DEFECTS OF DEVELOPMENT. PLASTIC SURGERY

Congenital abnormalities of the organism’s growth, throughout which its features are broken or unattainable, are referred to as maldevelopments. These malformations, which deform the exterior type of a physique, particularly in the event that they on the identical time cut back viability of a person, are named congenital malformations.

Small deviations from the traditional kind of the organism construction, that are on the verge of physiologic variations, are named anomalies.

A majority of individuals with heavy congenital malformations require surgical remedy. Duly operative intervention for some defects is the one means for rescuing the lifetime of the affected person.

Congenital malformations are identified from historical instances, some tough infringements of physique type drew consideration to themselves through the prehistoric interval. A lot of the details show it. Rock work made many millennia in the past signify twins which “conjoined”. Within the Babylon’s cuneiform (3800–2000 B.C.), which is translated and revealed by the British museum in 1870, there’s the desk with 62 sorts of congenital malformations. It’s identified, that XI and XII dynasties pharaons (2400–1780 B.C.) had been sick with pes equinovarus. In the primary textual content of the Talmud edited in II centure B.C. a listing of greater than 100 sorts of congenital malformations is given.

Earlier than the event of such disciplines as embryology and comparative anatomy, the incidence of congenital malformations was defined by their paranatural origin, coupling with animals (hybrid principle), coupling throughout menses time, “maternal impressions”, that’s sudden and robust impressions of the pregnant lady. The latter principle was supported by many scientists not solely of historical instances but additionally through the Renaissance, for instance, Paracelsus and Ambruas Pare′. Probably the most excellent thinker Aristotle beneficial to protect pregnant lady towards disagreeable impressions and encompass her with lovely objects that may promote the fantastic thing about the kid. Even though Aristotle proved the absurdity of the “hybrid theories”, there are paperwork that as much as the XVII century in Denmark and America there have been circumstances of execution of girls who gave delivery to kids with congenital malformations and who had been accused in coupling with animals or the satan. Nevertheless, already in these days there have been materialistic approaches regarding remedy of congenital malformations. Hippocrates defined the incidence of some congenital malformations by mechanical affect on the uterus (trauma, compression).

Johan Meckel Jr. (1781–1833) gave especial contribution to the event of the doctrine about malformations. He described a major quantity of malformations, which had been identified earlier than, created classifications for the malformations, tried to elucidate the explanations and mechanism of their formation. Meckel concluded that almost all of malformations aren’t whims of nature however penalties of broken regular growth of physique, that to a sure extent is the recurrence of phylogenesis.

Genetic science significantly enriched our data in regards to the aetiology and pathogenesis of congenital malformations and expanded alternatives of their prevention. Direct switch of some congenital malformations from mother and father to kids was identified even in historical instances. Nevertheless, scientific proof of the genotype principle started solely within the XX century, specifically after the second discovery in 1890 by De-Frieze, Korens and Chermak of the fundamental legal guidelines of heredity of G. Mendel. To start with in 1955 about 1,500 hereditary ailments and congenital malformations grew to become identified. Genetic technique of researches have gotten the widest software after the invention of chromosomal aetiology of some already identified syndromes (Down’s, Klinefelter’s, Shereshevsky—Terner’s) in 1959.

The examine of questions regarding malformations proves that every deviation from the traditional organism construction must be studied from two sides: the way it arose and why it occurred. Comparative and experimental fetology reply these questions.

The explanations of incidence of congenital malformations could be inside (endogenic) and exterior (exogenic). Endogenic causes:

adjustments within the hereditary constructions (mutation);

over-matured sexual cells;

age of fogeys.

Mutations can happen on the genic, chromosomal and genome ranges. Genic mutations are related to adjustments within the inside construction of sure genes and predetermine transformation of 1 type of allele into one other. With chromosomal mutations the next can happen:

interchromosomal change of segments;

doubling of the chromosomal web site;

breaking of the chromosomes with the lack of part of the chromosomal materials.

Genome mutations are a change within the quantity of chromosomes. A rise within the quantity of chromosomes is noticed extra typically, much less typically — discount. Genome mutations are accompanied by adjustments within the phenotype of the fetus and can lead to involuntary abortion or chromosomal diseases like Down’s, Edwards’, and so forth.

Exterior causes for the incidence of congenital malformations could be divided into 4 teams:

bodily components;

chemical components;

infectious-inflammatory components;

psychological components.

Bodily components: radiation affect. There isn’t a direct proof regarding the doses of ionizing irradiation acquired by the trendy particular person. Nevertheless, in experiments on mammals it’s proved that ionizing irradiation is the explanation for a lot of congenital malformations. The same impact for an individual will not be excluded.

Mechanical components which trigger congenital malformations are as follows: amniotic conjoins, superfluous uterus stress or a tumour on the growing fetus, within the case of oligoamnios or a big myoma.

Chemical components embody adversarial affect of alcohol, lead, mercury, nicotine, benzene, chloroform, deficiency or discount of magnesium salts, iron, iodine within the organism, insufficiency of nutritional vitamins A, D, E, B₁, C, in addition to hormones. Inadequate provide of an organism with oxygen has nice worth.

Infectious-inflammatory components are infections in early phrases of being pregnant: rubella, measles, epidemic parotitis, infectious hepatites, smallpox, poliomyelitis, and so forth.

Unfavorable components, which affect the event of the fetus and trigger congenital malformations, are lengthy absence of being pregnant, synthetic abortion, in addition to tuberculosis, syphilis and different ailments.

Throughout warfare the quantity of deformities will increase, which is a psychological issue for the incidence of congenital malformations.

