NEONATAL SURGERY - 1976

2. SPECIAL SECTIONS

8. Congenital Malformations of Individual Organs and Systems

The majority of Congenital Malformations require surgical correction within the first hours, days, or weeks of a newborn's life. Neonatal surgery is, to a significant extent, the surgery of congenital malformations.

HEAD, Face, and Neck

   Cranial Hernias (Encephaloceles)

Congenital cranial hernias represent the protrusion of intracranial contents through bony defects of the Skull. They occur with an average frequency of one in every 4,000 to 8,000 newborns (A. F. Zverev, 1967; G. A. Bairov, 1968). These malformations result from impaired Development of the cranial bones and Brain during the Cytology/cytology/16.html">Early stages of Embryogenesis. The Etiology AND Pathogenesis of cranial hernias remain not fully elucidated to this day.

Depending on the localization of the cranial defect, hernias are classified into anterior, posterior, and basilar types.

In anterior cranial hernias, the defect typically localizes at the fusion sites of the embryonic facial and cranial bone buds. The presence of a defect at the junction of the frontal and nasal bones gives rise to an anterior nasofrontal hernia. In anterior nasoorbital hernias, the defect is located in the region of the inner canthus of the eye (T. G. Mareeva, 1970).

Posterior cranial hernias are characterized by the localization of the defect either above or below the external occipital protuberance, accordingly referred to as superior and inferior posterior cranial hernias.

In basilar (the rarest) forms of hernias, the defect is localized in the region of the anterior or middle cranial fossa, with the hernia sac protruding into the Nasal cavity.

Depending on The Nature of the hernia sac contents, cranial hernias are divided into meningocele, encephalocele, and encephalocystocele. The contents of a meningocele consist solely of the Meninges and CEREBROSPINAL FLUID. The dura mater generally does not extend into the hernia sac in cases of meningocele. In an encephalocele, the hernia contents include brain tissue and meninges. An encephalocystocele—the most severe form of cranial hernias—is characterized by the presence of meninges, brain tissue, and cerebral ventricles within the hernia sac.

Clinical presentation. The manifestations of congenital cranial hernias are in most cases characteristic, and Diagnosis presents no difficulties. The infant is born with a hernial protrusion in the bridge-of-the-Nose area, the occiput, or the nasal cavity (Fig. 31). In anterior encephaloceles, the orbits are widely spaced and obliquely positioned, and the facial Skeleton bones are deformed. In basal hernias, the contents protrude into one of the nasal passages, deform the nasal septum and nasal bones, and frequently cause respiratory distress. Obstruction of the lacrimal duct leads to constant epiphora (watering of the eye).

Class="center">Fig. 31. Newborn with an anterior encephalocele.

Diagnosis is established based on the presence of the hernial protrusion. Skull radiography in two projections is mandatory. Radiographs typically reveal defects in the cranial bones. The type of hernia is clarified by puncturing the protrusion and through pneumoencephalography. In cases of meningocele, cerebrospinal fluid is found within the protrusion. Following fluid aspiration, the hernia subsides. A reduction in the size of the hernial protrusion also occurs when fluid is withdrawn via lumbar puncture. As a rule, cranial hernia protrusions are pulsating.

Differential diagnosis is performed with dermoid cysts, vascular tumors, and, less commonly, cephalhematoma.

Dermoid cysts are frequently localized near the inner canthus of THE EYE AND, when large, can be mistaken for a cranial hernia. Unlike a hernia, dermoid cysts are firmer to the Touch and do not pulsate. Radiography reveals no bone defect. Spinal puncture does not alter the size of a dermoid cyst. The greatest diagnostic challenge is differentiating hernias with a narrow base and strangulated hernia contents from the cranial cavity. In such instances, the diagnosis is often clarified only during surgery.

The diagnosis of a vascular tumor (hemangioma, lymphangioma) is established based on inspection and Palpation. Hemangiomas are typically associated with altered Skin coloration. Palpation reveals no defect in the underlying bone. In doubtful cases, radiography is indicated.

Basilar hernias are differentiated from nasal polyps. Obtaining cerebrospinal fluid via puncture is decisive for diagnosing a hernia.

Treatment of cranial hernias is surgical. For small hernias where the protrusion is covered by unaltered skin, surgery is performed at the age of 1 to 3 years. During the newborn period, infants with large hernias are operated on when the skin over the hernia sac is altered and There is a risk of necrosis or rupture. Indications for emergency surgery include rupture or Necrosis of the hernia membranes accompanied by cerebrospinal fluid leakage. In cases of concomitant Hydrocephalus and large encephalocystoceles, indications for surgery are relative.

Surgical treatment consists of excising the hernial protrusion and performing cranioplasty of the cranial bone defect. The Procedure can be performed using extra- and intracranial Methods. In the extraplanar (extracranial) method, removal of the hernia sac and bone defect repair are performed without opening the cranial cavity. The extracranial method is indicated for small hernial protrusions and minor bone defects, as well as in cases of strangulated hernias.

