NEONATAL SURGERY - 1976

2. SPECIAL SECTIONS

8. Malformations of Individual Organs and Systems

Genitourinary system

   Emergency Urological Syndromes in Newborns

Emergency urological syndromes in newborns include disorders of urine production and urination, bleeding from the Urinary Tract, and the symptom of a palpable abdominal mass.

Complete cessation of urine production in newborns may result from anuria or Acute Urinary Retention.

Anuria — the cessation of the renal excretory function — is a symptom of many pathological conditions. Prerenal, renal, arenal, and subrenal forms of anuria are distinguished (Scheme 1).

Class="center">Scheme 1. Forms of anuria

Prerenal anuria is caused by extrarenal factors. It develops As a result of hemodynamic disorders and renal parenchyma ischemia in acute cardiovascular failure, Blood loss, renal vessel thrombosis, and Shock. Depending on The Nature of the shock-inducing factor, posttraumatic, reflex, hemolytic, toxic, and infectious anuria are distinguished. A frequent cause of ACUTE RENAL FAILURE in newborns is persistent vomiting and prolonged diarrhea, which lead to Water and Electrolyte imbalance, dehydration of the infant, and a decrease in circulating blood volume.

Arenal anuria is a consequence of renal agenesis or severe congenital renal diseases (Polycystic Kidney Disease, multicystic dysplastic kidney of a solitary kidney, etc.).

Renal anuria is associated with specific nephrotoxic or nephrosensitive damage. This type of anuria in newborns frequently occurs against the Background of infectious diseases, as a complication of Pneumonia, purulent-inflammatory processes, or Sepsis. A direct nephrotoxic effect may be exerted by sulfonamides and Antibiotics. Anuria of renal origin is observed in the terminal stages of various kidney diseases.

Subrenal anuria occurs when urine outflow from the kidney is impaired. Its most common cause is the occlusion of the ureteral lumen or compression of the urinary tract. Obstructive lesions can be localized at any level of the urinary tract. A rather frequent cause of subrenal anuria in newborns is uric acid diathesis, leading to ureteral obstruction by salts.

The clinical course of neonatal anuria is characterized by The Development of symptoms of renal failure, the identification of which is extremely difficult. Therefore, Treatment usually begins with symptomatic therapy aimed at stimulating renal function, correcting water-electrolyte balance and acid-base equilibrium. Simultaneously, measures are taken to eliminate azotemia, along with gastric and intestinal lavage using a 2% soda solution or fluids intended for peritoneal dialysis. If conservative treatment proves ineffective, indications arise for The Use of extracorporeal purification Methods: peritoneal dialysis and hemodialysis.

In pediatric urological practice, a fractional method of peritoneal dialysis is used by introducing 100 — 150 ml of dialyzing fluid into the Abdominal cavity, changing it every 45 — 60 min. For this purpose, the anterior abdominal wall is punctured with a trocar, and a multi-hole catheter tube is inserted into the abdominal cavity. The duration of the Procedure is 12 — 24 hours. The following COMPOSITION OF THE dialyzing fluid is recommended (calculated per 20 L): glucose — 30 g, NaCl — 160 g, NaHCO₃ — 20 g, KCl — 4 g, MgCl₂ — 2 g, CaCl₂ — 2 g, NaH₂PO₄ — 1 g. To each liter of solution, 1250 IU of heparin and 100,000 IU of penicillin are added.

During dialysis, careful monitoring of The amount of administered and withdrawn solution is carried out. Fluid retention in the ABDOMINAL CAVITY AND its entry into the bloodstream can lead to hyperhydration and pulmonary edema. The most frequent cause of complications is the blockage of the drainage tube by air or the occlusion of its lumen by intestinal loops. In such cases, the drain is flushed or pulled back slightly. Sometimes it is necessary to replace it.

Peritoneal dialysis makes it possible to extract a significant amount of urea from the blood. However, this method removes only 12 — 15% of toxic substances (S. D. Goligorsky, N. T. Terekhov, 1969). Hemodialysis is a more effective method. In recent years, it has become quite widespread in the treatment of acute renal failure in older children. During the neonatal period, hemodialysis is used very rarely, mainly due to the difficulties associated with connecting the patient to the machine.

Acute urinary retention in newborns occurs as a result of mechanical obstacles along the lower urinary tract. In acute urinary retention, the Urinary Bladder is overfilled, distended, and contoured above the Pubic Symphysis. The infants are restless and crying. In most cases, the causes of acute urinary retention in newborns are congenital in nature (Scheme 2).

Scheme 2. Causes of acute urinary retention

In infants During the first hours of life, acute urinary retention may occur in the presence of a thin membrane in the area of the orificium urethrae externum, which is easily ruptured with a bougie without applying force. Most frequently, such a membrane is observed in glandular hypospadias. However, in this case, acute urinary retention may also occur in the absence of a membrane and is caused by a marked narrowing of the external urethral meatus, the presence of which is detected only upon careful examination as a barely noticeable pinpoint depression on the ventral surface of the glans Penis.

To eliminate acute urinary retention, immediate meatotomy is required to ensure normal urine outflow (Fig. 96).

Fig. 96. Meatotomy.

a — glandular hypospadias; at the apex of the glans There is a blind-ending depression; the external urethral meatus is located in the coronal sulcus region as a barely noticeable dot; b — an instrument blade is inserted 2 — 4 mm into the stenosed external meatus. By compressing the blades, the Skin forming the posterior wall of the external meatus is crushed; c — the BOUNDARIES OF THE compressed, necrotic Tissues are defined; d — an incision is made with a scalpel in the compression zone; e — catgut sutures are applied to the edges of the mucosal membrane and skin; stenosis is eliminated.

Difficult breech delivery can cause edema of the foreskin and impaired normal urination. Phimosis itself may act as an independent cause of acute urinary retention. Another potential cause of acute urinary retention is bladder calcinosis, with calcifications located near the internal urethral orifice. Large ectopic ureteroceles may prolapse into the Urethra, leading to acute urinary retention; in female infants with a wide and short urethra, ureteroceles can prolapse externally and undergo necrosis if prolonged strangulation occurs. A prolapsing ureterovesical anomaly can simulate a bladder or vaginal tumor.

Infravesical obstruction is most commonly manifested by symptoms of chronic urinary retention. In cases of posterior urethral Valves, acute retention is also possible.

Inflammatory processes of the external genitalia, such as acute vulvovaginitis in girls and balanoposthitis in boys, frequently disrupt the normal act of Micturition and cause acute urinary retention.

Acute urinary retention requires immediate medical intervention. Physical examination of the infant and bimanual Palpation are sufficient in the vast majority of cases to determine the underlying cause of the retention. In select cases, to identify the etiological factor accurately, additional investigations such as plain abdominal radiography of the Urinary System and ureterocystography become necessary.

