Brown-McLean syndrome in long-term contact lens wearers

It is not uncommon to observe corneal changes in eyes that have undergone cataract extraction.
Basement membrane dystrophy is commonly seen, as are discrete areas of epithelial oedema around the limbus adjacent to a corneal section and in the region overlying the corneal section itself. However, in some cases, corneal oedema is seen in the periphery of the cornea in areas away from the corneal section.
This finding was first described by Brown and McLean in 1969. The syndrome they gave their name to is described as a rare condition1 (only 100 plus cases in the literature in the last 34 years). It is characterised as corneal oedema which involves the peripheral cornea from the limbus into the cornea for about 2-3mm but sparing the superior portion of the cornea between 10 and 2 o'clock. The oedema is said to start inferiorly and spread circumferentially, with other authors describing the whole of the corneal circumference being involved in severe cases.
Underlying this area of oedema is an orange-brown endothelial pigmentation. What is so interesting about this corneal oedema is that, despite the central cornea being prone to some degree of transient swelling, it is usually spared from permanent decompensation. It is known to occur following all types of cataract surgery, intracapsular, extracapsular or phakoemulsification and whether an intraocular implant has been inserted or not.
Further cases have been described in patients who have undergone other intraocular surgery such as pars plana lensectomies and vitrectomies and on the donor cornea of a patient who had undergone penetrating keratoplasty for bullous keratopathy following a previous intracapsular cataract extraction.2 It has even been described in cases where the patient has not undergone any intraocular surgery as in the case reported by Brown seen in a person with Marfan's syndrome.
Case presentation
Female, 79 years old: 2001
The patient was referred to the contact lens clinic from another hospital in the region. She had a past history of severe uveitis. She had undergone a right cataract extraction without an intraocular implant (date not known) and a left cataract extraction in 1984 using an extracapsular approach with a Pearce posterior chamber lens. The right eye had been corrected with a contact lens following cataract surgery.
Visual acuity in the right eye was 6/6 with a Persecon E 7.90/10.30 +7.25D lens from Ciba Vision. Left visual acuity was 6/36 due to chronic cystoid macular oedema following the surgery to this eye. The patient's IOPs were 18 mmHg right and left and the patient was on timoptol 0.25 per cent bd. The patient also used FML to the left eye when required. There was evidence of an attempted YAG capsulotomy in the left eye and the pupil was distorted.
The contact lens fit was found to be acceptable although the lens sat rather low and the inferior edge just crossed the limbus. Fluorescein showed a central pool and the lens condition was seen to be one of multiple scratches and some front surface drying. The patient was wearing the lens for 17 hours per day.
Slit lamp examination revealed an area of circumferential corneal epithelial oedema and bullae formation with underlying thickened stroma and folds at Descemet's level. This area covered the complete superior half of the cornea and was about 2.50mm wide from the limbus inwards (Figure 1). Centrally there was a hurricane keratopathy and some degree of hypoxia was noticed. Inferiorly in the region of normal cornea there was orange-brown pigmentation on the endothelium. However, no pigmentation was noticed underlying the peripheral corneal oedema at this stage.
Refraction revealed the following:

R +6.75DS (6/7.5); L -4.75/+2.75 x 147 (6/30) 'comes and goes'
Keratometry R 8.10 along 5, 7.95 along 112

At this point, a diagnosis of Brown-McLean syndrome was made.
The patient was given a spectacle prescription for the right aphakic eye and followed at regular intervals until July 2001 when it was felt that the cornea would benefit from a lens with a greater Dk. The lens was ordered from the department's usual rigid lens supplier, Jack Allen Contact Lenses London, to the prescription 7.95 CBAS 10.30 +7.50D material Dk100. The repeat lens fit was identical to the Persecon lens and VA was 6/6. Two months later, on review, the area of corneal oedema showed greater stromal thickness, with an increase in the epithelial component and for the first time some punctate staining was seen within this area. The patient was warned of the potential risk of infection and review date set for one month. At this visit the patient was still wearing the lens for 16-17 hours a day; VA was 6/6- but there was no sign of any further Hurricane keratopathy or central corneal oedema. A new finding however, was an increase in the endothelial pigmentation which was now visible underlying the oedematous area of the cornea. At this point, the patient was put on four-monthly review and has remained on this review period to date with no change in her corneal appearance or her ability to wear a contact lens on this eye.

