NEONATAL SURGERY - 1976

2. SPECIAL SECTIONS

8. Malformations of Individual Organs and Systems

Peripheral Vessels (Blood and Lymphatic Vessels)

Malformations of the peripheral Vessels of the extremities are among the rare yet severe conditions that lead to profound local and systemic changes, marked functional disorders, and, if left untreated in a timely manner, permanent disability in the child. The task of the physician attending such a patient during the neonatal period is to establish a correct Diagnosis and guide the parents regarding further management.

Clinical experience shows that general pediatricians are insufficiently familiar with the Clinical presentation and diagnosis of peripheral vascular malformations of the extremities in children, particularly During the first weeks of life. As a result, children are referred to specialized surgical departments late, which worsens Treatment outcomes. Until recently, the majority of such patients were diagnosed with "racemose hemangioma" or "elephantiasis." Frequently, pathologically altered vessels were excised without adequate indications. Only after the publication of works by M. V. Volkov, A. A. Arenberg (1964), G. A. Bairov et al. (1965), L. M. Boychenko (1965), P. P. Alekseyev (1966), A. V. Bondarchuk (1968), Yu. A. Tikhonov (1970), G. A. Fedoreyev (1971), A. V. Pokrovskiy et al. (1971), Olivier (1957), Malan, Piglionisi (1964), and others, did the Etiology, Pathogenesis, Clinical Features, and diagnostic principles of these malformations become clear, and objective indications for various treatment Methods were developed.

ETIOLOGY AND PATHOGENESIS. Most researchers currently attribute the causes of peripheral vascular malformations of the extremities to impaired Embryogenesis and subsequent abnormal development of vascular trunks and walls. The latter result from both genetic anomalies and teratogenic influences on the fetus during the first months of Pregnancy. The specific hemodynamics within a pathologically formed vessel lead to stasis, secondary trophic disorders, and the corresponding clinical picture.

Currently, peripheral vascular malformations in children are classified into: a) venous malformations, b) arterial malformations, c) arteriovenous malformations, d) lymphatic vessel malformations, and e) combined vascular malformations (Yu. A. Tikhonov, 1970).

Venous Malformations

Malformations of the superficial Veins OF THE extremities (congenital phlebectasia) are most commonly observed in children during the first days and weeks of life. In this condition, varicose dilation of the subcutaneous veins—typically of the lower extremities—is noted immediately after birth or during the first weeks and months of life. The pathologically altered vessels appear as thickened, tortuous trunks, nodes, or conglomerates. Combinations of Various Forms of varicosity are frequent. In the majority of patients, the Skin overlying the dilated vessels is thinned and cyanotic at birth. Upon Palpation, the expanded vessels are easily compressible. The "sponge" sign is characteristic—a significant reduction in the limb volume upon compression and its rapid restoration after the release of pressure. In severe cases, an increase in limb length and secondary changes in Muscles and bones may occur. Subsequently, pain during movement develops, the limb increases in volume, and phleboliths form within the anomalous vessels.

The diagnosis is refined using specialized diagnostic methods. On plain radiographs of the affected extremities, characteristic findings include Muscle atrophy, bone curvature, and bone thinning. Venous pressure in the abnormally developed vessels is generally slightly elevated or remains normal. Blood oxygen saturation also remains within normal limits, and only in isolated patients with a marked impairment of tissue oxygen uptake is a 2–3% increase in venous blood oxygen saturation observed. Contrast examination of the pathological veins—venography—is decisive for establishing the diagnosis. The radiograph clarifies the extent and localization of venous involvement, the state of the deep veins, and the presence of anastomoses with the Arterial System. The choice of treatment method can be determined only after a comprehensive evaluation of the child, including contrast venography.

Following discharge from the maternity hospital, an infant with superficial venous malformations of the extremities must be examined by a pediatric surgeon and registered for further follow-up.

Treatment of the malformation is surgical, involving the excision of the abnormally developed veins. Depending on the extent of the vascular involvement and the child's condition, the surgery is performed at the age of 1–3 years. Timely Surgical treatment allows for the cure of the majority of patients with superficial venous malformations of the extremities.

A rarer malformation of the deep veins is Klippel-Trenaunay syndrome. The Essence of the malformation lies in the narrowing of a venous segment, its complete absence, or the compression of the venous wall by an abnormally formed muscle, artery, or embryonic band. The presence of this obstruction leads to impaired venous outflow from the deep veins of the limb and The Development of a clinical picture characterized by a triad of symptoms: cutaneous vascular or pigmented nevi, varicose veins, and limb hypertrophy. The veins of the lower extremities are typically affected. At birth, the clinical manifestations of the malformation may be negligible or entirely absent. In newborns, vascular or pigmented skin spots on the affected lower leg are observed more frequently and earlier than other manifestations of Klippel-Trenaunay syndrome. Varicose veins in these patients are localized predominantly on the lateral aspect of the limb, which is explained by the persistence of the marginal vein lumen, a vessel that normally obliterates during vascular development. Later, a feeling of heaviness in the limb, rapid fatigue, and edema appear. Subsequently, trophic disorders develop, progressing to ulceration. In some patients, the disease is accompanied by Hematuria and intestinal bleeding. Unlike superficial venous malformations, Klippel-Trenaunay syndrome is characterized by a significant increase in venous pressure distal to the site of obstruction. Contrast venography clarifies the presence and localization of the blood flow obstruction.

Upon noting hyperpigmented or vascular spots and varicose veins on a newborn's limb, the maternity hospital physician refers the infant to a surgeon, who performs special examinations if necessary to clarify the diagnosis.

Treatment for Klippel-Trenaunay syndrome is surgical, consisting of relieving the cause of venous compression or creating a bypass anastomosis. Excision of varicositically transformed veins in such children is contraindicated, as they serve as the sole pathways for venous drainage.

