PRENATAL AND PERINATAL PATHOLOGY
SEPSIS
Neurohumoral changes during pregnancy can determine anomalies in embryonal development and pregnancy course. The following pathologies refer to the pregnancy period: early and late gestosis, extrauterine pregnancy, spontaneous abortion, preterm delivery, hydatidiform mole, chorioepithelioma, placental polyp, puerperal infection.
Pregnancy toxicoses (gestoses). Among the early gestoses the most frequently occur the following ones: sickness of pregnancy, excessive sickness of pregnancy, allergic reactions, hyperptyalism and others. Early gestoses occur on the 1st-3d month of pregnancy and are determined by excessive irritation of the nerve centers and cerebral cortex depression or jump of estrogen and progesterone concentration in the blood.
To the late gestoses are referred edema of pregnant, nephropathy, preeclampsia and eclampsia. They occur and declare themselves more frequently from the 32th-34th week of pregnancy. In the studies they are also called “EPH-gestosis” – edema, proteinuria, hypertension or preeclampsia.
Eclampsia is one of the pregnancy toxicoses which develops in the second half of pregnancy, childbirth and puerperal period. Clinically eclampsia is determined by the renal and liver insufficiency, major epilepsy with syncope.
Etiology and pathogenesis. Autointoxication with products which are secreted by fetal tissues and excrements is considered to be the cause of eclampsia. Eclampsia appears on the background of renal insufficiency, endocrine balance disturbance (hypophysis hyperfunctioning, adrenal and in-thyroid glands insufficiency). Allergic eclampsia theory is worth mentioning according to that the pregnant female’s organism is sensitized by the fetus and excremental albuminous products.
Anatomical pathology. At the time of the partition jaundice, edemas, full-blown changes in liver and kidneys.
Liver is enlarged, striped in looks – on the yellow background (fatty degeneration) there are numerous flat subcapsular hemorrhages. The surface of the incision is pale, clayey, with numerous hemorrhages. With the help of the microscope hemorrhages, necroses in the peripheric sites of particles, fibrinogenous small venous thrombosis, albuminous and fatty degeneration of the hepatocytes.
Kidneys are enlarged, slack, crust layer is pale, gummy, a little thickened, cerebral one is sharply plethoric.
With the help of histology dystrophy and tubule epithelium necrosis, excrement cells embolism of the vas capillares glomerulares, fibrinoid necrosis of the capillary walls, stroma hemorrhages, sometimes a picture of mesangium glomerulonephritis with laying the immune complexes on the basic membrane and mesangium cells proliferation are detected. In the serious cases necrotic nephrosis with acute renal insufficiency develops.
Numerous hemorrhages combined with small venous thrombosis as well as necrotic and dystrophic changes apart from liver and kidneys are detected in the celebral, lungs, heart tissue, serous membranes. In the placenta changes are found which are the consequence of its ischemia: intensive deciduocellular nodi formation, thickening of the basic trophoblast membrane, cytotrophoblast hyperplasia, infarctions. Premature placenta exfoliation is often observed. Infants are born premature with the features of hypoxia and intrauterine fetal hypotrophy, sometimes intrauterine fetal death occurs. Parturient women die of liver-renal insufficiency and hemorrhages to the vital organs.
Extrauterine pregnancy is characterized by the fetus development outside the uterine cavity – in the tube (tubal pregnancy), in the ovary (ovarian pregnancy) or in the abdominal cavity (abdominal pregnancy).
Extrauterine pregnancy.
Development of the extrauterine pregnancy is determined by abnormality of the uterine tubes permeability (chronic inflammation, congenital luminal narrowing, tumors) which make fertilized ovum translocation from ampullar tube part to the uterine cavity difficult.
Tubal pregnancy can be ampullar which develops in the abdominal tube part, interstitial – in the part of the tube located in the uterus wall depth, and isthmic – in the place of the anatomic tube constriction.
Tubal pregnancy
If the tube breaks along the lower rib in the consequence of the fetal egg growth then the latter falls between the uterine ligament folia and interligament pregnancy develops.
Tubal pregnancy
During the tubal pregnancy in the mucous tube, on the place of the fetal egg attachment and in the endometrium decidual reaction appears – appearance of big cells rich in glycogen. The fetus is attached to the endometrium with chorion. As the wall of the tube is thin, the choria grow through the endometrium, muscular layer reaches the serous membrane. The wall becomes friable and the fetus is torn away (2nd-3d pregnancy months) – it is called maldeveloped tubal pregnancy. The tube rapture is accompanied with the hemorrhage into abdominal cavity which can result in the woman’s death. Sometimes a thrombus tampons the rapture hole (masked rapture); if the latter drops out, recurring hemorrhages are possible.
If the torn-away egg is left in the tube lumen – incomplete tubal abortion. In the cases it dies and its membranes are impregnated with blood – it’s a blood mole, and if the fetus falls into the abdominal cavity through the ampullar tube part, a complete tubal abortion comes.
Tubal pregnancy
Reimplantation is possible in this case, the development of the secondary abdominal pregnancy. The fetus dies more often, embalm (papyraceous fetus) and limes (lithopedion), or resolves.
Tubal pregnancy
When the histologic study of the tube extracted by means of the operation, pregnancy features are displayed – chorionic villi, decidual cells. Decidual reaction in the endometrium takes place, the uterus enlarges a little.
Miscarriage (spontaneous abortion) is a spontaneous fetus wastage and fetus excretion out of the endometrium before the 28th week from the conception moment. Abortion before 14th week is considered early, from 14th to 28th weeks – the late one. Miscarriage between the 28th and the 38th week is called preterm delivery. At the time of the fetus wastage the whole fetal egg (fetus and membranes) is excreted out of the endometrium. The latter can be intact or torn. During the preterm delivery the fetus is born first and then the membranes and placenta (afterbirth). Histologically chorionic villi, decidual cells and fetuc membranes are detected among the grumes.
