Review Article

Anesthetic Management in Mucopolysaccharidoses

Table 3

Involvement of organs and systems.

ā€‰Clinical manifestations

Upper airwaysFacial coarseness, hypertelorism, sunken nasal dorsum, anteverted nostrils, thickened lips, gingival hyperplasia [19] and dental distortions. Swollen tongue, epiglottis, tonsils and adenoids [47], pharyngomalacia (anterior position of larynx [48]).

Lower airwaysExpanded larynx and tracheobronchomalacia [49] (enlarged cartilage). Recurrent pneumonia and restrictive lung disease that results in lung failure, Obstructive Sleep Apnea and sudden death for central apnea [5]. Rhinitis, tonsillitis, laryngitis and otitis media [4, 47].

CardiacValvular disease, cardiomyopathy, right ventricular dysfunction and congestive heart failure [2, 27].
Coronary vessel flow impairment and cardiac ischemia [20, 50].

Orthopedic-Bone ChangesShort neck, joints stiffness [47], odontoid instability [48], hypo plastic mandible, thoracic cage restriction for kyphoscoliosis [24].
Hip dysplasia, valgus knee, spinal cord compression and atlanto-axial instability [51, 52].

OthersMental retardation [14] and hyperactive, aggressive behavior [53]. Communicating hydrocephalus.
Hearing loss [2], corneal opacities [54, 55].
Hepatosplenomegaly, umbilical and inguinal hernias [39, 40].