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 Table of Contents    
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 3  |  Page : 198-199  

Delayed sclerotomy wound dehiscence after lensectomy and vitrectomy in Marfan syndrome


Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida, USA

Date of Web Publication20-Nov-2015

Correspondence Address:
Dr. Jayanth Sridhar
900 NW 17th Street, Miami, Florida
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-620X.169893

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   Abstract 

Marfan syndrome (MFS) is associated with abnormal fibrillin development that can cause morbidity and mortality. A case of acute onset hypotony due to sclerotomy wound dehiscence 13 years after 20-gauge pars plana vitrectomy and lensectomy is reported in a patient with MFS. Slit lamp examination revealed a leaking sclerotomy wound and intraoperatively the source was noted to be the prior sclerotomy site. On postoperative follow-up, the patient's vision returned to baseline, and intraocular pressure normalized. Twenty-gauge sclerotomy wound dehiscence may occur years after surgery, especially in patients with abnormal collagen as in MFS.

Keywords: Hypotony, Marfan syndrome, vitrectomy


How to cite this article:
Sridhar J, Chang JS, Aziz HA, Erickson BP. Delayed sclerotomy wound dehiscence after lensectomy and vitrectomy in Marfan syndrome. Oman J Ophthalmol 2015;8:198-9

How to cite this URL:
Sridhar J, Chang JS, Aziz HA, Erickson BP. Delayed sclerotomy wound dehiscence after lensectomy and vitrectomy in Marfan syndrome. Oman J Ophthalmol [serial online] 2015 [cited 2020 Aug 15];8:198-9. Available from: http://www.ojoonline.org/text.asp?2015/8/3/198/169893


   Introduction Top


Marfan syndrome (MFS) is a primarily autosomal dominant disorder characterized by skeletal abnormalities, cardiovascular anomalies, and ocular findings, including ectopia lentis.[1] Lens subluxation frequently requires surgical intervention, which may be accomplished by either an anterior or posterior approach to lensectomy, with or without placement of an intraocular lens.[2] Recognized postsurgical complications include retinal detachment, glaucoma, vitreous hemorrhage, and lens implant decentration. Here, we describe a patient with MFS presenting 13 years after 20-gauge pars plana vitrectomy and lensectomy with hypotony due to sclerotomy wound dehiscence, a rare postoperative occurrence.


   Case Report Top


A 19-year-old woman with MFS presented with new onset eye pain the same morning after rubbing her right eye. She had a medical history of aortic valve surgery with a mechanical valve, scoliosis rendering her wheelchair bound, and high palate [Figure 1]a. Her past ocular history included pars plana vitrectomy and lensectomy in the right eye 13 years prior and multiple retinal detachments in the left eye with eventual enucleation for a blind painful eye. Her last documented visual acuity 1-year prior in the right eye was 20/40 with aphakic correction.
Figure 1: Nineteen-year-old woman with Marfan syndrome and hypotony, right eye. (a) High palate typical of Marfan syndrome. (b) Seidel positive area of conjunctiva superotemporal to cornea, right eye. (c) Local peritomy reveals dehisced scleral wound with active leakage, right eye

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On initial examination, visual acuity was 20/400 in the right eye with a nonmeasurable intraocular pressure and the globe soft to palpation. Slit lamp examination was notable for a Seidel positive area in the superotemporal quadrant [Figure 1]b. Gentle B-scan ultrasonography through the lid revealed a shallow choroidal detachment and the patient was diagnosed with a presumed wound leak from an old sclerotomy wound.

The patient was taken to the operating room where a superotemporal peritomy was opened, revealing an actively leaking dehisced scleral wound in the pars plana [Figure 1]c. The scleral wound was closed with a 9–0 nylon suture and the overlying conjunctiva was closed.

Two months after wound closure, the patient's visual acuity had improved to 20/30 with an intraocular pressure of 13 mm Hg, resolution of the choroidal detachment, and no evidence of scleral wound leakage.