The abortion very often comes after the fetus has died as a result of incomplete immersion of the fetus egg into the endometrium as a result of its incompetence. The latter was mainly determined by the atrophy resulted from previous abortions, inflammation. Separation and extrusion of the fetus egg is often caused by the early fetal death when the mother has different diseases (syphilis, serious infections, intoxications, avitaminoses). Miscarriage also develops along with congenital maldevelopment which are incompatible with life. It remains a mystery how the mother’s organism detects the deformities of the embryo.
According to the data of the embryologists Svyetlov P.G., Dyban A.P., the fetal death more frequently comes during a certain period of gestation. For the human embryo such periods of special sensitivity to the pathogenic agents are implantation which coincide with the 15th day of gestation and placentation – every 3th-6th week. These periods of the most fetus sensitivity to the influence of the disturbing agents are called the first and the second critical periods. But the embryo death in most cases doesn’t come right after the damage but after some time for the first critical period – the 4th week of gestation, for the second one – the 8th-11th week of gestation.
Artificial abortion is carried out according to the medical indication or the undesirable pregnancy. If such abortion is either carried out outside the medical establishments in insanitation which can lead to sepsis and criminal investigation or is not registered as surgical operation and it is called criminal. It is proved that embryo is very troubled before the artificial abortion, his heart beating speeding up; it contracts as if trying to become less, unnoticed, hides in the most remote corner. He reacts to the abortion as to death which is coming closer. The abortion complications are: sterility, hemorrhages, sepsis.
Trophoblastic tumor includes hydatidiform mole, invasive hydatidiform mole, chorioncarcinoma, trophoblastoma of the placental site. The source of the disease is placental tissues. It is more often found among the pregnant in the age before 16 or after 35 years.
Hydatidiform mole (mola hydatiosa) is a hydropic and cystic degeneration of the placental chorionic villi during gestation.
One form of gestational trophoblastic disease, a molar pregnancy, results from abnormal fertilization of the ovum. A complete hydatidiform mole, shown here, occurs when the ovum is lacking a maternal complement of chromosomes and is fertilized by a haploid sperm, usually containing an X chromosome. Duplication of this chromosome set typically yields a 46, XX karyotype that is paternally derived. No fetus develops, but there is an abnormal placenta consisting of a mass of tissue with grape-like, swollen chorionic villi. [http://library.med.utah.edu]
The pathologist determines the histologic diagnosis of the trophoblastic disease; most importantly, if choriocarcinoma is present. In this complete hydatidiform mole shown here there is atypical trophoblastic proliferation, but chorionic villi are still present. The patient is then followed with serial serum HCG levels after evacuation of the mole to make sure there is no residual trophoblastic disease. A small number of complete moles are followed by development of invasive mole or a choriocarcinoma. [http://library.med.utah.edu]
The number of villi increases they become large in the shape of moles filled with transparent liquid and resemble a bunch of grapes. The disease manifests itself through vaginal hemorrhages, sometimes with elimination of the hydatidiform villi during the first term. The uterus is enlarged and extremely high level of chorionic gonadotrophin is displayed. The fetus dies. The trophoblast proliferates, lytic activity rises which leads to the growing of the villi into the deep layers of the uterus (moimetrium), sometimes to the serous membrane (chorioadenoma destruens). In such cases urinal hemorrhages are observed a few weeks after the ablation of hydatidiform mole.
This is a partial hydatidiform mole. Note that there are only scattered grape-like villi. Such a partial mole occurs when an ovum is fertilized by two haploid sets of paternal chromosomes. This may occur with dispermy when two sperms fertilize a single ovum, or when a diploid sperm fertilizes an ovum, or if a haploid sperm fertilizes a diploid ovum. The result is triploidy with 69 chromosomes. Since a maternal set of chromosomes is present, a fetus develops, but it is malformed, and the pregnancy rarely goes to term. Only some of the villi are grape-like. Choriocarcinoma is a rare outcome of a partial mole. [http://library.med.utah.edu]
Along with it lung, vagina metastases are found which can disappear especially after the chemotherapy course. If the villi have grown into the veins, tissue placental pulmonary embolism occurs. Hydatidiform mole can be complicated with chorioepithelioma. The cause of hydatidiform mole is unknown, it is often connected with follicular ovary cyst and perhaps appears on the background of the harmonic dysfunction of the rest. Cystic transformation of the placental villi with formation of hydatidiform mole can be also determined by domination of the father’s chromosomes in the embryonic karyotype.
In partial moles, some villi appear normal, whereas others are swollen, avascular, and grape-like (though not as large as a complete mole). There is minimal trophoblastic proliferation. In fact, most placentas in cases of triploidy do not have grossly recognizable grape-like villi. [http://library.med.utah.edu]
Chorionepithelioma (chorioncarcinoma) is a malignant tumor from the trophoblast epithelium. It develops of the remnants of placenta after abortions (25%), deliveries complicated by the hydatidiform mole (50%), clinically normal delivery (22%), ectopic pregnancy, especially with chorioadenoma destruens (invasive mole). Chorioncarcinoma can develop in the lungs as a result of placental embolism, in the ovary with teratogen, urinary bladder, partial septum of testis, testicles. Typical clynical symptom is the appearance of the metrorrhagias. The tumor is hormonally active, extremely malignant, accompanied with the uterus enlargement with evident decidual reaction in the endometrium.
Some time ago this tumor was called deciduoma, it was considered to originate from decidual tissue of the gravid uterus. In 1886 Nikiforov M.N. and Marshan proved that it develops of the chorionic villi epithelium. It looks like a variegated fluffy nodus in the myometrium, the vessels in the form of cavities. There are no stroma and own vessels. It feeds from blood which flows out the tissues, destroyed by it. It is of a dark-brown colour due to hematogenous pigments. It consists of the cyto- and syncytiotrophoblast cells – light epithelium Langhans cells, among which there are many gigantic cells with numerous mitoses; polymorphous dark syncytium cells are located at the periphery. It gives metastases to the lungs in the early period.
