Nonimmune hydrops fetalis is becoming a predominant form of fetal hydrops due to the declining incidence of immune hydrops fetalis triggered by Rh isoimmunization. Infantile polycystic kidney appeared to be related to hydrops fetalis whether it is causal or merely coincidental and may represent another entry to differential diagnoses. Infantile polycystic kidney was diagnosed by an elevated maternal serum alpha-fetoprotein (AFP) value coupled with an ultrasonographic abnormality scanned as a multicystic mass with ascites in the fetal abdomen antenatally. This study presents a case of infantile polycystic kidney that resulted in a stillborn baby with hydrops fetalis and extensive placental calcification; it was the first case in Korea in which nonimmune hydrops fetalis was associated with infantile polycystic kidney in consecutive siblings by autosomal recessive inheritance in one family. In addition, this paper comprehensively reviews the incidence, etiology, prenatal diagnosis and proper management of nonimmune hydrops fetalis.
Adult ; Female ; Fetal Death/complications ; Fetal Diseases/*complications/pathology ; Human ; Hydrops Fetalis/*complications/pathology ; Pedigree ; Polycystic Kidney Diseases/*complications/pathology ; Pregnancy ; Recurrence

The causes of congenital hydrocephalus vary widely and have an important effect in determining the future counseling of affected cases. We analyzed the postmortem findings of 49 autopsies diagnosed as hydrocephalus to find the causes and related conditions. The cases were collected during the last 10 years (1981-1990). The cases consisted of 25 cases associated with various congenital malformations of the central nervous system (CNS), 14 cases of posthemorrhagic hydrocephalus, 4 cases of postinfection hydrocephalus, and 2 cases of hydranencephaly. Four cases were associated with supracerebellar arachnoid cyst (1) and unknown causes (3). Twenty-five cases associated with congenital malformation of the CNS consisted of 10 cases of holotelencephaly, 5 cases of stenosis of the Sylvian aqueduct, 4 cases of agenesis of the corpus callosum, and 3 cases each of Dandy-Walker malformation and Arnold-Chiari malformation. Various malformations of other organs were associated with these cases. Cardiovascular malformations were common, consisting of 11 cases of patent ductus arteriosus (PDA), 7 cases of atrial septal defect (ASD), and 6 cases of patent foramen ovale (PFO). Among the anomalies of the respiratory system, abnormal lobation was commonly associated, as well as hypoplasia of the lung. Gastrointestinal malformations included Meckel's diverticulum, diaphragmatic hernia, mobile intestine, and midline small liver. Cryptorchidism was the most common malformation in the genitourinary system. Holotelencephaly cases showed multiple craniofacial anomalies, as well as other malformations in the central nervous system. Skeletal malformations included polydactyly, simian crease, and flexion deformity. There were no specific constellations of malformations in these different groups of CNS malformations associated with hydrocephalus.
*Abnormalities, Multiple/pathology ; Autopsy ; Central Nervous System/abnormalities ; Cerebral Hemorrhage/complications ; Female ; Fetal Diseases ; Herpes Simplex/complications ; Humans ; Hydrocephalus/complications/embryology/*etiology/pathology ; Infant, Newborn ; Male

OBJECTIVETo investigate the clinicopathological characteristics, diagnostic criteria and differential diagnosis of placental chorioangioma.

METHODSTwenty-five cases of placental chorioangioma were analyzed for their clinical data, histomorphology and immumohisto chemical staining. Relevant literature was reviewed.

RESULTSThe average age of the 25 patients was 29 years. Fourteen patients had full-term pregnancy, 10 had preterm labor, and 1 had intrauterine fetal death. Nineteen patients had pregnancy complications. The tumors presented as red or dusty pink nodules with clear borders. The tumor size ranged from 1 to 16 cm. Microscopically, the tumors possessed abundant capillaries or cavernous blood spaces lined by hyperplastic endothelial cells. These cells were positive for CD34 and Ki-67 index < 10%.

CONCLUSIONSPlacental chorioangioma is a rare benign tumor of the placenta, and is associated with various pregnancy complications. Misdiagnosis of cell-rich type tumor should be avoided.

