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Objective: The objective of this study was to compare reference textbooks and the computer database, OSSUM, for accuracy and ease of use in the diagnosis of skeletal dysplasias. Materials and methods. Twenty cases of clinically and and radiologically established skeletal dysplasias were evaluated as unknowns by four pediatric radiologists. Readers 1 and 2 evaluated group A (10 cases) using reference texts and group B (10 cases) using OSSUM. Readers 3 and 4 evaluated group B using reference texts. The radiologists independently listed their roentgenographic findings, the top three diagnoses, confidence level, difficulty level, and time spent on each case., Results: The correct diagnosis was made in 68% of both the reference text cases and the OSSUM cases. Difficulty level was significantly higher (3.5 vs 2.9, P = 0.0013) and confidence significantly lower (3.3 vs. 2.3, P = 0.0001) when using OSSUM. Average time spent on cases was 25 min with references and 30 min with OSSUM (P > 0.05). However, there was a decrease in both the time (38 min vs 23 min, P = 0.05) and the difficulty (3.9 vs 3.1, P = 0.001) between the first five and the last five cases. The composite of four readers correctly identified 90% of the skeletal dysplasias when the results of both methods were combined., Conclusions: In the ability to reach a correct diagnosis, no difference was detected between the OSSUM and reference texts methods. The increased time necessary, greater difficulty and decreased confidence levels with OSSUM are expected to improve with increasing program familiarity. Use of both textbooks and the database was complementary.

Osteopathia striata with cranial sclerosis (OS-CS) is a specific bone dysplasia manifested by hypertelorism, flat nasal bridge, frontal bossing, large head, hypoplastic maxilla, palate anomalies, chronic otitis media, hearing deficits, nasal obstruction, and neurological changes of deafness, facial palsy, ophthalmoplegia, and mental retardation. We will review the clinical and radiologic findings in a new patient from birth to 20 years; this is believed to be the thirty-fifth patient reported. OS-CS is 2.5 times more common in females and occurs as an autosomal dominant condition or a sporadic dominant mutation with patients presenting for evaluation from the newborn period to the fifth decade. Skeletal abnormalities are distinctive including sclerosis of the skull base and calvarium, linear striated densities in the long bones and pelvis, and poor development of the mastoid and sinus air cells. Radionuclide bone scans with SPECT indicated in our patient increased bone turnover which was supported by biochemical findings of increased pyridinoline excretion. The major complications are due to constriction of essential foramina at the skull base. The condition is not life-threatening but can produce disability.

Within this decade it has been determined that primary gastritis in both children and adults is frequently associated with infection of the gastric mucosa with Campylobacter pylori. It is characterized by a chronic inflammatory process in which the mucosa of the gastric antrum is typically most severely involved. Other regions of the stomach may be involved and associated peptic ulcers of the stomach and duodenal bulb are frequent. A case of C. pylori gastritis is reported in which involvement of the gastric fundus and body produced severe rugal hypertrophy that resembled Menetrier's disease.

Esophageal atresia with tracheosophageal fistula may be associated rarely with distal esophageal stenosis. Three patients are reported with this combination of esophageal anomalies. In addition the clinical and radiologic features of 24 patients previously reported in the literature are reviewed. Careful evaluation of the distal esophagus during postoperative contrast studies in patients with esophageal atresia should be obtained to exclude distal stenosis. The presence of unrecognized distal esophageal stenosis may lead to complications of postoperative anastomotic leaks, poor healing of the anastomosis, aspiration, and impaction of a solid food bolus proximal to the stenosis.

Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

The diagnosis of Wilms' tumor is usually made by excretory urography after the patient presents with an abdominal mass. A diagnosis prior to the appearance of an obvious mass might result in an improved cure rate and less morbid therapy. The three cases described illustrate the difficulty in making an earlier diagnosis since pyelograms were "normal" prior to detection of the abdominal mass.