Delayed sclerotomy wound dehiscence after lensectomy and vitrectomy in Marfan syndrome.

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.

Introduction

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

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 1a]. 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

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 1b]. 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 1c]. 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

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.[25] There is evidence both supporting and arguing against the placement of an intraocular lens implant.[56]

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

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.