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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 95-97

Bilateral sequential manual small incision cataract surgery as the management of phacomorphic glaucoma in a rare case of Werner's syndrome


1 Department of Glaucoma, Sadguru Netra Chikitsalay and Post Graduate Institute of Ophthalmology, Shri Sadguru Seva Sangh Trust, Jankikund, District Satna, Chitrakoot, Madhya Pradesh, India
2 Director and Trustee, Quality Assurance, Sadguru Netra Chikitsalaya, Shri Sadguru Seva Sangh Trust, Jankikund, District Satna, Chitrakoot, Madhya Pradesh, India

Date of Submission22-Feb-2021
Date of Acceptance30-Jun-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Rakesh Shakya
Department of Glaucoma, Sadguru Netra Chikitsalay and Post Graduate Institute of Ophthalmology, Chitrakoot - 210 204, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_450_21

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  Abstract 


We present a case of Werner's syndrome (WS), which is a rare progressive hereditary disorder characterized by accelerated aging. Bilateral cataracts are one of the cardinal signs of WS. The clinical characteristics of our patient met the diagnostic criteria of “Probable WS” according to the International Registry of Werner Syndrome. Our patient presented with bilateral phacomorphic glaucoma. Previous reports involving single case of WS, cataract surgery was performed by clear corneal phacoemulsification. Our patient underwent immediate bilateral sequential manual small incision cataract surgery (IBSCS) with intraocular lens implantation. We highlight the fact that bilateral cataract surgery with Manual small incision cataract surgery (M-SICS) for phacomorphic glaucoma is a safe choice for patients with Werner's syndrome.

Keywords: M-SICS, phacomorphic glaucoma, Werner's syndrome


How to cite this article:
Shakya R, Ahluwalia NS, Paes R, Parikh DV, Jain B K. Bilateral sequential manual small incision cataract surgery as the management of phacomorphic glaucoma in a rare case of Werner's syndrome. Indian J Ophthalmol Case Rep 2022;2:95-7

How to cite this URL:
Shakya R, Ahluwalia NS, Paes R, Parikh DV, Jain B K. Bilateral sequential manual small incision cataract surgery as the management of phacomorphic glaucoma in a rare case of Werner's syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Dec 3];2:95-7. Available from: https://www.ijoreports.in/text.asp?2022/2/1/95/334951



Werner's syndrome (WS) is a rare progressive genetic disorder. Unusually accelerated aging (progeria) characterizes WS, which is typically recognized by the third or fourth decades of life.[1] Bilateral cataracts are one of the cardinal signs of WS.[2] We report the successful management of a WS patient who presented with bilateral phacomorphic glaucoma.


  Case Report Top


A 33-year-old patient presented with complaints of diminution of vision in both eyes for the past 15 days that was insidious in onset and painful in nature. The patient had associated mental retardation. The patient's height was 143 cm, and he weighed 30 kg. He had an “old man look,” with a bird-like face [Figure 1]a, pinched nose, alopecia; graying of beard; thin extremities with atrophied, hyperpigmented skin; decreased subcutaneous fat; and flat feet. He also had a high-pitched voice with a loss of dentition [Figure 1]b and [Figure 1]c. The patient had no family members with similar features and no history of consanguinity. Our patient met three cardinal and two additional signs from the diagnostic criteria suggested by International Registry of Werner Syndrome [Table 1]. Hence, we diagnosed this patient as “Probable WS.”
Figure 1: (a) The bird like face, pinched nose, greying of eyebrows, moustache and beard, alopecia. (b) And (c) Showing loss of teeth except one pre-molar of mandible

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Table 1: Adapted from The International Registry of WS (www.wernersyndrome.org)

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On ocular examination of both the eyes, it was found that his vision had light perception with accurate projection of light in both the eyes. The anterior segment examination showed bilateral circumciliary congestion with corneal edema and a shallow anterior chamber. The pupils were fixed, mid-dilated, and sluggishly reacting to light. In addition, the right eye also showed iris atrophic patches with posterior synechiae. Both eyes had intumescent mature cataracts with no view to the fundus [Figure 2]a and [Figure 2]b. Intra ocular pressure (IOP) by Goldmann Applanation Tonometer (GAT) in the right eye was 44 mmHg and left eye was 32 mmHg. No angle structures were visible on gonioscopy with four mirror Sussman gonioprism on indentation [Figure 2]c and [Figure 2]d.
Figure 2: Pre-operative and Post-operative image (a) Right eye showing corneal haze, posterior synechiae inferiorly and mature cataract. (b) Left eye showing mature cataract, (c) Gonioscopy of right eye and (d) left eye showing closed angle (e) Right eye and (f) left eye on post op day one showing well apposed scleral tunnel wound, clear cornea and PCIOL in bag, (g) and (h) Post-operative fundus photos showing Glaucomatous changes

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On the basis of the ocular examination, a provisional diagnosis of bilateral phacomorphic glaucoma was made. The patient was admitted for treatment with a plan for cataract surgery with iol implantation once the cornea edema got resolved.