Surgical Technique. Patient position: supine. Anesthesia: endotracheal narcosis. The skin and subcutaneous tissue are incised using a circumscribing incision placed close to the Base of the hernial protrusion. The edges of the outer bone defect are isolated, the periosteum is incised, and the hernia stalk is freed. Then, the hernia stalk is dissected up to the level of the inner bone defect, ligated at the base with a synthetic (capron) ligature, tied off, and transected. The stump is invaginated into the cranial cavity. The bone defect is covered with surrounding Tissues, or a simultaneous bone graft is performed. Reconstruction of the nose, if necessary, is performed at an older age. Following extracranial removal of cranial hernias, cerebrospinal fluid leakage and hernia recurrence are not uncommon.

The intracranial approach involves accessing the hernia gateway from within the cranial cavity, repairing the bone defect from the inside, and subsequently excising the hernial protrusion. In newborns, intracranial repair techniques are generally not employed. The outcomes of plasty in cases of small hernias and the absence of concomitant brain damage are favorable in the majority of patients.

Robin Sequence

Underdevelopment of the Mandible (retrognathia) combined with cleft palate and Tongue displacement (glossoptosis)

was described by Robin (1929, 1934), after whom this syndrome is named. Mandibular hypoplasia in Robin sequence differs from true micrognathia in that facial proportions are restored in most cases within the first few years of life. In true micrognathia, jaw dimensions do not correct with age. The pathogenesis of Robin sequence is shown schematically in Fig. 32.

Fig. 32. Development of Robin sequence.

Anatomical relations in a 9-week embryo. The Maxilla, developing rapidly, outgrows the mandible in length (a). The top right inset shows a frontal section through the embryonic skull at the same age. The tongue lies between the palatine shelves. Arrows indicate the direction of growth of the palatine shelves and the descent of the tongue. Anatomical relations in a newborn (b). Normal proportionality between the upper and lower jaws.

In a newborn with Robin sequence (c), the anatomical relations of the 9-week embryo persist: mandibular hypoplasia, cleft hard palate, and a tongue positioned upward toward the cleft, obstructing the laryngeal inlet.

After birth, the hypoplastic mandible continues to grow forward, reaching normal dimensions by 2 ¹/₂ — 3 years of age (d). The tongue also returns to its normal position. Thus, of the factors contributing to Robin sequence, only the cleft hard palate remains.

Clinical presentation. The condition manifests in newborns and infants with respiratory distress and asphyxia. The primary role in respiratory impairment is attributed to the tongue, which is displaced upward against the cleft hard palate, blocking the laryngeal inlet. Pressing down on the tongue with a spatula temporarily restores its normal position, which immediately relieves dyspnea. Diagnosis is straightforward. The small lower jaw is striking, giving the patient's face a characteristic appearance (Fig. 33). The infant is restless and cyanotic, presenting with stridor and inspiratory dyspnea. Feeding exacerbates asphyxia. Constant Hypoxia is aggravated by secondary Pneumonia, which is a frequent cause of death in these patients.

Fig. 33. Robin sequence in a newborn, showing marked microretrognathia.

Various degrees of tongue size, palatal clefting, and jaw proportion in Robin sequence have been described. The prognosis is not always directly related to the severity of individual symptoms. With a wide palatal cleft and a small tongue, there is a genuine risk of asphyxia from glossoptosis; however, positioning the tongue correctly forward ensures normal Respiration and feeding. Conversely, with a narrow cleft and a large tongue, the risk of glossoptosis is minimal, but swallowing is impaired, making feeding a major challenge.

Treatment. Clinical observations show that after birth, the underdeveloped mandible continues to grow anteriorly, reaching normal size by 2 ¹/₂ — 3 years of age. At the same time, the tongue returns to its normal position. Therefore, the goal of treatment is to maintain adequate respiration and Nutrition During the first months of life. This is achieved through various methods. The infant is placed prone on a plastic support molded to the contour of the anterior body and mounted on a frame so that the head and lower jaw hang freely downward. This corrects glossoptosis and upper airway obstruction (Bromley, Burston, 1966) while reducing the risk of regurgitation and aspiration pneumonia. The infant's head is supported by a rest or bonnet bandage (Dennison, 1967) to allow unobstructed breathing. During feeding, the infant is placed in a lateral position, and the lower jaw is advanced manually to facilitate swallowing. Initially, feeding with plain Water is recommended, as its aspiration does not carry the risk of pneumonia. Premature and debilitated infants require gavage feeding. The infant is kept in this position for 2 to 4 months until the suck-swallow reflex recovers and respiration normalizes.

Temporary correction of glossoptosis involves placing a heavy suture through the tongue and applying traction, securing the end of the thread to the chest wall with adhesive tape. Duhamel (1957) passes two horizontal sutures through the tongue, bringing the ends out through the Cheeks to be secured over skin bolsters. Prolonged fixation (up to a month or longer) is required. The sutures frequently cut through the lingual tissue, leading to recurrent asphyxia.

