LASIK - BMJ Case Reports

Unexpected outcome ( positive or negative) including adverse drug reactions

CASE REPORT

Urrets-Zavalia syndrome after laser-assisted in situ keratomileusis (LASIK) Kavitha Avadhani,1 Gaurav Prakash,2 Dhruv Srivastava,3 Anitha Shakunthala4 1

Uvea and Ocular Inflammation Services, NMC Specialty Hospital, Abu Dhabi, United Arab Emirates 2 Department of Cornea and Refractive Surgery Services, NMC Specialty Hospital, Abu Dhabi, United Arab Emirates 3 NMC Specialty Hospital, Abu Dhabi, United Arab Emirates 4 Glaucoma Services, NMC Specialty Hospital, Abu Dhabi, United Arab Emirates Correspondence to Dr Gaurav Prakash, [email protected] Accepted 24 June 2015

SUMMARY We report a case of fixed, non-reactive pupil (UrretsZavalia syndrome) in a 34-year-old patient following laser-assisted in situ keratomileusis (LASIK) caused probably by intermittent postoperative intraocular pressure fluctuations. This case highlights the possibility of a fixed and dilated pupil even after LASIK, a technically non-intraocular surgery, which is due to a preventable cause: postoperative intraocular pressure fluctuations. To the best of our knowledge, this is the first report of Urrets-Zavalia syndrome following LASIK. BACKGROUND A postoperative fixed and dilated pupil is a wellreported clinical entity with reports following various ophthalmic surgeries such as penetrating keratoplasty (PKP), cataract surgery, trabeculectomy and deep anterior lamellar keratoplasty.1–4 Initially, it was thought to be associated with keratoconus, however, cases with other aetiologies have also been seen. We report a case of fixed, non-reactive pupil, the lower end of the spectrum of Urrets-Zavalia syndrome, in a patient following laser-assisted in situ keratomileusis (LASIK). To the best of our knowledge, there has been no other report of such an occurrence.

CASE PRESENTATION A 37-year-old man with anisometropia presented to us for elective vision correction. He had an uncorrected distance visual acuity (UDVA) of OD 20/200 improving to 20/20 with a refraction of −5.50 DS/−2.0 DC at 30° and OS 20/25 improving with −0.25 DS to 20/20. The anterior segment evaluation was normal. The pupillary reaction was brisk for direct and consensual response. The intraocular pressure (IOP) was 15 mm Hg OU on applanation tonometry. Dilated retinal evaluation was normal and the cup-disc ratio was 0.3 OU, with a healthy neuro-retinal rim.

INVESTIGATIONS

To cite: Avadhani K, Prakash G, Srivastava D, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2015210977

The preoperative corneal topography was performed on a Scheimpflug+Placido device (Sirius, Costruzione Strumenti Oftalmici, Italy) and was normal for both eyes (figures 1 and 2). We measure scotopic (0.4 lux), mesopic (4 lux) and photopic (40 lux) pupillary diameters in all cases undergoing LASIK using the inbuilt pupillometer in the Sirius device. The preoperative pupil diameters were as follows for scotopic, mesopic and photopic illuminations: 3.74, 3.50 and 2.86 mm, respectively, for the right eye (figure 3A), and 3.42, 3.02 and

