Retinal Detachment in Marfan Syndrome

Loewenstein, Anat; Is, Barequet; E, De Juan; Ih, Maumenee

doi:10.1097/00006982-200007000-00006

Cited by 35 publications

(9 citation statements)

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“…The exact pathogenesis of RD in MFS remains unknown, but increased AL, a previous intraocular surgery, and EL causing vitreous traction seem to be associated risk factors for RD. It still seems that this potentially sight‐threatening complication is managed better today, independently of whether the eye is phakic or aphakic (Loewenstein et al. 2000).…”

Section: Discussionmentioning

confidence: 99%

A descriptive study of ocular characteristics in Marfan syndrome

Konradsen¹,

Zetterström²

2013

Acta Ophthalmologica

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Section: Discussionmentioning

confidence: 99%

A descriptive study of ocular characteristics in Marfan syndrome

Konradsen¹,

Zetterström²

2013

Acta Ophthalmologica

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“…The same is true for retinal detachment. Here, as for lens surgery, major progress in vitreo-retinal surgery in the past decades has massively improved the prognosis [117], with cumulative reattachment rates of close to 90% [118].…”

Section: Ophthalmologic Treatment Optionsmentioning

confidence: 99%

Marfan syndrome: what internists and pediatric or adult cardiologists need to know

Kaemmerer

Oechslin²,

Seidel

et al. 2005

Expert Review of Cardiovascular Therapy

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Marfan syndrome (MFS) is one of the most frequent connective tissue disorders, showing striking pleiotropism and clinical variability. There is autosomal dominant inheritance with complete penetrance but variable expression. Approximately 25% of MFS patients have no family history of the syndrome and represent sporadic cases due to new mutations. This hazardous condition is often associated with premature cardiovascular death unless surveillance and management are optimized. The fibrillin gene (FBN1) encodes the structure of the connective tissue protein fibrillin. MFS is caused by mutations in the fibrillin gene, located on chromosome 15 at locus 15q21. Fibrillin abnormalities reduce the structural integrity of different body systems, primarily involving the heart valves, blood vessels, lungs, bones, tendons, ligaments, cartilages, eyes, skin, spinal dura and the CNS. Patients with MFS are likely to have too little fibrillin within these structures, resulting in clinically relevant problems. For example, in the aortic wall, deficient fibrillin may trigger progressive aortic ectasia and may result in aortic dissection.

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“…The risk is further increased with younger age, male sex, and myopia which are invariably associated with Marfan's syndrome. [9][10][11] The limbal or anterior route of surgery was preferable in our series of cases since it requires less instrumentation, and as already mentioned, it just required the use of a single instrument for aspiration of lens and capsular bag. Further, limited anterior vitrectomy without the insertion of a posterior infusion line can minimize the occurrence of trauma and vitreous traction to the vitreous base which can lead to retinal detachment.…”

Section: Discussionmentioning

confidence: 99%

Modified technique of endocapsular lens aspiration for severely subluxated lenses

Khokhar

Aron

Yadav

et al. 2017

Eye

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PurposeSeverely subluxated crystalline lenses pose a difficult situation to anterior segment surgeons and can only be managed surgically by removal of the lens as well as the capsular bag. Several techniques have been described in literature for the management of such cases. We describe a modified technique of endocapsular lens aspiration by the limbal route for lens extraction through small incisions on the cornea.Patients and methodsThirty-two eyes of 16 consecutive patients with severely subluxated crystalline lenses were recruited in the study. All eyes underwent a modified technique of lens aspiration within the capsular bag using a single instrument, vitrectomy cutter, and irrigation cannula, followed by sacrificing of the capsular bag. The patients were either left aphakic or implanted with an open loop anterior chamber intraocular lens (ACIOL Kelman Multiflex) and prospectively followed up for a period of 3 months.ResultsThe mean age of the patients was 9 years 3 months±3 years (range 5-15 yrs). All eyes underwent complete lens aspiration within the capsular bag with no dislocation of the lens matter. ACIOL was inserted in 22 eyes (68.7%) and 10 eyes (31.2%) were left aphakic. All the surgeries were uneventful. The mean best corrected visual acuity (BCVA) at 3 months post surgery was 0.47±0.11 logMAR which was significantly better than pre-operative BCVA (P=0.001). The percentage endothelial cell loss at 3 months was 7.1%. There was no evidence of glaucoma, corneal decompensation, or retinal detachment. The astigmatism which increased from 1.45D±086 preoperatively to 3.76D±2.02 1 week post-operatively due to sutures reduced to 1.97D±0.81 post suture removal at 3 months.ConclusionThe modified technique of endocapsular lens aspiration proves to be a simple and effective method of removal of the lens-capsular bag complex in severely subluxated lenses.

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Retinal Detachment in Marfan Syndrome

Abstract: The results of RD operations done in the past in Marfan patients were worse when the eye was aphakic. In most cases operated more recently, the prognosis for successful RD repair was good regardless of whether the eye was phakic.

Cited by 35 publications

References 0 publications

A descriptive study of ocular characteristics in Marfan syndrome

A descriptive study of ocular characteristics in Marfan syndrome

Marfan syndrome: what internists and pediatric or adult cardiologists need to know

Modified technique of endocapsular lens aspiration for severely subluxated lenses

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