Relief of acute urinary retention is achieved through urinary bladder catheterization. This manipulation is relatively safe, reliable, and effective. In rare instances where catheter insertion is impossible due to an impassable obstruction or difficulties in locating the external urethral orifice, bladder puncture (suprapubic aspiration) or the creation of an epicystostomy (suprapubic cystostomy) is performed. The underlying cause of acute urinary retention may be addressed simultaneously with the placement of the suprapubic tube or, in technically challenging conditions, during a subsequent surgical stage.

Macroscopic Hematuria. Macroscopic hematuria in newborns indicates serious abnormalities occurring within the body or the urinary system Organs (Diagram 3).

Diagram 3. Causes of macroscopic hematuria

One of the most frequent causes of gross hematuria during the first days of a child's life is uric acid infarct, caused by increased excretion of urates. The urine acquires a dark yellow or even reddish hue, and a sediment of precipitated uric acid salts frequently remains on the diapers. Excessive urate excretion can be a cause of neonatal death. According to Fanconi, autopsies of infants who died within the first 2 to 5 days reveal brick-red streaks extending from the renal pyramids to the cortex in 50% of cases — characteristic of uric acid infarcts.

Gross hematuria may be a symptom of hemorrhagic disease of the newborn. The primary signs of this condition include hemorrhages in various locations (skin, mucous membranes, umbilical cord, melena neonatorum, etc.), including bleeding from the urogenital organs. A therapeutic effect is achieved through general supportive measures (vitamin K injections, blood transfusions, etc.).

Renal vein thrombosis in neonates may develop as a result of Hypoxia during difficult labor. Some authors note a particular susceptibility in infants born to mothers with Diabetes Mellitus. Renal vein thrombosis leads to Hemorrhagic Infarction of the kidney, the MAIN CLINICAL MANIFESTATIONS of which are profuse gross hematuria and azotemia. Pronounced skin pallor, decreased Hemoglobin levels, lowered blood pressure, and dehydration are characteristic. In bilateral cases, azotemia rapidly progresses, accompanied by oliguria. Congestive phenomena in the kidney lead to edema in the lumbar region and an enlarged kidney size. Correlation of clinical, laboratory, and radiological findings AIDS in establishing the Diagnosis. Intravenous pyelography typically reveals a non-functioning kidney on the affected side. In cases of pronounced dehydration and a drop in blood pressure, depression of function in the unaffected contralateral kidney may occur. In questionable cases, retrograde pyelography is indicated, which reveals a normal pelvicalyceal system or filling defects within the renal cavities caused by blood clots. If retrograde pyelography is necessary, precautions must be observed, as the congested, edematous, and bleeding renal parenchyma is easily perforated by the catheter, potentially allowing contrast medium extravasation.

Treatment of renal vein thrombosis is conservative. To prevent infection, antibiotics are prescribed, and metabolic acidosis, dehydration, and anemia are corrected. If the Adrenal gland is involved, steroid therapy is indicated.

The question of indications for surgical intervention remains a subject of debate. Proponents of surgery argue that it is necessary due to the risk of thrombosis extending to the contralateral renal vein. The experience of others (Rickham, 1969) confirms the validity of a conservative management approach.

Complete bilateral renal infarction secondary to renal vein thrombosis is a virtually incurable condition.

Renal artery thrombosis in newborns is rare. The primary symptoms are gross hematuria and Heart Failure. Intravenous pyelography demonstrates a lack of function on the affected side. Renal angiography holds the greatest diagnostic value for this pathology. The only definitive treatment is emergency nephrectomy.

Macroscopic hematuria in newborns may be caused by a congenital neoplasm.

Macroscopic hematuria originating from the lower urinary tract during the neonatal period is observed in bladder calcinosis and is associated with the detachment of calcifications. Diagnostic Procedures in such cases include radiological and endoscopic examination methods. Management is typically conservative.

Palpable mass symptom. The presence of a palpable abdominal mass can result from Developmental Anomalies and Diseases of the urinary system, pathological processes within the abdominal cavity (intestinal, mesenteric, or ovarian tumors, intestinal duplication), and retroperitoneal processes (teratoma, cyst, paranephritis, adrenal rupture, etc.).

In rare instances, the "palpable mass symptom" in newborns is caused by a wandering (floating) Spleen (Daum et al., 1964), as well as by Ovarian Cysts that are prone to torsion and rupture (Ahmed, 1971).

According to E. A. Ostropolskaya (1974), out of 82 infants monitored during the neonatal period for an abdominal mass, 66 were found to have Various Forms of urological pathology. Among these, Hydronephrosis (23), infravesical obstruction (19), cystic disorders (polycystic kidney disease — 9, multicystic dysplastic kidney — 5), and Wilms Tumor (7) predominated. These findings are illustrated in Diagram 4.

Diagram 4. Causes of the palpable mass symptom

The Clinical presentation features of this symptom depend on the Nature of the underlying disease and are discussed in detail within the sections describing the respective nosological entities (congenital hydronephrosis, polycystic kidney disease, multicystic kidney, infravesical obstruction, etc.).

In addition to the aforementioned processes, a palpable mass in newborns may result from massive Adrenal Hemorrhage. This condition is characteristic of the neonatal period, during which the Adrenal Glands are significantly enlarged and richly vascularized. Hemorrhage occurs within or around the gland and is typically the result of stress responses. The onset of massive hemorrhage in large, full-term infants immediately after birth is regarded as the result of birth trauma, whereas in premature infants it develops against the background of hypoxia. Adrenal hemorrhage may also occur during the prenatal period, in which case the infant is born with a pronounced hematoma.

In such patients, alongside the symptom of a palpable tumor on one or both sides, there are signs of internal hemorrhage: progressive pallor, falling blood pressure, and a collapse-like state.

Adrenal hemorrhage is frequently combined with renal vein thrombosis, which occurs due to extensive anastomoses between the two venous systems. In such cases, gross hematuria joins the aforementioned clinical picture.

Thus, a palpable tumor in the lumbar region may result from A wide variety of pathological renal and extrarenal processes.

Surgical management is determined by the nature of the pathological process. In massive adrenal hemorrhage, conservative measures aimed at restoring blood loss, administering hemostatic agents, and steroid therapy are often quite effective. Surgery is recommended only if the hematoma ruptures into the abdominal cavity.