Arterial Malformations

Congenital arteriovenous fistulas and arterial aneurysms (Parkes Weber syndrome) are the most common. Parkes Weber syndrome is the most severe congenital vascular malformation of the extremities. The core of the malformation is the abnormal formation of both the arterial and venous systems, between which multiple wide shunts persist. Arterial blood bypasses the capillaries and is shunted through these connections into the veins, leading to venous Hypertension, impaired trophics of the affected limb, and increased cardiac workload. Clinical manifestations comprise both local and general symptoms. In newborns, local symptoms predominate—pronounced Hypertrophy of the affected organ, varicosity of the affected limb's veins, and the presence of hyperpigmented or vascular spots at the sites of anomalous vascular development. Pulsation of the dilated veins and enhanced pulsation of the affected limb's vessels are characteristic. Auscultation frequently reveals a bruit over the site of anastomosis development and along the major vessels. Special examinations reveal a distinct elevation in skin Temperature over the lesion site and a sharp increase in venous blood oxygen content. Angiography, which is mandatory in such patients, identifies arteriovenous fistulas.

Treatment is surgical, involving ligation of the fistula or excision of the anomalous vessels. In cases of mild circulatory impairment, the initial clinical signs of the malformation may appear at the age of several months.

Lymphatic Vessel Malformations

Congenital lymphedema may manifest in newborns as chronic, typically slowly progressive edema of the subcutaneous adipose tissue of the affected limb or genitalia. The underlying cause of the malformation is a congenital anomaly of lymphatic vessel development. These vessels may be hypoplastic, hyperplastic, or entirely absent. Secondary impairment of lymphatic drainage due to a mechanical barrier (amniotic bands, intrauterine scarring) is also possible. The affected limb—most commonly the lower one—is uniformly increased in volume due to pronounced subcutaneous edema. Initially, the distal parts are typically affected. Concomitant true and partial gigantism of the abnormal organ may be present. In secondary lymphatic drainage disorders in patients with congenital scars or amniotic bands proximal to the obstruction, the limb develops normally, whereas the distal parts are markedly edematous. In the Cytology/cytology/16.html">Early stages of the disease, the edema may be intermittent.

Treatment. Prompt conservative treatment is recommended (A. A. Troshkov, 1969), consisting of daily massage, Electrophoresis with a 5% potassium chloride solution (every other day for 30 sessions, 2–3 courses per year), and electrophoresis with a 3% Trypsin solution from the negative pole (in the same volume). According to A. A. Troshkov, such conservative treatment resulted in the complete cure of 5 children.

Tight bandaging is contraindicated to prevent ischemia and progression of the lesion.

When elephantiasis is localized in the genital area, accompanied by urinary passage disorders or marked compression of the corpora cavernosa by edema—which may impair their development—surgical treatment is indicated regardless of the child's age. Elective surgery is recommended after the age of 2 years.

In Conclusion, it should be noted that congenital peripheral vascular malformations of the extremities are severe conditions that lead to patient disability. Because these malformations manifest in the first days and weeks of Life in the majority of cases, the maternity hospital physician must make a correct and timely diagnosis. Further management strategy is formulated by a specialist surgeon after the diagnosis is refined. Only timely surgical intervention can cure the patient.

Musculoskeletal System

Congenital Malformations of The Musculoskeletal System account for the majority of human congenital anomalies, with the upper and lower limbs most frequently affected. Together with spinal malformations and certain systemic Disorders of the osteoarticular apparatus, they comprise approximately half of all congenital diseases and birth defects in children.

The etiology of musculoskeletal malformations is diverse, involving the interplay of exogenous and endogenous factors, as well as genetic disruptions. Observations by V. A. Sturm (1968) demonstrated that exogenous factors underlie 10 — 12% of congenital disorders and malformations, while endogenous factors account for approximately 12 — 15%, and hereditary (genetic) factors for no more than 4 — 6%. E. K. Nikiforova notes that deformations are hereditary in 5 — 7% of patients.

Timely recognition and early treatment of congenital deformities in newborns help prevent disease progression. The preventive approach in modern pediatric orthopedics is grounded in the necessity of diagnosing congenital deformities directly in the maternity hospital. Treatment is initiated from the first days of a child's life, whereas surgical intervention is reserved for cases where conservative measures prove ineffective or unfeasible (M. V. Volkov, 1957).

Congenital hip dislocation

This malformation affects all Components of the hip joint: the acetabulum, the femoral HEAD and Neck, the proximal Femur, the Joint Capsule and ligaments, and the surrounding musculature. Hip Dysplasia occurs in 16 per 1,000 newborns, whereas hip dislocation affects 5 per 100.

Pathogenesis. Attempts to link the onset of dislocation to birth trauma have been unsuccessful; for instance, M. V. Volkov observed 25 infants with pre-dislocation and hip dislocation who were delivered via Cesarean Section.

Several authors attribute the development of congenital hip dislocation to delayed INTRAUTERINE DEVELOPMENT OF a normally formed hip joint.

Recent observations have demonstrated that fetal position plays a significant role in the development of congenital hip dislocation. Intrauterine adduction of the lower limbs leads to overstretching of the posteroinferior joint capsule and abnormal alignment of joint structures in the fetus, subsequently resulting in developmental delay. Observations by M. V. Volkov, R. L. Gorbunova, and I. P. Elizarova (1966) revealed that 50% of mothers who gave birth to children with hip pre-dislocation experienced oligohydramnios and breech presentation, which led to marked compression and adduction of the hips.

Genetic studies provide strong evidence that Hip Dysplasia and congenital hip dislocation are genetically linked pathologies.

Dysplasia is a genetically determined developmental anomaly that, under predisposing conditions, can lead to The formation of a congenital hip dislocation.

Diverse interpretations regarding THE ORIGIN OF hip dislocation have led to inconsistencies in terminology. The abnormal Formation of the acetabulum and the proximal femur in congenital hip dislocation is primarily caused by the lack of proper contact between them. Based on METABOLISM/2.html">THE CONCEPT OF primary hip dislocation (pre-dislocation), it is considered that if infants are born with a lax hip joint capsule and exhibit dislocation and reduction of the femoral head within the acetabulum, the condition should be termed hip pre-dislocation. If the femoral head is displaced upward and outward, without extending beyond the limbus, it is classified as subluxation. In cases where the femoral head completely loses contact with the acetabulum, the limbus inverts into the acetabulum, the head moves past the limbus, and a true hip dislocation occurs.

