Congenital pediatric airway problems

Published on 07/02/2015 by admin

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Last modified 07/02/2015

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Congenital pediatric airway problems

Wayne H. Wallender, DO

The upper airway is more easily compromised in infants and children than in adults. Anatomically, compared with an adult, the tongue of an infant or child is relatively larger within the mouth, the larynx is more cephalad, the glottic opening and airways are narrower, the arytenoid cartilages are more prominent, and the occiput is larger, resulting in greater resistance to airflow. Infants’ and children’s respiratory physiology further complicates the management of the airway. Because infants have higher weight-adjusted basal metabolic rates, O₂ consumption and CO₂ production rates are higher. In addition, the functional residual reserve of infants, per kilogram, is less than that of adults. Congenital abnormalities associated with airway problems can cause further difficulties with airway management.

Management of the difficult pediatric airway should include spontaneous ventilation with the consideration that direct laryngoscopy has a significant failure rate. As a general approach, these patients can be divided into those who will be difficult to intubate but can be ventilated by mask and those who are difficult or impossible to ventilate by mask. The latter group poses a more difficult anesthetic challenge and may require emergency tracheostomy. If a child can be ventilated by mask, then a number of options can be safely employed until the trachea is successfully intubated. The anesthetic goals are always a safe induction and intubation as well as a safe extubation.

Congenital abnormalities of the airway

Mucopolysaccharidoses

Hurler syndrome is associated with severe mental retardation, gargoyle facies, deafness, stiff joints, dwarfism, pectus excavatum, kyphoscoliosis, abnormal tracheobronchial cartilage, hepatosplenomegaly, severe cardiac valvular disease, and early coronary artery disease. Children with Hunter syndrome often have course facial features, deafness, hypertrichosis, stiff joints, macrocephaly, and carpal tunnel syndrome. Children with Morquio syndrome often appear healthy at birth; however, as the child ages, manifestations may include coarse facial features, prognathism, odontoid hypoplasia, atlantoaxial instability resulting from thoracic or lumbar kyphosis, aortic valve incompetence, hepatomegaly, inguinal hernias, mixed hearing loss, ocular complications, and limb abnormalities.

Upper airway obstruction and difficult intubation are common in children with mucopolysaccharidosis because of infiltration of lymphoid tissue, enlarged tongue, small mouth, and profuse thick secretions. A difficult airway should be expected and, as the infant ages, the airway may become even more difficult to manage.

Pierre robin syndrome

Children with Pierre Robin syndrome may have cleft palate, micrognathia, glossoptosis (posterior displacement of the tongue), and congenital heart disease. Affected infants can present with significant airway problems almost immediately after birth. Intubation may be very difficult and should initially be attempted with the infant awake. Tracheostomy should be considered early. Airway obstruction may be relieved by positioning the patient prone, pulling the tongue forward, or using a nasopharyngeal airway. A suture may be placed to maintain the tongue position.

Treacher collins syndrome

This syndrome is the most common of the mandibulofacial synostoses. Clinical features include micrognathia, aplastic zygomatic arches, microstomia (small mouth), choanal atresia, and congenital heart disease. These children usually present with less severe airway and intubation difficulties than are seen in children with Pierre Robin deformities.

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