In the most severe cases, tracheostomy and gastrostomy are performed, and in recent years, prolonged nasotracheal intubation has been adopted. The prognosis worsens when Robin sequence is associated with other congenital anomalies, the most dangerous being laryngotracheomalacia—namely, hypoplasia of the laryngeal and tracheal cartilages. In such cases, even tracheostomy fails to secure a patent airway, and the infant dies of asphyxia (S. Ya. Doletsky, B. V. Gavryushov, 1962).

Posterior artery of the choanae

Choanal atresia is caused by congenital membranes or partitions between the posterior nasal apertures and the nasopharynx. Unilateral anomalies are more common, occurring on the right side in 60 — 66% of cases (Pracy, 1969). Atresia may be bony, cartilaginous, or membranous (Connective Tissue). The Development of choanal atresia is shown schematically in Fig. 34.

Fig. 34. Development of choanal atresia.

During development, the embryonic nasal pits deepen and approach the Oral Cavity (a). By the 7th week of gestation, the tissue separating the nasal pits from the oral cavity becomes markedly thinned, consisting only of a bilayered epithelium—the nasobuccal membrane (b). Following rupture of the membrane (c), the nasal pits open freely into the oral cavity directly behind the maxillary arch (d). Choanal atresia in the newborn (e) results from the persistence of the embryonic nasobuccal membrane.

Clinical presentation. Bilateral choanal atresia precludes nasal breathing; because newborns are obligate nasal breathers, severe asphyxia develops immediately. Symptoms appear at birth and include inspiratory dyspnea with retractions of the chest wall, cutaneous and mucosal cyanosis, and motor restlessness.

The diagnosis is confirmed by nasal cannulation. A definitive picture of choanal atresia is obtained on lateral skull radiographs following the injection of 1 cm ³ of iodolipol into the nasal passages (Schaffer, 1960).

Unilateral atresia does not cause severe respiratory distress and is usually diagnosed later in life. Characteristic signs include the inability to breathe through one nostril and persistent nasal discharge. Upper respiratory infections are frequent in these children.

Treatment. To relieve asphyxia, it is usually sufficient to open the infant's Mouth and depress the tongue with a spatula or insert an oral airway. Alternatively, the chin can be taped to the chest with adhesive strips. For bilateral choanal atresia, surgical intervention is performed promptly upon diagnosis. The membranous septum is incised using a paracentesis needle or ophthalmic scalpel via the inferior nasal meatus under local anesthesia with 1 : 300,000 epinephrine solution to minimize bleeding. Bony atresia requires a narrow chisel to create openings in the partitions via a transnasal approach. This procedure is recommended under endotracheal anesthesia to prevent aspiration of Blood and bone chips. A major hazard is the association of choanal atresia with narrowed nasal passages caused by a low cranial base. Forced attempts at perforation in such patients can result in destruction of the anterior wall of the sphenoid sinus or even pituitary injury. Intact plastic tubing is inserted into the newly created apertures for 2 to 3 days, followed by bougienage of the nasal passages with a rubber catheter to prevent restenosis. Catheterization is repeated daily and subsequently at intervals of 2 to 4 days for a month.

Pracy (1969) considers transpalatal choanotomy to be the surgery of choice.

Surgical technique. The procedure is performed under endotracheal anesthesia with the patient in the supine position and the head extended. A U-shaped incision, anteriorly based, is used to elevate the palatal mucosa, and the palate is trephined using a burr. This provides unobstructed access to the posterior choanae. A narrow trephine is used to create openings through the bony partitions occluding the nasal passages, into which plastic tubes are placed for up to one month.

Schaffer (1960) recommends performing this procedure at 11 to 12 months of age. Prior to this, the infant is fed using a bottle with frequent pauses, and in cases of recurrent asphyxia, a tracheostomy is performed.

Cleft Lip and Palate

Cleft lip and palate are common congenital malformations resulting from the failure of fusion of the embryonic facial prominences that form the nose, upper lip, and palate. They occur in approximately 1 in 700 to 1,000 newborns (G. I. Semenchenko, 1965; I. N. Yurenev, 1970; I. I. Kasparova, 1973; Swenson, 1969).

Embryogenesis. The formation of the face, hard palate, and soft palate occurs during the 2nd month of intrauterine development from seven primary processes: the frontonasal, maxillary, nasal, and mandibular processes. The nose, intermaxillary process, and upper jaw are formed through the fusion of the paired maxillary and medial nasal processes. The latter also participate in the Formation of the palate, the main part of which consists of the palatine processes of the maxilla. The absence or delay of fusion of these embryonic processes determines the specific Morphology of the defect.

The term "cleft" is the most appropriate as it reflects not only the morphological Nature of the defect, but also its etiopathogenesis.

Cleft Lip

Cleft lip can be complete or incomplete, unilateral or bilateral. In incomplete cleft lip, the defect is confined to the soft tissues. Complete cleft lip involves a defect of the alveolar ridge and nasal passages, and in a high percentage of cases, is associated with a cleft palate. Such patients typically exhibit deformation of the nasal tip, alar cartilages, and nasal passages on the affected side (S. D. Ternovsky, 1952).