Adult ; Diagnosis, Differential ; Endothelial Cells ; pathology ; Female ; Fetal Death ; Hemangioma ; pathology ; Humans ; Infant, Newborn ; Placenta ; pathology ; Placenta Diseases ; pathology ; Pregnancy ; Pregnancy Complications, Neoplastic ; pathology ; Stillbirth

Holoprosencephaly (HPE) and polycystic kidney disease (PKD) are genetically heterogeneous anomalies which can make up part of various syndromes or chromosomal anomalies. Due to the rapid lethality prognosis, early and precise prenatal diagnosis would be of great value. This case report describes extensive PKD involvement, already present in utero, in a patient with HPE and subdural effusion visible by MR imaging. The detailed anatomic information obtained by the MR imaging can guide the surgical planning and can aid antenatal counseling.
Adult ; Female ; Fetal Death ; Holoprosencephaly/complications/*diagnosis/embryology/pathology ; Humans ; Magnetic Resonance Imaging/*methods ; Polycystic Kidney Diseases/complications/*diagnosis/embryology/pathology ; Pregnancy ; Prenatal Diagnosis/*methods

Intrahepatic cholestasis of pregnancy (ICP) is an unique complication in pregnancy, which usually manifests in the second or third trimester, and mainly harms the fetus. Its pathogenesis is not yet clear, and placental pathological changes are insufficient to explain the clinical phenomenon.Recent studies had shown that the important cause of perinatal deaths may be the damage to the placental structure and function caused by the high bile acid level. In addition, the change of placental structure and function, umbilical cord factors, and endocrine changes can also cause the fetal development and intrauterine hypoxia. In recent years related researches focus on the toxic effect of bile acid on fetus heart, lungs, brain, liver, and other important organs, the placental vascular pathology, hemodynamic changes, umbilical cord blood vessel factors and the endocrine changes.
Bile Acids and Salts ; metabolism ; Cholestasis, Intrahepatic ; metabolism ; pathology ; Female ; Fetal Diseases ; etiology ; metabolism ; Fetus ; metabolism ; Humans ; Maternal-Fetal Exchange ; Placenta ; pathology ; Pregnancy ; Pregnancy Complications ; metabolism ; pathology ; Umbilical Cord ; metabolism ; pathology

We report three autopsy cases of congenital cytomegalovirus (CMV) infection in fetuses with a review of literature. The clinical manifestations in these cases of congenital CMV infection include intrauterine fetal death, hydrops fetalis, and CMV pneumonia associated with cardiovascular defect. The pathological characteristics were as follows: 1) the kidney was the most frequently involved organ, followed by lung and liver, 2) CMV inclusions were found predominantly in epithelial cells and to a lesser degree in endothelial cells, 3) intrahepatic bile duct epithelial cells were frequently involved, and 4) inflammatory reaction around CMV inclusions was not prominent in the early stage of pregnancy. Diagnostic confirmation was obtained by in situ hybridization (ISH) using a biotinylated CMV-DNA probe, which demonstrated intranuclear inclusions and sometimes recognized cells that did not show intranuclear inclusion.
Autopsy ; Case Report ; Cytomegalovirus Infections/virology ; Cytomegalovirus Infections/pathology* ; Cytomegalovirus Infections/congenital* ; Female ; Fetal Diseases ; Human ; Male ; Pregnancy ; Pregnancy Complications, Infectious

We report three autopsy cases of congenital cytomegalovirus (CMV) infection in fetuses with a review of literature. The clinical manifestations in these cases of congenital CMV infection include intrauterine fetal death, hydrops fetalis, and CMV pneumonia associated with cardiovascular defect. The pathological characteristics were as follows: 1) the kidney was the most frequently involved organ, followed by lung and liver, 2) CMV inclusions were found predominantly in epithelial cells and to a lesser degree in endothelial cells, 3) intrahepatic bile duct epithelial cells were frequently involved, and 4) inflammatory reaction around CMV inclusions was not prominent in the early stage of pregnancy. Diagnostic confirmation was obtained by in situ hybridization (ISH) using a biotinylated CMV-DNA probe, which demonstrated intranuclear inclusions and sometimes recognized cells that did not show intranuclear inclusion.
Autopsy ; Case Report ; Cytomegalovirus Infections/virology ; Cytomegalovirus Infections/pathology* ; Cytomegalovirus Infections/congenital* ; Female ; Fetal Diseases ; Human ; Male ; Pregnancy ; Pregnancy Complications, Infectious

