The use of radionuclides in the investigation of conjoined twins.

Three sets of thoraco-omphalopagous conjoined twins were investigated. The clinical findings indicated a complex shared heart in two sets, and separate cardiovascular systems in the other. These assessments were confirmed by dynamic imaging after bolus injection of Tc-99m(Sn)colloid or Tc-99m millimicrospheres. Static images were then used to establish the configurations of the shared livers. The results of these studies were in keeping with the angiographic and autopsy findings in the two sets with complex cardiac anomalies and with the surgical findings during successful separation of the third set.

Placentae of light for dates infants born to underweight mothers at term: a morphometric study.

Fifteen light for dates infants and their placentae were compared to 15 well-grown infants and their placentae. The former were born to thin, underweight women while the latter were born to women of normal weight. The light for dates infants were symmetrically growth retarded but not wasted at delivery and their placentae had a reduced weight, volume, chorionic surface area, percentage parenchyma and total villous surface area. The peripheral villous surface area and volume of peripheral villous trophoblast, fetal capillaries and connective tissue was also reduced in the placentae of light for dates infants, suggesting retarded placental growth in the latter half pregnancy. In contrast, the stem villous surface area and volume of stem villous trophoblast, fetal capillaries and connective tissue was similar in both groups of placentae, suggesting the same rate of growth in early pregnancy. There were no differences in the volume of fibrin or infarcts. The ratio of total villous surface area to infant weight, length and head circumference was reduced in the light for dates infants. This may restrict the materno-fetal oxygen exchange, and thereby increase the risk of fetal hypoxia during labour. It is concluded that the placentae of light for dates infants born at term to underweight women are both absolutely and relatively small with a reduced villous surface area.

Ploidy analysis on Wilms' tumour touch imprints using ethidium bromide and automated image analysis integrated confocal laser scanning microscopy.

Although image analysis (IA) is increasingly being used to quantitate nuclear DNA, comparative data between fluorescence methods of IA and flow cytometry (FCM) is limited. In this study fluorescence IA was compared with FCM data in a series of Wilms' tumour touch preparations. Airdried touch imprints that had previously been Giemsa stained were restained with ethidium bromide. Confocal fluorescence images were obtained with a confocal laser scanning microscope and assessed by a fully automated IA package. Data was collected from 400 nuclei per imprint. The resulting DNA histograms were analysed and ploidy status and DNA indices determined using standard criteria. Results were compared with those derived from FCM analysis of nuclear suspensions. Ten of twelve tumours were concordant by both techniques. However in two cases assessed as diploid by FCM, IA identified aneuploidy. Excellent correlation between DNA indices as assessed by both techniques was observed (r = 0.987). In the three cases for which both unstained and Giemsa stained touch imprints were available for IA, the histogram configurations did not differ significantly. Fluorescence IA is an accurate and sensitive technique for DNA quantitation, which appears at least comparable to FCM assessment and which has a number of important advantages.

Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature.

Primary pulmonary tumors are infrequent in childhood, therefore an accurate diagnosis and treatment is often delayed. We review the English language literature and report the clinical and pathological features of eight tumors arising in the lungs of pre-adolescent children, accessioned between 1960 and 1991 in the pathology department of a children's hospital in South Africa. The ratio of pulmonary primary tumors to secondary neoplasms and to non-neoplastic lesions of the lung examined during this period was 1:5:60. Over the last 31 years we received three plasma cell granulomas, two pleuro-pulmonary blastomas, a mucoepidermoid carcinoma, an endobronchial fibrosarcoma, and a hemangioma. All patients presented with cough unresponsive to medical treatment. The incidence and spread of primary lung tumors in children was similar to that reported from other centers. Plasma cell granuloma is the most common primary tumor in the lungs of children. Aggressive behavior is most frequently encountered with pleuro-pulmonary blastoma and rhabdomyosarcoma, and because of their association with cystic lesions careful examination of lungs is required in such cases. Most other malignant neoplasms, such as muco-epidermoid carcinoma and primary fibrosarcoma, are usually of a low grade of malignancy. A decreasing incidence of bronchogenic carcinoma seems to be reported during the first two decades of life.

Intestinal obstruction in the newborn with congenital syphilis.

