Pathological anatomy of intrauterine infections

Relevance of the problem

Intrauterine infections are the main cause of perinatal mortality. So, 80% of infectious fetopathies end fatally. Surviving children usually have severe lesions of various organs, especially the brain and liver.

Classification

Intrauterine infections are classified according to the following basic principles:

I. Time of infection

1. Prenatal ( antenatal ) infections (synonym - infectious kimatopathy ) - intrauterine infections that occur during infection of the fetus before birth (in the prenatal or antenatal period).
2. Intranatal infections - intrauterine infections that develop as a result of infection of the fetus during childbirth (in the intrapartum period).

II. Etiological principle

1. Viral intrauterine infections
2. Bacterial intrauterine infections
3. Fungal intrauterine infections ( intrauterine mycoses )
4. Intrauterine protozoa .

Prenatal infections are divided into the following four types:

1. Infectious blastopathies - infectious kimatopathies that occur during blastogenesis (1-15 days of fetal life). Most infectious blastopathies result in the death of the ovum and the development of spontaneous abortion.
2. Infectious embryopathies - infectious kimatopathies that develop during embryogenesis (16-75 days). Infectious embryopathies lead either to the death of the embryo, or to the formation of congenital malformations.
3. Early infectious fetopathies - infectious kimatopathies that occur in the early period of fetogenesis (76-180 days). Early infectious fetopathies, as a rule, are accompanied by a delay in the development of the fetus (low birth weight is characteristic); malformations of organs during this period are rarely formed.
4. Late infectious fetopathies are infectious kimatopathies that develop in the late fetal period (181-280 days of fetal life). Late infectious fetopathies are mainly manifested by generalized processes resembling sepsis; in this case, brain damage predominates.

Source of infection

The source for intrauterine infections is the mother: pregnant in case of antenatal infection or a woman in labor with intrapartum infection.

Infection Mechanisms

Two mechanisms of intrauterine infection are distinguished: (I) antenatal ( prenatal ) and (II) intrapartum .

Ways of antenatal infection: (1) transplacental , (2) ascending , (3) descending .

Transplacental Path

Transplacental pathway (synonyms: vertical , hematogenous ).

There are two options for transplacental infection:

1. With the development of placentitis
2. Without the development of placentitis.

With the development of placentitis, an infection of the extraplacental membranes and amniotic fluid can occur a second time, followed by infection of the fetus due to contact of infected water with its skin or mucous membranes (aspiration or ingestion of water).

Ascending infection

An ascending infection is the penetration of the pathogen from the vagina and cervical canal into the membranes, then into the amniotic fluid.

Further, there are three options for the spread of infection and infection of the embryo / fetus:

1. Contact skin lesion (pathogen penetrates the embryo / fetus through the skin).
2. When infected water is swallowed, the pathogen enters the fetus through the mucous membranes of the gastrointestinal tract.
3. With water aspiration, primary damage to the mucous membranes of the respiratory tract occurs.

Descending Path

The descending path is infection of the ovum when the infection enters the uterine cavity through the fallopian tubes with salpingitis of an infectious origin or from the peritoneal cavity if there is a focus of infection in it.

Intranatal infection

Intranatal infection - infection during childbirth during the passage of the fetus through the infected birth canal. The intranatal infection mechanism is characteristic of herpes simplex type II , eye gonorrhea ( blenorrhea ), viral hepatitis B , congenital syphilis .

For infectious fetopathies, the following tissue changes in the fetus are characteristic:

I. Specific Changes

1. The formation of tissue dysplasia , primarily due to a delay in the differentiation (maturation) of tissues .
2. Extramedullary proliferation of myeloid tissue in the interstitium of various organs, which is a kind of analogue of the immune response. Extramedullary hematopoiesis as a protective and adaptive process is observed in the body of a full-term baby, usually before the age of 3 months of fetal life. Starting from 3 months of age, cellular immune responses are manifested by the formation of lymphohistiocytic infiltrate in the tissue ( immune inflammation ).
3. Mesenchymosis - excessive proliferation of connective tissue as a manifestation of the regenerative process .

II. Nonspecific changes

1. Alterative processes in the form of necrosis ( destructive changes ) or severe dystrophy.
2. Inflammatory response : exudative or productive inflammation.
3. Hemorrhagic syndrome in the form of hemorrhages in the skin, mucous membranes and internal organs (the result of damage to the walls of blood vessels - infectious vasculitis ).

Etiology

Cytomegalovirus (CMV) is a DNA virus from the Herpesviridae family. The virus is able to infect various human cells, but has a pronounced tropism for salivary gland epithelium.