Placental pathology is classified due to localization and character of the pathologic process. Pathologic process caestle on the basic membrane (deciduas basalis), intervillous lacuna, fetus part of placenta (villi, chorionic plate), umbilical cord, outplacental fetus membranes. Inflammatory processes and blood-circulation disorders are the most often found in the placenta. Disturbance of the villiferous tree are often found which lead to the placental hypoplasia, insufficient vascularization of the villi.
Infectional processes in the placenta appear as a result of penetration of the microorganisms (viruses, bacteria, protozoas, etc.) into the placenta. They distinguish:
o ascending way of infectioning – through the uterus and the cervix uteri which takes place along with early moving of waters and long-lasting anhydrous period;
o hematogenic from the maternal blood-circulation;
o descending through the uterus tubes.
Premature and/or prolonged rupture of fetal membranes (PROM) increases the risk for intrauterine infection, because bacteria can pass into the normally sealed amniotic cavity. This leads to acute inflammation as seen here as an acute chorioamnionitis within the fetal membranes. The fetus may become infected and suffer intrauterine fetal demise. Also, the inflammation may lead to premature labor and premature birth. [http://library.med.utah.edu]
The inflammation caestle in the decidual membranes, in the villi, in the intervillous lacuna, chorionic and amniotic membranes, in the umbilical cord. Depending on the causative agent inflammatory cellular infiltration is presented by leukocytes, lymphocytes, plasma and gigantic cells, histiocytes, etc. Inflammatory processes in placenta can cause fetus, uterus infection, preterm delivery, anomalies of the following pregnancies.
Placental villitis is shown here with a small microabscess containing mostly neutrophils in a case of congenital infection with Listeria monocytogenes. Listeriosis is generally not life-threatening to the mother, but is potentially a cause for fetal demise. [http://library.med.utah.edu]
Placental villitis at the bottom is seen in conjunction with hydropic change at the top in this placenta with congenital cytomegalovirus (CMV) infection. Both of these microscopic changes can occur together. Congenital infection is a common cause for hydrops fetalis. [http://library.med.utah.edu]
Placental villitis seen here is accompanied by a couple of enlarged cells with mauve intranuclear inclusions typical for congenital cytomegalovirus infection. Such inclusions may be difficult to find, and the inflammation can be focal, with lymphocytes, plasma cells, and occasional neutrophils. [http://library.med.utah.edu]
Placental blastodisk anomalies manifest themselves through the change of form, appearance of the elevation or limbus which surround placenta. In such cases hemorrhages in placenta, preterm delivery or stillbirth are observed. According to the changes in the localization of the placenta attachment the following variants of anomalies are distinguished: marginal or central placental presentation relative to the cervix uteri internal fauces. Such anomalies can cause hemorrhages and lead to the fetal and mother’s death. Anomalies of exfoliation manifest themselves through adherences or early exfoliations which lead to the metrorrhagias.
Blood-circulation disorders in placenta manifest themselves through diffuse ischemia, diffuse hyperemia, hemorrhages, edema, perivilliferous fibrin deposition, thromboses, infarctions.
Here is a placental infarct. A small infarct may be of no consequence to the fetus, but if more than a third or half of the placental parenchyma is infarcted or lost in some fashion, then the blood supply to the fetus can become severely compromised and fetal demise may occur. [http://library.med.utah.edu]
Here is a placenta cut in cross sections to reveal pale infarction involving over half of the parenchyma. This was so extensive that fetal demise resulted from the infarction. [http://library.med.utah.edu]
Microscopically, this placental infarct demonstrates pale, necrotic chorionic villi. [http://library.med.utah.edu]
The cross section of the placenta seen here is quite abnormal, with a pale tan appearance. This represents the consequence of diffuse fibrin deposition, also associated with a condition known as maternal floor infarction, and sometimes called a “gitterinfarkt”. [http://library.med.utah.edu]
Diffuse ischemia of placenta is observed with hemolytic anemia, posthemorrhagic conditions, intrauterine fetal death. Placental ischemia can cause anemia as well as the fetal death. Diffuse hyperemia of placenta takes place accompanied with mother’s hypoxic conditions, blood outflow derangements through the umbilical vein as a result of nodi formation in the umbilical cord. Hemorrhages from the placenta occur with early exfoliation, placental presentation. Placental edema develops At the time of hemolytic disease, nephropathies, infectious diseases. Thromboses develop during gestoses and cause infarctions formation. Perivilliferous fibrin deposition is observed at the periphery of placenta in the form of close daffodil bonfires with fibrosis and vessels obliteration.
The decidua is at the left, from which extends into the villi extensive deposits of fibrin, and the villi are becoming infarcted. This is known as maternal floor infarction. This condition can cause uteroplacental insufficiency and fetal demise. It is uncommon, but this condition has a tendency to recur in subsequent pregnancies. [http://library.med.utah.edu]
Umbilical cord pathology manifests itself through the change of length (a short one – less than
The amniotic cavity has been opened here to reveal the normal fetal surface of the placenta at the upper right. The umbilical cord inserts centrally into the placental disk. The abnormal finding here is a “nuchal cord” in which one or more loops of umbilical cord are wrapped around the baby’s neck. [http://library.med.utah.edu]
Here is a cross section of an umbilical cord in which there is only a single artery and a vein: a “two vessel” umbilical cord. Such cords occur in about 1% of births. A two vessel cord has signficance because there is a greater likelihood that other congenital anomalies will be present in such cases. [http://library.med.utah.edu]
Seen above and below are examples of “velamentous” insertion of the umbilical cord in which the major umbilical vessels separate in the fetal membranes before reaching the placental disk. Such a condition is of no major consequence in utero, but could lead to a greater chance for cord trauma with bleeding during delivery. Dividing membranes are see at the left in the twin placenta below. [http://library.med.utah.edu]
This is a true knot of the umbilical cord. Such knots are more likely to occur with abnormally long umbilical cords that may develop with increased fetal movement. Such a knot could constrict the blood vessels and lead to fetal demise. [http://library.med.utah.edu]
This is another true umbilical cord knot. [http://library.med.utah.edu]
The fetus at the left is macerated from prolonged demise in utero. The cause of the demise in this case is the marked twisting, or torsion, of the umbilical cord. A macerated placenta is present at the right. [http://library.med.utah.edu]
This umbilical cord shows marked spiralling, or torsion. This can potentially compromise blood flow to fetus. [http://library.med.utah.edu]
Anomalies of the amnion development manifest themselves through enlargement (over
The twins’ placentas distinguish depending on the kind of ovum fertilization: dizygotic twins have a dichorionic diamniotic placenta, monoovular twins have a monochorionic placenta.
