Ventilatory failure (pediatrics)

Pathophysiology
Respiratory failure is classified based on the pathophysiological mechanisms that lead to hypoxemia a/nebo hypercapnia. In a simplified view, these are disorders of ventilation, distribution, diffusion and perfusion. The following abnormalities lead to hypoxemia and/or hypercapnia: Searchtool right.svg See Ventilatory failure (pathophysiology) for more detailed information.
 * V/Q nepoměr (ventilation-perfusion ratio)
 * intrapulmonary PL shunts (intrapulmonary shunt)
 * hypoventilation
 * abnormal gas exchange at the alveolocapillary membrane
 * reduced concentration of inspired O 2
 * increased venous blood desaturation with cardiac dysfunction plus one or more of the above 5 factors.

Definitions and terms
Arterial hypoxemia can be caused by a low O2 concentration in the inspired mixture, hypoventilation, oxygen diffusion disorder, ventilation-perfusion imbalance, shunt, desaturation of mixed venous blood. The causes of arterial hypoxemia are basically the same as those of cyanosis.

*does not apply to cyanotic heart defects **does not apply in the presence of chronic lung disease

CNS lesions

 * pharmacy → narcotics, barbiturates, sedatives, anesthetics, poisons
 * neuroinfection→ meningitis encephalitis, abscess
 * hypercapnia
 * alkalosis
 * tumors
 * trauma
 * intracranial hypertension
 * central alveolar hypoventilation
 * central sleep apnea

Neuromuscular lesions

 * pharmacy → curariform drugs, aminoglycosides
 * tumors
 * tetanus
 * trauma
 * myasthenia gravis
 * multiple sclerosis
 * muscular dystrophy
 * Guillain-Barré syndrome
 * motor neuron disease
 * bilateral lesions of the phrenic nerve

Upper respiratory tract lesions
infection → retropharyngeal abscess, papular tonsillitis, epiglottitis, laryngotracheitis, diphtheria
 * tonsillar adenoid hyperplasia
 * VVV
 * trauma → burns, smoke inhalation, foreign body
 * polyps
 * tumors
 * paralysis of the vocal cords
 * acquired laryngomalacia
 * acquired tracheomalacia
 * swelling of the mucous membrane -> Quincke's angioneurotic edema, hereditary angioneurotic edema
 * obstructive sleep apnea

Chest wall lesions

 * severe kyphoscoliosis
 * severe spondylitis
 * trauma → burns with keloids
 * expansion of the interpleural space → PNO, hemothorax, fluidothorax, chylothorax
 * scleroderma

Lesions of the lower respiratory tract

 * infection → bronchiolitis,bronchopneumonia, abscess, bronchiectasis
 * VVV
 * trauma → lung contusion
 * distress syndromes → RDS from immaturity, ALI/ARDS
 * bronchial asthma
 * interstitial lung disease
 * atelectasis
 * cystic fibrosis
 * pulmonary embolization
 * Heart Failure

Other

 * hyperglycemia
 * ion imbalance → hyponatremia, hypocalcemia, hypomagnesemia, hypophosphatemia
 * myxedema
 * Pickwick syndrome

Anamnesis

 * Factors that may increase the risk of respiratory failure: young age, prematurity, immunodeficiency, chronic lung or heart disease (cystic fibrosis, bronchopulmonary dysplasia, uncorrected VCC), neuromuscular disease spinal muscular atrophy (pediatrics) = M. Werdnig–Hoffmann, myasthenia gravis, Guillain–Barré syndrome), severe sepsis sepsis (can lead to respiratory failure in the context of pulmonary edema, ARDS ARDS or with extremely increased O2 consumption duringtachypnea.
 * The indication of the duration of the difficulties is important. The course of RSV infection may worsen after 3-5 days of a relatively mild course of respiratory infection. If the patient has pain, we must think of pleurisy or aspiration of a foreign body.
 * With symptoms of neuromuscular weakness or paralysis, we must rule out the following diseases: myasthenia gravis (bulbar dysfunction), Guillain–Barré syndrome (distal paresis and hyporeflexia), apnea due to cervical spinal cord injury.
 * A patient arriving with respiratory insufficiency could also aspirate due to convulsions.
 * In patients with chronic hypercapnia, we find headaches at night or early in the morning after waking up.