Dysplasia refers to Developmental anomalies of the hip joint observed during the first months of life that typically do not progress to hip dislocation when preventive measures are applied (M. V. Volkov, G. M. Ter-Egiazarova, G. P. Yukina, 1972).

Clinical presentation. The diagnosis of congenital hip dislocation must be established within the first days and weeks of life. To achieve this, all newborns are examined by a pediatrician or orthopedist in the maternity hospital. A follow-up preventive examination is conducted at 3 months of age by a pediatric surgeon (orthopedist) at the regional outpatient clinic.

The principal early symptoms of congenital hip dislocation include the Ortolani (telescoping/click) sign, limitation of hip abduction, Asymmetry of the gluteal folds, shortening of the lower limb, and external Rotation of the leg.

Class="center">Early signs of congenital hip dislocation: shortening of the thigh on the affected side (top left); limitation of hip abduction (top right); asymmetry of the skin creases on the thigh (bottom left).

Dennison positioning for a newborn presenting with Robin sequence.

Samoylovich table designed for the treatment of diseases and INJURIES OF THE lower extremities in newborns. For femoral fractures, the table facilitates Blount traction (left), whereas for pathological and congenital hip dislocations, it allows for titrated axial traction of the limb combined with simultaneous abduction (right).

Surgical table for newborns equipped with a Water-heated mattress (left). ERGA MP electroroentgenographic unit. Electroroentgenography is widely utilized in the clinical diagnosis of bone, soft tissue, and pulmonary pathologies in neonates. Color Vision experiments have demonstrated that yellowish and greenish tinted papers, rather than standard white bond paper, yield superior print quality (right).

   The telescoping sign (the "click" sign according to V. O. Marx, sign of instability) is elicited with the infant in the supine position (Fig. 105). The child's hips and knees are flexed; the physician's thumbs are placed on the medial aspect and the remaining fingers on the lateral aspect of the thighs, with the tip of the third finger pressing against the greater trochanter. When applying axial traction, exerting pressure with the thumb toward abduction, and abducting the hips, the reduction of the femoral head into the acetabulum can be felt (a). This sign can also be demonstrated using an alternative maneuver that induces dislocation rather than reduction. Instead of axial traction, pressure is applied to the knee region while simultaneously adducing the hips (b). A positive sign is accompanied by a piston-like gliding sensation of the femoral head. The telescoping sign indicates hip joint instability; it is characteristic of newborns and may resolve spontaneously without treatment by the 7th to 10th day. In some instances, it persists longer (up to 3 months), in which case it becomes pathognomonic for congenital hip dislocation.

Fig. 105. Demonstration of the telescoping sign in a newborn with congenital hip dislocation.

Limited hip abduction is observed in all children with congenital hip dislocation. To elicit this sign, the physician gently and gradually abducts the infant's legs, flexed at the knee and hip joints. Normally, hip abduction in the first months of life ranges up to 80°. This sign is particularly striking in unilateral involvement. It may also be noted in spastic paralysis, congenital coxa vara, and pathological hip dislocation.

Asymmetry of the gluteal folds is assessed with the infant in the prone position. Attention is directed to the level of the gluteal folds—in unilateral involvement, they may lie at different levels and vary in depth. This sign is present in 50% of patients. Some authors note a difference in the number and depth of skin creases on the inner aspect of the thighs. This sign is inconstant and typical only of pronounced hip dislocation.

Shortening of the lower limb is likewise an inconstant sign. In the first months of life, it is rarely detected and occurs only in high hip dislocations. With the infant in the supine position, the legs are flexed at the knee and hip joints and brought together. Leg length discrepancy is judged by the relative levels of the knee joints.

External rotation of the lower limb occurs in unilateral dislocation on the affected side. This feature is particularly noticeable during infant Sleep and is sometimes brought to attention by mothers. However, it should be kept in mind that external rotation may also occur with normally developed hip joints.

If signs of congenital hip dislocation or hip dysplasia are present, radiographic examination is mandatory regardless of the child's age.

Radiographic findings. An anteroposterior radiograph is taken with the limbs in a neutral position. Positioning of the infant in the apparatus must be strictly symmetrical. The radiograph is evaluated for the shape of the acetabular roof, the acetabular angle, the distance from the center of rotation of the femoral head to Hilgenreiner's horizontal line, and the Development of the ossification centers of the femoral head (Fig. 106).

Fig. 106. Radiographic diagnosis of congenital hip dislocation.

In a healthy infant, the angle between the line drawn through the triradiate cartilages and the line of the acetabular roof (the acetabular index) reaches 30°, while the distance from the center of rotation of the head—located 1–1.5 mm above the midpoint of the metaphyseal plate—to the horizontal line is always constant at 8–12 mm (a). In subluxation, the acetabular index exceeds 30°, the distance is decreased, and the head is displaced laterally relative to the level of the acetabular roof (b). In dislocation of the hip, the acetabular index is greater than 30°, the distance is less than 8 mm, and the femoral head is displaced laterally and upward (c).

   In a normal hip joint, the acetabular roof in the first days and months of life is slanted and appears flat. As the child grows, a characteristic bony prominence forms. The value of the acetabular angle is determined by drawing a horizontal line through the triradiate cartilages and a line along the acetabular roof. This value defines the degree of inclination of the bony acetabular roof. Normally in newborns, the acetabular angle reaches up to 30° and gradually decreases with age. In congenital hip dislocation, even without marked Displacement of the head, the acetabular angle is always greater than 30°.

In the first months of life, the ossification centers of the femoral heads are not visualized. They become visible only by 3 months of age, and later in the presence of pathology. Therefore, determining the center of rotation of the femoral head is of great importance; it is located at a point 1–1.5 mm above the midpoint of the metaphyseal plate visible on the radiograph. In a normal hip joint, the center of the head and the center of the acetabulum coincide and do not change position regardless of THE POSITION OF the hip. In congenital hip dislocation, when the head is displaced, its center does not coincide with the center of the acetabulum. The distance from the center of the head to Hilgenreiner's horizontal line is normally 8–11 mm and remains constant in all positions of the hip.