Epidemiology

Source of infection : (1) a mother with a cytomegalovirus infection (usually in such cases the infection proceeds easily as an acute respiratory viral infection) or (2) a virus carrier (a persistent form of a cytomegalovirus infection in the mother that does not manifest itself clinically).

Infection mechanisms : (1) transplacental infection ( antenatal cytomegalovirus infection ), (2) if an infected genital tract is swallowed or aspirated, an intrapartum cytomegalovirus infection develops during childbirth.

Classification

The following options for intrauterine CMV infection are distinguished:

1. A generalized form that occurs with damage to various organs (more common).
2. The localized form is cytomegalovirus sialadenitis .

Pathological Anatomy

The prevalence of the process with cytomegalovirus infection depends primarily on the maturity of the fetal / child's immune system. At 3 months of age, his body becomes capable of a full-fledged immune response. In this case, lymphohistiocytic inflammatory infiltrate is formed in the tissues. In the fetus and under the age of 3 months, a erythromyelocytic reaction occurs in a similar situation, i.e. a center of extramedullary hematopoiesis is formed with an insufficient number of immunocompetent cells. Therefore, under the age of 3 months, as a rule, a generalized form of cytomegalovirus infection develops, in children older than 3 months the localized form dominates.

Eye of an owl (bull's eye). Cytomegalovirus-infected cells are sharply enlarged, primarily due to the nucleus, where the viral particles are concentrated. The center of the nucleus is occupied by a large basophilic viral inclusion, heterochromatin is located on the inner surface of the karyolemma, a bright zone is revealed between the inclusion and chromatin. This kind of core is called the "eye of an owl" or the "eye of a bull."

Generalized form

In the generalized form of intrauterine cytomegalovirus infection, the leading is liver damage ( cytomegalovirus cholangiohepatitis ). In this case, the bile ducts are blocked by cytomegalovirus-infected cholangioepithelial cells. Due to a violation of the outflow of bile, obstructive jaundice is formed, discolored feces and dark urine appear, the liver grows, and a hemorrhagic rash can occur. In some cases, lung damage ( interstitial cytomegalovirus pneumonia ) and central nervous system ( cytomegalovirus encephalomyelitis ) come to the fore. The forecast is unfavorable. Surviving children show signs of damage to internal organs and the brain in the form of hydro- and microcephaly.

Localized Form

Cytomegalovirus sialadenitis usually proceeds chronically. At the same time, fibrosis develops in the stroma of the salivary glands, atrophy of parenchymal elements occurs, and functional insufficiency of the glands in the form of hyposialia (a decrease in salivation) occurs. Hyposalivation contributes to various infectious processes in the tissues of the oral cavity (caries, periodontitis, stomatitis), since one of the main functions of saliva is its biostatic effect.

Etiology

Intrauterine herpes simplex is more often caused by the herpes simplex virus ( simplex virus ) type II, less commonly the type I virus. Simplexvirus is a DNA-containing virus from the Herpesviridae family.

Epidemiology

Source of infection. Chronic recurrent herpetic infection of the mother (genital herpes or asymptomatic carriage of the virus). During pregnancy, the virus often activates with a latent form of infection. With exacerbation of herpes in the last months of pregnancy, the risk of infection of the fetus reaches 40%.

Infection mechanisms : (1) intranatal (more common), (2) transplacental infection, (3) ascending path.

Classification

I. The prevalence of lesions

1. Generalized form
2. Meningoencephalitis
3. Mucocutaneous form .

II. Infection period

1. Intranatal herpes simplex develops during infection of the fetus during childbirth (occurs most often), manifests itself within 1-2 weeks. of life.
2. Prenatal ( antenatal ) herpes simplex , which develops during infection in the prenatal period (less common), the baby is born with signs of herpes infection.

Pathological Anatomy

The pathognomonic sign of herpes simplex is a vesicular ( vesicular ) rash on the skin and mucous membranes, but it does not appear in all cases.

1. The generalized form occurs more often without a vesicular rash on the skin and mucous membranes. In this case, multiple necrosis develops in various organs, including the brain. With liver damage ( herpetic hepatitis ), parenchymal jaundice develops. In severe cases, hemorrhagic manifestations in the form of a hemorrhagic rash on the skin and gastrointestinal bleeding are possible. Mortality reaches 80%.

2. Herpetic meningoencephalitis - acute destructive meningoencephalitis with localization of necrosis mainly under the ependyma of the ventricles and in the frontal lobes. Mortality is 50%.

