Types of congenital defects and deformities of the nose - treatment of nasal abnormalities in newborns and child care. Strabismus in infants: normal or pathological

33 reported hereditary diseases with growth retardation. Attention is drawn to their phenotypic similarity and the real difficulties of distinguishing them from each other. It is proposed to select two groups and a table differential diagnosis .

Substantial part hereditary pathology childhood clinically expressed by a sharp retardation of children's growth. The variety of nosological forms and the relatively low frequency of many of them create great difficulties in the process of differential diagnosis of these conditions.

From the standpoint of differential diagnosis, it is advisable to divide a large group of diseases into separate subgroups - growth retardation against the background of a sharp disproportion of the skeleton and growth retardation with a proportional shape of the skeleton.

A. Differential diagnosis diseases accompanied by stunting and severe skeletal disproportion

This group is extremely heterogeneous. It includes diseases classified according to the classification of M.V. Volkov, E.M. Meersov et al. to epiphyseal dysplasia - pseudochondroplasia, etc.; physeal - achondroplasia, etc.; spondyloepimetaphyseal - parastrematic dysplasia, etc.; diaphyseal dysplasia - imperfect bone formation, etc.; representatives of mixed forms systemic diseases skeleton - Ellis-van-Creveld disease; mucopolysaccharidoses.

Most nosological forms are characterized by the manifestation of clinical signs from birth or from the first months of life. In a number of diseases (Seckel syndrome, Russell-Silver syndrome, etc.), children are born with a short body length. Below is a brief clinical characteristics diseases.

Achondroplasia. Dwarfism with pronounced shortening of the limbs. Pronounced frontal tubercles. Sunken bridge of the nose. Prognathism. Waddle, "duck" gait. Lumbar lordosis. In most cases, patients have normal intelligence.

X-ray findings: shortening of the proximal parts of the limbs. Kyphosis. Shortening of the femoral neck. Elongation of the fibula. Narrowing of the distance between the roots of the arches of the lumbar vertebrae. Frequency - 1: 10.000. Type of inheritance: autosomal dominant. About 80% of cases are sporadic (fresh mutations). Average age fathers of probands increased.

Hypochondroplasia. Growth retardation is mainly observed after 3-4 years. Sharp shortening of the limbs. Face without pathological features. Wide rib cage. Lordosis. Sometimes - slight flexion contractures in the elbow joints.

Rg - shortening of the limbs, some lengthening of the fibula, wide hands, disruption of the structure of the vertebral bodies. The type of inheritance is autosomal dominant. An increase in the age of fathers was noted.

Parastrematic dysplasia. Sharp growth retardation (average adult height 90-110 cm) in combination with multiple skeletal deformities. There is a "twisting" of the bones around the axis. Short neck. Kyphoscoliosis. Varus and valgus deformities of the legs. Multiple contractures of large joints.

Rg - coarse trabecular bone structure with areas of dense dots and streaks - “flaky” bones. Zones of enchondral ossification are transparent and expanded. The vertebral bodies are flattened. The pelvic bones are dysplastic. The metaphyses and epiphyses of the tubular bones are deformed. The type of inheritance is autosomal dominant.

Langer's mesomelic dysplasia. A sharp retardation in growth with pronounced shortening of the limbs, especially the forearms. Intelligence preserved.

Rg - hypoplasia of the ulna and fibula.

Type of inheritance: autosomal dominant.

Rhizomelic dysplasia. Growth retardation with sharp shortening of the proximal limbs. Microcephaly. Low nasal septum. Mental retardation. 70% of patients have cataracts. Multiple joint contractures.

Rg - vertebral dysplasia. Disturbance of the trabecular structure of long bones. Curvature of tubular bones.

Type of inheritance: autosomal dominant, autosomal recessive.

Camptomelic dysplasia. The name comes from the Greek words: kamptos - bend, melos - limbs. Prenatal growth deficiency. The length of children at birth is 35-49 cm. Small face with a low nasal septum. Dolichocephaly. Disproportionally short limbs. Hypoplasia of the scapula. Kyphoscoliosis.

Rg - curvature of the tibia, shortening of the fibula. Thin, short collarbones. Incomplete development of cartilage. The type of inheritance is autosomal recessive.

