<p>Meier-Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the clinical triad of microtia, patellar hypo- or aplasia, and proportionate short stature. Due to its phenotypic variability and frequent craniofacial anomalies, anesthetic management may be challenging. We report the perioperative management of a 5-year-old boy with genetically confirmed MGS undergoing adenoidectomy, otomicroscopy, and bilateral tympanostomy tube insertion. Preoperative airway evaluation was unremarkable, with no classical predictors of a difficult airway. However, videolaryngoscopy during induction revealed an anterior and deeply positioned glottis with soft, collapsible surrounding structures. Initial intubation with a flexible stylet was unsuccessful; a second attempt using a stiffer stylet was successful under direct visualization. The remaining perioperative course, including extubation and postoperative monitoring, was uneventful. A multidisciplinary planning and individualized anesthetic management enabled safe ambulatory care. To our knowledge, this is the first published case describing anesthetic management in a patient with MGS.</p>

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Anesthetic management in a child with Meier-Gorlin syndrome: a case report

  • Christine Gaik,
  • Kalvis Kreiss,
  • Astrid Morin,
  • Katharina Toussaint,
  • Hinnerk Wulf,
  • Nicolas Schmitt

摘要

Meier-Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the clinical triad of microtia, patellar hypo- or aplasia, and proportionate short stature. Due to its phenotypic variability and frequent craniofacial anomalies, anesthetic management may be challenging. We report the perioperative management of a 5-year-old boy with genetically confirmed MGS undergoing adenoidectomy, otomicroscopy, and bilateral tympanostomy tube insertion. Preoperative airway evaluation was unremarkable, with no classical predictors of a difficult airway. However, videolaryngoscopy during induction revealed an anterior and deeply positioned glottis with soft, collapsible surrounding structures. Initial intubation with a flexible stylet was unsuccessful; a second attempt using a stiffer stylet was successful under direct visualization. The remaining perioperative course, including extubation and postoperative monitoring, was uneventful. A multidisciplinary planning and individualized anesthetic management enabled safe ambulatory care. To our knowledge, this is the first published case describing anesthetic management in a patient with MGS.