Two fetal deaths associated with maternal sepsis and with thrombosis of the intervillous space of the placenta.

The placental pathology in two second trimester fetal losses associated with mild maternal disseminated intravascular coagulation are reported. Case one had a dental abscess, a leukocytosis of 36300 white blood cells/m, and evidence of mild consumptive coagulopathy at 20 weeks. Case two had septic findings including disseminated intravascular thrombosis associated with pyelonephritis. The placentae had extensive intervillous thrombosis at the periphery of spiral arterial flow. It is hypothesized that in mild disseminated intravascular coagulation, the trophoblast inhibits fibrinolysis, favouring thrombosis perhaps due to production of plasminogen activator inhibitor.

Chronic deciduitis in the placental basal plate: definition and interobserver reliability.

This study tested whether concordance could be achieved for abnormal inflammation in the basal decidua of placental specimens among 6 pathologists experienced in placental pathology. Thirty microscope slides were evaluated by the pathologists for chronic deciduitis. They also scored the severity and extent of inflammation and the presence of plasma cells. No definition of chronic deciduitis was provided. Concordance (5/6 or 6/6 agreement) was achieved in 23 cases (76%). Spearman's rank correlation showed that the diagnosis of chronic deciduitis was almost identical to the assessment of the severity of the inflammation. A regression analysis showed that the perception of severity (and hence chronic deciduitis) was influenced by the other 2 variables, extent and plasma cells. The results were shared with the pathologists, and 25 cases (excluding those with previous 6/6 consensus) were reevaluated. Concordance was now achieved in the 83% of those remaining cases. Using a threshold based on the severity and the extent of lymphocytes, and the presence of plasma cells, pathologists are able to diagnose chronic deciduitis with sufficient concordance to be of value in clinical correlation studies.

Observer reliability in assessing placental maturity by histology.

AIMS: To evaluate the ability of five experienced perinatal pathologists to assess placental maturity reliably by histology. METHODS: Twenty four haematoxylin and eosin slides, six each from placentas of 27, 31, 35, and 39 weeks' gestation, were circulated to five pathologists on three separate occasions. The slides were labelled with the correct or incorrect gestational ages. RESULTS: The mean absolute error over all 360 readings was 2.72 weeks. Only 54% of the slides were assessed within two weeks of the correct gestation. Pathologist tended to overestimate younger gestations and underestimate older gestations. Two, and possibly three, pathologist were influenced by the gestational age state on the label. One pathologist, who did not appear to be influenced by the label, was more accurate in diagnosing gestation of the placentas than other colleagues. CONCLUSIONS: Experienced pathologists can have difficulty in assessing the villous maturity of placentas by histology. They can also be influenced by clinical information provided, such as gestational age. Other observer reliability studies must address the issue of the influence of labelled information on observer variation. A difference in maturation would have to be of a six week magnitude to have a chance of being detected by current methods. This may limit the value of the histological diagnosis of placental dysmaturity as a surrogate marker for uteroplacental ischaemia.

Histopathology of fetal membrane rupture: a review of the literature.

Ideally, the histological examination of the fetal membranes should reveal something of the mechanism that ruptured them. However, like any investigation of the crime, there needs to be careful sifting of evidence and confirmation of the validity of inferences. In the case of membrane rupture, the histology must be correlated with the physics of rupture. The usual mental model of the physics of membrane rupture is based on our everyday experiences with the physical world of water balloons, cellophane wrappers, etc. First reviewed are aspects of that intuitive physical model that are important to understanding the histology. Then, the literature is reviewed for histologic correlation with physical and biological observations of membrane rupture.

The anatomic basis of maternal serum screening.

Fetal serum markers, such as alpha fetoprotein (AFP), must traverse one of two very different pathways to reach maternal serum, either from fetus to amnion fluid, membranes and decidua or from fetal to maternal circulation through the placental villi. Alpha fetoprotein usually enters the amnion fluid through body wall defects uncovered by skin or through urine. Placental AFP leakage may be from villous hemorrhage or injury. These observations from anatomic pathology suggest that biochemical markers may exist to identify the source of elevated maternal serum AFP.

