Radiograph review revisited.
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Diagnosis, Differential↗
Biomedical subjects
J B Wyly
Publications and source records attributed to J B Wyly.
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.
Bone Diseases, Developmental↗
Imaging manifestations of cat-scratch disease.
Cat-scratch disease affects an estimated 22,000 people in the United States each year, more than half of whom are children or adolescents [1]. It is caused by Bartonella henselae, a gram-negative bacillus usually introduced by the scratch of a cat [2]. In the past, diagnosis was made if three of the following four criteria were met: (1) history of cat exposure with inoculation, (2) positive skin test: (3) absent laboratory and histopathologic evidence of other diseases, and (4) biopsy findings of granulomatous inflammation [3]. Recent identification of the causative organism has led to new diagnostic tests, including serum assays for B. henselae antibodies [4]. Although response of the disease to antibiotics is poor, spontaneous recovery generally occurs within months to years [3].
Adolescent↗
Osteopathia striata with cranial sclerosis.
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.
Adult↗
Adjuvant chemotherapy for advanced nasopharyngeal carcinoma in childhood.
Seven children with advanced nasopharyngeal carcinoma younger than 20 years of age diagnosed between 1975 and 1986 (inclusive) were treated with a uniform adjuvant chemotherapy regimen, which consisted of vincristine (1.5 mg/m2; day 1), doxorubicin (45 mg/m2; day 1), 5-fluorouracil (8 mg/kg; days 1 through 5), and cyclophosphamide (7 mg/kg; days 1 through 5). This combination chemotherapy was given for 12 to 24 months after completion of radiation therapy. The radiation doses to the primary sites ranged from 6000 cGy to a maximum of 6800 cGy. The radiation doses for neck prophylaxis ranged from 4500 cGy to a total of 5000 cGy. Involved sites were irradiated to at least an additional boost of 1000 cGy. One patient had an external dose 6000 cGy to the primary site boosted with brachytherapy of 3000 cGy at the surface of an ovoid. After chemotherapy myelosuppression occurred in all patients and was tolerable. All seven patients are surviving, six disease-free, for 22 months to 12 years (median, 4 years). This study suggests that the combination of radiation therapy and chemotherapy as used here has acceptable toxicity and is effective and further suggests that children with nasopharyngeal carcinoma, even in its advanced stage at diagnosis, may be curable.
Adolescent↗
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.
Female↗
Prostaglandin-induced hyperostosis. A case report.
The use of prostaglandin-E1 (PGE1) to maintain patency of the ductus arteriosus in infants with ductal-dependent congenital heart disease is now well established. A 2.5-month-old child with cyanotic heart disease who required long-term PGE1 infusions; developed widespread periosteal reactions during the course of therapy. Prostaglandin-induced subperiosteal hyperostosis should now be considered in the differential diagnosis of neonatal cortical proliferation.
Alprostadil↗
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.
Biopsy↗
Spinal abnormalities in pediatric patients: MR imaging findings compared with clinical, myelographic, and surgical findings.
Eighty-one pediatric patients with a variety of spinal disorders, including suspected dysrhaphism, scoliosis, neoplasia, and neurofibromatosis, underwent magnetic resonance (MR) imaging. The results were retrospectively compared with those of myelography followed by computed tomography (CT) and surgery. In patients with dysrhaphism, most abnormalities, including hydromyelia, inclusion tumors, and sites of cord tether, were demonstrated with MR imaging. Diastematomyelia and small hydromyelic cavities were indistinguishable on routine coronal and sagittal T1-weighted images; axial images with T2 weighting were optimal for this differentiation. MR imaging did not enable direct visualization of a thickened filum or evaluation of tethering with a thin, dorsally positioned neural placode. Congenital or severe scoliosis required lengthy studies with multiple planes of imaging or myelography and CT. Milder curvatures were readily evaluated with MR imaging, and neoplastic lesions, with the exception of intrathecal tumor seeding, were adequately defined.
Adolescent↗
Cholelithiasis after spinal fusion for scoliosis in children.
