Aleyna Calap OCAK, Şemsi Mustafa AKSOY, Burak NALBANT
Journal of Comprehensive Surgery - 2026;4(3):74-76
Mucopolysaccharidosis type IV A (Morquio A syndrome) is a rare lysosomal storage disorder characterized by glycosaminoglycan accumulation, causing progressive skeletal deformities and complex airway abnormalities that complicate anesthetic management. We report the perioperative airway management of a 27-year-old woman with MPS type IV A undergoing elective posterior cervical laminectomy for cervical myelopathy secondary to odontoid hypoplasia. Despite a preoperative Mallampati score of II, tracheal intubation was unexpectedly difficult. Attempts using both video laryngoscopy and a Macintosh laryngoscope failed because of a markedly enlarged, thickened epiglottis obstructing glottic visualization. Intubation was successfully achieved with a Miller laryngoscope. Anesthesia was maintained with total intravenous anesthesia, and the patient was extubated after complete recovery and a successful cuff leak test. Postoperative upper airway edema resolved with medical treatment. This case highlights that conventional airway assessment may underestimate airway difficulty in MPS type IV A and emphasizes the importance of alternative airway devices, meticulous perioperative planning, and vigilant postoperative monitoring.