Physical examination
The frequency of respiratory failure in young children is higher than in older children and adults for a number of reasons.
 * The position the patient takes spontaneously. (The smallest children cannot use the compensatory position, as they are often carried by their parents in their arms, i.e. in a position that does not respect their compensatory requirements).
 * Patients with pneumonia take the so-called. splinting position of the chest, i.e. they try to keep the chest stretched and developed as much as possible.
 * With severe bronchial obstruction, we see orthopnea in children (an image of objective shortness of breath associated with the involvement of the axillary muscles and a relief position, usually sitting with the upper limbs retracted). Orthopnea is otherwise rare in childhood.
 * In case of severe obstruction of the upper respiratory tract, children take the so-called. sniffin position ("sniffing" position).
 * Assessment of chest shape:
 * The increased anteroposterior dimension together with the bulging of the supraclavicular pits is referred to as the volume of the pulmonum auctum and is found in severe asthma or cystic fibrosis. Scoliosis and pectus excavatum represent a significant reduction in the compliance of the chest wall and restriction of the lung parenchyma.
 * Chest asymmetry can also be found in pneumothorax, paresis of the diaphragm, alar pneumonia, in atelectasis of the entire lung wing, in large unilateral fluidothorax.
 * Painless expansion of the connective tissue of the distal joints of the phalanges = "club fingers " is a non-specific finding that indicates chronic hypoxia (VVV heart with PL shunt, cystic fibrosis).
 * Under pathological circumstances, we can find intensified breathing(pneumonia, increased work of breathing, acute bronchitis) or weakened breathing (effusion, atelectasis, pneumothorax). The so-called tube breathing it is characterized by exhalation noisier than inhalation, reminiscent of the sound "ch". Physiologically, it is heard above the trachea, in pathological conditions it is found above the infiltrated lung tissue (with "complete" obturation of the alveoli). Secondary breath murmurs include moist phenomena - crackles and crepitus - they are audible during inspiration and originate in the alveoli. Crackles are also moist phenomena audible during inspiration and early expiration, originating in the bronchi. Wheezing and wheezing are audible during expiration, they also originate in the bronchi (the cause is inflammatory edema of the mucous membrane or spasm of the smooth muscle of the bronchi).

Specifics of breathing in children

 * The airways are narrower, and even a slight swelling in an inflammatory condition can lead to critical obstruction.
 * Newborns and infants up to 6 months of age breathe mainly through the nose due to the close proximity of the epiglottis and nasopharynx. Nasal congestion at this age can lead to significant respiratory distress.
 * Small children have a large tongue that fills their small oropharynx. Their larynx is located higher, i.e. opposite the C3−C4 vertebrae (in older children and adults, the larynx is opposite C6–C7).
 * The epiglottis is long, slender and U-shaped
 * The narrowest place in children < 8 years is the subglottic space (ring cartilage area), in children > 8 years then the glottis
 * The trachea has a third diameter compared to adults.
 * Infants and young children have significantly fewer alveoli than children > 8 years of age. The number of alveoli increases from 20 million to 300 million around the age of 8. Therefore, small children have a smaller area for gas exchange.
 * There are less developed elastic fibers in the alveoli. The alveoli therefore have a greater tendency to collapse, resulting in a ventilation-perfusion imbalance.
 * Small children are characterized by high compliance of the chest wall and, on the contrary, low compliance of the lungs . The intercostal muscles are immature, they show uncoordinated movements during REM sleep, the ribs are placed horizontally, the diaphragm works "more", but less efficiently.
 * Children < 6 years of age have a value of the so-called closing capacity (the volume of air in the lungs that prevents the collapse of the small airways) higher than the FRC. This fact explains the predisposition of young children to the formation of atelectasis.
 * Collateral ventilation is not fully developed. Therefore, atelectasis is far more common in children than in adults. During childhood, anatomical channels are formed that allow collateral ventilation of the alveoli. These collaterals exist between alveoli (pores of Kohn), bronchioles and alveoli (Lambert's ducts), additional bronchioles represent additional collaterals. Collateral ventilation enables gas exchange even with distal airway obstruction.
 * The respiratory center in the smallest children is immature. This poses a risk for breathing irregularities and apnea. Reflexes inhibiting respiration predominate, especially the Hering-Breuer reflex, which is a response to lung expansion. Risk factors are sleep, cold, medication, various metabolic deviations.

Changes in the breathing pattern in relation to the patient's age

 * Toddlers and older children achieve the necessary minute ventilation with '''restrictive lung disease by increasing the respiratory rate at a reduced tidal volume.
 * Conversely, with obstruction the necessary gas exchange is achieved by deepening the respiratory volume at a reduced respiratory rate
 * Newborns and infants have an almost horizontal position of the ribs, i.e. the chest is already physiologically in the inspiratory position. Therefore, when the minute ventilation needs to be increased, the breathing frequency is practically always increased without an adequate increase in the respiratory volume. Thus, the universal response of children of this age group to respiratory distress is tachypnea. This pathophysiological note must be taken into account when analgosedation is indicated. Sedation of the child limits compensatory tachypnea and may thus induce ventilatory failure in previously compensated respiratory distress.

