Standard method of diagnosis versus use of a computer database in the evaluation of skeletal dysplasias.

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.

Fibromatoses of childhood: the spectrum of radiographic findings.

Fibrous tumor of childhood include several disorders with variable biologic behavior. In the review by Coffin and Dehner [1] of 190 soft-tissue neoplasms in 183 children, 27% were fibroblastic or myofibroblastic in origin. Although nearly all fibrous lesions are benign, they may be locally aggressive. The purpose of this essay is to describe clinical characteristics and to illustrate radiologic features of commonly encountered fibrous lesions of childhood. Familiarity with the presentation and variable appearance may aid the radiologist in suggesting the diagnosis of fibromatosis.

Scrotal swelling: unusual first presentation of Crohn's disease.

Crohn's disease often is detected in adolescents with nonspecific gastrointestinal complaints. Extraintestinal complications are common but usually follow the onset of the bowel complaints. We present an unusual case in which scrotal swelling was the first symptom in a patient discovered to have Crohn's disease.

Cystic dysplasia of the testis associated with multicystic dysplastic kidney.

Cystic dysplasia of the testis (CDT) is a rare congenital defect characterized by the formation of multiple irregular cystic spaces in the mediastinum testis. Co-existent genitourinary lesions have commonly been associated with this lesion and have included absence of the ipsilateral kidney, duplication anomalies, and cryptorchidism. We describe the first case in which multicystic dysplastic kidney (MCDK) is associated with CDT.

Pediatric blunt abdominal trauma: periportal tracking at CT.

Periportal zones of decreased attenuation at computed tomography (CT) have been described in a variety of disorders. In the setting of blunt abdominal trauma, the zones have been attributed to dissection of blood along the portal tracts. Because of the observation of isolated periportal tracking (PPT) in children after blunt trauma, the authors retrospectively reviewed CT scans of the abdomen obtained in 114 children to determine the frequency of PPT, liver injury, and peritoneal fluid. PPT was present in 22% of patients (25 of 114); it was associated with liver injury in 10 and was the only liver abnormality in 15. Pathologic correlation was available in two patients: In one it revealed marked periportal lymphedema and in the other, PPT of blood. This study indicates that both hemorrhage and lymphatic edema may be represented as PPT in children after blunt abdominal injury.

The normal and abnormal scrotum in children: evaluation with color Doppler sonography.

The use of color Doppler sonography to evaluate the symptomatic testes in children with scrotal pain or swelling was prospectively studied with a fourth-generation color sonographic unit with a 7-MHz linear transducer. The 32 patients were 1 day to 18 years old (mean age, 8.6 years). Results were correlated with scintigraphic findings in 23 patients, with the final diagnosis established by surgery in 12 patients, and with clinical follow-up in all patients. Eight cases of testicular torsion, including two of acute torsion and six of late torsion, were correctly detected by color Doppler sonography and confirmed surgically. In the remaining patients, perfusion of the testis was correctly detected by color Doppler examination. The final diagnoses in these patients included torsion of the appendix testis (15 patients), epididymitis (five patients), epididymo-orchitis (one patient), yolk sac tumor of the testis (one patient), hydrocele (one patient), and local reaction to an insect bite (one patient). The ability to detect blood flow in the normal contralateral testis was also evaluated in 28 patients. Blood flow was demonstrated in normal testes larger than 1 cm3. Detection of flow in the very small normal prepubertal testis was often difficult, and no flow was identified in one testis. Flow was identified in central arteries in only six of 13 testes smaller than 1 cm3. We conclude that color Doppler sonography is helpful in the initial evaluation of pediatric testes, providing accurate evaluation of the involved hemi-scrotum in our patients and also providing the benefit of both structural and flow information. Until our sensitivity to low-velocity flow improves, we would not suggest the exclusive use of color Doppler sonography in the evaluation of testicular perfusion in the prepubertal patient. We advocate the addition of testicular scintigraphy to corroborate the presence of testicular perfusion when flow in intratesticular arteries cannot be established with certainty by color Doppler sonography.

Melanotic neuroectodermal tumor of infancy. MR findings and a review of the literature.

Melanotic neuroectodermal tumor of infancy is an uncommon neoplasm occurring primarily in the child one year or less in age. Difficulty in deciding the cellular origin of this tumor has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumor, to list a few. Electron microscopy and histochemical studies, however, have now established the neural crest as the most likely origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. Within the skull, there is a predilection for the anterior fontanel. The following is a case report of a young child with melanotic neuroectodermal tumor of infancy arising at the anterior fontanel. Included is a discussion of magnetic resonance (MR) findings, which to our knowledge, have not been previously reported in this tumor.

Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice in childhood.

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.

Ewing's sarcoma of a lower extremity in an infant. A therapeutic dilemma.

A 5.5-month-old infant with Ewing's sarcoma of the left femur is described. The clinical and the pathologic features in this infant are presented in detail, and the dilemma faced in diagnosis and therapy of Ewing's sarcoma in infants is discussed. It is suggested that Ewing's sarcoma in an infant with a lower extremity lesion may be adequately managed without primary amputation.