Clinical presentation. Cleft lip is accompanied by pronounced cosmetic and functional impairments. On the affected side, there is hypoplasia of the Muscles, mucous membrane, and skeletal bone framework. In complete clefts, the anatomical changes are more severe and frequently extend to the Tissues of the pharyngeal ring. The most severe impairments are observed in patients with complete bilateral cleft lip.

Incomplete forms of cleft lip generally do not cause functional impairments. However, complete cleft lip, particularly when combined with cleft palate, often causes respiratory and feeding difficulties. The continuous broad communication between the nasopharynx and cooled, insufficiently humidified air leads to catarrhal symptoms and disrupts the rate and depth of respiration, predisposing the child to pneumonia. The infant struggles to breastfeed; milk constantly enters the nasal passages, creating favorable conditions for inflammatory complications and the spread of infection to the Middle ear.

Treatment. In the absence of contraindications (birth trauma, severe prematurity, serious associated malformations, etc.), surgery is performed within the first 3 to 4 days of life by mobile teams of pediatric dentists in the maternity hospital or specialized departments. For infants with contraindications during the neonatal period, lip repair (cheiloplasty) is postponed until 3 months of age (S. Ya. Doletsky, Yu. F. Isakov, 1970).

Modern surgical techniques aim at the anatomical reconstruction of the upper lip components, correction of nasal and nasal septal deformities, and the creation of the nasal passage and oral vestibule. The procedure should involve minimal trauma to surrounding tissues to prevent subsequent deformities as the child grows. The Limberg method, modified by L. M. Obukhova et al., best satisfies these requirements; its principle is based on the transposition of opposing triangular or rectangular flaps.

Preoperative preparation is carried out according to general guidelines. Endotracheal anesthesia is the method of choice. In select cases, when an experienced surgeon is available, local anesthesia may be used.

Surgical technique. In incomplete unilateral cleft lip (Fig. 35, a), the incision on the lateral segment of the lip is made along the mucocutaneous border, extending from the convexity point of Cupid's line (point 1) to the apex of the cleft at the base of the nasal opening (point 2). The incision length is 5 — 6 mm. Next, the skin is incised from point 2 to point 3, which corresponds to the center of the orbicularis oris Muscle whorl. In newborns, this incision also does not exceed 5 — 6 mm in length. The incision is then extended to the apex of the cleft (point 4). The next step involves incising the vermilion border of the lateral lip fragment at a 45° angle from point 1 to point 5, continuing along the cleft margin to the apex (point 6). The skin and mucous membrane are dissected to mobilize the created triangular flap of the lateral fragment. On the medial half of the lip, the inflection point of Cupid's line is identified (point 7), from which a horizontal skin incision 5 — 6 mm long is made toward the PROJECTION OF THE concavity point on the midline of the face (point 8). Points 7 and 4 at the cleft apex are connected along the mucocutaneous border. If there is deformity of the nasal ala, the incision is extended into the nasal opening. The vermilion border of the medial half of the lip is incised from point 7 at a 45° angle toward Cupid's line to its midpoint (point 9), after which points 6 and 9 are connected. The skin and mucosa between the incision lines are excised. The tissues of the medial half of the lip are undermined, mobilizing the skin, mucosa, and muscle. The flaps are then transposed (point 7 is apposed to point 1). The first step is to reconstruct Cupid's line. The oral vestibular mucosa is sutured with fine catgut threads. Muscle edges and skin are best approximated using fine synthetic sutures. Excellent cosmetic results are achieved when the skin is sutured with horsehair.

Fig. 35. Cheiloplasty for Various Forms of cleft lip.

In complete unilateral cleft lip (Fig. 35, b), flap creation is performed According to the technique described above. From the upper edge of the base of the lateral triangular flap, an incision is made along the border of the skin and vermilion border up to the base of the nose, terminating in a semi-oval on the inner surface of the nasal ala. The nasal ala is mobilized. The nasal passage is formed using a flap turned toward the nasal septum and sutured to the mucosa of the medial lip fragment. A rectangular flap with a base at the transitional fold of the lateral fragment is harvested from the vermilion border and mucosa, rotated by 180°, and sutured to the mucosa of the alveolar ridge, thereby creating a mucosal lining at the site of the cleft. To advance the buccal mucosa onto the upper lip, the Limberg "hockey stick" incision is used. Lip reconstruction begins by placing sutures on the mucosa and vermilion border, and lastly on the skin.

In complete bilateral cleft lip, repair is performed using the L. E. Frolova modification (Fig. 35, c). The First stage involves making a "hockey stick" incision in the oral cavity. The mucous membrane is mobilized. A trapezoidal incision is made along the vermilion border of the median lip segment, and the tissues are undermined. Incisions on the lateral lip fragments are made similarly to those for complete unilateral cleft repair. The deformed nasal alae are corrected, and the nasal passages, oral vestibule, and Cupid's line are reconstructed. To ensure unobstructed breathing in the postoperative period, rubber tubes are inserted into the nasal passages for 2 — 3 days. In cases of extensive bilateral cleft Lips, the surgery is performed in two stages.