DISORDERS OF SEXUAL Development

Human sex is the result of the Interaction of a complex of genetic, hormonal, psychosexual, and other factors. Between 4 and 7 indicators are cited for sex identification: genetic sex, Gonadal Sex, hormonal sex, morphological sex (phenotype), legal (social) sex, and psychological sex (A. Grollman, 1969; N. T. Starkova et al., 1971). In a healthy individual, these criteria correspond with one another. Deviations in the development of any of them lead to pathology in sex differentiation. A condition characterized by a "contradiction" between general appearance, external genitalia, and Gonads is termed Hermaphroditism. It is customary to distinguish between true hermaphroditism (the gonads of such individuals contain both testicular and ovarian morphological elements) and pseudohermaphroditism. In the latter, the gonads are differentiated, but neither the physique nor the external or internal genitalia correspond to them. In female pseudohermaphroditism, the gonads are Ovaries, whereas in male pseudohermaphroditism, they are Testes.

Discrepancies between gonadal sex and phenotype occur in cases of sexual dysgenesis and agenesis.

The information that determines an individual's sex is encoded in the Chromosomes. Genetic sex is established at the moment of Fertilization upon the fusion of the ovum and the sperm.

The male karyotype consists of 22 pairs of somatic chromosomes and a pair of sex chromosomes, X and Y. The female karyotype contains 22 pairs of somatic chromosomes and two X chromosomes. All ova contain a single X chromosome. Half of the spermatozoa contain an X chromosome, while the other half contain a Y chromosome. Consequently, in 50% of cases, an ovum may be fertilized by a sperm carrying an X chromosome, and in 50% by a sperm carrying a Y chromosome. In the first scenario, a zygota (fertilized egg) containing two X chromosomes is formed; In the second scenario, the zygote contains one X chromosome and one Y chromosome. This stage of sex formation is called Sex Determination. A chromosomal set of the zygote containing two X chromosomes determines female-type gonad differentiation (ovaries). A set of sex chromosomes of the XY type determines the development of male-type gonads—testes.

Initially, embryonic gonads are ambisexual, i.e., capable of developing into either male or female organs. Up to the 7th–8th week of intrauterine development, the gonads of male and female embryos have the same Structure, consisting of two layers: the cortex and the medulla. The anatomical relationships of the urogenital sinus and the stages of sex formation are shown schematically in Fig. 97.

Fig. 97. Stages of sex formation.

a — anatomical relationships in an 8-week human embryo: B — Wolffian duct; M — Müllerian duct; b — relationships of the Wolffian and Müllerian ducts and the primordial gonad: 1 — cortical layer; 2 — medullary substance.

At The First stage of sex formation, sex determination takes place. The development of internal genitalia depends on the information encoded in the chromosomes.

During female sex formation (c), under METABOLISM/18.html">The Influence of information embedded in the fertilized ovum (XX), Atrophy of the medullary substance of the primordial gonad and development of its cortical layer occur.

During male sex formation (e), under the influence of information embedded in the fertilized ovum (XY), atrophy of the cortical layer of the primordial gonad and development of its medullary substance occur.

Further sex formation proceeds under the influence of Hormones from the developed embryonic gonad, the fetal adrenal cortex, and maternal hormones (estrogens), which condition the autonomous tendency of each embryo and fetus toward feminization.

During female sex formation (d, e), the Ovary secretes hormones that, together with maternal estrogens, determine the development of internal and external genitalia. The "anti-Wolffian" substance (AW) secreted by the ovary causes atrophy of the Wolffian duct.

During male sex formation (g, h), under the influence of testicular androgens and the fetal adrenal cortex—which determine the resistance of Embryonic and Fetal tissues to the feminizing effects of maternal hormones—male internal and external genitalia are formed. The "anti-Müllerian" substance (AM) secreted by the testes causes atrophy of the Müllerian duct.

The formation of external genitalia occurs from the 12th to the 20th week. In boys, the genital tubercle gives rise to the glans penis and the corpus spongiosum of the urethra. On the lower surface of the genital tubercle, a urethral groove appears, from which the urethral trough is formed. Along both sides of the latter lie the genital folds, which elongate during differentiation, fuse with one another, and form the urethra. During this period, the urogenital orifice opens at the Base of the penis. Later, when the corpus spongiosum of the urethra is formed from the mesenchyme of the genital tubercle, the urogenital orifice shifts to the glans penis. After the fusion of the genital folds, the joining genital swellings form the cutaneous part of the Scrotum.

In girls, the genital tubercle forms the Clitoris; the urogenital sinus remains open and transforms into the Vestibule of the Vagina, while the Labia minora develop from the genital folds. The Labia Majora are formed from the outer labial swellings.

By the time of birth, the formation of both internal and external genitalia is typically fully complete.

The Emergence of pathology in sex formation depends on The Nature and timing of the pathological (teratogenic) factor. Such factors may include radiation, physical, chemical, or biological (infectious) agents, or hormonal dysregulation in the mother's body, among others. Pathological exposure at the stage of genetic sex determination leads to Chromosomal Sex anomalies, the clinical picture of which depends on the pathology of cellular chromosomes.

Disruption of sexual differentiation at the stage of gonadal ridge formation, depending on the extent of the lesion, leads to the development of gonadal bisexuality (true hermaphroditism) or gonadal dysgenesis and even agenesis (Fig. 98). In the latter case, regardless of genetic sex, internal and external genitalia form According to the female type due to the autonomous tendency of any fetus toward feminization. The same occurs in sexual dysgenesis of chromosomal origin (Shereshevsky–Turner syndrome).

Fig. 98. Pathogenesis of gonadal agenesis.

At the stage of sex determination (a), the primary gonad is destroyed as a result of exposure to exo- or endogenous damaging factors (b). Under the influence of maternal hormones (c), which drive the autonomous tendency of each fetus toward feminization, derivatives of the Müllerian duct develop (d). With a 46,XY karyotype, a phenotypically female child is born—exhibiting a genotype-phenotype mismatch.

At the Third Stage of sex differentiation (7th–12th week of intrauterine life), in the event of teratogenic exposure, the formation of internal and external genitalia is completed by maternal estrogens. A physically normal-appearing female is born. If the fetus had a genetic male sex in such a situation, the inadequacy of its testicles leads to the development of mixed-type external genitalia.

At the stage of external genitalia differentiation (12th–20th week of intrauterine life), the gonads and internal reproductive organs are fully formed. For the proper development of external genitalia, adequate amounts of isosexual Sex hormones are required. The greatest danger during this period is hormonal dysregulation in the mother or proband, which induces the development of pseudohermaphroditism.

Female pseudohermaphroditism is characterized by a karyotype with a set of XX sex chromosomes, fully differentiated ovaries, typically female internal reproductive organs, but virilized external genitalia. Virilization of the external genitalia may occur in the presence of a virilizing tumor in the mother (androsteroma, arrhenoblastoma, etc.), when the mother takes high doses of sex hormones during Pregnancy (it has been noted that not only Male Sex Hormones can cause fetal virilization, but also progesterone and estrogens), and in congenital adrenal hyperplasia (Fig. 99).

