," and 47% have asked a resident to leave the program. Directors' most frequently expressed concern was threat of diminished residency numbers due to decreased funding.

Hepatobiliary cystadenoma: a rare hepatic tumor in a child.

This report describes a 3-year-old child with a hepatobiliary cystadenoma. Cystadenomas are benign, multilocular, cystic neoplasms that usually occur in middle-aged women. The patients may be asymptomatic, but often there are vague abdominal complaints related to extrinsic compression of the stomach, duodenum, or biliary tree. Recurrence following incomplete excision and the presence of carcinoma within otherwise benign cysts has been documented. Complete resection is the therapy of choice, and thorough histopathologic evaluation is imperative.

Radiographic findings after pectus excavatum repair.

Postoperative radiographic changes after pectus repair have not previously been described. We reviewed the chest radiographs of 65 children who underwent surgical repair of pectus excavatum between early 1985 and mid-1992. After operation, 64 children showed atelectasis, 38 had pleural effusions, 30 showed a congestion or edema pattern, 12 had pneumothorax, 21 showed fixation strut tilt or rotation, and 4 had pneumomediastinum. Despite the sometimes worrying radiographic appearance, these children uniformly did well clinically.

Neonatal craniopharyngioma: CT and ultrasonographic features.

A case of craniopharyngioma in a neonate is reported and the literature on neonatal craniopharyngioma reviewed. First reported in 1952 and previously considered rare, the diagnosis of these neonatal tumors has increased with the use of noninvasive imaging including CT and ultrasound. The CT and ultrasound features of this uncommon but potentially treatable entity are described.

Arachnoid cysts in a brother and sister.

We describe a brother and sister with microcephaly and mental retardation who were shown by cranial CT scan to have almost identical unilateral arachnoid cysts. The occurrence in sibs suggests the possibility of a genetic basis for at least some cases of arachnoid cysts.

The role of ultrasound in the management of vein of Galen aneurysms in infancy.

Ultrasonography can be the key imaging modality for neonatal patients with vein of Galen aneurysms. Ultrasound can be used to diagnose the condition, monitor transtorcular embolization procedures, and follow-up these patients after embolization to assess the effectiveness of embolization and to check for complications.

Neonatal dural venous sinus thrombosis associated with central venous catheterization: CT and MR studies.

We report a case of extensive thrombosis of dural venous sinuses associated with placement of a central venous catheter in the jugular vein. Cases such as this one, though uncommon, underscore a potentially devastating complication associated with central venous catheterization and emphasize caution in catheter placement particularly in neonates.

Idiopathic arterial calcification of infancy: sonographic and magnetic resonance findings with pathologic correlation.

Idiopathic arterial calcification of infancy is a rare condition characterized by extensive arterial calcification and stenoses of large and medium sized arteries. We report the sonographic and magnetic resonance angiographic findings of this entity and correlate them with the findings at autopsy.

Prevalence of deep venous thrombosis in the lower extremities of children in the intensive care unit.

PURPOSE: To determine the prevalence of lower extremity deep venous thrombosis (LE-DVT) in children who spent at least 72 h in the pediatric intensive care unit (ICU). MATERIALS AND METHODS: Children up to the age of 17 years who spent at least 72 h in the ICU underwent lower extremity venous ultrasound at the end of their stay. Prevalence range for the sample size was calculated with a confidence interval of 95%. RESULTS: Among 76 children who spent 3-141 days in the ICU, the prevalence of acute (and silent) DVT was 4% (confidence interval 0-9%). All three affected children had femoral venous catheters in that leg during their ICU stay (17 unaffected children also had catheters). CONCLUSION: Children in an ICU setting are at significantly lower risk for thrombosis than adults in the same setting.

Asymmetric radiographic findings in the pediatric chest: approach to early diagnosis.

Several conditions in children produce a mass effect within one hemithorax, which may be life threatening. The different radiographic appearances of these conditions provide a basis on which they can be classified. The three major categories include a large cystic hemithorax, a large lucent hemithorax, and a large opaque hemithorax. Some disorders may be seen within more than one major group. Within the large cystic hemithorax group, congenital cystic adenomatoid malformation and congenital diaphragmatic hernia may be life-threatening conditions that require emergency surgery. The large lucent hemithorax is usually caused by pneumothorax, partial bronchial occlusion, or compensatory hyperinflation of the unobstructed lung. The majority of cases within the large opaque hemithorax group involve pleural fluid collections. Because many conditions that cause a mass effect require intervention or surgery, accurate interpretation of the plain chest radiograph is essential for an early diagnosis. Classification of radiographic features into these three groups often allows accurate diagnosis without additional studies.