During the first 2 — 3 days of the postoperative period, administering Antibiotics to the infant is recommended. To detect any potential hyperthermia in a timely manner, body Temperature is measured every 3 hours. Feeding is carried out using expressed breast milk delivered via a bottle with an adequately sized nipple hole or with a spoon. Sutures are removed on days 7 — 8. On the 2nd to 3rd day after suture removal, the infant may be put to the mother's breast.

Cleft Palate

Cleft palate can be complete or incomplete. In complete clefts, the fissure involves all PARTS OF THE palate, extends onto the alveolar process of the maxilla, and is combined with a cleft lip. Both unilateral and bilateral clefts of the palate can occur.

In incomplete cleft palate, depending on the extent of the fissure, a distinction is made between clefts of the soft palate, the soft and partially hard palate, the entire soft and hard palate, and occult (submucous) cleft palate, characterized by a short soft palate and a bony cleft hidden beneath the intact mucous membrane. Cleft palate, especially complete clefting, is typically associated with impaired skeletal and soft tissue development of the maxilla.

Clinical presentation. The diagnosis of cleft palate is established upon inspection of the oral cavity. In newborns with cleft palate, feeding and respiratory disturbances come to the foreground. The palatal cleft disrupts the suckling mechanism; milk frequently enters the Trachea or regurgitates through the nasal passages. This creates favorable conditions for inflammatory processes in the nasopharynx, aspiration pneumonia, and middle ear infections. The primary objective for the maternity hospital physician is to establish effective infant feeding. With small clefts limited to the soft palate, the infant often adapts and nurses well. If this is not possible, attempts are made to feed the infant using a bottle. Infants with complete cleft palate are fed with a spoon.

Treatment. Upon discharge from the maternity hospital, the infant is referred to a surgeon and an orthodontist, who determine the necessity and timing for fabricating a floating obturator, prepare the child for surgery, and establish the schedule for surgical intervention. Radical correction of the malformation is possible only through surgery. Depending on the severity of the defect, it is performed between 2 and 4 years of age.

Macrostomia

Macrostomia is a rare congenital malformation characterized by a soft tissue defect extending from the angle of the mouth toward the external acoustic meatus. Macrostomia can be unilateral or bilateral. The half of the face on the affected side is frequently hypoplastic. Treatment is surgical and is performed after one year of age. In extensive defects, the newborn is unable to nurse, necessitating a transition to spoon-feeding or tube-feeding.

Macroglossia

This term refers to enlargement of the tongue. Macroglossia can be true, i.e., caused by Hypertrophy of the tongue muscles (often associated with Down syndrome), or it may be associated with the presence of a tumor, such as an angioma or lymphangioma. In true macroglossia, the tongue is uniformly enlarged and does not fit in the mouth. The tongue musculature has a normal appearance. When a tumor is present, the shape and size of the tongue depend on the tumor's Location and dimensions. Treatment is surgical. In the neonatal period, indications for surgery include concomitant swallowing or breathing difficulties. For true macroglossia, a wedge-shaped excision of the tongue is performed. If a tumor is present, it is excised.

Ankyloglossia (Tongue-Tie)

This defect involves a fold of mucous membrane extending from the tip of the ventral surface of the tongue to the floor of the Oral Cavity and the alveolar process of the mandible. A short frenulum restricts tongue mobility, leading to poor nursing in infants. Treatment is surgical. Frenotomy in newborns is indicated when there is marked tethering of the tongue and impaired nursing. A thin frenulum is sectioned without subsequent suturing. In the case of a thick, muscular frenulum, 1 or 2 catgut sutures may be required after division.

Coloboma

Coloboma refers to an oblique facial cleft—a developmental defect of the soft tissues of the cheek, lower eyelid, and upper lip. A coloboma may be total or partial, unilateral or bilateral. Treatment is surgical, typically performed at 1 to 2 years of age. In newborns, a coloboma makes nursing difficult, necessitating feeding by spoon or via a nasogastric tube.

Congenital Stridor

The term "congenital stridor" encompasses a group of conditions characterized by whistling, noisy breathing that begins at birth or shortly thereafter. Stridulous breathing occurs due to narrowing or deformation of the glottis, Larynx, or trachea, the presence of an obstruction in their lumen, or abnormal functioning of the vocal cords. Congenital stridor may be caused by local pathology or Central Nervous system disorders.

Laryngomalacia. This group includes cases of underdevelopment of the cartilaginous structures of the larynx and excessive laxity of the laryngeal tissues, which collapse during inspiration.¹ A variant of this pathology is a floppy, pliable epiglottis that occludes the glottis during inspiration. Changes may also involve the arytenoid cartilages, which sag inward upon inspiration and narrow the laryngeal lumen.

¹ This pathology is also known by other names: congenital laryngeal weakness, inspiratory laryngeal collapse, simple congenital laryngeal stridor, etc.