Based on the aforementioned signs, Differential diagnosis OF various degrees of hip joint developmental impairment is possible.

Upon detecting early signs of congenital hip dislocation, an orthopedic regimen or The Use of one of the braces (see below) is prescribed, and in 3–4 weeks a repeat anteroposterior radiograph with hip abduction is performed. If the radiograph shows an increase in the distance, it should be considered that the patient has a hip subluxation, and as a result of head centralization, the hip position is normalizing. If the distance decreases, hip dislocation is suspected, because upon leg abduction, reduction of the head into the acetabulum did not occur. If the distance remains unchanged, the hip joint is considered normal but not yet mature. Most often, this radiographic pattern is observed in infants in the first months of life when preluxation of the hip has resolved.

Treatment. Treatment of a newborn with congenital hip pathology begins from the first days of life (Fig. 107). In preluxation of the hip, wide swaddling with a soft abducting bandage and therapeutic gymnastics aimed at eliminating limited hip abduction are prescribed. If joint stabilization occurs within 2–3 weeks (the Ortolani/telescoping sign disappears), wide swaddling can be continued for 2 months along with therapeutic gymnastics.

Fig. 107. Methods of treatment of congenital hip dislocation.

Subluxation is treated with a Frejka pillow (a), a custom-made abduction brace (b), a Wilensky splint (c); dislocation is treated with a CITO harness brace (d), Pavlik harness (e), and a Pavlik harness combined with plaster boots that enhance hip abduction (f) (after Bart

Physicians' confidence in the safety of early treatment has led to the occasional application of an abduction brace when dysplasia or hip dislocation is suspected. This is unjustified, especially if the brace is applied in a position of extreme hip abduction. Such a position may lead to Impaired blood supply to the proximal end of the femur, avascular Necrosis of the femoral head, deformity, and the subsequent development of deforming arthrosis. Therefore, targeted differentiated treatment should be initiated only when there is no doubt about the diagnosis (G. M. Ter-Egiazarov, G. P. Yukina, 1972).

In cases where preluxation of the hip has not resolved and subluxation or dislocation of the hip has developed, conservative treatment aimed at the slow and gentle elimination of adduction contracture is indicated. This is achieved using various spreader braces (Wilensky splint, custom-made spreader brace, etc.). If the femoral head is well centered in the acetabulum within the brace, fixation is continued for 4–6 months depending on The rate of joint formation. Control radiographs are taken approximately once every 3 months.

In established hip dislocation, hip abduction must be gradual and reach 80–90°, which is necessary for the reduction of the femoral head into the acetabulum. For this purpose, the CITO splint, Pavlik harness, etc., are used. Following centralization of the femoral head, treatment is continued for an average of about a year. In cases of incomplete abduction in a Pavlik harness, Barta (1972) applies plaster casts to the lower legs. Under the action of this additional weight, the adduction contracture relaxes, and reduction of the dislocation occurs.

Congenital Clubfoot

Congenital clubfoot occurs in 1 out of every 1,000 newborns. Congenital contracture of the FOOT joints is characterized by three main components: equinus (plantar flexion of the foot at the ankle joint), supination of the foot (inward rotation with lowering of the lateral border), and adduction (adduction of the foot with a simultaneous increase in the longitudinal arch).

   The deformity can be typical or atypical. The latter includes clubfoot associated with Arthrogryposis, amniotic bands, and bone defects.

Treatment begins from the first days of the child's life. In the first weeks of life, until the skin becomes stronger, corrective gymnastics and bandaging with a soft flannel bandage according to Finckh-Eettingen are performed 6–7 times a day (Fig. 108). From the 2nd month of life, treatment with serial plaster casts is initiated, which are changed every 7–10 days. In the majority of patients, complete correction of the deformity is achieved by 6–7 months of age.

Fig. 108. Bandaging of the limb according to Fink-Ettingen for congenital clubfoot (a) and congenital Flatfoot (b).

Congenital Flatfoot

This deformity is the opposite of clubfoot and consists in the lowering of the midfoot, its pronation, some abduction, and a valgus position of the heel. According to T. S. Zatsepin, congenital flatfoot accounts for 11.5% of congenital Foot deformities.

The arch of the foot is flat, without contours, and convex in severe cases. The outer edge of the foot is raised, while the inner edge is lowered. The forefoot is abducted, and the heel is deflected outward. The foot is strongly dorsiflexed and, in the most pronounced cases, lies against the anterior surface of the lower leg. Skin folds form along the outer surface of the ankle joint.

Treatment. Correction of congenital flatfoot is less difficult than that of clubfoot, since the deformity is not as stable. Conservative treatment begins from the first days of the child's life and is carried out in the same way as for clubfoot, only in the opposite direction. During the first weeks of life, Therapeutic Exercises are performed 6 to 7 times a day. For severe deformity, bandaging with a soft flannel bandage is indicated. If there is no tendency toward correction, one should proceed to the application of serial corrective plaster casts, changed every 7 to 10 days until the position of the foot is completely corrected.

Congenital Muscular Torticollis

Congenital muscular torticollis—shortening of the sternocleidomastoid muscle on one or both sides—ranks third in frequency (after congenital hip dislocation and congenital clubfoot).

The cause of torticollis is microtrauma or overstraining of young, immature tendon tissue during childbirth, followed by a peculiar reaction of fibrous tissue with its hyperproduction (S. Ya. Doletsky, G. Ya. Fishchenko, 1968).

Clinical picture. In the first days of the child's birth, the symptoms of torticollis are not pronounced. By the end of the 2nd to the beginning of the 3rd week of life, a "tumor" appears—the sternocleidomastoid muscle thickens and becomes denser in the middle and lower thirds. The head tilts toward the affected side, and facial asymmetry is visible in A number of patients.

Diagnosis is based on the characteristic signs of the anomaly.

Difficulties may arise in bilateral lesions, which are quite rare and manifest as a forward head tilt, limited mobility in the cervical spine in all directions, and increased cervical lordosis (O. V. Dolnitsky, V. A. Skiban, 1974).