Epidermal Growth Factor ; physiology ; Fetal Diseases ; therapy ; Hernia, Diaphragmatic ; complications ; therapy ; Hernias, Diaphragmatic, Congenital ; Humans ; Lung ; abnormalities ; pathology ; Prenatal Diagnosis ; Pulmonary Surfactants ; pharmacology ; Tumor Necrosis Factor-alpha ; physiology

OBJECTIVE: To investigate the relationship between homocysteine (HCY) and congenital heart defects, and to observe the toxic effect of different doses of HCY on embryonic heart development in mammalian. METHODS: A total of 30 SD pregnant rats were randomly divided into 3 groups: a high dose group [200 mg/(kg.d)], a low dose group [(100 mg/(kg.d)] and a control group (equal volume of physiologic saline, n=10 in each group). The HCY or vehicle was given intraperitoneally from 7 to 20 days after uterineincision delivery. The contents of HCY in serum were analyzed by high performance liquid chromatogram electrochem before the pregnancy and 20 days after the pregnancy. The structure changes of the newborn rats heart were observed by stereoscope. The ultrastructure changes of cadiomyocyte were observed through transmission electron microscope. RESULTS: Comparing with the control, serum HCY in rats 20 days after pregnancy was significantly increased in the high or low dose group [(30.47 ± 1.12), (20.90 ± 1.08)vs(10.98 ± 0.77)μmol/L, P<0.01)], indicating that the hyperhomocysteinemia animal model was successfully established. The incidence rate of congenital heart defects in neonatal was significantly increased in the high or low dose group(14.13%, 9.57% vs 0.76%, P<0.01). The number of apoptotic cells were significantly increased in the high dose group. CONCLUSION Hyperhomocysteinemia may exert toxic effect on embryonic heart development in pregnancy rats, which led to congenital heart defects in the newborn rats. Hyperhomocysteinemia induced cardiomyocyte apoptosis may, at least partially, contribute to the heart defects.
Animals ; Apoptosis ; drug effects ; Female ; Fetal Diseases ; chemically induced ; Heart Defects, Congenital ; chemically induced ; pathology ; Hyperhomocysteinemia ; complications ; Myocytes, Cardiac ; pathology ; Pregnancy ; Pregnancy Complications ; Random Allocation ; Rats ; Rats, Sprague-Dawley

Acute atherosis is unique vascular changes of the placenta associated with poor placentation. It is characterized by subendothelial lipid-filled foam cells, fibrinoid necrosis of the arterial wall, perivascular lymphocytic infiltration, and it is histologically similar to early-stage atherosclerosis. Acute atherosis is rare in normal pregnancies, but is frequently observed in non- transformed spiral arteries in abnormal pregnancies, such as preeclampsia, small for gestational age (SGA), fetal death, spontaneous preterm labor and preterm premature rupture of membranes. In preeclampsia, spiral arteries fail to develop physiologic transformation and retain thick walls and a narrow lumen. Failure of physiologic transformation of spiral arteries is believed to be the main cause of uteroplacental ischemia, which can lead to the production of anti-angiogenic factors and induce endothelial dysfunction and eventually predispose the pregnancy to preeclampsia. Acute atherosis is more frequently observed in the spiral arteries of the decidua of the placenta (parietalis or basalis) than in the decidual or myometrial segments of the placental bed. The presence and deeper location of acute atherosis is associated with poorer pregnancy outcomes, more severe disease, earlier onset of preeclampsia, and a greater frequency of SGA neonates in patients with preeclampsia. Moreover, the idea that the presence of acute atherosis in the placenta may increase the risk of future cardiovascular disease in women with a history of preeclampsia is of growing concern. Therefore, placental examination is crucial for retrospective investigation of pregnancy complications and outcomes, and accurate placental pathology based on universal diagnostic criteria in patients with abnormal pregnancies is essential for clinicopathologic correlation.
Arteries* ; Atherosclerosis ; Cardiovascular Diseases ; Cholesterol ; Decidua ; Female ; Fetal Death ; Foam Cells ; Gestational Age ; Humans ; Infant, Newborn ; Ischemia ; Membranes ; Necrosis ; Obstetric Labor, Premature ; Pathology ; Placenta ; Placentation ; Pre-Eclampsia ; Pregnancy ; Pregnancy Complications ; Pregnancy Outcome ; Retrospective Studies ; Rupture