3. The congenital mucocutaneous form is characterized by a vesicular rash throughout the skin, including the palms and soles, as well as on the mucous membranes. The vesicles are constantly poured over 2 weeks. - 1.5 months The prognosis is favorable, but generalization of the infection and death is possible.

Etiology

The causative agent of the disease is a DNA virus from the Hepadnaviridae family.

Epidemiology

Source of infection . A mother can infect a baby if she is (1) sick with viral hepatitis B, especially in the last trimester of pregnancy, or (2) a carrier. In general, the risk of perinatal infection is 40-50%.

The mechanism of infection . (1) The main mechanism of infection is infection during childbirth, which occurs in two ways: from blood-containing amniotic fluid through macerated skin and mucous membranes of the fetus, as well as when passing through the birth canal. (2) Transplacental transmission of the virus occurs in 10% of cases of viral hepatitis B in children, mainly in regions with a high prevalence of the virus. The risk of infection increases sharply (up to 95%) when an HBe antigen is detected in the mother’s blood. (3) In rare cases, infection of the baby occurs immediately after birth, but most likely not through milk, but when the mother’s blood from the nipple cracks gets on the macerated mucous membrane of the baby’s mouth (parenteral infection mechanism). However, such a disease, according to formal signs, cannot be attributed to intrauterine infection.

Pathological Anatomy

In general, intrauterine viral hepatitis B proceeds similarly to adult disease.

The features of this infection in early childhood include the following:

1. The shorter the preicteric period , the harder the subsequent course of the disease (in older children and adults, on the contrary).
2. In the icteric period , in addition to the enlarged liver, an increase in the spleen ( splenomegaly ) is more often than in other age groups.
3. The convalescence period is usually longer.
4. Chronic forms of the disease are much more often formed (when infected in the first year of life, chronic viral hepatitis B occurs in 90% of cases, while infection in an adult state occurs in 10%), and therefore cirrhosis and primary liver cancer .
5. Primary liver cancer can develop in the first year of a child’s life and even in utero; unlike primary liver cancer in adults, it is less associated with cirrhosis.
6. In a micromorphological study, multinuclear giant hepatocytes ( giant cell hepatitis ) are often found in the tissue of the affected liver, but they cannot be considered pathognomonic for intrauterine hepatitis B (they can also be found in intrauterine herpetic hepatitis , with intrauterine measles and measles rubella ).

In addition to viral hepatitis B, intrauterine infection is also caused by hepatitis C and D RNA viruses ( delta ). Particularly difficult for children in their first year of life is co-infection with hepatitis B and D viruses, often in the form of a malignant necrotic form ( progressive massive liver necrosis ), in which children usually die in 2-3 days with symptoms of acute liver failure .

Etiology

The causative agent of rubella (measles rubella) is an RNA virus from the family Togaviridae (genus Rubivirus ). The rubella Latin name is rubeola .

Epidemiology

The source of infection is (1) a sick rubella mother or (2) a virus carrier (with latent viral infection). The disease in a pregnant woman proceeds easily in the form of catarrh of the upper respiratory tract, cervical lymphadenitis, spotted-papular rash and mild signs of a general intoxication reaction. Infection occurs during the incubation period (with the most intense viremia).

The infection mechanism is antenatal, the pathogen is transplacental.

Children who have had intrauterine rubella present a great epidemiological danger, as for months have been sources of infection. The main incidence of rubella is recorded in children of the first decade of life. Diseases of adolescents and adults are extremely rare. This is due to the fact that most people suffer rubella infection in the first years of life in a subclinical ( inapparent , latent ) form . By 10 years of age, more than 50% of the population is infected with rubella virus, and by 20 years of age - 80-95%. However, among adults there are always people who are not infected with the virus and are not immune to rubella. Among women of childbearing age, they are from 1 to 30%.

In the second half of the 20th century, two measles rubella epidemics were registered in the world: in 1964 - 1965 . in the USA (more than 1.8 million people were ill, including 50 thousand pregnant women who gave birth to 20 thousand children with congenital defects) and in Japan in 1965 (about a quarter of the country's population was ill).

Characteristics of acquired rubella. Subclinical , erased and typical forms of infection are distinguished. A typical form is characterized by the appearance in the prodromal period of catarrh of the upper respiratory tract (ARVI) and cervical lymphadenitis (damage to the posterior cervical, occipital and parotid nodes). Lymphadenitis develops 1-3 days before the rash appears and persists until it resolves. In the midst of a fever, rubella exanthema appears in the form of spotted-papular elements located throughout the skin. The rash disappears after 2-3 days, leaving no trace. In adults, measles rubella can occur in severe form.