Treatment. Conservative treatment begins from the moment symptoms appear. Treatment consists of corrective positioning, gentle (non-forceful) passive therapeutic exercises, physiotherapy (electrophoresis with potassium iodide, UHF), and massage of the healthy sternocleidomastoid muscle. Due to the immaturity of the tissue of the affected muscle, forceful manual correction (redression) and massage on the affected side, recommended by some authors, are contraindicated, as they can lead to an increase in the hyperregenerative scar process. Surgery is performed in children older than one year.

Congenital High Scapula

In 1891, Sprengel reported 4 cases of such a deformity, and since then it has been known as Sprengel's deformity. In congenital high scapula, there is its delayed development caused by defects in the Ossification of the spine and the formation of the chest wall. The scapula is rotated around the sagittal plane, its lower angle is brought closer to the spine, and its outer edge is tilted downward.

According to Kirmisson's theory, the cause of the high scapula is a delay in its descent. As a result of various embryogenesis disorders, the scapula remains at the level of the IV or V cervical segment.

Clinical picture. The shoulder girdle on the affected side is somewhat higher than on the healthy one. The dimensions of the scapula are reduced, and the upper angle protrudes on the side of the neck. Synostosis with the VI or VII cervical vertebra is possible. Due to the improper position of the scapula, a limitation of movements in the shoulder joint is revealed—raising the arm is possible only up to 90°.

Treatment. From the first days of the child's life, conservative measures are used in the form of corrective gymnastics, positioning for arm abduction, and massage. For complete correction of the scapula position and restoration of movements in the shoulder joint, surgical treatment is necessary, which is indicated at 5 to 6 years of age.

Congenital Dislocation of the Radial Head

Congenital dislocation of the radial head is a rare developmental anomaly. The main clinical manifestation is moderate deformity and limitation of limb function. The head of the radius may be dislocated anteriorly, laterally, or posteriorly. Limitations of flexion and extension are noted. Rotational Movements of the forearm are usually not affected, though supination may be limited.

During the neonatal period, active range-of-motion exercises and massage are contraindicated due to the possible trauma to muscles and periarticular Tissues. Surgical treatment is performed at the age of 3 to 6 years.

Congenital Clubhand

Congenital clubhand is a consequence of the shortening of the ligaments on the palmar-radial side, shortening of muscle tendons, and underdevelopment or absence of the radius or ulna. In the first case, congenital clubhand can be regarded as a contracture of the wrist joint in a position of flexion and adduction, similar to congenital clubfoot. Since this form of clubhand is often combined with contractures in other joints, it can be considered an atypical form of arthrogryposis.

The absence or underdevelopment of the radius can be unilateral or bilateral, and is often accompanied by the absence of the thumb and corresponding Carpal Bones, as well as underdevelopment of the muscles on the radial side of the forearm. Due to the lack of support, the hand shifts toward the radial side and assumes an angle relative to the forearm. X-rays show the complete absence of the radius or underdevelopment of its peripheral section, along with shortening and curving of the ulna.

With congenital underdevelopment or absence of the ulna, the forearm is shortened, the hand is shifted somewhat toward the ulnar side and forms an angle with the forearm. Complete absence of the ulna is extremely rare; usually, underdevelopment of the distal part of the bone is noted. The pisiform, triquetrum, and hamate bones, the IV and V fingers, and the Muscles of the ulnar edge of the forearm may be absent.

Treating congenital clubhand is a challenging task. Restoring hand function is possible only through surgical intervention. Conservative measures begin within the first days of a child's life, aiming to correct contractures and prevent their progression. A complex of modeling and corrective exercises, massage, and gentle manual reduction combined with fixing plaster casts or fast-Setting plastic splints is prescribed.

Various braces are used subsequently. Children suffering from clubhand must wear orthoses for an extended period. Surgery is indicated at the age of 10–12 years.

Congenital radioulnar synostosis

This deformity results from the incomplete Separation of the forearm bones from the common mass of mesenchymal tissue during early embryonic development.

The clinical presentation is typical. While flexion and extension movements in the elbow joint are fully preserved, rotational movements of the forearm are absent, and the forearm remains fixed in pronation. The synostosis is always located in the proximal forearm and varies in extent. In some cases, synostosis manifests during the first months of life only clinically, without radiographic signs of bony fusion. Such cases warrant intensive mobilization of rotational movements to bring the forearm into a mid-position between pronation and supination. If bony fusion is present, surgical treatment is indicated at the age of 3–4 years.

Syndactyly

Syndactyly refers to the complete or partial fusion of two or more fingers. The deformity stems from an arrest in hand development during early embryogenesis; therefore, it is more accurate to describe it as complete or partial non-separation of the digits. Webbed, cutaneous, osseous, and tip (terminal) forms of syndactyly are distinguished.

In the webbed form of syndactyly, the fingers are connected by a skin bridge resembling a web. This bridge may connect all fingers or just two—the thumb and index finger. Finger mobility in this form remains largely preserved.

The cutaneous form of syndactyly is the most common. In this condition, two or more fingers are completely fused from the base to the nail. Finger mobility is significantly impaired, allowing only simultaneous movements.

In the osseous form of syndactyly, the fingers are fused either across a single phalanx or along the entire length of the digit.

In the tip (terminal) form of syndactyly, the distal PARTS OF THE fingers remain unseparated while the bases are not fused. This form can be either cutaneous or osseous and is frequently associated with underdevelopment, digital deformities, and amniotic bands.

Treatment for syndactyly is surgical. Determining the optimal timing for surgery is crucial for successfully restoring digital shape and hand function. For webbed and cutaneous forms, surgery is postponed until 5–6 years of age. For the terminal form, especially in complex deformities, surgery is performed within the first 2 years of life to prevent further digital deformity and restore function.