Embryogenesis. The exact cause of abnormal development of the laryngeal cartilages remains unknown. Given that these cartilaginous structures acquire normal Structure with age, one can speak of their transient immaturity in early infancy (S. Ya. Doletsky, 1968). Individual cases have been associated with micrognathia and even Pectus excavatum. In all likelihood, these observations reflect a systemic underdevelopment of cartilaginous tissue in the body.

Clinical presentation. The initial signs of stridor are typically detected immediately after birth. Breathing is dry and wheezing. The noise usually occurs during inspiration, but in severe cases, it may also accompany expiration. Stridulous phenomena increase with heightened motor activity in the infant and may disappear at rest. When agitated, breathing is accompanied by retractions of the suprasternal notch, intercostal spaces, and Sternum. Stridor worsens when the infant lies on their back and diminishes in the prone position. It should be noted that this unusual breathing pattern distresses parents far more than it inconveniences the child. Phonation is unaffected. The voice is loud and clear. Skin coloration remains normal. Appetite is not reduced, and the infant gains weight well.

Treatment. Stridor generally resolves spontaneously between 6 months and 1 year of age, though in some cases it persists into older childhood. Schaffer (1960) described two children in whom stridulous breathing when agitated persisted up to 6 years of age in one and up to 8 years in the other. These children were healthy and not prone to respiratory infections. This form of stridor requires no specific treatment. Children should be evaluated to rule out organic pathology. If none is found, the infant is monitored at home. Parents are reassured that such phenomena are entirely harmless and in most cases resolve with age. If the infant stops gaining weight, appears lethargic and ill, develops cyanosis, or the voice begins to lose strength and change timbre, hospitalization is necessary to determine the cause of the stridor and, if possible, correct it.

Congenital Laryngeal Synechiae (Webs). This pathology involves the presence of adhesions or webs, typically localized at the level of the vocal cords and occasionally located in the supra- or subglottic region. In some cases, such webs significantly obstruct the glottic lumen, causing respiratory impairment.

Embryogenesis. The larynx develops from the caudal part of the Pharynx at the 5th to 6th week of embryonic development (Patten, 1959). At the beginning of the 7th week of embryogenesis, parallel to the development of the laryngeal cartilages, active proliferation of the laryngeal epithelium occurs, accompanied by the temporary obliteration of its lumen. Several weeks later, the walls of the laryngeal lumen begin to grow more rapidly, and the airway is soon re-established. Isolated epithelial remnants may persist as adhesions or webs into the postnatal period. Complete laryngeal atresia, where the glottis is sealed by a thick fibrous plate resembling Cartilage in consistency, is extremely rare.

Clinical presentation. Symptoms manifest at birth. Since webs are located in the glottic area in the majority of cases, phonation is usually affected. The infant's voice is weak and hoarse, or sometimes entirely absent. The severity of stridor and respiratory distress varies depending on the degree of airway obstruction. A definitive diagnosis is established following direct laryngoscopy.

Treatment. The Need for reconstructive extralaryngeal surgery arises extremely rarely. In most patients, thin webs can be divided during laryngoscopy. Thicker webs require repeated bougienage. Recently, cryotherapy techniques have been successfully employed. In severe cases, tracheostomy and prolonged laryngeal cannulation are necessary to reform the laryngeal lumen.

Maternity ward physicians and delivery personnel should keep in mind the possibility of complete closure of the laryngeal lumen. In such cases, the newborn's life can be saved only by emergency tracheostomy or perforation of the membrane under laryngoscopic guidance.

Congenital Subglottic Stenosis. Most commonly, the stenosis is located 2 to 3 mm below the glottis. Occasionally, the cricoid cartilage is involved, appearing deformed and narrowing the airway lumen.

Clinical presentation. Significant stenosis presents with stridulous breathing accompanied by varying degrees of hypoxia. With moderate stenosis, stridor occurs only when the narrowing is exacerbated by submucosal inflammatory edema triggered by a respiratory infection. Such children suffer from frequent episodes of "false croup," often complicated by laryngotracheobronchitis. Unlike true croup, the voice is unaffected in this condition because the narrowing is located below the glottis. The diagnosis is established via direct laryngoscopy or lateral radiography of the larynx.

Treatment. Laryngeal growth with age improves respiratory conditions, but in cases of pronounced stenosis, indications for tracheostomy and surgical dilation of the stenosis may arise. Tracheostomy is sometimes resorted to during respiratory infections when conservative measures for subglottic laryngitis (steam inhalation, soda and ephedrine aerosols, mucosal decongestants, hydrocortisone, etc.) fail to improve breathing.

Laryngeal Cysts and Neoplasms. Complete closure of the laryngeal lumen by a congenital cyst can result in the infant's death from asphyxia immediately after birth. Incomplete obstruction leads to the symptoms of stridulous breathing. Cysts may originate from the Glands of the laryngeal mucosa and are localized on

the laryngeal walls or in the region of the vocal cords. In some cases, cysts form due to prolapse of the mucous membrane of the laryngeal ventricle (laryngeal saccule) resulting from underdevelopment of the connective tissue framework of its walls.