Classification

Three forms of intrauterine rubella are distinguished:

1. Rubeolar embryopathy ( Gregg syndrome ) with infection in the first trimester of pregnancy.
2. Rubeolar fetopathy , developing during infection of the fetus in the second or third trimesters.
3. Rubeolar embryofetopathy .

The affected fetus either dies in utero, or is born with signs of intrauterine rubella. However, fetal damage does not occur in all cases of the mother’s disease, especially after 8 weeks. pregnancy. In this case, the placenta is almost always affected ( rubeolar placentitis ).

Pathological Anatomy

The virus inhibits the mitotic activity of embryonic cells, resulting in the development of hypoplasia of the primordia of organs and other disorders of morphogenesis. The frequency of organ defects depends on the gestational age at which infection occurs: with infection in a period of up to 4 weeks. defects are formed in 60% of cases, 5-8 weeks. - in 25%, 9-12 weeks. - in 8% (data of the Guide to the epidemiology of infectious diseases. - M., 1993). With rubella infection after three months. In pregnancy, the incidence of anomalies corresponds to the average in the population.

A child is born with signs of general hypoplasia (weight loss is usually 15-30%), anemia and thrombocytopenia, multiple hemorrhages and congenital malformations.

Rubeolar Embryopathy

Rubeolar embryopathy was described in 1941 by the Australian ophthalmologist N. M. Gregg and is called Gragg syndrome . Gragg syndrome is characterized by a triad ( Gregg triad ) in the form of damage to the eyes, organ of hearing and heart. However, damage to the organ of hearing is rare.

1. Damage to the eyeball : microphthalmia and loss of transparency of the lens ( cataract ) occur more often, less often - narrowing of the venous sinus of the sclera (Schlemm canal) with the development of glaucoma , clouding of the cornea and vitreous humor, retinal damage and atrophy of the optic nerve.

2. Heart damage : congenital defects of the interventricular and atrial septa, malformations of the great vessels (open botall duct, stenosis of the aortic arch).

3. Damage to the organ of hearing in the form of persistent deafness due to impaired differentiation of the organ of Corti (up to the complete lysis of its bookmark). Similar changes develop in the vestibular apparatus.

In addition, tooth defects (enamel hypoplasia, late teething) and the brain (microcephaly, less commonly hydrocephalus) often develop. Tooth abnormalities occur in 50%, and the brain - in 10% of cases. Defects of other organs rarely develop.

Rubeolar fetopathy

Rubeolar fetopathy can be observed independently or combined with embryopathy. Usually a baby is born prematurely with dry, flaky skin, often with a hemorrhagic rash . Rubeolar fetopathy proceeds in the form of a generalized process involving almost all organs and tissues.

The most severe is damage to the brain ( rubeolar encephalitis ), eyes ( rubeolar ophthalmitis ) and pancreas ( rubeolar pancreatitis ).

1. Rubeolar encephalitis. Two main forms of rubeolar encephalitis are distinguished: (1) productive necrotic form ( destructive-productive rubeolar encephalitis ), usually combined with productive leptomeningitis ; (2) damage to the brain in the form of a slow infection ( progressive rubeolar panencephalitis ), the manifestation of which occurs in the second decade of life and always ends fatally.

2. Rubeolar ophthalmitis. Productive-necrotic changes are formed in various parts of the eyeball, leading to retinal detachment, the development of cataracts and glaucoma.

3. Rubeolar pancreatitis. With the development of interstitial pancreatitis against fibrosis, atrophy of the islets of Langerhans occurs with a picture of insulin-dependent diabetes mellitus (measles rubella virus is one of the most active diabetogenic viruses).

Perinatal and early infant mortality in case of rubeolar embryopathy / fetopathy is extremely high.

Etiology

Listeriosis is an infectious disease caused by bacteria of the genus Listeria , especially L. monocytogenes . Sometimes the term Listerella is used to refer to these microorganisms, and the disease is accordingly called listeriellosis .

Epidemiology

Source of infection . In intrauterine listeriosis, the source of infection is an infected mother, whose listeriosis can manifest itself in different ways ( carriage , erased form or a form with pronounced manifestations , primarily urogenital listeriosis ). In prenatal infections, the source of infection for humans is primarily the external environment and sick animals ( saprozoonosis ). In some cases, the source may be another person (patient or carrier).

Mechanisms of infection . Intrauterine listeriosis develops with transplacental and ascending ways of antenatal infection and with infection during childbirth. In addition, extrauterine listeriosis of newborns can develop when a child is infected in the first days of life from a mother, medical personnel or infected objects of the external environment. In this case, listeria enters the newborn's body by alimentary, aerogenic and contact routes.