The fetal surface of a normal term twin placenta is seen here. The dividing membranes between the amniotic cavities occupied by the two fetuses are seen between the cord insertions. [http://library.med.utah.edu]
This is a section through a dividing membrane of a twin placenta to reveal a thin cuboidal layer of amnion on each side. In the center are two chorions plastered together. Thus, this is a “diamnionic-dichorionic” twin placenta (di-di placenta), which makes the likelihood of dizygous twinning much more probable than monozygous twinning. [http://library.med.utah.edu]
This is another twin placenta that has a normal discoid shape with dividing membranes that separate the fetal amnionic cavities into equal halves. It is not possible grossly to determine whether this is monochorionic or dichorionic. [http://library.med.utah.edu]
Two strips of dividing membrane are seen here from a twin placenta. In this case, the dividing membranes have an amnion on each surface, but no visible chorion, so this is a “diamnionic-monochorionic” twin placenta (di-mono placenta). Monochorionic placentas imply that monozygous twinning is present. [http://library.med.utah.edu]
Only a single amnionic cavity is present in this twin placenta. Note that the two umbilical cords join and share circulation. Monoamnionic twins have more potential problems. In this case, one twin was an “asymmetric” acardiac twin supported by the heart of the remaining complete twin. [http://library.med.utah.edu]
The placental blood vessels have been injected with a white fluid to reveal the anastomosis across the dividing membranes of a monochorionic twin placenta in a case of twin-twin transfusion syndrome. In general, this syndrome can be suspected when one twin is at least 25% larger than the other. [http://library.med.utah.edu]
Anastomoses between the twins’ vessels is formed in the placenta. In case of unilateral direction of such anastomoses placental transfusion syndrome develops: one of the twins becomes a donor, another – a recipient. With this syndrome a twin-donor death rate is rather high.
Placental polyp develops in the endometrium from the remnants of placenta pieces after deliveries or abortions. Histologically it is made of villi, decidual tissue, fibrin clots which become organized. In the place where the polyp is attached connective tissue site is formed. Placental polyp slows down postnatal involution of uterus, contributes to development of endometritis, is accompanied by metrorrhagias.
Afterbirth infection of the uterus is the most often determined with streptococcosis, staphylococcosis, colon bacillus. Purulent endometritis (endometritis pyrylenta) occurs. Endometritis can develop before delivery (endometritis sub partum), during delivery (endometritis intra partum), and after delivery (endometritis post partum). Afterbirth infection more frequently occurs exogenously (nonobservance of the aseptics rules) or endogenously (antenatal endometritis). Endometritis very often causes uterine sepsis. Septical endometritis is of purulent, diphtheritic or suppurative character; endometrium surface is covered with taupe incrustation. Lymphangitis, phlebitis, thrombophlebitis develop. Metritis, perimetritis, pelvic peritonitis often develop.
PRENATAL PATHOLOGY
Prenantal (antenatal) pathology includes pathologic processes of the human embryo, beginning with fertilization and ending with delivery. Prenatal period lasts 280 days, or 40 weeks. The whole development from fertilization to delivery is called kinetogenesis which is preceded by progenesis – period of male and female sex cells (gametes) ripening before the fertilization. Kinetogenesis period is divided into three periods – blastogenesis, lasts from fertilization to the 15th day of pregnancy, when the fertilized ovum division takes place and it ends up with embryo- and trophoblast elimination; embryogenesis – from the 16th to the 75th day of pregnancy when the main organogenesis takes place amnion and chorion are formed; fetogenesis – lasts from the 76th to the 280th day of pregnancy when differentiation and ripening of the fetal tissue take place, placenta is formed, ends up with delivery. Fetogenesis period can be divided into early fetal (from the 76th to the 180th day), at the end of this period fetus becomes viable, and late fetal (from the 181st to the 280th day), when the fetus becomes mature. Pathology which occurs during the kinetogenesis period is called kinetopathy and it is correspondingly divided into blastosis, embryopathy, early and late fetopathy.
The reasons for kinetopathy according to the latest data: 20% deformities (main kinetogenesis period pathology) are connected with gene mutations, 10% – with chromosome aberration, 10% – with the exogenous factors influence, 60% – of ambiguous etiology. German measles, rubeola, chickenpox, mononucleosis, parotitis, hepatitis, influenza, poliomyelitis, pale treponema, toxoplasmosis, tuberculosis microbacterium belong to the exogenous factors.
Apart from infection agents kinetopathies can be caused by radiation energy, some pharmaceutical preparations (tolidomide, cytostatic drugs), hormones, vitamins, alcohol, drugs, hypoxia.
Gametopathies. During protogenesis pathology of gametes may occur – gametopathies. They are manifested through nuclear substance and sex cells cytoplasm pathology. Nucleus changes are characterized by hereditary apparatus of gamete pathology. Gene, chromosome and genomic mutations are distinguished that are the cause of congenital maldevelopment (deformity). Deformities are not viable and end up with spontaneous abortion. Gamete cytoplasm pathology as a rule results in sterility (infertility).
