Primary cardiac lipoblastoma.

Lipoblastoma is a benign adipose tumor in children that has been described in various anatomic locations, most commonly the extremities. We describe the case of a 17-month-old boy diagnosed with cardiac lipoblastoma, a previously unreported primary cardiac tumor in children. Our patient presented with symptoms of coughing, wheezing, and hoarseness and was found to have a large mediastinal mass, which narrowed the left mainstem bronchus and compressed the right atrium and superior vena cava, causing superior vena cava syndrome. Surgical exploration revealed an intrapericardial soft tissue mass arising from the area of the posterior interatrial septum. Grossly, the resected mass was lobulated, pale yellow, and fatty with focal areas of gray myxoid tissue. Microscopically, the tumor consisted of both immature and mature adipocytes, with focal vascular myxoid areas containing lipoblasts, diagnostic of lipoblastoma. Two months after surgery, the patient was in good health without evidence of recurrence.

Lobar emphysema due to anomalous aortic origin of the left pulmonary artery.

The unusual case of an infant with aortic origin of the left pulmonary artery is presented. The patient developed a rare complication of lobar emphysema due to bronchial compression from the enlarged right pulmonary artery. Operative anastomosis of the left pulmonary artery to the pulmonary trunk was successful, with subsequent resolution of the lobar emphysema.

Sternal defects associated with congenital pericardial and cardiac defects.

We describe three cases of sternal defects of varying severity associated with other congenital anomalies. In the most severe case, both anterior and posterior defects were seen, with near-absence of the sternum and pericardium continuous with a large omphalocele. This resulted in external location of organs usually confined within the thoracic and abdominal cavities. A ventricular septal defect was present, and the arterial duct was absent. The course of the ascending aorta was anomalous. The baby had anencephaly and rachischisis. In the intermediate case, a proximal sternal cleft was associated with shortening of the sternum, and absence of the manubrium. Anterior pericardial and diaphragmatic defects were seen, while a scalp defect and an encephalocele were present on the posterior aspect of the head. This baby had tricuspid atresia. The remaining case had only an anterior defect with a shortened sternum. A supra-umbilical omphalocele contained a left ventricular diverticulum without interposing pericardium or diaphragm. Ventricular and atrial septal defects were present. The first two cases can be considered as representing failure of development of both an anterior and a posterior midline field. The third case, much milder than the other two, represents failure of development of an anterior field.

Carbon dioxide (CO2) angiography in children.

BACKGROUND: When iodinated contrast material is contraindicated, carbon dioxide (CO2) gas can be injected intravascularly to produce high-quality digital subtraction angiograms. OBJECTIVE: CO2 angiography, although previously described in adults, has never before been reported in children. MATERIALS AND METHODS: We present three children with renal transplants who required renal angiography. Because of elevated creatinine levels, iodinated contrast was not used to search for possible renal artery stenosis. Instead, CO2 angiography was used to evaluate the renal artery anastomosis. RESULTS: In all three cases, the renal artery anastomosis was clearly visualized using CO2 angiography and showed no evidence of renal artery stenosis. CONCLUSION: Digital CO2 angiography is an effective method for pediatric renal angiography. The technique can easily be adapted for virtually any angiographic laboratory capable of digital subtraction imaging. Digital CO2 angiography also lacks the risks of a conventional iodinated contrast medium, namely nephrotoxicity, allergic reaction and volume overload.

Tetralogy of Fallot with absent pulmonary valve and bronchial compression: treatment with endobronchial stents.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac lesion. The lesion includes ventricular septal defect, overriding aorta, and absence of the pulmonary valve, with resultant pulmonary incompetence. It has been suggested that the pulmonary incompetence induces intrauterine dilatation of the pulmonary artery, which leads to tracheobronchial compression. One of the presenting features in infants with APVS is severe airway obstruction, which may be difficult to manage. We report an infant who benefited from bilateral endobronchial endoscopic stent placement.

Cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency in a patient with HTLV-I.

The clinical spectrum of retroviruses is expanding rapidly. Human T-cell lymphotropic virus type I (HTLV-I) was the first retrovirus to be described, and its role had been established in adult T-cell leukemia/lymphoma and tropical spastic paraparesis. We report the case of a 35-year-old woman with HTLV-I and the unusual combination of cutaneous T-cell lymphoma, tropical spastic paraparesis, cerebral vasculitis, and protein S deficiency. We discuss the relationship of all her diseases to HTLV-I.