Classification

Two forms of intrauterine listeriosis are distinguished:

1. Generalized intrauterine listeriosis ( granulomatous sepsis of the newborn ).
2. Isolated damage to individual organs (gastrointestinal tract, lungs, liver, brain and its membranes).

Pathological Anatomy

Often, intrauterine listeriosis is combined with fetal asphyxia , since the state of hypoxia is favorable for the reproduction of these microorganisms (Listeria - facultative anaerobes). More often, listeriosis proceeds as a generalized infection. At the same time, granulomas ( listeriomas ) are formed in various internal organs, on the skin and mucous membranes, therefore, generalized intrauterine listeriosis is also called granulomatous sepsis of newborns .

Macromorphologically, listeriomas look like small yellowish papules (nodules) surrounded by a red corolla. When localized on the skin and mucous membranes, they are elements of a rash (papular exanthema and enanthema).

Micromorphologically in the center of a typical listerioma there is a focus of necrosis, along the periphery - ordinary histiocytes, rarely epithelioid macrophages are found. Around granulomas, there are signs of pronounced hyperemia and minor diapedetic hemorrhages (extravasation).

Usually newborns die in the first days after birth. Mortality with intrauterine listeriosis reaches 70%.

Etiology

Currently, tuberculosis is called an infectious disease caused by three types of bacteria of the genus Mycobacterium : M. tuberculosis , M. bovis and M. africanum .

Epidemiology

Source of infection . The source of infection is a mother with tuberculous endometritis , sometimes with other forms of tuberculosis. Infection usually occurs no earlier than IV months. pregnancy.

The mechanism of infection . (1) The main mechanism of infection is transplacental (with this, tuberculous placentitis develops). (2) Less commonly, the ascending path is realized.

Pathological Anatomy

1. With the transplacental mechanism of infection, primary affect is absent. In various organs, primarily in the liver and spleen, foci of caseous necrosis occur.

2. When aspirated or swallowed infected water in the lungs, multiple small primary affects are formed . In the wall of the stomach or intestines, primary affect is also a focus of caseous necrosis. In both cases, regional caseous lymphadenitis develops. If children with multiple primary affects survive, then secondary hematogenous generalization of the process develops.

Intrauterine tuberculosis usually ends with the death of children in the first years of life.

Etiology

The causative agent is the bacterium Treponema pallidum .

Epidemiology

The source of infection is a mother who has syphilis both before pregnancy and during pregnancy. Since secondary syphilis occurs with symptoms of spirochemia, the greatest risk is the birth of a sick child in pregnant women in the secondary period of syphilis. In addition, the infection of the fetus occurs mainly in the first years after infection of the mother, in the future, the risk of intrauterine syphilis decreases sharply.

Mechanisms of infection . Two mechanisms of infection of the fetus with syphilis are distinguished: (1) antenatal (transplacental route of infection) and (2) intranatal, if the mother became ill in the last months of pregnancy.

Despite the early penetration of pale treponema into the fetus, pathological changes in its organs and tissues develop only in the V-VI months of pregnancy. Therefore, adequate therapy in the early stages of pregnancy can ensure the birth of a healthy baby.

Classification

There are four main forms of intrauterine syphilis:

1. Syphilis of the fetus .
2. Early congenital syphilis (from the moment of birth to 4 years) [early congenital syphilis is also divided into syphilis of infants and early childhood syphilis , i.e. from 1 to 4 years].
3. Late congenital syphilis (in children 4 years of age and older).
4. Latent congenital syphilis , found in children of all ages.

In the above classification, the most common at present in Russia, the age limit for the separation of congenital syphilis into early and late variants is the age of 4 years. In international classifications, this boundary corresponds to the age of 2 years, but this discrepancy does not play a significant role.

Pathological Anatomy

Placental changes

Syphilitic placentitis is accompanied by an increase in the mass of the placenta (in typical cases, the fetal-placental coefficient is 1: 3). Microscopically the most significant are three types of changes: (1) fibroplasia - the growth of fibrous connective tissue in the stroma of the villi with their subsequent thickening, (2) lymphohistiocytic inflammatory infiltration of the villi, (3) productive panvasculitis with cicatricial obliteration of the lumen of the affected vessels. Hyperconcentration of treponemal tissue in the placenta does not occur.

Fetal syphilis

Syphilitic embryopathy does not develop. Late miscarriages and birth on the VI — VII months are characteristic. pregnancy macerated fetus. The largest number of pale treponemas are found in the liver, spleen and adrenal glands. Major changes are detected in the bones, skin and internal organs.