Clinical presentation. Cyst size ranges from a few millimeters in diameter to the size of a large cherry. They are occasionally multilocular. Their walls consist of epithelialized connective tissue. The content is a gelatinous, transparent fluid. In rare cases, tumor-like formations of the Upper Respiratory Tract can cause asphyxia or stridulous breathing in newborns (A. I. Kuselman, V. P. Gorbachev, 1972). Laryngeal neoplasms described include fibromas and vascular tumors—hemangiomas—located on the vocal cords, the lingual surface of the epiglottis, the arytenoid cartilages, the aryepiglottic folds, and within the subglottic space.

Diagnosis of laryngeal cysts and neoplasms is performed during direct laryngoscopy. Radiographic examination, particularly tomography, can provide substantial assistance. When performing endoscopic examinations in newborns with laryngeal narrowing, one must exercise extreme caution and be mindful of the risk of subglottic edema, which easily develops even after mild mucosal trauma.

Treatment. Laryngeal cysts and neoplasms can be removed with forceps during direct laryngoscopy or via electrocoagulation. For inoperable hemangiomas, cryotherapy is recommended, as it leaves more delicate scars that cause minimal deformation of the larynx.

Neurogenic stridor. This condition may be caused by various central nervous system regulatory disorders. It most commonly manifests in newborns who have suffered central nervous system birth trauma and typically resolves spontaneously within the first few months of life. One variant of neurogenic stridor is vocal cord paralysis resulting from recurrent laryngeal nerve injury during delivery. Diagnosis is performed using direct laryngoscopy. No special treatment is required.

Rare causes of stridor may include tracheomalacia and tracheal stenosis resulting from structural Malformations of the tracheal wall, as well as tracheal compression caused by tumors and cysts of the neck and Mediastinum, an enlarged Thymus, congenital goiter, and aberrant Blood Vessels. Tracheal compression most frequently occurs in congenital aortic anomalies, specifically a right-sided aortic arch or a double aortic arch. Rarer causes of tracheal compression may include an aberrant right Subclavian Artery originating from the left branch or descending aortic arch, as well as an anomalous brachiocephalic trunk and common carotid artery.

Tracheomalacia as a cause of stridor can be confirmed via mediastinoscopy, which has recently found application in newborns (W. Tischer, G. Schwock, 1974). According to the authors, mediastinoscopy performed in these cases also serves a therapeutic purpose, as the scar tissue formation developing in the paratracheal tissue following the procedure prevents future tracheal flattening.

Cysts and sinuses of the neck

Cysts and sinuses of the neck during the neonatal period are primarily of diagnostic interest. A distinction is made between median and lateral cervical cysts and sinuses.

Median cysts and sinuses of the neck develop due to disruptions in the formation of The Thyroid Gland, the median rudiment of which descends from the anterior wall of the oropharyngeal cavity down the neck to its final anatomical location. Along this descent pathway, an embryonic tract persists (ductus thyreoglossus), which normally obliterates by the end of the 8th week of embryonic development. If this involution of the ductus thyreoglossus is disrupted by birth, it may remain fully or partially patent. Depending on this, cysts or sinuses are formed.

Lateral cysts and sinuses of the neck, according to the majority of authors, result from abnormal embryological development of the branchial or pharyngeal apparatus (Snyder, 1969).

In newborns, only sinuses manifest; cysts are typically diagnosed in children over 1 year of age. Cervical sinuses appear as funnel-shaped depressions in the skin. In the case of a median sinus, it is sometimes possible to palpate a fibrous cord extending toward the Hyoid bone. Serous or serosanguineous fluid continuously discharges from the sinus. Periodic inflammation of the sinus may occur. Treatment is surgical, and the procedure is performed in children older than 3 years.

Thorax and thoracic Organs

Congenital malformations of the chest wall and thoracic organs are not uncommon in children, though they are not always diagnosed during the neonatal period. Late diagnosis and delayed correction lead to severe functional impairments and childhood disability.

Sternum malformations

The development of the sternum during embryogenesis and the main variants of its formation defects are illustrated in Fig. 36.

Fig. 36. Development of the sternum and major variants of its malformations.

The formation of the sternum begins with the appearance of two cord-like accumulations of mesenchymal Cells (a). Once these cords acquire a precartilaginous structure, they begin to converge toward the midline. Fusion starts at the cranial end (b). By the 9th week (c), the sternum fuses along the midline and acquires a cartilaginous structure (d).

Depending on the stage at which embryonic development is arrested, various malformations are formed: complete sternal cleft (e), upper sternal cleft (f), and lower sternal cleft (g). Lower sternal clefts are frequently accompanied by associated anomalies — a midline paraumbilical defect of the anterior abdominal wall, a defect in the anterior Diaphragm, and a defect in the diaphragmatic Pericardium (h).

