NEONATAL SURGERY - 1976

2. SPECIAL SECTIONS

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6. Syndromes of Relative Immaturity

   The most striking clinical illustration of the theory concerning the Relative immaturity of Organs and Tissues in a newborn infant is found in syndromes that occur exclusively in neonates. These include physiological jaundice of the newborn, hemorrhagic syndrome, thermoregulation disorder syndrome, and calcinosis. Physiological jaundice is not a surgical problem and is discussed herein only in the context of the Cytology/practical/136.html">Differential Diagnosis OF congenital Anomalies of the Bile ducts. The thermoregulation disorder syndrome is described in the section covering preoperative preparation and postoperative management.

Hemorrhagic Syndrome

The predisposition to hemorrhagic manifestations in newborns is caused by A number of factors, among which vitamin K deficiency and increased permeability of the Capillary Wall play the primary role. Vitamin K is synthesized in the intestine with the participation of symbiotic microflora. During the first week of life, this flora does not yet perform this function, resulting in a decreased Blood level of vitamin K in the infant's first days of life. A lesser role is played by a deficiency in prothrombin, factor VII (proconvertin), and factor X. The reduction in these factors leads to slower blood clotting and a tendency toward bleeding. In most infants, this temporary decrease in blood clotting causes no clinical symptoms. In isolated cases, when the levels of these factors drop below a certain critical threshold, various hemorrhages occur, which are referred to in pediatric literature as manifestations of "hemorrhagic disease of the newborn" (D. Bobev, I. Ivanova, 1969; E. Ch. Novikova et al., 1971, etc.) (Fig. 25).

Fig. 25. Onset time of the hemorrhagic syndrome.

Bleeding occurs on the 3rd to 7th day of the infant's life, when the levels of blood clotting factors drop below the critical threshold.

   The most frequent and formidable manifestation of hemorrhagic disease is melena neonatorum. In melena, diapedetic bleeding occurs from the capillaries of the gastric and intestinal mucosa into the lumen of the gastrointestinal tract. The onset of the disease coincides temporally with the maximum drop in blood vitamin K levels. As a rule, infants in the first week of life are affected (most commonly on the 3rd or 4th day). Without apparent prodromal signs, the infant suddenly develops vomiting containing blood and blood-tinged stool. The blood in the stool has a crimson hue because the entire gastrointestinal mucosa is bleeding, and altered blood from the upper PARTS OF THE intestine mixes with unchanged blood from the lower parts of the bowel. In most cases, the bleeding is substantial, and the infant may die unless emergency measures are taken.

Upon admission, the infant's condition depends on the extent of blood loss. In cases of massive Hemorrhage, the infant appears pale. The body Temperature in most patients is not elevated.

The diagnosis is established based on the typical clinical picture and the time of onset. Differential diagnosis should be carried out to rule out swallowed blood syndrome. In such cases, blood in the stool results from swallowing maternal blood from cracked nipples during feeding or from bleeding in the Oral Cavity and nasopharynx. Examination of the mother's breasts and the infant's nasopharynx provides the initial data necessary for diagnosis.

Infants with melena neonatorum require urgent Treatment, which consists of replacing blood loss and stopping the hemorrhage. The administration of Vikasol (menadione) is effective (1 ml of a 0.3% solution 3 times a day intramuscularly). Simultaneously, transfusions of compatible, freshly prepared blood are prescribed (25 — 50 — 100 ml depending on the degree of blood loss). Administering Vikasol for 2 — 3 days and performing one or two blood transfusions generally succeed in halting the hemorrhage and curing the infant. Subsequently, the children develop normally, and the tendency toward bleeding disappears.

Other manifestations of hemorrhagic disease of the newborn include bleeding from the umbilical stump, Lungs, and Urogenital System, intracranial hemorrhages, and Adrenal Hemorrhage (Willi, 1957). The Etiology AND Pathogenesis of these bleedings are identical. Characteristic is the timing of clinical symptoms, which, as in melena neonatorum, coincides with the maximum decrease in the levels of vitamin K and other clotting factors.

Bleeding from the umbilical fossa is a frequent manifestation of the hemorrhagic syndrome. The bleeding begins on the 2nd to 4th day of the infant's life, often without apparent cause. Upon examination, it is usually impossible to locate a bleeding vessel. Attempts to stop the bleeding by suturing the umbilical cord or applying a pressure bandage are unsuccessful. The administration of Vikasol and blood transfusions is effective.

Bleeding from other organs is treated according to General Principles.

It is important to remember that any hemorrhage in an infant during the first week of life may be caused by a blood clotting disorder, and Pathogenetic Therapy should be prescribed when necessary.