1. Bone lesions . The most common and reliable sign of fetal syphilis is the detection of V-VI months. intrauterine development of a lesion of the skeletal system in the form of osteochondritis , less commonly osteoperiostitis .

2. Skin lesions . The skin of stillborn fruits is macerated, eroded in places, the epidermis is exfoliated by layers due to the autolytic process . Due to the underdevelopment of subcutaneous fat, the skin is easily creased, wrinkled, especially on the face, which becomes like the face of an old man.

3. Lesions of the internal organs . Among syphilitic visceritis, the lesions of (1) the liver ( “flint liver” ), (2) the spleen and (3) the lungs ( “white pneumonia” of Virchow ) have the greatest significance in syphilis of the fetus. Changes in them correspond to those with early congenital syphilis.

Early Congenital Syphilis

With early and late congenital syphilis, systemic manifestations of the disease occur due to active spirochemia.

There are two types of early congenital syphilis:

1. Congenital Syphilis in infants
2. Congenital syphilis of early childhood .

I. Congenital Syphilis in infants

Signs of the disease are detected either immediately after the birth of the baby, or within the first 2 months. of life. Rashes contain many pale treponemas and are therefore very contagious. There are no specific granulomas , because only by 6–7 months of age does the child develop the ability to provide a mature response to treponema in the form of granulomas formation.

Congenital syphilis of infancy is manifested by lesions of (1) bones, (2) skin and (3) mucous membranes (primarily the nose, larynx and oral cavity), as well as a number of (4) internal organs.

1. Bone damage is the most common manifestation of early congenital syphilis at present. Sometimes it is the only sign of congenital syphilis. The following forms of bone damage in early congenital syphilis are distinguished: (1) syphilitic osteochondritis, (2) syphilitic periostitis or osteoperiostitis, (3) syphilitic dactylitis, (4) focal fibrosing syphilitic osteomyelitis.

Syphilitic osteochondritis . It occurs in almost all cases of early congenital syphilis. The pathological process is localized in the metaphysis of long tubular bones, most often of the upper limbs, involving both bone and cartilage tissue (hence the term osteochondritis ). Osteochondritis occurs from the V month of fetal life and persists until 12 months of postnatal ontogenesis. In 85% of cases, osteochondritis is detected in the first 3 months. extrauterine life, much less often in children older than 3 months. After a year, manifestations of osteochondritis are rare. On the border of the pineal gland and metaphysis, i.e. at the junction of the red cancellous bone tissue and the bluish cartilage tissue, a yellowish strip of calcified cartilaginous tissue is revealed. This morphological feature is called the Wegener band . Its borders are uneven, serrated. The width is different depending on the severity of the process. Between the Wegener's strip and the bone tissue of the metaphysis, the growth of granulation tissue occurs. Necrosis of granulation tissue can lead to a pathological fracture in the metaphysis. This pathological fracture is called Parro disease .

Syphilitic periostitis / osteoperiostitis is observed in 75% of cases of early congenital syphilis. May occur in isolation from osteochondritis. More often the long tubular bones of the limbs and the flat bones of the skull are affected. Bones thicken due to neoplasm of unevenly calcified fibrous tissue from the periosteum ( ossifying periostitis ). The process in the bones of the skull can lead to its deformation. The most common variant of cranial deformity in syphilitic periostitis / osteoperiostitis is a buttock- shaped skull with sharply enlarged frontal and parietal tubercles, separated by a longitudinal hollow.

Syphilitic dactylitis is a cylindrical or spindle-shaped thickening of the proximal, less often middle phalanges of the fingers. Often, several phalanges are affected. Soft tissue of the brush does not change.

Focal fibrosing syphilitic osteomyelitis is extremely rare. The diaphyses of long tubular bones are affected in the form of an overgrowth of granulation tissue, during the maturation of which a coarse-fibrous (scar) tissue is formed.

2. Skin lesions in early congenital syphilis are manifested in the following forms: (1) syphilitic pemphigoid (syphilitic pemphigus), (2) Hochsinger infiltration and Robinson-Fournier scars, (3) roseous, papular and pustular syphilis. Syphilitic pemphigoid and Hochsinger infiltration are specific manifestations of congenital syphilis. They do not occur with the acquired form of this disease.

Syphilitic pemphigoid - the formation of blisters on the palms and soles, flexion surfaces of the forearms and legs, on the face, less often on the body. Occasionally, bullous rashes are located on the entire surface of the skin. Bubbles contain serous, purulent or hemorrhagic exudate with an abundance of treponema. Such elements are extremely contagious. After opening them, erosion is formed. Syphilitic pemphigus must be distinguished from pemphigoid neonates of staphylococcal etiology.