Polydactyly

Polydactyly is sometimes hereditary and affects both the hands and feet. Marginal polydactyly is the most common type. Duplication of the first digit (thumb) occurs on the radial side, while duplication of the fifth digit (little finger) appears on the ulnar side. In rare instances, multiple digit duplications occur, bringing the total number of fingers on a hand up to ten. An accessory finger sometimes hangs loosely by a skin pedicle, whereas in other cases, it represents a normally functioning digit with a developed metacarpophalangeal joint.

Treatment. Accessory digits are removed during the first months of life. Rudimentary, freely hanging fingers are excised (amputation by tying a thread around the base should be avoided). When both fingers are functional, the marginal one is excised.

Occasionally, digital hypoplasia occurs where the normal number of fingers is present, but the metacarpal bone and Phalanges are absent. The base of such a finger is sharply narrowed, causing it to hang loosely. In these cases, soft tissue surgical correction is indicated within the first weeks of life to prevent circulatory disturbances.

Osteoplastic surgeries can be undertaken later in childhood.

Amniotic Bands

Amniotic bands are congenital thread-like constrictions along limb segments that involve soft tissues to varying depths, sometimes reaching the bone. It has been proven that, excluding genetically determined cases, congenital constricting bands result from amnion rupture. Strands originating from the amnion and chorion constrict the fetal limbs (Field, Krag, 1973).

Deep amniotic bands cause not only cosmetic defects but also various trophic disorders (lymphedema, trophic ulcers, skin anesthesia). They occur most frequently on the lower leg, forearm, and fingers.

Treatment is surgical. Pronounced trophic disorders warrant emergency surgery, which involves excising the band in one or two stages (Fig. 109). Z-plasty or triangular flapplasty is sometimes necessary.

Fig. 109. Amniotic band of the upper third of the right lower leg in a newborn.

a — extreme degree of blood supply impairment. Severe limb edema, lymphovenous stasis. Threat of spontaneous limb amputation; b — the same patient after The First stage of the surgery—excising a semicircle of the amniotic band. Multiple incisions on the skin of the lower leg and foot; c — the same patient after the Second Stage of the surgery—excising the second semicircle of the amniotic band; edema has practically resolved.

Congenital spinal deformities

Congenital spinal deformities manifest as Scoliosis or Kyphosis. Congenital scoliosis results from the presence of a wedge vertebra or an accessory hemivertebra and is characterized by a localized curvature of a short segment of the spine (Fig. 110). Rapid progression of the deformity is observed in cases of non-synostosed hemivertebrae and rib involvement.

Fig. 110. Electro-roentgenogram of the thoracic spine of a 3-month-old infant. Diagnosis: congenital kyphoscoliosis due to hypoplasia and vertebral blocks. Anteroposterior (a) and lateral (b) projections in mirror image.

Congenital kyphosis is caused by a posterior accessory hemivertebra, a wedge vertebra, or a partial fusion of the anterior aspects of the vertebral bodies.

Treatment. For scoliosis, treatment aimed at strengthening the back muscles is indicated from the first weeks of life. As soon as the infant is able to hold their head up, they should be placed prone on a hard surface, and back muscle gymnastics and massage should be performed. This approach achieves stabilization and, in some cases, correction of the deformity (Walch, Kühn, Paschohl, 1972).

Surgical intervention is sometimes necessary at around one year of age (G. A. Bairov). Treatment for kyphosis is surgical in the majority of cases.

Arthrogryposis

Arthrogryposis is a severe developmental disorder characterized by marked contractures of all limb joints. The Clinical presentation of arthrogryposis in a newborn is quite typical. The joints are fixed in a contracted position, and range of motion is severely restricted. Clubhand and clubfoot are observed. The hip joints present with flexion contractures. The upper limbs are extended and internally rotated. Hip dislocations are frequently noted.

The cause of arthrogryposis is presumably a delay and distortion of embryogenesis. Arthrogryposis is not classified among clearly Hereditary diseases.

Treatment is initiated in the first days after life. Therapeutic measures are directed toward restoring joint mobility using passive therapeutic exercises, eliminating contractures, and correcting abnormal limb positioning. Surgical treatment is indicated at 5 to 6 years of age.

Osteogenesis Imperfecta (Congenital Fragility of Bones)

Osteogenesis imperfecta is a systemic skeletal disorder associated with an anomaly in Bone Formation. Two forms of the disease are distinguished: congenital osteogenesis imperfecta, in which bone fractures occur during the intrauterine period in the fetus, and late osteogenesis imperfecta, where fractures and deformities develop after birth.

The etiology and pathogenesis of the condition remain unclear. Some authors associate it with Central Nervous system disorders, while others link it to

Pathology of the Endocrine System, which is supported by the cessation or significant reduction of the number of fractures after Puberty. Still others consider this disease to be a consequence of impaired phosphorus-calcium metabolism. The hypothesis that has gained the most supporters explains the development of the disease as a defect in the Skeletal System, osteoblast dysfunction, and periosteal dysplasia.

Clinical presentation. Osteogenesis imperfecta is characterized by multiple fractures and bone deformities (Fig. 111). Fractures result from trivial causes—such as during swaddling, dressing the child, etc. A distinctive feature of these fractures is the absence of displacement along the longitudinal axis and rapid healing.

Another frequently encountered sign is the blue color of the sclerae. Deafness develops by the age of 20 to 30 years as a result of rapidly progressing otosclerosis.

In the congenital form of the disease, the physical development of children is severely impaired. In some cases, the newborn infant proves to be non-viable or dies in the first years of life from a secondary infection. The course is more favorable in the late form of the disease. However, marked deformities of the Skull, Ribs, and limbs are noted. Upon completion of puberty, fractures cease completely or become relatively rare.

Fig. 111. Radiograph of a newborn with osteogenesis imperfecta. Multiple fractures of the BONES OF THE lower (a) and upper (b) extremities.