Diastrophic dysplasia. The name comes from the geological term diastrophism, which refers to the processes of bending of the Earth's crust that result in the formation of mountains and oceans. Prenatal growth deficiency. Severe growth retardation in later life. Significant shortening of the limbs. Kyphoscoliosis. Clubfoot. Limitation of movements in the finger joints. Sometimes there is a cleft palate, hypertrophy of the ear cartilages, and subluxation of the cervical vertebrae.

Rg - calcification and ossification of ear cartilage. Ankylosis of the proximal interphalangeal joints. Shortening and thickening of tubular bones. Subluxation of the hip joint. The type of inheritance is autosomal recessive.

Metatrophic dysplasia. Severe growth retardation with shortening of limbs. Narrow chest with short ribs. Kyphoscoliosis. Limitation of joint mobility.

Rg - platyspondyly, increase in intervertebral spaces. Wide metaphyses. Hypoplasia pelvic bones. The type of inheritance is autosomal recessive.

Pseudoachondroplasia. Growth retardation is observed mainly in the second year of life. The height of adults does not exceed 130 cm. There is a sharp shortening of the limbs, especially the proximal parts. Kyphoscoliosis. Lordosis. Rocking gait. Hallux valgus and viral deformity lower limbs. Increased joint mobility. There are no anomalies of the face or skull.

Rg- wide pelvis. The wings of the ilium are rectangular. The femoral heads are small. The epiphyses are small, the metaphyses have uneven contours, with areas of rarefaction. Delay in the formation of ossification nuclei of the carpal bones. Type of inheritance: the disease is genetically heterogeneous, both autosomal dominant and autosomal recessive forms are found.

Schmid's metaphyseal chondrodysplasia. This is the most common form of metaphyseal chondrodysplasia. Stunting moderate severity(adult height - 130-160 cm). The first signs appear in the second year of life. Significant varus curvature of the legs. "Duck" gait. Lumbar lordosis. Rg - changes in the metaphyses of tubular bones, especially the lower extremities - the contours are uneven, fringed, extensive zones of uneven rarefaction. The type of inheritance is autosomal dominant.

Imperfect bone formation. One of the most common and well-known hereditary lesions of the skeletal system. The pathology is genetically heterogeneous. It is divided into various clinical and genetic types, the main of which are - congenital form(type B roller) and late (Lobstein syndrome).

Congenital form of Vrolik- prenatal growth deficiency. Multiple intrauterine and postnatal fractures, especially affecting long bones, ribs and clavicles. Secondary deformation and shortening of the bones of the limbs. Blue sclera. Megacephaly. Late closure of fontanelles and skull sutures. Extreme softness - “rubbery” quality of the skull. The course is severe, usually children die in the first months of life. The type of inheritance is autosomal recessive.

Late Lobstein form- pathological fragility of bones. Growth retardation. Blue sclera. Hearing loss. Keeled or funnel-shaped deformity of the chest. Kyphosis. Deformation of the pelvic bones. Saber shins. Dentin hypoplasia. Increased joint mobility.

Rg - thinning of the compact layer of tubular bones. Osteoporosis. The type of inheritance is autosomal dominant.

Bloom's syndrome. Prenatal growth retardation is combined with skin changes. Congenital telangiectatic erythema in the form of a butterfly is observed on the face and forearms. Sharply increased light sensitivity skin. There are areas of skin hyperpigmentation and café-au-lait spots. Small narrow face. Premature wrinkles. Hypogenitalism, cryptorchidism. High timbre of voice. The type of inheritance is autosomal recessive.

Ear-palato-finger syndrome. Growth retardation. Violation of psychomotor development and speech development. Prominent forehead. Hypertelorism. Anti-Mongoloid eye shape. Small nose and mouth. Cleft palate. Conducted deafness. Widely spaced toes. Limitation of movements in the elbow joints due to subluxation of the radial head.

Rg - hypoplasia of the facial bones.

Type of inheritance: recessive, linked to the X chromosome.

Weil-Marchesani syndrome. Growth retardation. Brachycephaly. Hypoplasia of the upper jaw. Hypodactyly. Gothic palate. Brachydactyly. Lens subluxation, secondary glaucoma. The type of inheritance is autosomal recessive.

