Optometry in Practice Vol 7 (2006) 1722

Posterior Staphyloma: Can it be Retinal Detachment or Tumour?

Nonavinakere P Manjunatha1 MD MRCOphth (Lon), Shrivatsa P Desai1 MS FRCS, Aravind Reddy2 MS FRCS and Siddharth Goel1 MRCOphth (Lon) FRCS (Edn) FRCS (Gls)
1Doncaster

and Bassetlaw NHS Foundation Trust, UK Accepted for publication 23 January 2006

2Bradford

Teaching Hospitals NHS Foundation Trust, UK

Posterior staphyloma is often a feature of pathological myopia. The complex optics in high refractive error and the difficulties in viewing the fundus in myopic eyes can lead to a mistaken diagnosis of retinal detachment or tumour on routine examination. This can cause anxiety to patients. We report two such cases. Curtin (1977) suggested a classification for posterior staphyloma and we have used it in the description of our cases.

Case 2
A 72-year-old woman was referred urgently to the emergency eye clinic at Bradford Royal Infirmary by the optometrist who had noticed an elevated area in the right fundus and suspected choroidal melanoma. Once again it was a routine eye check-up and the patient was asymptomatic. There was a history of bilateral myopia and the right eye was amblyopic. On examination the patients best corrected visual acuity was 6/60 in the right eye and 6/6 in the left eye. The refraction was 7.00DS/2.00DC axis 135 in the right eye and 1.75DS in the left eye. Anterior segments of both eyes were unremarkable. Examination showed myopic fundal degeneration in both eyes but posterior staphyloma only in the right eye. It was of Curtin type V with an area of ectasia around the disc, extending 1 disc diameter above the disc to about 5 disc diameters inferiorly (Curtin 1977). No staphyloma was found in the other eye. Ultrasound B-scan of the right eye showed only staphyloma but no tumour (Figure 3). The axial lengths of the right and left eye were 29.3mm and 22.48mm respectively. The patient was reassured and discharged.

Case 1
A 45-year-old female was referred urgently by her optometrist with suspicion of bilateral retinal detachments to the emergency eye clinic at Doncaster Royal Infirmary. She had been for a routine check-up and was asymptomatic. There was a history of high myopia and in the past she had had right-eye squint surgery. The vision had been poor in both eyes since childhood. Her best corrected visual acuity was 6/24 in the right eye and 6/36 in the left eye. The refraction was 31.00DS/1.50DC axis 10 in the right eye and 31.00DS/2.00DC axis 180 in the left eye. Anterior segments of both eyes were unremarkable except for deep anterior chambers. Examination showed myopic degeneration in both eyes. Posterior staphyloma Curtin type VIII was seen in both eyes (Curtin 1977; Figure 1). There was an area of ectasia centred around the disc extending 4 disc diameters nasal to the optic nerve and temporally to within 1 disc diameter of the macula with single step along the nasal wall of the staphyloma. Neither retinal detachment nor retinal tears could be seen in either eye. The axial lengths were: right eye 35.79mm, left eye 35.55mm. Ultrasound B-scan confirmed the staphyloma and retinal detachment was ruled out (Figure 2). The patient was reassured and discharged back to the optician.

Discussion
The word myopia is derived from the ancient Greek myops, half-closing the eyes, a reference to the stenopaeic effect achieved by so doing. It is a refractive condition that results when the image of a distant object is focused in front of the retina by the relaxed eye. Myopia can be classified into simple myopia and pathological myopia. Simple myopia is the most common eye disorder worldwide. Pathological myopia, also called progressive myopia, involves structural alterations to the globe, which may threaten sight and ocular health. Pathological myopia is due to the development of structural defects in the posterior segment of the eye. It comprises 2% of all myopias. It is thought to result from a

Address for correspondence: Mr NP Manjunatha, Ophthalmology Department, Doncaster Royal Infirmary, Armthorpe Road, Doncaster, South Yorkshire, DN2 5LT, UK.

