Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc
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Case Report

Volume 8, Number 7, July 2017, pages 203-206

Anesthetic Management of a 13-Year-Old Adolescent With Mucolipidosis Type II for Total Hip Arthroplasty


Table 1. Reports of Anesthetic Care for Patients With ML II
Authors and referencePatient demographicsIntraoperative managementPostoperative management
ID: internal diameter; GA: general anesthesia; LMA: laryngeal mask airway; ETT: endotracheal tube.
Mahfouz and George [14]A 5-year-old girl for gingivectomy and dental extractionsAirway progressively obstructed despite use of an oropharyngeal airway and a jaw thrust maneuver. Bag-valve-mask ventilation was difficult. A #2 LMA was placed which successfully maintained the airway. Endotracheal intubation was deemed necessary to guard against the risk of aspiration. Laryngoscopy was difficult due to limited neck movement and macroglossia. The LMA was reintroduced after three failed attempts at endotracheal intubation. Nasal intubation failed due to nasal bleeding from congested and hypertrophied nasal tissue and large adenoids and rapid desaturation. Following placement of a sand bag under the patient’s shoulder, oral endotracheal intubation was possible with a 4 mm ID ETT.The patient’s trachea was extubated when fully conscious and the recovery was uneventful.
Bains et al [15]Three siblings with multiple anesthetic proceduresAt 9 years of age, the eldest sibling underwent GA for removal of grommets. Bag-valve-mask ventilation was difficult despite a range of oropharyngeal airways. An LMA failed to provide a completely effective airway. A range of straight and curved laryngoscope blades revealed only the posterior aspect of the airway and no glottis structures were identified with the Belscope® blade and prism. The grommets were removed under face mask anesthesia with a semi-obstructed airway.
The middle child underwent repair of an umbilical hernia at 8 years of age. The airway was easy to maintain with bag-valve-mask and oropharyngeal airway. A large epiglottis was seen with direct laryngoscopy using a straight laryngoscope blade and a 4.0 mm ID ETT tracheal tube was passed under the epiglottis into the trachea.
The youngest child also underwent removal of grommets. His airway was easy to maintain with bag-valve-mask and an oropharyngeal airway followed by an LMA. Laryngoscopy was attempted with a range of straight and curved laryngoscopy blades, but only the epiglottis could be seen. The larynx was seen with the Belscope® blade with prism.
Postoperative outcomes were uneventful.
Two of the three children demonstrated increasing difficulty with airway management as they grew older.