Ellis van Creveld disease. (Chondroectodermal dysplasia). Dwarf stature with normal body length and shortened limbs. Polydactyly. Hypoplasia of teeth and nails. Alopecia. Sometimes - congenital heart defects. Short upper lip.

Rg - shortening of the distal limbs. Slow development of ossification nuclei. Polydactyly, Multiple exostoses. The type of inheritance is autosomal recessive.

Mucopolysaccharidoses. Diseases are hereditary disorders metabolism of glycosaminoglycans and belong to storage diseases - lysosomal diseases. A number of types of mucopolysaccharidoses are clinically characterized by systemic damage musculoskeletal system. This differential diagnostic group includes those with clearly defined growth disorders.

Hurler syndrome. Caused by a defect in the enzyme iduronidase. Clinically manifested from the first months of life. Sharp deformations of the skeleton and skull. Rough facial features. Hypertelorism. Epicanthus. A wide nose with a flattened bridge and everted nostrils. Big and thick lips. Often open mouth, large tongue. Small, widely spaced teeth. Chronic rhinitis. Severe growth retardation. Short neck. Kyphosis with hump in the lower thoracic and upper lumbar region. Big belly. Hepatosplenomegaly. Wide brushes with short fingers. Flexion contractures. Mental retardation. Inguinal and umbilical hernias. Hirsutism. Cloudiness of the cornea. Deafness.

Rg - cuboidal vertebral bodies. Kyphosis. Thickening of the collarbones and shoulder blades. Deformation of the pelvic ring. Flattening, reduction of the femoral heads. Delay in the formation of ossification nuclei. Severe deformities of the facial bones. Increased urinary excretion of dermatan sulfate and heparan sulfate. The type of inheritance is autosomal recessive.

Hunter syndrome. Caused by iduronate sulfatase deficiency. Clinical signs appear at 2-4 years of age. Growth retardation. Moderate bone deformities. Rough facial features. Hypertelorism. Flat bridge of the nose with large nostrils. Thick lips. Macroglossia. Widely spaced teeth. Short neck. Joint contractures occur. Deafness. Mental retardation. Hepatosplenomegaly. Abdominal hernias. Hypertrichosis.

Rg - changes similar to Hurler syndrome, but less pronounced. Increased urinary excretion of dermatan sulfate and heparan sulfate. Type of inheritance: recessive, linked to the X chromosome.

Morquio syndrome. Caused by a deficiency of the enzyme chondroitin-6-sulfate-N-acetylglucosamine-4-sulfate sulfatase. Clinical symptoms appears at 1-3 years of age. Growth retardation. Significant deformations of the skeleton, especially the chest. Wide mouth. Speaker upper jaw. Short nose. Widely spaced teeth. Short neck. Kyphosis. Sharp keeled deformity of the chest. Movements in the joints upper limb limited. Valgus deformity legs and feet. Intelligence is normal. Cloudiness of the cornea. Hearing loss. Tendency to colds. Hernias. Hepatomegaly. Cardiopathy (sometimes).

Rg - platyspondyly. Severe osteoporosis. Kyphosis, scoliosis. Dental hypoplasia. Expansion of metaphyses. The femoral heads are flattened and fragmented. Delay of ossification nuclei of the wrist. Cone-shaped narrowing of the proximal ends metacarpal bones. Increased urinary excretion of keratan sulfate. Type of inheritance: autosomal recessive.

Maroteaux-Lamy syndrome. Caused by a defect in the enzyme arylsulfatase. First Clinical signs appear at 1-3 years of age. Severe growth retardation. Macrocephaly. Rough face. Hypertelorism. A big nose, thick lips. Macroglossia. Short neck. Barrel chest. Kyphosis (sometimes). Flexion contractures in joints. Valgus deformity of the legs. Clouding of the cornea leading to blindness. Deafness (sometimes). Inguinal, umbilical hernias. Hepatosplenomegaly. Intelligence is unchanged.

Rg - deformation of the pelvic ring. Thinning of the femoral neck. Round biconvex shape of the vertebrae, concave back surface lumbar vertebrae. Increased urinary excretion of dermatan sulfate. Type of inheritance: autosomal recessive.

B. Differential diagnosis of diseases accompanied by severe growth retardation with a proportional skeletal shape

The vast majority of diseases included in this differential diagnostic group are characterized by low height at birth. In the future, as children develop, the growth retardation increases, but the physique remains proportional.