Blastopathies. They are the most frequently caused by chromosome aberrations accompanied with environmental influence (mother’s endocrine diseases, hypoxia, intoxications, etc.). To blastopathy belong: blastocyte implantation disturbance (extrauterine pregnancy), twin deformities, solitary deformities, placenta and umbilical cord formation deformities. Twin deformities are connected with appearance of two or more independent growing centres during division. If centres of growth are in close location and have common intermediate zone, than two conjoined twins develop. If the conjoined twins are identical, symmetrically developed, they are called diplopagus (from Gr. diplos – double, pagus – to connect), if the twins are asymmetrically developed, it is heteropagus. The twin lesser in size is called teratic parasite. Sometimes such twin is found in the body of the bigger one – “fetus in fetu”. In
The degree of twins conjugation can be different – from minor conjoined superficial tissues to such degree when only heads and limbs are separated. To determine the localization of twins conjugation a word pagus was added to the anatomic name of the site of conjugation – craniopagus, thoracopagus, ischiopagus, etc.
Heteropagus
Heteropagus
Diplopagus. This is an uncommon complication of monozygous twinning in which there is fusion of the twins. The popular term is “siamese” twins. The scientific term applicable in the case shown here is craniothoracopagus, or twins joined at the head and chest. There is only one brain, and the hearts and gastrointestinal tracts are fused as well. The location and amount of fusion can vary. [http://library.med.utah.edu]
Diplopagus.
This is an example of thoracopagus. Note the large omphalocele in the lower abdomen shared by these monozygous twins. These twins shared a heart and liver and several other organs were partially fused. Attempts at separation of such twins can either be viewed as heroic new technology or expensive futility. The survival rate with significant fusion of organs is essentially nil. [http://library.med.utah.edu]
Diplopagus. Chang and Eng Bunker.
This is another abnormality of twinning in which one fetus is essentially a poorly formed blob attached to, or separate from, a complete fetus. This condition is called acardius-acephalus because there is typically no heart or brain in the “blob” twin. The photograph here reveals an acardiac twin that consists mainly of just lower extremities. Such a non-viable twin can be surgically removed. [http://library.med.utah.edu]
This is a more complete acardiac twin in which actual body regions are present. There were few poorly formed internal organs present. Nonetheless, it is non-viable. [http://library.med.utah.edu]
Embryopathy is an embryonic period pathology from the 16th to the 75th day of pregnancy, during which the main organogenesis is completed. To the embryopathies belong mostly congenital maldevelopment – deformities. With the embryo’s development the ability to react to different pathogenic influences with disturbance of morphogenesis is gradually developed. This ability is called dysontogenesis. It was found out that different teratogenic agents can cause the same deformity. Along with it the same teratogenic agent can cause different deformities of development influencing during different periods of embryogenesis. There is a certain period of time for each organ during which under the influence of a teratogenic agent hypoplasia of this organ occurs. This period of time is called teratogenic termination period (from Lat. teratos – deformity and terminus – boundary). So, it is a certain period of time during which anlage and formation of different organs is performed and the influence of teratogenic factors in this period causes the disturbance of this process which results in deformities. Morphogenesis of different organs is carried out during different periods of embryogenesis and during this time the organs are the most susceptible to the teratogenic agent effect. Teratogenic agents are the viruses – bacteria, toxins, alcohol, medicine, hormones, vitamins, penetrating radiation, etc.
Congenital maldevelopment is persistent morphologic changes of the organs which appeared as a result of the region’s or organism’s morphogenesis disturbance and they are beyond the measures of normal variations. To the congenital maldevelopment belong:
1) absence of any organ or region – agenesia, aplasia;
2) underdevelopment of an organ – hypoplasia;
3) excessive development – hyperplasia;
4) change in forms: conjugated organs, arthrodesia or stenosis of apertures or canals, nonclosure of embryonic fissures – persistence, eversion – ectropion;
5) change in organs’ location – ectopia;
6) persistence of embryonic (provisional) organs, more frequently of branchial arches or their remnants.
Apart from the pathology of organs their can be congenital maldevelopment with disturbance in differentiation of separate tissues of:
o skeletal muscles – congenital Oppenheim’s myopathy;
o connective tissue – Marphan’s disease;
o skin – ichthyosis congenita;
o bones of cartilage genesis – congenital chondrodysplasia.
Congenital maldevelopment can be simple – when one organ is involved, complicated – a few organs of one system and numerous – organs of few systems.
This is anencephaly. This condition occurs when there is failure of formation of the fetal cranial vault. The brain cannot form properly when exposed to amniotic fluid.
The first thing to notice is the features of intrauterine fetal demise: skin slippage and reddening. These features are those of maceration and not trauma or birth defect. Note the shortened lower extremities, known as phocomelia. It was idiopathic in this case, but in the 1950’s the drug thalidomide was responsible for many cases when pregnant women took it. Thus, it is very important that pregnant mothers be advised that drugs (including cigarette smoking and alcohol consumption) may have profound effects on the fetus. [http://library.med.utah.edu]
Amelia
The most serious consequence of oligohydramnios is pulmonary hypoplasia. Note the extremely small lungs in this case on each side of the heart in the middle of the chest. The chest cavity is opened here at autopsy and the lung appear small in comparison to chest size, but in utero the constriction from diminished amnionic fluid would have decreased the chest cavity size. [http://library.med.utah.edu]
Horseshoe kidney
The skull is opened here to reveal the “semilobar” form of holoprosencephaly, because there is a small cleft representing an attempt to separate the hemispheres. There is no gyral pattern here because this stillborn fetus was under 20 weeks gestation. Holoprosencephaly is a grave condition with little or no brain function. Holoprosencephaly can be associated with chromosomal anomalies (such as trisomy 13), with maternal diabetes mellitus, and can be seen sporadically. . [http://library.med.utah.edu]
Here is an example of syndactyly in which the 3rd and 4th fingers are fused into one large digit. This particular pattern can be seen with triploidy (69 chromosomes).
