Aneurysm in newborns

Aneurysms are bulges in the arterial or venous walls or ventricles of the heart due to their thinning or stretching. Due to these processes, a so-called aneurysmal bursa is formed, which can press on nearby structures. Pathology does not have an attachment to age and is able to appear, both in children and the elderly. But the aneurysm in newborns is mainly congenital in nature: such a violation is rare, but in some cases can lead to the development of unfavorable and even threatening consequences. It is detected more often accidentally, when performing ultrasound or X-ray examination.

Epidemiology

In general, aneurysms are not a very rare pathology: they are found in about 5% of the world's population. However, it is not known how many people in the world live without knowing they have an aneurysm.

Among all patients with this disease, the smallest number are children. They account for no more than 2% of the total statistical figure. At the same time, the development of complications occurs only in 3% of cases, although it almost always leads to a fatal outcome.

Any artery or vein can theoretically be affected by an aneurysm, but most often the pathology develops in large arterial trunks, as well as in the area of branching arteries.

The most common forms of this disease in newborns:

• cerebral artery aneurysm;
• aortic aneurysm;
• cardiac aneurysm.

Pathologic dilations of peripheral vessels are less frequently detected.

Causes of the neonatal aneurysms

There are a number of potential causes of aneurysms in newborns. In most cases, the pathology is congenital - for example, there is a weakness of connective tissue structures. Also, the tendency to aneurysm can be inherited by the child. Most often, the disorder is found in children with genetic lesions of connective tissue:

• Marfan syndrome;
• MASS phenotype;
• Ehlers-Danlos, Stickler syndrome;
• Lewis-Dietz, Bils syndromes (congenital contractural arachnodactyly);
• with a familial arterial aneurysm;
• familial pathological arterial tortuosity;
• with osteogenesis imperfecta;
• Alport syndrome;
• benign joint hypermobility;
• chondrodystrophies;
• with an elastic pseudoxanthoma.

In addition, the intrauterine development of the fetus plays an important role. There are many factors that can have an unfavorable effect at the stage of pregnancy, which directly affects the formation of aneurysms in newborns.

Risk factors

• Hereditary factors:
  • Close relatives have had similar vascular pathologies (not just aneurysms);
  • maternal aneurysms;
  • -previous pregnancies have resulted in the birth of children with congenital aneurysms.
• Negative effects on the fetus in the first trimester of pregnancy:
  • alcohol consumption, smoking, drug addiction;
  • infectious diseases (acute respiratory viral infections, influenza, coronavirus infection, rubella);
  • taking medications with teratogenic effect.
• Influence of radiation, chemical substances (in particular, work in harmful production).
• Chronic pathologies of the expectant mother:
  • Metabolic disorders, hormonal disorders;
  • systemic connective tissue disorders;
  • hypertension, obesity, diabetes mellitus;
  • Chronic infectious processes, including latent (asymptomatic carrier).

Pathogenesis

Depending on the mechanism of occurrence, neonatal aneurysms are conventionally divided into true, false, and delaminating aneurysms.

True aneurysms are caused by structural changes in the vascular wall, which create prerequisites for thinning and dilation of the affected segment. Detachable aneurysms in newborns are practically not encountered, as they are formed at a more mature age, and false aneurysms arise under the influence of trauma to the vascular wall, violation of its integrity.

In terms of pathomorphologic features, aneurysms are divided into local and diffuse. In diffuse lesions, there is a pronounced bulge of the vessel, cylindrical or spindle-shaped configuration. In localized lesions, there is a sac-like, navel-shaped or funnel-shaped dilation limited by peculiar orifices.

Congenital vascular disorders are the result of abnormal development of the network of arteries or veins during embryonic formation, which can be associated with gene mutations, infectious and inflammatory diseases, toxic effects (exo or endogenous) at the stage of carrying the baby. [1]

In most cases, doctors cannot explain why aneurysms occur in newborn children, more typical for people over 45 years of age. Unlike adults, such risk factors as smoking, atherosclerosis, hypertension, etc. Do not apply to children.

The congenital origin of aneurysms is considered particularly relevant. However, there are no clear references to the detection of this pathology in fetuses during autopsy or screening ultrasound.

Many specialists insist on the involvement of infectious processes and head injuries as the root causes of early formation of pathologic vascular dilations.

Symptoms of the neonatal aneurysms

Aneurysm in newborns occurs very rarely, and the present symptomatology is associated mainly with the fact that aneurysmal expansion exerts pressure on neighboring structures, complicates their work, disrupts blood supply.

