Bilateral sequential pseudophakic aqueous misdirection syndrome presenting as a myopic shift in refraction

Swati Singh; Avnindra Gupta; Mahipal Singh Sachdev; Nirmala Sudhamala

doi:10.4103/pajo.pajo_107_21

CASE REPORT

Year : 2021 | Volume : 3 | Issue : 1 | Page : 35

Bilateral sequential pseudophakic aqueous misdirection syndrome presenting as a myopic shift in refraction

Swati Singh, Avnindra Gupta, Mahipal Singh Sachdev, Nirmala Sudhamala
Cataract and Glaucoma Services, Centre for Sight, New Delhi, India

Date of Submission	29-Aug-2021
Date of Acceptance	27-Sep-2021
Date of Web Publication	22-Oct-2021

Correspondence Address:
Dr. Swati Singh
B - 5/24, Safdarjung Enclave, New Delhi - 110 029
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_107_21

Abstract

A 63-year-old woman with high hyperopia developed acute myopia of − 5.50 D with axial shallowing of anterior chamber and high intraocular pressure (IOP) in her left eye after 2 weeks of phacoemulsification surgery. She developed a myopic shift after 4 weeks in the right eye also which was operated 8 months ago for cataract. Laser peripheral iridotomy was ineffective in both eyes. Cycloplegic therapy was started for presumed aqueous misdirection syndrome (AMS) with antiglaucoma drugs in both eyes. A laser capsulo-hyaloidotomy was attempted in LE with no result. Pars plana vitrectomy (PPV) with iridectomy-zonulectomy-hyloidectomy (IZH) was done in both eyes after 2 years of onset and multiple recurrences associated with atropine withdrawal. Pseudophakic AMS can present as a myopic shift in refraction without a significant rise in IOP. Cycloplegic therapy is not well tolerated by multifocal intraocular lens patients. PPV with IZH has been effective in preventing relapses in our patient for 20 months (RE) and 23 months (LE).

Keywords: Hypermetropia, myopic shift, pseudophakia, secondary glaucoma, sequential aqueous misdirection syndrome

How to cite this article:
Singh S, Gupta A, Sachdev MS, Sudhamala N. Bilateral sequential pseudophakic aqueous misdirection syndrome presenting as a myopic shift in refraction. Pan Am J Ophthalmol 2021;3:35

How to cite this URL:
Singh S, Gupta A, Sachdev MS, Sudhamala N. Bilateral sequential pseudophakic aqueous misdirection syndrome presenting as a myopic shift in refraction. Pan Am J Ophthalmol [serial online] 2021 [cited 2022 May 28];3:35. Available from: https://www.thepajo.org/text.asp?2021/3/1/35/329087

Introduction

Aqueous misdirection syndrome (AMS), described first as malignant glaucoma by von Graefe in 1869, is a rare disorder considered to be a complication of incisional eye surgery^[1],[2],[3] but reported to occur after noninvasive ocular procedures and even spontaneously.^[4] An abnormal relationship between anterior hyaloid face, ciliary body, and lens is implicated which creates a block to the forward movement of aqueous into anterior chamber and directs it into or behind the vitreous cavity pushing the lens–iris diaphragm anteriorly causing a myopic shift in refraction and secondary angle-closure glaucoma.^[2],[3] Quigley et al. proposed the choroidal expansion theory to explain the disorder.^[4] Shallow anterior chamber, short axial length, disproportionally large crystalline lens, and preexisting narrow angles or primary angle-closure glaucoma are known risk factors for AMS.^[3],[4] Pseudophakic aqueous misdirection can present as induced myopia with normal or raised intraocular pressure (IOP).^[5],[6] Contrary to the clinical presentation of typical malignant glaucoma, this variety of AMS often creates diagnostic dilemmas. We report a case of acute onset aqueous misdirection after uneventful phacoemulsification in one eye followed by similar signs and symptoms in pseudophakic fellow eye which was successfully managed by pars plana vitrectomy (PPV) and iridectomy-zonulectomy-hyloidectomy (IZH).

Patient Information and Clinical Findings

A 63-year-old hyperopic female with no significant medical history had uneventful phacoemulsification with extended depth of focus intraocular lens (IOL) implantation (ZXR00) in the right eye (target refraction-0.3 D) followed by left eye (target refraction-0.96 D) within a gap of 7 months. Best-corrected visual acuity in RE at 1 month of surgery was 6/6 with − 0.75 D and remained stable till LE surgery was done. After 2 weeks of LE phacoemulsification, her vision deteriorated to counting fingers at 3 m from 6/12 on a postoperative day 1. Examination of LE showed diffusely shallow AC and large myopic shift (−5.50 D) with raised IOP (40 mm).

Diagnostic assessment

Scheimpflug imaging and IOL master 700 elicited changes in anterior chamber dimensions. Fundus examination was within normal limits with a CD ratio of 0.3:1 in BE.

