Biomedical subjects
Publications and source records attributed to R H Cleveland.
Gonadal dysgenesis and coarctation of the aorta were found in four patients to be associated with premature sternal fusion but possible etiologic relationship to shield chest deformity in suggested.
An abnormality which has received little attention but may be easily recognized on radiographs of the hand of patients with Turner's syndrome is described. Eleven of thirty-one patients (35.5%) with Turner's syndrome were shown on radiographs of the hand to have a visually detectable smallness on the bone surface area of the carpus when compared to the area of the second through fifth metacarpals. Values for the "C/M" ratio (the area of the carpals divided by the area of the second through fifth metacarpals) were calculated for films of 31 individuals with gonadal dysgenesis and compared with those from bone age-matched films of seventy-six individuals with normal development of the hand and wrist. A consistent difference with minimal overlap was documented. For all of the films of patients with Turner's syndrome the C/M ratio averaged 89% of the value in the films of the controls. In the 11 patients in whose films smallness was visually apparent, the C/M ratio averaged 82% of controls while in those where the diminution was detected only by planimetry the C/M ratio averaged 91% of controls. The incidence of a diminished C/M ratio was compared with the incidence of four other previously described signs of Turner's syndrome; although not seen as often as the coarse reticular pattern or delayed bone age, the C/M sign was recognizable without planimetry at least as commonly as the "metacarpal sign," and a decreased carpal angle and/or Madelung's deformity. Taken together, these several criteria suggest the radiographic diagnosis of Turner's syndrome in more than 93% of cases.
This multicenter study was designed to determine if CT can assess operability in stage III neuroblastoma. Nineteen children (11 boys, eight girls), aged 2-51 months, considered to have, by conventional clinical and radiographic examinations, localized neuroblastoma that crossed the midline were examined by CT. After intravenous and oral contrast media enhancements, CT was able to show the relation of the tumor to the adjacent vital vessels: aorta, celiac axis, and superior mesenteric artery. In all 15 patients, tumors that encased the aorta or its major branches were unresectable. However, three of four tumors crossing the midline but not coming into contact with the aorta or adherent only to one side of the vessel could be resected. Because surgery currently plays the major role in the treatment of neuroblastoma, the relation of the tumor to the aorta and great vessels is a more reliable and important factor in predicting the outcome of these children than the extension and location of the tumor with reference to the midline. CT after intravenous contrast enhancement can establish this relation and assess resectability.
Each of four obese adolescent females were thought to have pre-sternal anterior chest wall "masses" when evaluated by lateral chest radiograph. All had normal physical examinations. The apparent chest wall masses were found to be an artifact created by partial midline contact of both, large breasts, surrounded by a partial midline air space adding contrast to the adjacent soft tissue. Manual abduction of the breasts was followed by disappearance of the apparent radiographic abnormality.
Low-dose transaxial tomography is a technique that can produce cross-sectional images of the hips and femurs in children to permit calculation of the angle of femoral anteversion. Transaxial tomography was compared with computed tomography in terms of measured radiation dose and image quality. Transaxial tomography was found to require at least 90% less radiation dose, and the images were judged to be acceptable for the determination of anteversion.
Toxic epidermal necrolysis is a cutaneous disorder with high morbidity and mortality. Esophageal stricture has rarely been reported following recovery from this abnormality. A case is presented demonstrating the occurrence of an esophageal stricture following successful therapy. New methods of treatment are changing the mortality and morbidity such that esophageal stricture will be observed more frequently.
This retrospective study of 470 children undergoing barium upper gastrointestinal examinations was performed with three goals in mind: (1) to document the incidence of gastroesophageal reflux discovered during standardized upper gastrointestinal examination; (2) to compare the amount of gastroesophageal reflux detected in patients with symptoms suggestive of reflux as opposed to those who had no such symptoms; and (3) to ascertain the effect on reflux of the presence of a lower esophageal "beak." About 65% of the children studied had gastroesophageal reflux. Reflux was seen more commonly when symptoms of reflux were present (75.9%) than when not (36.8%). The amount of reflux seen over 5 min was greater if symptoms were present (mean incidence 2.72 bouts) than when not (mean incidence 0.76 bouts). Most importantly, there was a significant decrease in the amount of reflux seen with increase in patient age; age-related criteria for "acceptable" gastroesophageal reflux are presented. The presence of an esophageal "beak" is associated with an increased amount of reflux (94.4%) as opposed to no such "beak" (67.8%). The data suggest that gastroesophageal reflux is present in a large percentage of pediatric patients, whether there are symptoms to suggest reflux or not. Since reflux diminishes with increasing age, age-related criteria for an "acceptable" amount of reflux should be used rather than a universal judgment based on three episodes. Reflux to the cervical esophagus occurs frequently, both with and without symptoms of reflux, and may not be a reliable solitary indication for therapy. An esophageal "beak" is associated with an increase in reflux and may have important prognostic implications.