Comments
This patient's presentation of Brown-McLean syndrome was somewhat unusual compared to that described as the classic appearance. In the first instance there was no sparing of the superior region of the cornea. While superior sparing does not always occur, it is usually associated with cases that involve the whole circumference of the cornea, whereas in this case this was clearly not so. Secondly there was no brown-orange pigment noticed underlying the area of oedema when the patient first presented, with this only appearing several months later at review. Despite these unusual differences there was no hesitation in diagnosing the condition given the location and appearance of corneal oedema.
Despite the striking appearance of corneal oedema (Figures 2 and 3), it has been said that there is no reason why these patients should not wear contact lenses successfully. This case study reviews such a patient who for many years remained undiagnosed and as such had little or no trouble. Within the contact lens department there are a number of patients who have been diagnosed with Brown-McLean syndrome with various degrees of expression. The syndrome probably goes unrecognised in its early stages and in mild cases may even be misdiagnosed. With such an advanced case, however, there is a risk of infection1 and it is important that the patient is fully informed of the condition and the possible implications and that follow-up is very regular. The transient nature of central corneal changes requires a degree of caution married to an understanding of the condition such that decisions on the contact lens fit are not made inappropriately. Ultimately, these patients can be managed with contact lens correction3 but it does require a truly combined effort on behalf of the practitioner and the patient.

References
1 Todd W Gothard et al. Clinical findings in Brown-McLean Syndrome. Am J Ophthalmol, 1993;115:729-737.
2 Sugar A. Brown-McLean syndrome occurring in a corneal graft. Cornea, 1997;16:493-494.
3 Tuft S J et al. Peripheral corneal oedema following cataract extraction (Brown-McLean syndrome). Eye, 1992; 6:502-505.

Steve Johns is principal optometrist at the North Devon District Hospital BarnstapleIt is not uncommon to observe corneal changes in eyes that have undergone cataract extraction.
Basement membrane dystrophy is commonly seen, as are discrete areas of epithelial oedema around the limbus adjacent to a corneal section and in the region overlying the corneal section itself. However, in some cases, corneal oedema is seen in the periphery of the cornea in areas away from the corneal section.
This finding was first described by Brown and McLean in 1969. The syndrome they gave their name to is described as a rare condition1 (only 100 plus cases in the literature in the last 34 years). It is characterised as corneal oedema which involves the peripheral cornea from the limbus into the cornea for about 2-3mm but sparing the superior portion of the cornea between 10 and 2 o'clock. The oedema is said to start inferiorly and spread circumferentially, with other authors describing the whole of the corneal circumference being involved in severe cases.
Underlying this area of oedema is an orange-brown endothelial pigmentation. What is so interesting about this corneal oedema is that, despite the central cornea being prone to some degree of transient swelling, it is usually spared from permanent decompensation. It is known to occur following all types of cataract surgery, intracapsular, extracapsular or phakoemulsification and whether an intraocular implant has been inserted or not.
Further cases have been described in patients who have undergone other intraocular surgery such as pars plana lensectomies and vitrectomies and on the donor cornea of a patient who had undergone penetrating keratoplasty for bullous keratopathy following a previous intracapsular cataract extraction.2 It has even been described in cases where the patient has not undergone any intraocular surgery as in the case reported by Brown seen in a person with Marfan's syndrome.
Case presentation
Female, 79 years old: 2001
The patient was referred to the contact lens clinic from another hospital in the region. She had a past history of severe uveitis. She had undergone a right cataract extraction without an intraocular implant (date not known) and a left cataract extraction in 1984 using an extracapsular approach with a Pearce posterior chamber lens. The right eye had been corrected with a contact lens following cataract surgery.
Visual acuity in the right eye was 6/6 with a Persecon E 7.90/10.30 +7.25D lens from Ciba Vision. Left visual acuity was 6/36 due to chronic cystoid macular oedema following the surgery to this eye. The patient's IOPs were 18 mmHg right and left and the patient was on timoptol 0.25 per cent bd. The patient also used FML to the left eye when required. There was evidence of an attempted YAG capsulotomy in the left eye and the pupil was distorted.
The contact lens fit was found to be acceptable although the lens sat rather low and the inferior edge just crossed the limbus. Fluorescein showed a central pool and the lens condition was seen to be one of multiple scratches and some front surface drying. The patient was wearing the lens for 17 hours per day.
Slit lamp examination revealed an area of circumferential corneal epithelial oedema and bullae formation with underlying thickened stroma and folds at Descemet's level. This area covered the complete superior half of the cornea and was about 2.50mm wide from the limbus inwards (Figure 1). Centrally there was a hurricane keratopathy and some degree of hypoxia was noticed. Inferiorly in the region of normal cornea there was orange-brown pigmentation on the endothelium. However, no pigmentation was noticed underlying the peripheral corneal oedema at this stage.
Refraction revealed the following:

R +6.75DS (6/7.5); L -4.75/+2.75 x 147 (6/30) 'comes and goes'
Keratometry R 8.10 along 5, 7.95 along 112

At this point, a diagnosis of Brown-McLean syndrome was made.
The patient was given a spectacle prescription for the right aphakic eye and followed at regular intervals until July 2001 when it was felt that the cornea would benefit from a lens with a greater Dk. The lens was ordered from the department's usual rigid lens supplier, Jack Allen Contact Lenses London, to the prescription 7.95 CBAS 10.30 +7.50D material Dk100. The repeat lens fit was identical to the Persecon lens and VA was 6/6. Two months later, on review, the area of corneal oedema showed greater stromal thickness, with an increase in the epithelial component and for the first time some punctate staining was seen within this area. The patient was warned of the potential risk of infection and review date set for one month. At this visit the patient was still wearing the lens for 16-17 hours a day; VA was 6/6- but there was no sign of any further Hurricane keratopathy or central corneal oedema. A new finding however, was an increase in the endothelial pigmentation which was now visible underlying the oedematous area of the cornea. At this point, the patient was put on four-monthly review and has remained on this review period to date with no change in her corneal appearance or her ability to wear a contact lens on this eye.

Comments
This patient's presentation of Brown-McLean syndrome was somewhat unusual compared to that described as the classic appearance. In the first instance there was no sparing of the superior region of the cornea. While superior sparing does not always occur, it is usually associated with cases that involve the whole circumference of the cornea, whereas in this case this was clearly not so. Secondly there was no brown-orange pigment noticed underlying the area of oedema when the patient first presented, with this only appearing several months later at review. Despite these unusual differences there was no hesitation in diagnosing the condition given the location and appearance of corneal oedema.
Despite the striking appearance of corneal oedema (Figures 2 and 3), it has been said that there is no reason why these patients should not wear contact lenses successfully. This case study reviews such a patient who for many years remained undiagnosed and as such had little or no trouble. Within the contact lens department there are a number of patients who have been diagnosed with Brown-McLean syndrome with various degrees of expression. The syndrome probably goes unrecognised in its early stages and in mild cases may even be misdiagnosed. With such an advanced case, however, there is a risk of infection1 and it is important that the patient is fully informed of the condition and the possible implications and that follow-up is very regular. The transient nature of central corneal changes requires a degree of caution married to an understanding of the condition such that decisions on the contact lens fit are not made inappropriately. Ultimately, these patients can be managed with contact lens correction3 but it does require a truly combined effort on behalf of the practitioner and the patient.

References
1 Todd W Gothard et al. Clinical findings in Brown-McLean Syndrome. Am J Ophthalmol, 1993;115:729-737.
2 Sugar A. Brown-McLean syndrome occurring in a corneal graft. Cornea, 1997;16:493-494.
3 Tuft S J et al. Peripheral corneal oedema following cataract extraction (Brown-McLean syndrome). Eye, 1992; 6:502-505.

Steve Johns is principal optometrist at the North Devon District Hospital Barnstaple