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Year : 2022  |  Volume : 4  |  Issue : 1  |  Page : 25

Late presentation of isolated spherophakia in an elderly woman with bilateral angle closure and cataract managed by lens extraction and retropupillary iris-claw lens implantation

Cataract and Glaucoma Services, Sankara Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission25-Jan-2022
Date of Decision13-Mar-2022
Date of Acceptance14-Mar-2022
Date of Web Publication19-May-2022

Correspondence Address:
Mithun Thulasidas
Cataract and Glaucoma Services, Sankara Eye Hospital, Sathy Road, Sivanandapuram, Coimbatore - 641 035, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/pajo.pajo_6_22

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Spherophakia is usually associated with systemic disorders but may also present as an isolated entity. Spherophakia patients often present in adolescence or before 50 years of age. Here, we describe a case of isolated spherophakia in a 61-year-old woman with bilateral angle closure and cataract managed by lens extraction and retropupillary iris-claw lens implantation.

Keywords: Angle-closure, iris-claw, microspherophakia, retropupillary iris-claw, spherophakia

How to cite this article:
Sasidharan A, Thulasidas M. Late presentation of isolated spherophakia in an elderly woman with bilateral angle closure and cataract managed by lens extraction and retropupillary iris-claw lens implantation. Pan Am J Ophthalmol 2022;4:25

How to cite this URL:
Sasidharan A, Thulasidas M. Late presentation of isolated spherophakia in an elderly woman with bilateral angle closure and cataract managed by lens extraction and retropupillary iris-claw lens implantation. Pan Am J Ophthalmol [serial online] 2022 [cited 2023 Sep 21];4:25. Available from: https://www.thepajo.org/text.asp?2022/4/1/25/345497

  Introduction Top

Spherophakia is a rare congenital condition of the eye in which the crystalline lens is more spherical in shape, with an increased anteroposterior diameter and decreased equatorial diameter.[1] The condition may be isolated, familial, or associated with systemic disorders. Usually, spherophakia patients present in adolescence or early adulthood and those with systemic associations may present earlier.[2] We present a case of isolated spherophakia in a 61-year-old woman with bilateral angle closure and visually significant cataract.

  Case Report Top

A 61-year-old woman presented with decreased vision in both eyes (BE) for 3 months. She had no history of systemic illness and was not on any medications. On examination, the corrected distance visual acuity (CDVA) was 20/200 in the right eye (RE) and 20/100 in the left eye (LE). Retinoscopy was not possible due to the dull fundal glow. The intraocular pressure (IOP) was 40 mmHg and 25 mmHg on the Goldmann applanation tonometer in RE and LE, respectively. The anterior segment examination revealed clear corneas, shallow anterior chamber, round reacting pupils, and grade 2 nuclear sclerosis cataract in BE. Gonioscopy showed appositional angle closure with no peripheral anterior synechiae (PAS) in BE. Undilated fundus examination showed a cup-disc ratio of 0.4 with a healthy neuroretinal rim and normal macula in BE. The patient was given syrup glycerol 30 ml followed by systemic acetazolamide 250 mg. Laser peripheral iridotomy (LPI) was performed in BE for relieving the pupillary block. One hour later, the IOP came down to 18 mmHg and 13 mmHg in RE and LE, respectively. At 1 week follow-up, the IOP was 22 mmHg in RE and 21 mmHg in LE. The LPI was patent in BE. On dilated examination, the cataractous lens was found to be small and spherical and subluxated anteriorly into the pupillary plane, suggestive of microspherophakia [Figure 1]. The axial length was 22.08 mm and 21.90 mm in RE and LE, respectively. A complete systemic examination of the patient revealed no abnormalities regarding systemic conditions such as Weill–Marchesani's syndrome, Marfan's syndrome, and homocysteinemia. A diagnosis of isolated spherophakia was made.
Figure 1: Slit-lamp photograph showing spherical cataractous lens subluxated anteriorly into the pupillary plane in the (a) right eye and (b) left eye, suggestive of spherophakia

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The patient underwent phacoemulsification with limited anterior vitrectomy and retropupillary iris-claw lens implantation (+22.50 diopters, Excelens, Excel Optics Pvt. Ltd., Chennai, India) in BE [Figure 2]. At 6 weeks follow-up, the CDVA was 20/20 in BE with an IOP of 18 mmHg in RE and 16 mmHg in LE. Gonioscopy revealed open angles in BE with no PAS.
Figure 2: Slit-lamp photograph showing retropupillary iris-claw intraocular lens implanted in the (a) right eye and (b) left eye

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  Discussion Top

Spherophakia is usually associated with systemic disorders such as Weill–Marchesani's' syndrome, homocysteinemia, Marfan's syndrome, Alport's syndrome, and Klinefelter's syndrome.[2] In our case, the patient did not have any findings suggestive of systemic association. Bilateral angle closure in isolated spherophakia is uncommon.[3],[4] It can occur due to different mechanisms such as pupillary block by the spherical lens, ciliary body irritation by the dislocated lens, or complete subluxation of the lens in the anterior chamber.[3],[4] Untreated pupillary block may lead to PAS formation and irreversible trabecular damage.[3]

Spherophakia patients often present before 50 years of age.[2] In the present case, spherophakia was not suspected initially given the age of the patient (>60 years) and lack of any significant past history. Spherophakia was diagnosed only after the dilated examination post-LPI. To the best of our knowledge, this is the first reported case of spherophakia with a late presentation in an elderly woman.

