Biomedical subjects
Publications and source records attributed to R Solenberger.
We have described two infants, aged 2 months and 5 months, who had malignant hyperthermia, an entity not generally believed to occur during infancy. Both children were under general anesthesia when MH occurred, and both responded well to administration of dantrolene and sodium bicarbonate.
We describe the clinical courses of four infants with infantile myofibromatosis (IM). This entity is a mesenchymal disorder of early infancy characterized by the formation of tumors in skin, muscle, viscera, bone, and subcutaneous tissues. Previously known as congenital generalized fibromatosis, IM was formerly thought to be a rare condition that was frequently fatal. The majority of the 170 affected patients we describe have been diagnosed since 1980. Furthermore, the mortality rate for these patients is less than 15%. Our review includes the clinical manifestations, as well as histopathologic features, and discusses the prognosis in affected infants. We found that infants with solitary lesions or multiple lesions without visceral involvement generally have a benign course. However, in patients with the multicentric form of the disorder and visceral involvement, 73% have died. Because the lesions may not be easily discernible and most spontaneously resolve, the condition is underdiagnosed and underreported. IM is the most common fibrous tumor of infancy and must be considered when evaluating children who present with either solitary or multiple tumors, particularly during the neonatal period.
A totally implantable venous access system consisting of a subcutaneously placed stainless steel portal with a silicone diaphragm attached to a Silastic elastomer central venous catheter was inserted in 39 children with poor venous access. The devices were utilized for blood drawing and the administration of antineoplastic drugs, parenteral nutritional fluids, antibiotics, and blood products. Cumulative duration of access was 6,724 days (range 5-491, median 172 days). Major complications necessitating removal of the device included catheter sepsis or pocket abscess (7%), catheter clot not responsive to enzyme declotting (5%), and catheter occlusion by too tight a ligature (2%). The system provided easy venous access and permitted full physical activity.