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Awake airway endoscopy in mucopolysaccharidosis: a case report
Sofia Fernandes1, Jo O Borges2, Marta Martins3
1Centro Hospitalar Universit..rio Lisboa Norte, Department of Anesthesiology, Lisbon, Portugal.
Abstract:
Mucopolysaccharidosis (MPS) are a group of rare genetic inherited diseases with a progressive course due to the accumulation of glycosaminoglycans resulting in anatomic abnormalities and organ dysfunction, including the respiratory, cardiovascular, skeletal, and neurological systems that can increase the risk of anesthesia complications. Clinical manifestations are variable, multisystemic, and include severe morphological changes. The anesthetic management of these patients is complex, particularly airway management, which can be planned to include a fiberoptic airway investigation prior to surgery. We present two cases of patients with MPS type VI and VII who underwent fiberoptic airway mapping under conscious sedation, with no complications. Since MPS is a rare but challenging disease concerning the airway management, we propose a safe and effective anesthetic technique that could be used for fiberoptic bronchoscopy and allow fiberoptic-assisted tracheal intubation at the time of surgery.
Insights
Mucopolysaccharidosis (MPS) patients face anesthesia risks due to progressive genetic disorders. Fiberoptic airway mapping under conscious sedation offers a safe approach for managing complex airways in MPS types VI and VII.
Area of Science:
- Anesthesiology
- Rare Genetic Diseases
- Airway Management
Background:
- Mucopolysaccharidosis (MPS) comprises rare inherited disorders characterized by progressive glycosaminoglycan accumulation.
- Multisystemic manifestations in MPS, including respiratory and skeletal changes, complicate anesthetic management and increase risks.
- Airway management is particularly challenging in MPS patients due to potential anatomical abnormalities.
Observation:
- Two cases of patients with MPS type VI and VII underwent fiberoptic airway mapping.
- The procedure was performed under conscious sedation.
- No complications were observed during the fiberoptic airway mapping.
Findings:
- Fiberoptic airway mapping under conscious sedation is a safe and effective technique for evaluating complex airways in MPS patients.
- This pre-operative assessment allows for better planning of anesthetic and surgical interventions.
Implications:
- Proposes a safe anesthetic technique for fiberoptic bronchoscopy and tracheal intubation in MPS patients.
- Highlights the importance of pre-operative airway assessment in managing rare genetic disorders.
- Aims to improve anesthetic outcomes and reduce complications in patients with Mucopolysaccharidosis.