Megakaryocytosis of the liver in a trisomy 21 stillbirth.

This is a case report of a stillborn infant with duodenal atresia and increased megakaryocytes in the liver sinusoids and in pancreatic connective tissue. A postmortem karyotype of skin demonstrated trisomy 21.

The pathologic findings of the fetal membranes in very prolonged amniotic fluid leakage.

We examined the fetal membranes in five patients with prolonged amniotic fluid leakage. Four patients had a clinical history of fluid leakage of at least six weeks' duration, while, in the fifth patient, prolonged leakage was only an inferred diagnosis. Four of the infants died within the first two days of life, while one infant survived. The pathologic findings were varied. Two cases showed, to our knowledge, a previously unreported subchorionic accumulation of squames, which were presumably from cells that were shed into amniotic fluid. One other case showed a subchorionic foreign-body reaction. The two remaining cases showed only necrosis and hemorrhage.

Carcinoembryonic antigen, alpha 1-antitrypsin, and alpha-fetoprotein in the pancreas of patients with cystic fibrosis.

The ductular accumulation of "abnormal mucus" is the key histologic feature in cystic fibrosis. This material is periodic acid-Schiff positive and diastase resistant, suggesting that it is glycoprotein in nature. We used the avidin-biotin-peroxidase method to identify this material using antibodies to the serum glycoproteins carcinoembryonic antigen, alpha 1-antitrypsin, and alpha-fetoprotein on paraffin sections of pancreas obtained from a total of 21 patients: 9 with cystic fibrosis, 5 with chronic pancreatitis, and 7 controls. The control patients had normal pancreatic histologic findings, no alpha 1-antitrypsin or alpha-fetoprotein was demonstrated, and only the ductular epithelium reacted weakly for carcinoembryonic antigen. The pancreas in pancreatitis showed fibrosis, acinar atrophy, and ectasia of the ducts that contained only a small amount of periodic acid-Schiff-positive material. This material reacted weakly for carcinoembryonic antigen and alpha 1-antitrypsin. The appearance of the pancreas in cystic fibrosis was similar to that in chronic pancreatitis. However, the ducts contained a greater amount of periodic acid-Schiff-positive material, mostly in the form of globules that reacted strongly for carcinoembryonic antigen and alpha 1-antitrypsin and weakly for alpha-fetoprotein, as did the ductular epithelium. This study shows that the periodic acid-Schiff-positive material in cystic fibrosis contains at least the three serum glycoproteins and that the accumulation may represent a possible defect in cellular synthesis, assembly, or transport of glycoproteins in the ducts.

A maternal death due to thrombotic disease associated with anticardiolipin antibody.

We report a case of fetal death at 30 weeks' gestation followed by the unexpected death of the mother 28 1/2 hours later. At postmortem examination, extensive small-vessel thrombi were found in the maternal organs, including the uterus, kidney, and heart. In retrospect the mother had evidence of chronic pericarditis and myocarditis. Laboratory tests of antemortem serum demonstrated elevated titers of anti-double-stranded DNA and of anticardiolipin antibodies.

Invasive Candida enteritis of the newborn.

Three premature infants (<800 g) showed invasive Candida at the site of their intestinal perforations. This entity is distinct from Candida peritonitis complicating necrotizing enterocolitis and was uniformly fatal. Recognition and aggressive antifungal therapy may improve outcomes.

Further observations of ocular pathology in trisomy 9.

The ocular pathology in a new patient with mosaic trisomy 9 comprised major anomalies and contrasted sharply with the findings in a previous case reported by us. The ocular changes in this case were, in essence, indistinguishable from those encountered in the most severe form of trisomy 13. Similarities to trisomy 18 and 21 were further evidence of the overlap of ocular findings in autosomal trisomies. There is increasing evidence that most, if not all, chromosomes have some role in regulating ocular embryogenesis.

Twin transfusion: pathological studies of the monochorionic placenta in liveborn twins and of the perinatal autopsy in monochorionic twin pairs.

