Airway management in mucopolysaccharidosis: a retrospective case series review

Murat Tümer1, Aysun Ankay Yılbaş1, Sehend Debbağ1

  • 1Hacettepe University Faculty of Medicine, Department of Anesthesiology and Reanimation, Ankara, Turkey.

Insights

Managing airways in mucopolysaccharidosis patients undergoing surgery presents challenges. Video laryngoscopy appears safe, but alternative airway management techniques are crucial, especially for older patients.

Area of Science:

  • Anesthesiology
  • Pediatric Surgery
  • Genetic Disorders

Background:

  • Mucopolysaccharidosis (MPS) encompasses a group of rare genetic disorders.
  • Airway management in MPS patients undergoing surgery is often complex.
  • This study reviews surgical airway management in MPS patients.

Observation:

  • A retrospective review of 31 surgeries in MPS patients (2015-2019) was conducted.
  • The mean patient age was 127.6 months; MPS-IV and MPS-VI were most frequent.
  • Orthopedic surgeries were most common, with difficult intubation in 10 procedures.

Findings:

  • Difficult intubation occurred in 10 procedures, exclusively in patients over 36 months old.
  • Video laryngoscopy was the primary intubation method utilized.
  • Seventeen patients required intensive care unit (ICU) follow-up.

Implications:

  • Video laryngoscopy demonstrates safety in MPS patients for airway management.
  • Anesthesiologists must maintain preparedness for alternative airway techniques.
  • Age over 36 months may be a risk factor for difficult intubation in MPS patients.

Related Concept Videos

Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
239
Chronic Obstructive Pulmonary Disease-V: Management01:29

Chronic Obstructive Pulmonary Disease-V: Management

Managing Chronic Obstructive Pulmonary Disease (COPD) involves a multifaceted approach to reduce symptoms, prevent exacerbations, improve overall health status, and slow disease progression. Key strategies include lifestyle modifications, pharmacotherapy, supportive therapies, and, in some cases, surgery. Here is an overview of the primary COPD management strategies:
Smoking Cessation
2.7K
Acute Respiratory Failure-V01:29

Acute Respiratory Failure-V

The treatment for acute respiratory failure varies based on factors like the underlying cause, overall health, and severity. A collaborative healthcare team is essential for early detection, often through arterial blood gas analysis. Identifying the cause is the primary goal, with treatment strategies adjusted for ventilation/perfusion (V/Q) mismatch, shunting, or diffusion impairment.
Ensure that patients are monitored continuously for their response to therapy, including changes in...
232
Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques01:30

Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques

Airway management is essential in emergency and surgical medicine, ensuring ventilation and oxygenation in patients who cannot maintain their own airway. Clinicians use a range of techniques and devices to secure the airway, depending on the patient’s condition and the clinical context. Key methods include endotracheal intubation, rapid sequence intubation (RSI), supraglottic airway devices, and advanced visualization aids. In cases where these approaches fail, surgical airway...
143
Cardiopulmonary Resuscitation II: ACLS Airway Management01:22

Cardiopulmonary Resuscitation II: ACLS Airway Management

Airway management is a key skill in emergency and critical care settings, as maintaining a clear airway is essential for adequate oxygenation and ventilation.Head Tilt-Chin Lift TechniqueThe head tilt-chin lift maneuver is an essential technique primarily used in patients without suspected cervical spine injuries. To perform this maneuver, one hand is placed on the patient’s forehead, and gentle pressure is applied backward to tilt the head. The fingertips of the other hand are positioned...
177
Chronic Obstructive Pulmonary Disease-V: Nursing Management01:30

Chronic Obstructive Pulmonary Disease-V: Nursing Management

Nursing management of Chronic Obstructive Pulmonary Disease (COPD) is crucial for providing thorough care and support to patients. Nurses play an integral role in this process through detailed assessment, careful planning, targeted interventions, and ongoing evaluation. Here's an overview of the critical steps in nursing management for COPD.
Assessment
4.4K