Here is a ventral abdominal wall defect. This defect involves the region of the umbilical cord, so this is an omphalocele. Note that there is a thin membrane covering the herniated abdominal contents (loops of bowel can be seen under the membrane). This defect would have to be repaired over a period of time. Since the bowel has mainly developed outside of the abdominal cavity, it is malrotated and the cavity is not properly formed (too small).
Spina bifida
The meconium-filled bowel ends in a blind pouch. This is atresia of the bowel. Such a defect, like many anomalies, often happens along with other anomalies. Bowel atresias are accompanied by polyhydramnios, since the swallowing and absorption of amniotic fluid by the fetus is impaired. [http://library.med.utah.edu]
Accessory spleen
Fetopathies are pathologies of the fetal period, from the 76th to the 280th day of pregnancy, during which the basic tissue differentiation of the organs is carried out. Two types of manifestations are typical: blood-circulation disorders, dystrophy and necroses, mutated immune reactions and compensatory and time-serving processes. Disturbances of tissue morphogenesis are typical of early fetopathies, reactive reactions – of the late ones. Fetus infection takes place in ascending way through genital organs and placenta and in a descending hematogenic way, mainly with either salpingitis or ovaritis. Morphologically infectional fetopathies manifest through generalization of the inflammatory process with numerous foci of reactive necroses, granuloma formation, hemorrhagic syndrome resulted from vasculitis, hemolytic jaundice, retention of foci of extramedullar hematosis, accidental involution (athrophy) of the retrosternal gland (thymus), general hypothrophy, prematurity. As a rule, such infants die during their first months of life. If they survive, persistent changes in the organs remain that cause disability.
Noninfectious fetopathies can be early or late. To the early ones belong: hypertrophic pylorostenosis, megacolon, megaurethra, agenesis, hypoplasia or hyperplasia of bile ductules, polycystic lung disease, polycystic renal disease, etc., to the late ones – hemolytic disease of the infants, fetal mucoviscidosis, endocardial fibroelastosis, diabetic and alcoholic fetopathy, etc.
Diabetic fetopathy
Alcoholic fetopathy is characterized by small fetal weight, microcranium, microgyria, polygyria. Sizes of head and brain are diminished, gyri are narrow, numerous, sulci are deep. These changes are often combined with other congenital maldevelopment – maldevelopment of brain, cardiovascular system, and urinary system. Such children are slow in their mental and physical development.
Fetal alcohol syndrome
PATHOLOGIES OF THE INFANTS
Perinatal period (period “around delivery”) lasts from the 154th day of the intrauterine life of fetus (22 weeks of pregnancy) to the first week of the extrauterine independent life. Infant is a neonate which has begun to breathe by itself. Stillborn is a fetus which doesn’t breathe at the moment of birth and it could not be stimulated artificially although the heart beating can be observed during some time. Stillbirth and death of infants during the first seven days after delivery are called perinatal mortality. Perinatal period and corresponding pathology and mortality can be divided into antenatal (prenatal), intranatal (during the delivery), postnatal (postpartum) or neonatal.
Features of prematurity: gestation duration is less than 38 weeks, the weight of fetus is less than
Features of postmaturity: gestation duration is over 41 weeks, dryness, desquamation and partial maceration of skin, general hypothrophy, anemia, water, umbilical cord and membranes are imbued with meconium into bottle green because of hypoxia.
To the pathology of infants belong asphyxia, pneumopathies, birth trauma, hemorrhagic and hemolytic disease of infants.
Birth trauma is a mechanic injury of tissues and organs of the fetus during delivery. The causes which determine it are divided into three groups. The first group is those laid in the condition of fetus itself: fragility of tissues at prematurity or postmaturity, congenital maldevelopments which are accompanied by venous hyperemia, hemorrhagic syndrome, fetopathies, hypoxia. The second group is determined by pathologies in the mother’s maternal passages: rigidity of the maternal passages tissues, inclination of pelvis, contracted pelvis, tumors, olygoamnios, early pouring-out of the waters. The third group is those laid in the course of delivery – accelerated and prolonged labor.
Morphology of the birth traumas.
Cephalic tumor appears in the part of head that adjoins the pelvic outlet. It is determined by disorders of blood-circulation and lymphokinesis. The tissues of the latter become dropsy, swollen, can suppurate.
Cephalohematoma is a hemorrhage under the cranial bones, it is always restricted to the one bone site. External hematoma is the most frequently found. It resolves slowly, undergoes organization and petrification. If there is a purulence, meningitis can develop.
Hemorrhage into the meninges and brain. Epidural, subarachnoid and intracerebral hemorrhages are distinguished. Epidural hemorrhages (internal cephalohematoma) are always massive. They can take place when there are traumas of cranial bones and dura mater of brain. Subdural hemorrhages most frequently occur along with laceration of tentorium of cerebellum, of crescent, they are as a rule massive and located on the surface of celebrum. Subarachnoid hemorrhages are mostly determined by rupture of small veins. Unlike asphyctic, traumatic subarachnoid hemorrhages are always massive.
Birth trauma
Intracerbral hemorrhages are caused by rupture of terminal veins, can lead to development of hematomas. Intraventricular hemorrhages are most frequently observed among the premature infants.
Spinal cord trauma is a result of injury of the spine mostly on the level of the IV cervical vertebra and is accompanied by development of descending subdural hemorrhages.
Among the skeleton bones clavicle is most frequently injured (fracture of clavicle). Paralyses of arms and diaphragm of the infants are determined by trauma of root of cervical plexus and brachial plexus. Rapture and hemorrhage into the nodding muscle results in torticollis. Among the internals liver and adrenal glands are most frequently injured.