Cytolytic response to HIV in patients with HIV disease treated with extracorporeal photochemotherapy: preliminary study.

Extracorporeal photochemotherapy (photopheresis), an immunomodulatory therapy that targets circulating T helper lymphocytes, has been applied to the management of human immunodeficiency virus (HIV) disease. Any therapy that exerts its actions on CD4+ T cells has the potential of exacerbating HIV infection. Therefore, it was necessary to observe immune function during treatment. Because cytotoxic T lymphocytes (CTL) and natural-killer cells are thought to play an important role in the response against HIV infection, we examined the effect of photopheresis on HIV cytolytic activity. The study group consisted of seven patients with late-stage HIV disease who had not received any previous treatment for HIV infection. Patients were treated exclusively with photopheresis on two consecutive days each month for 14-32 months (average, 25 months). Peripheral lymphocytes, collected at various points during treatment, were used as effectors in a 51Cr release assay. Epstein-Barr virus (EBV)-transformed autologous B cell lines transfected with recombinant vaccinia vectors that expressed the HIV env (gp120, gp41) and gag (p24) proteins were used as target cells. All seven patients demonstrated relatively constant levels of cytolysis (>10% above controls) during treatment in the context of stable CD4+ T cell counts and a stable clinical status. These results suggest that extracorporeal photochemotherapy did not impair the cytolytic response to HIV infection and may have enhanced it in some patients.

Extracorporeal photochemotherapy in a patient with Ki-1-positive anaplastic large-cell lymphoma.

A 57-year-old man with Ki-1 anaplastic large-cell lymphoma treated with extracorporeal photochemotherapy is described.

Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis.

A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. Physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.

Right ventricular outflow muscle in tetralogy of Fallot: Histologic and immunohistochemical monoclonal antibody analysis.

To evaluate progressive muscular right ventricular outflow tract (RVOT) obstruction in tetralogy of Fallot (TOF), we hypothesized that this tissue would demonstrate more prominent selected immunohistochemical markers of myogenous cell differentiation, growth factor/receptor, and extracellular matrix. Sections of formalin-fixed, paraffin-embedded myectomy tissue obtained from RVOT at the time of surgical correction of TOF (n = 32; ages = 3 months through 13 years) were compared with age-matched tissue from the RVOT of normal control hearts (n = 12) obtained at autopsy after non-cardiac death. Examining by light microscopy slides stained with a combination of hematoxylin and eosin and elastic trichrome revealed cardiomyocyte (CM) hypertrophy, extensive myofiber disarray, trabeculation, multinucleation (more than two nuclei per myocyte), fibrosis, and thick-walled coronary arteries within the myocardium of TOF tissue. The endocardium from TOF specimens was thickened and hypercellular with prominent fibrosis and elastosis. Mitosis was not observed. At the interface between the endocardium and myocardium, the TOF specimens demonstrated myocyte dispolarity (orientation of CMs perpendicular to the endocardial surface), vascularization, and fibrosis. Immunohistochemical studies were performed using monoclonal antibodies to vimentin, desmin, muscle-specific actin (MSA), epidermal growth factor (EGF), epidermal growth factor receptor (EGFR), and laminin. Compared to the tissue from controls, TOF tissue showed a pattern of upregulated expression of epitopes within the endocardium and adjacent subendocardial myocardium. Decoration for MSA, vimentin, desmin, and EGFR highlighted the zonal nature of this tissue hyperactivity. Laminin prominently outlined endocardial cells, subendocardial CMs, and interface vessels in TOF tissue compared to the remainder of the myocardium and tissue from controls. Overall, changes in TOF were age-related, with older patients showing less zonal myocardial reactivity. These findings provide evidence for an ongoing, complex remodeling of the RVOT muscle in TOF.

"Meandering" pulmonary veins: report of a case in an asymptomatic 12-year-old girl.

We report a case of a healthy, asymptomatic 12-year-old girl in whom anomalous pulmonary venous connection to the left atrium occurred as an isolated congenital anomaly. This condition, termed "meandering" pulmonary veins, is closely related to scimitar syndrome and is usually associated with hypogenesis of the right lung and dextropositioning of the heart. To our knowledge, ours is only the second reported case of meandering pulmonary veins occurring as an isolated anomaly.

Maturation of the central nervous system: comparison of equine and other species.

