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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
Source publication
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 intraoperative...
Contexts in source publication
Context 1
... 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. ...
Context 2
... 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. ...
Citations
... If a wound leak is still detected at the end of surgery, absorbable sutures can be placed, especially in the setting of leaking silicone oil. Leakage from sclerotomies is more likely in highly myopic eyes with low scleral rigidity, in eyes with scarred conjunctiva or sclera from previous surgery, in Marfan's syndrome [36], and in young children [19]. ...
Purpose. To summarise the surgical advances and evolution of small gauge vitrectomy and discuss its principles and application in modern vitreoretinal surgery. The advent of microincisional vitrectomy systems (MIVS) has created a paradigm shift away from twenty-gauge vitrectomy systems, which have been the gold standard in the surgical management of vitreoretinal diseases for over thirty years. Advances in biomedical engineering and surgical techniques have overcome the technical hurdles of shifting to smaller gauge instrumentation and sutureless surgery, improving surgical capabilities and expanding the indications for MIVS.
... Common pathophysiologic mechanisms include trauma, suture malfunction, scleral melt, obesity, old age and prolonged steroid use [1,11]. Delayed wound dehiscence can occur due to abnormal collagen structure in systemic disorders, such as in Marfan syndrome [12] and diabetes [13]. A number of growth factors and their associated receptors, including epidermal growth factor, transforming growth factor-β, keratinocyte growth factor, hepatocyte growth factor, fibroblast growth factor and platelet-derived growth factor, regulate functions including mitosis, differentiation, motility and apoptosis, which play a vital role in corneal wound healing, mediating the proliferation of epithelial and stromal tissue and affecting the remodelling of the extracellular matrix [13]. ...
Purpose
To report a case of inadvertent bleb formation presenting 18 months after pars plana M3 Molteno implant tube obstruction in a patient with mixed mechanism glaucoma.
Materials and Methods
An 84-year-old Caucasian male with mixed mechanism glaucoma underwent slit-lamp examination, gonioscopy, colour anterior segment photography and anterior segment optical coherence tomography (AS-OCT).
Results
An inadvertent bleb developed 18 months after pars plana implant tube re-positioning with a 6/0 Vicryl tie ligature. The bleb was located in the area anterior to the implant plate; it was characterised by a thin, transparent, avascular and multi-cystic wall, with a visible stoma at the posterior edge of the bleb. The bleb was functioning as demonstrated by an intraocular pressure of 6 mm Hg at presentation and a punctate fluorescein uptake pattern of the bleb wall. The bleb over the plate of the Molteno implant was non-functioning, likely secondary to tube obstruction by vitreous in the early postoperative period. AS-OCT showed a tract from the anterior chamber commencing at an entry wound through a corneal tunnel to the posterior stoma at the base of the inadvertent bleb.
Conclusions
We hypothesise that the pathophysiologic factors resulting in an inadvertent bleb are a result of a combination of apoptosis, late-onset wound dehiscence and internal gaping of a centrally placed corneal wound. In addition, aqueous hydrodynamic factors may play a role.
A man in his 50s presented to the eye emergency department on three separate occasions complaining of blurred vision and discomfort in the left eye. He had a history of Marfan syndrome and had undergone bilateral 20-gauge (G) pars plana vitrectomy and lensectomy 20 years prior for ectopia lentis. On examination he had epithelial corneal oedema, raised intraocular pressure >40 mm Hg and conjunctival chemosis, which later appeared as a bleb-like conjunctival elevation. Acute treatment with oral acetazolamide and topical ocular hypotensive agents produced a marked reduction in intraocular pressure to 2–4 mm Hg. A presumed diagnosis of a leaking scleral wound was made. He underwent scleral exploration under general anaesthesia and a leaking sclerotomy was uncovered. The defect was repaired successfully using a scleral patch graft. Late dehiscence of a sclerotomy has been reported rarely in patients with Marfan syndrome. This is the first reported case to present atypically with intermittent episodes of raised intraocular pressure rather than with hypotony.