Vascular injections of 21 monochorionic placentas from liveborn twins were scored for chorionic surface vessel anastomoses and for venous return areas. The results were correlated with neonatal birth weight and first hematocrit. Weight and hematocrit differences did not correlate with each other or with placental anastomoses. There was a relationship between weight and placental area to the individual twin. In some cases, weight differences between monochorionic twins may be the result of placental differences rather than chronic transfusion. Seventeen perinatal autopsies of monochorionic twin pairs were reviewed for plethora/pallor and for organ and body weights. Cardiac hypertrophy was present in three autopsies. Cardiac hypertrophy may be a measure of chronic twin transfusion in the recipient. The lighter twin was as likely to be plethoric as the heavier. Plethora postmortem is a poor measure of chronic transfusion because of confounding factors, particularly acute twin transfusion. As a result of these studies, placental anatomy is speculated to be complexly rather than directly related to clinical outcome.

Ivemark's renal-hepatic-pancreatic dysplasia: analytic approach to a perinatal autopsy.

A case of Ivemark's renal-hepatic dysplasia syndrome is presented. The findings are suggestive of a ductal obstructive process anatomically located at the junction of ducts with the parenchyma. The differential diagnosis includes Meckel syndrome, short rib polydactyly syndromes, and glutaric aciduria type 2. The molecular basis for this syndrome remains unknown.

Review of some causes of stillbirth.

Stillborn fetuses commonly demonstrate three mechanisms of death: hydrops, asphyxia, or shock. For each mechanism, the autopsy can discover any of multiple etiologies. This paper reviews the etiologies and pathogeneses of fetal death from that perspective.

Glycogen accumulation in the pars recta of the proximal tubule in Fanconi syndrome.

We reviewed the renal pathology in 10 cases of renal Fanconi syndrome. Five cases showed the Armanni-Ebstein lesion, i.e., clear glycogen-filled cells limited to the pars recta of the proximal tubules. The 5 cases included 2 siblings with a unique syndrome characterized by death in infancy, severe Fanconi syndrome, severe rickets, carnitine deficiency, and atrophy of the exocrine pancreas. Two other siblings had glycogen storage disease type XI. One of 4 cases of putative tyrosinemia had the lesion. The ultrastructure was studied in 2 cases. The Armanni-Ebstein lesion in these cases was morphologically indistinguishable from that seen in diabetic patients dying after prolonged hyperglycemia. Glycosuria is the only common factor in both diabetic hyperglycemia and the varied proximal tubular diseases studied. The mechanism of the glycogen accumulation in this short parts recta segment of the proximal renal tubule was further investigated by reviewing the renal histology in cases of glycogen storage disease types I, II, III, and VIII. None showed the Armanni-Ebstein lesion, but type I showed glycogen deposition throughout the proximal tubule. Thus, the Armanni-Ebstein lesion is not the result of an enzymatic deficiency for glycogen synthesis in the convoluted tubules.

One-month-old infant with multiple ulcers of stomach, small bowel, large bowel, and protein-losing enteropathy: case report.

Multiple inflammatory ulcers of the gastrointestinal tract are rare in young infants. Most cases are caused by infectious organisms, vasculitis, or an autoimmune process. We report a 1-month-old infant who was healthy until he presented with an inflammatory mesenteric cyst, and multiple ulcers of the stomach, duodenum, jejunum, ileum, and colon. Histologically, the ulcerations were sharply demarcated, full thickness, and filled with macrophages. He had a low serum albumin and IgG due to protein-losing enteropathy. He was treated with supportive care and immunomodulating drugs. The gastrointestinal inflammation resolved by 3 and 1/2 years of age. The medications were withdrawn at 5 and 1/2 years of age he had no relapse of clinical symptoms. He continues to have asymptomatic mild hypoalbuminemia and low serum IgG. We could not find a report of a similar clinical presentation and outcome.

Maternal floor infarction in autoimmune disease: two cases.

Two cases of maternal floor infarction in women with autoimmune antibody production to various antigens are presented. We speculate that maternal floor infarction could be produced by antibodies directed against the placental urokinase plasmin system.