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BRIEF COMMUNICATION
Year : 2019  |  Volume : 26  |  Issue : 1  |  Page : 33-36  

Fibrin glue-assisted intraocular lens fixation in weill–Marchesani syndrome


1 Department of Cataract, Glaucoma, and Anterior Segment, Raipur, Chhattisgarh, India
2 Department of Vitreo-Retiina, Glaucoma, and Anterior Segment, Raipur, Chhattisgarh, India

Date of Web Publication24-Apr-2019

Correspondence Address:
Charudutt Kalamkar
Shri Ganesh Vinayak Eye Hospital, Opposite Colours Mall, Dhamtari Road, Raipur - 492 001, Chhattisgarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/meajo.MEAJO_162_16

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   Abstract 


PURPOSE: To report visual and intraocular pressure (IOP) outcomes of 4 eyes in 2 patients with Weill Marchesani Syndrome having ocular fearures of spherophakia and secondary glaucoma who underwent fibrin glue assisted intrascleral fixation of intraocular lens (IOL).
METHODS: Detailed anterior and posterior segment evaluation assessing best corrected visual acuity (BCVA), IOP, central corneal thickness was done in all. Lensectomy, vitrectomy with glued Intrascleral fixation of 3 piece intraocular lens was done. Post operative BCVA and IOP were assessed.
RESULTS: Visual acuity and IOP control improved post-operatively.
CONCLUSIONS: Glued IOL implantation is an effective method to visually rehabilitate and control glaucoma in patients with Weill Marchesani Syndrome.

Keywords: Glued intra ocular lens, spherophakia, Weil–Marchesani syndrome


How to cite this article:
Kalamkar C, Radke NV, Mukherjee A, Radke SN. Fibrin glue-assisted intraocular lens fixation in weill–Marchesani syndrome. Middle East Afr J Ophthalmol 2019;26:33-6

How to cite this URL:
Kalamkar C, Radke NV, Mukherjee A, Radke SN. Fibrin glue-assisted intraocular lens fixation in weill–Marchesani syndrome. Middle East Afr J Ophthalmol [serial online] 2019 [cited 2019 May 21];26:33-6. Available from: http://www.meajo.org/text.asp?2019/26/1/33/256962




   Introduction Top


Weill–Marchesani syndrome (WMS) is a multisystem disorder with both autosomal dominant and recessive modes of inheritance and is characterized by short stature, brachydactyly, joint stiffness, and characteristic eye abnormalities such as microspherophakia, ectopia lentis, and glaucoma.[1] We report outcomes of four eyes with glued intraocular lens (IOL) fixation in patients with WMS. To the best of our knowledge, this is the first case report of fibrin glue-assisted intrascleral haptic fixated IOL in patients with WMS.


   Case Reports Top


Case 1 (Both eyes)

A 44-year-old male presented with gradual blurring of vision in both eyes (OU) for 1 year. On ocular examination, the patient had best-corrected visual acuity (BCVA) of finger counting 4 meter in both eyes and near vision (N) was <N36. Central corneal thickness (CCT) adjusted intraocular pressures (IOPs) were 26 mm Hg in the right eye (OD) and 24 mm Hg in the left eye (OS). CCT was OD 623 micron and OS 632 micron. Anterior segment evaluation on slit lamp revealed bilateral subluxated lens with spherophakia [Figure 1]a. Angles were closed on gonioscopy. Both eyes had 0.6 cup:disc ratio with neuroretinal rim (NRR) thinning. Macula and peripheral retinal examination was normal. He was on three antiglaucoma medications (AGMs) at the time of presentation.
Figure 1: Preoperative and postoperative clinical photographs. (a) Right eye of Case 1 with spherophakia; (b) Left eye of Case 2 with spherophakia status post trab and patent superior peripheral iridectomy; (c) Glued intraocular lens in situ in the right eye of Case 1; (d) Glued intraocular lens in situ in left eye of Case 2; (e and f) Circled inset showing intrascleral portion of haptic of the glued intraocular lens on anterior segment examination

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Case 2 (Both eyes)

A 42-year-old male presented with OU for the last 4 years. He had a history of surgery in OU 3 years back, for which documents were not available. BCVA was OD finger counting at 1 meter, <N36 and 6/60, N18 in OS. CCT adjusted IOP in OD was 28 mm Hg and 22 mm Hg in OS. CCT in OD was 618 micron and 610 micron in OS. Both eyes had superior 12 'o' clock flat blebs with patent peripheral iridectomy (PI) [Figure 1]b. Angles were closed on gonioscopy. He had bilateral subluxated cataractous lens with spherophakia [Figure 1]b. Both eyes had 0.9 cup: disc ratio with NRR thinning in all quadrants. Macula and peripheral retinal examination was normal. The patient was on four topical AGM for the past 1 year, which was advised elsewhere.