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NP Manjunatha, SP Desai, A Reddy & S Goel

combination of genetic and environmental factors (Grossniklaus & Green 1992). Pathological myopes may present with decreased visual acuity, strabismus, openangle glaucoma, premature lenticular opacification, and increased axial length. Features of myopic degeneration include one or more of the following: vitreous syneresis, tilted discs, posterior staphyloma, myopic crescent, patchy choroidal atrophy within the posterior pole, breaks in Bruchs membrane with accompanying choroidal atrophy known as lacquer cracks, subretinal neovascular membrane, Fuchs spots and retinal breaks or detachments. A staphyloma is a protrusion of the outer coat of the eye with incarceration of uveal tissue. It is derived from the Greek word which means a bunch of grapes. It can be classified anatomically into corneal, ciliary, intercalary, equatorial and posterior staphyloma (Duke-Elder 1965). A discussion on the first four is out of the scope of this article. A histopathological study (Grossniklaus & Green 1992) reported posterior staphyloma in 35.4% of pathological myopia eyes. The prevalence increases to 71.4% if axial length is 33.536.6mm (Curtin & Karlin 1970). There is a tendency for posterior staphyloma to expand and deepen with age (Curtin 1977). The posterior staphyloma is believed to result from slowly progressive chorioretinal stretching, mechanical tearing and atrophy (Albert & Jakobiec 1994). Sclera has also been shown to be abnormal in eyes with pathological myopia. There is thinning of scleral collagen bundles and decreased number of collagen striations (Curtin & Teng 1958). In 1977 Curtin proposed a classification and described 10 types of posterior staphyloma. These were classified according to the fundus area in which the ectasia was located. The types of staphyloma differed not only in the area of fundus involvement, but also in their size, shape and depth, the abruptness of their margins and the associated changes in the appearance of the optic nerve and retinal vessels. The first five varieties were called primary posterior staphylomas and the remaining five types compound staphylomas. In type I the area of ectasia is between 2 and 5 disc diameters nasal to the optic nerve and extending temporally to macula or several disc diameters beyond it. Type VIII is distinguished by the presence of tiers or steps across the wall of a primary type I staphyloma. These steps or terraces may be single or multiple and are almost invariably found along the nasal wall of the staphyloma. At these abrupt edges the retinal vessels sharply bend and disappear in the shadow. The deeper the staphyloma, the sharper or more abrupt are the margins (Curtin 1977). The ectatic areas appear relatively pale in comparison to the uninvolved areas of the fundus. All of the above features

can give the impression of a retinal detachment, as in our case no. 1. In type V primary staphyloma the ectatic area is inferior to the optic nerve (Curtin 1977). The posterior staphyloma is deepest at the disc and at the inferior edge the retina might look elevated, which mimics an elevated lesion or tumour, as happened in case no. 2. Myopes can have an increased risk of developing retinal detachment and the prevalence in pathological myopia is 11.4% (Curtin & Teng 1958). The retinal tear might be present at or originate from an area of posterior staphyloma and might lead to retinal detachment. But most of these patients are symptomatic (Phillips & Dobbie 1963). We have illustrated two different problems encountered in optometric practice worth taking note of. In symptomatic myopic patients retinal detachment should always be kept in mind while examining the fundus. Elevated retinal lesion even in an asymptomatic eye should be carefully examined to make a correct diagnosis and, if need be, referred to ophthalmologists for confirmation or exclusion of the initial diagnosis. The difficulties of visualisation of the fundus in patients with high refractive errors and the presence of staphyloma in pathological myopia can lead to presumptive diagnosis of retinal detachment or tumour, as illustrated by our two cases. A thorough assessment, including biomicroscopic fundus examination and biometry, can avoid needless anxiety and stress to patients.

Acknowledgement
We are grateful to Ms Laura Ferguson for photography.

References
Albert DM, Jakobiec FA (1994) Principles and Practice of Ophthalmology. Philadelphia: WB Saunders Curtin BJ (1977) The posterior staphyloma of pathologic myopia. Trans Am Ophthalmol Soc 75, 6784 Curtin BJ, Teng CL (1958) Scleral changes on pathological myopia. Trans Am Acad Ophthalmol Otolaryngol 62, 77790 Curtin BJ, Karlin DB (1970) Axial length measurements and fundus changes of the myopic eye. Part 1. The posterior fundus. Trans Am Ophthamol Soc 68, 31234 Duke-Elder S (1965) Systems of Ophthalmology, vol. 8 (part 2), pp 9989. London: Henry Kimpton Grossniklaus HE, Green WR (1992) Pathologic findings in pathologic myopia. Retina 12, 12733 Phillips CI, Dobbie JG (1963) Posterior staphyloma and retinal detachment. Am J Ophthalmol 55, 3325

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Posterior Staphyloma: Can it be Retinal Detachment or Tumour?