In most cases, the pathology in infants proceeds without certain symptoms and is detected only after several years or even decades, more often accidentally, when performing diagnostic manipulations for other diseases or conditions. However, sometimes children voice complaints about small aching pains or tingling in the area of the pathologic formation.

The clinical picture, if any, depends largely on the localization of the affected vessel.

• Aneurysm of the heart in newborns is caused by intrauterine developmental abnormalities. Due to abnormal cardiac function, signs of heart failure occur. The probability of lethal outcome depends on the localization of the pathologically-altered area. [2], [3]
• Aneurysm of the atrial septum in a newborn is manifested by the presence of a protrusion of the thinning membrane between the two atria. In most newborns, cardiac function and blood flow are not impaired, and the pathology is detected incidentally. The risks increase if a defect is formed between the left and right atria, which creates an increased load on the right atrium. Symptomatology manifests itself only over the years: the baby is more often sick with viral infections, has less endurance compared to peers. There may be a visible bulge in the chest area (cardiac localization zone). [4], [5]
• Aneurysm of the vein of Galen in newborns is accompanied by various disorders of the cerebral circulation system, which is expressed in the intertwining of defective vessels, the formation of nodules and nodules of different configurations, local dilatation of the walls of the vein of Galen. Such lesions account for about 30% of malformations diagnosed in newborn babies and children up to 3 years old. Pathology is quite dangerous, often entails a lethal outcome. At an older age, the problem can cause intracranial hemorrhage, cerebral circulation disorders of the ischemic type. [6], [7]
• Brain aneurysm in a newborn creates significant obstacles to normal blood circulation and brain function. The problem is accompanied by motor impairment, headaches, convulsive seizures, sensory impairment. Lethality rates for this pathology are estimated at 10-30%. [8], [9]
• Aortic aneurysm in newborns in the first time practically does not make itself known, manifesting itself only in school or adolescence. The first signs of pathology: chest pain, a feeling of compression behind the sternum, difficulty breathing, dry constant cough (due to pressure on the respiratory tract). Some children "sits down" voice, there are problems with swallowing. When such an aneurysm ruptures in childhood, in almost 100% of cases the patient dies. [10], [11], [12]

Complications and consequences

The main threatening complication of neonatal aneurysm is rupture of the affected vessel. This condition is characterized by the following symptoms:

• a sharp drop in blood pressure readings;
• acute pain in the area of localization of pathology;
• sudden onset of shortness of breath;
• heart palpitations;
• skin changes (sudden pallor or blueness);
• motor and cognitive disorientation;
• loss of pain response.

If the child does not receive emergency medical attention, there is a fatal outcome.

If hemorrhage occurs in the brain tissue, it causes impulse conduction disorder, disruption of all vital systems of the body. It is important to understand that the rupture of an aneurysm is an extremely severe violation of the vascular system. But in many cases - with a favorable course of pathology and timely intervention - there is a rapid recovery of the child's body, which practically does not affect its development.

Another unfavorable consequence is thrombosis with further detachment of the clot and development of heart attack or stroke.

Diagnostics of the neonatal aneurysms

Diagnosing an aneurysm in newborns is quite difficult, since the pathology requires mandatory thorough instrumental studies. With the help of radiography, it is possible to consider the lesion of the thoracic segment of the aorta, and on a multislice radiograph it is possible to clearly trace a pulsating aneurysmal shadow that is not isolated from the aortic vessel.

In most infants, the problem is detected incidentally during an ultrasound or x-ray. Targeted ultrasound is performed with color Doppler mapping and energy Doppler.

If the newborn baby is suspected of having disorders on the part of the vascular network, then they start with objective diagnostics:

• carefully examine the newborn, assess the color of the skin, motor activity, the presence of edema;
• count heart rate and respiratory rate;
• determine the pulsation of the peripheral arteries;
• percussion assess the borders of cardiac dullness, localization of the heart and liver;
• auscultation determines the presence of heart murmurs, pulmonary rales;
• palpatorily determine the size of the liver and spleen;
• detect other congenital abnormalities.

The following tests may be ordered:

• general clinical blood work;
• blood chemistry;
• study of the acid-base state of the blood.

Instrumental diagnostics most often include:

• pulse oximetry (measurements of preductal and postductal saturation with simultaneous measurement of blood pressure);
• Radiography (with assessment of cardiac dimensions and the state of the pulmonary vascular pattern);
• electrocardiography (cardiac electrical axis statics, coronary overload, rhythm disturbances, etc.);
• aortography, Echo-CG, etc.

Differential diagnosis

Aneurysm in newborns is differentiated with such pathologies:

Interventricular septal defects, interatrial septal defects, open ductus arteriosus, pulmonary vein drainage defect, incomplete atrioventricular communication;

• pulmonary artery stenosis;
• stenosis, coarctation of the aorta;
• dextracardia, vascular localization defects, double aortic arch.