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Adolescent↗
Maintenance of vascular access patency in pediatrics.
The patency of vascular access shunts and fistulae has been prolonged by a combined surgical and radiological approach that includes percutaneous transluminal angioplasty (PTA), surgical revision, thrombectomy, and thrombolysis. Over the last 3 years, 35 vascular accesses in 27 patients were found to have angiographic abnormality. PTA was performed 32 times on 19 accesses and 7 PTAs resulted in patent accesses by the end of the study. Surgical revision was performed 9 times on 8 accesses and 2 of the surgical revisions resulted in a patent access by the end of the study. Concerning Thomas femoral shunts, PTA prolonged the patency by 2.2 months and surgical revision by 3.8 months per procedure. Concerning arteriovenous (AV) fistulae, PTA prolonged the patency by 4.3 months and surgical revision by 3.5 months per procedure. A combination of procedures effectively doubles the duration of patency of Thomas femoral shunts and almost triples the duration of patency of AV fistulae in children. Forty-one percent of these accesses remain open 1 year following the initiation of these procedures.
Adolescent↗
Enterocyst formation: a late complication of neonatal necrotizing enterocolitis.
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Colonic Diseases↗
MR imaging of renal transplants.
Fifty-six MR studies were obtained in 32 renal transplant patients, by using T1-weighted, spin-echo, and inversion-recovery pulse sequences. The findings, particularly the loss of corticomedullary differentiation, and the extent of vascular penetration into the renal parenchyma, were compared with the clinical and histologic diagnosis of transplant rejection. Thirteen MR studies on 11 patients with clinically normal renal transplants demonstrated normal corticomedullary differentiation. Renal vessels extended into the parenchyma in all 11 patients and to the cortex in 38%. In 37 MR studies on 22 patients with a clinical or histologic diagnosis of acute and/or chronic transplant rejection, the corticomedullary differentiation was normal in 8%, faint in 24%, and absent in 68%. Renal parenchymal vessels were visualized in 32%, but extended to the level of the cortex in only 8%. In 68% of the studies with transplant rejection, no parenchymal vessels were seen. When the corticomedullary differentiation was either faint or absent, the vascular pattern was normal in 6%; in 68% of cases no parenchymal vessels could be identified. We conclude the corticomedullary differentiation and the renal vascular pattern are useful parameters in the evaluation of renal transplant rejection.
Female↗
Therapeutic catheter procedures in pediatrics.
There is a large spectrum of interventional catheter procedures being performed presently in children. The procedures that offer a greater advantage than surgical techniques and that will continue to be established procedures include percutaneous angioplasty of the pulmonary valve, peripheral pulmonary artery and caval stenosis, embolizations of pulmonary collaterals and pulmonary arteriovenous malformations, angioplasty of vascular access shunt, embolization of bronchial arteries for hemoptysis, and percutaneous nephrostomy and abscess drainage procedures. Others that have been successful but not yet routinely established in children include thrombolysis, renal artery angioplasty, embolization of peripheral arteriovenous malformations, dilatation of the urinary and gastrointestinal tract stenosis, and percutaneous biopsy.
Angioplasty, Balloon↗
Refluxing urethral ectopic ureters: recognition by the cyclic voiding cystourethrogram.
Reflux into an ectopic ureter that opens into a girl's urethra has been difficult to demonstrate and has been thought to occur in only about a third of the patients with this anomaly. In an attempt to more reliably demonstrate this reflux, 12 girls aged 1 day to 6 years, who were suspected of having an ectopic ureter, were studied using a method of cyclic filling and voiding during the cystourethrogram. Reflux into their urethral ectopic ureter was shown in 11 (92%). The technique of cyclic voiding cystourethrography should be used in all girls in whom an ectopic ureter is suspected, either because of signs or symptoms or because of a dilated upper pole of a duplex collecting system seen on an excretory urogram or a sonogram. It should also be used when a conventional voiding cystourethrogram has shown no or only minimal reflux.
Child↗