A 16-year-old tall, thin boy with high myopia and a downward and inward displacement of both lenses is being prepared for lens extraction. The anaesthetist asks for specific advice before administering general anaesthesia. The chief concern is:
- A Risk of fatal thromboembolic episodes due to friable platelets and defective fibrinolysis ✓
- B Malignant hyperthermia triggered by succinylcholine
- C Severe bradycardia from the oculocardiac reflex
- D Raised intracranial pressure complicating intubation
Correct answer: A. Risk of fatal thromboembolic episodes due to friable platelets and defective fibrinolysis
Explanation
Inferonasal lens subluxation in a marfanoid habitus with myopia points to homocystinuria. Patients have abnormal platelet adhesiveness and increased thrombotic tendency, so general anaesthesia carries a documented risk of fatal thromboembolism, and elective lens surgery is preferably done under general anaesthesia only when unavoidable, with careful hydration and avoidance of prolonged immobility. Malignant hyperthermia, oculocardiac reflex and raised intracranial pressure are not features of this metabolic disorder.
Reference: Parsons' Diseases of the Eye, 23rd ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.