Figure 1 Fundus pictures showing posterior staphyloma in case no. 1. Left, Fundus picture of right eye. Right, fundus picture of left eye.

Figure 2 Ultrasound B-scan pictures showing posterior staphyloma in case no. 1. Left, ultrasound B-scan picture of right eye. Right, ultrasound B-scan picture of left eye.

Figure 3 Ultrasound picture of right eye showing posterior staphyloma with inferior elevated area in case no. 2.

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Comment
Manjunatha et al. present two cases of posterior staphyloma associated with pathological myopia presenting initially to the optometrist. Both cases were referred urgently and the diagnosis of posterior staphyloma was confirmed by B-scan ultrasound biometry. The authors describe the pathology of posterior staphyloma, describing it as an ectasia a thinning of scleral tissue. From the figures, it can be seen that the staphylomatous area is atrophic. This is due to the loss of the choriocapillaris and subsequent atrophy of the retinal pigment epithelium and photoreceptors. Staphyloma can also be complicated by breaks in Bruchs membrane that heal to form lacquer cracks, and, in some instances, subretinal neovascularisation and disciform scarring. Atrophy of photoreceptors of course leads to visual field defects. However, visual field defects are also associated with local detachments and with tumours. Visual field testing is also difficult in pathologically myopic eyes because of the reduced visual acuity and the constriction caused by high-minus corrections, so visual field testing is unlikely to help differentiate posterior staphylomas from tumours or retinal detachments. The authors suggest that detachments are usually symptomatic, which is a fair point, although not a universal rule, especially in an eye that may be amblyopic and suppressed. It is also worth pointing out that the authors were confident of their diagnosis only after performing B-scan ultrasound scans. Unfortunately, ultrasound scanners are not in the typical optometrists armamentarium and so prompt or urgent referral seems appropriate in these cases. The authors themselves concede that an ultrasound scan is indicated, even if the referral does have the unfortunate side-effect of causing needless anxiety and stress. However, if the optometrist includes posterior staphyloma in the differential diagnosis and tells the patient that this is a possibility (which should now be the case given this timely article!), fears can perhaps be allayed.

It is unclear from the report whether the optometrists used either fundus biomicroscopy (with eg a Volk lens) or headband indirect fundoscopy. The authors argue that stereoscopic fundus examination would have helped in these cases. I agree with this view, although again, one of the cases exhibited a convex fundal elevation rather than a concave depression. It would be difficult to rule out a tumour with confidence and maybe a detachment in such cases, so once more, prompt referral seems the appropriate course of action, at least for the case with a staphylomatous elevation. It is perhaps worth mentioning here that optometrists should bear in mind the useful sign of anterior vitreous tobacco dust as an indicator of a retinal tear (though not an exudative detachment). One issue that I would like to bring to optometrists attention is the appearance of the optic discs in these highly myopic eyes. Both eyes show tilted discs and peripapillary myopic degeneration, which makes them extremely difficult to assess, especially from the point of view of glaucoma. Given the difficulties associated with visual field testing discussed above, high myopes can be difficult to manage in this situation. Serial stereoscopic optic disc photography would be the ideal, but I would urge practitioners to have a good look using fundus biomicroscopy and at least to draw a diagram of the disc, paying particular attention to where the disc stops and the myopic degeneration starts, as well as the contour of any cupping. This is an interesting article and the authors are to be congratulated for reminding practising optometrists of this entity. I hope you enjoyed reading it as much as I did.

Dr Russell J. Watkins Specialist Registrar in Histopathology Leeds General Infirmary

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Posterior Staphyloma: Can it be Retinal Detachment or Tumour?

Multiple Choice Questions

This paper is reference C-3114. One credit is available. Please use the inserted answer sheet. Copies can be obtained from Optometry in Practice Administration, PO Box 6, Skelmersdale, Lancashire WN8 9FW. There is only one correct answer for each question.

1. (a) (b) (c) (d) 2.

