Anterior Segment Parameters in Patients With Wilson Disease

Kara, Necip; Seyyar, Sevim Ayça; Saygılı, Oğuzhan; Seyyar, Mustafa; Gülşen, Murat Taner; Güngör, Kıvanç

doi:10.1097/ico.0000000000001422

Cited by 5 publications

(2 citation statements)

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“…In addition to KFR, the evaluation of anterior segment in WD patients showed differences with healthy population. In a study published by Kara et al, 22 patients with WD (mean age 29 years old) were compared to 22 age-and sex-matched controls [64]. WD patients had significantly higher CCT (p < 0.001), keratometric values (p = 0.011), anterior and posterior corneal elevation (p = 0.029 and p = 0.002, respectively), horizontal visible iris diameter (p = 0.001) and anterior chamber depth (p = 0.01) compared to controls.…”

Section: Discussionmentioning

confidence: 99%

Eye Involvement in Wilson’s Disease: A Review of the Literature

Chevalier

Mauget‐Faÿsse

Vasseur

et al. 2022

JCM

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Wilson’s disease (WD) is an autosomal recessive genetic disorder due to a mutation of the ATP7B gene, resulting in impaired hepatic copper excretion and accumulation in various tissues. Ocular findings are one of the hallmarks of the disease. Many ophthalmological manifestations have been described and new techniques are currently available to improve their diagnosis and to follow their evolution. We have performed a systematic PubMed search to summarize available data of the recent literature on the most frequent ophthalmological disorders associated with WD, and to discuss the newest techniques used for their detection and follow-up during treatment. In total, 49 articles were retained for this review. The most common ocular findings seen in WD patients are Kayser–Fleischer ring (KFR) and sunflower cataracts. Other ocular manifestations may involve retinal tissue, visual systems and eye mobility. Diagnosis and follow-up under decoppering treatment of these ocular findings are generally easily performed with slit-lamp examination (SLE). However, new techniques are available for the precocious detection of ocular findings due to WD and may be of great value for non-experimented ophthalmologists and non-ophthalmologists practitioners. Among those techniques, anterior segment optical coherence tomography (AS-OCT) and Scheimpflug imaging are discussed.

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Section: Discussionmentioning

confidence: 99%

Eye Involvement in Wilson’s Disease: A Review of the Literature

Chevalier

Mauget‐Faÿsse

Vasseur

et al. 2022

JCM

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“…12 Also 360-degree Scheimpflug imaging is reported to be a useful method in detecting and documenting even minute inflammatory retrocorneal deposits, which may be indicative of upcoming allograft rejection after Descemet membrane endothelial keratoplasty. 13 Anterior segment parameters were recently investigated in WD patients by Kara et al 14 They reported that WD patients had thinner CCT, higher keratometric values and shallower ACDs than normal controls. They also concluded that CCT of the WD patients with KF ring was thinner than the patients without KF ring.…”

Section: Discussionmentioning

confidence: 99%

Evaluation and grading of Kayser–Fleischer ring in Wilson disease by Scheimpflug camera

Değirmenci

Palamar

2020

European Journal of Ophthalmology

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Purpose: Evaluation the anterior segment parameters of Wilson disease patients with Kayser–Fleischer ring, the diagnostic power of Scheimpflug imaging for Kayser–Fleischer ring and suggest a scoring system. Materials and Methods: A total of 44 eyes of 22 Wilson disease patients with Kayser–Fleischer ring and 40 right eyes of 40 healthy age matched subjects were enrolled to the study. Serum ceruloplasmin and urine copper/24 hours levels were recorded. Anterior segment parameters including steep and flat keratometry, corneal thickness at central, 2, 4, 6, 8 and 10 mm, anterior chamber angle width, volume and depth, corneal volume, pupillary diameter were evaluated by Scheimpflug imaging. Images of cornea were scored according to Kayser–Fleischer ring size. Results: Serum ceruloplasmin level was below 10 mg/dL in 17 patients and was 12, 18.5, 20, 22, 37 mg/dl in the remaining five patients. Urinary copper/24 hours was 249.55 ± 304.14 (23–1050) µg/day. Central corneal thickness and corneal thickness at 2 mm were statistically different ( p values 0.02, 0.04, respectively). Scheimpflug images apparently showed Kayser–Fleischer ring as a hyper-reflective band at the corneal endothelial surface. Kayser–Fleischer ring in 24 eyes was grade 1, 16 eyes were grade 2 and 4 eyes were grade 3. Conclusion: Scheimpflug imaging seems to be a helpful diagnostic tool for detecting and grading the Kayser–Fleischer ring. Corneal thickness in Wilson disease patients with Kayser–Fleischer ring tends to be higher, so that the possible affection in corneal thickness should be kept in mind for clinical evaluation of these patients.

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