title: Obstructive airway in Morquio A syndrome, the past, the present and the future.
abstract: Patients with severe tracheal obstruction in Morquio A syndrome are at risk of dying of sleep apnea and related complications. Tracheal obstruction also leads to life-threatening complications during anesthesia as a result of the difficulty in managing the upper airway due to factors inherent to the Morquio A syndrome, compounded by the difficulty in intubating the trachea. A detailed description of the obstructive pathology of the trachea is not available in the literature probably due to lack of a homogenous group of Morquio A patients to study at any one particular center. We present a series of cases with significant tracheal obstruction who were unrecognized due to the difficulty in interpreting tracheal narrowing airway symptoms. Our goal is to provide the guidelines in the management of these patients that allow earlier recognition and intervention of tracheal obstruction. Sagittal MRI images of the cervical spine of 28 Morquio A patients (12±8.14years) showed that19/28 (67.9%) patients had at least 25% tracheal narrowing and that narrowing worsened with age (all 8 patients over 15years had greater than 50% narrowing). Eight out of 28 patients were categorized as severe (>75%) tracheal narrowing when images were evaluated in neutral head and neck position. Of the 19 patients with tracheal narrowing, compression by the tortuous brachiocephalic artery was the most common cause (n=15). Evidence of such tracheal narrowing was evident as early as at 2years of age. The etiology of tracheal impingement by the brachiocephalic artery in Morquio A appears to be due to a combination of the narrow thoracic inlet crowding structures and the disproportionate growth of trachea and brachiocephalic artery in relationship to the chest cavity leading to tracheal tortuosity. In conclusion, tracheal narrowing, often due to impression from the crossing tortuous brachiocephalic artery, increases with age in Morquio A patients. Greater attention to the trachea is needed when evaluating cervical spine MRIs as well as other imaging and clinical investigations, with the goal of establishing a timely treatment protocol to reduce the mortality rate in this patient population.
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States. Electronic address: stomatsu@nemours.org.
authors.forename: Shunji
authors.initials: S
authors.lastname: Tomatsu
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Lauren W
authors.initials: LW
authors.lastname: Averill
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Kazuki
authors.initials: K
authors.lastname: Sawamoto
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: William G
authors.initials: WG
authors.lastname: Mackenzie
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Michael B
authors.initials: MB
authors.lastname: Bober
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Christian
authors.initials: C
authors.lastname: Pizarro
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Goff
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States.
authors.forename: Li
authors.initials: L
authors.lastname: Xie
authors.affiliation: Department of Pediatrics, Gifu University, Gifu, Japan.
authors.forename: Tadao
authors.initials: T
authors.lastname: Orii
authors.affiliation: Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, United States. Electronic address: Mary.Theroux@nemours.org.
authors.forename: Mary
authors.initials: M
authors.lastname: Theroux
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000473:pathology*; D012189:Retrospective Studies; D014132:Trachea / Q000473:pathology; D055815:Young Adult
keywords: Brachiocephalic artery; Disproportionate growth; MRI; Morquio A syndrome; Tracheal obstruction