Hochsinger infiltration usually develops not immediately after birth, but at 8-10 weeks. extrauterine life. The process is localized on the palms, soles, buttocks, but especially often in the lips and chin. It begins with the occurrence of erythema, then infiltration (compaction) develops, as a result of which the skin folds are smoothed, the skin loses its elasticity. The lips are thickened, yellowish red, the mucous membrane and the red border are tense. Usually, the lesion surface is macerated and wet, and many treponemas are found on it. When screaming or mechanical injury to the lips, superficial and deep cracks form, extending to the red border. Cracks are usually located perpendicular to the oval of the mouth, bleed and quickly crust over. After 2-3 months. even without treatment, Hochsinger infiltration is gradually resolved, but in the place of deep cracks, especially in the corners of the mouth, radar scars ( Robinson-Fournier scars ) remain for life. Radial cracks at the entrance to the nasal cavity, around the anus, as well as skin cracks in the interdigital spaces are characteristic. Diffuse edema, thickening and redness develops on the skin of the soles, while the skin becomes smooth and shiny ( “glossy heels” or “varnish soles” ). Diffuse infiltration in the forehead, where the skin is tense, is characterized by dryness, shine and stagnant erythematous coloration with a copper tint, is also characteristic of facial skin. The extension of the process to the superciliary arches is accompanied by a thinning of the eyebrows. On the scalp, hair thinns and falls out.

Roseolous syphilides on the skin of the body are rare, sometimes they undergo peeling or merge, which is not typical for a roseolous rash with acquired syphilitic infection. Papular and pustular syphilis are also rarely formed.

3. The defeat of the mucous membranes . In infants, syphilitic rhinitis may be the earliest manifestation of congenital syphilis. The mucous membrane of the nose with signs of edema, hyperemia, sometimes eroded, with a significant amount of mucus, an admixture of pus and blood. The discharge shrinks into massive crusts, sometimes completely covering the nasal passages. A long-existing process can lead to the destruction of the bone-cartilaginous part of the nose and its deformation ( saddle nose ). In some cases, the nose has a saddle shape at birth.

Damage to the mucous membrane of the larynx is manifested by diffuse infiltration and often occurs in the form of ulcerative laryngitis with hoarseness of the voice. With the transition to cartilage, perichondritis and destruction of the cartilage with the subsequent formation of stenosis are possible.

4. Syphilitic visceritis . The liver is enlarged 1.5-2 times, the section has small yellowish foci, sometimes the growth of fibrous tissue ( "flint liver" ). Interestingly, severe parenchymal jaundice in syphilitic hepatitis is not described. The spleen is sharply increased. Lungs are large, dense [catarrh-desquamative pneumonia with hepatization ( Virchow's “white pneumonia” ) and interstitial process].

II. Congenital Syphilis in Early Childhood

Specific for congenital syphilis in young children are (1) damage to the nervous system and (2) organ of vision.

1. Damage to the nervous system . Three forms of lesions of the brain and its membranes are distinguished with this variant of congenital syphilis: (1) leptomeningitis , (2) meningoencephalitis , (3) hydrocephalus . Hydrocephalus is a complication of leptomeningitis, often detected already at birth or develops at the 3rd month. life proceeds acutely or chronically. It is characterized by an increase in the skull, tension of the fontanelles, divergence of cranial sutures, protrusion of the eyeballs.

2. Damage to the organ of vision . The organ of vision is affected in the form of the development of cataracts, uveitis, retinitis and optic atrophy. Atrophy of the optic nerve leads to loss of vision.

Newborns and infants with early intrauterine syphilis, as a rule, die from secondary infectious diseases (pneumonia, intestinal infections, sepsis).

Late Congenital Syphilis

Late congenital syphilis often manifests itself at the age of 14-15 years, and sometimes at older ages. It is regarded as a relapse of syphilis, transferred in early childhood or infancy, as well as a manifestation of a long and asymptomatic process. Signs of late congenital syphilis are identical to those with acquired tertiary syphilis. Reliable signs of late congenital syphilis include the Hutchinson triad (or tetrad ): parenchymal (interstitial) keratitis, syphilitic labyrinthitis and Hutchinson-Fournier teeth. These signs of the Hutchinson triad are rare at the same time. Usually one of them comes to light. A number of authors in this symptom complex include syphilitic drives, thereby expanding the triad to tetrad.