In numerous nations the frequency of congenital malformations ranges from 1 for 83 newborns to 1 for 1587 newborns, i.e. on the common 1 congenital malformations for each 213 newborns. In the identical nation the quantity of congenital malformations relies upon upon race. In America the folks of the white race have the incidence of congenital malformations 2 instances as a lot than for the black race (5.72— in whites and— 3.17 in negros for 1,000 newborns). Congenital malformations of the auricle for folks of the yellow race are noticed extra typically than for the black race and is much more typically than for white folks.

Classification of Congenital Malformations and Anomalies

It’s accepted to tell apart single congenital malformations which concern infringements of growth of 1 organism and double (a number of) deformities, which consists in infringement of growth of two and extra fetuses that are within the uterus on the identical time.

There are distinguished the next teams of single anomalies:

anomalies of the sizes of the complete organism or sure organs:underdevelopment (hypogenesis, hypoplasia) or absence (agenesis, aplasia) of an organ or tissue; superfluous growth of an organ or tissue (hypergenesia, hyperplasia);

anomalies of the quantity of organs each in progress (polydactyly), and discount (bidactyly);

anomalies of the shape (talipes);

anomalies related with the stopped growth of organs, preservation of atavistic attributes, accretion or narrowing in uncommon locations: atresia, or stenosis of the anus; nonunion in corresponding zones of the growing fetus (mind hernias);

anomalies of localization — reverse lodging of organs (heterotopia) — coronary heart, appendix, liver, and so forth.;

Anomalies related to infringements of genital growth(true or synthetic hermaphroditism).

Double (A number of) Anomalies

If full division of a germ has taken place, monoovular twins develop and ship. In case of incomplete division of two main stria double anomalies come up. The embryos seem conjoined in variable positions (merging of the pinnacle, higher, center or decrease components).

If the foetuses are an identical in dimension, they’re referred to as symmetric; if one among them is kind of underdeveloped and related with one other, fully developed fetus, the anomaly is uneven (for instance, amorphous — shapeless anomaly, or polymelia, when just some extremities remained from the foetus which grew along with the opposite regular foetus.

Double anomalies are distinguished based on the way in which of cytotrophy: autositic — every fetus has an unbiased system of blood circulation;

omphalositic — every fetus nourishes by umbilical vessels;

parasitic — one of many fetus (parasite) has no unbiased blood circulation, thus on the physique of the fairly developed fetus there’s one other, much less developed fetus.

Based on the diploma of viability and life expectancy:

organisms which perish through the early germinal interval;

organisms viable through the intra-uterine interval however are notcapable of unbiased existence after delivery (some malformations of the center and the lung, GIT ailments, majority of the double anomalies;

organisms that are born fairly viable and might proceed to liveafter delivery (anomalies of the extremities, partial giantism, and so forth).

In 1975 on the XXIX World Meeting of Public Well being Companies, the classification was adopted the idea of which is anatomical-physiological precept of dividing an individual’s physique into the techniques of organs:

A. Congenital malformations of organs and techniques:

malformations of the CNS and sense organs;

malformations of the face and neck;

malformations of the cardiovascular system;

malformations of the organs of digestion;

malformations of the osteomuscular system;

malformations of the urinary system;

malformations of the genitals;

malformations of the endocrine glands;

malformations of the pores and skin and its derivatives;

malformations of the afterbirth;

different malformations.

B. A number of congenital malformations:

chromosomal syndromes;

genic syndromes;

syndromes that are predetermined by exogenous components;

syndromes of unsure aetiology;

a number of malformations of non-specified aetiology.

The quantity of congenital malformations reaches greater than 1,500, on this lecture we will focus our consideration solely on these noticed extra typically and provides some examples from every anatomic system.

The a part of congenital malformations within the CNS makes greater than 30% from all malformations noticed in kids. Anencephalia is the absence of the mind, bones of the arch of the cranium (acrania) and mushy covers of the pinnacle. With congenital malformations of growth of the arch of the cranium, mind and its membranes, mind hernias happen. Relying upon the contents, mind hernias are divided into 4 teams:

meningocele is a tumour-like formation is the medulla membranes stuffed with liquid;

encephalocele — the tumour accommodates mind tissue, arachnoid and vascular membranes;

cephaloma — extrusion of the modified mind substance;

encephalocystocele is a tumour which just like the mind tissue, accommodates spinal liquid.

Mind hernias are positioned in typical locations: within the entrance of the nostril bridge (anterior mind hernia), posterior, beneath or above the occipital protuberance (posterior mind hernia — superior if greater occipital protuberance, and inferior if beneath it). Apertures within the bones are often spherical with clean and glossy edges. They’re at all times smaller than the idea of the extrusion. Extrusions, as a rule, have a spherical type, elastic consistency, ceaselessly fluctuate. When pressed on, it decreases, pulsates. An amazing quantity of youngsters with mind hernias die quickly after delivery on account of meningitis or oedema of the mind.

Remedy: within the case of small hernias operation is indicated — they expose the idea of the extrusion, tie it, delete the entire extruding tissue and perform plasty of the defect within the cranium cavity.

Atelocephalia — absence of the massive hemispheres and subcortex nuclei — extreme defect of growth, which is seldom noticed.

Nanocephalia — discount within the mass and sizes of the mind (greater than 5 cm).

Macrocephalia (megalocephalia) — uncommon enhance in mass and dimension of the mind ceaselessly accompanied by oligophrenia.

Hydrocephalia — congenital oedema of the mind, superfluous accumulation within the ventricular system or arachnoid area of spinal liquid accompanied by atrophy of the mind substance.

Defects of Improvement of the Face and the Neck

Infringements of growth of the face, the auricle and the neck are various based on each aetiology and incidence and severity diploma. A few of them are accompanied by solely beauty defects (for instance, double lip) or stay completely imperceptible (epicant), others result in extreme purposeful infringement within the neonatal interval, inflicting excessive lethality in kids. Defects of this group mix with infringements of growth of different techniques organs, the residing prognosis relies upon upon their severity.