Hemolytic disease of the infants develops with blood incompatibility between the mother and the fetus (mother is Rh-negative and child is Rh-positive). From the mother’s blood anti-Rh antibodies penetrate to the child’s blood and attack red blood cells. It develops during the second and the following gestations because immunization of the network (antibody titer) grows with gestation. Thee main types of disease are distinguished – edematous, anemic and jaundice diseases. Manifestation of their certain forms depends on the period and amount of penetration of the mother’s antibodies into the blood of fetus.
When early massive penetration of the antibodies takes place, in some cases early fetopathy develops and antenatal death of the 5-7-month-old fetus, in the others – chronic fetopathy in the form of heavy edematous form of the hemolytic disease with maldevelopment of the tissue ripening. Pathologoanatomic changes with the intrauterine fetal death manifest through maceration and autolysis. Maceration (from the lat. maceratio – maceration) is softening of the tissues by the water. Along with it edema of face and peeling of the epidermic tissue in big layers. Autolysis (from the gr. autolis – by oneself, lisis – dissolution) is an autodigestion, disintegration of tissues of the organism which takes place under aseptic conditions and effect of their own enzymes. Organs and tissues disintefrate till the formation of the uniform mass of the murrey colour. If it is chronic edematous form, the skin of the infant is pale, half-transparent, glossy, partly macerated, with solitary petechial hemorrhages. Hypodermic cellulose, cerebral tissue and cerebral membranes are sharply dropsical, in the body cavity is transsudate (hydrops factus universalis). Liver and spleen are greatly enlarged, retrosternal gland is atrophied. The heart is enlarged due to myocardium hyperplasia, lungs are diminished. With the help of the microscope foci of extramedullar hematosis with the dominance of erythroblasts (erythroblastosis) in the liver, spleen, lymph nodi, kidneys and petechial hemorrhages, dystrophic and necrobiotic changes in the internals.
During the later and moderate penetration of the mother’s antibodies into the blood of fetus anemic form of the hemolytic disease of the infants develops, it is more frequent observed with the premature infants. Paleness, slight pitting edemas are observed. There is no icteritiousness. The internals are anemic. Liver and spleen are slightly enlarged, they contain microscopic displays of marked erythroblastosis.
Icteritous form seldom develops itrauterinally, because placenta is able to remove bilirubin from the organism of fetus. During the massive penetration of the antibodies at delivery time a heavy postnatal icterous form of the hemolytic disease of the infants develops (icterus neonatorum gravis). Jaundice appears at the end of the first or on the second day after delivery and grows quickly. Penetration of the indirect toxic bilirubin into the brain causes the damage of ganglionic cells till their very necrosis – bilirubinic encephalopathy.
Bilirubinic encephalopathy.
The changes develop mostly in the subcortical sections – hypostones, nuclei of the bottom of the diamond-shaped fossa, inferior olives, pale nucleus and nuclei of the cerebellum. Hypostones, nuclei of the bottom of the diamond-shaped fossa, inferior olives, pale nucleus and nuclei of the cerebellum are intensively coloured yellow – nuclear icterus. Erythroblastosis, hemosiderosis, biliarystases and thrombi, sometimes even gallstones; in the kidneys – bilirubinic infarctions are observed in the liver. Spleen is enlarged, dense. Microscopically hemosiderosis and erythroblastosis are found in it.
The children who overcame hemolytic disease can have considerable defects in the development of the central nervous system (CNS) that results in mental deficiency.
SEPSIS
A sepsis is the special form of infectious disease which often has hard development and is characterized by high lethality. Its polyethiologity (very much many microorganisms can be reason of illness), special reaction of the immune system on an infection, clinical (there is no recurrence in development, not depending on an exciter manifestation of sepsis of the same types), epidemiologys (not contagious disease), pathomorphological features, is characterized (the local and general changes do not have the specific manifestation).
In pathogenic of sepsis an important place is taken to bacteriaemia. Development of sepsis is predefined by the special reaction of macroorganism often it is hyperergic reaction, absence of immunoreaction, acyclicity of development, advantage of general reaction on the hit of microorganisms.
Pathomorphology of sepsis is presented by the local and general manifestation. The local changes develop in the hearth of penetration of microorganisms (septic hearth) or on the way of their distribution:
o lymphangitis,
o lymphadenitis,
o lymphotrombosis,
o phlebitis,
o thrombophlebitis.
A septic hearth more frequent shows up festering inflammation. The general changes are presented by dystrophic, inflammatory, hyperplastical processes in different organs. The dystrophic and inflammatory (intermediate or interstitial inflammation) changes develop in parenchimatous organs and vessels, that predetermines the increase vascular – tissue penetrating and development of hemorragic syndrome and hemolytic icterus.
Hyperplastic processes mainly develop in the lymphoid and hemopoetic system: a generalized lymphadenopathy is the increase of lymphatic knots; septic spleen – acutely megascopic, rose, loose, gives large scrape of pulpa; hyperplasia of marrow and his metaplasia; leukocytosis with development even leukemoid reaction.
Classification of sepsis is based on etiology, entrances gate clinical-morphological manifestation.
After etiology a sepsis can be related to the different microorganisms (by bacteria, fungi, and others like that). Today more frequent meets staphylococcus and pseudomonas aeruginosa sepsis.
After an entrances gate a sepsis is: surgical, therapeutic, wound, umbilical, fallopian, otogenic, odontogenic, tonsilogenic, urology, criptogene (an entrances gate not is known). Lately began to select a paratherapeutic sepsis, when an infection is brought in an organism during implementation of medical manipulations: incubation (an entrances gate is lungs), cannulation, imposition of vascular shunts, and others like that.
After clinical-morphological features distinguish the following forms of sepsis:
(1) septicaemia,
(2) septicopyemia,
(3) septic (bacterial) endocarditis,
(4) chroniosepsis.