Four newborn patients with congenital syphilis and intestinal obstruction are discussed in this report. In three cases, the obstruction was due to inspissated meconium, simulating the features of meconium ileus in one, meconium plug in the second, and associated with perforation of the terminal ileum in the third. An exocrine pancreatic insufficiency and a motility disturbance of the intestine due to syphilitic infection during fetal life could be the probable etiologic factor. The fourth patient had multiple ileal stenoses. Ischemia due to the prenatal syphilitic arteritis of the bowel wall can be responsible for this pathology. Attention is drawn to the association between congenital syphilis and neonatal intestinal obstruction.

A histological grading system for the evaluation of co-existing NID with Hirschsprung's disease.

The significance of dysplastic features in the surgical pullthrough segment of bowel in patients with Hirschsprung's disease (HD) has not yet been clarified. The aim of this study was to evaluate prospectively the ganglionated proximal bowel in 26 patients with HD (January 1988 through January 1991). The significance of dysplastic features and their influence on post operative outcome were evaluated by means of a newly devised histological scoring system based on the morphological features. Functional outcome was assessed clinically at follow-up interview. Comparison was with control specimens from 22 patients undergoing unrelated bowel surgery and a further 5 patients with neuronal intestinal dysplasia (NID). Results indicated a wide spectrum of histologically identified dysplastic features in patients with NID, the ganglionated bowel of HD and controls. Although individual abnormal features were noted in the control group, significant degrees of dysplasia were absent. The overall degree of dysplasia was less striking than that observed in NID and in the 5 patients in whom NID co-existed with HD. Dysplasia of the ENS in residual bowel could be correlated with postoperative dysfunction in 4 out of 5 patients (80%) with HD and features of co-existing NID. In addition, milder symptoms were noted in 50% of patients having a borderline score (5-6/12). This study emphasizes the relationship between clinical obstructive symptoms and a high degree of dysplasia within the ENS. A histological grading system is of value in evaluating the spectrum of abnormal findings and prospectively identifying those with functional significance in patients with NID co-existing with HD.

Degenerative leiomyopathy in African children: a review of current perspectives.

The syndrome of degenerative leiomyopathy causing intestinal obstruction clinically manifests in young indigenous African children as massive megacolon without aganglionosis. Eighteen children have been seen over a 16 year period from a localized geographic area. There were 10 males and 8 females having a mean age of 9.5 years at presentation and a mean duration of symptoms of 4.3 years. The youngest was 6 months old. All had progressive abdominal distension and infrequent stooling but 11 had intermittent diarrhoea and 9 had colicky abdominal pain. Gross gaseous distension of the large bowel with extension into small intestine occurred in 9 and this extended into stomach and oesophagus in 4. Biopsy of the dilated, thin walled bowel showed smooth muscle degeneration and necrosis with replacement by fibrous tissue. Neuronal cells of Auerbach's plexus tend to be displaced into the circular layer of smooth muscle with mild inflammatory changes. Some small arteries show medial fibrosis with subintimal fibroblastic proliferation. Acetylcholinesterase and immunohistochemical staining with neural and muscle markers are within normal limits. One child died while 14 have been maintained on prokinetic agents, low residue diets, laxatives and enemas. Nine children have required surgical intervention of whom 4 had volvulus and 3 adhesive bowel obstruction. Degenerative leiomyopathy is a distinctive entity with classical clinical and histological features. The aetiology is still obscure.

Placental histology related to fetal brain sonography.

BACKGROUND: Chronic hypoxia and inflammatory processes can induce placental disturbances that may indirectly lead to perinatal brain injury. OBJECTIVE: To study histological features of the placenta in relation to echogenicity changes in the periventricular white matter, ventricular system and basal ganglia/thalami of the fetal brain. DESIGN: Prospective study of 77 fetuses between 26 and 34 weeks gestational age with their placentas. The pregnancies were complicated by hypertensive disorders (n=42) or preterm labour (n=35). RESULTS: Of the placentas 79% showed uteroplacental hypoperfusion, inflammation or a combination. Transvaginal ultrasound examination of the brain revealed echogenicity changes in 73% of the fetuses (44 mild, 29 moderate). Moderate brain echogenicity changes (periventricular echodensity (PVE) grade IB: increased echogenicity brighter than choroid plexus, intraventricular echodensity (IVE) grade II and III: echodensity filling ventricle respectively <50% and ≥50%; basal ganglia/thalamic echodensity (BGTE): locally increased echogenicity within basal ganglia/thalami) were equally distributed over cases with uteroplacental hypoperfusion and inflammatory features in the placenta. PVE grade IB was always associated with placental pathology. The sensitivity and negative predictive value of placental pathology for moderate echogenicity changes were high (0.91 and 0.88, respectively), while the specificity and positive predictive value were low (0.27 and 0.34, respectively). CONCLUSIONS: Normal placental histology predicted no or mild echogenicity changes, supporting the view that the latter are physiological. Placental pathology was always present in cases with grade IB PVE, presumed to represent mild or early forms of white matter injury. Both uteroplacental hypoperfusion and inflammatory features were seen in placentas from pregnancies with hypertensive disorders.

