Anesthesia Considerations in Management of the Patient with Achondroplasia

Introduction

Achondroplasia is the most prevalent form of dwarfism and presents distinctive challenges in anesthesia management due to skeletal dysplasia
• Preoperative assessment should include airway examination and cardiovascular assessment, as there is an increased risk of respiratory and cardiovascular complications in achondroplasia patients during anesthesia.
• Intraoperative measures involve careful positioning and appropriate equipment selection to address potential complications.
• Patients with achondroplasia often exhibit anatomical variations such as shortened neck with limited mobility and distorted vertebral anatomy.

Case Description

28-year-old, 4'4'', 52.2kg male, history of achondroplasia, asthma, anxiety, post-traumatic stress disorder (PTSD), sagittal plane imbalance, spinal stenosis, and obstructive sleep apnea (OSA).
• Medications: alprazolam, aspirin, brexpiprazole, fluvoxamine, gabapentin, morphine, nortriptyline
• Allergies: amoxicillin, cefaclor and tramadol
• Presenting for revision lumbar posterior spinal fusion (PSF), Refusion/Regrafting Fusion T7-L4 and myocutaneous flap closure due to wound dehiscence and hematoma at the surgical site.
• After arrival to the OR, standard ASA monitors were placed followed by placement of a preinduction arterial line.

Induction: Preoxygenation, Propofol 150mg and Succinylcholine 80mg. The patient was difficult to mask ventilate by a single clinician and subsequently began to desaturate to the mid-80s.

• He required a two-person bag-mask technique to achieve adequate ventilation, and his oxygen saturation returned to 100%.
• He was successfully intubated with a size 7.0 ETT using a McGrath video laryngoscope on the first attempt.
• The rest of the case was completed uneventfully, and he was extubated and transferred to the recovery room.

Conclusion

• This case serves as a reminder that patients with achondroplasia typically present with anatomical anomalies which may predispose them to a difficult airway increasing the risk of respiratory complications, particularly during intubation.
• Anesthetic challenges in this population may also include difficult neuraxial access.
• Careful consideration of anatomical and physiological differences is crucial for optimizing perioperative outcomes.
• During anesthesia, maintaining appropriate intravascular volume through fluid administration helps prevent hypovolemia, which can exacerbate the risk of spinal cord compression.
• With a meticulous approach to preoperative assessment, tailored intraoperative care based on patient co-morbidities and anatomical variation, complications can be minimized, ensuring safe and effective perioperative care for this patient population.

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