Deformities of the face happen, primarily, as the results of regular accretion of the clefts stopping within the germ, which happen at this era. Accretion happens, as a rule, on the second month of the intrauterine life.

Cleft lip or hare lip is noticed in one among 2,500 newborns. It may be unilateral or bilateral. Unilateral cleft could be on the appropriate or left (extra typically on the left). Remedy is surgical. Completely different plastic operations are utilized relying upon the defect. Operations could be carried out 2 days after delivery, however extra typically six months after delivery.

Macrostomia — abnormally massive dimension of the mouth; could be unilateral or bilateral. It’s accompanied by hypersalivation. Remedy — stitching collectively the corners of the mouth.

Palatoschisis — cleft palate, “wolf mouth” could be full (cracks within the mushy and laborious palate), partial (solely within the mushy or solely within the laborious palate), midline, uni- or bilateral, by or submucous. There may be 1 case in 1,000 newborns. The reason being delayed accretion of maxillary processes and vomer.

Remedy relies upon upon the severity, presence of different defects, consists of not solely surgical remedy (correction) but additionally common dispensary supervision of the kids’s physician and the logopedist and if mandatory different consultants (neuropsychiatrist, otolaryngologist). Cleft palate in kids on the age of as much as 6 months is corrected with obturator, plastic operations are carried out for kids of three– 6 years outdated.

Congenital vascular tumours of the face and neck — hemangioma and lymphangioma are ceaselessly noticed.

Hemangioma — purple-red, typically cyanotic shade spots or tumours.

Lymphangioma — clinically seems as tumescence which typically reaches huge sizes. The pores and skin above the tumour is skinny and a colorless liquid seems by it.

Congenital defects of the auricles are noticed seldom, 1–2 circumstances in 10,000 newborns. These remoted infringements of growth have relatively small scientific worth. The infringements of growth of the auricle with syndromes vary from deformations and infringements of the reduction to tough deforming dysplasias and aplasias.

Congenital Defects of Improvement of the Neck

Brief neck — truncation of the neck often happens on account of flattening of vertebrae corpus or intervertebral cartilages. It’s noticed with many chromosomal ailments.

Congenital muscular wryneck — shortenning of the sternocleidomastoid muscle due to focal fibrosis; subsequently the kid’s head has an inclination to the broken muscle. The explanations are unknown. Defect happens typically — 12% of all defects of the support-motor system. Remedy is surgical in early childhood.

Median cysts and fistulae of the neck are cavities from the rest of the thyrolingual passage. They find beneath the pores and skin on the web site of midline of the neck, between the thyroid cartilage and lingual bone. Dimension of the cysts is 1 cm.

Lateral cysts of the neck find alongside the sting of the posterior stomach of the digastric muscle or alongside the anterior line of the sternocleidomastoid muscle. Lateral cysts of the neck type from the nonreduced remainders of two branchial cleft and pharyngeal recess.

Defects of the Thorax

Full cleft of the sternum is noticed seldom, mixed with the center prolapse.

Extrusion of the sternum doesn’t trigger the change within the organs of the thorax and isn’t topic to surgical remedy.

Absence or superfluous quantity of ribs has no scientific shows and is extra typically recognized casually.

Defects of Improvement of the Thoracic Organs

Coronary heart and vessel ailments is a bunch of probably the most widespread defects, which totals tens of nosological varieties. Incidence: 6–10 circumstances in 1,000 newborns.

Acardia is the absence of the center. It’s noticed solely in uneven free twins.

Cardiac ectopia is lodging of coronary heart outdoors of the chest cavity. Cervical, stomach and extrasternal ectopia are distinguished. The latter makes up 51.2% of all cardiac ectopia. Stomach ectopia often has an asymptomatic course, different defects lead to loss of life.

Dextracardia — coronary heart location on the appropriate. It’s noticed in inverse location of all inside organs and is a uncommon remoted defect.

Coarctation of the aorta — narrowing of the isthmus of the aorta. 1 case in 6,500 newborns is noticed. This defect is twice as typically in males than in ladies. Coarctation of the aorta seems as hypertension of the higher a part of the physique and hypotension of the decrease one. First attributes are noticed on the age of 10 months. Remedy is surgical with full correction of the defect.

Aortic stenosis. The narrowing could be noticed each on the valve degree and on the preliminary division of the left ventricle, much less typically — greater than the valves.

Defect of interatrial membranes is among the most widespread coronary heart ailments: 1 case in 1,000 newborns. It’s noticed in ladies extra typically than in males.

Three-chambered coronary heart with one normal atrium is the absence of the interatrial membrane. Sufficient uncommon and often deadly defect which is related to different coronary heart and huge vessels ailments. Remedy is surgical.

Fallot’s tetrad — stenosis of the pulmonary trunk, excessive defect of the interventricular membrane with the diameter of two cm, dextroposition of the aorta. The fourth element of the defect is hypertrophy of the appropriate ventricle. Scientific shows: cyanosis (amplifies with years), dyspnea, tough systolic noise within the second and fourth intercostal on the left. Prognosis is unfavourable. Extra typically kids die on the early age from infringements of blood circulation within the mind, however typically dwell 20–30 years. Remedy is surgical.

Fallot’s triad — valval stenosis of the pulmonary artery related to the interatrial membrane defect and hypertrophy of the appropriate ventricle.

Transposition of vessels — the aorta comes out from the appropriate ventricle, the pulmonary arteries — from the left one. With the absence of shunts (defects of the membrane, open arterial passage) the defect will not be appropriate with life. Prognosis is unfavourable. Youngsters die beneath the age of 1. Remedy is surgical.

Anomalies of the valves are typically the compound parts of complicated congenital coronary heart ailments. Foraminous valves, valves with recesses, in addition to enhance and reduce within the quantity of apertures of valves aren’t accompanied by haemodynamic infringements. Aplasia of the apertures of the semilunar valves, particularly the aorta, is extra typically noticed.

