Conjoined twinning is a rare obstetric event that has fascinated physicians and laypersons alike for centuries. When this event occurs, early diagnosis, close prenatal management, and the proper route of delivery will assure the best possible outcome for mother and affected infants. Magnetic resonance imaging (MRI) is used both as an ancillary technique to the sonography for the antepartum diagnosis and in the preoperative planning for the surgical separation of conjoined twins. This report describes the MRI findings in conjoined twins of the thoraco-omphalopagus symmetricus type, a condition incompatible with extrauterine life. The twins were delivered at 21 weeks gestation. The MRI was used to evaluate the anatomic nature of the internal anomalies. These findings were then used to develop an autopsy approach plan that preserved the external phenotype. After dissection, the specimen was preserved by using polyethylene glycol and ultimately prepared for use as a teaching specimen.

Autopsy↗

PubMedDate not supplied

Study of the malformation of ductal plate of the liver in Meckel syndrome and review of other syndromes presenting with this anomaly.

Meckel syndrome (MIM 249.000) is an autosomal recessive disorder with a variable spectrum of anomalies. Since the first reports of this syndrome, very broad diagnostic criteria have been proposed, but the process of defining them continues. It is probable that at least two of three manifestations, including cystic kidney dysplasia, occipital encephalocele or other anomaly of the central nervous system, and postaxial polydactyly occur in most cases. Arrest of the development of intrahepatic bile ducts at the stage of the bilaminar plate formation or ductal plate malformation is considered of high diagnostic value in Meckel syndrome, but there is no complete agreement in the literature about its occurrence. The aims of this investigation were to study the prevalence and morphologic patterns of ductal plate malformation of the liver in Meckel syndrome by evaluating the dilatation of primitive biliary structures and the increase in connective tissue of the portal tract. Archival data files from four German centers (Berlin, Freiburg, Heidelberg, Mainz) were reviewed. Liver sections of 30 well-studied fetuses with Meckel syndrome were immunostained with antibodies against cytokeratins (intermediate filaments of the cytoskeleton) and factor VIII (an endothelial cell marker) and were evaluated both qualitatively and quantitatively. Cystic kidney dysplasia, occipital encephalocele, and postaxial polydactyly were found in 100%, 90%, and 83.3% of the fetuses, respectively. Ductal plate malformation of the liver was a constant anomaly in Meckel syndrome, seen as frequently as renal lesions. We observed essentially two kinds of hepatic lesions: 23 cases showed mainly a cystic dilatation of primitive biliary structures with little portal fibrosis, while 7 cases showed mainly rings of interrupted curved lumina around a central fibrovascular axis and pronounced portal fibrosis. In these seven cases an abnormal pattern of the portal vein, with many small and closely spaced branches of the portal vein (the so-called pollard willow pattern), was also seen. With respect to other fetal developmental anomalies, no difference between the two types of lesions was found. We also provide a potentially useful comprehensive review of other genetic syndromes in which ductal plate malformations may occur.

Abnormalities, Multiple↗

PubMedDate not supplied

Huge fetal sacrococcygeal teratoma with a completely formed eye and intratumoral DNA ploidy heterogeneity.

Preterm infants and newborns with sacrococcygeal teratomas are at high risk for prenatal and perinatal complications. The prognosis depends on size and histology of the tumor, degree of prematurity, associated malformations, route of delivery, and prompt and complete surgical removal. Virtually any tissue can be present in a sacrococcygeal teratoma, but to date, ocular lens has been documented only as lentinoids (lens-like cells), whereas flow-cytometric data have been variably interpreted. We describe a case of a sacrococcygeal teratoma in an infant of 29 weeks gestational age that is remarkable for the weight (4500 g), the presence of a completely formed eye, and intratumoral DNA ploidy heterogeneity.

Choristoma↗

PubMedDate not supplied

Alterations of the intestinal mucosal block in ulcerative colitis and Crohn's disease--immunological and ultrastructural findings, and considerations of the pathogenesis.

The intestinal mucosa is the very site of the chronic inflammatory lesion in ulcerative colitis (UC) and in Crohn's disease (CD). The lamina mucosa together with its own and associated cellular and humoral protective properties can be described as the mucosal block which is functionally related to the intestinal and systemic immune system. Deteriorations of this mucosal block probably are of pathogenetic significance in UC and CD. This paper will summarize observations on the systemic immune system and local immune phenomena and their possible relations to the tissue injury in the gut. An immunopathogenetic concept intends to explain the chronicity of the diseases: epithelial insufficiencies of the mucosal block are followed by strong antigenic intrusion and stimulation of the local immune system with disturbance of the local immune homeostasis and the formation of immune complexes. The interactions of phagocytes with immune complexes cause the release of degrading lysosomal enzymes, which leads to continuous injury of the mucosal block and therefore to a perpetuation of the disease.

Antigen-Antibody Complex↗

PubMedDate not supplied

Evidence for local immune complexes in ulcerative colitis.

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Adolescent↗