Sir,

Werner syndrome is a rare disorder characterized by premature ageing. It is associated with bilateral cataract. Impaired wound healing is a feature of this syndrome.1

We reported a female who had presented with this rare disorder and undertook lensectomy and subsequent bleb formation and blebitis several years later. Ultrasound biomicroscopy (UBM) was carried out to evaluate the aetiology of bleb formation.

Case report

A 28-year-old woman presented with a complaint of a red eye. She had a history of bilateral lensectomy through the scleral tunnel 10 years ago.

Best-corrected visual acuity (BCVA) was 20/200 in the involved eye, with an intra-ocular pressure (IOP) of 4 mm Hg. During slit-lamp examination it was discovered that there was a thin, cystic, white elevated conjunctival bleb with surrounding conjunctival injection (Figure 1). A diagnosis of spontaneous bleb formation after lensectomy with subsequent blebitis was made.

Figure 1
figure 1

Slit lamp photograph showing a thin cystic white elevated conjunctival bleb with surrounding conjunctival injection.

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She had a characteristic dermatological pathology (atrophic skin and subcutaneous atrophy) and characteristic bird-like facies of Werner syndrome (Figure 2). Her scalp hairs were thin and she had a high-pitched voice.

Figure 2
figure 2

Characteristic atrophic skin, wrinkles and characteristic bird facies of the patient.

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Ultrasound biomicroscopy was carried out and it showed the internal and external openings of the fistula and its tract (Figure 3). Blebitis was resolved with topical antibiotics.

Figure 3
figure 3

Ultrasound biomicroscopy of patients showing internal and external opening of the fistula and its tract.

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Comment

Werner syndrome is a rare autosomal recessive disorder characterized by many features of premature ageing, including scleroderma-like skin changes, premature greying of the scalp hair, bilateral cataracts, and some endocrinologic disorders. The syndrome is most common in patients of Japanese origin.2

Cataract surgery in Werner syndrome may be complicated by wound dehiscence or corneal endothelial decompensation. There are some reports of unplanned filtering bleb in these patients.3, 4

Two of 18 eyes from patients with Werner syndrome in Jonas's4 study were complicated with unplanned filtering bleb. He used extracapsular cataract surgery. However, Kocabora3 showed that Werner's syndrome cataracts can be safely managed with the current phacoemulsification and temporal clear corneal small-incision surgery techniques.

Ultrasound biomicroscopy has been shown to be useful in showing an aetiology of a spontaneous filtering bleb, such as Terrien's marginal degeneration and Axenfeld syndrome, after cyclophotocoagulation or sutured posterior chamber intraocular lenses.5 UBM might be helpful in differentiating a filtering bleb from a possible conjunctival cyst. It is also useful for surgical planning if deemed necessary.

Ophthalmologists should be careful with the creation of well-constructed wounds in Werner syndrome. By reporting this case, we also highlight the use of UBM to visualize and evaluate the fistula. We recommend the use of UBM for the early diagnosis of the aetiology of bleb and the position of the fistula in this syndrome. Moreover, emergent diagnosis and treatment of presumed blebitis is to be kept in mind.