Defects of Improvement of the Respiratory System

Defects of the throat aren’t frequent and seem typically as infringements of growth of the cartilaginous constructions (thyroid cartilage, epiglottis), similar to aplasia, hypoplasia, infringements of accretion, and so forth.

Tracheomegalia is a rise within the trachea, primarily due to an enlargement of its aperture. Remedy: conservative.

Pulmonary agenesia (aplasia) — absence of the lung and the primary bronchial tube. It happens extra typically in boys. Left-side agenesia is noticed twice as typically than the right-side one. Remedy is symptomatic.

Pulmonary hypoplasia is a relatively uniform discount of pulmonary mass and quantity.

Bronchogenic (disontogenic) cysts — spherical cavities of various sizes separated from the encircling pulmonary tissue. Probably the most typically lung defect. Cysts could be solitary and a number of.

Congenital diaphragmal hernias is a transferring of the stomach organs into the chest cavity. It may be true or synthetic (extra typically). In case of synthetic hernias the hernial bag is absent. Remedy is surgical, with the presence of synthetic hernias — emergency.

Defects of Improvement of the Digestive Organs

Within the esophagus atresia is noticed, which is often accompanied with the formation of tracheoesophageal fistula. The next defects might develop:

two segments, which blindly come to an finish;

one phase, that involves an finish blindly, and the second,

which opens into the trachea;

two segments, which open into the trachea;

anastomosis between the trachea and esophagus.These defects are accompanied by aspiration pneumonia.

Within the abdomen stenosis of the pylorus (pylorostenosis) is noticed extra typically.

Within the intestines stenosis and atresia, single or a number of, type.

Extra typically they find within the duodenum and on the place of transition from the ileum into the cecum, in addition to on the distal areas of the rectum. Full or partial doubling of the intestines each the small and huge, and doubling of the appendix are sometimes noticed.

Dolichosigma is a congenital prolongation of the gut with out its enlargement. The sigmoid colon, as a rule, varieties 2–3 further folds and extra.

Megasigmoid is a frequent defect (Hirschsprung’s illness). This enlargement and hypertrophy of part of the colon, typically the sigmoid, is related to its peristalsis infringement. Remedy is surgical, consists in removing of the aganglionic zones and pathological modified expanded websites situated above the positioning.

Two defects of growth of the intestines are related with the preservation of the embryonic constructions. It’s hernia of the umbilical twine and fistula on the navel space. With partial preservation of passage, Meckel’s diverticulum varieties. It’s much like a glove’s finger and exits from the ilea at a distance of 25 cm and extra from the Bauhin’s valve.

Congenital defects of growth of the biliary tracts encompass an uncommon place of the gallbladder and its dimension, incidence of cysts of the widespread biliary tract and atresia or stenosis of one of many biliary tracts. The latter is related with intrauterine hepatitis subsequently biliary cirrhosis of the liver takes place.

Anorectal anomalies are noticed in 0.25–0.66% of the circumstances, twice as typically in ladies than in boys. The next anorectal anomalies are distinguished:

Ectopia of the anus (perineal and vestibular).

Congenital fistulae within the sexual and urinary techniques or in theperineum within the case of usually developed anus.

Congenital narrowing of the anus, rectum, anus and rectum atthe identical time; happens extra typically in boys.

Atresia:

easy;

with fistulae within the sexual or urinary system;

casuistics (congenital cloaca, atresia and doubling of the rectum).

Defects of Improvement of the Urinogenital System

Defects of growth of the urinogenital system are ceaselessly noticed. They make 30% all circumstances of congenital defects. Anomaly of the quantity of kidneys can seem bilateral arenia (renal agenesia), that’s the absence of kidneys. Boys are extra typically affected. The kid has a attribute face (“Potter face”): extensively positioned eyes, low positioned huge ears, thick nostril, insignificant epicant.

Unilateral arenia is the presence of 1 kidney. It could possibly have a traditional construction or have defect of growth: dysplasia, hypoplasia, ectopia, doubling. Clinically these defects seem by accident or within the case of illness of 1 kidney.

The extra kidney has a traditional construction with a separated excretion and vascular system. Often it’s smaller in dimension and positioned beneath the traditional kidney.

Anomalies of place, type and orientation of the kidneys are as follows:

— dystopia (ectopia) — irregular location of the kidney. With easy dystopia the kidney is positioned on the identical facet however in an uncommon place, with cross — it’s shifted to the midline with a crossing of the ureter. Dystopia is critical to distinguish from nephroptosis, throughout which the renal artery leaves from the standard place, and the ureter has a traditional size. Accretion of the kidneys could be symmetric and uneven; they will develop collectively on the higher, decrease and completely different poles. Horseshoe, biscuit-like, L- or S-like kidneys type. This defect is noticed twice as typically in males. Virtually all accretion of the kidneys are malrelated. Anomalies of the renal construction are as follows:

а) renal dysplasia could be easy and cystomic, after the localization — cortical, medullar, cortico-medullar; after the spreading — focus, segmentary and whole, uni- or bilateral;

b) renal polycystosis is a congenital defect characterised by a number of cysts within the renal parenchyma with the absence of dysplasia.

Agenesia of the urinary bladder is a defect which leads to loss of life.

Doubling of the bladder — anomaly which is seldom noticed.

Diverticula of the bladder is extrusion of its partitions which come to a blind finish. Diverticula are single and a number of.

Extrophy (ectopia) of the bladder is a congenital crack of the bladder and stomach wall. The posterior wall of the bladder which is roofed with a velvety mucous membrane extrudes by the defect within the stomach muscular tissues to the skin. The opening of the ureter is gaping.

Patent urachus — an open passage of the alantois:

patent urachus from the navel to the bladder with a urinaryfistula which opens into the navel;

urachus obliterated within the umbilical phase; defect has no scientific shows;

partially patent urachus with an obliteration of the ends and open center space (cyst urachus).

