Stifling the sneeze in ACHOO syndrome-What the anesthesiologist should know.

Abstract

Dear Editor, A 32-year-old male, a known patient of ocular amyloidosis, was posted for superficial keratectomy (removal of the corneal epithelium up to Bowman membrane) of the right eye under topical anesthesia. This patient had a history of photic sneeze reflex (PSR) since 12 years of age and was a diagnosed case of autosomal dominant compelling helio-ophthalmic outburst (ACHOO) syndrome. This condition is characterized by uncontrollable sneezing episodes on exposure to bright light or periocular injection including peribulbar block or eyelid infiltration. He suffered from continuous sneezing episodes lasting around 5 min even in response to slit-lamp examination. This was usually followed by a refractory period during which the ophthalmic examination was conducted. He had no other systemic illness. On the day of the procedure, he developed an irrepressible sneezing reflex after cleaning and draping of the right eye in the operating room. The ambient light had stimulated the PSR. The anesthesiologist was called for help. On clinical examination, the patient’s vitals were stable with a heart rate of 62/min, SpO2 98%, and blood pressure 100/70 mmHg. His sneezing fit had ceased but the patient was extremely anxious. An intravenous line was secured and he was administered midazolam 1 mg and fentanyl 50 μg intravenously. He became calm and allowed the gentle insertion of a wire speculum but still could not look at the light of the operating microscope though there were no more sneezing episodes. He was given another 50 μg fentanyl intravenously and reassured. The procedure was completed smoothly in 10 min. No further drugs were administered nor were there any sneezing episodes. A syringe filled with injection pheniramine maleate 22.75 mg was kept ready but was not required. Five days later, he was posted for superficial keratectomy of the other eye under monitored anesthesia care. The same anesthetic technique was followed and he was administered midazolam 1 mg and fentanyl 100 μg in small aliquots even before skin preparation. The procedure was completed uneventfully under topical anesthesia without a single sneezing episode. Sneezing fit after exposure to bright light or “sun sneezing” has been reported in about 25% of the population. These patients are also prone to a sternutatory reflex after local anesthetic injection under sedation.[1] Unexpected or sudden head movement during peribulbar block or eye surgery can cause globe perforation or vascular injury and retrobulbar hemorrhage.[2] PSR is a primitive brainstem reflex, sometimes considered a variant of the trigemino-cardiac reflex with the center in the trigeminal nucleus and adjacent reticular formation. The afferent arc is from the first (ophthalmic) and second (maxillary) division of the trigeminal nerve (VN) and the efferent is the vagus nerve. A cross-talk between the optic nerve (IIN) and VN has also been hypothesized.[3] No known treatment exists except avoidance. Few authors have found deep sedation, eyelid infiltration, midazolam, and propofol to increase the risk while opioids, dexmedetomidine, and antihistamines have been found to reduce the risk.[2,4] Some have even suggested PSR as an indication for general anesthesia in ophthalmic surgery.[5] It is important for anesthesiologists routinely caring for patients for ophthalmic surgery to be aware of this reflex as it can have severe sight-threatening consequences. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.