Fatal meningococcal disease in childhood: an autopsy study of 86 cases.

A retrospective study of the pathology in 86 consecutive autopsies of fatal meningococcal infection in children, performed at the Red Cross War Memorial Children's Hospital during the 19-year period from 1973 to 1991, was undertaken. The most prominent pathological changes found at autopsy were those of an overwhelming bacterial infection with evidence of disseminated intravascular coagulopathy in many organs of the body. The skin, adrenal glands and central nervous system were most commonly involved. Acute myocarditis occurred in 23 cases (27%) and was diagnosed almost exclusively histologically. In only one case was it diagnosed clinically. In addition, the nutritional status and the morphological expression of immune reactivity of our hospital population was better than expected.

Castleman's disease in children: the experience of a children's hospital in Africa.

As the incidence of childhood Castleman's disease (CD) is reported to be rare in Western communities, the recent occurrence of three cases in non-HIV infected prepubertal children stimulated a 10-year (1983-1993) retrospective investigation into the conditions at a children's hospital that serves a predominantly developing community. Retrospective histologic examination revealed six cases of CD among children 3-11 years of age and another five probable cases having histological features of CD without adequate clinical and laboratory data. Three CD cases were associated with malignancy (Kaposi's sarcoma in two and a gastric B cell lymphoma in the other). Two CD cases were plasma cell type, one hyaline vascular, and three intermediate. One child with recurrent plasma cell-type features in a groin node had a Kaposi's sarcoma in the thigh. The five probable cases of CD all had intermediate histological features. Four of the 11 cases being reported came from a geographic locality that provides about 3% of the laboratory's case material and these included both cases that developed Kaposi's sarcoma. All patients who developed malignancy had the plasma cell variant. As all cases have been identified in developing communities, where the disease may often be misdiagnosed as nonspecific reactive lymphadenopathy, and there is an evident association with malignancy, it is recommended that children in these areas with the plasma cell variant of CD be closely monitored for subsequent malignancy.

Virus peneumonia following measles: a virological and histological study of autopsy material.

Adenovirus, measles virus and herpesvirus were found to be responsible for the serious non-bacterial bronchiolar and interstitial necrosis in post-measles pneumonia in underprivlieged non-White children less than 4 years of age in Cape Town. Secondary bacterial bronchopneumonia, possibly antibiotically suppressed, proved to be less important in accounting for the extensive lung damage in 18 fatal cases that were examined virologically and histologically.

Immunohistochemical characterization of Burkitt's lymphoma.

Cytoplasmic immunoglobulins and muramidase (lysozyme) were demonstrated in formalin-fixed tissues by an immunoperoxidase procedure in 3 cases of Burkitt's lymphoma. The Burkitt cells were strongly positive with the full panel of monospecific antisera against human immunoglobulin components (kappa and lambda light chains, gamma, alpha and micron heavy chains). The 'starry-sky' macrophages were weakly positive with antimuramidase antiserum and strongly positive with the antisera against immunoglobulins, thus demonstrating their phagocytic and histiocytic nature. The reasons for the polyclonal increase in immunoglobulins are discussed.

Cardiac failure in a case of aneurysm of the vein of Galen associated with diffuse meningeal angiectasia.

A case of aneurysm of the vein of Galen associated with diffuse meningeal angiectasia is described and compared with a similar reported case. The current literature dealing with arteriovenous malformation of the vein of Galen in young children is reviewed. The need for proper clinical and angiographic examination of all newborn infants with congestive cardiac failure due to extracardiac causes is stressed. Attention is drawn to the association of meningeal angiectasia with arteriovenous malformation of the vein of Galen.