Fig. 99. Virilization of external genitalia in girls with congenital adrenal hyperplasia.

The degree of virilization depends on the level of adrenal-secreted androgens (a). Clitoral hypertrophy. Normal vulva (b). More pronounced clitoral hypertrophy. The common urogenital opening opens onto the Perineum (c). The phallus resembles a penis. The common urogenital orifice opens at its apex. The vagina empties into a urogenital sinus resembling the urethra. The labia majora are fused (d).

Male pseudohermaphroditism is characterized by an XY set of Sex Chromosomes and formed yet functionally insufficiently active testicles. In these cases, the external genitalia develop under the influence of maternal estrogens. The degree of feminization of the external genitalia depends on androgen deficiency (Table 19).

Table 19. Cytology/practical/136.html">Differential diagnosis OF sex development disorders in newborns

Turner Syndrome

Turner syndrome belongs to a multi-symptom group of disorders united under the common term of gonadal dysgenesis.

Various forms of dysgenesis present with a diverse range of sex chromosome pathologies, most commonly XO or mosaic structures such as XO/XX, XO/XY, XO/XYY, XO/XXX, XO/XX/XXX, XO/XY/XXY, as well as structural aberrations of the X chromosome: long arm duplication, short arm deletion, and ring X chromosome. Newborns with Turner syndrome frequently exhibit prenatal dystrophy, and lymphedema of the hands and feet. Due to nuchal skin edema, the neck is significantly

thickened. Other findings include a low posterior hairline, nail and tooth dystrophy, auricular deformities, a broad Flat chest, micro- and retrognathia, a high-arched palate, occasionally Syndactyly, shortening of the 4th metacarpal or 3rd, 4th, and 5th Phalanges of the feet, a positive metacarpal sign, and a single transverse palmar crease. Anomalies of The Cardiovascular system, Kidneys, eyes, and other organs are common. The internal and external genitalia develop along female lines. The gonads are dysgenetic, presenting as Connective Tissue streaks, with germinal epithelium absent.

Sex chromatin is not detected in the nuclei of buccal epithelium Cells. There is no Pathogenetic Therapy for this syndrome.

Klinefelter syndrome

Newborns with Klinefelter syndrome are phenotypic males; cryptorchidism is sometimes observed (in 0.1% of cases) (N. S. Kazey, 1970; Ferguson-Smith, 1966). Concomitant Developmental anomalies of the cardiovascular, osteoarticular, and other systems are characteristic. Sex chromatin is positive, with 1 to 2, 3, or more bodies depending on the number of chromosomes in the karyotype. The karyotype may be XXY, XYY, XY/XXY, XXY/XO/XY, XXXXY, etc.

Klinefelter syndrome and trisomy X syndrome do not require therapy during the neonatal period. Such patients should be monitored by an endocrinologist.

True Hermaphroditism

The term "hermaphroditism" originates from the name of an ancient Greek god, the child of Hermes and Aphrodite, who was depicted as a female figure with well-developed breasts and male genitalia. The term "true hermaphroditism" implies the presence of both ovarian and testicular tissue in a single individual. Testicular and ovarian gonads may exist either as separate organs or as a single combined organ. Depending on their combinations, hermaphroditism is classified as bilateral (each side has an ovotestis, or an ovary and a Testis), unilateral (one side has a normal gonad, while the other has an ovotestis), and lateral (one side has a testis, and the other has an ovary).

The structure of the reproductive tracts in such patients depends on the STRUCTURE OF THE gonad on the respective side. If a testis is present, an Epididymis and vas deferens may be found on its side; on the side of the ovary, a fallopian tube. Sometimes the fallopian tube and Uterus are found within an inguinal hernia sac or even in the scrotum.

Female Pseudohermaphroditism

Adrenogenital syndrome (congenital adrenal hyperplasia). The disease is inherited. The genetic pathology induces Impairment of the 21-hydroxylase and 11-hydroxylase enzyme systems, which are necessary for the synthesis of glucocorticoid and mineralocorticoid Hormones of the adrenal cortex. As a result, the release of adrenocorticotropic hormone (ACTH) by the hypothalamic-pituitary system is disinhibited, leading to adrenal cortex hyperplasia and increased Synthesis and Secretion of androgens with virilizing effects.

The androgenic Influence of the adrenal secretion manifests from the 3rd to the 5th month of intrauterine development. By this time, the gonads and reproductive ducts are already fully differentiated, and the external genitalia begin to form. If the proband is a male, the elevated amount of androgens does not interfere with the proper Differentiation of the external genitalia, and a phenotypically male child is born. However, if a genetically female Organism is exposed to high doses of androgens, the clinical picture of female pseudohermaphroditism develops.

Clinical presentation. The appearance of the external genitalia depends on the degree of fusion of the labia. Several types of genital structure are distinguished in such patients: 1) normal vulva; 2) deepened, funnel-shaped vestibule; 3) a common urogenital opening of varying sizes in the perineum; 4) the urogenital opening opens at the tip of the clitoris. Androgen excess exerts an anabolic effect, manifested by accelerated bone differentiation. The bone age of such a newborn may correspond to that of a 6-month-old, a 1-year-old, or even a 2-year-old.

The deficiency of glucocorticoid and mineralocorticoid hormones of the adrenal cortex in such patients manifests as hyponatremia, hyperkalemia, exicosis, toxicosis, hypotrophy, muscular adynamia, regurgitation, and frequent vomiting. Hyperpigmentation of the skin of the external genitalia, areolae, and other skin areas subject to friction from clothing is observed.

Depending on the severity of steroid hormone deficiency, three main forms of the disease are distinguished: uncomplicated, salt-losing, and hypertensive. In the uncomplicated and salt-losing forms, blood pressure is decreased, while in the hypertensive form, it is elevated.

Treatment. Congenital adrenal hyperplasia is the only form of hermaphroditism for which pathogenetically substantiated treatment exists. It includes hormone replacement therapy with glucocorticoid and mineralocorticoid activity (prednisolone, hydrocortisone, DOCA, etc.) in doses sufficient to eliminate hyponatremia and hyperkalemia, and to reduce The excretion of 17-ketosteroids to a level corresponding to the degree of tissue maturity of the newborn (their "bone" age). Under the influence of these hormones, the infant's regurgitation ceases, dehydration decreases, and weight gain is observed. Concurrently with the improvement of the general condition and normalization of the electrolyte balance, symptoms of virilization also decrease. Definitive correction of the external genitalia is performed surgically in the postneonatal period.