Respiratory Distress Clinic
• Cheyne-Stokes breathing : it has an ascending (crescendo) phase, when the frequency and depth of breathing increases, and a descending phase (decrescendo), which ends with an apneic pause (apnea is not a condition, however). The cause is a delayed reaction of the respiratory centers to changes in blood gases. Cerebral, thalamic, or hypothalamic control of breathing is impaired, but brainstem control is still intact. This occurs when there is insufficient perfusion of the CNS or when respiration is controlled by a lack of oxygen. The Cheyne-Stokes respiratory pattern is found in CNS injury, intoxication (depression of the respiratory center) uremia, immaturity, heart failure, increased intracranial pressure.
 * In hypoxemia, we expect respiratory stimulation, i.e. tachypnea and/or dyspnea. The exception is premature babies, who on the contrary react with hypoventilation and apnea. Reduced respiratory effort in a child with hypoxemia most likely indicates CNS depression, a neuromuscular disorder, but the possibility of exhausting respiratory effort in a patient with prolonged respiratory distress must also be taken into account.
 * Respiratory tachypnea is usually caused by the presence of fluid in the alveoli or interstitium.
 * Primary tachypnea in a child with minimal chest retraction and no secondary auscultation phenomena most likely indicates a non-respiratory cause (CNS pathology including intoxications, sepsis, respiratory compensation MAC = Kussmaul breathing).
 * In these cases, we often find reduced values ​​of glycemia, calcium and hypocapnia. On the contrary, we can demonstrate hypercapnia in heart defects with significant pulmonary congestion.
 * Respiratory distress in heart failure can only be associated with tachypnea without chest retraction. In advanced pulmonary edema, however, we find rales (intraalveolar edema), wheezing (bronchial wall edema), retraction of the soft parts of the chest.
 * Involvement of auxiliary respiratory muscles = retraction, indicative of reduced lung compliance or airway obstruction . Likewise, retraction/falling in of the jugular (in eupnoea, the jugular fossa does not retract), retraction of the supra or infraclavicular fossae, intercostal, epigastrium, attachment of the diaphragm and alar flexure = expansion of the nostrils leads to a reduction in the resistance to the passage of air and thereby reduces the work of breathing.
 * A special sign of respiratory distress is grunting Retraction with grunting means reduced lung compliance and loss of FRC. Grunting is caused by the glottis clenching mechanism. It is an attempt to increase autoPEEP and thereby increase functional residual capacity. A restrictive disorder is characterized by an accelerated rate and reduced tidal volume.
 * Retraction with inspiratory stridoris accompanied by obstruction of the extrathoracic airways, mixed stridor means obstruction of the intra + extrathoracic airways (larynx, trachea), wheezing with the auscultatory finding of squeaks/gurgles is accompanied by obstruction of the lower, i.e. intrathoracic airways. * Mild obstruction of the airways will be manifested by a reduced respiratory rate and, conversely, an increased respiratory volume. Severe airway obstruction is characterized by increased respiratory rate, chest retraction, involvement of accessory muscles, anxiety, cyanosis. It should always be remembered that the resolution of severe upper airway obstruction may be followed by the development of pulmonary edema.
 * On the other hand, it should be noted that compensatory expiratory effort in a dyspneic patient may be counterproductive in extreme cases. Patient anxiety increases oxygen consumption, strenuous respiratory effort can cause dynamic airway obstruction. If it lasts for a long time, respiratory muscle fatigue develops, hypoventilation occurs with a rise in pCO2. Forced inspiration will significantly increase intratracheal negative pressure, which can worsen upper airway obstruction, while positive intrapleural pressure during forced expiration can lead to intrathoracic airway collapse. When the closing capacity is exceeded, smaller or larger lung regions collapse.
 * A critically serious form of breathing is gasping which is characterized by a low and irregular respiratory rate, variable respiratory volume, and respiratory pauses. It occurs in the terminal phase of hypoxia, sepsis or shock from any cause, before cardiopulmonary arrest. This breathing pattern is predictive for the immediate initiation of cardiopulmonary resuscitation.
 * The respiratory compensation of severe MAC (diabetes mellitus, intoxication) is pronounced hyperpnea with extremely increased minute ventilation, the so-called Kussmaul breathing. Mistaking it for dyspnea in pneumonia or bronchitis is dangerous for the patient in this case!
 * Within the CNS causes of respiratory distress, two specific breathing patterns should be noted:

• Biot's breathing : periodic breathing with respiratory effort followed by apnea. It is an expression of damage to the neurons controlling breathing. It is accompanied by CNS involvement in the region of the posterior fossa of the cranium or brainstem, it can be observed when the irritability of the respiratory center decreases during meningitis or encephalitis.

Other clinical signs of respiratory distress are changes in auscultatory phenomena on the lungs and cyanosis. We also often find disorders of consciousness - anxiety, agitation (arising as a result of hypoxia with subsequent sympathetic activation), somnolence (developing as a result of fatigue or hypoperfusion of the CNS). Cardiovascular symptomatology is also characteristic of respiratory distress (tachycardia, bradycardia, hypotension or mild hypertension, arrhythmia).

Resources

 * HAVRÁNEK, Jiří: Ventilation failure.

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