Remedy for a complete patent urachus is surgical through the first days and months of life.

Hypospadia — the decrease crack of the urethra. One case in 300 new child boys is noticed. There are distinguished 5 types of this defect: “hypospadia with out hypospadia”, coronal, penile, scrotal and perineal. For all types of hypospadia the curvature of the penis, shift of the exterior aperture of the urethra are typical. Relying upon the shape the exterior aperture could be positioned on the penis from the underside floor of the caput to the perineum. Within the case of perineal and scrotal varieties, the scrotum splits, cryptorchism, broad go to the urethra, reminding the doorway to the vagina, are noticed. It could possibly result in misguided figuring out the affected person’s intercourse. In 8– 10% of the circumstances, hypospadia is accompanied with hermaphroditism. Remedy is surgical.

Epispadia — prime crack of the urethra. It’s accompanied with curvature of the penis, its pulling up and retraction into the encircling tissue.

Aphalia (agenesia, aplasia) of the penis is a really uncommon defect (one case in 30 million newborns). Thus, the ureter opens into the rectum or on the pores and skin of the perineum.

Macrophallus — enhance within the penis — defect that’s seldom noticed. Sexual glands, as a rule, aren’t modified.

Microphallus — sharp shortening of the penis. It’s accompanied by quick corpus cavernous and a small caput, cryptorchism.

Phimosis is congenital narrowing of the prepuce, stopping its being drawn again over the glans. It’s sophisticated by balanoposthitis, formation of concrements, ulcers and sores of the prepuce. Remedy is conservative and surgical through the neonatal interval.

Paraphimosis — pinching of the prepuce. This defect is a complication of phimosis. Remedy consists in pressing operation.

Cryptorchism — delay within the testicles reducing into the scrotum. If one testicle doesn’t decrease — monorchism, with whole absence of the testicles — anorchism. Complication of those defects: infertility. Remedy is surgical: reducing the testicle into the scrotum as much as 2– 3 years of age.

Congenital defects of feminine genitals are ceaselessly noticed.

Vaginal atresia (lumbar membrane of the vagina) is mixed with atresia of the anus, completely different urinogenital fistulae and anomalies of the urinary system.

Anomalies of the uterus:

cervical agenesia is noticed seldom;

agenesia of the uterus — full absence of the uterine corpus

with regular feminine karyotype — noticed extraordinarily seldom, it seems throughout sexual maturing in reference to amenorrhea.

Hypoplasia of the uterus (rudimentary uterus, infantility). There are three levels of anomaly: germinal uterus (size as much as 3 cm); childish (3–3.5 cm) and teenage (5–7 cm). The uterus thus has a superfluous ahead bend and a conic cervix. This defect is accompanied by menstrual cycle problems and infertility.

Doubling of the uterus (bifurcation of the uterus) — the cervix and the each vaginas are grown collectively.

The double uterus is characterised by the presence of two separated uteruses, every of which joins the corresponding a part of the doubled vagina.

Saddle-shaped uterus is the defect the place the underside of the uterus doesn’t have a regular rounding. Anomaly of the ovaries is characterised by their absence (agenesia), underdevelopment (hypoplasia), infringement of growth (dysgenesis). Absence of two ovaries (anovaria) is described solely in nonviable fetuses. It’s often accompanied with underdevelopment of the genitals.

Synthetic hermaphroditism is discrepancy between construction of the gonads and the construction of the exterior genitals.

Synthetic male hermaphroditism — the sufferers have testicles, however the exterior genitals are fashioned by the feminine kind. Three types of anomaly are distinguished:

feminism the place the sufferers have feminine kind of structure;

virilism or masculinism; kind of structure is masculine;

eunuchoidism (eunuchoid kind of structure) — absence of the event of breast and secondary pilosis.

Synthetic feminine hermaphroditism — sufferers have ovaries, and exterior genitals develop by the masculine kind. Extra typically attributes of feminine hermaphroditism are noticed in circumstances of pronounced hyperplasia of the cortex of the adrenal glands. Remedy — hormonal, needs to be appointed as quickly as attainable.

True hermaphroditism (ambisexuality, bisexuality) — presence in a single organism of intercourse cells of each sexes, and in addition two sexual techniques. Remedy: hormonal and operative, relying on the need of the affected person.

Defects of Improvement of the Locomotor System

Based on the Worldwide Classification and Nomenclature of the Constitutional Illnesses 5 primary teams of skeletal ailments are distinguished:

Osteochondrodysplasia — infringement of progress and growth of the cartilage or bone.

Distosis of the bones.

Idiopathic osteolysis — lisis of bones with secondary deformations.

Chromosomal aberrations with uncommon skeletal anomalies.

Major metabolic defects (infringement of metabolism).

An enhance within the quantity of vertebrae is noticed within the lumbar and sacral areas of the spine extra typically.

Further sphenoidal vertebrae or half-vertebrae. The defect is characterised by the presence of lateral or posterior further halfvertebrae noticed within the thorax a part of the spine extra typically.

Patent (aplasia) vertebrae arculus with aplasia of the spinous course of (spina bifida).

Kyphosis — curvature of the backbone, with backward convexity. It may be whole or native.

Lordosis — curvature of the backbone with convexity wanting anteriorly.

Flat again — obturated physiologic curvatures of the backbone.

Scoliosis — lateral curvature of the backbone which mixes with its torsion.

Anomalies of the ribs are noticed seldom. Aplasia or further ribs are largely met.

Anomalies of the Extremities

Discount defects consist within the stoppage of formation or inadequate formation (inadequate progress) of the skeleton components.

Cross-section discount defects of the extremities (congenital amputations). Noticed at any degree of the extremity: shoulder, hip, forearm, shin, wrist, fingers and phalanxes. Distal a part of the extremity (beneath the amputation) is absent fully. Remedy relies upon upon the extent of amputation and consists in prosthetics.