Other forms of female pseudohermaphroditism are characterized by a structure of the external genitalia similar to that described above, but the clinical course lacks symptoms of adrenal insufficiency. The diagnosis is established based on the female chromosomal sex and the exclusion of adrenogenital syndrome. The presence of ovaries confirms the diagnosis and rules out true hermaphroditism. Attempts have been made to perform endoscopy of the urogenital sinus in newborns with pseudohermaphroditism (Suli, Nicole, 1966). Treatment consists of corrective surgery.

Male pseudohermaphroditism

The gonads in newborns suffering from male pseudohermaphroditism are represented by testes. The external genitalia are of a mixed type. There is a specific form of male pseudohermaphroditism, the so-called testicular feminization syndrome. In this form, the gonads are fully differentiated testes, but they produce hormones perceived by tissues as estrogens. This leads to the development of female-type genitalia. In a newborn, testicular feminization syndrome can be suspected only if testes are palpated within the labia majora or the Inguinal Canal.

Medical tactics and treatment. The child's sex must be established during the neonatal period, as changing the sex after infancy can cause severe psychiatric disorders. Difficulties in choosing the social sex are compounded by the lack of absolute indicators. Even the type of gonads, sex chromatin, or the number and type of sex chromosomes are not always factors that determine sex. More important from the perspective of the individual and their living environment are secondary sexual characteristics, such as body build, external genitalia, breast development, voice timbre, and facial Hair growth.

A newborn female with congenital adrenal hyperplasia, regardless of how virilized her genitalia may be, should be raised as a girl, since virilization decreases as the body becomes saturated with the missing hormones.

In other cases, one is guided by the degree of virilization of the external genitalia, as this specific sign makes it possible to predict the direction in which Puberty will proceed. In cases of significant virilization, the child is given a male name and raised as a boy.

In complex cases of hermaphroditism, it is preferable to choose the female sex, as hormonal therapy and Surgical treatment are easier to manage subsequently. In patients with male pseudohermaphroditism, insufficiently differentiated testes are recommended to be removed to prevent potential enhancement of their virilizing effect and malignant transformation. In patients with testicular feminization whose testes are located within the labia majora, the question of testicle removal can be postponed to an older age, since these testes are easily palpable and the risk of malignancy is not as high.

Exstrophic anomalies

Disruption of the ingrowth of the primary mesoderm into the allantois at various stages of Embryogenesis leads to severe Malformations of the lower URINARY AND REPRODUCTIVE systems, collectively termed "exstrophic anomalies."

The most frequent of these is bladder exstrophy combined with epispadias. The formation of this malformation temporally corresponds to a later stage (16 mm) of embryonic development. Due to the underdevelopment of the Abdominal Muscles, the primary urogenital sinus remains exposed externally, uncovered by the anterior abdominal wall.

Depending on the degree of underdevelopment of the mesodermal tissue, malformations of varying severity arise. The mildest degree of underdevelopment is manifested by epispadias and an upper bladder cleft. The most severe forms are cloacal exstrophy and vesicointestinal fissure.

Bladder exstrophy occurs in 1 out of every 30,000 births, more frequently in males.

Clinical presentation. The malformation manifests as the absence of the anterior abdominal wall and the anterior wall of the urinary bladder. The mucous membrane of the posterior bladder wall is exposed and fills the defect in the suprapubic region. In the vast majority of cases, exstrophy is accompanied by complete epispadias. In males, the penis is underdeveloped, drawn toward the abdomen; when pulled downward, a groove of the urethral mucosa, which adheres tightly to the cavernous bodies of the penis, is clearly traceable on its anterior surface. The glans penis is split, and the prepuce covers it like an apron only along the posterior surface.

In females, there is a clefting of the labia majora and minora and the glans clitoris, between the two halves of which a strip of the split urethral mucosa can be traced. The vaginal and anal openings are usually displaced anteriorly. Bladder exstrophy is frequently combined with Anomalies of the internal genitalia, such as cryptorchidism and prostate hypoplasia in males, and vaginal duplication and bicornuate uterus in females.

In bladder exstrophy, there is a significant Separation of the BONES OF THE pubic symphysis, with a diastasis that can reach 6–7 cm or more.

The dimensions of the exstrophic bladder vary within a fairly wide range—from 2 to 6–8 cm in diameter—and do not change significantly with age. Its circumference is limited by an aponeurotic ring, the diameter of which is often significantly smaller than the diameter of the bulging bladder mucosa.

To determine the true dimensions of the exstrophic bladder, it is advisable to measure it while the child strains and cries, as the increase in intra-abdominal pressure ensures maximum stretching of the mucous membrane.

At birth, the exposed bladder mucosa is smooth, elastic, and free of marked hyperemia. Soon after birth, its character changes significantly due to constant irritation and the development of an inflammatory process. It becomes loose, edematous, hyperemic, and bleeds easily. A tendency toward metaplasia rapidly develops, and polypoid proliferations appear. The surrounding skin becomes macerated, and Pyoderma may occur. The inflammatory reaction is not limited to external manifestations; the process typically spreads to the upper urinary tract, becoming complicated by ascending Pyelonephritis.

Care for children with bladder exstrophy is difficult. Even adherence to aseptic rules does not guarantee protection against inflammatory and septic complications.

Treatment. Bladder exstrophy requires surgical treatment. Surgery during the neonatal period is quite complex, but according to some authors (G. A. Bairov, 1966; Rickham, 1969), it has certain advantages. These consist in the absence of secondary Changes in the mucous membrane and surrounding skin, which negatively affect the outcome of the plasty. The moral and ethical aspect of the physician's responsibility to the child and their parents is also of considerable importance.

Reconstructive surgeries are justified only in cases of large dimensions of the exstrophic bladder in girls. The presence of pronounced dilatation of the upper urinary tract is a contraindication to the intervention. In newborns, it is advisable to perform the plastic surgery in two stages. The first stage involves bilateral posterior iliac osteotomy to normalize the anatomical relationships of the pelvic ring; the subsequent stage, performed a week later, consists of bladder plasty. Most authors postpone the operation for Ureter transplantation or the transplantation of the bladder wall with its orifices until 1 year of age.

Epispadias is a rarer manifestation of exstrophic disorders and results from the underdevelopment of the inferior umbilical membrane, limited to the pubic region.

Epispadias is 5 times more common in boys than in girls and, according to the current Classification, is subdivided into three forms: glandular, penile, and total, depending on the Location OF THE urethral meatus. Total epispadias prevails in frequency over the other forms. In girls, total epispadias is observed in the vast majority of cases; however, some authors distinguish clitoric and subsymphyseal forms by analogy with male epispadias.