General physical examination demonstrated short stature (brachymorphy) (Case 1: 146 cm, Case 2: 138 cm), brachycephaly, brachydactyly, and stiff joints [Figure 2]a,[Figure 2]b,[Figure 2]c. On cardiology evaluation, Case 1 had pulmonary stenosis and tricuspid regurgitation. Case 2 did not have any cardiac anomalies.
Figure 2: Anthropometry of patients with Weill–Marchesani syndrome. Two brothers with spherophakia. (a) Short stature, brachycephaly; (b) Brachydactily of upper extremity with stiff joints; (c) Brachydactily involving lower extremity

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Based on these findings, diagnosis of WMS with spherophakia with subluxated lens and secondary glaucoma was made in the both cases. It was noteworthy that Case 2 had failed trabeculectomy surgery with flat blebs in OU.

Management

A written informed consent was obtained from the patients, and the study adhered to the tenets of Declaration of Helsinki. A pars plana lensectomy with vitrectomy was done. Intrascleral fixation of three-piece IOL: Acrysof® multi-piece IOL model no. MA60AC (Alcon Fortworth Texas, United States) or Aurolab three-piece rigid PMMA lens B3602 (Aurolab, Madurai, India) was done using technique described by Agarwal et al.[2] IOL power calculations were done using multiple formulas, namely, Barrett's universal formula and SRK-T formula. Limited peritomy is made 180° away from each other, and limbus-based partial thickness sclera flaps are raised similarly 180° away from each other. 21G needle is used to make pars plicata sclerotomies under the sclera flaps. After vitrectomy through these sclerotomies, the three-piece IOL is inserted through a clear corneal incision or scleral tunnel, and the haptics are externalized using microsurgical technology forceps. The externalized haptics are tucked into a scleral tunnel (Scharioth's tunnel) fashioned with a 26G needle at the edge of the sclera flap running parallel to the limbus. The sclera flaps are apposed with the help of fibrin glue after drying the sclera bed. The conjunctival flaps are similarly apposed in the end over the scleral flaps.

Surgical PI was performed in Case 1. In Case 2, surgical wound construction was done to avoid the previous superior trabeculectomy site. The second eye of both the patients was operated 2 weeks after the first eye in a similar manner.

Postoperative 3-month follow-up

CASE 1 – BCVA was 6/12, N6 OU. IOP was 16 mm Hg in OU with one AGM [Table 1].
Table 1: Visual and intraocular pressure outcomes

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Glued IOLs were well-centered and stable with well-positioned intrascleral haptics [Figure 1]c, [Figure 1]e, and [Figure 1]f. Posterior segment evaluation was stable without any new changes.

CASE 2 – BCVA in OD was 6/24, N8 and that in OS was 6/18, N8. IOP was 14 and 12 mm of Hg in OD and in OS, respectively, with two AGMs [Table 1]. Glued IOLs were well-centered and stable [Figure 1]d.


   Discussion Top


We searched PubMed using the keywords WMS and Glued IOL. To the best of our knowledge these are the first reported cases of fibrin glue assisted intra-scleral haptic fixated IOLs in patients with WMS. Similar to published literature, in our cases, systemic features included short stature, stiff joints, brachy-cephaly and brachy-dactyly. WMS is occasionally associated with cardiac anomalies like mitral valve prolapse, aortic and pulmonary valve stenosis, ventricular septal defects, and QTc prolongation.[1],[3] One of our cases had Tricuspid regurgitation and pulmonary stenosis while the other had normal cardiac examination findings.

Spherophakia is usually associated with anterior subluxation or dislocation of the spherical lens, leading to pupillary block glaucoma. Long-standing shallowing of anterior chamber also leads to permanent synechial angle closure. Both these mechanisms jointly contribute to the elevated IOP.