Therapeutic intervention

After starting antiglaucoma drugs, laser peripheral iridotomy (PI) was done in the left eye which proved ineffective. Suspecting aqueous misdirection, atropine sulfate 1% eyedrop was started in qid dosage which reformed the AC and improved vision in a week. Gonioscopy done at this time showed open angles with no peripheral anterior synechiae (PAS) in both eyes (BE). Atropine withdrawal after 2 weeks in LE resulted in shallow AC with myopic shift in BE [Figure 1]a, [Figure 2]a and [Figure 2]b and raised IOP (34 mm Hg) in LE. YAG laser PI was done in RE also and cycloplegic-antiglaucoma therapy was started in BE. In the next 2 years, atropine withdrawal was attempted thrice, but relapses happened in one of the eyes [Figure 3]a and [Figure 3]b. During this period, IOP remained normal in RE after withdrawal of glaucoma medication, while LE required a single glaucoma drug. Repeat gonioscopy showed PAS formation (one quadrant) in LE [Figure 1]c, but RE angles remained open [Figure 2]c. YAG laser capsulo-hyaloidotomy was tried in LE but was unsuccessful. Total PPV with surgical IZH along with a capsulotomy [Figure 1]b, [Figure 1]d and [Figure 2]d was done in both eyes within a gap of 3 months [Table 1].

Figure 1: Left eye (a) Oblique illumination showing axially shallow anterior chamber. (b) Diffuse illumination picture after pars plana vitrectomy and irido-zonulo-hyaloidectomy. (c) Gonioscopy showing peripheral anterior synechiae in inferior quadrant. (d) Slit lamp picture showing patent surgical fistula connecting the two chambers

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Figure 2: (a) Diffuse illumination of the right eye at presentation. (b) Oblique slit illumination showing axial shallowing of anterior chamber. (c) Right eye gonioscopy picture showing open angle. (d) Right eye slit-lamp picture after pars plana vitrectomy showing surgical iridectomy and capsulotomy opening

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Figure 3: (a) Right eye Scheimpflug image during a recurrence showing shallow anterior chamber before starting cycloplegic therapy. (b) Right eye Scheimpflug image showing deepened anterior chamber post cycloplegic therapy

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Table 1: Biometric details on first visit before RE surgery and changes in refraction and AC depth of both eyes during the follow-up period of three years after left cataract surgery.

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Follow-up and outcome

Both eyes maintain deep AC and stable refraction with IOP controlled on one drug in LE at 20 months (RE) and 23 months (LE) follow-up.

Discussion

The pathophysiology of AMS is still not well understood. The disease has a range of clinical presentations which may be missed if the examiner is not aware of what to look for. Our patient had biometric risk factors for the development of AMS, but no complications were noted after RE surgery. However, the LE developed acute postoperative aqueous misdirection with high IOP and a myopic shift was noted in the fellow eye also within a month.

Involvement of fellow eye in aqueous misdirection is known, but no clear explanation exists for the same. A migration of fluid to the contralateral subarachnoid space with a forward shift of vitreous and the lens is a proposed mechanism for affection of fellow eye.^[7],[8] In our case, there is a possibility that RE also had a subtle simultaneous AMS when LE was going through the acute episode which was overlooked, and probably, the administration of topical atropine in LE had some effect on RE also via systemic route. Therefore, within a fortnight of stopping atropine, the patient had a rebound event in both eyes.

Moinul et al. in their case series reported 7 eyes of 4 patients presenting with aqueous misdirection as large myopic shifts with shallow AC and normal IOP within 1 month of cataract surgery.^[6] Three patients in their study had sequential pseudophakic AMS with a gap of 1–2 weeks in the onset of symptoms in fellow eye. Laser treatment was successful in one eye, whereas the rest 6 eyes were managed with total PPV and IZH.^[6]

Pharmacotherapy is the initial line of treatment and has been reported to cure only up to 50% of cases, but many of these patients need cycloplegics for lifetime which may not be acceptable to all.^[2],[9],[10] Since our patient was initially unwilling to undergo a second surgery, the cycloplegic was continued, but the excessive glare exacerbated by the rings of EDOF IOL increased her distress over time. With the onset of synechial angle closure, an early surgery was planned after failed laser treatment. Debrouwere et al. have reported 100% success after complete vitrectomy with iridectomy-zonulectomy in their series, but their follow-up period was short (longest follow-up 228 days).^[10] In our case, surgical therapy involving complete vitrectomy and IZH was successful in breaking the cycle of aqueous misdirection by making the eye unicameral and stabilized refraction in both eyes. Our patient has successfully completed a follow-up of 600 days (RE) and 690 days (LE) without a relapse.

Informed consent

Written informed consent has been obtained from the patient for publication of case report and de-identified pictures.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgments

The authors would like to thank Mr. Arun Upadhyay, Chief Optometrist, and Mr. Rishikesh Kumar, Senior Optometrist, Centre For Sight, B - 5/24, Safdarjung Enclave, New Delhi - 110 029.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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