During the neonatal period, this anomaly is of interest in terms of timely and accurate diagnosis. While a single physical examination is sufficient for males to establish the diagnosis and determine the form of the anomaly, it presents certain difficulties in females.

Clinical presentation. Epispadias in boys manifests as penile deformity, the severity of which is directly dependent on the form. Total epispadias is characterized by clefting of the urethra throughout its entire length; the anterior segment of the urinary bladder sphincter is also partially involved. The penis is deformed and drawn toward the anterior abdominal wall. The foreskin covers the glans only on the posterior surface and hangs down like an apron. When the penis is retracted downward, a mucosal groove of the cleft urethra is clearly visible on its anterior surface, transitioning at the base into a wide, funnel-shaped opening leading into the bladder cavity. Continuous urine leakage due to total incontinence quickly causes skin irritation and maceration, requiring the most meticulous care.

In incomplete epispadias, penile deformity is less pronounced, the urethra is not cleft along its entire length, and the splitting is limited to the area of the glans up to the coronary sulcus (in the glandular form) or the penile shaft (in the penile form). Urinary control is usually preserved in these forms; however, sphincter weakness is often noted, manifested by moderate urine leakage during straining and crying in the infant.

In newborn girls, the initial examination reveals no obvious anatomical defects, and only a careful examination with spread labia majora helps establish the correct diagnosis. Epispadias in girls is characterized by clefting of the labia majora and minora, splitting of the clitoral glans into two separate halves, and complete clefting of the urethra along its entire length. The external urethral meatus is displaced anteriorly and represented by a wide, funnel-shaped entrance into the bladder, from which urine continuously leaks. The vaginal opening is also dystopic and shifted anteriorly. Palpation of the pubic region reveals moderate divergence of the pubic bones.

Treatment. During the neonatal period, only timely and correct diagnosis is required, along with special care to protect the skin from irritation, maceration, and pyoderma. The best results are achieved with open management accompanied by lubrication of the surrounding skin areas with neutral ointments. Frequent diaper changes using pre-ironed diapers are necessary. Plastic surgeries aimed at reconstructing the bladder neck and urethra are performed at an older age (3–5 years).

High bladder cleft is among the rare anomalies. In this condition, the urinary bladder has an internal cavity and is covered by the anterior abdominal wall, except for a small area of splitting along the linea alba, projecting to the region of the bladder dome. The part of the bladder adjacent to this zone is exstrophic. The urethra and the neck of the bladder with the sphincter mechanism are normally formed, which greatly facilitates plastic surgery aimed at closing the defect of the bladder and the anterior abdominal wall.

Cloacal exstrophy, or vesicointestinal fissure, is the most severe combined developmental anomaly within the group of exstrophic disorders. The condition is extremely rare and is described by most authors as a casuistic observation. It arises from early embryonic development disturbances (5 mm stage) prior to the Formation of the urorectal septum. Cloacal exstrophy exhibits considerable Variability in manifestation while retaining the core Clinical Features of the anomaly. The exstrophic bladder is divided into two halves by a bowel passing vertically through it. The intestinal mucosa, similar to the vesicular mucosa, is everted, and intestinal contents are exposed. The exstrophic bowel segment represents the ileocecal region, with the upper opening corresponding to the terminal section of the ileum and the lower opening corresponding to the colon segment, extending 2–3 cm anterior to the sacrum and ending blindly. Directly above the exstrophic bladder lies a large embryonic umbilical hernia, usually containing the Small Intestine and frequently a portion of the Liver.

This developmental anomaly is frequently combined with abnormalities of the upper urinary tracts, internal genital organs, as well as anomalies of other systems and organs.

The severity and extent of the lesion usually render attempts at surgical correction in the neonatal period unpromising, and in most cases, treatment is strictly conservative.

In exceptional cases, when the child's general condition is good and the embryonic hernia is relatively small, surgery may be performed, the MAIN STAGES OF which are: 1) repair of the embryonic hernia, 2) mobilization of the exstrophic bowel segment and, depending on local conditions, either its preservation and tubularization (in which case the terminal blind end of the colon is mobilized and brought out as a perineal or abdominal colostomy), or resection of the bowel and creation of an ileostomy on its proximal segment, 3) bladder reconstruction by suturing its halves together. Radical reconstruction of the anomaly is usually not achievable in the neonatal period, and in cases of recovery, children undergo repeated interventions at an older age.

External genital anomalies in girls

Disruption of the normal Development of the Müllerian ducts during early embryonic stages leads to reproductive tract anomalies. Three forms of uterine and vaginal developmental anomalies are distinguished: complete or partial absence of these organs, duplication, and varying degrees of organ underdevelopment (hypoplasia).

Atresia of the vulva and hymen. Congenital closures of the vulva are the result of an intrauterine inflammatory process (I. I. Bogorov, 1966). Hymenal atresias are combined with atresias of the anterior vagina in the majority of cases.

Upon examination, the hymen appears as a solid barrier (imperforate hymen). The membrane may be located at various levels within the vagina; most frequently, it is found at the border of the upper and middle thirds.

As a result, hydrocolpos develops, which is detected after birth by a midline mass in the lower abdomen and infant restlessness. The distended vagina may obstruct urination due to bladder displacement. Excretory urography frequently reveals dilation of the upper urinary tracts.

The membrane closing the vaginal outlet is excised via a circular incision. The vagina is drained with tubes for several days. Bougienage may be required to prevent the development of stenosis.

Hymenal polyp. Under the influence of maternal estrogens, the newborn's hymen is thickened and edematous, and is clearly visible upon perineal examination. Occasionally, a polyp is found in its lower section. Histologically, the protrusion consists of edematous connective tissue covered with squamous epithelium. The polyp may resolve spontaneously.

Vaginal cysts originate from remnants of Gartner's ducts located on both sides of the vaginal wall. They prolapse into the vaginal lumen and simulate hydrocolpos. Compression of the urethra by vaginal cysts obstructs urination and necessitates their early excision.

Paraurethral cysts. In newborns, cyst formation from paraurethral glands is possible. Upon examination, cysts are found near the external urethral meatus, resembling a prolapsing ureterocele. Excretory urography aids in differential diagnosis. The cysts are surgically removed.

External genital anomalies in boys

Congenital tightness of the foreskin (phimosis). Narrowing of the foreskin that prevents it from being retracted behind the glans penis without force is called phimosis. In newborns, epithelial adhesion of the foreskin to the glans is a physiological phenomenon. In markedly pronounced phimosis, difficulty in urination and involvement of the overlying urinary tracts in the pathological process are observed. Along with congenital phimosis, in 0.5–1% of observations, foreskin stenosing occurs as a result of inflammatory conditions in the area of the glans penis and preputial sac (acquired phimosis).