Congenital Chondrodystrophy (Chondrogenesis Imperfecta)

Chondrodystrophy is a systemic disease based on a developmental defect of the embryonic chondroblastic system. The etiology remains unclear to this day. The essence of the disease lies in the impairment of enchondral Development of the skeletal system. The bones of the limbs and the Base of the skull—bones of primary ossification—are affected. The flat bones of the cranial vault and the clavicles, which ossify via Connective Tissue, are not involved in the process. As a result of the defective, disordered arrangement of the growth Cartilage Cells, the regularity of ossification is disrupted as early as the 3rd to 4th week of embryonic development, and longitudinal bone growth is stunted. Periosteal and endosteal ossification are not impaired. The disease manifests already during the intrauterine period. Some fetuses die in utero, while others prove non-viable as a result of premature birth. Children born at term are fully viable and, despite developing deformities, survive into old age.

Clinical presentation. The primary sign of chondrodystrophy is limb shortening. The upper limbs in a newborn barely reach the umbilicus. The proximal segments of the limbs are shortened to a greater degree. As a result of normal periosteal growth, all tubular bones are thickened, curved, and nodular. The epiphyses are widened and deformed. Macrocephaly is observed. The frontal and parietal tubers bulge, and the cranial vault overhangs the face.

Treatment aims to prevent and correct developing deformities. It is necessary to prevent children from standing and walking at an early age. Unlocked orthotic braces are recommended for deformities. Established deformities are managed with corrective osteotomies. Recently, attempts have been made to use anabolic hormonal preparations to stimulate longitudinal bone growth.

Spinal Hernias

Congenital spinal hernias, characterized by the protrusion of Spinal Cord elements through defects in the vertebral arches, occur in an average of one in 3,000 newborns (S. D. Tornovsky, 1959). This malformation results from a disruption in the formation of the spinal canal during early stages of embryogenesis.

Depending on The Nature of the hernial sac contents, distinction is made between meningocele (protrusion of the spinal Meninges only), myelomeningocele (the hernial sac contains both meninges and spinal cord elements), myelocystocele (containing a deformed spinal cord within the hernial sac), rachischisis (complete cleft of the spine and spinal cord), and Spina bifida occulta (hidden non-fusion of the vertebral arches).

In most cases of spinal hernias, the hernial protrusion is localized in the lumbosacral region of the spine, though thoracic and cervical localizations of the defect are also possible.

Clinical presentation. Diagnosis is generally straightforward. The infant is born with a tumor-like mass whose Location corresponds to the spinal defect. Depending on the size and Nature of the defect, the hernial protrusion is covered either by normal or sharply thinned skin. In some patients, rupture of the membranes occurs during labor, resulting in CEREBROSPINAL FLUID leakage present at birth. In other cases, the hernial membranes rapidly undergo necrosis and rupture, accompanied by cerebrospinal fluid leakage and subsequent infection.

In rachischisis, the spinal cord is completely exposed and lies at the base of the wound. Spina bifida occulta often remains unrecognized during the neonatal period. In doubtful cases, the diagnosis is clarified using radiography. Radiographs of the spine readily reveal vertebral defects. In the majority of infants with spinal hernias, distinct neurological symptoms are evident at birth, including paresis and paralysis of the lower extremities, impaired sphincter function of the bladder and rectum, and trophic disorders, all of which significantly worsen the prognosis. In a high percentage of cases, cerebrospinal fluid Circulation disorders rapidly ensue, leading to the development of Hydrocephalus.

Treatment. Surgical elimination of the hernial protrusion, plastic repair of the vertebral defect, and shunting of the Lateral ventricles into the right atrium using a Holter valve in patients with hydrocephalus are indicated in the first days of life. Early surgical treatment eliminates the possibility of cerebrospinal fluid leakage and infection, preventing irreversible Brain damage. In the absence of skin changes over the hernial protrusion and in the absence of neurological disorders, the surgery is postponed to a later age. Treatment of patients with paresis and paralysis presents considerable difficul-

ties and is of low efficacy. The prognosis depends on the extent of spinal cord and brain damage. Infants with rachischisis are, in most cases, nonviable.

The best treatment outcomes for all forms of spinal hernia through emergency surgery in newborns, followed by comprehensive management by a surgeon, orthopedist, urologist, and neurosurgeon, are reported by Zachary (1971).

Sacrococcygeal Teratomas

A large number of works in both domestic and foreign literature are devoted to the etiology, diagnosis, and treatment of sacrococcygeal teratomas (S. D. Ternovsky, 1959; F. G. Uglov, R. A. Mursalova, 1959; A. G. Pugachev, V. V. Gavryushov, 1963; Gross, 1951; Hunt, 1968; Burl, 1970, et al.).

Embryogenesis. The occurrence of teratomas is explained by The complexity of the embryonic development of the caudal end of the human embryo, where all three germ layers—endoderm, mesoderm, and ectoderm—meet during embryogenesis. Therefore, among the theories explaining the origin of sacrococcygeal teratomas, the most widely accepted is The Theory of late-cleaving or improperly segregated blastomeres (N. N. Petrov, 1903). This viewpoint is supported by the presence of derivative elements from all three germ layers within teratomas.

Over the period from 1957 to 1972, we observed 24 newborns with sacrococcygeal teratomas.

Identifying the exact cause of teratoma development is not feasible. In 20% of cases, the teratoma is combined with other developmental defects, such as spinal hernias, skull bone malformations, and Urogenital System anomalies. In our observations, such a combination occurred in 5 patients.

Clinical presentation. Diagnosis of sacrococcygeal teratomas presents no difficulties in the majority of cases. The infant is born with a tumor-like mass in the sacrococcygeal region that displaces the anus anteriorly and is frequently fused with the coccyx (Fig. 112). The teratoma may be smooth or nodular, covered with normal or necrotic skin. Necrosis, sharp thinning of the skin, or infection of the cystic cavities occurred in 15 of our patients. The consistency of the tumor varies depending on its contents. Certain difficulties arise in the differential diagnosis between teratomas and anterior sacral meningoceles. When large in size, the latter extend downward between the sacrum and the rectum and can be mistaken for a teratoid tumor. Radiography of the sacrum and the tumor in two projections helps establish the correct diagnosis in these cases. In anterior spinal hernias, radiographs reveal Anomalies of the sacral vertebrae. In teratomas, radiographs frequently demonstrate dense inclusions, ranging from bone fragments to entire bones. Applying pressure to the tumor in cases of spinal hernia causes an increase in intracranial pressure, synchronous bulging of the fontanelle, and infant restlessness.