Late morphologic consequences of measles: a lethal and debilitating lung disease among the poor.

Pneumonia that occurs within 28 days of the onset of measles rash is a common cause of severe pulmonary morbidity and/or death among poor children. The prevalence of such pneumonia can be related to the effectiveness of measles immunization programs. For 20 of 57 new cases of bronchiectasis in children undergoing bronchography, a strong causal relationship to measles was found. The lungs of 21 unselected children who died in the wake of measles were examined. Severe necrosis of bronchi and bronchioles was found in those children who had developed intercurrent adenovirus and herpesvirus infections. Bacterial suppuration produced a less severe necrosis. It is suggested that intercurrent adenovirus and herpesvirus infections that occur following measles are the most important initiating causes of follicular bronchiectasis in childhood. The severity of these supervening infections may be mediated by the transient immune suppression that occurs as a consequence of both measles and inadequate nutrition.

Childhood pulmonary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma.

Two fatal childhood cases of the rare pulmonary blastoma are reported. One was associated with a congenital cystic adenomatoid malformation. Both neoplasms extended to involve visceral pleura and were entirely composed of blastemal and mesenchymal elements without recognizable neoplastic epithelial components. The mesenchymal component in both instances consisted of malignant rhabdomyoblasts, undifferentiated mesenchyme, and differentiated, apparently benign, cartilage. Review of the literature suggests that these features may be specific for the childhood forms of pulmonary blastoma. It is further suggested that pulmonary blastoma, malignant mesenchymoma of the lung, and primary pulmonary rhabdomyosarcoma may have a common pathogenetic origin.

Factors associated with fatal cases of measles. A retrospective autopsy study.

Clinical and laboratory records at Red Cross War Memorial Children's Hospital, Cape Town, for the period 1976-1982 were reviewed to determine factors associated with fatal cases of measles. Pneumonia was found to be the commonest lethal complication of measles. Supervening infections by both viral (especially adenovirus and herpesvirus) and bacterial (especially Klebsiella species and Pseudomonas) agents played a prominent role in causing the deaths of children who had recently been infected with measles. Severe malnutrition was present with almost equal frequency among those dying of measles and those dying from other causes, but was significantly (P less than 0,02) less common in measles patients who survived. Peripheral lymphopenia and depletion of T-cell zones in the lymph nodes and spleen were more common in those who died from measles than in others.

Familial haemophagocytic reticulosis. Report of 2 cases in sibs.

Two cases of familial haemophagocytic reticulosis occurring in sibs are described. The disease is characterised by a widespread proliferation of histocytes showing haemophagocytosis and it usually manifests clinically with haematological abnormalities. The initial presentation may be that of a meningo-encephalitic illness, the result of a lymphohistiocytic infiltration of the meninges and cerebral tissues. The clinical course and pathological features are described and the differential diagnosis is discussed.

Mesenchymal hamartoma of the chest wall: a cooperative study with review of the literature.

The clinicopathologic features of three examples of mesenchymal hamartoma of the chest wall are described. The entity has been recorded under a number of names including osteochondroma, osteochondrosarcoma, benign chondroblastoma, mesenchymoma, and chondromatous hamartoma. The condition is manifest at birth or shortly thereafter with deformity of the chest wall and respiratory distress. Radiographic examination reveals a well-defined, partly calcified mass involving one or more ribs. The tumor is composed predominantly of chondroid tissue with large endothelium-lined blood spaces and immature mesenchyme with osteoclastic giant cells and osteoid. We review the literature and suggest that the lesion should be distinguished from aneurysmal bone cyst, chondroma, and other mesenchymal neoplasms. In order to avoid local recurrence the recommended treatment is complete surgical resection.

Pluripotential melanoblastoma, a unifying concept on malignancies arising in congenital melanocytic nevi: report of two cases.

Congenital melanocytic nevi are benign lesions present at birth and considered to be caused by a maldevelopment of the neural crest. The malignant potential of the congenital melanocytic nevi have been extensively addressed by several authors, and malignant melanoma is the most frequent neoplasm arising in these lesions. The present report describes two patients with congenital melanocytic nevi in which malignant melanoma with undifferentiated areas showing rhabdomyoblastic differentiation developed. The findings suggest that these mixed neoplasms may be recapitulating the differentiation potential of the ectomesenchyme-neural crest cells. We advocate the term "melanoblastoma" when referring to them.