Cross-section terminal aplasia (hemimelia, extromelia) — absence of the distal a part of the extremity (amputation kind) at any degree: hemibrachia — absence of the forearm, achyria — arms, apodia — foot, adactylia — fingers, oligodactylia — a number of fingers, aphalangia — phalanxes.

Focomelia (seal-like extremities) — full or partial absence of the proximal components of the extremity. 3 varieties are distinguished: proximal — aplasia of the humeral or femoral bone; distal — aplasia of the bones of the forearm, shin; full — aplasia of the entire lengthy tubular bones. Palms or toes on this case are hooked up on to the trunk, reminding a seal’s fin.

Amelia — full absence of the extremities. Absence of each higher extremities — abrachia, one of many higher extremities — monobrachia, each of the decrease extremities — apous, one of many decrease extremities — monoapous. Remedy is with the assistance of prosthetics.

Splitting of the hand, foot (ectrodactylia, chelate hand, chelate foot, lobster hand) — aplasia of sure central parts of the hand, foot with the presence of deep sulcus as an alternative of absent bones. Monodactylism — presence of 1 finger on the hand or foot.

Polydactylia — enhance within the quantity of fingers on the hand or foot.

Syndactylia — incomplete discount or absence of discount of the interdigital membranes.

Syrinomelia (sympodia, symmelia, caudal regression syndrome) — merging of the decrease extremities. Each mushy tissues and bones can merge.

Congenital talipes (inside talipes) — steady adducting-bending rigor contraction of the toes, which is related with congenital underdevelopment and shortened inside and posterior teams of ligaments, corresponding flexors and muscular tendons, in addition to with infringements of muscular synergism. The method is often bilateral. Primary scientific attributes: equines (plantar bending of the foot on the ankle joint), supination (returning of the plantar surfaces of the foot with reducing of the exterior edge as help), adduction (discount of the anterior a part of the foot) enhance within the arch of the foot (empty foot); flat toes — flat arches — frequent congenital defect.

Horse foot — rigor contraction of the ankle joint in place of superfluous plantar bending; subsequently throughout strolling resistance falls on the fingers and the heads of the metatarsal bone. Remedy consists in handbook correction of the foot inside the first days after delivery, therapeutic massage and bandaging with mushy bandage into the appropriate place. At 5 years the closed correction of the foot (redressment) is carried out and to maintain it within the appropriate place a plaster bandage is utilized. If conservative measures fail, they perform surgical remedy on the tendons of the toes or resort to sphenoidal or crescent resection of the bones. These operations are carried out upon the top of bone progress.

PLASTIC OR RESTORATIVE SURGERY

Cosmetic surgery has existed because the historical instances and corrects, primarily, defects which disfigures an individual. In historic paperwork there’s a certificates regarding software of tissue transplantation with the aim of correcting face defects. Greater than 8 thousand years in the past in historical Egypt operations regarding the nostril restoration had been carried out. Within the e-book of the excellent Indian scientist Sushruta who lived in 1000 B.C., is written about plasty of the nostril by taking a pores and skin orifice on the pedicle from the brow or cheek and returning it to the place of defect. This sort of plasty is known as “Indian plasty”. That point the circumstances of rhinoplasty in Rome, Greece, Egypt are described. The explanation for growing pores and skin graftings in historical folks, apparently, was the customized of incision off noses in criminals and prisoners of warfare.

In Europe cosmetic surgery started to develop through the Renaissance. In Italy in 1450 a navy physician Branka began to hold out rhinoplasty with the assistance of native tissue (pores and skin of the brow, cheeks). His son Antony transferred a flap on the pedicle from the distant websites. He used the flap on the pedicle from the pores and skin of the shoulder. The tactic is named “Italian plasty”. In 1802 M. І. Pyrogov instructed osteoplastic operation and developed the strategy of making a functioning stump in case of amputation of the foot. In 1865 R. Okay. Shimanovsky revealed in Kiev a handbook on pores and skin grafting. In 1872 S. M. Yanovich-Chainsky supplied a option to transplant pores and skin course of in a web site of granulation. V. P. Filatov supplied the strategy of pores and skin grafting with the assistance of tubed pedicle flap, which is extensively unfold. The works on cosmetic surgery: M. V. Sklifosovsky, M. M. Petrov, A. A. Limberg, M. A. Bogoraz, M. M. Blokhin, and so forth are well-known.

The cosmetic surgery makes use of a lot of strategies for eliminating defects because of which the organs restore. Relying upon the very fact whether or not the personal tissue is transferred into the organism, or it’s taken from a donor, whether or not it’s international for the residing organism, the next sorts of plasty are distinguished:

— autoplasty — transplantation of tissues taken from the identical affected person. Throughout autoplasty the tissue can switch on the physique with preservation of the nutritious pedicle with a mother or father foundation (involuntary plasty), or is transferred on one other place fully from the distant websites of the physique (free plasty);

— isoplasty — transplantation of tissues or organs that are taken from monoovular twins. Tissues and organs of such twins are genetically relatively related, subsequently tissue restoration happens in addition to with autoplasty;

— homoplasty — transplantation of tissues and organs from one particular person to a different one. Thus, as a rule, it’s carried out in a free approach;

— heteroplasty — transplantation of tissues and organs taken from people of one other type. For instance, from an animal to folks

(xenoplasty);

— alloplasty — if the transplanting tissue will not be of animal origin (steel, artificial supplies).

Relying upon the type of transplanting tissue, pores and skin, muscular, tendinous, nervous, bone, cartilaginous, vascular and organ plasty are distinguished. Completely different sorts of tissues and organs could be mixed additionally: skeletal-muscular plasty, liver or pancreas transplantation, and so forth.