Fig. 112. Newborn with a giant sacrococcygeal teratoma.

Teratoid tumors may be localized in the retroperitoneal space. In such cases, particularly with hourglass-shaped tumors, bulging in the sacrococcygeal region may be minimal or entirely absent. The clinical picture in these patients is dominated by symptoms of abdominal organ compression (intestinal obstruction, urinary retention, etc.). We encountered this type of retroperitoneal tumor localization three times, and in 5 other cases, the tumor was hourglass-shaped. Clarification of the tumor's localization and size in these cases is achieved through digital rectal examination. Some patients present with additional tumor nodes in the pelvic cavity or deep within the gluteal muscles. When clinical manifestations of the tumor are ambiguous, primary diagnostic importance is attributed to abdominal radiography, barium-contrast bowel studies, and contrast studies of the Kidneys, the results of which provide insight into the tumor size, as well as the relationship and displacement of abdominal Organs (Grewe,

1969).

Treatment. Sacrococcygeal teratomas are radically removed surgically. Regarding the optimal timing for Surgical Treatment of teratomas, a consensus does not yet exist in the literature. For instance, S. D. Ternovsky, A. E. Zvyagintsev (1965), and Yu. F. Isakov (1971) recommend performing the operation at 6–8 months of age, citing the high surgical trauma of the intervention. Others (I. M. Dudnik, 1966; V. F. Gorlinov, 1967; Burl, Dillard, 1970), in the absence of contraindications on the part of the infant (severe concomitant diseases and developmental defects rendering the child nonviable), excise sacrococcygeal teratomas in the first days of life. Our experience allows us to endorse this latter viewpoint. Modern Anesthetic Management, correction of Homeostasis parameters, proper surgical technique, and other advances make it possible to successfully operate on newborns during their first days of life. Removal of the tumor prevents malignant transformation, and early restoration of anatomical relationships promotes the normalization of rectal function.

Malignant transformation is a frequent and most formidable complication of teratoid tumors, including sacrococcygeal teratomas (O. V. Blagoveshchenskaya, 1957; M. L. Lipkind, A. P. Malinin, 1962; Donnelan, Swenson, 1968). There is a distinct and notable increase in the rate of malignant tumor transformation with the patient's age. Malignancy occurred in 7% of patients under 4 months of age and in 42% of older children (Waldhausen et al., 1963). According to Hunt (1968), malignant transformation of the tumor was detected in 50–60% of children aged 4 months to 5 years. Teratoma malignancy occurred in 4 of our 24 patients. The age of these patients ranged from several hours to 1 month from birth, which confirms the possibility of intrauterine tumor malignancy and the necessity of its earliest possible removal.

Preparation of the patient for surgery follows general guidelines. The removal of a sacrococcygeal teratoma is, in most cases, associated with significant blood loss; therefore, at least 300–500 ml of freshly prepared citrated blood must be available prior to surgery.

Preoperative preparation depends on the infant's condition and lasts from several hours to several days. Special attention is paid to improving blood clotting, preventing and, if necessary, treating Pneumonia, and normalizing homeostasis parameters.

Before surgery, a thick rubber tube is inserted into the rectum, and a catheter is placed into the Urethra. Blood loss Prevention is achieved through prophylactic electrocoagulation of Blood Vessels.

Surgical technique. The child is positioned either prone with elevated legs or flat on the abdomen. An arcuate skin incision is made 2–3 cm posterior to the anal orifice. The ends of the incision reach the PROJECTION OF THE greater trochanters. An angular incision with its base facing the anal orifice may also be used. The width of the skin flaps depends on the tumor size. After dissecting the skin and subcutaneous tissue, the tumor is bluntly and sharply isolated from surrounding tissues while avoiding damage to the teratoid cysts. The most challenging step of the operation is separating the tumor from the posterior wall of the rectum, particularly the coccyx. The use of hydrodissection and a rubber tube within the intestinal lumen facilitates the separation of the tumor from the bowel wall. In the event of an intestinal injury, the defect is closed with a double-row nylon suture. If the teratoma is fused with the coccyx, the latter is excised. Resection of the coccyx tip facilitates radical tumor removal and prevents recurrence. According to Waldhausen (1963), when the coccyx was left intact, tumor recurrence occurred in 7 out of 19 patients. Similar results were reported by Gross (1951) and Pathak (1967).

In our observations, the coccyx was not removed in 9 children, three of whom subsequently returned with a tumor recurrence.

Following tumor excision and meticulous hemostasis, the anatomical relationships of the pelvic muscles and organs are restored to the maximum possible extent. Excess skin is excised. An active drainage system for wound secretion is placed at the base of the wound. The skin edges are approximated with interrupted sutures.

We place great emphasis on active aspiration of wound secretions during the first 2 days following sacrococcygeal teratoma resection. This simple Procedure promotes rapid tissue apposition, prevents fluid accumulation, and facilitates primary wound healing.

In cases of a high retroperitoneal tumor location, the presence of accessory cysts, or a dumbbell-shaped tumor, complete excision via a perineal approach is not feasible. These patients require a combined approach—specifically, a perineal approach in conjunction with a midline laparotomy.

Following surgery, the child is placed in a prone position to prevent urine and feces from contaminating the wound. Postoperative management follows standard protocols. Special attention is paid to preventing Shock during the first hours after surgery, which is achieved primarily through proper analgesia and timely blood volume replacement. Blood transfusions are continued throughout the first few hours postoperatively. Blood loss in these patients must be over-replaced by 15 — 20 mL. Failure to adhere to this rule worsens treatment outcomes. Sutures are removed on the 8 — 9th day.

Of the 24 newborns operated on in our clinic for sacrococcygeal teratomas, 3 died in the first days after surgery, while the rest were discharged in satisfactory condition. During long-term follow-up, 3 patients experienced tumor recurrence. Following reoperation, 2 children, in whom the recurrent tumors underwent malignant transformation, died; 1 child was discharged healthy and is currently developing satisfactorily.