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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 59  |  Issue : 3  |  Page : 295-297

Experience with manual small incision cataract surgery in the management of isolated bilateral microspherophakia


1 Department of Intraocular Lens and Cataract Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
2 Department of General Ophthalmology, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
3 Department of Vitreo-Retina Services, Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai, Tamil Nadu, India

Date of Submission04-Feb-2021
Date of Acceptance02-Jun-2021
Date of Web Publication09-Sep-2021

Correspondence Address:
Dr. Mulasthanam Sai Dheera
Aravind Eye Hospital and Post Graduate Institute of Ophthalmology, Madurai - 625 020, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_15_21

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  Abstract 


Introduction: We report a case of isolated microspherophakia,managed by an anterior segment surgeon with good visual recovery. Case Description: A 36-year-old female who presented with complaints of defective vision was diagnosed with bilateral isolated microspherophakic lens with cataractous changes. She underwent manual small incision cataract surgery (SICS) uneventful, utilizing capsular tension ring within the bag placement of lens in one eye, whereas in the other eye, an iris fixation of the intraocular lens using sutures was done. Conclusion: In cases of MSP, clear lens extraction, phacoemulsification, and pars plana lensectomy have been described for lenticular myopia and cataract. However, we reiterate the use of simpler techniques like SICS, especially when challenged by advanced cataract or a resource-poor setting. We report a good visual recovery in our patient, substantiating that such complicated cases can be handled by an anterior segment surgeon even in such a challenging context.

Keywords: Complicated cataract, microspherophakia, small incision cataract surgery


How to cite this article:
Sankarananthan R, Dheera MS, Shekhar M, Sen S. Experience with manual small incision cataract surgery in the management of isolated bilateral microspherophakia. TNOA J Ophthalmic Sci Res 2021;59:295-7

How to cite this URL:
Sankarananthan R, Dheera MS, Shekhar M, Sen S. Experience with manual small incision cataract surgery in the management of isolated bilateral microspherophakia. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Oct 20];59:295-7. Available from: https://www.tnoajosr.com/text.asp?2021/59/3/295/325722




  Introduction Top


Microspherophakia (MSP) is a rare abnormality of the eye which results from defective development of zonules, leading to formation of small spherical lens without any proper corticonuclear demarcation. MSP is usually bilateral and is associated with lens dislocation/subluxation, high myopia, and inverse glaucoma due to lens displacement into the anterior chamber.[1] It may be isolated, familial, or associated with systemic conditions such as Marfan's syndrome, Weill–Marchesani syndrome, hyperlysinemia, and congenital rubella syndrome. Surgical management of such lenses in the setting of a harder cataract proves to be a bit tedious and challenging owing to extremely weak zonules. We describe the management of such a case of MSP with bilateral cataractous lenses.


  Case Report Top


A 36-year-old female presented with complaints of gradually progressive defective vision for 3 years, which was more in the left eye (OS) than right eye (OD). She had no history of using glasses in the past. On examination, the patient had uncorrected visual acuity of 3/60 OD and 2/60 OS, which improved with 19 DS bilaterally to 6/60 in OD and to 5/60 in OS. Intraocular pressures were measured as 18 mmHg in both eyes (OU).

Investigations

Slit-lamp examination revealed iridodonesis OU. On complete pupillary dilation, OU revealed a spherical cataractous lens of nucleus sclerosis Grade 3, associated with gross phacodonesis. The edges of the lens were visible, with thin stretched-out zonules OU, however, there was no gross subluxation [Figure 1]a and [Figure 1]b. Gonioscopic examination OU showed highly pigmented angles with minimal peripheral anterior synechiae. Posterior segment examination was within normal limits OU. During optical biometry, lens thickness was found to be about 4.58 mm OD and 5.09 mm OS. The anterior chamber depths were about 3.08 mm OD and 3.02 mm OS, with axial lengths of 22.27 mm and 21.99 mm, respectively, in her OD and OS. Her systemic workup, including serology, was found to be within normal limits. Her entire family was screened for the same and was found to have no similar ocular findings.
Figure 1: (a) Slit-lamp photograph showing microspherophakic cataractous lens in the left eye. (b) Intraoperative picture showing microspherophakic lens with zonular dialysis

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Diagnosis

The patient was diagnosed with bilateral isolated MSP with lenticular myopia and nucleus sclerosis.

Treatment and outcome

Manual small incision cataract surgery was planned for the OS, in view of the harder grade of cataract in the presence of weak zonules and an elastic anterior lens capsule. After making an adequate superior sclerocorneal tunnel, a continuous curvilinear capsulorhexis was carefully made, capsule hooks were placed to stabilize the bag, and the nucleus was maneuvered out bimanually. Cortex aspiration was done tangentially, after introducing a capsular tension ring (CTR) into the lax bag to achieve distension. A rigid three-piece polymethyl methacrylate (PMMA) intraocular lens (IOL) was placed in the bag. Postoperatively, the IOL was well centered, with minimal anterior chamber inflammation [Figure 2].
Figure 2: Minimal corneal edema with inflammation on the 1st postoperative day in the left eye

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The patient underwent SICS in the other eye 15 days later, but because of rhexis extension to periphery due to lack of zonular support, CTR and IOL could not be placed in the bag. After nucleus extraction and cortical aspiration, iris fixation of IOL was performed with 9-0 prolene. Postoperatively, there was minimal corneal edema with intraocular inflammation, which subsided with medical management using topical cycloplegic and steroid.