   Discussion Top


Ectopia lentis has been reported to occur in 60–80% of patients with MFS.[3] Indications for surgical intervention include poor visual acuity, significant anisometropia, lens-induced glaucoma or uveitis, and complete dislocation.[4] Although a pars plana approach is more commonly described, anterior lensectomy with limited anterior vitrectomy has been shown to have similar outcomes.[2],[5] There is evidence both supporting and arguing against the placement of an intraocular lens implant.[5],[6]

Well-described complications of pars plana lensectomy in MFS include retinal detachment, vitreous hemorrhage, lens implant decentration, and glaucoma.[7] One similar case was noted in the literature, where Mancino et al. described a 34-year-old woman in Italy with MFS, who developed chronic hypotony 20 years after 20-gauge pars plana lensectomy.[8] However, that patient required surgical intervention with a scleral graft and autologous conjunctival epithelial transplant whereas our patient was able to have the sclerotomy closed with nylon suture.

MFS is a connective tissue disorder secondary to mutations in the FBN1 gene encoding fibrillin, resulting in poor tensile strength in the involved tissue.[1] Fibrillin has been noted on histopathological examination to be present in the normal sclera.[9] This possibly explains the poor wound healing and eventual dehiscence observed in both the previous case and our case. Similarities between the previous case and our case also include both patients with MFS undergoing lensectomy as children (14-year-old and 9-year-old, respectively). Patients with MFS typically undergo progressive axial elongation of the globe throughout childhood, which may alter the healing characteristics of well-approximated scleral wounds over time.[10]


   Conclusions Top


Delayed 20-gauge sclerotomy wound dehiscence appears to be an extremely rare postoperative risk of lensectomy patients with MFS. Clinicians should maintain this entity on the differential when assessing the MFS patient with hypotony.

Acknowledgments

The authors have no proprietary or financial interest in any of the work discussed in this manuscript. There are no disclosures for any author. This research is funded in part by NIH Center Core Grant P30EY014801, Research to Prevent Blindness Unrestricted Grant, Department of Defense (DOD-Grant#W81XWH-09-1-0675).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-85.  Back to cited text no. 1
    
2.
Miraldi Utz V, Coussa RG, Traboulsi EI. Surgical management of lens subluxation in Marfan syndrome. J AAPOS 2014;18:140-6.  Back to cited text no. 2
    
3.
Neely DE, Plager DA. Management of ectopia lentis in children. J Pediatr Ophthalmol Strabismus 2004;41:289-94.  Back to cited text no. 3
    
4.
Shortt AJ, Lanigan B, O'Keefe M. Pars plana lensectomy for the management of ectopia lentis in children. J Pediatr Ophthalmol Strabismus 2004;41:289-94.  Back to cited text no. 4
    
5.
Plager DA, Yang S, Neely D, Sprunger D, Sondhi N. Complications in the first year following cataract surgery with and without IOL in infants and older children. J AAPOS 2002;6:9-14.  Back to cited text no. 5
    
6.
Morrison D, Sternberg P, Donahue S. Anterior chamber intraocular lens (ACIOL) placement after pars plana lensectomy in pediatric Marfan syndrome. J AAPOS 2005;9:240-2.  Back to cited text no. 6
    
7.
Fan F, Luo Y, Liu X, Lu Y, Zheng T. Risk factors for postoperative complications in lensectomy-vitrectomy with or without intraocular lens placement in ectopia lentis associated with Marfan syndrome. Br J Ophthalmol 2014;98:1338-42.  Back to cited text no. 7
    
8.
Mancino R, Aiello F, Ceccarelli S, Marchese C, Varesi C, Nucci C, et al. Autologous conjunctival epithelium transplantation and scleral patch graft for postlensectomy wound leakage in Marfan syndrome. Eur J Ophthalmol 2012;22:830-3.  Back to cited text no. 8
    
9.
Wheatley HM, Traboulsi EI, Flowers BE, Maumenee IH, Azar D, Pyeritz RE, et al. Immunohistochemical localization of fibrillin in human ocular tissues. Relevance to the Marfan syndrome. Arch Ophthalmol 1995;113:103-9.  Back to cited text no. 9
    
10.
Konradsen TR, Zetterström C. A descriptive study of ocular characteristics in Marfan syndrome. Acta Ophthalmol 2013;91:751-5.  Back to cited text no. 10
    


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