Tissue Incompatibility and Methods of its Prevention

Throughout tissue and organ transplantation from one particular person to a different the actual engraftment by no means takes place. The primary difficulties throughout transplantation aren’t due to scarcity of operative method (lately it has develop into excellent and surgeons are capable of substitute nearly complete complexes of organs), however they consist within the immunological nature of every particular person which genetically differs from one other. Exceptions are solely monoovular twins. Allo- and xenogenic transplants on account of protecting immunological reactions are rejected by the recipient’s organism. Sure chemical structural parameters, by which the donor and recipient’s cells differ, are often chargeable for this rejection. They’re referred to as transplantation antigens or antigenes of tissue incompatibility. Antigens of blood teams erythrocytes and leukocytes belong to them. The latter make the primary complicated of antigenes of tissue incompatibility — HLA system in an individual (human leukocyte antigen). The cytotoxic motion of Т-killers is directed on these antigens.

Genetic researches concluded that transplantation antigens of an individual have 5 locuses — serologically they’re decided as A, B, C — and lymphocytes, decided as D and DB. Antigens of each locus are allelic; their inheritance is carried out codominant based on Mendel’s legal guidelines. Every particular person can have solely two antigens of 1 locus. For homozygous people for each locus one transplantation antigen is critical. The existence of dense couplings throughout inheritance is proved. Crossing-over is noticed seldom.

Locus A supervises the inheritance of 20 antigens, locus B — 42, locus C — 8, locus D — 12, sublocus DB — 10 completely different antigens.

Statistical likelihood of reliability that two unrelated people could be an identical is just too small (about 1%). For shut family members this opportunity is a little bit greater and reaches 25% for sisters and brothers.

The weaker the compatibility of НLA — antigens of the donor and recipient — the stronger the pronounced protecting response — response of rejection in a recipient. The success of transplantation of organs and tissues relies upon initially upon true alternative of the donor by transplantation antigens, that’s typing of those antigens.

At present there are three strategies of kind:

leukoagglutination response;

lymphatic take a look at;

blended leukocytes response.

Prevention of incompatibility of tissues and organs is carried out by three primary instructions:

Collection of the donor and recipient based on isoserological properties. However appropriate pairs are noticed one in some tens of 1000’s, which troubles the selection.

Adjustments in immunobiological response of the recipient to transplant. It’s essential to do not forget that oppression of the protecting immune response leads to weakening and destruction of the transplant from the best an infection, for instance flu. There have been makes an attempt to irradiate the recipient. Nevertheless, the rejection was not noticed solely with the dose inflicting radiation illness. The applying of various chemical, organic and hormonal (steroid) hormones doesn’t consequence within the mandatory impact, they solely delay the life time period of the transplant, with out offering engraftment.

Affect of various components on transplant causes weakeningof inverse response of the corresponding organism and reduces its antigenic properties. The transplantation of low-differentiated tissue (cornea, bone, cartilage, fascia) happens higher than transplantation of complicated tissue with intensive metabolism. Many means have been utilized to scale back the antigenic properties of transplants (irradiation, motion of varied chemical substances, and so forth.), however the perfect outcomes have been acquired with the appliance of the lyophilic drying technique. It’s marked that tissue taken from corpses (that are within the “existance” stage), have much less pronounced properties and yield the perfect outcomes. However the organs acquired from an alive donor heal higher.

Pores and skin transplantation is probably the most important division of cosmetic surgery. Autoplasty is utilized extra typically. There are two sorts: flap protection of the wound and free pores and skin grafting.

Flap protection of the wound — the pores and skin flap on the pedicle, which nourishes it, along with fatty tissue wherein blood vessels go which provide the flap are discovered. The flap’s pores and skin needs to be broad, unbent with out superfluous tightness, uncompressed by a bandage. The most straightforward type of pores and skin grafting is freshening and tighting the sides of the wound. For this function further incisions are made, a triangular, oval and other forms of flaps are created, that are transferred on the nutritious pedicle.

Plasty on the pedicle is utilized when the tissue positioned close by will not be sufficient. Pores and skin flaps are taken close to the defect, or transferred from distant websites of the physique (hand, breast, neck, and so forth.).

For cheiloplasty and meloplasty they apply Joseph’s, Esser’s, Lexer’s strategies (they’re utilized seldom due to the formation of further scars). The “Italian” technique of plasty on the pedicle is justified. On the shoulder or forearm they minimize a tongue-like flap. The wound beneath the flap is sutured, the hand is dropped at the face and the flap is sutured to the tissue of the nostril. After therapeutic, the pedicle is separated and a mandatory organ is developed.

Bridge-like flap plasty based on G. V. Sklifosovsky and Zontag. The flap has nutritious legs. This sort of plastic yields good outcomes, however is restricted regarding software.

Plasty based on V. P. Filatov. They minimize the flap on the stomach, buttock and suture as a tube. One of many ends is squeezed for five–10 min, in order that the blood circulation comes from the alternative finish. Then they minimize one finish, suture within the web site of anatomic snuffbox on the hand. After therapeutic the flap is moved to the defect and sutured. In 3 weeks the flap is separated from the anatomic snuffbox and positioned above the defect. The size of the flap shouldn’t be better than 10 cm. With the assistance of this technique plasty of the nostril, lips, cheeks, ears is carried out; it’s utilized for “wolf mouth”, noma, and so forth. For rhinoplasty it’s attainable to make use of additionally “4 legged flap”. For plasty of ears auto- and homocartilage are implanted into the Filatov’s tube flap. The Filatov’s tube flap is utilized for plasty of the esophagus, vagina, urethra, and so forth. Extensive recognition of this technique is acquired in plasty of trophic ulcers and extremities stumps which don’t heal. The defect of this technique consists in important thickness of the flap and heterogeneity of the pores and skin shade.

