Anesthetic care and perioperative complications of children with Morquio syndrome

• Published in: Pediatric anesthesia 2012-09, Vol.22 (9), p.901-907
• Main Authors: Theroux, Mary C., Nerker, Tanvi, Ditro, Colleen, Mackenzie, William G.
• Format: Article
• Language: English
• Subjects: Adolescent; Airway Management; Anesthesia - adverse effects; Anesthesia, Conduction; Anesthesiology; Bronchi - abnormalities; Child; Child, Preschool; Children & youth; difficult airway; Female; Glidescope; Humans; Infant; Intubation, Intratracheal; Male; Morquio syndrome; MPS type IVA; mucopolysaccharidoses; Mucopolysaccharidosis IV - surgery; pediatrics; Perioperative Care - methods; Preanesthetic Medication; Retrospective Studies; Spinal Fusion; Trachea - abnormalities
• ISSN: 1155-5645; 1460-9592
• DOI: 10.1111/j.1460-9592.2012.03904.x

Summary Objectives:  Our objective was to make recommendations based on our experience and findings from this study regarding the anesthetic care of children with Morquio syndrome (MS). We emphasize information not readily available in the Anesthesiology literature. Aim:  To describe the unique nature of difficulties, especially the relationship of the head and neck to airway patency. In addition, we aim to examine 83 intubations performed in 28 patients and report on observed preferences. Background:  Much of the available literature in Anesthesiology consists of case reports of single or small groups of cases, many describing a nonhomogenous population inclusive of many mucopolysaccharidoses. Methods/Materials:  We retrospectively studied 28 children with MS who underwent 108 surgical procedures at our pediatric hospital, which provides multidisciplinary, comprehensive care to children with skeletal dysplasia. Results:  Cervical fusion was performed in 22 of 28 patients in our study. Eight children after cervical fusion became difficult to intubate for subsequent surgical procedures. In addition, we found airway abnormalities including tortuous appearance of the trachea and bronchi, evident on chest radiograph, as a result of the abnormalities in the hyaline cartilage and deposits of glycosaminoglycans. Conclusion:  Morquio syndrome results in abnormalities of not only upper airway but also of large airways. Information from 83 intubations of 108 anesthetics (in 28 patients) shows a preference for Glidescope when intubating children with MS. Displacing the tongue anteriorly prior to intubation by manual retraction using a ring forceps or a piece of gauze helps to access the larynx in children with MS.