There are no universally accepted guidelines for managing secondary angle-closure glaucoma in spherophakia. Some authors suggest LPI ± glaucoma medications and further trabeculectomy only if IOP is not controlled. Lens extraction was considered only in the case of lens subluxation or persistent shallow anterior chamber following trabeculectomy.[5] Another school of thought suggested that the crystalline lens, the pathological cause for glaucoma in spherophakia, should be removed initially.[6] However, in our case, the patient presented with both secondary angle closure and visually significant cataract. LPI was initially performed to relieve the pupillary block and control the IOP. The presence of cataract with anterior subluxation warranted an immediate lens extraction.

The choice of the IOL depends mainly on the surgeon and patient factors. Angle-supported anterior chamber lenses (ACIOL) and iris-claw lenses (prepupillary and retropupillary) are commonly used options. Posterior chamber IOL (PCIOL) ± capsular tension ring/segments and scleral-fixated IOL (SFIOL) have also been described.[2] ACIOLs have been reported to be associated with corneal endothelial cell loss, PAS formation, and glaucoma due to excess pressure caused on the iris root.[7] A properly sized ACIOL when planned rather than implanted in a complicated case has better outcomes.[2] PCIOL implantation is debatable as the zonules are developmentally weak, and the possibility of the bag lens complex falling into the vitreous cannot be ruled out.[8] The use of a capsular tension ring/segment has been described to strengthen the bag and reduce the risk of IOL dislocation.[2] SFIOL implantation has significant advantages such as the more biological location in the eye closer to the plane of the crystalline lens away from the cornea.[9] However, the technique needs more surgical expertise, and complications may occur due to transscleral sutures, such as suture erosion and haptic extrusion.[2] Retropupillary iris-claw lens implantation in eyes with inadequate zonular support is an effective procedure with fewer complications. The shift in the placement of iris-claw lens from prepupillary to a more physiological retropupillary position and change in the optic design from biconvex to convex-concave have given better functional outcomes and improved safety. In our case, a retropupillary iris-claw lens (+22.50 diopters, Excelens, Excel Optics Pvt. Ltd., Chennai, India) made of polymethyl methacrylate with an optic diameter of 8 mm and A-constant of 117.2 (Sanders–Retzlaff–Kraff/Theoretical formula) was implanted in BE. One of the major complications associated with iris-claw lens is the disenclavation of the lens, which is found to occur due to incorrect primary haptic enclavation or atrophic iris at the site of enclavation, and can be easily re-enclavated with proper techniques.[10] However, in the present case, no such complication associated with the iris-claw lens was encountered. The CDVA was 20/20 in BE, and the IOP was well-controlled at 6 weeks follow-up.

  Conclusion Top

Spherophakia should be included in the differential diagnosis of bilateral angle closure, even in the elderly age group. Retropupillary iris-claw lens is an effective and safe option in such cases when put under experienced surgical hands. However, a longer follow-up is mandatory to monitor the IOP.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Macken PL, Pavlin CJ, Tuli R, Trope GE. Ultrasound biomicroscopic features of spherophakia. Aust N Z J Ophthalmol 1995;23:217-20.  Back to cited text no. 1
Khokhar S, Pillay G, Sen S, Agarwal E. Clinical spectrum and surgical outcomes in spherophakia: A prospective interventional study. Eye (Lond) 2018;32:527-36.  Back to cited text no. 2
Johnson GJ, Bosanquet RC. Spherophakia in a New-Foundland family: 8 years experience. Can J Ophthalmol 1983;18:159-164.  Back to cited text no. 3
Johnson VP, Grayson M, Christian JC. Dominant microspherophakia. Arch Ophthalmol 1971;85:534-7.  Back to cited text no. 4
Senthil S, Rao HL, Hoang NT, Jonnadula GB, Addepalli UK, Mandal AK, et al. Glaucoma in microspherophakia: Presenting features and treatment outcomes. J Glaucoma 2014;23:262-7.  Back to cited text no. 5
Yang J, Fan Q, Chen J, Wang A, Cai L, Sheng H, et al. The efficacy of lens removal plus IOL implantation for the treatment of spherophakia with secondary glaucoma. Br J Ophthalmol 2016;100:1087-92.  Back to cited text no. 6
Ellerton CR, Rattigan SM, Chapman FM, Chitkara DK, Smerdon DL. Secondary implantation of open-loop, flexible, anterior chamber intraocular lenses. J Cataract Refract Surg 1996;22:951-4.  Back to cited text no. 7
Khokhar S, Pangtey MS, Sony P, Panda A. Phacoemulsification in a case of microspherophakia. J Cataract Refract Surg 2003;29:845-7.  Back to cited text no. 8
Brunette I, Stulting RD, Rinne JR, Waring JO, Gemmil M. Penetrating keratoplasty with anterior or posterior chamber intraocular lens implantation. Arch Ophthalmol 1994;112:1311-9.  Back to cited text no. 9
Thulasidas M. Retropupillary iris-claw intraocular lenses: A literature review. Clin Ophthalmol 2021;15:2727-39.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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