Anesthetic Management of a Patient With Myhre Syndrome
Anesthesia Progress
|December 8, 2025
Summary
Myhre syndrome patients may face difficult endotracheal intubation due to airway complications. Careful preoperative assessment and preparation are crucial for safe anesthesia management in these individuals.
Area of Science:
- Medical Genetics
- Anesthesiology
- Connective Tissue Diseases
Background:
- Myhre syndrome (MS) is a rare genetic disorder affecting connective tissues.
- Key features include intellectual disability, growth deficiency, and craniofacial abnormalities.
- Airway anomalies like laryngotracheal and choanal stenosis are common in MS.
Purpose of the Study:
- To highlight the challenges of endotracheal intubation in a patient with Myhre syndrome.
- To emphasize the need for specialized airway management strategies in MS patients.
Main Methods:
- A case report of a 17-year-old female with Myhre syndrome undergoing dental treatment.
- Airway assessment revealing trismus, macroglossia, and limited neck flexion.
- Endotracheal intubation performed using a flexible fiber-optic bronchoscope.
Main Results:
- Successful endotracheal intubation was achieved using a fiber-optic bronchoscope.
- The patient's specific anatomical features presented significant intubation challenges.
Conclusions:
- Patients with Myhre syndrome require thorough preoperative airway evaluation.
- Anesthesia providers must be prepared for potentially difficult tracheal intubation in MS cases.
- Flexible fiber-optic bronchoscopy is a valuable tool for managing difficult airways in Myhre syndrome.
Related Concept Videos
01:24General Anesthesia: Overview
552
Anesthesia is a medical procedure that uses drugs for CNS suppression to enable painless surgeries and procedures. The selection of anesthetics is influenced by their pharmacokinetic properties, side effects, and patient characteristics. Various types of anesthesia include general, local, regional, spinal, and inhalational.
General anesthesia induces unconsciousness in the whole body, while the others target specific areas or sensations. It is administered to minimize adverse effects, maintain...
General anesthesia induces unconsciousness in the whole body, while the others target specific areas or sensations. It is administered to minimize adverse effects, maintain...
552
01:24Parenteral Anesthetics: Overview
546
Intravenous anesthetics are drugs administered parenterally to induce anesthesia or sedation. Propofol is a widely used agent formulated as a 1% emulsion in soybean oil, glycerol, and egg phosphatide. It induces rapid anesthesia primarily due to its rapid distribution from the bloodstream to target tissues and is metabolized in the liver. However, it can cause significant pain on injection and hypertriglyceridemia. Fospropofol, a water-based prodrug of propofol, lacks these adverse effects.
546
01:31Skeletal Muscle Relaxants: Therapeutic Uses
859
Skeletal muscle relaxants are used to relax muscle tone and alleviate painful muscle contractions. However, the choice of skeletal muscle relaxants depends on the duration of the surgical procedure in order to minimize potential side effects. Skeletal muscle relaxants like neuromuscular blocking agents [NMBAs] are commonly employed as adjuvants alongside general anesthetics in clinical settings. NMBAs are also used to maintain controlled ventilation during surgery of the larynx or pharynx...
859
01:20Inhalational Anesthetics: Overview
955
Inhalation anesthetics are drugs that induce general anesthesia upon inhalation. They work by increasing the sensitivity of GABAA receptors or inhibiting NMDA receptors, leading to a decrease in central nervous system activity. The depth of anesthesia can be rapidly adjusted by changing the concentration of the inhaled gas. Some common examples of inhalational anesthetics include volatile liquids like isoflurane, desflurane, sevoflurane and gases like xenon and nitrous oxide. Isoflurane, a...
955
01:12Local Anesthetics: Adverse Effects
739
While local anesthetics are generally safe and well-tolerated, they can occasionally cause adverse effects that vary in severity. Local anesthetics can induce toxicity at two distinct levels. They can either produce local effects through direct contact with the neural elements or be absorbed into the bloodstream from the injection site, leading to systemic effects.
Once absorbed into the systemic circulation, local anesthetics can affect the organs that depend on the functioning of sodium...
Once absorbed into the systemic circulation, local anesthetics can affect the organs that depend on the functioning of sodium...
739
01:21Skeletal Muscle Relaxants: Adverse Effects
761
Skeletal muscle relaxants are widely used for muscle paralysis and relieving pain following any muscle injury or stiffness. However, depending on the drug type, they can have adverse effects that range from mild to severe. Usually, nondepolarizing neuromuscular blockers have minimal side effects. For example, drugs like d-tubocurarine, cisatracurium, and rocuronium cause hypotension, whereas drugs like baclofen, when stopped abruptly, can lead to the recurrence of spastic conditions.
Unlike...
Unlike...
761