Clinically, two forms of phimosis are distinguished: hypertrophic, when the foreskin is elongated and protrudes forward like a trunk, and atrophic, characterized by skin tightly embracing the glans.

Adhesion of the foreskin layers in newborns is a physiological phenomenon and does not require treatment. To prevent pathological phimosis, the outer layer of the foreskin is pulled backward enough to expose the external urethral meatus, adhesions between the glans and the inner layer of the foreskin are separated with a probe, the glans is lubricated with petrolatum, and the foreskin is pulled back over it. In rare cases, with significant narrowing of the foreskin, difficulty in urination, and failure of conservative measures, surgical treatment is undertaken in newborns. The primary surgical method is circular circumcision of the foreskin (circumcision) (Fig. 100). The foreskin is clamped with forceps jaws. Leaving an excess of foreskin layers posteriorly ensures the preservation of the frenulum of the penis. After removing the clamp, an incision is made through the crushed tissue area, and it is completely removed. The foreskin layers are approximated with interrupted fine catgut sutures.

Fig. 100. Circumcision for phimosis.

Along the midline, the prepuce is crushed with a straight Kocher clamp down to the level of the coronal sulcus. The avascular strip is then incised (a). Excess foreskin is excised following crushing with a curved clamp (b). Catgut sutures are placed in the wound (c).

Infravesical obstruction

Obstructions located in the urethra and bladder neck are collectively termed "infravesical obstruction." Obstructive uropathies represent one of the most challenging problems in neonatal surgery.

The kidneys begin to secrete urine in the 14-week fetus, and an outflow obstruction from the bladder already during the intrauterine period causes dilatation of the urinary tract. In infravesical obstruction, the bladder responds to persistent distension with muscular hypertrophy. Detrusor hypertrophy manifests as trabeculation of the mucous membrane, whereas Hypertrophy of the circular Muscles of the bladder neck results in its narrowing. Subsequently, areas of muscular hypertrophy alternate with areas of wall thinning, giving the bladder a honeycombed appearance. Bladder capacity increases, its base is sometimes palpable above the umbilicus, and diverticula frequently form. Due to persistently elevated pressure in the urinary tract, the Ureters become dilated and tortuous. The rise in intravesical pressure leads to vesicoureteral reflux. The contractility of the ureters is preserved, but a dilated ureter is incapable of transporting urine into the bladder because its walls do not come into contact with one another, making the movement of urine oscillatory. Enlargement of the renal pelvis against the background of marked hydroureter is not very pronounced, except in cases with concomitant pelviureteric junction obstruction, where pelvic enlargement can be significant.

The renal parenchyma is thinned, and cystic Dysplasia is frequent. Shortly after birth, infection sets in, exacerbating the destructive process within the kidneys.

A rare but formidable complication of infravesical obstruction is urinary ascites. Urine may be present in the peritoneal cavity of such patients at birth, or ascites may develop during the first days of life. In the majority of patients, spontaneous ruptures of the bladder or a hydronephrotic kidney are discovered, but in A number of cases, no communication between the peritoneal cavity and the urinary tract can be found. The Etiology AND PATHOGENESIS of this condition remain unclear. Evidently, the posterior parietal Peritoneum in a newborn has a greater permeability than that of adults and older children. Therefore, in the presence of neonatal ascites and the absence of liver pathology, all attention is directed toward the urinary system. Mortality from urinary ascites is high, reaching 70% (Johnston, 1966; Thompson, 1972).

The most frequent cause of infravesical obstruction in neonates is posterior urethral valves. Valves occur exclusively in males. In recent years, this problem has received considerable attention (A. Ya. Pytel, S. D. Goligorsky, 1970; S. Ya. Doletsky, I. A. Korolkova, Yu. P. Terekhov et al., 1971; Williams, 1968; Hendren, 1971).

The embryological Origin of the valves remains not fully understood. Some urologists consider them to be hypertrophied folds of the normal urethra, while others attribute them to pathological alterations of the Wolffian duct or remnants of the urogenital membrane.

Currently, most urologists adhere to the classification of Young (1929), who divided posterior urethral valves into three types based on their Anatomical Structure and location within the urethra (Fig. 101). Type 1 valves appear as two sail-like cusps descending from the distal end of the verumontanum downward and laterally along the urethral walls; during retrograde catheterization, they collapse and do not cause a sensation of obstruction. When urine flows into the urethra, the valve cusps approximate, creating an obstruction. Sometimes the valves originate not from the verumontanum, but somewhat more distally from the urethral crest, with their cusps extending into the area of the external sphincter.

Fig. 101. Posterior urethral valves (according to Young's classification).

Type 1 posterior urethral valves: a — two valve cusps originate from the distal end of the verumontanum and descend inferiorly and laterally along the urethral walls; b — valve cusps originate from the verumontanum; c — only a single valve cusp is present.

Type 2 posterior urethral valves represent mucosal folds extending from the proximal part of the verumontanum upward and laterally toward the bladder neck (d).

Type 3 represents a stenotic narrowing of the urethra in the form of a membrane, which may be located below, above, or at the level of the verumontanum (e, f).

The Anatomical structure of the valves varies from thin, transparent membranes to dense fibrous cusps. A valve may be expressed unilaterally only. Type 2 consists of mucosal folds extending from the proximal portion of the verumontanum upward and laterally toward the bladder neck. As an independent cause of obstruction, this type of valve is questionable. It is believed that these folds form as a result of posterior urethral dilatation associated with Type 1 and Type 3 valves.

Type 3 is encountered less frequently than Type 1 and presents as a stenotic narrowing of the urethra in the form of a membrane. This type of obstruction does not possess valve-like properties, as it impedes both antegrade and retrograde movement through the urethra. Posterior urethral valves are connective tissue plates covered on both sides by epithelium.

Clinical presentation. In newborns, the leading manifestation of urethral valves consists of symptoms of urinary tract infection. General symptoms appear: vomiting, diarrhea, hyperthermia, convulsions, jaundice, and hemorrhagic diathesis. Upon examination of the infant, attention is drawn to an enlarged abdomen; sometimes enlarged, ballottable kidneys can be palpated, and the urinary bladder is felt as a dense mass upon bimanual examination. Micturition is infrequent, and urinary dribbling is occasionally noted. The urinary stream is weak and intermittent; the infant is restless and strains during urination. The presence of a strained stream does not contradict the diagnosis, as the hypertrophied detrusor compensates for the existing obstruction by generating increased intravesical pressure. The urine is cloudy, containing high amounts of protein and pus. Severe water and electrolyte balance disorders and pronounced metabolic acidosis are frequently observed. Blood urea and residual nitrogen are usually elevated, but following the correction of dehydration and electrolyte disturbances, as well as a decrease in the severity of the inflammatory process, they may drop close to normal values.