Which of the following is a posterior staphyloma often mistakenly diagnosed as? optic atrophy retinal detachment advanced glaucomatous optic nerve head damage all of the above Which of the following tests is routinely employed in a hospital eye department to confirm or exclude a diagnosis of posterior staphyloma? direct ophthalmoscopy gonioscopy ultrasound b-scan pachymetry Which of the following statements is true regarding pathological myopia? it comprises 2% of all myopias it can present with early cataracts choroidal atrophy is common all of the above

4. (a) (b) (c)

How is type VIII staphyloma distinguished from type I? the presence of an associated retinal break the ectatic area is inferior to the optic nerve the presence of tiers or steps along the wall of the staphyloma (d) an axial length of >29.3mm as measured by ultrasound b-scan 5. (a) (b) (c) (d) 6. Which of the following statements is true regarding retinal detachments in pathological myopes? a tear can originate from an area of posterior staphyloma there is no increased risk of retinal detachment in pathological myopes most of these patients are asymptomatic the prevalence is <10% What proportion of pathological myopic eyes were reported to have staphylomas in a histopathological study? <20% between 20% and 30% between 30% and 40% >40%

(a) (b) (c) (d) 3. (a) (b) (c) (d)

(a) (b) (c) (d)

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Optometry in Practice Vol 7 (2006) 2334

Recent Changes in Medicines Legislation That Affect Optometrists

Lucy C Titcomb1 BSc MRPharmS MCPP and John G Lawrenson2 BSc PhD MCOptom
1Birmingham

& Midland Eye Centre, UK 2Department of Optometry and Visual Science, City University, London, UK Accepted for publication 26 January 2006

The rules governing the use and supply of medicines in the UK are laid down in the Medicines Act (1968). Optometrists have traditionally been granted exemptions from the general rules laid down in the Act to allow them to use certain prescription-only medicines (POMs) in the course of their professional practice and in particular circumstances to supply POMs to their patients. The list of drugs to which exemptions apply has changed little in the years since the establishment of the Medicines Act; however, in 2005 significant changes to medicines legislation occurred. These changes included: an update to the list of POMs available to all registered optometrists. a relaxation of the rules governing the supply of pharmacy (P) and general sales list (GSL) medicines by optometrists the establishment of a list of further POMs that can be used and supplied by optometrists who have completed extended training In parallel with these changes, a further development arose out of a wider National Health Service (NHS) policy initiative to extend the authority to prescribe to nonmedical healthcare professionals. The rationale underpinning this policy was described in the NHS Plan (2000) and included: (1) improving patient access to medicines; (2) breaking down traditional demarcations between clinical roles; and (3) making better use of the knowledge and clinical skills of non-medical healthcare professionals. Two distinct categories of prescriber have been defined in legislation: 1. independent prescribers, who are responsible for assessing the patient, making a diagnosis and drawing up a clinical management plan 2. supplementary prescribers, who have the authority to prescribe within the parameters of an agreed management plan for patients whose condition has been diagnosed by an independent prescriber

Nurses and pharmacists were the first healthcare professionals to train as supplementary prescribers and in 2005 this opportunity was extended to other professional groups, including optometrists. This article will summarise these changes to medicines legislation and discuss their implications for the care that optometrists can provide to their patients.

Update to the List of POMs Available to All Registered Optometrists

The list of POMs available to optometrists (Table 1) has only been updated sporadically in the years since it was first established. Since the last revision took place in 1978, the list has been in need of major review. Several drugs in the list, for example, the antimuscarinic hyoscine, the non-steroidal anti-inflammatory drug oxyphenbutazone and the miotics bethanechol, neostigmine and physostigmine, were no longer commercially available as ophthalmic formulations in the UK. In the case of antimicrobial preparations, chloramphenicol was the only POM available to optometrists for treating superficial infections of the eye or as prophylaxis following ocular surface injury. Although framycetin sulphate was listed, it was of little clinical value as its use was restricted to administration only. The legal process for amending POMs available to professional groups through exemptions involves a period of public consultation that is administered by the Medicines and Healthcare Products Regulatory Agency (MHRA), followed by consideration by the Committee for the Safety of Medicines (CSM). The consultation document MLX 306 summarised the rationale for updating the list and proposed removing framycetin and adding the antibacterial agent fusidic acid to the POMs available to optometrists for sale and supply. The outcome of this process was the establishment of an updated list which was embodied in a new statutory instrument (SI 2005 766) which came into force on 7 April 2005 (Table 2).

Address for correspondence: Mrs LC Titcomb, Pharmacy Department, Birmingham & Midland Eye Centre, Sandwell & West Birmingahm NHS Trust, Dudley Road, Birmingham B18 7QH, UK

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