Free Pores and skin Grafting

The most straightforward strategies of free pores and skin transplantation are Reverden’s, Yanovich—Tchainsky’s strategies: with a razor they minimize slices of the pores and skin with the scale of two–5 mm along with papillary layer and place them on the granulating floor.

The flap technique based on Tirsh is as follows — the flaps of the pores and skin are positioned on the floor of preliminary deleted granulation. Above a dry bandage is utilized or this place is put within the ATU isolator.

The tactic of plasty with the assistance of a perforated flap. They take a pores and skin flap, make perforating apertures in a chessboard order, then suture the flap. Lately plastic pores and skin flaps taken with a dermatome (handbook, electrical, pneumatic, and so forth.) are utilized.

Pores and skin homoplasty is utilized for sufferers with burns wherein it’s unattainable to hold out autoplasty due to the extent of damage. For homoplasty they use each recent and preserved transplants.

Xenoplasty. For this sort of plasty typically pig pores and skin is used. In our clinic xenoplasty is used even for sufferers with wounds that don’t heal for a very long time, for sufferers with diabetes. Often such pig pores and skin flap rejects, however the defect significantly decreases in dimension and subsequently epithelizes quick.

Bone Tissue Transplantation

Transplantation of the bone could be carried out on a nutritious pedicle from mushy tissue and as free transplanted auto-, homo- and heterotransplants.

Involuntary bone plasty for the primary time was executed by M. І.

Pyrogov throughout osteoplastic operations of the shin with the appliance of the heel for help functions. Autograft without spending a dime bone plasty is taken from the tibia extra typically.

Homoplasty of the bones is utilized after lyophilization or quick freezing on the temperatures of –70 …–196°C . Such a bone is preserved on the temperature of –25 …–30°C . Such a bone graft stimulates the regeneration of the recipient’s bone and resolves in 2–3 years.

Bone marrow transplantation is utilized as autoplasty and homoplasty. With autoplasty the bone marrow is taken a few days earlier than the operation or irradiation. It’s preserved by the strategy of freezing. After irradiation with hypoplasia of the bone marrow they defreeze the transplant and substitute it to the identical affected person. It’s higher to enter bone marrow intercostally even throughout homoplasty, which is extensively utilized for radiation illness.

Myoplasty

Myoplasty is extensively used to cease bleedings from parenchymatous organs (the liver, the kidneys). They use the rectus abdominus muscular tissues, and so forth. They seal up the medulla membrane sinus, osteomyelitic cavities.

Tendon Grafting

This plasty is utilized primarily with autotendons for regenerating the ligamentous equipment of the joints, for instance, cruciate ligaments of the knee joint.

Plasty of the Fascia

The broad fascia of the hips, which may shut defects of anterior stomach wall throughout central hernias, defects of the diaphragm, esophagus are used extra typically for plasty of fascia. Strips of fascia are used for strengthening the anus, and so forth.

Plasty of the Omentum

The massive omentum is used to shut the wounded parenchymatous organs which bleed, for closing huge perforating apertures of the abdomen and duodenum, for the creation of vascular natural anastimosis, with cirrhosis of the liver and obliterating endarteritis of the decrease extremities.

Peripheral Nerves Grafting

4 sorts of operations are used:

neurolysis;

neuroraphia;

neurotization;

nervous trunk grafting.

The case when the broken nerve is united finish to finish by making use of perineural stitches made from skinny silk is right. Whether it is unattainable to attach the ends of the nerve, they resort to transplants utilization, largely homoplasty.

Angioplasty

There are various means of stitching vessels collectively. All of them are directed on stopping the narrowing of apertures of vessels and the incidence of a blood clot in it. In clinics very often stitching gadgets that enable to stitch an aperture of a vessel from 2 as much as 15 mm are used. To exchange the defect they apply auto-, homo-, or allografts. It’s higher to make use of a vein within the case of autoplasty. Lister and N. O. Bogoraz carried out the vein transplantation for the primary time in clinic. However it’s essential to do not forget that veins have valves, subsequently veins needs to be circled. Lately arterial shunts are used, that’s making use of roundabout vascular anastomosis.

Organ Transplantation

The thyroid gland transplantation was carried out for the primary time by Schiff and Kocher in 1883. With the absence and inadequate perform of the gland, individuals are transplanted free items from one other particular person leading to excitation of the glands important exercise within the recipient. It’s higher to transplant the glands on a vascular pedicle; the parathyroid glands, ovaries, pancreas and different endocrine organs could be changed.

At present as a result of growth of microsurgery and technical coaching of surgeons, qualitative anaesthesia there appeared alternatives regarding the transplantation of not solely remoted organs but additionally complete complexes of organs (coronary heart with lungs, liver with pancreas, and so forth.).

Renal transplantation have been carried out already for a very long time. There may be an proof {that a} transplanted kidney lives in a recipient’s organism greater than 10 years, particularly if the donor is a monoovular twin.

As a result of broad growth of vascular surgical procedure and software of synthetic blood circulation apparatuses there is a chance for the center transplantation operations. Some thousand of operations have been carried out already on the earth. However the issues regarding the authorized legal guidelines come up. The truth is, for good outcomes it’s essential to transplant a “beating” coronary heart. Due to this fact, when getting a coronary heart from the donor it’s essential to have the family members’ written approval.

Microsurgions transplant the fingers, the hand, the suture torn off higher and decrease extremities with constructive distant outcomes.

Alloplasty in Surgical procedure

For the primary time in 1542 Vesalius renewed circulation within the artery with the assistance of a cane tube. At present in vascular surgical procedure numerous synthetic vessels of lavsan, teflon, dacron and different artificial supplies are very extensively used. Over 50 years traumatologists efficiently join bones with the assistance of steel nails, screws, stirrups, wires, silk, kapron, and so forth. Orthopedists apply synthetic joints, vascular surgeons — coronary heart valves, and so forth.