In our cases, angles were closed due to long-standing nature of the disease. Surgical removal of lenses helped in lowering of IOP to normal ranges, suggesting the dual mechanism of glaucoma. Similar to our cases, increased CCT has been published in literature, highlighting the importance of this parameter in measuring accurate IOP.[4],[5]

Trabeculectomy alone without removal of the spherophakic lens may not lead to long-term IOP reduction. Senthil et al. reported a success rate of 61% with trabeculectomy in microspherophakic patients.[6] Case 2 had undergone trabeculectomy in both eyes, but IOP was above the desired target. This case highlights the importance of step-by-step management of glaucoma in WMS, first step being the removal of lens. If IOP does not fall to target levels despite the use of AGMs, trabeculectomy should be performed. In our patients, after removal of lenses, AGMs achieved target IOP. Literature similarly advocates lensectomy as the primary modality of treatment.[7],[8],[9] It would be beneficial to do a prophylactic laser iridotomy before the development of permanent angle closure in such patients.

The advantages of glued IOL over anterior chamber IOL are the more physiological location, reduced iris tissue irritation, reduced risk of glaucoma, and less chances of corneal endothelial touch and decompensation. The advantages of glued IOL over scleral-fixated IOL are the elimination of suture-related complications such as erosion or breakage, reduced risk of IOL dislocation, tilting, or decentration. Fixing haptics in intrascleral tunnels eliminates the need of special IOLs with eyelets in haptics as any three-piece IOL can be used for this surgery. It has been shown to be safe even in pediatric patients.[10] All four eyes of our cases had stable well-centered glued IOLs with no complications. Short-term follow-up of 3 months is a limitation of the study, but the initial results are promising.

Treatment of WMS associated spherophakia with ectopia lentis, and secondary glaucoma is aimed at removing the underlying cause by lens removal. Lensectomy leads to control of IOP in majority of cases. Glued IOL is a good modality for these cases with minimal risk of complications. Screening of family members and importance of early treatment should be emphasized.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Faivre L, Dollfus H, Lyonnet S, Alembik Y, Mégarbané A, Samples J, et al. Clinical homogeneity and genetic heterogeneity in Weill-Marchesani syndrome. Am J Med Genet A 2003;123A: 204-7.  Back to cited text no. 1
    
2.
Agarwal A, Kumar DA, Jacob S, Baid C, Agarwal A, Srinivasan S, et al. Fibrin glue-assisted sutureless posterior chamber intraocular lens implantation in eyes with deficient posterior capsules. J Cataract Refract Surg 2008;34:1433-8.  Back to cited text no. 2
    
3.
Kojuri J, Razeghinejad MR, Aslani A. Cardiac findings in Weill-Marchesani syndrome. Am J Med Genet A 2007;143A: 2062-4.  Back to cited text no. 3
    
4.
Razeghinejad MR, Safavian H. Central corneal thickness in patients with weill-marchesani syndrome. Am J Ophthalmol 2006;142:507-8.  Back to cited text no. 4
    
5.
Roszkowska AM, Aragona P. Corneal microstructural analysis in Weill-Marchesani syndrome by in vivo confocal microscopy. Open Ophthalmol J 2011;5:48-50.  Back to cited text no. 5
    
6.
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. 6
    
7.
Harasymowycz P, Wilson R. Surgical treatment of advanced chronic angle closure glaucoma in Weill-Marchesani syndrome. J Pediatr Ophthalmol Strabismus 2004;41:295-9.  Back to cited text no. 7
    
8.
Goel N, Sharma R, Sawhney A, Mandal M, Choudhry RM. Lensectomy, vitrectomy, and transvitreal ciliary body photocoagulation as primary treatment for glaucoma in microspherophakia. J AAPOS 2015;19:366-8.  Back to cited text no. 8
    
9.
Guo H, Wu X, Cai K, Qiao Z. Weill-Marchesani syndrome with advanced glaucoma and corneal endothelial dysfunction: A case report and literature review. BMC Ophthalmol 2015;15:3.  Back to cited text no. 9
    
10.
Kumar DA, Agarwal A, Prakash D, Prakash G, Jacob S, Agarwal A, et al. Glued intrascleral fixation of posterior chamber intraocular lens in children. Am J Ophthalmol 2012;153:594-601, 601.e1-2.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]



 

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