Complete sternal cleft — a rare malformation caused by the failure of the sternal bands to fuse during the 9th to 10th weeks of embryonic development. As a result, the child retains a cleft along the entire length of the sternum. The Heart and great vessels are covered only by soft tissues.

Surgical treatment is indicated and performed during the 2nd to 3rd weeks of life. The sternal bands are brought together and sutured to one another. Prior to surgery, gentle traction is applied to the infant's shoulders, which brings the halves of the sternum closer together and facilitates the subsequent surgical intervention (Belling, 1970).

Upper sternal clefts are more commonly observed in children. Due to the failure of the upper sternum to fuse, the heart and great vessels appear to be located in the neck when the child strains, cries, or coughs. Consequently, this anomaly is sometimes described as cervicothoracic ectopia cordis (U. Tsuplov, G. Kislitsin, 1971).

Surgical treatment is indicated. The operation is performed during the neonatal period, as the technical difficulties and trauma associated with the procedure increase as the child grows (Sabiston, 1958). The defect is most frequently closed using cartilage grafts by suturing the sternal bands following chondrotomy of the 1st, 2nd, and 3rd Ribs. The cartilages are incised obliquely (Fig. 37).

Fig. 37. Surgery for an upper sternal cleft.

a — freshening of the defect margins; b — division of the cartilages; c — approximation and suturing of the defect edges.

Lower sternal clefts vary in extent. Associated malformations are frequent, including a midline supraumbilical defect of the anterior abdominal wall, a defect of the anterior diaphragm, a defect of the diaphragmatic pericardium, and various intracardiac defects. Malformations of other organs may also be present.

Repair of the anterior chest and abdominal wall defect is performed during the neonatal period. The surgical procedure involves approximating the sternal cleft and suturing the sternal halves together. Congenital heart defects are operated on at a later stage.

Pectus excavatum is a congenital deformity of the sternum and adjacent ribs, forming a funnel-shaped depression below the manubrium sterni. Most authors attribute the etiology of this anomaly to congenital hypoplasia of the costal cartilages, maldevelopment of the lower section of the

sternum during the intrauterine period, as well as shortening of the sternodiaphragmatic ligament.

Due to the sternal depression, the thoracic cavity volume in the infant decreases, which affects the function of the intrathoracic organs. In newborns with pectus excavatum, clinical manifestations of functional impairment are usually absent. Based on the depth of the funnel, G. A. Bairov determines the degree of deformity using the formulas:

(where a is the sagittal diameter, and b is the frontal diameter at the level of the deformity).

External signs of the anomaly, highly characteristic in older children, are less pronounced in newborns. In a young infant, attention is drawn to a flattened chest wall, flared costal margins, and a funnel-shaped sternal depression of varying magnitude. As a result of tracheal and esophageal compression or displacement, regurgitation and vomiting may occur. Radiographic examination in newborns reveals certain signs of intrathoracic organ compression: leftward Displacement of the cardiac silhouette and mediastinum, decreased aeration of the lower lung fields, and so forth.

Treatment of pectus excavatum during the neonatal period is aimed at preventing the progression of the depression and functional disorders. To this end, infants undergo Therapeutic Exercises designed to expand the thoracic cavity and massage of the Respiratory Muscles.

Indications for surgical intervention are established after 2 to 3 years of age (Uebermuth, 1957; Geisbe, 1969).

Pectus carinatum results from congenital hypoplasia of the costal cartilages and sternum. In this anomaly, the lower section of the sternum, along with the costal cartilages, protrudes forward in a keel-like fashion, accompanied by depressions in the lateral aspects of the chest wall on both sides of the sternum. Consequently, the volume of the thoracic cavity decreases, and the organs within it are compressed. The deformity tends to progress as the child grows.

The diagnosis in a newborn is established based on the identification of chest wall deformity, as well as signs of cardiac and pulmonary compression or displacement.

Treatment during the neonatal period is aimed at preventing the progression of the deformity: for this purpose, the infant is prescribed therapeutic physical training and chest wall massage.

Rib Malformations

Congenital absence of ribs is typically associated with the absence or hypoplasia of the chest wall muscles, as well as with other malformations and anomalies of The Musculoskeletal System. Most frequently, the uppermost or lowermost ribs are absent, which has little impact on pulmonary function. When the defect is localized in the middle sections of the thoracic cavity, lung function may be impaired. The rib defect extends from the sternum to the posterior axillary line and can be either bilateral or unilateral. The absence of the II, III, IV, and V ribs is marked by partial or complete hypoplasia of the pectoralis major muscle. This is frequently accompanied by ipsilateral vertebral hypoplasia and kyphoscoliosis. Large defects in the middle sections of the chest wall may lead to pulmonary hernia formation, accompanied by paradoxical respiration and mediastinal flutter. In some children, respiratory disorders resolve with age.

Treatment is directed at replacing the rib defect and eliminating respiratory complications. In neonates, this is achieved by applying pads or binders to close the defect. In cases where insufficiency progresses, surgical reconstruction of the defect using homo- or autologous bone is indicated.