Scapuloiliac dysostosis.
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Biomedical subjects
Publications and source records attributed to J F Holt.
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Infantile cortical hyperostosis (Caffey-Silverman syndrome) is an uncommon and not well-understood disorder. Most authors confine cortical hyperostosis strictly to infants under six months of age as originally described by Caffey. Others indicate they have seen the same clinical, radiographic, and pathologic problem in older children as initial presentations or as a recurrence where the infantile phase had possibly been unrecognized. This is a report of idiopathic cortical hyperostosis (ICH) in an 11-year-old girl who had no history of the disorder as an infant. The report confirms others who have documented cortical hyperostosis in older children and postulates that it is the same entity as infantile cortical hyperostosis.
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Visualization of the suprasternal fossa in the newborn is due to suprasternal retraction. It is seen in 59% of patients with respiratory distress syndrome of the premature prior to intubation, and in 5% of patients with conditions such as pneumonia, meconium aspiration, and transient tachypnea of the newborn. The lower compliance of the lungs in patients with respiratory distress syndrome and increased compliance of the chest wall in premature infants accounts for the higher incidence of accentuation of the fossa. Visualization of the suprasternal fossa can simulate the distended proximal pouch of esophageal atresia. The two can be differentiated in the lateral view where the pretracheal location of the fossa can be appreciated.
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Two cases of abnormal collection of fat in the abdomen are reported. One involved the omentum, the other involved the mesocolon. Both patients had few symptoms with strikingly similar radiological findings of increased radiolucency due to fat surrounding the small bowel.
The jet phenomenon is a normal finding in excretory urography. The presence of such a jet is a good sign that vesico-ureteral reflux is not present. Of 19 ureters with a jet sign, only one had minimal transient ureteral reflux, while in 39 ureters without a jet sign 15 had reflux.
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Twenty-one cases of neonatal pneumoperitoneum seen at the C.S. Mott Children's Hospital between 1970 and 1974 were reviewed. Of 14 cases with autopsy or surgically proven gastrointestinal perforation, six did not have an intraperitoneal air-fluid level on the initial cross-table lateral roentgenogram while eight did. Four cases of pneumoperitoneum secondary to mechanically assisted ventilation and air leak phenomenon had no air-fluid level. A postmortem study in one premature infant cadaver suggests that the air-fluid level may not be reliably demonstrated when the amount of intraperitoneal fluid is small. These data support the contention that an intraperitoneal air-fluid level does not uniformly occur in perforated viscus and that its absence, even in the presence of mechanically assisted ventilation, does not exclude the necessity for surgical intervention. In some cases, water-soluble contrast examination of the gastrointestinal tract appears to be a more reliable diagnostic adjunct to document a perforated viscus, when a definite intraperitoneal air-fluid level is not present.
Characteristic radiographic changes were seen in six of 14 children who received radiation therapy to the epiphyseal plate of a long bone. These changes, which include metaphyseal sclerosis, metaphyseal fraying, and epiphyseal plate widening, resemble rickets. In three patients, these changes were followed by development of a broad metaphyseal band of increased density. The absence of metaphyseal changes may indicate sterilization of cartilage cells and may be predictive of significant limb shortening.
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For some reasons skull findings in Jansen's Metaphyseal Dysplasia have been largely neglected. A survey of the seven known cases (three of them being primarily observed and described by two of the authors) disclose important and constant alterations, namely pronounced basilar thickening and sclerosis, prominent supraorbital and zygomatic arches, underdevelopment of the paranasal sinuses with sclerosis of the adjacent bone, and hypoplasia of the mandible. These alterations give rise to distinct and fairly specific features. The relationship to other craniotubular disorders, such as craniodiaphyseal dysplasia, craniometaphyseal dysplasia, and frontometaphyseal dysplasia is discussed.
The ratio of patellar length to width was analyzed in comparison with subjective examination using radiographs of the affected knees in patients with hemophilia, juvenile rheumatoid arthritis (JRA), and controls to determine the usefulness of this measurement in differentiating the two diseases. A statistically significant difference was found between the proportion of JRA patients, controls, and patients with hemophiliac arthritis involving the knee. It was difficult to distinguish hemophilia from JRA by subjective analysis of the appearance of the knee using standard criteria. The patellar ratio is a more objective criterion which should be helpful in the radiological differentiation of JRA from hemophilia affecting the knee.
The relationship of thoracic spine length and birth weight was evaluated in newborn infants of 26-41 weeks gestational age, and standards of thoracic spine length were established. Thoracic spine length is an accurate index of trunk length in the: if the gestational age is known, a markedly lengthened thoracic spine suggests that the mother is diabetic, while a markedly shortened thoracic spine suggest retarded intrauterine growth.
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