Forty-seven females with duplication of the uterus and cervix were retrospectively studied and their associated urinary tract malformations were analyzed. In 31 of the patients duplication of the müllerian ducts was diagnosed during the work-up for major genitourinary malformations that corresponded to three main complexes of anomalies: cloacal anomalies (16 patients), exstrophic deformities (4 patients), and renal malformation on the same side as an occluded müllerian duct (11 patients). Another five patients with normal kidneys had incomplete müllerian duct fusion discovered incidentally at autopsy, surgery, or during the work-up for urinary tract infections. None of the 11 women examined by excretory urography following the discovery of duplication of müllerian ducts by hysterosalpingography had urinary tract abnormalities. Therefore, although failure of fusion of the müllerian ducts frequently coexists with other genitourinary anomalies, the associated malformations manifest themselves clinically. Women who are found to have duplication of the reproductive tract during the work-up for infertility and who are otherwise asymptomatic and have a normal physical examination do not require urographic investigation.
Forty-seven females with duplication of the uterus and cervix were retrospectively studied to obtain a better understanding of the reasons for failure of fusion of the müllerian ducts by analyzing the associated genitourinary malformations. Thirty-one of the 47 patients had major genitourinary malformations that corresponded to three main complexes of anomalies. Cloacal anomalies were present in 16 patients, exstrophy in two, and combined cloacal and exstrophic deformities in another two. Renal malformations ipsilateral to a unilateral occluded müllerian duct were seen in 11 patients, and in four of these an ectopic ureter opened into the occluded duct. From the analysis of these anomalies it is proposed that duplication of the female genital system, when not an isolated event, may be secondary to interference of the normal process of müllerian fusion by a persistent cloacal duct, an abnormal pelvic girdle, or an ectopic ureter.
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Between 1969 and 1979 five children were found to have lumbar disc disease and were evaluated for clinically unsuspected thoracic spine abnormalities. Of these five children, two had Scheuermann's disease and one had disc space narrowing associated with Schmorl's nodes. One other had narrowed disc spaces without bony defects, and one had a normal thoracic spine. A unitary concept of childhood lumbar disc disease, Schmorl's nodes and Scheuermann's disease is suggested.
In a review of all infants admitted to our hospital with hyaline membrane disease during a recent period, we observed that two distinct groups developed complete pulmonary opacification during the course of their disease. One group (9% of the total) developed a classic "white-out" of stage II bronchopulmonary dysplasia early in the course of their disease while critically ill. The prognosis for this group was poor. The other group (10% of the total) developed an almost identical radiographic finding later in the course of their disease as they were clinically improving and being weaned from the respirator. In this group the "white-out" was transient and not related to clinical deterioration; rather, it was secondary to decreased ventilatory pressures. The prognosis for this group was quite good.
The bronchial angles in children from birth to age 18 years were measured. Symmetry of angles was found to age 15 years. After that age, an aortic indentation was generally noted on the trachea with an increased left bronchial angle. Hence, the relatively equal incidence of right- and left-sided aspiration of foreign bodies in smaller children as opposed to adults.
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Knowledge of the transitory pleural thickening seen around thoracotomy tubes can avoid confusion with more serious pleural and pulmonary conditions. In this study of 60 children treated with chest tubes, nine had a local pleural reaction severe enough to produce findings on x-ray films taken up to ten days after removal of the tube.
Five children with six congenital pseudarthroses of the long bones of the forearm are described. Four cases were the result of neurofibromatosis, one was related to fibrous dysplasia, and one appears to be idiopathic. Whereas congenital pseudarthrosis of the leg primarily involves the tibia, congenital pseudarthrosis of the forearm primarily involves the radius. Four of the children were treated with bone grafts of the radius, three requiring repeated grafting. The etiology, differential diagnosis, long term prognosis, and treatment correlate with the data of similar lesions in the tibia. Thus the reason for poor fusion is probably unrelated to weight bearing but is rather the result of underlying bone pathology.
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