Preoperative preparation with intravenous injection of mannitol 20% w/v 100 ml was given over 45 min on admission and 1 h before surgery. Tablet acetazolamide 125 mg QID, tablet ranitidine 75 mg bis in die. Topical timolol eye drop continued BID.

The patient underwent immediate sequential bilateral cataract surgery (manual small incision cataract surgery) under general anesthesia with strict asepsis. The surgery protocols were derived from the previously published guidelines of the International Society of Bilateral Cataract Surgeons.[3] Both eyes were operated by a single experienced surgeon in the same operation room. Utmost care was taken to maintain strict asepsis intraoperatively. Each eye was treated as a separate procedure with complete segregation of the two procedures. Both the surgeon and the assistant rescrubbed after completion of surgery in one eye. A new trolley with separate fresh autoclaved instruments, high molecular weight OVD (sodium hyaluronate 1.4%), irrigation line, and balanced salt solution was used.

Postoperatively the patient was put on moxifloxacin 0.5% eye drops six times per day for 2 weeks, prednisolone 1% eye drops every 1 h for the first day and then tapering doses for 1 month, and homatropine 2% eye drops thrice a day for 1 week, and timolol maleate 0.5% in both eyes.

Visual acuity on the first post-op day was 6/60 in his right eye and 6/36 in his left eye. The scleral tunnel wounds were well opposed [Figure 2]e and [Figure 2]f. Fundus of right eye had a 0.7:1 cup–disc ratio with inferior rim thinning and left eye with a 0.5:1 cup–disc ratio with a healthy neuro-retinal rim, mild pallor was present in both eyes, and macula was normal in both eyes [Figure 2]g and [Figure 2]h. On last follow-up of 3 months, the patient had a vision of 6/36 in right eye and 6/24 in left eye. IOP was 16 mmHg in the right eye and 13 mmHg in the left eye.


  Discussion Top


WS is a rare, progressive hereditary disorder characterized by the appearance of unusual and accelerated aging.[4] In most cases, the inheritance is autosomal recessive, but sporadic cases have also been reported. Although the disorder is typically recognized by the third or fourth decades of life, certain characteristic findings are present during adolescence and early adulthood. Affected individuals have short stature and low weight relative to height. By age 25, individuals with the disorder typically experience early graying and alopecia, as well as cataract develops at a relatively younger age in these patients.[2]

It has a global incidence rate of less than 1 in 100,000 live births; only a handful of cases of WS have been reported till date. To the best of our knowledge, there is no existing literature on the safety of M-SICS in eyes with WS. We report the outcomes of M-SICS in eyes with WS presenting with phacomorphic glaucoma.

In previous case reports of WS, cataract surgery was done by clear corneal phacoemulsification and the corneal tunnel was sutured with nylon suture.[5],[6] Kusumesh et al. has reported a case of hypermature cataract successfully managed with clear corneal phacoemulsification.[6] In our patient, the presence of intumescent lens made phacoemulsification challenging considering the associated risk of rhexis run away and intraoperative endothelial damage. In debilitated patients and special situations, requiring general anesthesia, immediate bilateral sequential manual small incision cataract surgery may be the ideal procedure.[7]

In our case, M-SICS permitted a traumatic extraction of the nucleus with well apposed scleral tunnels and stable wound postoperatively, also there was no corneal edema suggestive of minimal intraoperative endothelial damage.


  Conclusion Top


We highlight the fact that immediate sequential bilateral cataract surgery with M-SICS for phacomorphic glaucoma is a safe alternative for patients with WS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Nakura J, Wijsman EM, Miki T, Kamino K, Yu CE, Oshima J, et al. Homozygosity mapping of the Werner syndrome locus (WRN). Genomics 1994;23:600-8.  Back to cited text no. 1
    
2.
Ruprecht KW. Ophthalmological aspects in patients with Werner's syndrome. Arch Gerontol Geriatr 1989;9:263-70.  Back to cited text no. 2
    
3.
International Society of Bilateral Cataract Surgeons. General Principles for Excellence in ISBCS. Available from: http://www. isbcs.org. [Last accessed on 2020 Nov 01].  Back to cited text no. 3
    
4.
Goto M, Matsuura M. Secular trends towards delayed onsets of pathologies and prolonged longevities in Japanese patients with Werner syndrome Biosci Trends 2008;2:81-7.  Back to cited text no. 4
    
5.
Kemmanu V, Nagappa S, Hegde K, Yadav NK, Shetty BK. Endothelial cell study in a case of Werner's syndrome undergoing phacoemulsification and Yettrium-Aluminum-Garnet laser capsulotomy. Indian J Ophthalmol 2012;60:570-2.  Back to cited text no. 5
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6.
Kusumesh R, Sinha BP, Ambastha A, Thakur SK. Management of cataract in Werner syndrome. Indian J Ophthalmol 2018;66:1337-9.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Ganesh S, Brar S, Sreenath R. Immediate sequential bilateral cataract surgery: A 5-year retrospective analysis of 2470 eyes from a tertiary care eye center in South India. Indian J Ophthalmol 2017;65:358-64.  Back to cited text no. 7
[PUBMED]  [Full text]  


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