Subglottic foreign bodies in pediatric patients.

Impacted subglottic foreign bodies may produce upper airway obstruction and clinical signs simulating croup or asthma. We identified the roentgenologic and clinical features in six patients. In four of these patients, the parent had not observed the aspiration episode, so that the diagnosis was delayed. Roentgenologic studies demonstrated subglottic narrowing of the upper airway with a homogeneous, poorly defined radiodensity within the narrowed segment. These roentgenologic studies are usually diagnostic; therefore, if infants or young children present with stridor of undetermined cause, soft-tissue upper airway roentgenography is indicated.

Cystic neuroblastoma in infants: radiographic and pathologic features.

Cystic neuroblastoma is a rare form of neuroblastoma. Three cases of cystic neuroblastoma in the infant are reported with emphasis on the sonographic findings. In two cases, the tumor was demonstrated in the fetus. The pathologic features of the tumors are described. Development of cysts may be related to a prominent microcystic arrangement of tumor nests.

Adrenal abscess in the neonate.

Adrenal abscess in the neonate is a rare complication of adrenal hemorrhage. The radiographic and clinical findings of 12 previously published cases and two new cases of adrenal abscess in the newborn are presented. Sonography was the most helpful examination in distinguishing a suprarenal lesion from an intrarenal lesion and in demonstrating the morphology of the abscess.

Yersinia enterocolitica colitis in infants: radiographic changes.

Radiographic changes of Yersinia enterocolitica colitis in 3 infants are presented. Findings included superficial aphthoid ulcers, submucosal ulcers, pancolitis (manifested by toxic megacolon), and involvement of the terminal ileum.

Ultrasound diagnosis of hypertrophic pyloric stenosis: real-time application and the demonstration of a new sonographic sign.

The diagnosis of hypertrophic pyloric stenosis (HPS), a common problem of infancy, is usually based on medical history. When the diagnosis is in question a barium upper gastrointestinal series has been the diagnostic imaging procedure of choice. In this study real-time ultrasound was used in 27 infants in whom pyloric stenosis was suspected. The published criteria for the ultrasound diagnosis of pyloric stenosis were used to evaluate the results. Fourteen examinations were true-positive, 12 were true-negative, and one was false-negative. The authors termed the hypertrophied muscle, which was demonstrated in longitudinal section, the "ultrasonic cervix sign" of hypertrophic pyloric stenosis. It was concluded that real-time ultrasound is a simple and accurate method for the diagnosis of HPS and should be the initial imaging procedure.

Wilms tumor in horseshoe kidneys: radiologic diagnosis.

Two cases of nephroblastoma occurring in a horseshoe kidney are reported, and 32 cases from the literature are reviewed. The radiologic signs of horseshoe kidney may be difficult to evaluate with excretory urography when the mass is large. Rotational abnormalities of the opposite kidney that is not involved by tumor should suggest the possibility of an associated horseshoe kidney. Real-time ultrasonography and computed tomography are helpful in identifying the isthmus of the horseshoe kidney. Aortography confirms the presence of the horseshoe kidney and demonstrates the arterial supply to the isthmus and the tumor. Radionuclide scans demonstrate the isthmus when the tumor arises from an upper pole, but may not be diagnostic if the tumor arises from the isthmus.

Choledochal cysts in children: radiologic features.

Choledochal cysts, although uncommon, are found most frequently in children. Radiologic studies have always played an important role in diagnosis because of the nonspecific clinical features. The abdominal roentgenogram may show in the right upper abdomen a mass, the cystic nature of which can be demonstrated by intravenous urography, hepatic angiography, sonography, and computerized tomography. Large choledochal cysts will displace the duodenal loop inferiorly and to the left, as seen on upper gastrointestinal contrast studies. Oral cholecystography and intravenous cholangiography are currently not indicated because of the poor diagnostic yield and the significant toxicity of intravenous media in children. Operative cholangiography is desired by the surgeon for demonstration of the pathologic anatomy. Real-time sonography and cholescintigraphy with technetium Tc 99m iminodiacetic acid derivative allow specific preoperative diagnosis and will eliminate the need for more invasive studies, such as arteriography, endoscopic cholangiopancreatography, and percutaneous transhepatic cholangiography. The radiologic findings in six patients are summarized.

Focal nodular hyperplasia of the liver in children: a report of three new cases.

Three cases of focal nodular hyperplasia of the liver in children are presented. The angiographic findings of a hypervascular mass with dense, delayed capillary stain in combination with increased uptake of 99mTc sulfur colloid on radionuclide scans were present in two cases. Computed tomography, utilized in one case, demonstrated a well-circumscribed mass with an irregular stellate area of low density which corresponded to the central collagenous scar described pathologically. The ultrasonographic finding was that of a mass slightly less echogenic than surrounding normal liver tissue.