2.51 mm, respectively, for the left eye (figure 4A). As the patient was comfortable with the unaided vision in the left eye, it was decided only to operate on the right eye. The patient underwent uneventful LASIK surgery in the right eye. A superiorly hinged femtosecond-assisted flap with a targeted flap thickness of 120 microns was created with intralase 150 kHz (iFS) (Abbott Medical Optics, California, USA) using our standard parameters including a bed energy of 0.9 mJ. The excimer ablation was performed with Visx Star 4 (Abbott Medical Optics, California, USA), using a Variable spot size refractive (VSSR) algorithm. Postoperatively, the patient was prescribed topical moxifloxacin and prednisolone eye drops four times per day and carboxymethylcellulose drops 0.5% every hour. The postoperative recovery was good with a UDVA of 20/20 OD recorded both on the third postoperative day and 1 week postoperatively. The patient was informed about the protocol to taper the steroids (twice/ day×1 week, once/day×1 week, then none). For the first month after LASIK, we have a weekly follow-up protocol. Therefore, the patient was advised to review after a week. However, he was unable to review as advised owing to some personal emergency. When he finally came back after approximately 1 month of the surgery, he reported mild glare in the right eye. On examination, a fixed middilated pupil was noted in the right eye (figure 5A). There were a few pigments noted in the anterior chamber without any obvious cellular reaction, iris atrophy or posterior synechiae. The flap and the interface area were normal. The unoperated fellow eye was normal (figure 5B). There was no evidence of interface cleft or fluid clinically or on optical coherence tomography (figure 6A). Automated pupillometry revealed the pupillary diameters for the right eye to be 4.88 mm for scotopic, 4.86 mm for mesopic and 4.83 mm for photopic illuminations (figure 3B). The unoperated left eye’s response to illumination changes was maintained (figure 4B). IOP was 14 mm Hg with applanation tonometry (Tonopen, Reichert Technologies, New York, USA). The UDVA was maintained at 20/20 OD and 20/25 OS. The right pupil did not respond to either 1% tropicamide or to 2% pilocarpine eye drops (figure 5C), as tested 1 week apart, while the left pupil dilated and constricted, respectively, when the drops were applied. There was no accommodative response in the right pupil. The only relevant retrospective history from the interim period between the visits was the occurrence of off and on headaches. We attributed this symptom to possible

Avadhani K, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-210977

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Figure 1 Corneal topography at the pre-laser-assisted in situ keratomileusis assessment of the right eye.

transient increase in the IOP and started the patient on timolol eye drops 0.5% two times per day. The aim was to reduce undiagnosed spikes in IOP.

DIFFERENTIAL DIAGNOSIS A fixed and dilated pupil can be due to multiple causes: high IOP, posterior synechiae, Adie’s pupil, pharmacological dilation, systemic anticholinergics or traumatic mydriasis. There was no history of recent trauma, systemic medications or use of dilating drops. The IOP in a post-LASIK eye can be a false low, including when standard applanation tonometry with a prism diameter of 3.06 mm is performed. A Tonopen is very useful in this scenario. Multiple measurements with a Tonopen should be performed to confirm the IOP. In our case, the Tonopen IOP was

normal. The pupil did not react to pilocarpine or tropicamide, ruling out Adie’s pupil and pharmacological effects, respectively. The history of a recent surgery, intermittent headaches in the postoperative period, and signs of fixed non-reactive pupil led to the clinical diagnosis in this case to be Urrets-Zavalia syndrome.

TREATMENT The patient was asked to continue the timolol 0.5% eye drops and to be on regular follow-up.

OUTCOME AND FOLLOW-UP At 3 months’ follow-up, the patient’s vision was maintained at 20/20. The flap was well apposed. The glare became lesser

Figure 2 Corneal topography at the pre-laser-assisted in situ keratomileusis assessment of the left eye. 2



Figure 3 Automated pupillometry readings for the operated eye (OD). (A) Pre-laser-assisted in situ keratomileusis (LASIK), (B) 1 month post-LASIK and (C) 3 months post-LASIK.

subjectively, possibly due to early neuroadaptation, and the patient was comfortable. The pupil was still fixed, there (figure 5D) was a mild improvement in the pupillary reaction. The flaps were well apposed (figure 6B). The pupil diameter only reduced slightly to 4.69 mm for scotopic, 4.66 mm for mesopic and 4.63 mm for photopic illumination for the right eye (figure 3C). The left eye’s pupillary response to illumination was unaffected (figure 4C).