Pituitary dwarfism. Caused by dysfunction of the pituitary gland. The totality of data from modern clinical genetics and endocrinology has made it possible to establish that there are several various forms pituitary dwarfism.

Pituitary dwarfism, type I. It has now been established that the disease is caused by (isolated) growth hormone deficiency. A sharp retardation of growth, which becomes especially obvious in the first 2 years of life. Thick skin. Subtle voice. The type of inheritance is autosomal recessive.

Laron's disease. Patients had clinical signs of growth hormone deficiency with elevated level of this hormone in the blood serum. In this case, the formation of somatomedin in the liver appears to be affected.

Cornelia de Lange syndrome. Severe growth retardation. Brachycephaly. Microcephaly. Dense, fused eyebrows, long eyelashes. Hirsutism. Hypertelorism.

Short nose, sunken bridge. The distance between the nose and upper lip. Short stature hair on the forehead and back of the head. A bluish tint to the skin in the area of ​​the eyes, nose, lips, due to increased venous pattern. Small hands and feet. Clinocamptodactyly (sometimes). Contractures elbow joints. Mental retardation.

Rg - cone-shaped epiphyses, hypoplasia of the head radius, horizontal arrangement of ribs. Frequency among newborn children: 1: 30.000-1: 50.000. Type of inheritance: unclear, polygenic inheritance possible. Most cases in pedigrees are sporadic.

Seckel syndrome. Prenatal growth deficiency. Microcephaly. Narrow face. Low position ears. Nose in the shape of a bird's beak. Micrognathia. Coarse hair. Keeled chest. Scoliosis, kyphosis. Clinodactyly. Subluxation hip joints. Mental retardation, negativism, tearfulness. Malformations of the kidneys, liver, genitals. Hypergammaglobulinemia. Hyperaminoaciduria. Transverse groove on the palm.

Rg - digital impressions on the skull, lesser sella turcica. Hypoplasia of the radius and fibula.

Russell-Silver syndrome. Prenatal growth deficiency, later - its sharp lag. A small, triangular face with the corners of the mouth downturned. Hypoplasia lower jaw. Late closure of fontanelles and teething. Body asymmetry - hemihypertrophy or limb length asymmetry. Clinodactyly. Brachydactyly. Scoliosis, due to asymmetry of the torso. Cafe au lait spots on the skin. Precocious puberty.

Dubovich syndrome. Prenatal growth deficiency followed by retardation. Microcephaly. High forehead, wide nose with a flat bridge. Facial asymmetry (sometimes). Hypertelorism. Blepharophimosis. Ptosis. Micrognathia. Low position of the ears. Coarse hair. Polydactyly. Clinodactyly. Mental retardation (not always). High voice. On the skin - eczema and psoriasis. Hypospadias, cryptorchidism.

Rg - periosteal hyperostosis of long bones, various anomalies of the ribs.

The type of inheritance is autosomal recessive.

Rubinstein-Taybi syndrome. Short stature. Microcephaly. Hypertelorism. Prominent forehead. Ptosis. Strabismus. Long eyelashes. High palate. Beaked nose. Micrognathia. Anomalies of bite and position of teeth. Low position of the ears. Mental retardation. Wide terminal phalanges thumbs arms and legs. Brachydactyly. Polydactyly. Clinodactyly. Scoliosis. Hypermobility of joints. Cryptorchidism. Cataract, hypermetropia, optic atrophy (sometimes). Various vices internal organs. Transverse groove on the palm.

Rg - wide, thickened distal phalanges of the thumbs. Defects of the spine, sternum and ribs.

Type of inheritance: unclear. Most cases are sporadic.

Leprechuanism. Children are often born prematurely. There is marked retardation in height and weight. Microcephaly. Hypertelorism. Large, low-set, protruding ears. Grotesque facial features. Flat nose with wide nostrils. Large mouth with thick lips. Exophthalmos. Large hands and feet. Delayed psychomotor development. Cryptorchidism. Enlargement of the labia, clitoris. Umbilical, inguinal hernia. Skin folding. The course is severe - children usually die in the first year of life. Hyperinsulinemia. Low level alkaline phosphatase.

Rg - delay in the formation of ossification nuclei.

Type of inheritance: autosomal recessive.