The critical condition of the neonate dictates The Need for diagnostic measures performed under the cover of infusion and antibacterial therapy. Urine cultures are obtained for the proper Selection of antibiotics. If the infant presents in a compensated phase, without marked urinary retention, bladder catheterization is best avoided to prevent infection.

Radiological findings. Following the identification and correction of Homeostasis parameters, high-dose infusion pyelography is performed using a high concentration of contrast medium (4–5 ml per kg of the infant's weight). Radiographs reveal decreased renal function, with dilated, hydronephrotic pelvicalyceal systems. The ureters are usually poorly opacified, and the urinary bladder is enlarged.

Cystourethrography is a simple and informative diagnostic method that allows the presence and level of obstruction to be established. In cases of severe renal failure, it is the only feasible investigative method. X-rays are taken in the anteroposterior and oblique positions of the patient during micturition. The cystourethrogram demonstrates an enlarged, frequently trabeculated urinary bladder with diverticulum-like formations. In the presence of reflux, wide and tortuous ureters are traced. The prostatic urethra is dilated, and its proximal segment is separated from the bulbar urethra by an oval filling defect. Contrast medium reflux into the vas deferens and prostatic utricle is occasionally revealed. A concomitant enlargement of the posterior lip of the bladder neck is frequently detected. Although its obstructive effect is questionable, surgeons prefer to combine valve ablation with bladder neck resection.

Cystoscopy and urethroscopy are not widely used in newborns due to technical difficulties in performance. Radioisotope studies provide valuable information when renal concentration capacity is reduced.

Treatment. The diagnostic workup required to establish a diagnosis takes 1–2 days when properly organized. Administering intensive antibacterial therapy during this period helps normalize the water-electrolyte balance, reduce residual nitrogen levels, and ameliorate acidosis and toxicity. In cases of marked urinary retention, an indwelling urethral catheter is left in place.

Conservative treatment methods yield only temporary results. Permanent drainage is possible solely through surgical intervention—bilateral ureterostomy. This procedure, which is minimal in volume and trauma, provides reliable drainage of the upper urinary tract. When creating loop or cutaneous ureterostomies, the ureters are mobilized as high as possible. Drainage tubes are inserted into the renal pelvis for the immediate postoperative period; as soon as the stomas are formed, the drains are removed. Postoperative bladder drainage and periodic irrigation with antiseptic solutions facilitate the sanitation of the lower urinary tract.

Radical surgery is performed once the newborn's condition stabilizes. The valves are destroyed via a perineal approach, transurethral endoscopic resection, or a suprapubic approach. The suprapubic approach does not provide access to the level of the valves without dividing the pubic symphysis; this approach is traumatic and accompanied by significant blood loss. The endoscopic method requires specialized equipment and is technically challenging, as it necessitates a urethrotomy to introduce the urethroscope into a newborn. The perineal approach is the most effective (Johnston, 1966) (Fig. 102).

Fig. 102. Perineal approach for the excision of urethral valves.

A perineal incision is used to expose the urethra layer by layer, sling it with stay sutures, open it, and dilate it with bougies until an otoscope can be passed. Upon introducing the scope, the seminal colliculus is visualized first, followed by the valves, which unfold and occlude the urethral lumen. The valves are destroyed using an electrode. The urethra and skin wound are closed in layers. An indwelling catheter is left in the urethra for approximately one week.

Surgical technique. With the child in the lithotomy position, an arcuate perineal skin incision is made to expose the urethra. An incision is made in the posterior wall of the urethra directly over the tip of a metal bougie previously inserted into it. Stay sutures capturing the mucosa are placed on the edges of the incision. The posterior urethra is dilated with straight or slightly curved bougies up to Charrière size 13. The resulting 4.75 mm diameter allows for the Introduction of an otoscope into the prostatic urethra. An electric light source is attached to the scope. As the scope is advanced, first the seminal colliculus and then the valves become clearly visible. The valves are destroyed using a dielectrically insulated electrode passed through the scope. The urethral wound is closed with catgut sutures, and a catheter is left in the urethra for several days.

Postoperative control radiographs demonstrate that the urethra soon assumes a normal configuration. Further treatment depends on the degree of upper urinary tract decompensation and requires an individualized approach.

In mild cases, where the removal of valves alone is sufficient to normalize urinary flow through the tract, the diagnosis is rarely made during the neonatal period. As Williams and Johnston note, the earlier the valves manifest clinically, the more severe the damage to the upper urinary tracts. In cases of moderate ureteral dilation and persistent vesicoureteral reflux after valve ablation, an anti-reflux procedure is indicated, the technique of which depends on the anatomical Structure and Functional preservation of the affected ureteral orifice. In cases of ectopia and dilation, we prefer the Politano-Leadbetter anti-reflux procedure.

Reconstruction of significantly dilated ureters presents a difficult surgical challenge, though it is usually performed at an older age. There are isolated reports in the literature concerning the Surgical Treatment of dilated ureters in newborns. Hendren (1969–1971) described THE PRINCIPLE OF staged reconstruction of the decompensated urinary system. The author proposes early and complete reconstruction of the entire urinary tract, which includes valve ablation, revision of the bladder neck, staged tapering and straightening of the ureters with reimplantation into the bladder. If the renal pelvis is dilated, its resection is also performed. Using the principle of staged ureteral straightening and tapering, we have operated on 3 patients. Short-term outcomes are satisfactory (T. N. Kulikova, 1975).

Reconstructive procedures on the urinary tract demand high surgical skill, meticulous anesthesia, and optimal antibacterial therapy. Therefore, for surgeons lacking experience with newborns, high cutaneous ureterostomy is the method of choice in treating children with marked urinary tract decompensation. Following surgery, children with obstructive uropathies are indicated for prolonged (at least 1 year) antibacterial therapy guided by urine culture results.

In addition to posterior urethral valves, newborns may present with other anomalies that obstruct bladder emptying: bladder neck stenosis, hypertrophy of the seminal colliculus, urethral fibroelastosis, urethral diverticulum, urethral duplication, urethral tumor, and inflammatory conditions of the urethra. These conditions are rare findings in neonates. The physician's management strategy is fundamentally identical to that for valves—that is, the primary objective is adequate drainage of the urinary tract followed by the removal of anatomical and functional obstructions. For bladder neck stenosis, a V-plasty is performed.

Prognosis. The compensatory capacity of the newborn's kidneys, provided prompt and effective urinary tract drainage is established, allows for an almost complete restoration of their function. Advances in the rehabilitation of the decompensated urinary tract justify a more optimistic outlook for this patient group, which until recently was considered hopeless.