DISCUSSION Urrets-Zavalia syndrome, which encompasses a spectrum of clinical features from an isolated dilated pupil to additional posterior synechiae, iris atrophy and secondary glaucoma, etc, following PKP for keratoconus, was first reported by Alberto Urrets-Zavalia.5

Since then, many hypotheses have been proposed for the possible cause and predisposing factors of this condition. The most widely accepted explanation is ischaemic atrophy of the sphincter muscle with resultant pupillary dilation. Iris ischaemia may itself be the result of the postoperative rise in IOP, surgical trauma to the iris or even the result of toxins, in cases where the dilated pupil follows toxic anterior segment syndrome.6 We observed a fixed dilated pupil in a patient, following uneventful LASIK surgery. In the absence of posterior synechiae, iris atrophy and other signs seen in full blown cases of Urrets-Zavalia syndrome, we believe that our patient belonged to the lower end of the spectrum of this syndrome. We propose that a transient rise in IOP in the postoperative period causing iris ischaemia and subsequent iris atrophy was the possible cause of the condition. Although there have been no documented

Figure 4 Automated pupillometry readings for the unoperated eye (OS). (A) During pre-laser-assisted in situ keratomileusis (LASIK) testing, (B) at 1 month post-fellow eye LASIK and (C) at 3 months post-fellow eye LASIK. Avadhani K, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-210977

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Figure 5 Clinical photograph showing the right (A) and left (B) eyes at the presentation after 1 month. (C) Right eye after using 2% pilocarpine eye drops. (D) Right eye pupil at 3 month’s follow-up. raised IOP measurements during the postoperative period, we believe that the patient’s symptom of on and off headaches is an indicator of the transient increase in IOP. These episodes may have then triggered iris ischaemia. The patient subsequently developed a fixed dilated pupil that caused a glare. A fixed dilated pupil does not directly affect visual acuity; it only causes glare, haloes, etc, which can be disturbing in their own right. A known complication following intraocular (anterior segment) surgeries, this condition has never before been reported following LASIK. It may thus be wise to keep in mind the possibility of a fixed dilated pupil occurring even in cases of LASIK. Since IOP, a possible treatable and preventable entity, is believed to be the cause of the fixed dilated pupil, it is

important for ophthalmologists to be aware of this and treat any fluctuations in IOP so as to prevent Urrets-Zavalia syndrome.

Learning points ▸ A fixed dilated pupil may occur following laser-assisted in situ keratomileusis. ▸ Fluctuations in intraocular pressure leading to iris ischaemia may be the most probable cause for this occurrence. ▸ It is thus important to treat any fluctuations in intraocular pressure in the immediate postoperative period in order to prevent this complication. ▸ Vigilant follow-up is needed in the postoperative period to rule out this change. Contributors GP, KA and DS were involved in concept. KA, GP, AS and DS were responsible for follow-up and critical review. KA, GP and AS participated in literature search and writing of the manuscript. Competing interests None declared. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.

REFERENCES 1 2 3 4

Figure 6 Anterior segment optical coherence tomography scans of the right eye. (A) At 1 month post-laser-assisted in situ keratomileusis (LASIK), note the well-apposed flap and lack of interface fluid. (B) At 3 months follow-up showing normal, attached post-LASIK flap.

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Bertelsen TI, Seim V. The cause of irreversible mydriasis following keratoplasty in keratoconus: a preliminary report. Ophthalmic Surg 1974;5:56–8. Golnik KC, Hund PW, Apple DJ. Atonic pupil after cataract surgery. J Cataract Refract Surg 1995;21:170–5. Jain R, Assi A, Murdoch IE. Urrets-Zavalia syndrome following trabeculectomy. Br J Ophthalmol 2000;84:338–9. Bozkurt KT, Acar BE, Acar S. Fixed dilated pupil as a common complication of deep anterior lamellar keratoplasty complicated with Descemet membrane perforation. Eur J Ophthalmol 2013;23:164–70. Urrets Zavalia A. Fixed, dilated pupil, iris atrophy and secondary glaucoma. Am J Ophthalmol 1963;56:257–65. Spierer O, Lazar M. Urrets-Zavalia syndrome (fixed and dilated pupil following penetrating keratoplasty for keratoconus) and its variants. Surv Ophthalmol 2014;59:304–10.


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