Difficult airway approach in patient with Oro-Facial-Digital syndrome type II or Mohr syndrome: a case report

Abstract

Oro-facio-digital syndrome is a rare disorder of embryonic development. Patients may have deformities in the mouth, face, hands and feet. At least 11 different clinical forms of the syndrome have been described, including oro-facial-digital syndrome type II or Mohr syndrome. The approach of the airway in the oro-facial-digital syndrome can be a challenge because craniofacial changes such as leborine lip / cleft palate, present in the syndrome, are predictors of difficult airway in pediatrics. The case report describes a 4-month-old infant with Mohr Syndrome who was admitted with respiratory symptoms and during hospitalization presented cardiac arrest, with direct laryngoscopy performed several times, without success in orotracheal intubation. The anatomical differences explain the greater potential for technical difficulties of the airways in infants than in teenagers or adults. In general, the airways of infants differ from those of adults by five points: 1) the relatively large size of the child's tongue, in relation to the oropharynx; 2) the larynx has a more cephalic location on the neck; 3) the epiglottis is short, omega-shaped and angled over the larynx entrance; 4) the vocal cords are angled, consequently, a tracheal tube that has passed blindly can easily lodge in the anterior commissure instead of sliding into the trachea; 5) the larynx is funnel-shaped, with the narrowest portion located in the cricoid cartilage. The guidelines for difficult airways in pediatrics are essential tools in this context for the prevention of associated complications, so their mastery by intensive care professionals and anesthesiologists is essential. Sharing this information is important because there is little data in the literature about approaching the airway in a patient with Mohr syndrome.