This review covers the development and maturation of the cerebellum of the horse and compares this developmental sequence with that of man, mouse and chicken. These comparisons attempt to correlate morphological and neurochemical features, developmental time and functional performance necessary for survival at birth. Although there is great disparity between these 3 species, the basic anatomical structures of the cerebellum are present as are specific cellular groups, synapses and neurochemical markers. In addition to this structural homogeneity, other attributes of the cerebellum are its easily identified cellular populations and its well ordered pattern of growth and differentiation. The cerebella of the developing chick and mouse have been studied in great detail as they are amenable to experimental manipulations. The pattern of cellular differentiation appears to be reproducible from species to species and differs primarily as it relates to gestational age and functional requirements at birth. For instance, most of the large neurones of the cerebellar cortex differentiate early with small neurones and neuroglia differentiating later. Neurogenesis of the cerebellar cortex is fairly complete in the newborn foal and chick hatchling, but not in the human or rodent newborn.

Effects of placental pathology on maternal plasma progestagen and mammary secretion calcium concentrations and on neonatal adrenocortical function in the horse.

Between 1985 and 1990, 25 Thoroughbred mares were classified as having an abnormal pregnancy on the basis of precocious signs of impending parturition. Throughout the remainder of their pregnancies, plasma progestagen and mammary secretion calcium concentrations were monitored. After parturition, evidence of placental pathology was recorded and their foals were assessed with regard to adrenocortical function and behaviour. Seven pony mares were used to establish a model of placental pathology and, following induced placental separation, were subjected to the same protocol. Control animals consisted of 35 Thoroughbred mares and 39 pony mares, all considered to have normal pregnancies. Of the 25 abnormal mares, 16 had precociously increased plasma progestagen concentrations, 17 had precociously increased mammary calcium concentrations and 24 had an abnormal placenta. Eighteen live foals resulted, of which 11 appeared abnormal on the basis of behaviour. Adrenocortical function was assessed in 13; 4 had normo- and 3 hypofunction and 6 were intermediate. These results were independent of gestational age at delivery. Of 13 foals examined for plasma progestagens, 7 had normal and 6 abnormal concentrations. Following placental separation in the pony mares, maternal plasma progestagen concentrations increased in 4 of the 7 mares within 4-11 days and in 1 after 42 days; there was placental pathology in 3 of these mares, 1 of which aborted at 287 days gestation. There was no increment in progestagens in the remaining 2 mares, which aborted at 240 days and 271 days. It was concluded that placental dysfunction may be diagnosed from early plasma progestagen increments prior to 308 days gestation; and that foals that survive birth in these circumstances may be physiologically immature but often have normal or intermediate adrenocortical function, irrespective of gestational age. This is in contrast to foals born prematurely without maternal plasma progestagen or mammary secretion calcium increments.

The placenta as a determinant of fetal well-being in normal and abnormal equine pregnancies.

One hundred twenty-four horse placentas were evaluated according to gross appearance, weight and surface area. Grossly abnormal placentas were photographed, and histological sections were taken from abnormal appearing areas, and from representative areas (pregnant horn, non-pregnant horn, body and cervical pole) in both normal and abnormal placentas. Information about the resulting foals was obtained and correlations examined. Histological sections were reviewed and related to the resulting foals. Four normal fresh placentas were infused with dyes to ascertain the blood supply to various areas. Foal weight was statistically related to both allantochorionic surface area and allantochorionic weight. When normal placental surface area was divided by foal weight and related to gestational age, a statistical (P less than 0.0001 and r = 0.829) relationship emerged. Long cords were seen frequently with fetal demise. Histological abnormalities in the placenta were highly associated with fetal demise or fetal abnormalities, with only 2 normal Thoroughbred foals resulted from the 23 placentas exhibiting abnormal histology. Among the ponies, the same relationship held, with 3 normal ponies delivered from 9 abnormal placentas. Normal placentas were associated with normal offspring in most cases. Perfusion studies indicated that, in the four placentas studied, one umbilical artery supplied the cervical area and the non-pregnant horn, whereas the other supplied the pregnant horn. The body had a mixed supply from both arteries. This study evaluated the feto-placental unit in terms of normal and abnormal measurements and histology related to foal outcome. The presence of abnormal histological appearance of the placenta appeared to be more closely associated with foal abnormality than the percentage of placental area involved in the abnormal process. More information is needed before placental blood supply can be related to abnormal processes.