Smith-Lemli-Opitz syndrome. Prenatal growth deficiency followed by retardation. Microcephaly. Short snub nose. Increasing the distance between the nose and upper lip. Micrognathia. Cleft palate or uvula. Strabismus. Low position of the ears. Short neck. Syndactyly (cutaneous). Brachydactyly. Mental retardation. Pyloric stenosis, in early childhood vomiting is noted. Hypospadias, cryptorchidism. Transverse groove on the palm. Heart defects (sometimes). Hernias (sometimes). Type of inheritance: autosomal recessive.

Noonan syndrome. Growth retardation. Broad forehead. Hypertelorism. Ptosis. Epicanthus. Sad expression. High palate. Anomalies of teeth. Splitting of the tongue. Low position of the ears. Coarse hair. Low hair growth at the back of the head. Kyphoscoliosis. Clinodactyly. Autism. Wing-shaped fold on the neck. Delayed secondary sexual characteristics. Anomalies urinary tract. Hepatosplenomegaly (sometimes). Congenital lymphedema of the hands and feet. Karyotype is normal. Type of inheritance: autosomal dominant.

Hanhart syndrome. A sharp retardation of growth mainly from the second year of life. Obesity. Delayed secondary sexual characteristics. Delay in the formation of ossification nuclei. The type of inheritance is not clear.

Familial osteopetrosis. Growth disturbance. Macrocephaly. Prominent forehead. Ptosis. Strabismus. Anomalies of teeth, caries. Frequent multiple fractures. Deafness (sometimes). Cataract, atrophy optic nerve(Sometimes). Delayed psychomotor development. Hepatosplenomegaly. Anemia. Lymphocytosis.

Rg - diffuse osteosclerosis (marble bones). Partial aplasia of the distal phalanges. Type of inheritance: autosomal dominant, autosomal recessive.

Thus, among hereditary forms Growth retardation can be classified into 33 diseases, each of which is relatively rare. These diseases have many common phenotypic features. The proposed differential diagnostic tables can greatly facilitate the differentiation of similar diseases.

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Not all parents know that strabismus in infants is often physiological norm. In order to understand when you should immediately go to the doctor with such a problem, and when you should not worry, you need to understand why this happens.

What is the norm?

In an adult, the axes of the eyes normally coincide completely. Deviation from this is called strabismus, or strabismus. There is another clinical name - heterotropia. There are two main types of strabismus:

1. Converging. In this case, one or two eyes slant towards the bridge of the nose. In infants this is exactly the type observed (in 90% of cases).
2. Divergent. One or both eyes move towards the temple.

As a result of the fact that the newborn baby often experiences weakness oculomotor muscles, for this reason heterotropia develops.

Movement control is not always available to him at birth eyeballs. It is important for parents to know when this phenomenon occurs, since such a process cannot be started.

Only 9% of seven-year-old children of the total number of children with strabismus persist. Over time, the eye muscles become stronger, and there is no longer any reminder that the baby had strabismus.

The structural features of the skull bones and the wide bridge of the nose also lead to the fact that the child has some deviation. It goes away in a few months.

Causes of pathological strabismus

But there are a number of cases in which normalization does not occur. The causes of this pathology may be:

• birth complications;
• lack of oxygen during intrauterine development;
• infection and intoxication of the fetus;
• previous measles, scarlet fever or influenza;
• neurological abnormalities;
• hereditary predisposition;
• improper placement of toys above the bed.

Psycho-emotional stress (screaming, bright light, etc.) can lead to the temporary appearance of strabismus in a newborn.

If strobism is observed for more than six months, it leads to impaired visual acuity and the development of amblyopia.

When to go to the doctor?

Despite the fact that strabismus can go away a month after birth, or three, but normally this phenomenon should not be observed in a six-month-old baby.

It is at this age that strabismus refers to pathological condition, and is a reason to see a doctor.

Distinguish the following types diseases:

• by time of appearance - congenital or acquired;
• permanent and temporary;
• one-sided or alternating;
• convergent, divergent and vertical.

Separately, we should highlight the paralytic type, in which the eye does not move in a certain direction as a result of damage to a muscle or nerve.

How to prevent the disease?

To prevent strobism from causing vision loss, there is prevention of strabismus in infants.

