Unusual systemic, pseudomalignant manifestations of cat-scratch disease in siblings.
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Biomedical subjects
Publications and source records attributed to C A Galliani.
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The physical and mechanical effects of injecting crystalloid cardioplegic solution under various pressures and flows was studied (in canine hearts) to establish a safe method for administering it in the presence of normal coronary arteries. A constant pressure system (300 mm Hg = 15 psi) was maintained in the solution reservoir, and flows and pressures were varied with the use of cannulas of different inner diameters: 0.8, 1.35, 1.6, 2.3, 2.58, and 2.80 mm. Cardioplegia distribution was measured by 15 microns radioactive microspheres. Peak flow rate, total flow, and mean flow rate per infusion were measured by an inline electromagnetic flowmeter probe. Direct aortic root pressure, time to standstill, and myocardial temperatures were recorded by continuous monitoring. Cardiac isoenzymes were measured in the coronary sinus, peripheral blood, and directly in the myocardial tissue. Histologic changes in the left ventricle were examined by light microscopy. The results showed that the higher the flow and pressure, the shorter the prearrest period, the better the flow distribution, and the faster the myocardial temperature drop. Mean aortic root pressures higher than 110 mm Hg and peak flow rates greater than 1500 ml/min caused a higher incidence of mechanical-physical trauma to the vascular endothelium and the endocardium, but cellular protection was good. Low pressure (less than 30 mm Hg) and peak flows (less than 125 ml/min) showed a higher incidence of cellular (myocardial) ischemia, focal necrosis, and uneven flow distribution. An aortic root pressure of 61 +/- 5 mm Hg, a mean peak flow rate of 622 +/- 52 ml/min, and a total flow of 600 ml for the first injection seem to offer the best cellular protection with minimal physical injury to the endothelium and endocardium for a mean canine heart weight of 236 gm.
Ovarian cysts are common incidental findings in term infants and, if unusually large, may result in dystocia, torsion, or rupture. Torsion and infarction of a normal ovary tend to occur in older childhood. During a 4-month period, 3 cases of neonatal ovarian torsion were observed after antenatal ultrasonography had detected fetal pelvico-abdominal cystic lesions. The three infants were explored between 4 and 16 days of age. Ovarian torsion was right-sided in all 3, and 1 ovary had been autoamputated. The resected specimens were nontense, thin-walled cysts, filled with hemorrhagic fluid, that measured between 4.5 and 8 cm in diameter. Microscopically, focal calcification and widespread necrosis precluded recognition of underlying histologic landmarks. Neonatal ovarian cysts or cystic ovaries greater than 4 cm in diameter should be excised, even if asymptomatic, because they are prone to, or have undergone, torsion.
A novel surgical procedure was performed on a 2-day-old infant with hypoplastic left-heart syndrome. It consisted of partitioning the pulmonary trunk thereby creating two channels, one leading to the pulmonary arterial system and the other to the aorta, and atrial septectomy to relieve left atrial pressure and facilitate mixing of blood. The technique avoids the use of conduits.
The importance of histologic subclassification of tumors lies principally in the correlation between nosology and tumor biology, including response to therapy. Subclassifications that are practically achieved, reproducible and uniquely predictive are of great value. As new methods are engaged and new clinical data are reported, classifications may be changed. Subclassifications of solid tumors with proven therapeutic application are exemplified by renal tumors and lymphomas that are commonly referred to as "favorable" or "unfavorable histology." Subclassification of soft tissue sarcomas, neuroblastoma and tumors of the central nervous system are being investigated, but are presently of undetermined relevance. Classifications and subclassifications of solid tumors of children are presented in the context of prognostic relevance.
Malignant tumors of infants and children, unlike their adult counterparts, are commonly undifferentiated or show minimal evidence of differentiation. Because of this, many of these tumors pose a formidable dilemma to the general pathologist in distinguishing one from another. A broad sampling of the more common malignant neoplasms and of newly recognized histologic subtypes which are restricted more or less to the pediatric age group is presented. The minimal microscopic criteria required to establish a diagnosis are given using widely accepted pathologic classifications and seminal journal references as a guide. Ancillary microscopic features, helpful histochemical stains, immunohistochemistry, and ultrastructural morphology are described for most tumors. Histologic distinction from diagnostic mimickers is discussed. Where appropriate, histologic grading and tumor subclassification are presented and their prognostic relevance is reviewed.
Larynges from 17 patients with DiGeorge syndrome (DGS) and from 14 patients with tetralogy of Fallot (TOF) (11 non-DGS and 3 possible but unproven DGS) were dissected, measured, and compared to a control population of comparable body length. The patients with DGS and the 3 patients with TOF suspected of having DGS showed the following: small thyroid cartilages with increased anterior angle, abnormally short superior cornua, low ratio of mean superior cornual length to distance between superior cornual tips, and delayed time of maximal rate of increase in superior cornual length relative to increase in body length. The hypoplasia, delayed maximal growth rate and persistent fetal shape of the thyroid cartilage (predominantly a derivative of the fourth branchial arch) in DGS, indicates that the causative process in the syndrome affects not only the third and fourth branchial pouches, but also, by a contiguous field defect, other derivatives than great vessels of the fourth-sixth branchial arches.
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Thirty-six hearts of patients who died with indwelling Swan-Ganz catheters in place were prospectively examined to assess the incidence and extent of localized lesions in the right side of the heart. Bland mural thrombosis in the superior vena cava, the right atrium and the pulmonary artery was found in 22 of 36 cases (61%). Patients with catheter periods greater than 2 days had a greater incidence and extent of bland mural thrombosis (79%) than patients with short-term catheterization (41%, p less than 0.01). Anticoagulation had no influence on bland mural thrombosis. Valvular hemorrhage occurred in 31% and aseptic valvular vegetations in 8% of the hearts. No case of infective endocarditis was found. Four of 36 cases (11%) had evidence of pulmonary infarction that appeared to be unrelated to the lesions in the right side of the heart. Endocardial lesions were common complications of indwelling Swan-Ganz catheters but had no significant impact on the clinical courses of the patients.
The feasibility of endovascular diathermic vessel occlusion (EDVO) is demonstrated in canines. The technique was standardized by measuring current flow through a monopolar intravascular electrode. Between 150 and 350 mA were applied to a spider-shaped intravascular electrode producing venous occlusion without perivascular damage. With further refinements in instrumentation, EDVO holds promise as a quick, safe method of therapeutic vascular occlusion that may eventually be applied clinically for spermatic vein occlusion.
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A 10-week-old male was found to have a cavernous hemangioma in the right lower pulmonary lobe. Hemangiomas of the lung are infrequent and in pediatric patients exceptionally rare. This may explain why, among the catalogs of intrapulmonary cystic lesions in children, cavernous hemangioma is a conspicuously absent entity and is rarely imagined in the clinical differential diagnosis. We present one such case, establish its distinction from arteriovenous malformations, and briefly review the available literature.
Myositis (fasciitis) ossificans developed following minor trauma to the soft tissues of the posterior aspect of the right knee of a 5-month-old female infant. The clinical and pathological features of myositis ossificans are reviewed and the differential diagnosis of soft tissue ossification in early childhood is discussed.
A 6-year-old white female was found to have an adenoid cystic carcinoma originating in a lacrimal gland. Eighteen months following diagnosis, the tumor recurred. Conservative surgery has been the sole mode of therapy. To date, after four operations and quadrimenstral imaging surveillance, there is no sign of disease progression. Our purpose is to record the unusual occurrence of an adenoid cystic carcinoma of the lacrimal gland in a child. An interim report, 32 months after diagnosis, is presented.