Follow-up

At 1-month follow-up, the patient had a best-corrected visual acuity of 6/12 OU (with 1D cylindrical power) with a near vision of N10 OU (with +2.5 DS). The IOLs were stable OU [Figure 3]a and [Figure 3]b.
Figure 3: (a) Right eye showing iris fixated well-centered intraocular lens at 1-month postoperative follow-up. (b) Left eye showing well-centered intraocular lens placed in the bag at 1-month postoperative follow-up

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  Discussion Top


MSP is an autosomal recessive condition, though autosomal dominant inheritance has also been described. It occurs due to mutation in latent transforming growth factor-B binding protein-2 gene or ADAMTS17 gene. Patients with this condition usually present with gradually progressive diminution of vision (myopia or cataract), acute eye pain due to mechanical angle closure due to anterior migration of lens into anterior chamber, or occasional pupillary block. The triad of angle-closure glaucoma, refractory myopia, and shallow anterior chamber should evoke the suspicion of MSP. Although MSP may occur frequently as a part of several syndromes, isolated MSP is rare and its disease-causing mechanism remains unknown. Since 1901, isolated MSP disease was described in different ethnic groups, mostly in the Asian continent.[2] Ultrasound biomicroscopy may be useful in diagnosing difficult cases as it provides morphological details of anterior chamber, ciliary body, zonules, and lens.[3] Glaucoma is the most common complication and is found in 51% of patients, and the probability of glaucoma increases with age.[4] Laser iridotomy using neodymium: yttrium-aluminum-garnet laser has been said to have a prophylactic role in preventing acute angle-closure attacks.[5]

Therapeutic lens extraction is performed in cases of cataract, high myopia, intermittent pupillary block, and secondary intractable glaucoma. Clear lens extraction can be performed through anterior route (phacoemulsification) or through pars plana lensectomy, with or without IOL implantation.[6],[7],[8] However, management of a cataractous lens in MSP cases, especially by SICS, has rarely been documented. SICS has become a very productive alternative, especially in terms of restricted resources utilized and cost-effectiveness, to phacoemulsification in developing areas, in experienced hands. We demonstrated a substantial gain of functional vision in OUs of our patient, with successful implantation of IOL, following SICS.

The type of IOL chosen for refractive rehabilitation depends largely on the surgeon's choice and expertise. Angle-supported anterior chamber IOL and iris-fixated IOLs have been previously described in MSP with optimal outcomes.[9],[10] Placement of a posterior chamber IOL has been debated due to the presence of developmentally weak zonules and risk of bag IOL drop subsequently; however, successful PCIOL placement assisted with CTR and capsular tension segment has also been demonstrated previously.[11],[12],[13]

In summary, SICS is an effective method of surgical management of MSP cases having cataractous lenses, followed by either placement of IOL in the bag assisted with a CTR or implanting an iris-fixated IOL. A rigid PMMA IOL also helps in distending the bag and prevents future decentration and subluxation. Our case also reiterates the fact that MSP cases can be successfully handled by an anterior segment surgeon even in a setting without advanced resources, without significant complications.

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 her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Şimşek T, Beyazyıldız E, Şimşek E, Öztürk F. Isolated microspherophakia presenting with angle-closure glaucoma. Turk J Ophthalmol 2016;46:237-40.  Back to cited text no. 1
    
2.
Johnson VP, Grayson M, Christian JC. Dominant microspherophakia. Arch Ophthalmol 1971;85:534-7 passim.  Back to cited text no. 2
    
3.
Bitar MS, Farooq AV, Abbasian J. Challenges in diagnosing microspherophakia in a pediatric patient. JSM Ophthalmol 2016;4:1040.  Back to cited text no. 3
    
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. 4
    
5.
Zimmerman LE, Font RL. Congenital malformation of the eye. JAMA 1996;196:684.  Back to cited text no. 5
    
6.
Behndig A. Phacoaspiration in spherophakia with corneal touch. J Cataract Refract Surg 2002;28:189-91.  Back to cited text no. 6
    
7.
Willoughby CE, Wishart PK. Lensectomy in the management of glaucoma in spherophakia. J Cataract Refract Surg 2002;28:1061-4.  Back to cited text no. 7
    
8.
Kaushik S, Sachdev N, Pandav SS, Gupta A, Ram J. Bilateral acute angle closure glaucoma as a presentation of isolated microspherophakia in an adult: Case report. BMC Ophthalmol 2006;6:29.  Back to cited text no. 8
    
9.
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. 9
    
10.
Fouda SM, Al Aswad MA, Ibrahim BM, Bori A, Mattout HK. Retropupillary iris-claw intraocular lens for the surgical correction of aphakia in cases with microspherophakia. Indian J Ophthalmol 2016;64:884-7.  Back to cited text no. 10
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11.
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. 11
    
12.
Khokhar S, Gupta S, Kumar G, Rowe N. Capsular tension segment in a case of spherophakia. Cont Lens Anterior Eye 2012;35:230-2.  Back to cited text no. 12
    
13.
Groessl SA, Anderson CJ. Capsular tension ring in a patient with Weill-Marchesani syndrome. J Cataract Refract Surg 1998;24:1164-5.  Back to cited text no. 13
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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