If a baby has strabismus at the age of one month, then you need to do the following:

1. Hang bright toys above the center of the crib at such a distance that the baby would not be able to reach them with his hand.
2. Toys should only be large sizes.
3. Do gymnastics to strengthen the eye muscles. For this purpose, you need to take a large and bright rattle and move it from side to side so that the baby follows it with his eyes.
4. Pass at two months of age routine inspection specialist and follow all his recommendations.

Treatment

On this moment There are 25 types of strabismus. For this reason, only a specialist should treat it. In each case, only an individual approach is applied.

Such a disease should not be neglected, as vision may gradually decrease sharply.

Once diagnosed, treatment is as follows:

1. Until all symptoms are completely eliminated, the child is given corrective glasses or soft lenses.
2. To improve the functioning of the affected eye, the occlusion method is used. It consists of closing the healthy eye for a while and forcing the sick one to work.
3. A variety of techniques are used to restore binocular vision.
4. If the child turns four years old, then complex treatment orthopedic and acupuncture therapy is used.

If a paralytic form of strobism is detected, consultation with a pediatric neurologist is necessary!

If there is no effectiveness, the doctor may recommend surgery. It is carried out under general anesthesia. After this, the child undergoes rehabilitation and strengthens eye muscles with the help of special exercises.

The presence of strabismus in a newly born baby is not a reason to panic; for the first few months of his life he cannot focus his gaze.

But in most cases, by 4-6 months this phenomenon disappears without leaving a trace. Correct prevention will help to avoid the transition of physiological strabismus to pathology.

Passing through the birth canal, the baby’s entire body is greatly compressed, as a result of which the newborn’s head may have asymmetry and the face may be swollen.

Head of a newborn baby

The head of a newborn is relatively large; immediately after birth, almost every child can notice some deformation of the head, less often - asymmetry is obvious. As a rule, any such changes are temporary and they should not frighten young parents.

The main cause of deformation, as already mentioned, is the process of the little man passing through the birth canal. The fact is that the bones of the child’s skull are forced to shift slightly relative to each other during this difficult journey. For this reason, experts have identified a certain pattern: the larger the baby’s head, the more deformation it will be subject to. As a rule, a large head is characteristic of a large fruit.

Babies who were born with the help do not have a noticeable deformation of the head.

If you carefully and carefully feel the head of a newborn baby, you can easily detect the so-called fontanelles. They are a soft area of ​​skin between the bones of the skull; when you press lightly with your finger on such areas, you can feel some pulsation. The largest fontanel is located just above the top of the head, the second is slightly lower from the large one. As the baby grows, his fontanelles tighten; As a rule, by the age of one year they completely disappear.

Newborn baby's face

In the first hours after birth, the newborn’s face still retains traces of strong compression: the nose is flattened, the eyelids are slightly swollen, the skin is swollen, with a reddish tint. In the folds on the face (in the nose area), behind the ears there are small accumulations of a special secretion in the form of white/yellowish dots, thanks to this lubricating secretion it was easier for the child to pass through the birth canal. You shouldn’t remove such accumulations yourself; they will go away on their own over time.

We should also talk about the nose of a newborn. Immediately after birth, this olfactory organ will be slightly flattened and may appear to be very large. This condition, again, is explained by the journey that the baby had to go through. In a couple of days, the baby’s nose will become neat.

In the very first minutes after birth, all nasal cavity the newborn is filled with amniotic fluid, which doctors remove themselves using a special suction. The baby's nasal cartilage is very soft. The nasal passages are relatively narrow, have a large number of blood vessels, therefore, if the vessels dilate (i.e., the mucous membrane swells), then the baby’s breathing becomes significantly more difficult. If breathing is difficult, the baby will often pause during feeding to inhale air through the mouth. inevitable.

Experts say that a newborn is able to distinguish smells almost immediately after birth. Even they can boast of a good sense of smell. In this regard, a young mother should remember: experiments with eau de toilettes/deodorants are best left for later if she wants to breastfeed her baby for a long time.

If the baby is large, then, most likely, some deformation of the face will be visually noticeable: during the birth process, not only the bones of the skull, but also the bones on the child’s face shifted. A young mother will not find such a face attractive, but by the time she is discharged, the baby’s skin will have time to smooth out and he will appear before his dad (and other relatives) in his very beauty.

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