Treatment of the neonatal aneurysms

Therapeutic tactics cannot be the same, as it depends on the growth rate, size and localization of the pathologically enlarged area. When indicated, surgical correction of the vascular defect, drug therapy of circulatory insufficiency is prescribed. Eliminate and prevent the most likely complications.

As an auxiliary treatment, oxygen therapy, vitamin therapy, cardiotrophy, sanitation of infectious foci are carried out.

Mandatory hospitalization is indicated when more in-depth diagnosis is needed, decompensated conditions, development of life-threatening conditions (heart rhythm disturbances, pulmonary hypertension).

With the development of circulatory insufficiency, it is possible to prescribe drugs such as diuretics, cardiac glycosides:

• Strophanthine for babies under 2 years of age is used in a dosage of 0.01 mg/kg, and older children - 0.007 mg/kg.
• Corglycone for babies under 2 years of age is used in the amount of 0.013 mg/kg, and for older children - 0.01 mg/kg.

Digoxin for children under 2 years of age is administered in the amount of 0.06-0.07 mg/kg orally or 0.04 mg/kg intravenously, and for children over 2 years of age the saturation dose equates to 0.05-0.06 mg/kg orally or 0.03-0.04 mg/kg intravenously. The maintenance dose for children is approximately 1/5 of the saturation dose.

Contraindications to the use of cardiac glycosides:

• bradycardia;
• atrioventricular block;
• paroxysmal ventricular tachycardia;
• anuria;
• aortic stenosis and coarctation;
• Fallot's tetrad.

Other medications:

• Dopamine at 4 mcg/kg/minute for 4-48 hours;
• Dobutamine in amounts of 2-10 mcg/kg/minute.

In the picture of pulmonary heart, oxygen therapy is performed, Eufylline, calcium channel blockers (Amlodipine, Verapamil) are administered, and in case of insufficiency of any origin, angiotensin-2 antagonists or ACE inhibitors are prescribed. If indicated: diuretics (Furosemide, Hypothiazide).

Against the background of decreasing pulmonary blood flow, it is possible to use myotropic antispasmodics (Drotaverine), beta-adrenoblockers (Bisoprolol, Propranolol).

Symptomatic treatment is selected individually, if necessary, place the baby in a specialized department of the hospital.

Surgical treatment

It should be noted that surgical intervention is resorted to relatively rarely, since surgery for a newborn carries many unfavorable consequences and risks. If the aneurysm is rapidly progressing, and the size of the affected segment of the vessel is large enough, it is recommended to perform surgical intervention with the placement of an artificial implant. The operation is performed in hospital conditions, in special clinics, where there is modern equipment and qualified medical staff, where everything is available for careful monitoring of the infant's condition.

Scheduled intervention is sufficient for most newborns; rarely is it necessary to resort to emergency surgery. After surgery, the child is placed on a dispensary record, he is under the supervision of pediatricians, vascular surgeons and cardiologists for a long time.

Prevention

Among the main recommended preventive rules to prevent aneurysms in newborns, the following are particularly important:

A woman should eat a full and high-quality diet throughout the reproductive period, and especially at the stage of planning pregnancy and carrying the baby;

• If necessary, doctors should advise the future mother to take vitamin and mineral preparations (folic acid and iodine are considered the most necessary);
• alcohol consumption, smoking and drug use should be completely eliminated;
• the likely effects of teratogenic substances (nitrates, pesticides, heavy metals, certain medications) should be eliminated;
• It is necessary to pay attention to the optimization of somatic women's health, which is normalization of weight, prevention of diabetes mellitus);
• It is important to prevent intrauterine infection (if necessary, a woman should be vaccinated against the rubella pathogen).

If there is a high risk of hereditary abnormalities in the fetus, a woman should necessarily consult a geneticist.

Forecast

The prognosis of the disease is ambiguous - primarily due to the rarity of the pathology. If neonatal aneurysm is characterized by a progressive nature and there is a possibility of an unfavorable course in the future, the positive outcome is questioned by specialists. In the majority (more than half) of children with pronounced symptomatology of pathology, various complications eventually arise, including strokes, heart attacks, heart failure, ruptures of pathologic dilation. Among the rarer complications: thrombosis, heart rhythm disturbances.

In general, the quality of prognosis may also depend on the timeliness of detection, size, localization, progression of the aneurysm, and the absence or presence of compression of adjacent structures.

Since there is a lack of practical information about cases of neonatal aneurysms, decisions regarding surgical intervention should be made only on an individualized basis.