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 The medications were withdrawn at 5 and 1/2 years of age he had no relapse of clinical symptoms.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.

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.

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.

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.

Fetal membrane histology in preterm premature rupture of membranes: comparison to controls, and between antibiotic and placebo treatment. The National Institute of Child Health and Human Development Maternal Fetal Medicine Units Network, Bethesda, MD, USA.

The objectives of this study were to test the hypotheses that antibiotic therapy will alter the histologic appearance of fetal membranes in preterm premature rupture of membranes (pPROM), and that the membrane histology will demonstrate distinct differences between term and preterm rupture of membranes. We also wished to test interobserver variability of pathologists. Placental membranes were sampled from 268 women participating in a randomized placebo-controlled trial of antibiotic therapy for pPROM at 24-32 weeks of gestation (cases) and from 4 control groups who were not in the randomized trial: (1) preterm labor without pPROM (n = 21), (2) term labor (n = 65), (3) term PROM (n = 21), and (4) term cesarean section (n = 27). The cases and controls were scored for 40 histologic features by pathologists blinded to the identity of each sample (case or control). pPROM histology of samples from patients receiving antibiotics and those receiving placebo was compared using a chi-squared test and with control groups using logistic regression. There were no histological differences between pPROM cases treated with antibiotic and those receiving placebo, nor with respect to duration of membrane rupture greater or less than 48 h. Concordance among pathologists was low for features other than acute inflammation. Logistic regression analysis controlled for race and pathologist, and demonstrated that all of the control groups had significantly fewer common markers of acute inflammation when compared with the pPROM cases. This study suggests that histopathologic evidence of infection is seen more frequently with pPROM than in preterm or term controls. The histologic features used in this study cannot be used to determine the effectiveness of antibiotic therapy.

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.

Candida funisitis: A clinicopathologic study of 32 cases.

We report on 32 cases of Candida funisitis and describe the associated clinicopathologic features. The Candida funisitis was characterized grossly by small, circumscribed, yellow-white nodules on the umbilical cord surface and, microscopically, by subamnionic microabscesses in which fungal organisms were demonstrable. Chorioamnionitis was present in all cases. Twenty-four (75%) of the 32 infants were premature. There were 7 perinatal deaths, all in immature fetuses. Five (16%) of the 32 fetuses had congenital candidiasis. Five (16%) of the mothers had a history of intrauterine foreign body, including intrauterine contraceptive device in three and cervical cerclage in two. The diagnosis of Candida funisitis should prompt a careful examination for fetal infection, even though it is associated with congenital candidiasis in only a minority of the cases.

Predictive value of fetal ultrasonography in the diagnosis of a lethal skeletal dysplasia.

BACKGROUND: Certain ultrasonographic findings identified in a fetus suspected of having a skeletal dysplasia may be predictive of a lethal outcome. METHODS: We evaluated 27 fetuses suspected of having a skeletal dysplasia using targeted ultrasonography between 16 and 31 weeks' gestation. Clinical examination and skeletal radiography were done after delivery. RESULTS: A skeletal dysplasia was confirmed and a diagnosis established in all but one case. The skeletal dysplasia was lethal in 23 cases and, in each case, the outcome was accurately predicted prenatally; however, three of the infants survived several months. In 11 of the 23 cases (48%), the specific diagnosis was correctly determined before birth. Ultrasonographic findings not considered to reflect a lethal outcome, were accurately predicted in two other cases. In an additional two, sonographic examination suggested a lethal osteochondrodysplasia, though both survived. Findings consistent with a lethal skeletal dysplasia included a femur length < 1st centile, combined with either a bell-shaped thorax, decreased bone echogenicity, or both. Using these criteria provided a positive-predictive value for neonatal deaths of 80% (20/25), and 92% (23/25) if the three that died in infancy were included. CONCLUSIONS: In the fetus suspected of having a skeletal dysplasia, certain findings on targeted ultrasonography frequently are predictive of a lethal outcome; the ability to predict this appears greatest when more than one of these abnormalities is present.

Endometrial stromal sarcoma involving the placenta.

This report presents two cases of endometrial sarcoma tumor detected in decidua submitted with placentas for pathologic evaluation. The first patient had low-grade endometrial stromal sarcoma discovered in the omentum and decidua at the time of a cesarean section for placenta previa. The second patient underwent a cesarean section for breech presentation and was noted to have a mass beneath the placenta that was histologically compatible with low-grade endometrial stromal sarcoma. We believe these to be the first reported cases of endometrial stromal sarcoma involving the decidua. These findings should elevate awareness that endometrial stromal tumors may be detected during placental examination.

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.

Histologic features of chorioamnion membrane rupture: development of methodology.

This study developed a set of histologic features that will allow subclassification of placentas with preterm premature rupture of membranes. Placentas were obtained from patients participating in a multi-institutional NICHD Maternal-Fetal Medicine Unit Network study of antimicrobial therapy after preterm premature rupture of membranes. The rupture site was sampled by inking the open sac margin and rolling a membrane strip in four quadrants from the ink to the placental margin. Independently, four pathologists used a provisional feature list to score the slides from 15 placentas. A concordance analysis was performed on those results. With those results, the slides were reviewed concurrently to discover the source of disagreements and to revise the feature list. The sampling method frequently demonstrated a rupture site with histology distinct from that of the remainder of the membranes. After review of the preliminary scoring results, 29 features of membrane histology present in preterm premature rupture could be objectively described with agreement among four pathologists. The feature list allows both novel and commonly recognized histologic features of fetal membranes to be recorded with objectivity. This list, with the described sampling technique, is presented as a tool for clinical correlation in studies of membrane rupture, especially in preterm, premature rupture.

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.

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.

Perinatal pathology of interhemispheric cyst with thinned posterior corpus callosum: four cases.

This paper presents four posterior, interhemispheric cerebral cysts found at perinatal autopsy. All cysts appeared to attenuate the overlying posterior corpus callosum. All had a base over the roof of the third ventricle and midbrain with a lining that resembled tela choroidea with the choroid plexus tufts projecting into the lumen of the cyst. Three cases had enlargement of the lateral ventricles. One case had complete communication between the cyst and the posterior lateral ventricles reminiscent of a holoprosencephaly confined to the posterior telencephalon. One case demonstrated a complete VATER association and two others had some features of the VATER association. We hypothesize that this latter relationship suggests an origin for the cysts during blastogenesis.

Foreign body reaction to exogenous pulmonary surfactant in an infant with hydrops and cardiomyopathy.

We present a hydropic infant who received exogenous surfactant and who had Noonan phenotype with hypertrophic cardiomyopathy. The infant had clinically diagnosed stridor for which bronchoscopy did not identify an origin. He died at 30 days of age. The bronchioles showed numerous eosinophilic plugs with a foreign body giant cell reaction. The plugs were positively immunostained with anti-aposurfactant protein B.

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.

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.