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No matter how many patients we see, there are always those patients who make our work experience more interesting and keep us on our toes so that we don't become complacent. This series presents six case reports of patients who fit this category and who demonstrate how wide ranging the use of contact lens fitting can be.
In hospital practice, we are used to seeing atypical cases, but I want to start with two who were, in my experience, unique. In both cases, neither I nor the various other specialists who have treated them have seen another like them. I have been lucky enough to be able to follow them both over several years. The last four patients in the series show how dependent our patients are on their contact lenses.
Eye rubbing
IP was a young girl who first attended the hospital at the age of seven years. The referring doctor stated that she had an asymmetrical orbital appearance with hollowing above the right eyelid and a bluish discoloration and queried whether this was from repetitive eyelid rubbing leading to fat atrophy.
Her parents reported that she had rubbed the upper part of the right eye since birth. They were concerned that she had a six month history of darkening of the skin around her right eye and an apparent sunken appearance of the eye. The eye wasn't red or sticky, she had no history of atopy and on questioning said her eye did not itch. She just wanted to rub it. She was well and there was no family history of eye problems.
On examination, she was found to have equal size orbits and no other facial asymmetry. However, her acuity was R 1.04 logMAR and L 0.0. There was no movement on cover test and no proptosis. Corneas were clear and the bulbar conjunctiva showed no sign of allergy.
Refraction
R -3.75/ -7.75 x 15 0.94
L Plano
Ks R 7.02 x 9, 6.01 x 99
L 7.92 x 173, 7.71 x 83
(Regular mires in both eyes)
Her full spectacle prescription was ordered and her parents were advised to patch her left eye for five hours a day and to pad the right eye at night to prevent her from rubbing it. Mast cell stabilisers were prescribed three times a day.
The diagnosis was made of orbital fat atrophy, and keratoconus due to repetitive eye rubbing. She was referred for a craniofacial assessment. They excluded Romberg's disease (also known as progressive hemifacial atrophy) which in its early stages usually involves the skin and subcutaneous tissue and later may involve the facial muscles and skeleton.
She was seen theree months later. The mast cell stabilisers had made no difference and she was now rubbing her left eye at night as well as the right. Acuity was R 0.56 and L 0.0.
The right refraction continued to increase to -3.50/-9.50x10 by age seven and three-quarters. With this prescription and continued patching, the right VA improved to 0.24 but by then, the left vision had reduced to 0.14 unaided and both K readings had altered, R 6.88 x 10, 5.91 x 100, L 7.95 x 159, 7.44 x 69. Slit-lamp examination remained clear throughout. The appearance of both eyes now showed some reduction of the peri-orbital fat though the right eye was far worse than the left (Figure 1). Daytime patching was reduced to two hours a day and her parents were advised to pad both eyes at night to prevent rubbing.
By eight years, the left K readings had again steepened. Slit lamp examination showed corneal striae in the right and early epithelial changes in the left. Trial contact lenses were ordered for both eyes which she successfully wore for several months.
One year later, aged nine, she developed right hydrops and became so upset that for a time, she stopped rubbing her eyes. Three months later the hydrops was beginning to resolve and she had stopped all drops. The right VA was 6/38 at 1/3m and there was a dense central scar (Figure 2). This slowly improved and six months after the hydrops occurred, she was able to see 1.0. However, she was complaining that her vision was worse and her left eye was now only able to see 0.24 unaided.
Refraction
R -5.00/-10.00 x 160 (0.9)
L 0.0/ -4.50 x 75
with K readings
R 6.85 x 160, 6.30 x 70
L 7.85 x 164, 6.93 x 75
Pachymetry measurements revealed reduced central corneal thickness (R>L) of CCT R 0.513mm (off centre), L 0.543mm.
New contact lenses were ordered:
R Rose K 6.40/9.30/-13.00 (0.7)
L Rose K 7.30/9.30/-2.25 (-0.1)
She was now aged 10 years. The scarring from the hydrops was resolving but she continued to rub her eyes in spite of the bilateral night time patching and the scare she had had with the hydrops. Her right eye again required a steeper contact lens.
With no other suggestions for treatment, she was referred to the psychology department. This she duly attended but they could find no likely cause of the constant eye rubbing.
Nine months later, the scar in the right eye was much resolved (Figure 3). She had superficial punctuate epitheliopathy over scarred area and splits in Descemet's membrane but the vision had improved to 0.3 with a contact lens.
By the following year, at age 11 years, although she was doing well with her lenses and her vision was good, her parents reported that she still rubbed her eyes a lot even while wearing her rigid lenses. Even her classmates were on her case and told her to stop each time they saw her rubbing the eyes. The left eye needed a steeper lens - Rose K 7.20/9.30/0.00 (-0.1).
She continued thus for another year when it was decided to refer her for a trial of hypnosis. This appeared to help a little. However, it was not until six months later that the eye-rubbing finally started to lessen. Now aged 13, she had had her ears pierced and instead of eye rubbing, she now 'fiddled' with her diamante ear studs.
Over the course of the next year, her corneas continued to steepen and the lenses have been refitted. The right acuity is 0.0 and Left 0.06 logMAR. Surprisingly, the right eye sees better than the left.
She is now aged 14 years. She will not leave the house without her makeup and she has completely stopped rubbing her eyes as the makeup would be ruined were she to do so. With hindsight, had makeup been prescribed for her as a seven-year-old, she may have avoided the problems her eye rubbing has incurred.
? Dr Lynne Speedwell is principal optometrist at Great Ormond Street Hospital