Pediatric syndromes with noncraniofacial anomalies impacting the airways

Abstract

Syndromes with noncraniofacial abnormalities can be a real challenge in terms of airway management. The key to success is effective preparation, presence of personnel with expertise in difficult pediatric airway management, regular training and familiarity with difficult intubation equipment, and teamwork. Considering that there are a very large number of syndromes, with variable phenotypic expression, the management strategy of every case will be dictated by the anatomical and functional airway as assessed on physical examination and subsidiary examinations before induction of anesthesia.

An Approach to the Airway Management in Children with Craniofacial Anomalies

“…The airway management plan of the infants and children with difficult airway has many proposed algorithms but not unified as in the American Society of Anaesthesiologists (ASA) adult difficult airway algorithm [4][5][6][7]. The awake intubation is no more a popular option in pediatric intubations except for some emergency situations where the patient is in severe distress and obstruction, as it carries its own disadvantages (increase in intracranial pressure ICP and intracerebral hemorrhage ICH, gagging, uncooperative kid), hence induction of anesthesia with preservation of spontaneous breathing is the cornerstone for a safe airway management in patients with craniofacial syndromes with suspected difficult airway.…”

“…Clinical considerations: Down's syndrome is a multisystem disease associated with generalized growth retardation and varying degrees of mental impairment. Its clinical hallmarks encompass generalized neuromuscular hypotonia, atlantoaxial instability often associated with congenital cardiac anomalies (in particular an atrioventricular septal defect, a patent ductus arteriosus, or a tetralogy of Fallot) and gastrointestinal malformations (duodenal atresia and Hirschsprung disease) [4,5,9]. In addition, children with DS compared to healthy ones are more prone to multiple health issues, including obstructive sleep apnea, recurrent infections, hypothyroidism, epilepsy, audiovestibular and visual impairment, hematopoietic disorders (including leukemia), anxiety disorders, and early-onset Alzheimer disease.…”

“…Screening patients at risk for cervical spine instability is a crucial step before airway management in order to avoid spinal injury, especially those with hyperlaxed spines who may require C-spine precautions with in-line-stabilization during intubation. This is performed via a lateral neck X-ray in flexion and extension positions [1,[4][5][6][7].…”

“…The An Approach to the Airway Management in Children with Craniofacial Anomalies DOI: http://dx.doi.org/10.5772/intechopen.93426 temporo-mandibular joint may get affected with restricted mobility. Deposition of mucopolysaccharides in the heart and vascular tissues may lead to cardiomyopathies, arrhythmias, and pulmonary hypertension [5].…”

“…It is a multi-loculated benign congenital tumor affecting preferably the head and neck. It can present as a neck mass on prenatal ultrasound [5]. Airway compromise can be the sequelae of direct compression from the mass and/or bony changes following mandibulomaxillary hypertrophy (Figure 8).…”