The Clinical presentation, diagnosis, and treatment of Liver and adrenal hemorrhages are detailed in the section "Birth injuries of Abdominal Organs."

Neonatal Calcinosis

Neonatal calcinosis is a specific condition occurring exclusively in newborn infants. In calcinosis, calcium salts are deposited in the soft tissues (predominantly the subcutaneous adipose tissue) of various regions. A characteristic feature of neonatal calcinosis is the absence of any

alterations in calcium METABOLISM. A distinction is made between metabolic and tissue calcinosis. Metabolic calcinosis is accompanied by the deposition of calcium salts resulting from various hypercalcemias (hyperparathyroidism, hypervitaminosis D, Bone tumors with bone destruction, impaired renal excretion of calcium salts, etc.). In newborns, metabolic calcinosis is practically nonexistent.

Tissue calcinosis occurs in association with various tissue changes. L. G. Safina (1964) divides tissue calcinosis into dystrophic and interstitial. Dystrophic calcinosis is the end stage of prolonged inflammatory and other processes (calcification of chronic inflammation foci, tuberculous Gums, parasite cysts, hematomas). In the neonatal period, dystrophic calcinosis is encountered in cases of prolonged purulent processes in the umbilical region.

Interstitial calcinosis is the deposition of calcium salts in the Skin, subcutaneous tissue, wall of the rectum, Urinary Bladder, etc. Primary local tissue changes in these cases cannot always be demonstrated. Interstitial calcinosis in newborns can be localized or generalized. In localized forms, the foci of salt deposition are confined to small areas. In generalized forms, calcium salts are deposited in extensive areas, not only in the subcutaneous tissue but also in the walls of adjacent organs.

The etiology of neonatal calcinosis has not yet been definitively clarified. The deposition of calcium salts is facilitated by intrauterine Hypoxia of various origins, birth asphyxia, and the injection of calcium salt solutions into the umbilical vessels for resuscitation purposes.

Neonatal calcinosis is a rare condition. According to L. G. Safina, it occurs in 1 out of 3,000 to 4,000 births. The onset of the disease dates back to birth or the first days of life. Calcium salt deposits are most frequently found in the subcutaneous tissue of the buttocks, Perineum, and the umbilical region. The second most common localization of calcinosis is the tissue along the Urinary Tract—paravesical, paraurethral, and pararenal (Fig. 26).

Fig. 26. Zones of the most frequent localization of calcinosis.

The initial clinical manifestations of calcinosis are areas of induration in the subcutaneous tissue. Secondary skin changes develop rapidly. The skin over the lesions becomes dense and blue-purple. The lesions have distinct margins and protrude slightly above the unaffected skin surface. Frequently, as early as the first few days, small yellowish calcification foci are visible through the thinned skin. After 4 to 6 days, the skin over the calcifications necroses, and crumb-like calcium deposits become visible at the bottom of the resulting ulcers. When calcifications are localized in the pararectal tissue, by the 2nd to 3rd week of life, an infiltration focus appears around the anus, inflammatory signs develop rapidly, and pararectal fistulas form that are refractory to conservative treatment. With concomitant involvement of the rectal wall, tenesmus, frequent stools, and admixtures of blood and pus in the feces are possible. Involvement of the umbilical region clinically manifests as prolonged inflammation of the umbilical fossa.

The most prognostically severe form is calcinosis of the urinary tract. The initial symptom in such patients is typically pyuria. Hematuria and symptoms of urosepsis develop rapidly. When calcifications are localized in the urethral wall or the ureteral orifices, urination disorders may occur. The diagnosis of calcinosis is refined by X-ray Examination. Direct and lateral radiographs of the affected area are performed. Radiographs reveal shadows of calcium density within the thickness of the affected tissues (Fig. 27).

27. Radiograph of a newborn. Diagnosis: calcinosis.

The Location of calcifications in the rectum and urinary tract is particularly dangerous because it can be complicated by perifocal inflammation and The Development of Sepsis.

Treatment

There is no pathogenetic treatment for calcinosis. Conservative therapy is ineffective. The only radical method is the surgical removal of calcium conglomerates within healthy tissue boundaries. In cases of limited lesions, this is achieved by Curettage of the affected tissue using a sharp surgical spoon. In other instances, extensive areas of the affected adipose tissue, abdominal wall, etc., must be excised, whenever possible. In calcinosis of the urinary tract where removal of calcifications is impossible, therapy aims to prevent complications—Antibiotics and general strengthening agents are prescribed. The deposition of calcium salts in interstitial neonatal calcinosis is a single-event process. Following the removal of calcium conglomerates, recurrence of the condition typically does not occur.