What are the anesthetic considerations for a patient with Parry‑Romberg syndrome, including airway management, seizure risk, and peri‑operative medication management?

Anesthetic Management of Parry-Romberg Syndrome

Patients with Parry-Romberg syndrome require meticulous airway planning with difficult airway equipment immediately available, seizure prophylaxis continuation, and extended postoperative monitoring due to the high risk of neurologic complications including seizures and potential airway compromise from hemifacial atrophy.

Preoperative Assessment and Planning

Airway Evaluation

• Anticipate difficult intubation due to hemifacial atrophy affecting subcutaneous tissue, muscles, and osteocartilaginous structures, which creates facial asymmetry and potential airway distortion 1.
• Have advanced airway equipment immediately available in the operating room, including video laryngoscopy, fiberoptic bronchoscope, and supraglottic airway devices, following difficult airway protocols 2.
• Document any previous anesthetic complications or airway difficulties from prior procedures 3.
• Measure the degree of facial asymmetry and mandibular involvement, as younger age at disease onset correlates with more severe deformity and potentially more challenging airway anatomy 4.

Neurologic Assessment

• Continue all antiepileptic medications through the perioperative period, including the morning of surgery, as Parry-Romberg syndrome is associated with recurrent seizures in a significant proportion of patients 5.
• Document seizure history, frequency, and most recent seizure activity 5, 4.
• Review neuroimaging if available, noting subcortical white matter changes and parenchymal calcifications that may increase seizure risk 5.
• Assess for associated neurologic manifestations including migraine and neuralgia 4, 1.

Systemic Considerations

• Evaluate for ophthalmologic involvement (enophthalmos, uveitis) that may affect positioning 1.
• Screen for cardiac, rheumatologic, and endocrinologic manifestations that have been reported with this syndrome 1.
• Consider whether the patient is on immunomodulators or steroids, as these have been used in some cases and may require stress-dose coverage 5.

Inpatient vs Outpatient Determination

• Strongly favor inpatient management given the neurologic complications, potential difficult airway, and need for extended monitoring 2.
• The combination of seizure risk and facial structural abnormalities necessitates access to emergency airway equipment, respiratory care equipment, and neurologic consultation 2.

Intraoperative Management

Anesthetic Technique Selection

• General anesthesia with a secure airway is mandatory rather than deep sedation, particularly given the facial structural abnormalities that may mechanically compromise the airway 2.
• For superficial procedures on unaffected body regions, consider regional anesthesia techniques (peripheral nerve blocks or neuraxial anesthesia) to avoid airway manipulation entirely 2, 6.
• If moderate sedation is used for minor procedures, continuous capnography monitoring is essential due to increased risk of undetected airway obstruction from facial asymmetry 2.

Airway Management

• Plan for awake fiberoptic intubation in patients with severe hemifacial atrophy or documented difficult airway 2.
• Avoid preoperative sedatives or use minimal doses with continuous monitoring, as respiratory depression may be poorly tolerated in patients with potential airway compromise 2.
• Position the patient carefully for intubation, accounting for facial asymmetry that may alter optimal head positioning 2.
• Extubate only when fully awake with complete reversal of neuromuscular blockade verified, preferably in semi-upright position 2, 6.

Medication Selection

• Avoid succinylcholine due to potential connective tissue abnormalities associated with the atrophic process, which creates risk for hyperkalemic response 7.
• Use non-depolarizing muscle relaxants such as rocuronium or vecuronium; avoid atracurium and mivacurium due to histamine release that may trigger hemodynamic instability in patients with tissue abnormalities 7.
• Minimize or avoid intraoperative opioids when possible, using multimodal analgesia instead, as respiratory depression may be poorly tolerated 2, 7.
• Carefully titrate anesthetic gases to avoid excessive respiratory depression while maintaining adequate depth 2.

Seizure Prophylaxis

• Maintain therapeutic antiepileptic drug levels throughout the perioperative period 5.
• Avoid anesthetic agents that lower seizure threshold (enflurane, ketamine in high doses) 5.
• Have benzodiazepines and additional antiepileptic medications immediately available 5.

Postoperative Management

Monitoring Requirements

• Admit all patients to ICU or high-dependency unit for continuous cardiopulmonary monitoring for at least 24-48 hours, even after seemingly uncomplicated procedures, due to seizure risk and potential delayed airway complications 7.
• Continuous pulse oximetry and respiratory rate monitoring are mandatory 2, 6.
• Neurologic checks every 2-4 hours to detect seizure activity or new neurologic deficits 5.

Respiratory Management

• Provide supplemental oxygen targeting SpO2 ≥95%, but monitor respiratory rate and effort closely as excessive oxygen can mask hypoventilation 7.
• Position patient semi-upright (30-45 degrees) to optimize respiratory mechanics and reduce risk of airway obstruction from facial asymmetry 6.
• Have emergency airway equipment at bedside given the difficult airway history 2.

Pain Management

• Prioritize non-opioid analgesics including acetaminophen and NSAIDs (if not contraindicated) 7.
• If opioids are required, use reduced doses (50% of standard dosing) with extended monitoring periods due to respiratory sensitivity 7.
• Consider regional anesthesia techniques (peripheral nerve blocks) for postoperative analgesia when anatomically feasible 2.
• Avoid morphine due to histamine release concerns in patients with tissue abnormalities 7.

Seizure Management

• Continue home antiepileptic medications as soon as oral intake is tolerated 5.
• If NPO status is prolonged, convert to intravenous formulations at equivalent doses 5.
• Maintain seizure precautions including padded side rails and suction equipment at bedside 5.

Critical Pitfalls to Avoid

• Never discharge to regular ward settings or same-day discharge, as these patients require extended monitoring even after routine procedures due to neurologic and respiratory risks 7.
• Do not assume normal airway anatomy based on external appearance alone; the atrophy extends to deeper structures including muscles and bones 1.
• Never discontinue antiepileptic medications perioperatively, as this significantly increases seizure risk in an already vulnerable population 5.
• Avoid aggressive fluid resuscitation that may worsen cerebral edema in patients with underlying white matter changes 5.
• Do not rely solely on pulse oximetry; use capnography when available to detect early respiratory compromise 2.
• Recognize that disease may still be active even if clinically stable, as progression can occur over 2-10 years 4.