1. Parenchymal keratitis is manifested by diffuse or focal clouding of the cornea, more intense in the center.

2. Syphilitic labyrinthitis ( syphilitic deafness ) is caused by bilateral damage to the auditory nerves and inner ear. If deafness occurs earlier (up to 4 years), then it is combined with difficulty speaking up to dumbness. Parenchymal keratitis and syphilitic labyrinthitis are an allergic reaction and are not associated with the direct influence of the infection.

3. Hutchinson's teeth - a change in the permanent upper central incisors. On their free (cutting) edge, semilunar sickle-shaped notches are formed, as a result of which the cutting surface of the incisors is somewhat narrowed. The neck of the teeth becomes wider, the teeth acquire a barrel-shaped or screwdriver shape. In the latter case, they are called Fournier teeth . The difference in the width of the neck and cutting surface should be at least 2 mm, otherwise this symptom cannot be considered reliable. Treatment of the mother in the last months of pregnancy or the baby in the first 3 months. life can prevent the formation of Hutchinson-Fournier teeth.

4. Syphilitic persecutes is a chronic synovitis of the knee joint, proceeding without damage to cartilage and pineal glands. In this case, the function of the joint is not impaired. The joint is enlarged, periarticular soft tissues are slightly edematous. The process may be symmetrical.

The characteristic signs of late congenital syphilis also include true saber-shaped tibia (a consequence of osteochondritis transferred in infancy) and saber-like forearms similar to them. They are characterized by a crescent anterior tibial bend.

Hidden Congenital Syphilis

Hidden congenital syphilis is called syphilis without signs of skin symptoms with the only manifestations in the form of (1) hepatosplenomegaly with lesions of the bone system or (2) only bone pathology.

Etiology

The causative agent is the intracellular simple organism Toxoplasma gondii . Cells filled with daughter parasite are called pseudocysts . They occur in the acute stage of the disease along with extracellularly located parasites. Toxoplasmas also form true cysts with their own bilayer membrane (they are found in chronic and latent course). In cysts, the pathogen can remain viable for many years. Sexual reproduction of toxoplasma can occur in the intestines of cats; in this case, oocysts are formed, secreted with feces into the external environment, where they can persist for a long time and become a source of human infection.

Epidemiology

The disease is widespread. From 5 to 50% of people are infected in different regions of the country, however, congenital toxoplasmosis occurs in no more than 0.5% of children born to infected mothers. The probability of infection of the fetus increases dramatically if the mother becomes infected during pregnancy.

Source of infection . Toxoplasmosis is an anthropozoonosis. The source of infection for humans is agricultural and domestic animals, especially cats.

Antenatal infection mechanism with transplacental transmission of the pathogen.

Once in the body, toxoplasmas are captured by macrophages, transferred to the lymph nodes, multiply there and penetrate into the bloodstream, spreading to different organs and tissues, are fixed in them and cause alterative-productive changes. Exudative inflammation , especially purulent, is not characteristic.

Classification

There are three forms of congenital toxoplasmosis ( toxoplasmosis fetopathy ):

1. When infected from the beginning of the ninth to 28 weeks, the baby is born with residual effects of the intrauterine meningoencephalitis and signs of cerebral hypoplasia .
2. From the 29th week until the birth, the baby is born with a detailed picture of meningoencephalitis .
3. Infection shortly before birth or during childbirth leads to the development of generalized toxoplasmosis .

Pathological Anatomy

Congenital toxoplasmosis occurs only in cases of infection of a woman during pregnancy, in the phase of parasitemia (it lasts 7-10 days). The most dangerous period is between the 10th and 24th weeks of pregnancy. During this period, the risk of infection of the fetus is combined with severe damage to the internal organs, especially the brain. In a chronic or latent form of infection in a woman, fetal infection does not occur, because it is protected by maternal antibodies. However, miscarriages often develop.

• Pathological anatomy
• Pathological anatomy of viral childhood infections
• Pathological anatomy of bacterial childhood infections
• Pathological anatomy of the prenatal period
• Pathological anatomy of the perinatal period
• Childhood tumors
• Alternative processes
• Destructive processes
• Circulatory disorders
• Inflammatory response
• Immunopathological processes
• Primary immunodeficiencies
• Adaptation and Compensation Processes
• Pathological anatomy of hemoblastosis
• Pathological anatomy of the female reproductive system

• Pathological anatomy of diseases of the fetus and child / Ed. T.E. Ivanovskaya, B.S. Gusman: In 2 vols .-- M., 1981.
• Strukov A.I., Serov V.V. Pathological anatomy.- M., 1995.