SEPTICAEMIA is characterized to the fasts (a few days), sometimes by fleeting development, expressed intoxication (high temperature disorder of consciousness) enhanceable reactivity of organism (hyperergy), sometimes by absence of septic abscess, by predominance of general changes in an organism: dystrophy and intermediate inflammation of parenchimatous organs (septic spleen, and others like that), vasculites, DIC syndrome, hyperplasia of the lymphoid and hemopoetical systems. Development of Septicaemia is often related to streptococcus. On a skin, mucuses membranes there is the expressed hemorragic syndrome icterus. Patients die from endotoxical shock, hemorrhages in suprarenal glands with development of acute suprarenal deficiency.
Hemorrhagic rash with septicemia.
A SEPTICOPYEMIA is characterized by predominance of festering processes in a gate and distribution them on all organism due to development of bacterial embolies by staphylococcuss, pseudomonas aeruginosa in lungs, liver, kidneys, marrow, synovial membranes, on the valves of heart, membranes and tissue of cerebrum, by the protracted development – a few weeks hyperplastical processes, intermediate inflammation expressed insignificantly. Among complications select the empyema of pleura, peritonitis, phlegmons of skin.
Septic emboli in blood vessels in the lungs.
A SEPTIC (BACTERIAL) ENDOCARDITIS develops as a result of septic damage of valves of heart with the hyperergic manifestation as a result of circulation of toxic immune complexes.
Etiology – more frequent aureus and albe staphylococcus green streptococcus, rarer is enterococcus.
Classification.
1. By character of development:
o acute is duration about 2 weeks,
o subacute – 3 months,
o chronic are months and years.
2. Presence or absence of base-line disease:
o primary septic endocarditis or illness of Chornoguzov – develops on the unchanged valves (20-30%),
o second septic endocarditis – develops on a background the defect of heart (rheumatic, atherosclerotic, syphilitic, borning), on prosthesis of valves.
A pathoanatomy is presented by the local and general changes. A polypous-ulcerous endocarditis which more frequent develops on an aortic valve belongs to the local manifestation, rarer – on mitral, and at drug addicts – on tricuspidal. Macroscopically find the considerable areas of necrosises and ulcering with destruction of valve, formation at them of defects, sometimes with fenestration, tearing off of particle of valve and development of tissue embolism. Trombotical masses which spread on an endocardium and wall of aorta deposite often in ulcers. If a septic endocarditis develops on the damaged valves, here the phenomena of sclerosis, hyalinosis early calcification leaves of valves, hypertrophy of myocardium, take place. The microscopic changes are presented by polymorphic-nuclear leukocyte, lympho-macrophage infiltration of wall of valve, presence of colonies of microorganisms, considerable deposites of salts of calcium, in trombotical masses.
This is infective endocarditis. The aortic valve demonstrates a large, irregular, reddish tan vegetation.Virulent organisms, such as Staphylococcus aureus, produce an “acute” bacterial endocarditis, while some organisms such as Streptococcus viridans produce a “subacute” bacterial endocarditis [http://library.med.utah.edu].
The more virulent bacteria causing the acute bacterial form of infective endocarditis can lead to serious destruction, as shown here in the aortic valve. Irregular reddish tan vegetations overlie valve cusps that are being destroyed. Portions of the vegetation can break off and become septic emboli. [http://library.med.utah.edu].
This angiogram demonstrates the aortic arch and great vessels. An embolus from a cardiac valvular vegetation from the left side of the heart can travel out the systemic circulation. Shown here is a septic embolus from infective endocarditis travelling up the left common carotid artery, which could result in a cerebral infarction and/or abscess. [http://library.med.utah.edu].
In this case, the infective endocarditis demonstrates how the infection tends to spread from the valve surface. Here, vegetations can be seen on the endocardial surfaces, and the infection is extending into to underlying myocardium. [http://library.med.utah.edu].
Here, infective endocarditis on the mitral valve has spread into the septum all the way to the tricuspid valve, producing a fistula. [http://library.med.utah.edu].
Microscopically, the valve in infective endocarditis demonstrates friable vegetations of fibrin and platelets (pink) mixed with inflammatory cells and bacterial colonies (blue). The friability explains how portions of the vegetation can break off and embolize. [http://library.med.utah.edu].
Here is a valve with infective endocarditis. The blue bacterial colonies on the lower left are extending into the pink connective tissue of the valve. Valves are relatively avascular, so high dose antibiotic therapy is needed to eradicate the infection. [http://library.med.utah.edu].
The general manifestations of endocarditis are:
(1) septic spleen (megascopic in sizes, tense capsule, gives considerable scrape, often there are infarcts, at chronic development is sclerosis and compression);
(2) generalized alterative-productive vasculitis especially in the vessels of microcirculation with development of plural petechial hemorrhages on a skin, mucuses and serosal membranes conjunctiva (a lower eyelid near an internal edge are Lukin-Libman spots – pathognomical sign);
(3) immune complex diffuse glomerulonephritis;
(4) artritis;
(5) are tromboembolical complications with development of infarcts in a spleen, kidneys, cerebrum, gangrene.
Before the peripheral manifestation take also the knots bulges on the hands of brush are knots of Osler, bulges of nail flanks («drumsticks»), cells of necrosis in a fatty hypoderm, dermatorrhagias and hypoderm (spots of Jeynuey), icterus.
Seen here in the finger at the right are small splinter hemorrhages in a patient with infective endocarditis. These hemorrhages are subungual, linear, dark red streaks. Similar hemorrhages can also appear with trauma. [http://library.med.utah.edu].
Another small linear splinter hemorrhage is seen here subungually on the left thumb of a patient with infective endocarditis and blood culture positive for Staphylococcus aureus. [http://library.med.utah.edu].
Figure. Knots of Osler.
Figure. Spots of Jeynuey.
Chroniosepsis form of sepsis, which has the following signs:
o long-term development,
o decreasing of reactivity of organism,
o presence septic hearth which lasted does not heal (carious tooth, chronic tonsillitis wound, with suppuration),
o chronic intoxication with exhaustion (pyoresorptive fever),
o brown atrophy of organs (hearts, livers, and others like that),
o atrophy and hemosiderosis of spleen,
o lardaceous of internalss.
